• Ning Zhou, Fanrong Wang, Li Yang, Qin Wang, Jun Liu, and Ying Chen.
• Department of Pathology, Sichuan Mianyang 404 Hospital, Mianyang, Sichuan Province, China.
• Medicine (Baltimore). 2023 Dec 15; 102 (50): e36624e36624.

IntroductionNeuroendocrine tumors usually arise from the gastrointestinal and pulmonary tracts and rarely from the skin. We report a unique case of high-grade neuroendocrine carcinoma with positive steroid hormone receptors in the primary skin of the groin.Case PresentationA 79-year-old female presented with a lump in her left inguinal region for 15 years that grew gradually. The tumor cells were arranged in sheets, solid nests, and bands within a rich network of thin-walled capillaries. Mucin was abundant in the stroma, and the tumor cells exhibited high-grade lesions, significant necrosis, and frequent mitosis, with small scattered foci of low-grade components. Immunohistochemistry revealed that the tumor cells diffusely and strongly expressed cytokeratin, synaptophysin, chromogranin A, GATA3, CAM5.2, and estrogen and progesterone receptors; partially expressed AR and GCDFP15.DiagnosisBased on pathological morphology, and immunohistochemical staining, it was confirmed as Primary high-grade neuroendocrine carcinoma with positive steroid hormone receptors arising in the inguinal skin. The patient underwent resection of the inguinal tumor and left inguinal lymph node dissection.InterventionsThe patient has been followed up for 16 months and has not undergone further examinations or received additional treatment. There is no evidence of tumor recurrence at the site of the original surgical resection, and the patient general condition is satisfactory.ConclusionsThe morphology of this tumor is unique and previously unreported, further expanding the possible pathogenesis and histological morphologies of this tumor type.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

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