INTRODUCTION
Endocrine Mucin Producing Sweat Gland Carcinoma (EMPSGC) is a rare, low-grade neuroendocrine cutaneous adnexal neoplasm. This tumor is more common in women than men, and the median age at presentation is 70 years.1 It predominantly involves the eyelids but can occur in other areas of the body. EMPSGC is thought to be a precursor of primary mucinous carcinoma (PCMC), and these two neoplasms share several histologic and immunologic characteristics.1-3 On histopathology, EMPSGC can resemble ductal carcinoma in situ and mucinous carcinoma of the breast.1,4 The tumor appears as a well-circumscribed dermal proliferation of neoplastic cells that may be cystic, multinodular, and express neuroendocrine markers. Papillary patterns and cribriform patterns, in which the cells of the tumor grow in a pseudorosette or lacelike pattern, are often observed.1,5 Clinically, they typically present as an asymptomatic and slow-growing nodule or papule. These tumors can morphologically resemble hidrocystomas and basal cell carcinoma, as they may appear nodular, pearly, pink flesh-colored, or contain telangiectasias.1,6-8 In some cases, the lesions may mimic a chalazion in clinical appearance.9
EMPSGC is locally aggressive but typically carries a low potential for recurrence and/or metastasis.1,4 However, compared to other non-melanoma skin cancers, the rate of metastasis for EMPSGC is usually higher.9 Current management for EMPSGC usually includes a metastatic workup in addition to either surgical excision with margins greater than 5 mm or Mohs micrographic surgery (MMS). However, clinical guidelines for optimal treatment of EMPSGC remain unestablished.4 For the treatment of EMPSGC, there are limited studies that compare the recurrence rates between MMS and traditional surgical excision. The aim of this study was to perform a systematic review of the literature to identify cases where EMPSGC was treated with MMS or surgical excision and to compare their recurrence rates.
EMPSGC is locally aggressive but typically carries a low potential for recurrence and/or metastasis.1,4 However, compared to other non-melanoma skin cancers, the rate of metastasis for EMPSGC is usually higher.9 Current management for EMPSGC usually includes a metastatic workup in addition to either surgical excision with margins greater than 5 mm or Mohs micrographic surgery (MMS). However, clinical guidelines for optimal treatment of EMPSGC remain unestablished.4 For the treatment of EMPSGC, there are limited studies that compare the recurrence rates between MMS and traditional surgical excision. The aim of this study was to perform a systematic review of the literature to identify cases where EMPSGC was treated with MMS or surgical excision and to compare their recurrence rates.