Clinicopathological Features and Immunohistochemical Phenotypes of Adenoid Cystic Carcinoma of the Uterine Cervix

  Objective  To investigate the clinicopathological and immunohistochemical features of adenoid cystic carcinoma (ACC) of the uterine cervix.

  Methods  Four cases who were diagnozed with ACC of the uterine cervix in the period from January 2003 to December 2013 were collected from the pathological databank and consultation database of Peking Union Medical Hospital. Immunohistochemical examination was conducted on the formalin-fixed, paraffin-embedded tissue specimens from the 4 patients. Clinical information, pathological features, treatment, and outcomes of these patients were summarized.

  Results  The average age of the 4 ACC patients was 61.5 years. The patients were mostly post-menopausal (3/4) and their chief complaint was vaginal bleeding (3/4). The tumors were primarily protruding masses (3/4). Histologically, 3 of the 4 cases were ACC combined with invasive squamous cell carcinoma, the other 1 was ACC alone. The growth pattern of ACC in all of the 4 cases was cribriform composed of luminal adenoepithelial and abluminal myoepithelial cells. Immunohistochemical findings supported the identification of 2 components in ACC:positive expression of CK7 in luminal adenoepithelial cells, and positive expressions of P63 and SMA in abluminal myoepithelial cells. C-MYB, a ACC-specific marker, was found with strong positive expression in all the 4 cases. Three of the 4 patients had total hysterectomy, the other 1 had conization. All were in stage I in terms of clinical staging. Postoperative radiotherapy was administered in all the 4 cases, combined with chemotherapy in 2. The mean follow-up period was 21.25 months, finding all the 4 patients in disease-free survival.

  Conclusions  ACC of the uterine cervix is a rare special type of adenocarcinoma, usually coexisting with other types of cervical tumors. The immunohistochemical findings in ACC of the uterine cervix are similar with ACC in other organs, but associated with poorer prognosis. Radiotherapy and chemotherapy after sugery is recommended. Early detection and treatment could improve survival.