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十二指肠神经内分泌肿瘤、腺癌及胃肠道间质瘤合并1型神经纤维瘤病:一种罕见情况。

Duodenal neuroendocrine tumor, adenocarcinoma and gastrointestinal stromal tumor in association with neurofibromatosis type 1: An unique occurrence.

作者信息

Shamila M A, Reddy P Somasekhara

机构信息

Department of Pathology, Sagar Hospital, Bengaluru, Karnataka, India.

出版信息

Indian J Pathol Microbiol. 2018 Jul-Sep;61(3):401-403. doi: 10.4103/IJPM.IJPM_534_17.

Abstract

Mixed endocrine-nonendocrine neoplasms are rare tumors and pose challenges in diagnosis and management. Neurofibromatosis-1 (NF-1) patients are known to develop malignancies. Although neuroendocrine tumors and gastro-intestinal stromal tumors are seen in NF-1, its association with adenocarcinoma is rare. The existence of these three entities with NF-1 is extremely rare. NF1 patients presenting with symptoms suggestive of malignancies should alert the physician for a detailed clinical work up. Diagnosis of these malignancies often requires a meticulous histopathological and immunohistochemical examination. We report the first case from Indian experience.

摘要

混合性内分泌-非内分泌肿瘤是罕见肿瘤,在诊断和管理方面存在挑战。已知1型神经纤维瘤病(NF-1)患者会发生恶性肿瘤。虽然在NF-1患者中可见神经内分泌肿瘤和胃肠道间质瘤,但其与腺癌的关联罕见。这三种实体与NF-1同时存在极为罕见。出现提示恶性肿瘤症状的NF1患者应提醒医生进行详细的临床检查。这些恶性肿瘤的诊断通常需要细致的组织病理学和免疫组织化学检查。我们报告了来自印度经验的首例病例。

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