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Vulvar Cancer

There are multiple different types of malignancies that can affect Affect The feeling-tone accompaniment of an idea or mental representation. It is the most direct psychic derivative of instinct and the psychic representative of the various bodily changes by means of which instincts manifest themselves. Psychiatric Assessment the vulva Vulva The vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy. The most common histologic type is squamous cell carcinoma Squamous cell carcinoma Cutaneous squamous cell carcinoma (cSCC) is caused by malignant proliferation of atypical keratinocytes. This condition is the 2nd most common skin malignancy and usually affects sun-exposed areas of fair-skinned patients. The cancer presents as a firm, erythematous, keratotic plaque or papule. Squamous Cell Carcinoma (SCC) (SCC), which accounts for approximately 75%–85% of all vulvar cancers. Other types include melanoma Melanoma Melanoma is a malignant tumor arising from melanocytes, the melanin-producing cells of the epidermis. These tumors are most common in fair-skinned individuals with a history of excessive sun exposure and sunburns. Melanoma, basal cell Basal Cell Erythema Multiforme carcinoma, sarcoma, malignancy Malignancy Hemothorax of the Bartholin glands Bartholin glands Mucus-secreting glands situated on the posterior and lateral aspect of the vestibule of the vagina. Vagina, Vulva, and Pelvic Floor: Anatomy, and Paget disease of the vulva Vulva The vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy (an adenocarcinoma). Squamous cell carcinoma is typically associated with either high-risk HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV) infection or lichen sclerosus Lichen Sclerosus Atrophy and shriveling of the skin of the vulva that is characterized by the whitish lichen sclerosus appearance, inflammation, and pruritus. Benign Vulvar Conditions. Vulvar cancer presents as vulvar lesions that can have a variety of appearances, which may include warty or nodular masses, scaly plaques, pigmented lesions, and ulcers; pruritus Pruritus An intense itching sensation that produces the urge to rub or scratch the skin to obtain relief. Atopic Dermatitis (Eczema) is also common. Diagnosis usually requires a biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma, and management is primarily with surgical excision. Unfortunately, lymph Lymph The interstitial fluid that is in the lymphatic system. Secondary Lymphatic Organs node metastasis Metastasis The transfer of a neoplasm from one organ or part of the body to another remote from the primary site. Grading, Staging, and Metastasis occurs early in the natural history of the disease and is associated with a poor prognosis Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Non-Hodgkin Lymphomas.

Last updated: 21 Sep, 2021

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Overview

Definition

Vulvar cancer is a malignancy Malignancy Hemothorax of the vulva Vulva The vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy, which includes structures of the external female genitalia: 

Epidemiology

Vulvar cancer is a rare cancer that constitutes only 0.3% of new cancer diagnoses.

  • Lifetime risk: 0.3%
  • Squamous cell carcinoma Squamous cell carcinoma Cutaneous squamous cell carcinoma (cSCC) is caused by malignant proliferation of atypical keratinocytes. This condition is the 2nd most common skin malignancy and usually affects sun-exposed areas of fair-skinned patients. The cancer presents as a firm, erythematous, keratotic plaque or papule. Squamous Cell Carcinoma (SCC) (SCC) is the most common histologic subtype: approximately 75%–85%
  • Incidence Incidence The number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time. Measures of Disease Frequency: approximately 5000–10,000 cases per year in the United States
  • Mortality Mortality All deaths reported in a given population. Measures of Health Status: approximately 1000–2000 deaths per year in the United States
  • Age: 
    • Typically diagnosed at 55–85 years of age
    • Most common age group affected: 65–75 years
  • Ethnicity: more common among White women

Risk factors

  • HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV) infection with high-risk subtypes:
    • Most important risk factor for SCC
    • High-risk HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV) subtypes: 16, 18, 31, 33, and 45
    • Approximately 70%–75% of HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV)-associated SCCs are HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV)-16.
  • Increasing age
  • Smoking Smoking Willful or deliberate act of inhaling and exhaling smoke from burning substances or agents held by hand. Interstitial Lung Diseases 
  • Lichen sclerosus Lichen Sclerosus Atrophy and shriveling of the skin of the vulva that is characterized by the whitish lichen sclerosus appearance, inflammation, and pruritus. Benign Vulvar Conditions (key risk factor for non– HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV)-associated SCC)
  • Squamous cell hyperplasia Hyperplasia An increase in the number of cells in a tissue or organ without tumor formation. It differs from hypertrophy, which is an increase in bulk without an increase in the number of cells. Cellular Adaptation
  • Prior pelvic radiation Radiation Emission or propagation of acoustic waves (sound), electromagnetic energy waves (such as light; radio waves; gamma rays; or x-rays), or a stream of subatomic particles (such as electrons; neutrons; protons; or alpha particles). Osteosarcoma
  • Immunodeficiency Immunodeficiency Chédiak-Higashi Syndrome

Classification

Classification by histologic subtype

Histologic subtypes of vulvar cancer include:

