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多中心性婴儿肌纤维瘤病,广泛累及仅限于骨骼。

Multicentric infantile myofibromatosis with extensive involvement limited to bone.

作者信息

Luque-Cabal María, Obregón-Martínez Estíbaliz, Díez-Blanco María, Álvarez-García Marta, Fernández-Pérez Gabriel

机构信息

Department of Radiology, Hospital Universitario Río Hortega, Valladolid, Spain.

Department of Clinical Pathology, Hospital Clínico Universitario de Valladolid, Valladolid, Spain.

出版信息

Skeletal Radiol. 2022 Jul;51(7):1503-1510. doi: 10.1007/s00256-021-03968-5. Epub 2021 Dec 4.

DOI:10.1007/s00256-021-03968-5
PMID:34865192
Abstract

Infantile myofibromatosis (IM) is the most common benign fibrous tumor of infancy, characterized by the development of single or multiple nodules in the skin, soft tissues, bone, and/or viscera. Multicentric forms are less frequent and can affect different tissues simultaneously and their prognosis depends on their extension and visceral involvement. Rarely, these forms are limited to the skeleton, in which case the absence of extraosseous lesions makes it difficult to suspect this entity. We present the case of an infant with multiple radiolucent lesions involving the skull, ribs, spine, and long bones, discovered in a radiological study performed after a minor trauma. A broad differential diagnosis was considered based on the osteolytic and polyostotic nature of the lesions on imaging studies. This report details and illustrates the typical radiological findings in bony involvement of IM, which suggest this disorder over other diagnostic options.

摘要

婴儿肌纤维瘤病(IM)是婴儿期最常见的良性纤维性肿瘤,其特征是在皮肤、软组织、骨骼和/或内脏中出现单个或多个结节。多中心型较为少见,可同时累及不同组织,其预后取决于病变范围和内脏受累情况。极少数情况下,这些类型仅限于骨骼,在这种情况下,由于没有骨外病变,很难怀疑这种疾病。我们报告一例婴儿病例,在一次轻微创伤后进行的放射学检查中发现,其颅骨、肋骨、脊柱和长骨有多个透光性病变。基于影像学研究中病变的溶骨性和多骨受累性质,考虑了广泛的鉴别诊断。本报告详细阐述并展示了IM骨受累的典型放射学表现,相较于其他诊断选项,这些表现提示了该疾病。

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