Vulvar Cancer

There are multiple different types of malignancies that can affect 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. 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), 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 carcinoma Basal cell carcinoma Basal cell carcinoma is the most common skin malignancy. This cancer arises from the basal layer of the epidermis. The lesions most commonly appear on the face as pearly nodules, often with telangiectatic blood vessels and ulceration in elderly individuals. Basal Cell Carcinoma, sarcoma, malignancy of the Bartholin glands, 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 (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. Papillomaviridae: HPV infection or lichen sclerosus. 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 is also common. Diagnosis usually requires a biopsy, and management is primarily with surgical excision. Unfortunately, lymph node metastasis occurs early in the natural history of the disease and is associated with a poor prognosis.

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Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

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Overview

Definition

Vulvar cancer is a malignancy 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, which includes structures of the external female genitalia: 

  • Labia majora and minora
  • Clitoris
  • Mons pubis
  • Greater vestibular glands (Bartholin glands)

Epidemiology

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

  • Lifetime risk: 0.3%
  • Squamous cell carcinoma (SCC) is the most common histologic subtype: approximately 75%–85%
  • Incidence: approximately 5000–10,000 cases per year in the United States
  • Mortality: 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. Papillomaviridae: 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. Papillomaviridae: 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. Papillomaviridae: 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. Papillomaviridae: HPV-16.
  • Increasing age
  • Smoking 
  • Lichen sclerosus (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. Papillomaviridae: HPV-associated SCC)
  • Squamous cell hyperplasia
  • Prior pelvic radiation
  • Immunodeficiency

Classification

Classification by histologic subtype

Histologic subtypes of vulvar cancer include:

  • Squamous cell carcinoma (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. Papillomaviridae: 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. Papillomaviridae: HPV-associated (keratinized type)
  • Melanoma (approximately 5%–10%)
  • Basal cell carcinoma (approximately 2%–8%)
  • Sarcomas (approximately 2%): soft tissue 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
    • Angiosarcoma: malignancies of blood and lymphatic vessels
    • Neurofibrosarcomas: malignancies of neural tissue
  • Adenocarcinoma or carcinoma of the Bartholin glands (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 (an adenocarcinoma originating from glandular cells, < 1%)

Staging Staging Cancer is the 2nd leading cause of death in the US after cardiovascular disease. Many malignancies are treatable or curable, but some may recur. Thus, all malignancies must be assigned a grade and stage in order to guide management and determine prognosis. Grading, Staging, and Metastasis

Vulvar cancer is staged on the basis of the size and location of the tumor, regional lymph node involvement, and the presence of metastasis.

  • Stage I: 
    • Tumor confined 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
    • No lymph node involvement
  • Stage II: 
    • Tumor with extension to adjacent perineal structures, such as:
      • Lower urethra
      • 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
      • Anus
    • No lymph node involvement
  • Stage III:
    • Tumor with extension to adjacent perineal structures
    • Metastasis to local lymph nodes (superficial or deep inguinal nodes)
  • Stage IV, which may be defined by the presence of any of the following:
    • Tumor invades to other regions, which may include:
      • Upper urethra
      • 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
      • Bladder
      • 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
      • 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. Structure of Bones
    • Any distant metastasis
    • Metastasis to beyond local lymph nodes (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. Papillomaviridae: 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. Papillomaviridae: 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. 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. Papillomaviridae: HPV types (6, 11) that cause genital warts Genital Warts 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)
    • 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. Papillomaviridae: HPV types (16 (especially), 18, 31, 33, and 45)
    • High association with concurrent cervical intraepithelial neoplasia (CIN)
  • 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. Papillomaviridae: HPV infections
    • 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, et al. 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. Papillomaviridae: HPV-associated 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 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. Papillomaviridae: 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. Papillomaviridae: 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. Papillomaviridae: HPV has the ability to affect 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. Papillomaviridae: HPV has 2 major oncoproteins:
    • E6: inactivates p53 tumor suppressor protein
    • E7: inactivates Rb tumor suppressor protein
  • After cells lose tumor suppressor proteins → unregulated proliferation → HSIL
  • Invasion through the basement membrane → 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. Papillomaviridae: HPV-associated 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 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. Papillomaviridae: HPV-associated SCC vulvar cancer most commonly occurs in the setting of lichen sclerosus.

  • Inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body's defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. 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 → SCC

Patterns of spread

  • Growth of primary tumor
  • Extension to surrounding organs
  • Embolization into regional lymph nodes: 
    • Superficial inguinal lymph node → deep inguinal lymph node → iliac lymph node
    • Spread beyond the deep inguinal nodes is considered “distant spread” → stage IV
  • Dissemination via hematologic and lymphatic vessels
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

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 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

  • Unifocal lesion (most commonly on the labia majora)
  • Lesions may have a variety of appearances, including:
    • Fleshy, nodular, or warty masses
    • Ulcerated
    • Scaly patches 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 (may be present even with small vulvar lesions)
  • Symptoms suggestive of advanced disease:
    • Vulvar pain Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain 
    • Dysuria
    • Dyschezia
    • Rectal bleeding
Squamous cell carcinoma of the vulva

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 immediately prior to surgical resection:
Planned surgical resection margins are marked.

