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表现为双侧鼓室球瘤的髓外造血——首例儿科双侧病例报告

Extramedullary Hematopoiesis Presenting as Bilateral Glomus Tympanicum-First Case Report in a Pediatric Patient and With Bilateral Presentation.

作者信息

Chen Leonard N, Espinel Alexandra, Reilly Brian, Luca Dragos C

机构信息

1 Department of Pathology, Medstar Georgetown University Hospital, Washington, District of Columbia.

2 Department of Otolaryngology, Children's National Medical Center, Washington, District of Columbia.

出版信息

Pediatr Dev Pathol. 2018 May-Jun;21(3):332-334. doi: 10.1177/1093526617704267. Epub 2017 May 10.

DOI:10.1177/1093526617704267
PMID:28488469
Abstract

To the best of our knowledge, this is the first case report of middle ear extramedullary hematopoiesis (EMH) in a pediatric patient as well as the first bilateral presentation reported in both children and adults. We report a 13-year-old African-American female with sickle cell disease who developed bilateral hearing loss, with magnetic resonance imaging findings consistent with bilateral glomus tympanicum (GT). Upon excisional biopsy, however, EMH was diagnosed histologically. Besides its novelty, this case highlights the importance of considering EMH in the differential diagnosis of GT including cases with bilateral presentation that may be otherwise highly suggestive of the familial form of GT.

摘要

据我们所知,这是首例关于小儿中耳髓外造血(EMH)的病例报告,也是首例在儿童和成人中均有报道的双侧发病病例。我们报告了一名患有镰状细胞病的13岁非裔美国女性,她出现了双侧听力丧失,磁共振成像结果与双侧鼓室球瘤(GT)相符。然而,经切除活检,组织学诊断为EMH。除了其新颖性外,该病例还强调了在GT的鉴别诊断中考虑EMH的重要性,包括双侧发病的病例,否则这些病例可能高度提示家族性GT。

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