| Summary: | Pain is considered as a vital parameter for any patient management. Type I neurofibromatosis (NF-1) is an autosomal dominant neurocutaneous disorder caused by loss-of-function mutations in the NF-1 gene. The presence of severe pain is an atypical feature of NF-1, and the possibility of malignant transformation with the development of a malignant peripheral nerve sheath tumor (MPNST) should be considered in such situation. These tumors are universally foreshadowed by the onset of severe pain. The present treatment options that can be used in the management of these patients have not been well described in the available literature. We report a case of NF-1 with atypical pain, its malignant transformation, and its management. This case report is unique as it attempts to correlate the mechanism and target therapeutic options in the management of pain in patients of NF with MPNST. We conclude by emphasizing that various available options for management of pain should be employed after understanding the pathophysiology behind the occurrence of pain in a clinical setting. Treatment of malignancy and management of pain should be done simultaneously.
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