Plexiform neurofibroma (PNF) is a rare variant of neurofibromatosis type1 (NF-1), which histopathologically, is a subtype of benign nerve sheath tumors, neurofibromas (NF). It develops as a result of proliferation in all parts of peripheral nervous system and can cause the functional damage, deformities, pain, considerable mortality, and morbidity and even the increasing risk of malignant transformation in some critical cases. Currently, the surgical intervention is the treatment of choice for PNF patients, which due to the tumor invasion, massive growth, and the chance of postoperative regrowth is not possible. The diagnosis of isolated tumor is an uncommon event. Considering the rarity of this neoplasm, herein, we describe a case of isolated PNF, so the purpose of this presenting is the rarity of recording. We describe a case of isolated plexiform neurofibroma presented with 7-year history of a slowly growing postauricular soft subcutaneous mass in a 14-year-old boy, which caused the right auricular deformity. After initial evaluation by imaging studies, the patient underwent to surgical resection of the mass and the diagnosis of plexiform neurofibroma was confirmed by histopathologic examination. Surgical excision of the mass had been done before which concluded the satisfactory result and based on oncologist diagnosis, further intervention such as radiotherapy or chemotherapy was not needed. The patient left the hospital with a clinical stability and was suggested to continue the regular follow-up. In conclusion, considering neurofibroma (NF) as differential diagnosis for subcutaneous masses in head and neck area is critical for early diagnosis and treatment procedure.
Current Problems in Cancer – Elsevier
Published: Mar 1, 2018
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