S1 guideline sweat gland carcinoma

The current classification of sweat gland carcinomas is based on histomorphological characteristics and distinguishes between more than 20 entities. Most patients are older, but some subtypes also affect middle-aged and younger patients. The majority of tumors arise de novo. Sweat gland carcinomas have nonspecific clinical features. The tumors are usually located in the head and neck area and on extremities, with the exception of extramammary Paget's carcinoma, which has an anogenital predilection. Sweat gland carcinomas occurring in the armpit may pose a histomorphological challenge in distinguishing them from metastatic or primary breast cancer. The diagnosis is made histopathologically via excisional biopsy. Histopathological subdifferentiation is essential for an accurate diagnosis. Metastasis initially occurs locally (per continuitatem), later to regional lymph nodes and distant organs. The treatment of choice is complete surgical excision with entire histopathological margin control (microscopically controlled surgery) or alternatively with wide local excision with a safety margin. Postoperative adjuvant radiotherapy is recommended for high-risk tumors. The data available for drug therapy of advanced tumors is generally weak, with the best results seen for chemotherapy using platinum derivatives. Furthermore, targeted therapies are possible, e.g., with HER2/neu or EGFR inhibitors, possibly in combination with chemotherapy. A risk-adapted regimen is recommended for the follow-up care.