Title: Multiple Plexiform Neurofibromas in Neurofibromatosis Type 1: A Case Report
Authors: Dr Meena Chauhan, Dr G R Tegta, Dr Renu Rattan, Dr G K Verma
DOI: https://dx.doi.org/10.18535/jmscr/v6i2.04
Abstract
Plexiform neurofibromas represent an uncommon variant (30%) of neurofibromatosis type 1 (NF-1). They account for substantial morbidity including disfigurement, functional impairment and may even be life threatening. More than one plexiform neurofibromas are uncommon in an individual of NF-1. We herewith, report a case of 50 years old female with NF 1 with multiple large plexiform neurofibromas over back, abdomen and left leg that began in early adulthood. Diagnosis of plexiform neurofibromas is usually made clinically, especially if classical signs of NF-1 are present. More than two diagnostic criteria for NF-1 were met, however in our case, the patient did not accept to undergo investigative work up and surgical resection and preferred to undergo clinical follow-up every 6 months. The current mainstay of the treatment of plexiform neurofibromas is surgical resection than medical therapy.
Keywords: Plexiform neurofibromas, Neurofibromatosis type 1, café-au-lait macules.
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