| Home > Publications database > S1 guideline sweat gland carcinoma. [S1‐Leitlinie Schweißdrüsenkarzinom.] |
| Journal Article | DKFZ-2026-02296 |
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2026
Wiley-Blackwell
Berlin
Abstract: The current classification is based on histomorphological characteristics and dis-tinguishes between more than 20 entities. Most patients are older, but somesubtypes also affect middle-aged and younger patients. The majority of tumorsarise de novo. Sweat gland carcinomas have nonspecific clinical features. Thetumors are usually located in the head and neck area and on extremities, withthe exception of extramammary Paget’s carcinoma, which has an anogenitalpredilection. Sweat gland carcinomas occurring in the armpit may pose a histo-morphological challenge in distinguishing them from metastatic or primarybreast cancer. The diagnosis is made histopathologically via excisional biopsy.Histopathological subdifferentiation is essential for an accurate diagnosis. Meta-stasis initially occurs locally (per continuitatem), later to regional lymph nodesand distant organs. The treatment of choice is complete surgical excision withentire histopathological margin control (microscopically controlled surgery) oralternatively with wide local excision with a safety margin. Postoperative adju-vant radiotherapy is recommended for high-risk tumors. The data available fordrug therapy of advanced tumors is generally weak, with the best results seenfor chemotherapy using platinum derivatives. Furthermore, targeted therapies arepossible, e.g., with HER2/neu- or EGFR-inhibitors, possibly in combination withchemotherapy. A risk-adapted regimen is recommended for the follow-up care.
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