Adenoid Cystic Carcinoma of the Vulva: Report on a Rare Malignancy

Abstract

Background:

Adenoid cystic carcinoma (ACC) is a malignant neoplasm involving mainly the exocrine glands. The most common site of involvement is the head and neck region. However, the neoplasm can be seen rarely arising in the breast, lacrimal gland, trachea–bronchial tree, lungs, cervix, brain and paranasal sinuses. Bartholin's glands in the vulva are yet another infrequent site for this neoplasm.

Case:

A 45-year-old woman presented with complaints of a gradually increasing swelling in the right side of vulva for the last 7 years. She had no other associated symptoms. On examination, a firm nontender swelling was found beneath the right labia minora, measuring 3 × 3 cm. Serum tumor markers as well as computerized tomography of the chest and ultrasound of the abdomen were within normal limits. Based on the clinical impression of a Bartholin's cyst, simple excision was performed. Histopathologic examination revealed adenoid cystic carcinoma. Subsequently, a wide local excision was performed, which showed a positive deep resection margin. She then underwent a modified radical vulvectomy with inguino–femoral lymph-node dissection.

Results:

Following the modified radical vulvectomy, there was no evidence of the tumor in the vulvectomy specimen as well as the lymph nodes.

Conclusions:

Establishing an early, accurate tissue diagnosis of vulvar ACC is imperative so that appropriate surgical management can be performed promptly to prevent local recurrences or distant metastases from developing.