title: Angiogenic and inflammatory factor expressions in cutaneomeningospinal angiomatosis (Cobb's syndrome): case report.
abstract: We report on a case of cervical cutaneomeningospinal angiomatosis (Cobb's syndrome), a rare somatic disorder, characterized by vascular abnormalities of the spinal cord, with a triad of associated vascular skin, muscle, bone, and dura involvement at the same somite. This case follows an 18-year-old male patient presenting with left extremity weakness and back cervical pain. Magnetic resonance imaging (MRI) revealed a spinal cord arteriovenous malformation (AVM) at the C3-C5 level. Cobb's syndrome was diagnosed by coexistence of cutaneous naevi in a dermatomal pattern and neurological signs of a spinal cord lesion together with cervical MRI and angiography. The patient underwent a combination of staged endovascular embolization and microsurgical resection. Multiple biopsies of the mass including the skin, muscle, dura, and spinal cord at the same somite revealed that the lesions had a similar pathology. Post-operative immunohistochemical characterizations on specimen included CD31, smooth muscle actin (SMA), vascular endothelial growth factor (VEGF), and matrix metalloproteinase (MMP-9). The unique associations of somatic and spinal cord lesion as well as angiogenic and inflammatory factor expressions in all specimens are reported.
authors.affiliation: Department of Neurosurgery, Xuanwu Hospital, Capital University of Medical Sciences, 45 Changchun Street, Xicheng District, Beijing 100053, China.
authors.forename: Peng
authors.initials: P
authors.lastname: Gao
authors.forename: Hongqi
authors.initials: H
authors.lastname: Zhang
authors.forename: Feng
authors.initials: F
authors.lastname: Ling
mesh_terms: D000293:Adolescent; D042501:Angiogenic Proteins / Q000096:biosynthesis*; D000798:Angiomatosis / Q000175:diagnosis / Q000378:metabolism* / Q000473:pathology*; D001165:Arteriovenous Malformations / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology; D003937:Diagnosis, Differential; D006801:Humans; D007249:Inflammation / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology; D018836:Inflammation Mediators / Q000378:metabolism*; D008297:Male; D013118:Spinal Cord Diseases / Q000175:diagnosis / Q000378:metabolism* / Q000473:pathology*; D013577:Syndrome
chemical_list: D042501:Angiogenic Proteins; D018836:Inflammation Mediators