Gorham–Stout Disease of the Skull Base With Hearing Loss: Dramatic Recovery and Antiangiogenic Therapy. Issue 5 (29th December 2015)

• Record Type: Journal Article
• Authors: Nozawa, Akifumi; Ozeki, Michio; Kuze, Bunya; Asano, Takahiko; Matsuoka, Kentaro; Fukao, Toshiyuki
• Is Part Of: Pediatric blood & cancer. Volume 63:Issue 5(2016:)
• Page Start: 931
• Page End: 934
• Publication Date: 2015-12-29
• Subjects: cerebrospinal fluid leakage -- interferon -- lymphatic malformation -- mammalian target of rapamycin -- sirolimus; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.25886
• Languages: English
• ISSNs: 1545-5009

Abstract

Abstract : Gorham–Stout disease (GSD) is a rare disorder of unknown etiology. We present a 6‐year‐old male with GSD involving the skull base who presented with recurrent cerebrospinal fluid (CSF) rhinorrhea, severe hearing loss, and facial palsy secondary to cerebellar herniation into the internal auditory canal. After 2 months of treatment with pegylated interferon (IFN) α‐2b (50 μg/week), his hearing recovered dramatically. Two years later, new bone formation appeared radiologically and IFN was switched to sirolimus. One year after the switch, CSF rhinorrhea disappeared. Antiangiogenic therapy might inhibit proliferation of vascular endothelial cells in osteolytic lesions and lead to new bone formation.