  • Squamous cell carcinoma Squamous cell carcinoma Cutaneous squamous cell carcinoma (cSCC) is caused by malignant proliferation of atypical keratinocytes. This condition is the 2nd most common skin malignancy and usually affects sun-exposed areas of fair-skinned patients. The cancer presents as a firm, erythematous, keratotic plaque or papule. Squamous Cell Carcinoma (SCC) (75%–85%): 
    • HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV)-associated (warty type)
    • Non– HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV)-associated (keratinized type)
  • Melanoma Melanoma Melanoma is a malignant tumor arising from melanocytes, the melanin-producing cells of the epidermis. These tumors are most common in fair-skinned individuals with a history of excessive sun exposure and sunburns. Melanoma (approximately 5%–10%)
  • Basal cell Basal Cell Erythema Multiforme carcinoma (approximately 2%–8%)
  • Sarcomas (approximately 2%): soft tissue Soft Tissue Soft Tissue Abscess malignancies 
    • Leiomyosarcomas: malignancies of smooth muscle tissue
    • Rhabdomyosarcomas: malignancies of skeletal muscle tissue
    • Liposarcomas: malignancies of adipose tissue Adipose tissue Adipose tissue is a specialized type of connective tissue that has both structural and highly complex metabolic functions, including energy storage, glucose homeostasis, and a multitude of endocrine capabilities. There are three types of adipose tissue, white adipose tissue, brown adipose tissue, and beige or “brite” adipose tissue, which is a transitional form. Adipose Tissue: Histology
    • Angiosarcoma: malignancies of blood and lymphatic vessels Lymphatic Vessels Tubular vessels that are involved in the transport of lymph and lymphocytes. Lymphatic Drainage System: Anatomy
    • Neurofibrosarcomas: malignancies of neural tissue
  • Adenocarcinoma or carcinoma of the Bartholin glands Bartholin glands Mucus-secreting glands situated on the posterior and lateral aspect of the vestibule of the vagina. Vagina, Vulva, and Pelvic Floor: Anatomy (approximately 1%)
  • Paget disease of the vulva Vulva The vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy (an adenocarcinoma originating from glandular cells, < 1%)

Staging Staging Methods which attempt to express in replicable terms the extent of the neoplasm in the patient. Grading, Staging, and Metastasis

Vulvar cancer is staged on the basis of the size and location of the tumor Tumor Inflammation, regional lymph Lymph The interstitial fluid that is in the lymphatic system. Secondary Lymphatic Organs node involvement, and the presence of metastasis Metastasis The transfer of a neoplasm from one organ or part of the body to another remote from the primary site. Grading, Staging, and Metastasis.

  • Stage I: 
  • Stage II: 
    • Tumor with extension Extension Examination of the Upper Limbs to adjacent perineal structures, such as:
      • Lower urethra Urethra A tube that transports urine from the urinary bladder to the outside of the body in both the sexes. It also has a reproductive function in the male by providing a passage for sperm. Urinary Tract: Anatomy
      • Lower vagina Vagina The vagina is the female genital canal, extending from the vulva externally to the cervix uteri internally. The structures have sexual, reproductive, and urinary functions and a rich blood supply, mainly arising from the internal iliac artery. Vagina, Vulva, and Pelvic Floor: Anatomy
      • Anus
    • No lymph Lymph The interstitial fluid that is in the lymphatic system. Secondary Lymphatic Organs node involvement
  • Stage III:
  • Stage IV, which may be defined by the presence of any of the following:
    • Tumor invades to other regions, which may include:
      • Upper urethra Urethra A tube that transports urine from the urinary bladder to the outside of the body in both the sexes. It also has a reproductive function in the male by providing a passage for sperm. Urinary Tract: Anatomy
      • Upper vagina Vagina The vagina is the female genital canal, extending from the vulva externally to the cervix uteri internally. The structures have sexual, reproductive, and urinary functions and a rich blood supply, mainly arising from the internal iliac artery. Vagina, Vulva, and Pelvic Floor: Anatomy
      • Bladder Bladder A musculomembranous sac along the urinary tract. Urine flows from the kidneys into the bladder via the ureters, and is held there until urination. Pyelonephritis and Perinephric Abscess
      • Rectum Rectum The rectum and anal canal are the most terminal parts of the lower GI tract/large intestine that form a functional unit and control defecation. Fecal continence is maintained by several important anatomic structures including rectal folds, anal valves, the sling-like puborectalis muscle, and internal and external anal sphincters. Rectum and Anal Canal: Anatomy
      • Pelvic bone Bone Bone is a compact type of hardened connective tissue composed of bone cells, membranes, an extracellular mineralized matrix, and central bone marrow. The 2 primary types of bone are compact and spongy. Bones: Structure and Types
    • Any distant metastasis Metastasis The transfer of a neoplasm from one organ or part of the body to another remote from the primary site. Grading, Staging, and Metastasis
    • Metastasis to beyond local lymph nodes Lymph Nodes They are oval or bean shaped bodies (1 – 30 mm in diameter) located along the lymphatic system. Lymphatic Drainage System: Anatomy (e.g., pelvic nodes)

Pathophysiology

Vulvar cancer develops when there is uncontrolled cellular proliferation in vulvar tissue. This cancer may progress from a premalignant lesion caused by an HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV) infection, or it may develop from other mutations, unrelated to HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV).

