BACKGROUND: Syringoid eccrine carcinoma (SEC) is an extremely rare malignant adnexal neoplasm derived from eccrine sweat glands, of unknown pathogenesis. We report a case of this rare entity presenting in the abdomen, which is the only one reported in this area and the only case of SEC in a patient with so many comorbidities.

CASE REPORT: A 58-year-old black male from Brazil reported a nodular lesion in the abdomen with a progressive increase in size and pain and local burning sensation. The histopathological examination showed a syringoid eccrine carcinoma.

CONCLUSIONS: We present a rare case of SEC and did an extensive literature review in order to describe the clinical characteristics, histopathological findings, immunohistochemical profile, treatments, and difficulties found in the diagnosis of this tumor. To avoid misdiagnosis, we gave special attention to biopsy quality.

Keywords: Carcinoembryonic Antigen, Diagnosis, Differential, Immunohistochemistry, Sweat Gland Neoplasms, Abdominal Wall, Carcinoma, Eccrine Glands, Rare Diseases, Syringoma