Vulva
Malignant neoplasms
Bartholin gland carcinoma

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

Revised: 5 October 2017, last major update March 2014

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

PubMed Search: "Bartholin's gland carcinoma" vulva

Cite this page: Nagarajan, P. Bartholin gland carcinoma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/vulvabartholinglandcarc.html. Accessed November 19th, 2017.
Definition / general
  • First documented in 1864
  • Often diagnosed at advanced stage due to late presentation and low clinical suspicion
  • Diagnostic criteria: (Obstet Gynecol 1972;39:489; Best Pract Res Clin Obstet Gynaecol 2003;17:609)
    • Compatible with origin from Bartholin gland, deep to the labia
    • Intact overlying skin
    • Transition between normal glandular tissue and carcinoma
    • No evidence of primary tumor elsewhere
Epidemiology
  • Mean age at diagnosis: 60 years (range 33 - 93 years)
  • Constitutes approximately 2 - 7% of vulvar and less than 1% of gynecologic malignancies (Surg Oncol 2013;22:117)
Sites
  • Posterolateral to labium majus, involving the lower part of the vulva
Pathophysiology
  • Human papillomavirus (HPV) infection has been identified as a major contributing factor in the development of squamous cell carcinoma of the Bartholin gland (Histopathology 2000;37:87)
Clinical features
  • Slow growing, painless, palpable or visible tumor posterior to the labium majus
  • Rarely, patients may experience rectal or vaginal pain and discomfort, bleeding (postcoital), dyspareunia and pruritus
Radiology description
Radiology images

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Transvaginal ultrasonography

Prognostic factors
  • Size and stage at the time diagnosis are the most important prognostic factors (J Clin Oncol 2008;26:884)
  • Larger size of the nodal metastasis and the presence of extracapsular invasion are also poor prognostic indicators (Gynecol Oncol 1992;45:313)
Case reports
Treatment
  • Surgical resection: (Surg Oncol 2013;22:117)
    • Tumors < 2 cm: hemivulvectomy
    • Tumors > 2 cm: total vulvectomy, with bilateral inguinal and femoral lymphadenectomy
    • Partial pelvic exenteration may be needed for large tumors
  • Adjuvant radiation therapy
Clinical images

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Irregular solid tumor with ulceration

Swelling on the right aspect of vulva

Gross images

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Bartholin gland tumor

Microscopic (histologic) description
  • Histology of the carcinoma varies based on site of origin
  • Squamous cell carcinomas (SCC) and adenocarcinomas are the most common malignant tumors arising from the Bartholin gland (~40% each), followed by adenoid cystic carcinoma (ACC, ~15%)
  • Other histological types (~5%) include:
    • Transitional cell carcinoma, adenosquamous carcinoma, poorly differentiated carcinoma, low grade epithelial - myoepithelial carcinoma, sarcoma, melanoma and clear cell carcinoma
  • Squamous cell carcinoma:
  • Adenocarcinoma:
    • Frequently arises at transition zone or from mucin producing acini (Jpn J Clin Oncol 2001;31:226)
    • Composed of columnar to cuboidal cells containing intracellular mucin
    • Pools of extravasated mucin may also be present
  • Adenoid cystic carcinoma:
    • May originate from myoepithelial cells
    • Tumor cells usually have low cytologic grade and are arranged in a cribriform pattern and the (pseudo) lumens are filled with mucin or hyalinized basement membrane material
    • Because of its tendency for extensive perineural invasion, ACC is associated with frequent local recurrence (Arch Gynecol Obstet 2008;278:473, Gynecol Oncol Case Rep 2012;4:16)
Microscopic (histologic) images

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Case of the Week #172:

Adenoid cystic carcinoma: H&E and c-kit



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Adenoid cystic carcinoma: H&E



Adenocarcinoma:

Well differentiated mucinous adenocarcinoma

CA19-9

CEA

p53

MIB1

Apocrine adenocarcinoma



Squamous cell carcinoma:

Squamous cell carcinoma - low power

Squamous cell carcinoma - medium power


Squamous cell carcinoma - high power


High molecular weight keratin

CK7

CK20

Positive stains
Negative stains
  • SCC: CEA, CK20
  • Adenocarcinoma: CEA
  • ACC: CK20
Electron microscopy description
Electron microscopy images

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Tumor cells undergoing central comedonecrosis

Dark and light secretory granules