Vulvar squamous intraepithelial lesions (SILs)

Vulvar SILs are abnormalities of the squamous epithelium Epithelium The epithelium is a complex of specialized cellular organizations arranged into sheets and lining cavities and covering the surfaces of the body. The cells exhibit polarity, having an apical and a basal pole. Structures important for the epithelial integrity and function involve the basement membrane, the semipermeable sheet on which the cells rest, and interdigitations, as well as cellular junctions. Surface Epithelium: Histology. Previously referred to as vulvar intraepithelial neoplasia (VIN), the preferred term is now SIL, which can be classified as:

  • Low-grade SIL (LSIL): 
    • Previously referred to as VIN1
    • Not considered a premalignant lesion (no significant risk of progression to vulvar cancer)
    • Generally associated with low-risk HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV) types (6, 11) that cause genital warts Warts Benign epidermal proliferations or tumors; some are viral in origin. Female Genitourinary Examination
    • Lesions are typically flat condylomas.
  • High-grade SIL (HSIL):
    • Previously referred to as VIN2 and VIN3
    • Premalignant lesions 
    • Significant risk of progression to SCC if untreated
    • Generally associated with high-risk HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV) types (16 (especially), 18, 31, 33, and 45)
    • High association with concurrent cervical intraepithelial neoplasia ( CIN CIN An increased tendency to acquire chromosome aberrations when various processes involved in chromosome replication, repair, or segregation are dysfunctional. Colorectal Cancer)
  • Differentiated VIN (dVIN):
    • Previously referred to as VIN simplex type
    • Premalignant lesions
    • Not associated with HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV) infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease
    • Refers to vulvar changes associated with vulvar dermatoses (primarily lichen sclerosus)
Low-grade sil (lsil)

Low-grade squamous intraepithelial lesion

Image: “Vulvar intra-epithelial neoplasia (VIN grade I)” by Kotsopoulos IC IC Inhaled Anesthetics, et al AL Amyloidosis. License: CC BY 2.0

HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV)-associated squamous cell carcinoma vulvar cancer

HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV)-associated SCC vulvar cancer is most commonly due to HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV)-16. HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV) has the ability to affect Affect The feeling-tone accompaniment of an idea or mental representation. It is the most direct psychic derivative of instinct and the psychic representative of the various bodily changes by means of which instincts manifest themselves. Psychiatric Assessment host cell protein expression:

  • HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV) has 2 major oncoproteins:
  • After cells lose tumor Tumor Inflammation suppressor proteins Proteins Linear polypeptides that are synthesized on ribosomes and may be further modified, crosslinked, cleaved, or assembled into complex proteins with several subunits. The specific sequence of amino acids determines the shape the polypeptide will take, during protein folding, and the function of the protein. Energy Homeostasis → unregulated proliferation → HSIL
  • Invasion through the basement membrane Basement membrane A darkly stained mat-like extracellular matrix (ecm) that separates cell layers, such as epithelium from endothelium or a layer of connective tissue. The ecm layer that supports an overlying epithelium or endothelium is called basal lamina. Basement membrane (bm) can be formed by the fusion of either two adjacent basal laminae or a basal lamina with an adjacent reticular lamina of connective tissue. Bm, composed mainly of type IV collagen; glycoprotein laminin; and proteoglycan, provides barriers as well as channels between interacting cell layers. Thin Basement Membrane Nephropathy (TBMN) → SCC

Non– HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV)-associated squamous cell carcinoma vulvar cancer

Non– HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV)-associated SCC vulvar cancer most commonly occurs in the setting of lichen sclerosus.

  • Inflammation or autoimmunity Autoimmunity Autoimmunity is a pathologic immune response toward self-antigens, resulting from a combination of factors: immunologic, genetic, and environmental. The immune system is equipped with self-tolerance, allowing immune cells such as T cells and B cells to recognize self-antigens and to not mount a reaction against them. Defects in this mechanism, along with environmental triggers (such as infections) and genetic susceptibility factors (most notable of which are the HLA genes) can lead to autoimmune diseases. Autoimmunity leads to the loss of cyclin-dependent kinase 2A (p16) → unregulated proliferation → dVIN
  • P53 remains intact
  • Invasion through basement membrane Basement membrane A darkly stained mat-like extracellular matrix (ecm) that separates cell layers, such as epithelium from endothelium or a layer of connective tissue. The ecm layer that supports an overlying epithelium or endothelium is called basal lamina. Basement membrane (bm) can be formed by the fusion of either two adjacent basal laminae or a basal lamina with an adjacent reticular lamina of connective tissue. Bm, composed mainly of type IV collagen; glycoprotein laminin; and proteoglycan, provides barriers as well as channels between interacting cell layers. Thin Basement Membrane Nephropathy (TBMN) → SCC

Patterns of spread

  • Growth of primary tumor Tumor Inflammation
  • Extension to surrounding organs
  • Embolization Embolization A method of hemostasis utilizing various agents such as gelfoam, silastic, metal, glass, or plastic pellets, autologous clot, fat, and muscle as emboli. It has been used in the treatment of spinal cord and intracranial arteriovenous malformations, renal arteriovenous fistulas, gastrointestinal bleeding, epistaxis, hypersplenism, certain highly vascular tumors, traumatic rupture of blood vessels, and control of operative hemorrhage. Gastrointestinal Bleeding into regional lymph nodes Lymph Nodes They are oval or bean shaped bodies (1 – 30 mm in diameter) located along the lymphatic system. Lymphatic Drainage System: Anatomy
  • Dissemination via hematologic and lymphatic vessels Lymphatic Vessels Tubular vessels that are involved in the transport of lymph and lymphocytes. Lymphatic Drainage System: Anatomy
Lymphatic drainage of the vulva

Lymphatic drainage of the vulva Vulva The vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy

Image by Lecturio.

Clinical Presentation

Vulvar cancer is often asymptomatic in its early stages. Early symptoms tend to be mild and are easily overlooked by the woman and/or her physician.

