Cranial Fasciitis Presenting as an Intracranial Mass in a 10-Year-Old Girl

Department of Pathology, University of Massachusetts Medical School, Worcester, MA 01605, USA.
Pediatric and Developmental Pathology (Impact Factor: 0.87). 11/2011; 15(2):146-50. DOI: 10.2350/11-02-0990-CR.1
Source: PubMed


Cranial fasciitis is a mass-forming lesion of myofibroblastic proliferation of the skull and overlying soft tissue and occurs most frequently in pediatric patients. Cranial fasciitis has the same histological features as nodular fasciitis, which appears in the subcutaneous tissue in other parts of the body. Cranial fasciitis can involve the outer table of the skull, and it occasionally extends through the skull to form a mass involving both soft tissue and the epidural space. Intracranial cranial fasciitis without a soft tissue mass is extremely rare. Here, we report a case of a 10-year-old girl who presented with a massive intracranial mass that caused midline shift of the brain. The lesion showed focal areas of osseous metaplasia and a cytogenic abnormality of t(17;18)(q25∶q12.2) that have not yet been reported.

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    ABSTRACT: 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.
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    ABSTRACT: Cranial fasciitis is a fibroblastic lesion found in the cranium of children three weeks to six years of age. It most commonly manifests as a solitary, rapid growing mass on the scalp with frequent involvement of underlying bone and occasional intracranial expansion. Patients with cranial fasciitis may present with a wide range of associated symptoms. Otologic symptoms such as otalgia, otorrhea, hearing loss and middle ear effusion are not frequently encountered. We present a case of cranial fasciitis with intracranial involvement and associated otologic symptoms in a four year old boy with subsequent follow up 14 years later.
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