Desmoid tumors in neurosurgery: a review of the literature

Abstract

Desmoid tumors (DTs) are rare myofibroblastic neoplasms, which are mostly sporadic, but sometimes associated with familial adenomatous polyposis syndrome. Neurosurgical cases of DT have been very scarce. We review the literature concerning neurosurgical DTs and describe the first case of a cicatricial DT after the resection of vestibular schwannoma, presenting as a painful swelling in the retrosigmoid scar. Contrary to other localizations in the body, standard-of-care wide margin resection cannot be performed in intracranial and spinal DTs. Therefore, maximally safe resection followed by radiotherapy when tumor margins are not free can be proposed as a treatment strategy in neurosurgical DTs.

Keywords: Aggressive fibromatosis; Cicatricial; Desmoid tumor; Desmoid-type fibromatosis.