Primary high-grade neuroendocrine carcinoma with positive steroid hormone receptors arising in the inguinal skin: A case report of An exceedingly rare entity

• Published in: Medicine (Baltimore) Vol. 102; no. 50; p. e36624
• Main Authors: Zhou, Ning, Wang, Fanrong, Yang, Li, Wang, Qin, Liu, Jun, Chen, Ying
• Format: Journal Article
• Language: English
• Published: United States Lippincott Williams & Wilkins 15.12.2023
• Subjects: Aged; Biomarkers, Tumor; Carcinoma, Neuroendocrine - pathology; Carcinoma, Neuroendocrine - surgery; Clinical Case Report; Female; Groin; Hormones; Humans; Neoplasm Recurrence, Local; Steroids
• ISSN: 0025-7974; 1536-5964
• DOI: 10.1097/MD.0000000000036624

Neuroendocrine 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. A 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. Based 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. The 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. The morphology of this tumor is unique and previously unreported, further expanding the possible pathogenesis and histological morphologies of this tumor type.