Image: “Microinvasive 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 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” by Sidor J, Diallo-Danebrock R, Eltze E, Lellé RJ. License: CC BY 2.0

Findings suggestive of non– 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 vulvar cancers

  • Basal cell carcinoma:
    • “Rodent ulcer”: rolled edges with central ulceration
    • Lesion may be pigmented, pearly, and/or gray.
  • Melanoma:
    • Usually a pigmented lesion
    • Characteristics similar to nonvulvar 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 (ABCDEs):
      • Asymmetric
      • Irregular Borders
      • Black Color
      • Diameter > 6 mm
      • Evolving/elevation
  • Paget disease:
    • Eczematoid appearance: 
      • Well demarcated
      • Raised edges
      • Red background
    • Usually multifocal
  • Bartholin gland carcinomas:
    • Mass in the region of the Bartholin glands (4:00 and 8:00 when viewing 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 as a clock face)
    • Mass may be irregular with solid components.
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 (also known as extramammary Paget disease):
Note the firm red mass on an eczematoid erythema 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 for lymphadenopathy.
    • Pap smear: should be up-to-date per screening guidelines owing to high rate of concurrent cervical pathology
  • Colposcopy: 
    • 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, 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, and cervix
    • 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 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. Structure and Function of the Skin, 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, and cervix in acetic acid and examine under magnification with a standard colposcope.
    • Allows for identification of:
      • Acetowhite changes (abnormalities) 
      • Abnormal vascular patterns
  • Cystoscopy: if there is concern for bladder involvement
  • Proctoscopy: if there is concern for rectal involvement

Biopsy

  • Should be obtained for any suspicious lesions because vulvar cancer can have a wide range of appearances
  • Indications:
    • Masses
    • Persistent ulceration 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. Structure and Function of the Skin color or elevation
  • Typically obtained with an in-office punch biopsy
  • Used to:
    • Confirm cancer diagnosis (rule out benign pathology such as lichen simplex chronicus)
    • 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
    • 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 or lung metastasis
  • Indications:
    • Symptoms suggestive of metastases (e.g., bowel or bladder dysfunction)
    • Tumors ≥ 4 cm
    • Clinically palpable lymph nodes
    • Individuals who are not candidates for surgery
  • Imaging methods:
    • Pelvic MRI
    • PET/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 showing an enlarged left inguinal lymph node
Right: SPECT scan showing focal tracer uptake in the left inguinal lymph 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 (site of injection).

Image: “Hybrid SPECT/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” 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, and chemotherapy is frequently used. The specific treatment plan depends on the individual’s disease and comorbidities.

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 node dissection”)
    • Debulking procedures
  • Surgery is tailored to the individual.

Chemoradiation and adjuvant therapy

  • Data regarding best practices are limited.
  • Radiation and/or chemotherapy 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

  • 5-year survival rates:
    • Stage 1: approximately 80%
    • Stage 2: approximately 60%
    • Stage 3: approximately 40%
    • Stage 4: approximately 10%–15%
  • Prognostic factors include:
    • Stage (which includes original tumor 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. Papillomaviridae: 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. Papillomaviridae: 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. Papillomaviridae: 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, genital warts Genital Warts 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)): 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. Papillomaviridae: 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 Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain, or bleeding. These warts 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. Papillomaviridae: HPV types 6 and 11 and are spread via sexual contact. Although classified as LSIL on biopsy, CA 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. Papillomaviridae: HPV infections and CA 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 (LS): chronic dermatologic disease that causes progressive thinning and fibrosis 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. Structure and Function of the Skin. Lichen sclerosus presents classically with itching and white plaques; as LS progresses, scarring 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. Management is with topical high-potency steroids.
  • 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. Structure and Function of the Skin disorder characterized by hyperkeratosis (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. Structure and Function of the Skin) that occurs secondary to chronic vulvar irritation. Lichen simplex chronicus may be caused by anything that triggers chronic itching or rubbing, such as atopic or contact dermatitis, 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. Structure and Function of the Skin 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. Structure and Function of the Skin markings and excoriations. Management is with good vulvar hygiene and topical steroids.
  • 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, 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. Structure and Function of the Skin 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. Structure and Function of the Skin 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 and supportive therapy (such as topical emollients).
  • 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,” are benign neoplasms composed of nevus Nevus 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 cells that are derived from melanocytes. Melanocytes are melanin-producing dendritic cells found within the epidermis, dermis, hair follicles, and other sites. A nevus Nevus 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 can usually be differentiated from a 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 because a nevus Nevus 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 tends to remain uniform in color and shape. Although dysplastic 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 are associated with a 3- to 20-fold risk of malignant transformation into 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, 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 Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain syndrome of the vestibule 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. Presentation is with symptoms of burning pain Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain 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 without an underlying organic cause. The pathophysiology is unknown. Individuals are diagnosed using a cotton-swab test, in which pain Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain 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 with a cotton swab. Individuals may be treated with education, pelvic floor 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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