Malignant peripheral nerve sheath tumour presenting as pathological fracture of proximal femur in neurofibromatosis type-1: a case report with brief literature search.

IF 1.4 Q3 EMERGENCY MEDICINE International Journal of Burns and Trauma Pub Date : 2024-08-25 eCollection Date: 2024-01-01 DOI:10.62347/IGKP3358
Mohd Hadi Aziz, Yasir Salam Siddiqui, Mohd Owais Ansari, Ahmad Masood Aziz, Adnan Anwer, Asad Khan, Mohd Julfiqar, Mohd Adnan, Mohammad Ibran
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Abstract

Neurofibromas, in association with NF-1, can undergo a malignant transformation, giving rise to Malignant peripheral nerve sheath tumours (MPNSTs), a relatively rare entity. Clinically, it presents with non-specific symptoms like pain and numbness, distinguishing it from other nerve lesions difficult. There is a lack of data on the occurrence of MPNST in NF-1 in children and adults, and distant metastasis to the brain and bones is reported only in a few cases. In this case report, we present the unusual presentation of MPNST with metastasis to the proximal femur and liver in a patient with NF-1 and its management. Patients and clinicians should be made aware of the relatively high risk of MPNST in NF-1, which is characterized by pain and rapid growth, and regular follow-up is needed for NF-1 patients for early diagnosis of malignant transformation. So, this case report is presented to enhance the understanding and awareness regarding this rare presentation.

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恶性周围神经鞘瘤表现为 1 型神经纤维瘤病股骨近端病理性骨折:病例报告及简要文献检索。
神经纤维瘤与 NF-1 相关联,可发生恶性转化,形成恶性周围神经鞘瘤(MPNST),这是一种相对罕见的实体肿瘤。在临床上,它表现为疼痛和麻木等非特异性症状,很难将其与其他神经病变区分开来。目前还缺乏关于儿童和成人 NF-1 中枢神经鞘瘤发生率的数据,仅有少数病例报告了向脑和骨骼的远处转移。在本病例报告中,我们介绍了一名 NF-1 患者 MPNST 转移至股骨近端和肝脏的异常表现及其处理方法。患者和临床医生应认识到,NF-1 患者发生 MPNST 的风险相对较高,其特点是疼痛和生长迅速,因此需要对 NF-1 患者进行定期随访,以早期诊断恶性转化。因此,本病例报告旨在提高人们对这种罕见表现的理解和认识。
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