Vulvar squamous cell carcinoma

  • Unifocal Unifocal Retinoblastoma lesion (most commonly on the labia majora)
  • Lesions may have a variety of appearances, including:
    • Fleshy, nodular, or warty masses
    • Ulcerated
    • Scaly patches Patches Vitiligo or plaques
  • Pruritus
  • Burning sensation
  • Vulvar bleeding
  • Lymphadenopathy Lymphadenopathy Lymphadenopathy is lymph node enlargement (> 1 cm) and is benign and self-limited in most patients. Etiologies include malignancy, infection, and autoimmune disorders, as well as iatrogenic causes such as the use of certain medications. Generalized lymphadenopathy often indicates underlying systemic disease. Lymphadenopathy in the groin Groin The external junctural region between the lower part of the abdomen and the thigh. Male Genitourinary Examination (may be present even with small vulvar lesions)
  • Symptoms suggestive of advanced disease:
Squamous cell carcinoma of the vulva

Squamous cell carcinoma Squamous cell carcinoma Cutaneous squamous cell carcinoma (cSCC) is caused by malignant proliferation of atypical keratinocytes. This condition is the 2nd most common skin malignancy and usually affects sun-exposed areas of fair-skinned patients. The cancer presents as a firm, erythematous, keratotic plaque or papule. Squamous Cell Carcinoma (SCC) of the vulva Vulva The vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy immediately prior to surgical resection:
Planned surgical resection margins are marked.

Image: “Microinvasive squamous cell carcinoma of the vulva Vulva The vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy” by Sidor J, Diallo-Danebrock R, Eltze E, Lellé RJ. License: CC BY 2.0

Findings suggestive of non–squamous cell carcinoma vulvar cancers

  • Basal cell Basal Cell Erythema Multiforme carcinoma:
  • Melanoma Melanoma Melanoma is a malignant tumor arising from melanocytes, the melanin-producing cells of the epidermis. These tumors are most common in fair-skinned individuals with a history of excessive sun exposure and sunburns. Melanoma:
    • Usually a pigmented lesion
    • Characteristics similar to nonvulvar melanoma (ABCDEs):
      • Asymmetric
      • Irregular Borders
      • Black Color
      • Diameter > 6  mm MM Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma
      • Evolving/elevation
  • Paget disease:
  • Bartholin gland carcinomas:
Paget disease of the vulva

Paget disease of the vulva Vulva The vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy (also known as extramammary Paget disease):
Note the firm red mass on an eczematoid erythema Erythema Redness of the skin produced by congestion of the capillaries. This condition may result from a variety of disease processes. Chalazion covering the mons pubis.

Image: “The clinical features of the patient with EMPD.” by Wang X, Yang W, Yang J. License: CC BY 2.0

Diagnosis

Vulvar cancer is a histologic diagnosis. Once the diagnosis is established, staging requires a combination of clinical and surgical assessments. 

Examination

  • Pelvic exam:
    • Identify any abnormal masses or lesions that should be biopsied.
    • Measure the size of any lesions.
    • Palpate lymph nodes Lymph Nodes They are oval or bean shaped bodies (1 – 30 mm in diameter) located along the lymphatic system. Lymphatic Drainage System: Anatomy for lymphadenopathy.
    • Pap smear Pap smear Cytological preparation of cells collected from a mucosal surface and stained with Papanicolaou stain. Cervical Cancer Screening: should be up-to-date per screening Screening Preoperative Care guidelines owing to high rate of concurrent cervical pathology
  • Colposcopy Colposcopy The examination, therapy or surgery of the cervix and vagina by means of a specially designed endoscope introduced vaginally. Cervical Cancer Screening: 
    • Performed on the vulva Vulva The vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy, vagina Vagina The vagina is the female genital canal, extending from the vulva externally to the cervix uteri internally. The structures have sexual, reproductive, and urinary functions and a rich blood supply, mainly arising from the internal iliac artery. Vagina, Vulva, and Pelvic Floor: Anatomy, and cervix Cervix The uterus, cervix, and fallopian tubes are part of the internal female reproductive system. The most inferior portion of the uterus is the cervix, which connects the uterine cavity to the vagina. Externally, the cervix is lined by stratified squamous cells; however, the cervical canal is lined by columnar epithelium. Uterus, Cervix, and Fallopian Tubes: Anatomy
    • Soak the vulva Vulva The vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy and perianal skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Skin: Structure and Functions, vagina Vagina The vagina is the female genital canal, extending from the vulva externally to the cervix uteri internally. The structures have sexual, reproductive, and urinary functions and a rich blood supply, mainly arising from the internal iliac artery. Vagina, Vulva, and Pelvic Floor: Anatomy, and cervix Cervix The uterus, cervix, and fallopian tubes are part of the internal female reproductive system. The most inferior portion of the uterus is the cervix, which connects the uterine cavity to the vagina. Externally, the cervix is lined by stratified squamous cells; however, the cervical canal is lined by columnar epithelium. Uterus, Cervix, and Fallopian Tubes: Anatomy in acetic acid and examine under magnification with a standard colposcope Colposcope Instruments inserted into the vagina for examination of the tissues of the vagina and cervix by means of a magnifying lens. Diagnostic Procedures in Gynecology.
    • Allows for identification Identification Defense Mechanisms of:
      • Acetowhite changes (abnormalities) 
      • Abnormal vascular patterns
  • Cystoscopy: if there is concern for bladder involvement
  • Proctoscopy: if there is concern for rectal involvement

Biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma

  • Should be obtained for any suspicious lesions because vulvar cancer can have a wide range of appearances
  • Indications:
    • Masses
    • Persistent ulceration Ulceration Corneal Abrasions, Erosion, and Ulcers or pruritic area
    • Change in skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Skin: Structure and Functions color or elevation
  • Typically obtained with an in-office punch biopsy Punch Biopsy Actinic Keratosis
  • Used to:
    • Confirm cancer diagnosis (rule out benign pathology such as lichen simplex chronicus Lichen Simplex Chronicus A benign vulvar skin disorder characterized by hyperkeratosis (thickening of the skin) that occurs secondary to chronic vulvar irritation Benign Vulvar Conditions)
    • Determine the histologic type of cancer
    • Assess depth of invasion

Imaging

Imaging of the abdominopelvic and/or thoracic cavities is often indicated to complement the physical exam and assist in staging and surgical planning.

