BACKGROUND Eccrine porocarcinoma (EPC) is a rare cutaneous neoplasm with high potential for morbidity and mortality. Due to its rarity, there is a paucity of data profiling diagnosis, work-up, and management. OBJECTIVE To consolidate reported information on demographics, diagnostics, clinical behavior, treatment modalities, and patient outcomes in EPC to provide a reference tool to optimize diagnosis and management. METHODS A comprehensive PubMed search was performed from 1963 to November 2017 using PRISMA guidelines. This yielded 155 articles detailing 206 cases of porocarcinoma. RESULTS Eccrine porocarcinoma most often presents in elderly patients on the head and neck or lower limbs. Metastatic disease at presentation is not uncommon (22%). Primary tumor location is significantly correlated with presence of metastasis (p = .038). The most common treatment is excision followed by Mohs micrographic surgery (MMS), although the outcomes after MMS were superior to those after surgical excision. CONCLUSION This systematic review of individual patient data reveals that all patients should have a histological diagnosis with imaging considered for high-risk cases. Primary tumor location should also be considered in diagnostic and therapeutic decision-making. Although wide local excision (WLE) is currently the first-line treatment, MMS is becoming increasingly used, with evidence indicating improved outcomes as compared to those seen with WLE.
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