Vulva, vagina & female urethra
Other carcinomas
Bartholin gland carcinoma

Editorial Board Member: Jennifer Bennett, M.D.
Editor-in-Chief: Debra Zynger, M.D.
Jutta Huvila, M.D.
C. Blake Gilks, M.D.

Topic Completed: 7 October 2020

Minor changes: 7 October 2020

Copyright: 2002-2020, PathologyOutlines.com, Inc.

PubMed Search: Bartholin gland[TI] carcinoma vulva

Jutta Huvila, M.D.
C. Blake Gilks, M.D.
Page views in 2019: 3,067
Page views in 2020 to date: 2,379
Cite this page: Huvila J, Gilks CB. Bartholin gland carcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/vulvabartholinglandcarc.html. Accessed October 28th, 2020.
Definition / general
  • Rare carcinoma arising from the Bartholin gland
  • These can be squamous cell carcinoma, adenocarcinoma, adenoid cystic carcinoma or other rare variants
Essential features
  • Carcinoma arising from Bartholin gland
  • Wide range of histologic types can be encountered but most are squamous cell carcinoma, adenocarcinoma or adenoid cystic carcinoma
  • Overlying epithelium should be uninvolved, i.e. exclude downgrowth of an overlying vulvar squamous cell carcinoma or Paget disease
  • Exclude metastasis to Bartholin gland from a genital or extragenital site
ICD coding
  • ICD-11: 2C70.Z & XH74S1 - malignant neoplasms of vulva, unspecified and adenocarcinoma, NOS
Epidemiology
  • Bartholin gland carcinomas arise in middle aged and elderly women and account for approximately 5% of vulvar malignancies (Surg Oncol 2013;22:117)
Sites
  • Diagnosis of Bartholin gland carcinoma can only be made if the anatomical location of the tumor is compatible with origin from Bartholin gland, i.e. posterolateral on the vulva (posterior to the labium major), without involvement of overlying squamous epithelium
Etiology
Clinical features
  • Painless mass in the region of Bartholin gland in an elderly woman
  • May be mistaken for a Bartholin gland cyst or abscess
Diagnosis
  • Biopsy of a mass in the region of Bartholin gland
Radiology description
Prognostic factors
  • Tumor size and stage are the most important, with prognosis similar to that of vulvar squamous cell carcinoma (Surg Oncol 2013;22:117, J Clin Oncol 2008;26:884)
  • Overall prognosis is favorable, especially for tumors localized to the vulva, without nodal metastasis
Case reports
Treatment
  • Surgical excision, (vulvectomy with unilateral or bilateral lymphadenectomy) often with adjuvant radiotherapy administered (similar to treatment of vulvar squamous cell carcinoma)
Gross description
  • Firm, irregularly shaped, unencapsulated mass deep to the vulvar skin
Frozen section description
  • Frozen section findings are not specific and are dependent on the tumor histologic type (e.g. squamous cell carcinoma, adenocarcinoma, adenoid cystic carcinoma)
  • Frozen section is typically not required as diagnosis will have been made based on preoperative biopsy
Microscopic (histologic) description
  • Approximately 85 - 90% of Bartholin gland carcinomas are squamous cell carcinomas, approximately 10% adenocarcinomas and < 5% adenoid cystic carcinomas (Gynecol Oncol 2001;82:247, Int J Gynecol Cancer 2016;26:785, Int J Gynecol Pathol 2019;38:189)
  • Additionally, several rare to exceedingly rare histological subtypes have been described, such as transitional cell carcinoma, neuroendocrine carcinoma, epithelial myoepithelial carcinoma, Merkel cell carcinoma and lymphoepithelioma-like carcinoma (Crit Rev Oncol Hematol 2017;117:1)
  • Histopathologic diagnostic criteria include the following (Obstet Gynecol 1972;39:489):
    • Tumor involves the anatomic region of the Bartholin gland and is histologically compatible with origin from the Bartholin gland
    • Areas of apparent transition from normal Bartholin gland elements to neoplastic ones are present
    • There is no evidence of primary tumor elsewhere
  • Histological features
    • Squamous cell carcinoma:
    • Adenocarcinomas:
      • Nonspecific features (adenocarcinoma NOS) are most common
      • May show mucinous differentiation or other growth patterns
    • Adenoid cystic carcinoma:
Microscopic (histologic) images

Contributed by Mehrane Nazeran, M.D. and Hugo Horling, M.D.
Papillary architecture Papillary architecture

Papillary architecture

p16 p16 p16

p16


Infiltrating carcinoma

Infiltrating carcinoma

Solid and glandular pattern

Solid and glandular pattern

Cribriform growth

Cribriform growth

Glandular pattern

Glandular pattern

Cytology description
  • Cytological examination is typically not used in diagnosis
  • It may be used to confirm lymph node metastasis
  • Cytological features reflect the histologic type of the carcinoma
Negative stains
Molecular / cytogenetics description
  • Adenoid cystic carcinomas show chromosomal rearrangements involving NFIB (also seen in adenoid cystic carcinomas arising at other anatomic sites) in most cases (Int J Gynecol Pathol 2017;36:289)
Sample pathology report
  • Vulva, biopsy:
    • Invasive squamous cell carcinoma, consistent with Bartholin gland carcinoma (see comment)
    • Comment: This squamous cell carcinoma shows strong diffuse p16 immunoreactivity, consistent with it being associated with high risk human papillomavirus. The clinical setting, i.e. location of the tumor mass and uninvolved overlying vulvar skin, is noted. While the histologic type of this tumor is compatible with it being a primary Bartholin gland carcinoma and there are adjacent benign Bartholin gland acini in this biopsy, clinical correlation is required to exclude a metastatic squamous cell carcinoma from another site.
Differential diagnosis
Board review style question #1

    Which of the following is true about squamous cell carcinoma of the Bartholin gland?

  1. Has a significantly worse prognosis than other vulvar squamous cell carcinomas
  2. Is often associated with human papillomavirus infection
  3. Is the second most common histological subtype of Bartholin gland carcinoma after adenocarcinoma
  4. Often presents with a painful mass similar to Bartholin gland abscess
Board review answer #1
B. Is often associated with human papillomavirus infection

Comment Here

Reference: Bartholin gland carcinoma
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