  • Can assess:
    • Tumor size
    • Extent of local progression
    • Enlarged lymph nodes Lymph Nodes They are oval or bean shaped bodies (1 – 30 mm in diameter) located along the lymphatic system. Lymphatic Drainage System: Anatomy
    • Liver Liver The liver is the largest gland in the human body. The liver is found in the superior right quadrant of the abdomen and weighs approximately 1.5 kilograms. Its main functions are detoxification, metabolism, nutrient storage (e.g., iron and vitamins), synthesis of coagulation factors, formation of bile, filtration, and storage of blood. Liver: Anatomy or lung metastasis Metastasis The transfer of a neoplasm from one organ or part of the body to another remote from the primary site. Grading, Staging, and Metastasis
  • Indications:
    • Symptoms suggestive of metastases (e.g., bowel or bladder dysfunction)
    • Tumors ≥ 4 cm
    • Clinically palpable lymph nodes Lymph Nodes They are oval or bean shaped bodies (1 – 30 mm in diameter) located along the lymphatic system. Lymphatic Drainage System: Anatomy
    • Individuals who are not candidates for surgery
  • Imaging methods:
    • Pelvic MRI
    • PET PET An imaging technique that combines a positron-emission tomography (PET) scanner and a ct X ray scanner. This establishes a precise anatomic localization in the same session. Nuclear Imaging/CT
    • Chest radiography
Ct images of the pelvis showing an enlarged left inguinal lymph node

Left: CT image of the pelvis Pelvis The pelvis consists of the bony pelvic girdle, the muscular and ligamentous pelvic floor, and the pelvic cavity, which contains viscera, vessels, and multiple nerves and muscles. The pelvic girdle, composed of 2 “hip” bones and the sacrum, is a ring-like bony structure of the axial skeleton that links the vertebral column with the lower extremities. Pelvis: Anatomy showing an enlarged left inguinal lymph Lymph The interstitial fluid that is in the lymphatic system. Secondary Lymphatic Organs node
Right: SPECT SPECT An imaging technique using a device which combines tomography, emission-computed, single-photon and tomography, x-ray computed in the same session. Nuclear Imaging scan showing focal tracer uptake in the left inguinal lymph Lymph The interstitial fluid that is in the lymphatic system. Secondary Lymphatic Organs node
Also note the intense tracer activity in the vulva Vulva The vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy (site of injection).

Image: “Hybrid SPECT SPECT An imaging technique using a device which combines tomography, emission-computed, single-photon and tomography, x-ray computed in the same session. Nuclear Imaging/CT images of the pelvis Pelvis The pelvis consists of the bony pelvic girdle, the muscular and ligamentous pelvic floor, and the pelvic cavity, which contains viscera, vessels, and multiple nerves and muscles. The pelvic girdle, composed of 2 “hip” bones and the sacrum, is a ring-like bony structure of the axial skeleton that links the vertebral column with the lower extremities. Pelvis: Anatomy” by Balasubramanian Harisankar CN, Mittal BR, Bhattacharya A, Dhaliwal LK. License: CC BY 2.5

Management and Prognosis

Vulvar cancer is treated primarily with surgery, though a combination of surgery, radiation Radiation Emission or propagation of acoustic waves (sound), electromagnetic energy waves (such as light; radio waves; gamma rays; or x-rays), or a stream of subatomic particles (such as electrons; neutrons; protons; or alpha particles). Osteosarcoma, and chemotherapy Chemotherapy Osteosarcoma is frequently used. The specific treatment plan depends on the individual’s disease and comorbidities Comorbidities The presence of co-existing or additional diseases with reference to an initial diagnosis or with reference to the index condition that is the subject of study. Comorbidity may affect the ability of affected individuals to function and also their survival; it may be used as a prognostic indicator for length of hospital stay, cost factors, and outcome or survival. St. Louis Encephalitis Virus.

Surgical management

  • Can include:
    • Excision of the primary lesion:
      • Wide local excision (stage IA and vulvar HSIL)
      • Modified radical vulvectomy: may be partial (i.e., hemivulvectomy) or complete
    • Lymphadenectomy (typically abbreviated LND for “ lymph Lymph The interstitial fluid that is in the lymphatic system. Secondary Lymphatic Organs node dissection”)
    • Debulking procedures
  • Surgery is tailored to the individual.

