Premium
Parasagittal cranial fasciitis following infratemporal fossa rhabdomyosarcoma
Author(s) -
Hattab Eyas M.,
Dvorscak Lauren E.,
Boaz Joel C.,
Douglas Annette C.,
Ulbright Thomas M.
Publication year - 2014
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/neup.12082
Subject(s) - medicine , nodular fasciitis , infratemporal fossa , craniotomy , rhabdomyosarcoma , fasciitis , lesion , anatomy , middle cranial fossa , sarcoma , skull , pathology , radiology
Cranial fasciitis is a rare lesion of young children characterized by proliferation of fibroblastic spindle cells. Most are scalp masses and are only rarely intracranial, where an association with radiation therapy is exceptional. We report a 32‐month‐old toddler with a facial rhabdomyosarcoma, diagnosed at 3 months of age, and treated with surgery, chemotherapy and brachytherapy. Brain MRI at 28 months revealed a large, left parasagittal, dural‐based, T2 hyperintense and T1 hypointense enhancing mass with superior sagittal sinus compression and bony hyperostosis. The mass was completely resected during an open craniotomy. Histologically, the lesion was comprised of loosely and haphazardly arranged bland spindle cells embedded in a myxoid background. Thick hyalinized collagen bundles were especially prominent. The spindle cells reacted for vimentin but not SMA , myogenin, MyoD1 or EMA . A diagnosis of cranial fasciitis was rendered. The role of radiation therapy in the pathogenesis of intracranial cranial fasciitis is discussed.