A Hidden Rarity: Incidental Posterior Mediastinal Neurofibroma with Middle Mediastinal and Lung Parenchymal Extension in a 20-year-old Male with Neurofibromatosis Type 1
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Date
2024-12
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Jaypee Brothers Medical Publishers Pvt. Ltd.
Abstract
Neurofibromatosis type 1 (NF1) is a rare genetic disorder characterized by multiple benign tumors of the nerves and skin, known as neurofibromas. Mediastinal involvement in NF1, particularly with anatomical extension to both posterior and middle mediastinum along with extension into lung parenchyma, is an uncommon occurrence. Here, we present the case of a 20-year-old male with a known history of NF1 who was incidentally found to have a mediastinal mass during a pre-surgical fitness evaluation. Histopathological analysis confirmed the mass to be a neurofibroma. This case highlights the importance of considering neurofibromas in the differential diagnoses of mediastinal masses in patients with NF1, even when they are asymptomatic. The potential risk for malignant transformation into a malignant peripheral nerve sheath tumor (MPNST) warranted surgical intervention and hence the patient was referred to a thoracic surgeon.
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Keywords
Asymptomatic mediastinal mass, Case report, Lung parenchymal involvement, Mediastinal neurofibroma, Multidisciplinary management, Neurofibromatosis type 1, Plexiform neurofibroma, Rare presentation, Thoracic neurofibroma
Citation
Paul B, Upadhya P, Nadaf Z, Ganesh RN.. A Hidden Rarity: Incidental Posterior Mediastinal Neurofibroma with Middle Mediastinal and Lung Parenchymal Extension in a 20-year-old Male with Neurofibromatosis Type 1 . The Indian Journal of Chest Diseases and Allied Sciences. 2024 Dec; 66(4): 148-151