Chemoradiation and adjuvant Adjuvant Substances that augment, stimulate, activate, potentiate, or modulate the immune response at either the cellular or humoral level. The classical agents (freund’s adjuvant, bcg, corynebacterium parvum, et al.) contain bacterial antigens. Some are endogenous (e.g., histamine, interferon, transfer factor, tuftsin, interleukin-1). Their mode of action is either non-specific, resulting in increased immune responsiveness to a wide variety of antigens, or antigen-specific, i.e., affecting a restricted type of immune response to a narrow group of antigens. The therapeutic efficacy of many biological response modifiers is related to their antigen-specific immunoadjuvanticity. Vaccination therapy

  • Data regarding best practices are limited.
  • Radiation and/or chemotherapy Chemotherapy Osteosarcoma may be recommended on an individual basis to women with:
    • Advanced disease
    • Disease that cannot be managed surgically
  • May be given before or after surgery

Prognosis Prognosis A prediction of the probable outcome of a disease based on a individual’s condition and the usual course of the disease as seen in similar situations. Non-Hodgkin Lymphomas

  • 5-year survival rates:
  • Prognostic factors include:
    • Stage (which includes original tumor Tumor Inflammation size, nodal involvement, and depth of invasion)
    • Capillary lymphatic space invasion
    • Older age
    • HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV) association ( HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV)-associated SCCs have improved outcomes over those not associated with HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV))

