11 March 2010 - Case #172
All cases are archived on our website. To view them sorted by case number, diagnosis or category, visit our main Case of the Month page. To subscribe or unsubscribe to Case of the Month or our other email lists, click here.
Thanks to Dr. Semir Vranic, Creighton University Medical Center, Nebraska (USA), for contributing this case. This case was reviewed in May 2020 by Dr. Jennifer Bennett, University of Chicago and Dr. Carlos Parra-Herran, University of Toronto.
Case #172
Clinical history:
A 73 year old woman presented with a vulvar mass.
Microscopic images:
What is your diagnosis?
Diagnosis: Primary Bartholin gland adenoid cystic carcinoma
Discussion:
Adenoid cystic carcinomas arise in various sites, including the salivary glands, lacrimal glands, breast, lung and trachea. In the vulva, < 75 cases have been reported.
Histologically, they show cribriform patterns of cell nests and columns with bland cytologic features, surrounding gland-like spaces with eosinophilic material that is PAS+ diastase resistant (J Surg Oncol 1985;30:132). Perineural and vascular invasion are common. Tumor cells are immunoreactive for low molecular weight keratin, CEA, S100 and type IV collagen (Arch Pathol Lab Med 2007;131:796). Tumor cells have been reported to be immunoreactive for c-kit at other sites (Mod Pathol 2006;19:1351).
The traditional treatment is wide local excision or vulvectomy, with or without nodal dissection. However, surgery may be debilitating and is associated with local recurrence and metastases (Arch Gynecol Obstet 2009;279:747, J Neurosurg 2005;102:543, Eur J Gynaecol Oncol 2009;30:317). As a result, radiation or chemotherapy have been suggested as an alternative, although there is no widespread consensus on treatment (Int J Gynecol Cancer 2007;17:661, Gynecol Oncol 2006;100:422). In the case presented, the patient died a few years later from kidney and liver metastases.
All cases are archived on our website. To view them sorted by case number, diagnosis or category, visit our main Case of the Month page. To subscribe or unsubscribe to Case of the Month or our other email lists, click here.
Thanks to Dr. Semir Vranic, Creighton University Medical Center, Nebraska (USA), for contributing this case. This case was reviewed in May 2020 by Dr. Jennifer Bennett, University of Chicago and Dr. Carlos Parra-Herran, University of Toronto.
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(1) We are updating the Bladder and Breast-nonmalignant chapters.
(2) Visit us at USCAP, Booth #1303.
Visit and follow our Blog to see recent updates to the website.
Case #172
Clinical history:
A 73 year old woman presented with a vulvar mass.
Microscopic images:
c-kit
What is your diagnosis?
Click here for diagnosis and discussion:
Diagnosis: Primary Bartholin gland adenoid cystic carcinoma
Discussion:
Adenoid cystic carcinomas arise in various sites, including the salivary glands, lacrimal glands, breast, lung and trachea. In the vulva, < 75 cases have been reported.
Histologically, they show cribriform patterns of cell nests and columns with bland cytologic features, surrounding gland-like spaces with eosinophilic material that is PAS+ diastase resistant (J Surg Oncol 1985;30:132). Perineural and vascular invasion are common. Tumor cells are immunoreactive for low molecular weight keratin, CEA, S100 and type IV collagen (Arch Pathol Lab Med 2007;131:796). Tumor cells have been reported to be immunoreactive for c-kit at other sites (Mod Pathol 2006;19:1351).
The traditional treatment is wide local excision or vulvectomy, with or without nodal dissection. However, surgery may be debilitating and is associated with local recurrence and metastases (Arch Gynecol Obstet 2009;279:747, J Neurosurg 2005;102:543, Eur J Gynaecol Oncol 2009;30:317). As a result, radiation or chemotherapy have been suggested as an alternative, although there is no widespread consensus on treatment (Int J Gynecol Cancer 2007;17:661, Gynecol Oncol 2006;100:422). In the case presented, the patient died a few years later from kidney and liver metastases.
