医学
丛状神经纤维瘤
神经纤维瘤病
组织病理学
神经纤维瘤
病理
神经鞘瘤
放射科
作者
Aditya Sriharsha Pedaprolu,Rajesh Gattani,Suhas Jajoo,Venkatesh Rewale,Swati Deshpande,Priya Chatterjee,Mehak Fayyaz Semy
摘要
Abstract Plexiform neurofibroma (PF) is a rare benign variant belonging to a subtype of neurofibromatosis type 1 that forms bulging or deforming masses arising from the peripheral nerve sheath. These masses involve surrounding connective tissue or dermal layers, leading to multiple cutaneous changes and certain characteristic appearances. It is these appearances that aid in the diagnosis of PF. We have encountered two distinct patients diagnosed with this disorder. While one patient was clinically and pathologically confirmed for PF, the other had no characteristic cutaneous changes. The diagnosis was made with postoperative histopathology and confirmed with an immunohistochemical examination. There are various modalities in the management of PFs, with surgery being a mainstay in the treatment of disfiguring large PFs, especially in resource-restrained settings. In view of high recurrence rates, postoperative clinical follow-up is a must. This paper describes these patients’ typical and atypical clinical presentation and subsequent management.
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