Vaginal Cancer

Primary vaginal cancers are malignant tumors that originate from cells in the 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. 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) is by far the most common (80%–85%); other histologic types include adenocarcinomas, sarcomas (including sarcoma botryoides, typically seen in children), and melanomas. Vaginal SCC is most commonly 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, while clear cell adenocarcinomas are associated with in utero exposure to diethylstilbestrol (DES). Individuals typically present with vaginal bleeding and/or an irregular mass or lesion on exam; other symptoms may include abnormal discharge, 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, and urinary or defecatory symptoms. A biopsy is required for diagnosis. 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 is based on tumor size, extent of local invasion, and metastasis. Management may be surgical for stage I disease, but surgery is typically avoided in advanced disease, which is instead managed with radiation and chemotherapy.

Last update:

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Table of Contents

Share this concept:

Share on facebook
Share on twitter
Share on linkedin
Share on reddit
Share on email
Share on whatsapp

Overview

Definition

Primary vaginal cancer is a malignant tumor arising from tissue of the 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 review

The 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 is:

  • A fibromuscular tube 
  • Lined with nonkeratinized stratified 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
  • Contains no glands within the vaginal wall itself
  • Located between the bladder and 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 in the female 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
Female pelvis cross section

A sectioned female 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 depicting the uterus in situ

Image by BioDigital, edited by Lecturio

Histologic classification

Vaginal malignancies may be either primary (originating from the 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 itself) or metastatic to the 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 from other primary sites.

Primary vaginal cancers:

The most common subtypes of primary vaginal cancer (originating in the 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) 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): approximately 80%–85%
    • “Typical” SCC (most common primary vaginal cancer)
    • Verrucous carcinoma: a rare variant of SCC that generally does not metastasize via blood or lymphatic vessels, though is locally aggressive
  • Adenocarcinoma (glandular malignancies): approximately 10%
    • Clear cell adenocarcinoma (2nd most common primary vaginal cancer)
    • Endodermal sinus tumor (also called a yolk sac tumor; tumor derived from germ cells)
  • Sarcomas (soft tissue malignancies): approximately 2.5%
    • Sarcoma botryoides (an embryonal rhabdomyosarcoma, which is a malignant tumor of skeletal muscle)
    • Leiomyosarcoma Leiomyosarcoma Uterine leiomyomas (or uterine fibroids) are benign tumors arising from smooth muscle cells in the uterine myometrium. Leiomyosarcomas, however, are malignant tumors, arising de novo (not from fibroids). Uterine Leiomyoma and Leiomyosarcoma (smooth muscle malignancy, very rare)
  • 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 2%
  • Other types: 1%–2% total 
    • Undifferentiated
    • Small cell
    • Lymphoma
    • Carcinoid

Metastatic cancer from other primary sites: 

  • Accounts for 80% of all malignancies in the 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
  • Occurs by:
    • Direct extension
    • Lymphatic/hematologic spread
  • Most common primary sites that metastasize to the 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:
    • Cervix
    • Endometrium/uterus
    • Vulva
    • Ovary
    • Breast
    • Rectum
    • Kidney

Epidemiology

Primary vaginal cancer:

  • Rare: accounts for only 1%–2% of all gynecologic malignancies
  • Age-adjusted incidence in the United States: approximately 1 per 100,000 population 
  • Sarcoma botryoides: most common vaginal cancer in children
  • Mean age at diagnosis: 
    • SCC: 60 years
    • Clear cell adenocarcinoma: 
      • 15–20 years (girls who were exposed to diethylstilbestrol (DES) in utero)
      • Late 60s–70s (women who were not exposed to DES in utero
    • Sarcoma botryoides: < 5 years (though it is possible in older girls and women)

