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.
[Show abstract][Hide abstract] 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.
[Show abstract][Hide abstract] ABSTRACT: Introduction
Cranial fasciitis is an exceedingly rare, benign, fibroblastic tumour of the skull of young children. It was first described in 1980 by Lauer and Enzinger as a subset of nodular fasciitis. There are fewer than 55 cases described in the literature.
We describe the presentation of a 2-year-old girl with a rapidly growing left temporal lesion, initially treated as an intramuscular haemangioma with propanolol. Following failure of response to this management, radiological and histological investigations indicated cranial fasciitis, and multidisciplinary surgery was undertaken via a bicoronal incision. Gross total resection was carried out, and the lesion was found to be adherent to but not infiltrating the dura. The petrous bone and zygomatic arch appeared destroyed, leaving a large temporal bony defect. This was repaired with a split calvarial graft from the outer table of the right parietal bone.
Histology confirmed a diagnosis of cranial fasciitis. She had an uneventful post-operative course and was discharged home well. A CT scan 8 months post-operatively showed no residual lesion and it was noted that the reconstruction had fully integrated and the zygomatic arch had reformed.
Child s Nervous System 07/2014; 30(12). DOI:10.1007/s00381-014-2488-2 · 1.11 Impact Factor
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