Adenoid Cystic Carcinoma of the Bartholin’s Gland: A Diagnostic Dilemma

Abstract

Adenoid cystic carcinomas of the Bartholin’s gland are extremely rare and are often misdiagnosed. There are currently no definite treatment guidelines. This article describes the case of a 33-year-old female who was managed at our centre for adenoid cystic carcinoma of the Bartholin’s gland. She presented with a prolonged history of a vulvar lesion which was eventually diagnosed as adenoid cystic carcinoma of the Bartholin’s gland. She was subsequently treated with wide local excision of the primary and inguinal lymph node dissection followed by adjuvant radiotherapy and chemotherapy. She had gross perineural invasion on MRI imaging. The present case highlights the diagnostic dilemma in this extremely rare cancer and the literature further explores the natural history and treatment options.

Adenoid Cystic Carcinoma of Bartholin’s Gland: A Case Report with Emphasis on Surgical Management

“…The mean age at diagnosis of a primary carcinoma of Bartholin’s gland is 50–60 years, with a range of 33–93 years [ 10 ]. Symptoms are nonspecific and include pain, swelling of the vulva, foreign-body or burning sensation, dyspareunia, feeling of pressure, or even abnormal bleeding [ 2 – 5 , 11 , 13 ]. Misdiagnosis is common, as these tumors mimic cysts or abscesses of Bartholin’s gland or sometimes even endometriosis, leading to a delay in diagnosis and treatment [ 3 , 5 , 7 , 11 , 13 ].…”

“…The most common sites of distant metastasis are the lungs. However, metastasis to the bones, the liver, and the brain have been reported [ 2 , 3 , 5 – 7 , 12 , 15 ].…”

“…Radiotherapy has been used as an adjuvant treatment for ACC of Bartholin’s gland. It is particularly recommended when resection margins are positive or there is perineural invasion [ 2 , 5 – 7 , 13 , 15 – 17 ]. Bernstein et al suspected that ACC of Bartholin’s gland may be less sensitive to radiation therapy, because all of their reported patients with positive surgical margins died due to distant metastasis despite receiving adjuvant radiation treatment [ 15 ].…”

“…Of all the possible histological subtypes of Bartholin’s gland carcinoma, the adenoid cystic carcinoma (ACC) of the Bartholin’s gland constitutes only about 10–15% and thus is an absolute rarity among gynecological tumors [ 1 – 7 ]. No more than approximately 350 cases have been reported in the literature [ 1 , 2 ].…”

“…Typically, ACCs occur in head and neck sites, such as the salivary glands, the lacrimal glands, the sinonasal region, and the pharynx, but they are also known to arise from other exocrine glands in rarer sites like the skin, the esophagus, the breast, or the uterine cervix [ 2 , 4 , 8 , 9 ]. ACCs of the head and neck region are typically characterized by slow, pervasive growth with often extensive perineural invasion, a tendency for local recurrences and distant metastasis, and are associated with poor prognosis [ 8 , 9 ].…”