Related videos

Etiology and Pathogenesis

Risk factors

  • SCC:
    • Infection 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:
      • Most important risk factor
      • High-risk types: 16 and 18
    • Squamous cell atypia of the cervix or 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 (see Vaginal squamous intraepithelial lesions (SILs) and vaginal intraepithelial neoplasia (VaIN) below)
    • Smoking
    • Multiple sexual partners
    • Increasing age
    • History of cervical or vulvar carcinoma
    • History of pelvic radiation
    • Immunosuppression
  • Clear cell adenocarcinoma: exposure in utero to DES 
    • The synthetic estrogen DES was given to pregnant women to prevent miscarriage in the 1950 and 1960s.
    • The daughters of women who took DES during pregnancy Pregnancy Pregnancy is the time period between fertilization of an oocyte and delivery of a fetus approximately 9 months later. The 1st sign of pregnancy is typically a missed menstrual period, after which, pregnancy should be confirmed clinically based on a positive β-hCG test (typically a qualitative urine test) and pelvic ultrasound. Pregnancy: Diagnosis, Maternal Physiology, and Routine Care are at risk for clear cell adenocarcinoma.
    • Because of this association, DES was discontinued in 1971.

Vaginal squamous intraepithelial lesions (SILs) and vaginal intraepithelial neoplasia (VaIN)

  • Vaginal squamous intraepithelial lesions:
    • Defined as squamous cell atypia without invasion
    • Traditionally referred to as VaIN; however, revised terminology was recommended in 2012
    • Classified according to the depth of epithelial involvement
    • SIL/VaIN considered a premalignant lesion:
      • Possible to resolve spontaneously
      • Risk of malignant transformation from SIL/VaIN to invasive vaginal carcinoma: approximately 10%
  • Low-grade squamous intraepithelial lesion (LSIL):
    • Involves < ⅓ of the vaginal 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 (depth)
    • Traditional nomenclature: VaIN-I
  • High-grade squamous intraepithelial lesion (HSIL):
    • Involves > ⅓ of the vaginal 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 (depth)
    • Traditional nomenclature: VaIN-II and VaIN-III
    • Higher risk of malignant progression
    • Carcinoma in situ: involves the full thickness of the 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

Pathogenesis

The pathogenesis of SCC is usually related 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 infections. The pathogenesis of other types is less well characterized.

  • 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 → LSIL/VaIN-I → HSIL/VaIN-II/III→ carcinoma in situ → invasion through the basement membrane = invasive cancer
  • 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 2 major oncoproteins:
    • E6 inactivates p53 tumor suppressor protein.
    • E7 inactivates Rb tumor suppressor protein.
  • After cells lose tumor suppressor proteins → unregulated proliferation → HSIL

Patterns of spread

  • Growth of primary tumor
  • Extension to surrounding organs
  • Embolization into lymph nodes: 
    • 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: communicates with lymphatic drainage from cervix → pelvic nodes → para-aortic nodes
    • 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: drains into the inguinal and femoral nodes → pelvic nodes → para-aortic nodes
  • Dissemination via hematologic and lymphatic vessels (usually late manifestation):
    • Lungs Lungs Lungs are the main organs of the respiratory system. Lungs are paired viscera located in the thoracic cavity and are composed of spongy tissue. The primary function of the lungs is to oxygenate blood and eliminate CO2. Lungs
    • 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
    • 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

Clinical Presentation

Symptoms

  • Vaginal bleeding (most common symptom), which is typically:
    • Postcoital
    • Postmenopausal
  • Abnormal vaginal discharge, which may be:
    • Watery
    • Malodorous
  • Pelvic 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
  • Lesions involving the anterior vaginal wall may present with:
    • Dysuria
    • Hematuria
    • Urgency
  • Lesions involving the posterior wall may present with:
    • Constipation Constipation Constipation is common and may be due to a variety of causes. Constipation is generally defined as bowel movement frequency < 3 times per week. Patients who are constipated often strain to pass hard stools. The condition is classified as primary (also known as idiopathic or functional constipation) or secondary, and as acute or chronic. Constipation
    • Dyschezia
    • Hematochezia (bright-red blood from the anus)
  • About 20% of women are asymptomatic at diagnosis (detected on screening pelvic exams).

Findings on exam

  • Vaginal mass:
    • Irregular shape
    • Solid components
    • Friable (bleeds easily)
    • Fungating mass
    • May constrict anatomy
    • Most commonly located on the posterior wall in the upper ⅓ of the 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, but may occur anywhere
    • Sarcoma botryoides: mass protruding from the 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 appearing as a grape-like cluster of tissue
  • Plaques
  • Ulcers
  • Extension of lesion to cervix
  • Inguinal 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

Diagnosis and Staging

Histologic examination of a biopsy is required for a formal diagnosis of vaginal cancer. Imaging findings help with staging and surgical planning. Lab assessment (aside from cytology/histology) is generally not helpful.

