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颅骨青少年砂粒体样骨化性纤维瘤。4例报告。

Juvenile psammomatoid ossifying fibroma of the neurocranium. Report of four cases.

作者信息

Hasselblatt Martin, Jundt Gernot, Greiner Christoph, Rama Burckhard, Schmäl Frank, Iglesias-Rozas José R, van de Nes Johannes A P, Paulus Werner

机构信息

Institute of Neuropathology, University Hospital Münster, Germany.

出版信息

J Neurosurg. 2005 Jun;102(6):1151-4. doi: 10.3171/jns.2005.102.6.1151.

Abstract

Juvenile psammomatoid ossifying fibroma (JPOF) is a benign fibroosseous lesion predominantly arising within the paranasal sinuses in children and young adults. Neurocranial occurrence is exceedingly rare and a location within the neurocranial portion of the temporal bone has not been described. The authors report on one case of sinonasal JPOF secondarily extending into the cranial cavity and three cases primarily affecting the neurocranial bones to increase clinical awareness of this uncommon tumor, which may be easily mistaken for meningioma. Moreover, the absence of activating missense mutations of the GNAS1 gene in two cases strongly argues against a relationship between JPOF and fibrous dysplasia.

摘要

青少年砂粒样骨化性纤维瘤(JPOF)是一种主要发生于儿童和青年成人鼻窦内的良性纤维骨性病变。发生于颅神经区域极为罕见,颞骨神经颅部的发病部位尚无报道。作者报告了1例鼻窦JPOF继发延伸至颅腔的病例以及3例主要累及神经颅骨的病例,以提高对这种可能易被误诊为脑膜瘤的罕见肿瘤的临床认识。此外,2例病例中未检测到GNAS1基因的激活错义突变,这有力地反驳了JPOF与骨纤维发育不良之间的关联。

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