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多中心性婴儿肌纤维瘤病:两例围产期病例。

Multicentric infantile myofibromatosis: two perinatal cases.

作者信息

Pelluard-Nehmé Fanny, Coatleven Frederic, Carles Dominique, Alberti Eve Marie, Briex Michel, Dallay Dominique

机构信息

Unité de Foetopathologie, Service d'Anatomie et Cytologie Pathologique, Centre Hospitalo-Universitaire de Bordeaux, Place Amélie Raba Léon, 33076, Bordeaux Cedex, France.

出版信息

Eur J Pediatr. 2007 Oct;166(10):997-1001. doi: 10.1007/s00431-006-0369-8. Epub 2006 Dec 21.

Abstract

Infantile myofibromatosis, the most common fibrous tumor of infancy, occurs in solitary, multiple, and generalized forms, with similar histology but different clinicopathologic and prognostic implications. This entity is a mesenchymal disorder characterized by the proliferation of fibrous tumors in the skin, muscles, viscera, bones, and subcutaneous tissues. Visceral lesions are associated with significant morbidity and mortality, generally within the first few months of life. They lead to failure to thrive, to infection, hemorrhage, or to the obstruction of vital organs. We describe two cases of multicentric myofibromatosis with significant in utero lesional growth, resulting in one fetal demise and one post-natal demise. To the best of our knowledge, this is the first report of a fetal death secondary to infantile myofibromatosis.

摘要

婴儿肌纤维瘤病是婴儿期最常见的纤维性肿瘤,有单发、多发和泛发型,组织学表现相似,但临床病理及预后意义不同。该疾病是一种间叶组织疾病,其特征为皮肤、肌肉、内脏、骨骼及皮下组织中纤维性肿瘤的增殖。内脏病变通常在出生后的头几个月内导致显著的发病率和死亡率。它们会导致发育不良、感染、出血或重要器官梗阻。我们描述了两例多中心性肌纤维瘤病病例,其在子宫内病灶显著生长,导致一例胎儿死亡和一例出生后死亡。据我们所知,这是首例继发于婴儿肌纤维瘤病的胎儿死亡报告。

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