Examinations and cytology

  • Pelvic exam:
    • Carefully assess all walls of the 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 (requires rotation of the speculum blades).
    • Palpate for any raised or hardened areas.
    • Identify any abnormal masses or lesions that should be biopsied.
    • Measure the size of any lesions.
    • Palpate lymph nodes for 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.
  • Cytology:
    • Vaginal cytology (i.e., vaginal Pap smear): should be obtained on any abnormal areas (or on the vaginal cuff if the woman has had a hysterectomy)
    • Cervical Pap smear: should be up to date per screening guidelines owing to high rate of concurrent cervical pathology
  • Colposcopy: 
    • Performed on the cervix, 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 potentially 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 (depending on presentation)
    • Soak the cervix and 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 in acetic acid and examine under magnification with a 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

  • Required for diagnosis (gold standard)
  • May be obtained with an in-office punch biopsy, though may require an exam under anesthesia Anesthesia Anesthesiology is the field of medicine that focuses on interventions that bring a state of anesthesia upon an individual. General anesthesia is characterized by a reversible loss of consciousness along with analgesia, amnesia, and muscle relaxation. Anesthesiology: History and Basic Concepts in cases such as:
    • Severe cervical stenosis
    • Abnormal vasculature raising concern for significant bleeding
  • Used to:
    • Confirm cancer diagnosis (rule out benign pathology)
    • Determine the histologic type of cancer
    • Assess depth of invasion

Imaging

Imaging of the abdominopelvic and/or thoracic cavities is 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
  • Imaging methods:
    • Pelvic MRI
    • PET/CT scan
    • Chest radiography
Ct scan of the pelvis showing a mass in the posterior vaginal wall

CT scan 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 a mass in the posterior vaginal wall (approximately 11.2 x 9.0 cm):
The mass was diagnosed as a vaginal leiomyosarcoma on histology.

Image: “A large primary retroperitoneal vaginal leiomyosarcoma: a case report.” by Xu Z, Zeng R, Liu J. License: CC BY 4.0

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

Vaginal cancer is staged using the TNM staging system. There are 4 major stages. 

  • TNM staging takes into account:
    • Tumor size
    • Extent of local invasion
    • Metastasis to lymph nodes
    • Distant metastasis
  • Clinical staging uses findings from:
    • Physical exam
    • Cystoscopy and proctoscopy
    • Chest and skeletal radiography
  • Surgical staging includes information obtained from:
    • Resected lesions
    • Resected lymph nodes

Individuals are staged based on their “highest” findings. For example, tumor in an individual with a tumor confined to the 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 but with positive lymph nodes is classified as stage III. Similarly, direct tumor invasion into the bladder mucosa is stage IV even if there is no lymph node involvement or distant metastasis.

Table: Vaginal cancer staging
Stage Extent of tumor invasion into surrounding tissue Metastasis
I Tumor is confined to the 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. None
II Tumor invades into paravaginal tissue, but does not extend all the way to the pelvic sidewall. None
III
  • Tumor invades into the pelvic sidewall.
  • Tumor causes hydronephrosis Hydronephrosis Hydronephrosis is dilation of the renal collecting system as a result of the obstruction of urine outflow. Hydronephrosis can be unilateral or bilateral. Nephrolithiasis is the most common cause of hydronephrosis in young adults, while prostatic hyperplasia and neoplasm are seen in older patients. Hydronephrosis or a nonfunctioning kidney owing to compression.
Metastasis to regional lymph nodes
IV
  • Direct tumor invasion into the mucosa of the bladder or 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
  • Direct tumor invasion outside the true 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
  • Metastasis to lymph nodes beyond the pelvic nodes
  • Metastasis to distant structures

Management and Prognosis

Management

Vaginal cancer is rare, so evidence on optimal treatment approaches is lacking. Treatment recommendations are generally adopted from similar cervical and anal cancers (which are more common).

