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1型神经纤维瘤病患者发生神经内分泌肿瘤的罕见病例:二者是否存在关联?

A Rare Case of Neuroendocrine Tumor in a Patient With Neurofibromatosis Type 1: Is There Any Association?

作者信息

Baddi Fatima Zahra, Ahsayen Fatima Zohra, Ramdani Hiba, Rhazari Meriem, Skiker Imane, Thouil Afaf, Kouismi Hatim

机构信息

Department of Respiratory Diseases, Mohammed VI University Hospital, Oujda, MAR.

Department of Pulmonology, Faculty of Medicine and Pharmacy, Mohammed I University, Oujda, MAR.

出版信息

Cureus. 2022 Sep 26;14(9):e29621. doi: 10.7759/cureus.29621. eCollection 2022 Sep.

Abstract

Neurofibromatosis type 1 (NF1) is an autosomal dominant condition characterized by café-au-lait spots, cutaneous neurofibromas, axillary and inguinal freckling, and iris Lisch nodules; however, the presentations vary greatly, even within families. NF1 is also a recognized risk factor for the development of malignancy particularly malignant peripheral nerve sheath tumors (MPNST), optic gliomas, other gliomas, and leukemia. Nevertheless, the occurrence of lung cancer in a patient with neurofibromatosis type 1 is a rare phenomenon. Here we present a case of neuroendocrine tumor in a patient with neurofibromatosis type 1, highlighting the association between the two diseases. This case report also aimed to raise awareness of possible malignancies in patients with neurofibromatosis type 1.

摘要

1型神经纤维瘤病(NF1)是一种常染色体显性疾病,其特征为咖啡斑、皮肤神经纤维瘤、腋窝和腹股沟雀斑以及虹膜Lisch结节;然而,即使在家族内部,其临床表现也差异很大。NF1也是发生恶性肿瘤尤其是恶性外周神经鞘瘤(MPNST)、视神经胶质瘤、其他胶质瘤和白血病的公认风险因素。然而,1型神经纤维瘤病患者发生肺癌是一种罕见现象。在此,我们报告一例1型神经纤维瘤病患者发生神经内分泌肿瘤的病例,强调这两种疾病之间的关联。本病例报告还旨在提高对1型神经纤维瘤病患者可能发生的恶性肿瘤的认识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/945b/9604765/0653259243e5/cureus-0014-00000029621-i01.jpg

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