Abstract
Malignant peripheral nerve sheath tumor (MPNST) of the scalp is rare. These lesions
are associated with neurofibromatosis type 1 (NF1), but patients had been reported
without NF1 also. We tried to analyze the difference between the clinical course and
outcome of the patient with MPNST having stigmata of NF1 and without it. We included
five patients treated over 3 years between July 2018 and July 2021 with diffuse scalp
MPNST. Two of these five patients with MPNST of the scalp had neurocutaneous stigmata
of NF1. Three were female and two males with an average age of 38.40 ± 18.48 years—the
youngest with NF1 being a 19-year-old female. We found dull aching pain as the most
typical complaint in all patients and a repeated episode of generalized seizure in
one patient. In these cases, two patients with NF1 have highly vascular tumors and
attained large sizes greater than 30 cm. These two cases required preoperative digital
subtraction angiography (DSA) and embolization with n-butyl acrylate. Total excision
of the tumor was done in all patients with radiotherapy. Metastases within 1 year
were noted in two patients with NF1, and one of these two succumbed to her illness.
The rest of the three patients without NF1 are under follow-up with no evidence of
disease with a maximum follow-up of 2 years. Large MPNST (size > 20 cm) are rare and
reported to have been associated with and without NF1. Patients with scalp MPNST with
NF1 can achieve larger size with fast progression of tumor size and higher chances
of recurrence and metastases.
Keywords
malignant peripheral nerve sheath tumor - diffuse scalp lesion - neurofibromatosis
type 1 - clinical course - treatment outcome