  • Treatment is individualized based on tumor location, size, and clinical stage.
  • Stage I disease is typically treated with: 
    • Surgical excision +/– radiation therapy
    • Radiation therapy alone
  • Stages II–IV are typically treated with radiation + chemotherapy:
    • Chemotherapy typically involves:
      • Fluorouracil
      • Cisplatin
    • Radiation therapy typically involves a combination of:
      • Brachytherapy
      • External-beam radiation therapy
  • Posttreatment surveillance to look for local recurrence:
    • Regular pelvic exams
    • Annual Pap smears
    • Colposcopy and biopsy if abnormalities are detected.

Surgical management in vaginal cancer

  • Surgery is associated with worse outcomes in SCC stages II–IV → generally avoided
    • Owing to local invasion, typically involves removal of adjacent structures, including bladder and bowel
    • Complication rates can be as high as 50% in total pelvic exenteration procedures.
  • Indications for surgery:
    • Verrucous carcinoma
    • Sarcoma botryoides
    • Well-differentiated leiomyosarcomas
    • 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
    • Palliative indications in advanced disease 
    • Fertility preservation: surgical transposition of the ovaries Ovaries Ovaries are the paired gonads of the female reproductive system that contain haploid gametes known as oocytes. The ovaries are located intraperitoneally in the pelvis, just posterior to the broad ligament, and are connected to the pelvic sidewall and to the uterus by ligaments. These organs function to secrete hormones (estrogen and progesterone) and to produce the female germ cells (oocytes). Ovaries out 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 in young women prior to initiating radiation therapy

Prognosis

  • The most important prognostic factors include:
    • Histologic type
    • Stage at presentation (especially tumor size and lymph node metastasis)
    • Age
  • 5-year survival rates based on stage:
    • Stage I: 75%–80%
    • Stage II: approximately 50%
    • Stage III: approximately 40%
    • Stage IV: approximately 10%–20%

Differential Diagnosis

Abnormal bleeding

The presenting symptom in vaginal cancer is typically postcoital or postemenopausal bleeding. The differential diagnosis for these presenting symptoms includes:

