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儿童颞骨朗格汉斯细胞组织细胞增生症:27 例临床和影像学研究。

Pediatric Langerhans cell histiocytosis of the temporal bone: clinical and imaging studies of 27 cases.

机构信息

Department of Radiology, Xinhua Hospital affiliated to Shanghai Jiao Tong University School of Medicine, Room 203, Building 5, No.1665 Kongjiang Road, Shanghai City, 200092, Yangpu District, China.

出版信息

World J Surg Oncol. 2018 Mar 27;16(1):72. doi: 10.1186/s12957-018-1366-x.

Abstract

BACKGROUND

We aimed to evaluate the clinical and imaging presentations of Langerhans cell histiocytosis (LCH) in the pediatric temporal bone.

METHODS

This retrospective study included 27 pediatric cases with pathological confirmed LCH of the temporal bone. The clinical and imaging features of the cases were analyzed. The involvement of ossicular chain and otic capsule was also evaluated.

RESULTS

A total of 38 lesions (27 cases) with 11 bilateral involvement were identified. For the 27 cases, the most common complaint was periauricular swelling (12/27, 44.4%), followed by otorrhea (9/27, 33.3%) and otalgia (5/27, 18.2%). The mastoid process was the most common involved subsite (31/38, 81.6%) among the 38 lesions. Ten (26.3%, 10/38) lesions belonged to the group of the diffuse involvement, 22 (57.9%, 22/38) were divided into the group of partial involvement and six (15.8%,6/38) localized lesions with punched-out appearance. Erosion of ossicular chains and otic capsule were found in three and seven lesions respectively.

CONCLUSION

The results indicate that the most common subsite for LCH of the pediatric temporal bone was the mastoid process. The location and extent of pediatric LCH of the temporal bone varied a lot between each other. The ossicular chains usually remain intact and the erosion of otic capsule can occur in some lesions.

摘要

背景

本研究旨在评估儿童颞骨朗格汉斯细胞组织细胞增生症(LCH)的临床和影像学表现。

方法

本回顾性研究纳入了 27 例经病理证实的颞骨朗格汉斯细胞组织细胞增生症患儿。分析了患儿的临床和影像学特征,并评估了听骨链和耳囊的受累情况。

结果

共发现 38 个病灶(27 例),其中 11 例为双侧受累。27 例患儿中,最常见的主诉是耳周肿胀(12/27,44.4%),其次是耳漏(9/27,33.3%)和耳痛(5/27,18.2%)。38 个病灶中,乳突是最常见的受累部位(31/38,81.6%)。10 个(26.3%,10/38)病灶属于弥漫性受累组,22 个(57.9%,22/38)病灶属于局限性受累组,6 个(15.8%,6/38)病灶属于局灶性穿凿样外观。3 个和 7 个病灶分别发现听骨链和耳囊侵蚀。

结论

结果表明,儿童颞骨朗格汉斯细胞组织细胞增生症最常见的受累部位是乳突。儿童颞骨朗格汉斯细胞组织细胞增生症的位置和范围彼此之间差异很大。听骨链通常保持完整,一些病灶可出现耳囊侵蚀。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2e32/5872572/96f14c4948cf/12957_2018_1366_Fig1_HTML.jpg

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