Differential Diagnosis

  • Condylomata acuminata Condylomata Acuminata Condylomata acuminata are a clinical manifestation of genital HPV infection. Condylomata acuminata are described as raised, pearly, flesh-colored, papular, cauliflower-like lesions seen in the anogenital region that may cause itching, pain, or bleeding. Condylomata Acuminata (Genital Warts) ( CA CA Condylomata acuminata are a clinical manifestation of genital HPV infection. Condylomata acuminata are described as raised, pearly, flesh-colored, papular, cauliflower-like lesions seen in the anogenital region that may cause itching, pain, or bleeding. Condylomata Acuminata (Genital Warts), genital warts Warts Benign epidermal proliferations or tumors; some are viral in origin. Female Genitourinary Examination): clinical manifestation of genital HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV) infection. Condylomata acuminata Condylomata Acuminata Condylomata acuminata are a clinical manifestation of genital HPV infection. Condylomata acuminata are described as raised, pearly, flesh-colored, papular, cauliflower-like lesions seen in the anogenital region that may cause itching, pain, or bleeding. Condylomata Acuminata (Genital Warts) are described as raised, pearly, flesh-colored, papular, “cauliflower like” lesions seen in the anogenital region that may cause itching, pain Pain An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. Pain: Types and Pathways, or bleeding. These warts Warts Benign epidermal proliferations or tumors; some are viral in origin. Female Genitourinary Examination are typically caused by the low-risk HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV) types 6 and 11 and are spread via sexual contact. Although classified as LSIL on biopsy, CA CA Condylomata acuminata are a clinical manifestation of genital HPV infection. Condylomata acuminata are described as raised, pearly, flesh-colored, papular, cauliflower-like lesions seen in the anogenital region that may cause itching, pain, or bleeding. Condylomata Acuminata (Genital Warts) are not considered premalignant lesions. HPV HPV Human papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation. Papillomavirus (HPV) infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease and CA CA Condylomata acuminata are a clinical manifestation of genital HPV infection. Condylomata acuminata are described as raised, pearly, flesh-colored, papular, cauliflower-like lesions seen in the anogenital region that may cause itching, pain, or bleeding. Condylomata Acuminata (Genital Warts) can be prevented through vaccination Vaccination Vaccination is the administration of a substance to induce the immune system to develop protection against a disease. Unlike passive immunization, which involves the administration of pre-performed antibodies, active immunization constitutes the administration of a vaccine to stimulate the body to produce its own antibodies. Vaccination.
  • Lichen sclerosus Lichen Sclerosus Atrophy and shriveling of the skin of the vulva that is characterized by the whitish lichen sclerosus appearance, inflammation, and pruritus. Benign Vulvar Conditions (LS): chronic dermatologic disease that causes progressive thinning and fibrosis Fibrosis Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury. Bronchiolitis Obliterans of the vulvar, perineal, and perianal skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Skin: Structure and Functions. Lichen sclerosus Lichen Sclerosus Atrophy and shriveling of the skin of the vulva that is characterized by the whitish lichen sclerosus appearance, inflammation, and pruritus. Benign Vulvar Conditions presents classically with itching and white plaques; as LS progresses, scarring Scarring Inflammation can distort the vulvar anatomy. The disorder itself is benign, but LS is associated with an increased risk for vulvar SCC. Diagnosis can be clinical, but often a biopsy is recommended to exclude malignancy Malignancy Hemothorax. Management is with topical high-potency steroids Steroids A group of polycyclic compounds closely related biochemically to terpenes. They include cholesterol, numerous hormones, precursors of certain vitamins, bile acids, alcohols (sterols), and certain natural drugs and poisons. Steroids have a common nucleus, a fused, reduced 17-carbon atom ring system, cyclopentanoperhydrophenanthrene. Most steroids also have two methyl groups and an aliphatic side-chain attached to the nucleus. Benign Liver Tumors.
  • Lichen simplex chronicus: benign vulvar skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Skin: Structure and Functions disorder characterized by hyperkeratosis Hyperkeratosis Ichthyosis Vulgaris (thickening of the skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Skin: Structure and Functions) that occurs secondary to chronic vulvar irritation. Lichen simplex chronicus may be caused by anything that triggers Triggers Hereditary Angioedema (C1 Esterase Inhibitor Deficiency) chronic itching or rubbing, such as atopic or contact dermatitis Contact dermatitis A type of acute or chronic skin reaction in which sensitivity is manifested by reactivity to materials or substances coming in contact with the skin. It may involve allergic or non-allergic mechanisms. Male Genitourinary Examination, vulvar eczema Eczema Atopic dermatitis, also known as eczema, is a chronic, relapsing, pruritic, inflammatory skin disease that occurs more frequently in children, although adults can also be affected. The condition is often associated with elevated serum levels of IgE and a personal or family history of atopy. Skin dryness, erythema, oozing, crusting, and lichenification are present. Atopic Dermatitis (Eczema), insect bites, or psychological disorders such as obsessive-compulsive disorder Obsessive-compulsive disorder Obsessive-compulsive disorder (OCD) is a condition characterized by obsessions (recurring and intrusive thoughts, urges, or images) and/or compulsions (repetitive actions the person is compelled to perform) that are time-consuming and associated with functional impairment. Obsessive-compulsive Disorder (OCD) ( OCD OCD Obsessive-compulsive disorder (OCD) is a condition characterized by obsessions (recurring and intrusive thoughts, urges, or images) and/or compulsions (repetitive actions the person is compelled to perform) that are time-consuming and associated with functional impairment. Obsessive-compulsive Disorder (OCD)). The skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Skin: Structure and Functions can appear thick, scaly, rough, or slightly erythematous, typically with accentuated skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Skin: Structure and Functions markings and excoriations Excoriations Excoriation is a linear abrasion produced by mechanical means (scratching, rubbing, or picking) that usually involves only the epidermis but can reach the papillary dermis. Secondary Skin Lesions. Management is with good vulvar hygiene and topical steroids Steroids A group of polycyclic compounds closely related biochemically to terpenes. They include cholesterol, numerous hormones, precursors of certain vitamins, bile acids, alcohols (sterols), and certain natural drugs and poisons. Steroids have a common nucleus, a fused, reduced 17-carbon atom ring system, cyclopentanoperhydrophenanthrene. Most steroids also have two methyl groups and an aliphatic side-chain attached to the nucleus. Benign Liver Tumors.
  • Lichen planus Lichen planus Lichen planus (LP) is an idiopathic, cell-mediated inflammatory skin disease. It is characterized by pruritic, flat-topped, papular, purple skin lesions commonly found on the flexural surfaces of the extremities. Other areas affected include genitalia, nails, scalp, and mucous membranes. Lichen Planus: idiopathic Idiopathic Dermatomyositis, cell-mediated inflammatory skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Skin: Structure and Functions disease. Lichen planus Lichen planus Lichen planus (LP) is an idiopathic, cell-mediated inflammatory skin disease. It is characterized by pruritic, flat-topped, papular, purple skin lesions commonly found on the flexural surfaces of the extremities. Other areas affected include genitalia, nails, scalp, and mucous membranes. Lichen Planus is characterized by pruritic, flat-topped, papular, purple skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Skin: Structure and Functions lesions commonly found on the flexural surfaces of the extremities. Other areas affected include genitalia, nails, scalp, and mucous membranes (most often in the mouth). Diagnosis is usually clinical, but it is confirmed with biopsy. Management is primarily with topical steroids Steroids A group of polycyclic compounds closely related biochemically to terpenes. They include cholesterol, numerous hormones, precursors of certain vitamins, bile acids, alcohols (sterols), and certain natural drugs and poisons. Steroids have a common nucleus, a fused, reduced 17-carbon atom ring system, cyclopentanoperhydrophenanthrene. Most steroids also have two methyl groups and an aliphatic side-chain attached to the nucleus. Benign Liver Tumors and supportive therapy (such as topical emollients Emollients Oleaginous substances used topically to soothe, soften or protect skin or mucous membranes. They are used also as vehicles for other dermatologic agents. Pityriasis Rosea).
  • Dysplastic or benign nevi Nevi Nevi (singular nevus), also known as “moles,” are benign neoplasms of the skin. Nevus is a non-specific medical term because it encompasses both congenital and acquired lesions, hyper- and hypopigmented lesions, and raised or flat lesions. Nevus/Nevi: Nevi, which are also known as “ moles Moles Primary Skin Lesions,” are benign neoplasms Neoplasms New abnormal growth of tissue. Malignant neoplasms show a greater degree of anaplasia and have the properties of invasion and metastasis, compared to benign neoplasms. Benign Bone Tumors composed of nevus cells that are derived from melanocytes Melanocytes Mammalian pigment cells that produce melanins, pigments found mainly in the epidermis, but also in the eyes and the hair, by a process called melanogenesis. Coloration can be altered by the number of melanocytes or the amount of pigment produced and stored in the organelles called melanosomes. The large non-mammalian melanin-containing cells are called melanophores. Skin: Structure and Functions. Melanocytes are melanin Melanin Insoluble polymers of tyrosine derivatives found in and causing darkness in skin (skin pigmentation), hair, and feathers providing protection against sunburn induced by sunlight. Carotenes contribute yellow and red coloration. Seborrheic Keratosis-producing dendritic cells Dendritic cells Specialized cells of the hematopoietic system that have branch-like extensions. They are found throughout the lymphatic system, and in non-lymphoid tissues such as skin and the epithelia of the intestinal, respiratory, and reproductive tracts. They trap and process antigens, and present them to T-cells, thereby stimulating cell-mediated immunity. They are different from the non-hematopoietic follicular dendritic cells, which have a similar morphology and immune system function, but with respect to humoral immunity (antibody production). Skin: Structure and Functions found within the epidermis Epidermis The external, nonvascular layer of the skin. It is made up, from within outward, of five layers of epithelium: (1) basal layer (stratum basale epidermidis); (2) spinous layer (stratum spinosum epidermidis); (3) granular layer (stratum granulosum epidermidis); (4) clear layer (stratum lucidum epidermidis); and (5) horny layer (stratum corneum epidermidis). Skin: Structure and Functions, dermis Dermis A layer of vascularized connective tissue underneath the epidermis. The surface of the dermis contains innervated papillae. Embedded in or beneath the dermis are sweat glands; hair follicles; and sebaceous glands. Skin: Structure and Functions, hair follicles, and other sites. A nevus can usually be differentiated from a melanoma because a nevus tends to remain uniform in color and shape. Although dysplastic nevi Dysplastic Nevi Clinically atypical nevi (usually exceeding 5 mm in diameter and having variable pigmentation and ill defined borders) with an increased risk for development of non-familial cutaneous malignant melanoma. Biopsies show melanocytic dysplasia. Nevi are clinically and histologically identical to the precursor lesions for melanoma in the b-k mole syndrome. Nevus/Nevi are associated with a 3- to 20-fold risk of malignant transformation Transformation Change brought about to an organism’s genetic composition by unidirectional transfer (transfection; transduction, genetic; conjugation, genetic, etc.) and incorporation of foreign DNA into prokaryotic or eukaryotic cells by recombination of part or all of that DNA into the cell’s genome. Bacteriology into melanoma, most nevi Nevi Nevi (singular nevus), also known as “moles,” are benign neoplasms of the skin. Nevus is a non-specific medical term because it encompasses both congenital and acquired lesions, hyper- and hypopigmented lesions, and raised or flat lesions. Nevus/Nevi have low malignant potential. 
  • Vulvar vestibulitis: chronic pain Chronic pain Aching sensation that persists for more than a few months. It may or may not be associated with trauma or disease, and may persist after the initial injury has healed. Its localization, character, and timing are more vague than with acute pain. Pain Management syndrome of the vestibule Vestibule An oval, bony chamber of the inner ear, part of the bony labyrinth. It is continuous with bony cochlea anteriorly, and semicircular canals posteriorly. The vestibule contains two communicating sacs (utricle and saccule) of the balancing apparatus. The oval window on its lateral wall is occupied by the base of the stapes of the middle ear. Ear: Anatomy of the vulva Vulva The vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy. Presentation Presentation The position or orientation of the fetus at near term or during obstetric labor, determined by its relation to the spine of the mother and the birth canal. The normal position is a vertical, cephalic presentation with the fetal vertex flexed on the neck. Normal and Abnormal Labor is with symptoms of burning pain Pain An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. Pain: Types and Pathways in the vulva Vulva The vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy without an underlying organic cause. The pathophysiology is unknown. Individuals are diagnosed using a cotton-swab test, in which pain Pain An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. Pain: Types and Pathways is reported when pressure is applied to the vulva Vulva The vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy with a cotton swab. Individuals may be treated with education, pelvic floor Pelvic floor Soft tissue formed mainly by the pelvic diaphragm, which is composed of the two levator ani and two coccygeus muscles. The pelvic diaphragm lies just below the pelvic aperture (outlet) and separates the pelvic cavity from the perineum. It extends between the pubic bone anteriorly and the coccyx posteriorly. Vagina, Vulva, and Pelvic Floor: Anatomy exercises, and psychotherapy Psychotherapy Psychotherapy is interpersonal treatment based on the understanding of psychological principles and mechanisms of mental disease. The treatment approach is often individualized, depending on the psychiatric condition(s) or circumstance. Psychotherapy.