  • Cervical cancer Cervical cancer Cervical cancer, or invasive cervical carcinoma (ICC), is the 3rd most common cancer in women in the world, with > 50% of the cases being fatal. In the United States, ICC is the 13th most common cancer and the cause of < 3% of all cancer deaths due to the slow progression of precursor lesions and, more importantly, effective cancer screening. Cervical Cancer: invasive cancer of the cervix (and the most common gynecologic cancer worldwide). There are 2 major histologic types of cervical cancer: SCC and adenocarcinoma, the vast majority of which are caused by 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 infections. Early cervical neoplasia is asymptomatic, though more advanced disease may present with abnormal bleeding (especially bleeding on contact). Diagnosis is made by Pap testing with cytology, 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 testing, and biopsy.
  • Endometrial cancer Endometrial Cancer Endometrial carcinoma (EC) is the most common gynecologic malignancy in the developed world, and it has several histologic types. Endometrioid carcinoma (known as type 1 EC) typically develops from atypical endometrial hyperplasia, is hormonally responsive, and carries a favorable prognosis. Endometrial Hyperplasia and Endometrial Cancer: cancer of the inner lining of the uterus (and the most common gynecologic cancer in the United States). Anything that increases estrogen exposure will increase the risk for endometrial cancer; these risks include obesity Obesity Obesity is a condition associated with excess body weight, specifically with the deposition of excessive adipose tissue. Obesity is considered a global epidemic. Major influences come from the western diet and sedentary lifestyles, but the exact mechanisms likely include a mixture of genetic and environmental factors. Obesity, chronic anovulation in reproductive-aged women, hormone replacement therapy, and tamoxifen use. Endometrial cancer Endometrial Cancer Endometrial carcinoma (EC) is the most common gynecologic malignancy in the developed world, and it has several histologic types. Endometrioid carcinoma (known as type 1 EC) typically develops from atypical endometrial hyperplasia, is hormonally responsive, and carries a favorable prognosis. Endometrial Hyperplasia and Endometrial Cancer is diagnosed with an endometrial biopsy; ultrasonography may show a thickened endometrial lining in postmenopausal women. Management is primarily surgical.
  • Endometrial atrophy: benign condition in which the endometrial lining becomes thin and atrophic because of prolonged states of low estrogen. With little to no fluid in the cavity, friction may lead to micro-erosions and a subsequent inflammatory reaction that typically presents with postmenopausal light bleeding or spotting. Endometrial atrophy is diagnosed on ultrasonography (which shows a thin endometrial lining) in the setting of a negative endometrial biopsy. No treatment is required.
  • Endometrial or cervical polyps: pedunculated or sessile projections of the endometrium that result from overgrowth of endometrial glands and stroma around a central vascular stalk. Although these polyps are usually benign, they can be malignant, particularly in postmenopausal women. Endometrial or cervical polyps present with abnormal uterine or postmenopausal bleeding, though many are asymptomatic. Endometrial polyps Endometrial polyps Endometrial polyps are pedunculated or sessile projections of the endometrium that result from overgrowth of endometrial glands and stroma around a central vascular stalk. Endometrial polyps are a few millimeters to a few centimeters in size, can occur anywhere within the uterine cavity, and, while usually benign, can be malignant, particularly in postmenopausal women. Endometrial Polyps are best diagnosed with saline-infusion sonography (SIS) and are usually treated with hysteroscopic resection. 
  • Leiomyomas (uterine fibroids): common, benign tumors arising from smooth muscle cells in the uterine myometrium. Leiomyomas typically present with abnormal bleeding, pelvic 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, and/or bulk symptoms. Fibroids are identified as a hypoechoic, well-circumscribed, round mass on pelvic ultrasonography. Leiomyomas of the vaginal wall are also possible, though extremely rare. 
  • Adenomyosis Adenomyosis Adenomyosis is a benign uterine condition characterized by the presence of ectopic endometrial glands and stroma within the myometrium. Adenomyosis is a common condition, affecting 20%-35% of women, and typically presents with heavy menstrual bleeding and dysmenorrhea. Adenomyosis: very common benign uterine condition characterized by the presence of ectopic endometrial glands and stroma within the myometrium. Adenomyosis Adenomyosis Adenomyosis is a benign uterine condition characterized by the presence of ectopic endometrial glands and stroma within the myometrium. Adenomyosis is a common condition, affecting 20%-35% of women, and typically presents with heavy menstrual bleeding and dysmenorrhea. Adenomyosis typically presents with heavy menstrual bleeding and dysmenorrhea. Diagnosis is either clinical or assisted with pelvic imaging, usually transvaginal ultrasonography or, occasionally, MRI. Management is based on the woman’s preference regarding future childbearing and may include hysterectomy, other surgical options, or medical hormonal suppression with progestins.
  • Vulvovaginitis Vulvovaginitis The term vulvovaginitis is used to describe an acute inflammation of the vulva and vagina. Vulvovaginitis can be caused by several infectious and non-infectious etiologies, and results from disruption of the normal vaginal environment. Common signs and symptoms include pain, pruritis, erythema, edema, vaginal discharge and dyspareunia. Vulvovaginitis: acute 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 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 and 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, most commonly due to Candida Candida Candida is a genus of dimorphic, opportunistic fungi. Candida albicans is part of the normal human flora and is the most common cause of candidiasis. The clinical presentation varies and can include localized mucocutaneous infections (e.g., oropharyngeal, esophageal, intertriginous, and vulvovaginal candidiasis) and invasive disease (e.g., candidemia, intraabdominal abscess, pericarditis, and meningitis). Candida/Candidiasis albicans, bacterial vaginosis, and Trichomonas vaginalis infections. Noninfectious causes include atrophic vaginitis and contact dermatitis. Common signs and symptoms include abnormal discharge, 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/dyspareunia, pruritus, erythema, and edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema of the affected region. Management depends on the etiology.
  • Cervicitis: 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 of the cervix, most commonly due to infections with Chlamydia Chlamydia Chlamydiae are obligate intracellular gram-negative bacteria. They lack a peptidoglycan layer and are best visualized using Giemsa stain. The family of Chlamydiaceae comprises 3 pathogens that can infect humans: Chlamydia trachomatis, Chlamydia psittaci, and Chlamydia pneumoniae. Chlamydia trachomatis and/or Neisseria Neisseria Neisseria is a genus of bacteria commonly present on mucosal surfaces. Several species exist, but only 2 are pathogenic to humans: N. gonorrhoeae and N. meningitidis. Neisseria species are non-motile, gram-negative diplococci most commonly isolated on modified Thayer-Martin (MTM) agar. Neisseria gonorrhoeae. Individuals are often asymptomatic, but they may present with a purulent abnormal discharge, pelvic 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, and irregular bleeding (especially contact bleeding). Diagnosis is with a nucleic acid amplification test (NAAT) and management is with antibiotics.

