Vulva
Malignant neoplasms
Carcinoma - general

Author: Priya Nagarajan, M.D., Ph.D. (see Authors page)
Editor: Sara Peters, Ph.D., M.D.

Revised: 8 October 2017, last major update June 2014

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Vulvar carcinoma [title] "loattrfree full text"[sb]

Cite this page: Nagarajan, P. Carcinoma - general. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/vulvacarcinomageneral.html. Accessed November 23rd, 2017.
Definition / general
  • Malignant tumors arising from the epithelial components of the vulva
  • Since most of the common malignancies affecting the vulva are discussed separately, this section will focus on
    • General aspects of vulvar carcinomas
    • Rare, unusual type of carcinomas that affect the vulva
Epidemiology
  • Rare tumors, accounting for 3 - 5% of female genital tract malignancies
  • About 2/3 of the cases affect older women (age > 70 years)
  • Squamous cell carcinomas (SCC) are the most common type (~85%)
  • Also basal cell, melanoma, adenocarcinoma
Sites
  • Labia majora and bartholin glands are most frequently affected
Etiopathogenesis
  • Most SCCs are related to human papillomavirus (HPV) infection (90% are HPV 16, 18)
  • Other causes include:
    • Lichen sclerosus
    • Chronic vulvar irritation (e.g. long term use of diapers)
    • Exposure to ionizing radiation, arsenic
    • Immune deficiency
    • Presence of hamartomatous lesions
Clinical features
  • Most tumors are slow growing and are frequently painless, until the lesions ulcerate
  • Other symptoms include itching, discomfort, bleeding, mass or swelling of the vulva or the groin
Diagnosis
  • Except for some of the well differentiated, keratinizing and warty forms of SCC, most vulvar tumors look clinically similar to each other
  • Histopathologic examination is essential for diagnosis
Laboratory
  • Except for nonspecific findings such as anemia, mild leukocytosis and rarely thrombocytopenia, routine laboratory investigations are not very useful in the diagnosis of vulvar tumors
  • Serum tumoral markers such as AFP, CEA, CA125, CA15.3 and CA19.9 should be within normal limits
Radiology description
  • MRI, CT scan, PET and ultrasound are often used (AJR Am J Roentgenol 2013;201:W147)
    • To determine the extent of tumor involvement and presence of nodal metastases prior to therapy
    • To assess tumor response to chemotherapy, radiation or both and
    • For posttreatment surveillance
Radiology images

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Before and after chemo

Prognostic factors
Management
  • Management of primary tumor and nodal metastases may be independent
  • For early lesions (primary tumor < 2 cm and no inguinal node involvement), wide local resection is the treatment of choice, aimed at achieving at least 1 cm of clear peripheral margins
  • For primary lesions > 2 cm, radical local excision (to achieve 5 cm resection margin) may be necessary, with or without inguinofemoral lymphadenectomy
  • If 2 or more inguinal lymph nodes are involved or when there is extracapsular tumor spread, pelvic and groin radiation is frequently needed
  • If radiologic imaging shows involvement of the retroperitoneal lymph nodes, surgical removal of enlarged lymph nodes is recommended, followed by radiotherapy
  • Although chemotherapy is not commonly used to treat vulvar carcinoma, it is increasingly used in neoadjuvant and palliative settings (Int J Gynaecol Obstet 2012;119:S90, Eur J Gynaecol Oncol 2011;32:505, J Gynecol Oncol 2014;25:22, Int J Gynecol Cancer 2012;22:865, Curr Oncol Rep 2013;15:573)
Histologic examination
  • Use of CAP synoptic reporting has become routine: (CAP: Vulva Protocol [Accessed 6 October 2017])
  • Common histopathologic types include:
    • Squamous cell carcinoma, graded as
      • GX: grade cannot be assessed
      • G1: well differentiated
      • G2: moderately differentiated
      • G3: poorly or undifferentiated
    • Verrucous or warty carcinoma
    • Paget disease of vulva
    • Adenocarcinoma, not otherwise specified (NOS)
    • Basal cell carcinoma, NOS
    • Bartholin gland carcinoma
  • Rare types of vulvar carcinomas:
    • Adenocarcinomas most often arise from the Bartholin glands but may also originate in the cutaneous eccrine or apocrine glands, Skene glands, minor vestibular glands, ectopic mammary tissue or even endometriotic implants
Case reports (rare tumor types)
Clinical images

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Before and after treatment

Microscopic (histologic) description
  • Intestinal type adenocarcinoma or adenocarcinoma of cloacagenic origin
    • Tumor is usually a moderately differentiated adenocarcinoma with goblet cells and variable amounts of necrosis, reminiscent of colonic adenocarcinoma
  • Adenocarcinoma of ectopic breast tissue
    • Tumor is similar to the mammary counterparts
    • Could be in situ, invasive or both
Microscopic (histologic) images

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Adenocarcinoma, CK20

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Mucinous adenocarcinoma: ER, BRST1

Positive stains
Negative stains
Differential diagnosis
  • Intestinal type adenocarcinoma or adenocarcinoma of cloacagenic origin: adenocarcinoma arising from the gastrointestinal tract must be ruled out
  • Adenocarcinoma of ectopic breast tissue: nonneoplastic mammary tissue is present adjacent to the adenocarcinoma
  • Tumors with prominent keratinization are often confused with dermatitis or eczema