General
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• 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
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• 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
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• Posterolateral to labium majus, involving the lower part of the vulva

Pathophysiology
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• Human papilloma virus infection has been identified as a major contributing factor in the development of squamous cell carcinoma of the Bartholin’s gland (Histopathology 2000;37:87)

Clinical features
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• 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
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• Ultrasound: (J Diag Med Son 2010;26:296)
  • Echogenic mass that may contain calcifications
• MRI: (Surg Oncol 2013;22:117)
  • T1-weighted: mass isotense to skeletal muscle
  • T2-weighted: enhancement with Gadolinium
• CT scan: (Surg Oncol 2013;22:117)
  • Indicated for any vulvar mass more than 2 cm in size
  • Also useful in detecting possible nodal spread
• F-18 FDG PET-CT: (Clin Nucl Med 2007;32:498)
  • Though the role of a PET-CT is not clear, it is essential for staging

Radiologic images
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Transvaginal ultrasonography

Prognostic factors
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• 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
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• 36 year old woman with adenoid cystic carcinoma of the Bartholin's gland (Eur J Gynaecol Oncol 2009;30:686)
• 49 year old woman with Merkel cell carcinoma of the Bartholin's gland (Gynecol Oncol 2005;97:928)
• 54 year old woman with adenoid cystic carcinoma of Bartholin's gland (Gynecol Obstet Invest 2005;59:54)
• 64 year old woman with adenoid cystic carcinoma of the Bartholin's gland (Arch Gynecol Obstet 2008;278:473)
• 68 year old woman with chemoradiotherapy with irinotecan (CPT-11) for adenoid cystic carcinoma of Bartholin's gland (Gynecol Oncol Case Rep 2012;4:16)
• 73 year old woman with vulvar mass (Case of the Week #172)
• 92 year old woman with mucinous adenocarcinoma of Bartholin gland treated with radiation therapy (Jpn J Clin Oncol 2001;31:226)
• Bartholin's gland squamous cell carcinoma (J Obstet Gynaecol 2012;32:318)

Treatment
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• 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's gland tumor

Micro description
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• 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’s 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:
  • Usually arises from vestibular duct orifice or at transition zone (J Obstet Gynaecol 2012;32:318)
  • Human papilloma virus infection has been identified as a major contributing factor in its carcinogenesis (Am J Pathol 1993;142:925; Int J Gynecol Pathol 2009;28:497; Histopathology 2000;37:87)
  • Usually well differentiated, composed of polygonal eosinophilic, keratinizing cells with intercellular bridges
  • Keratin pearls are frequently noted in well differentiated tumors
• 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)

Micro images
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H&E

       

Case of the Week #172: H&E and c-kit

Well differentiated mucinous adenocarcinoma

CA19-9

CEA

p53

MIB1

Apocrine adenocarcinoma

 

Squamous cell carcinoma-low power

   

Squamous cell carcinoma-medium power

     

Squamous cell carcinoma-high power

High molecular weight keratin

CK7

CK20

Positive stains
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• SCC: p63, HMCK, CK7
• Adenocarcinoma: CEA, mucin
• ACC: CEA, CD117, PAS-D; myoepithelial cells stain for S100, SMA and p63

Negative stains
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• SCC: CEA, CK20
• Adenocarcinoma: CEA
• ACC: CK20

Electron microscopy description
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• Not commonly performed; usually only to confirm organ of origin rather than type of tumor (Indian J Pathol Microbiol 2010;53:171)

Electron microscopy images
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Tumor cells undergoing central comedo-necrosis

Dark and light secretory granules

Differential diagnosis
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• Clinical differential diagnosis:
  • Angiomyofibroblastoma (Obstet Gynecol Sci 2013;56:349)
  • Bartholin gland cyst
  • Sarcoma botryoides
  • Superficial angiomyxoma (J Cutan Pathol 2010;37:672)
  • Vulvar hypertrophy with lymphedema (Arch Pathol Lab Med 2000;124:1697)

Additional references
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• Int J Gynecol Cancer 2006;16:1469, Int J Gynecol Pathol 2009;28:286, Rare Tumors 2013;5:e40

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