Syringocystadenocarcinoma Papilliferum: Clinicopathologic Analysis of Ten Cases

Abstract

Background

Syringocystadenocarcinoma papilliferum (SCACP) is an exceedingly rare cutaneous adnexal neoplasm. We aimed to investigate the clinicopathologic and immunophenotypic features of SCACP, and to discuss the prognosis of this rare entity.

Method

We retrospectively collected clinical, pathological and follow-up data of 10 cases with SCACP.

Results

There were 8 males and 2 females, with ages ranging from 26 to 74 years. The chest was most frequently involved. Histologically, one case only demonstrated SCACP in situ, nine cases presented with variable invasive components of adenocarcinoma and/or squamous cell carcinoma in addition to areas of in situ. Apocrine differentiation with decapitation was evident in 4 cases and mucinous metaplasia was noted in 1 cases. P63 was positive in invasive squamous cell carcinoma, while CK7 was variably positive in invasive adenocarcinoma. Regional lymph node metastasis was confirmed by pathological examination in four patients. Follow-up was available for 9 patients, ranging from 3 months to 112 months. Three patients died of the disease within 1 year after recurrences.

Conclusions

Due to high rates of regional lymph node metastasis and mortality in our patients, clinical behaviour of SCACP seems to be more aggressive than that previously reported.

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