Question

long answer of "location of brain/lungs/lacrimal gland"​

Answer 1

Adenoid cystic carcinoma of Bartholin’s gland is a very rare disease.

A 48-year-old premenopausal woman of Caucasian origin was delivered adjuvant pelvic and inguinal radiotherapy after prior complete left Bartholin’s gland tumor excision and inguinal lymph node dissection for adenoid cystic carcinoma of Bartholin’s gland with one metastatic inguinal lymph node.

Two years after primary treatment, she presented to the Emergency Room with acute headache, hypoacousia, decrease in visual acuity, and a decrease in right leg muscle strength. A cranial magnetic resonance imaging scan demonstrated three cystic brain lesions with associated perifocal edema. Chest and abdomen computed tomography scans and a magnetic resonance imaging scan of the pelvis did not find any metastatic or residual disease elsewhere. A physical examination found no local recurrence.

Bartholin’s gland carcinoma is a rare disease, accounting for 0.1% to 5% of all vulval carcinomas and representing barely 0.001% of gynecological cancers. Different histological variants have been described: adenocarcinomas, squamous cell carcinomas, transitional cell carcinomas and adenoid cystic carcinomas. The latter represent 10% to 15% of Bartholin’s gland tumors and arise usually from salivary, lacrimal and nasopharynx glands, and sometimes from mammary, skin and uterine cervix glands. The mean age at diagnosis is around 50 years with a range between 25 and 80 years. Distant metastasis has been found in only a few cases to the lungs, liver and brain. Here, we present the first case of Bartholin’s gland adenoid cystic carcinoma (ACC) with metastasis to the brain and lung.


Follow-up visits were organized every three months with CT scans of the chest, abdomen and pelvis performed every six months. In June 2011, two years after her primary treatment, she presented to the Emergency Room with acute headache, hypoacousia, decrease in visual acuity, and a decrease in right leg muscle strength.

A cranial MRI scan demonstrated three cystic brain lesions in the right parietal, left parieto-occipital and left temporal region with associated perifocal edema. Chest and abdomen CT scans and a MRI scan of the pelvis did not find any metastatic or residual disease elsewhere. A physical examination found no local recurrence.

Gadolinium-enhanced axial T1-weighted brain magnetic resonance imaging scans showing three metastatic cystic brain lesions in the right parieto-occipital and temporal regions with associated perifocal edema.

Stereotactic brain biopsies were undertaken. A pathology examination revealed the presence of ACC metastasis with characteristic tumor proliferation in a cribriform pattern composed of nests and columns of cells arranged concentrically around gland-like spaces filled with eosinophilic periodic acid-Schiff-positive material. These results were confirmed after immunological staining with specific antibodies, which showed that the brain lesions had similar characteristics as the initial ACC of Bartholin’s gland that was diagnosed two years earlier. Immunohistochemical characteristics were: cytokeratin (CK) AE1/AE3 positive, CK7 positive, epithelial membrane antigen (EMA) positive, S100 weakly positive, CK20 negative, estrogen receptor negative, progesterone receptor weakly positive.



Frontal and axial chest computed tomography scans showing adenoid cystic carcinoma metastasis (red arrow) in the right middle lobe of the lung.

An ACC of the Bartholin’s gland presents itself with similar symptoms as most vulval cancers. Patients usually complain of pruritus, a burning sensation and pain, which are explained by this slow-growing tumor’s tendency for local and perineural invasion. These symptoms can even be experienced before the physical appearance of any vulval mass. Among other nonspecific signs like bleeding, dyspareunia, and/or discharge from an abscess [4], the presence of an inguinal lymph node should be considered as being highly suspicious of malignancy