Aggressive digital papillary adenocarcinoma (Aggressive digital papillary adenoma and adenocarcinoma revisited)

In 1987 a clinicopathologic study by the Armed Forces Institute of Patholog y (AFIP) of rare sweat gland tumors, termed aggressive digital papillary ad enoma and adenocarcinoma, was published. Since that time, the AFIP has cont inued to collect these turners for study. Based on additional follow-up dat a, we think the original classification of these tumors requires revision. Sixty-seven cases of aggressive digital papillary adenoma and adenocarcinom a were studied according to their clinical characteristics and histologic f eatures. Fifty of these were originally diagnosed as adenoma and 17 as aden ocarcinoma. Follow up on 45 (67%) of the patients was obtained. None of the clinical or histologic parameters studied were found to be predictive of r ecurrence or metastasis, indicating that the originally proposed criteria f or distinguishing between benign (adenoma) and malignant (adenocarcinoma) d o not predict biologic behavior. When primary tumors were treated by subseq uent reexcision or amputation, only one recurred (5%), when not so treated, 11 recurred (50%) regardless of the original diagnosis (p <0.05), Metastas is occurred in six (14%) cases and in three cases led to the death of the p atient. Three of these metastatic cases had met the earlier criteria for ad enoma. Pulmonary metastases were observed in five cases. No effective treat ment for widespread metastatic disease has yet been developed. Because hist ologic features with prognostic significance could not be demonstrated in t his retrospective review, we propose that all aggressive digital papillary tumors be designated aggressive digital papillary adenocarcinoma.