Vaginal mass

Benign vaginal masses may include:

  • Vaginal inclusion or epidermal cysts: benign, small (approximately 1 cm) white or yellow cysts that can be located in the 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 or 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. Inclusion cysts occur when epithelial tissue becomes trapped under the surface after trauma. Epidermal cysts occur when sebaceous gland ducts become obstructed, causing secretions to accumulate under 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. These cysts are usually asymptomatic, but they may cause dyspareunia if they enlarge or become infected.
  • Gartner duct cysts: Gartner ducts are the embryologic remnants of the Wolffian (mesonephric) ducts, which typically regress in females in utero. If Gartner ducts persist and fill with fluid, they can become cysts (usually < 2 cm) on the anterolateral wall of the 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. These cysts are typically asymptomatic and discovered as incidental findings on gynecologic exams or imaging studies. If symptoms are present, they most commonly include dyspareunia and voiding disturbances.
  • Urethral diverticulum: focal outpouchings of the urethra that present as a vaginal mass on the anterior wall of the 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. Individuals will typically present with dysuria, postvoid dribbling, dyspareunia, recurrent urinary tract infections Urinary tract infections Urinary tract infections (UTIs) represent a wide spectrum of diseases, from self-limiting simple cystitis to severe pyelonephritis that can result in sepsis and death. Urinary tract infections are most commonly caused by Escherichia coli, but may also be caused by other bacteria and fungi. Urinary Tract Infections, and/or hematuria. Palpation of the mass may cause leakage of urine. Diagnosed clinically and with cystoscopy.
  • Vaginal endometriosis Endometriosis Endometriosis is a common disease in which patients have endometrial tissue implanted outside of the uterus. Endometrial implants can occur anywhere in the pelvis, including the ovaries, the broad and uterosacral ligaments, the pelvic peritoneum, and the urinary and gastrointestinal tracts. Endometriosis: Endometriosis is the ectopic implantation of endometrial tissue outside the uterine cavity. Although rare, it is possible for endometrial tissue to implant in the 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. The implant may present as a small blue, black, brown, or white lesion or as a larger cyst filled with dark fluid (known as a “chocolate cyst”). Other symptoms may include dyspareunia, dysmenorrhea, abnormal bleeding, and urinary/defecatory symptoms.

References

  1. Karam, A., Berek, J.S., Kidd, E. A. (2021). Vaginal cancer. UpToDate. Retrieved September 8, 2021, from https://www.uptodate.com/contents/vaginal-cancer 
  2. Holschneider, C., and Berek, J.S. (2021). Vaginal intraepithelial neoplasia. UpToDate. Retrieved September 8, 2021, from https://www.uptodate.com/contents/vaginal-intraepithelial-neoplasia 
  3. Bardawil, T. (2021). Vaginal cancer. Medscape. Retrieved September 8, 2021, from https://emedicine.medscape.com/article/269188-overview#showall 
  4. Kaltenecker, B. (2021). Vaginal cancer. StatPearls. Retrieved September 8, 2021, from https://www.statpearls.com/articlelibrary/viewarticle/30938/ 
  5. Schorge, J.O., Schaffer, J.I., et al. (2008). Chapter 32: Vaginal Cancer. In Williams Gynecology. McGraw-Hill Education, pp. 677–686.

Study on the Go

Lecturio Medical complements your studies with evidence-based learning strategies, video lectures, quiz questions, and more – all combined in one easy-to-use resource.

Learn even more with Lecturio:

Complement your med school studies with Lecturio’s all-in-one study companion, delivered with evidence-based learning strategies.

User Reviews

0.0

()

¡Hola!

Esta página está disponible en Español.

🍪 Lecturio is using cookies to improve your user experience. By continuing use of our service you agree upon our Data Privacy Statement.

Details