Plexiform’s perplexities: a tale of two plexiform neurofibromas

• Published in: Journal of surgical case reports Vol. 2024; no. 8; p. rjae486
• Main Authors: Pedaprolu, Aditya Sriharsha, Gattani, Rajesh, Jajoo, Suhas, Rewale, Venkatesh, Deshpande, Swati, Chatterjee, Priya, Semy, Mehak Fayyaz
• Format: Journal Article
• Language: English
• Published: England Oxford University Press 01.08.2024
• Subjects: Case Report; neurofibromatosis; neurofibroma; von Recklinghausen’s disease; nerve tumor; café-au-lait macules; plexiform neurofibroma
• ISSN: 2042-8812; 2042-8812
• DOI: 10.1093/jscr/rjae486

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.