Brain and lung metastasis of Bartholin’s gland adenoid cystic carcinoma: a case report

Abstract

IntroductionAdenoid cystic carcinoma of Bartholin’s gland is a very rare disease.Case presentationA 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.Stereotactic brain biopsies with pathology examination revealed the presence of adenoid cystic carcinoma metastasis. She thus received 30Gy of brain radiotherapy but, three months later, the brain lesions did not decrease in size and left mid lobular lung lesions appeared on her chest computed tomography scan. A mid left lobe lung excision was undertaken followed by chemotherapy consisting of six cycles of cyclophosphamide, adriamycin and cisplatin. Five months after beginning chemotherapy, the brain disease progressed and our patient died.ConclusionOur case report shows the difficulty in managing brain and lung metastasis of Bartholin’s gland adenoid cystic carcinoma as no consensus on the optimal treatment exists.

Adenoid Cystic Carcinoma of Vulva-11 Years’ Single-Institution Experience

“…Chemotherapy with 5-fluorouracil, cyclophosphamide, methotrexate, doxorubicin, dactinomycin, irinotecan, have been tried in individual cases, but the efficacy of each is difficult to assess due the rarity of this condition and relatively long survival without treatment. Ramanah et al [11] described a patient with confirmed brain and lung metastasis from ACC-vulva, treated with brain radiotherapy, lobectomy and chemotherapy with cyclophosphamide, adriamycin and cisplatin whose disease progressed to death.…”

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

“…The etiology of ACCBG is unknown, though a series of ACCBG cases by Copeland has suggested that pregnancy may be an independent risk factor [11]. ACCBGs can present as both painless and painful masses [10, 12] and are usually solitary and unilateral. Clinically, they are associated with pruritus and a burning sensation, which reflects its nature to invade the perineum but has also presented with bleeding, dyspareunia, and discharge from abscesses [13].…”

A Case of Adenoid Cystic Carcinoma Mimicking a Bartholin Cyst and Literature Review

“…Immunohistochemical staining may reveal low-molecular-weight keratins, carcinoembryonic antigen, lysozyme, alpha-antichymotrypsin, S100, and type IV collagen [ 11 ]. Ramanah et al took a step further by performing specific antibody stains of brain metastases from BG-ACC, which revealed positive cytokeratin (CK) AE1/AE3, CK7, and epithelial membrane antigen (EMA); weakly positive S100 and progesterone receptor; and negative CK20 and estrogen receptor [ 10 ].…”

“…Bones and lungs are the most common site of metastasis; other sites reported are liver, kidney, and brain [ 25 ]. Late recurrences and metastases are associated with poor prognosis [ 9 , 10 ].…”