References

  1. Berek, J.S., Karam, A. (2021). Vulvar cancer: epidemiology, diagnosis, histopathology, and treatment. UpToDate. Retrieved September 7, 2021, from https://www.uptodate.com/contents/vulvar-cancer-epidemiology-diagnosis-histopathology-and-treatment 
  2. Karam, A., Berek, J.S., Russo, A.L. (2021). Squamous cell carcinoma of the vulva: staging and surgical treatment. UpToDate. Retrieved September 7, 2021, from https://www.uptodate.com/contents/squamous-cell-carcinoma-of-the-vulva-staging-and-surgical-treatment 
  3. Karam, A., Berek, J.S., Russo, A.L. (2021). Squamous cell carcinoma of the vulva: medical therapy and prognosis. In UpToDate. Retrieved September 7, 2021, from https://www.uptodate.com/contents/squamous-cell-carcinoma-of-the-vulva-medical-therapy-and-prognosis 
  4. Holschneider, C.H. (2021). Vulvar squamous intraepithelial lesions (vulvar intraepithelial neoplasia). UpToDate. Retrieved September 7, 2021, from https://www.uptodate.com/contents/vulvar-squamous-intraepithelial-lesions-vulvar-intraepithelial-neoplasia 
  5. Creasman, W.T. (2021). Malignant vulvar lesions. Medscape. Retrieved September 7, 2021, from https://emedicine.medscape.com/article/264898-overview#showall 
  6. Canavan, T.P. (2002). Vulvar cancer. Am Fam Physician. 66(7),1269-1275. https://www.aafp.org/afp/2002/1001/p1269.html#sec-4 
  7. Capria, A., Tahir, N., Fatehi, M. (2021). Vulva cancer. StatPearls. https://www.ncbi.nlm.nih.gov/books/NBK567798/
  8. Alkatout, I., Schubert, M., Garbrecht, N., et al. (2015). Vulvar cancer: epidemiology, clinical presentation, and management options. Int J Womens Health 7:305–313. https://pubmed.ncbi.nlm.nih.gov/25848321/
  9. PDQ Adult Treatment Editorial Board. (2021). Vulvar Cancer Treatment (PDQ®): Health Professional Version. In: PDQ Cancer Information Summaries. https://www.ncbi.nlm.nih.gov/books/NBK65760/

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