Langerhans cell histiocytosis involving the temporal bone with destruction and subsequent reossification of the bony labyrinth boundaries.

IF 1.3 4区 医学 Q3 RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING Rofo-fortschritte Auf Dem Gebiet Der Rontgenstrahlen Und Der Bildgebenden Verfahren Pub Date : 2024-10-01 Epub Date: 2024-02-19 DOI:10.1055/a-2254-5536
Katja Döring, Philipp Ivanyi, Heinrich Lanfermann, Athanasia Warnecke, Anja Giesemann
{"title":"Langerhans cell histiocytosis involving the temporal bone with destruction and subsequent reossification of the bony labyrinth boundaries.","authors":"Katja Döring, Philipp Ivanyi, Heinrich Lanfermann, Athanasia Warnecke, Anja Giesemann","doi":"10.1055/a-2254-5536","DOIUrl":null,"url":null,"abstract":"<p><strong>Purpose: </strong> With an incidence between 1-9/100 000 per year, Langerhans cell histiocytosis (LCH) is a rather rare disease from the hemato-oncologic disease spectrum (Hayes et al. 2009). The tumorlike disease with proliferation of histiocytic cells may manifest as localized to one organ or disseminated with infiltration of a wide variety of organs. Approximately 25-30 % of these cases show involvement of the temporal bone (Ni et al. 2017).</p><p><strong>Case description: </strong> With vertigo persisting for three years, chronic mastoiditis, and acute progressive hearing loss bilaterally (r > l) for three weeks, a 41-year-old woman presented at an emergency department. The DVT showed extensive bony destruction of large parts of the temporal bone on both sides, involving the vestibular organ, the cochlea, and the internal auditory canal. To confirm the suspicion of a systemic inflammatory process, a PE was performed from the mastoid with bioptic confirmation of an LCH. Systemic therapy was initiated. Post-therapeutic imaging showed almost complete remission with reossification of the preexisting defect zones and the internal auditory canal and labyrinth structures again showed bony margins. Clinically, there was an improvement of the vegetative symptoms with remaining bilateral sensorineural hearing loss.</p><p><strong>Discussion: </strong> LCH of the temporal bone is a rare and often misdiagnosed disease due to its nonspecific clinical presentation. Awareness of temporal bone LCH and its occurrence in adults is essential for accurate and consistent diagnosis.</p><p><strong>Key points: </strong>  · LCH is a rather rare disease from the hemato-oncological spectrum. · Affection of the temporal bone, especially such an extensive one (as in this case report), is rather atypical in adulthood. · Use of systemic therapy resulted in remission. · There was complete reossification of the osseous structures post-therapy. · A cochlear implant was able to be implanted to compensate for hearing loss.</p>","PeriodicalId":21490,"journal":{"name":"Rofo-fortschritte Auf Dem Gebiet Der Rontgenstrahlen Und Der Bildgebenden Verfahren","volume":null,"pages":null},"PeriodicalIF":1.3000,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Rofo-fortschritte Auf Dem Gebiet Der Rontgenstrahlen Und Der Bildgebenden Verfahren","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1055/a-2254-5536","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/2/19 0:00:00","PubModel":"Epub","JCR":"Q3","JCRName":"RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING","Score":null,"Total":0}
引用次数: 0

Abstract

Purpose:  With an incidence between 1-9/100 000 per year, Langerhans cell histiocytosis (LCH) is a rather rare disease from the hemato-oncologic disease spectrum (Hayes et al. 2009). The tumorlike disease with proliferation of histiocytic cells may manifest as localized to one organ or disseminated with infiltration of a wide variety of organs. Approximately 25-30 % of these cases show involvement of the temporal bone (Ni et al. 2017).

Case description:  With vertigo persisting for three years, chronic mastoiditis, and acute progressive hearing loss bilaterally (r > l) for three weeks, a 41-year-old woman presented at an emergency department. The DVT showed extensive bony destruction of large parts of the temporal bone on both sides, involving the vestibular organ, the cochlea, and the internal auditory canal. To confirm the suspicion of a systemic inflammatory process, a PE was performed from the mastoid with bioptic confirmation of an LCH. Systemic therapy was initiated. Post-therapeutic imaging showed almost complete remission with reossification of the preexisting defect zones and the internal auditory canal and labyrinth structures again showed bony margins. Clinically, there was an improvement of the vegetative symptoms with remaining bilateral sensorineural hearing loss.

Discussion:  LCH of the temporal bone is a rare and often misdiagnosed disease due to its nonspecific clinical presentation. Awareness of temporal bone LCH and its occurrence in adults is essential for accurate and consistent diagnosis.

Key points:   · LCH is a rather rare disease from the hemato-oncological spectrum. · Affection of the temporal bone, especially such an extensive one (as in this case report), is rather atypical in adulthood. · Use of systemic therapy resulted in remission. · There was complete reossification of the osseous structures post-therapy. · A cochlear implant was able to be implanted to compensate for hearing loss.

查看原文
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
累及颞骨的朗格汉斯细胞组织细胞增生症,伴有骨性迷宫边界的破坏和随后的再化生。
目的:朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)每年的发病率在 1-9/10 万之间,是血液肿瘤疾病谱中一种相当罕见的疾病(Hayes 等,2009 年)。这种组织细胞增生的肿瘤性疾病可表现为局部器官或浸润多个器官的播散性疾病。其中约 25-30% 的病例表现为颞骨受累(Ni 等人,2017 年):一名 41 岁女性因持续三年的眩晕、慢性乳突炎和急性进行性双侧听力下降(r > l)三周到急诊科就诊。深静脉血栓显示两侧颞骨的大部分都有广泛的骨质破坏,涉及前庭器官、耳蜗和内耳道。为了证实系统性炎症过程的怀疑,对乳突进行了PE检查,并通过活检确认为LCH。随后开始了全身治疗。治疗后的影像学检查显示,患者的病情几乎完全缓解,原有的缺损区重新变小,内耳道和迷宫结构再次出现骨性边缘。临床上,患者的植物神经症状有所改善,但仍存在双侧感音神经性听力损失:讨论:颞骨 LCH 是一种罕见的疾病,由于其临床表现无特异性,常常被误诊。认识颞骨 LCH 及其在成人中的发生对于准确和一致的诊断至关重要:- 要点:LCH 是一种相当罕见的血液肿瘤疾病。- 颞骨受累,尤其是如此广泛的颞骨受累(如本病例报告中的情况),在成年期是相当不典型的。- 采用系统治疗后,病情得到缓解。- 治疗后骨质结构完全恢复。- 植入的人工耳蜗可以弥补听力损失。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 去求助
来源期刊
CiteScore
1.20
自引率
5.60%
发文量
340
期刊介绍: Die RöFo veröffentlicht Originalarbeiten, Übersichtsartikel und Fallberichte aus dem Bereich der Radiologie und den weiteren bildgebenden Verfahren in der Medizin. Es dürfen nur Arbeiten eingereicht werden, die noch nicht veröffentlicht sind und die auch nicht gleichzeitig einer anderen Zeitschrift zur Veröffentlichung angeboten wurden. Alle eingereichten Beiträge unterliegen einer sorgfältigen fachlichen Begutachtung. Gegründet 1896 – nur knapp 1 Jahr nach der Entdeckung der Röntgenstrahlen durch C.W. Röntgen – blickt die RöFo auf über 100 Jahre Erfahrung als wichtigstes Publikationsmedium in der deutschsprachigen Radiologie zurück. Sie ist damit die älteste radiologische Fachzeitschrift und schafft es erfolgreich, lange Kontinuität mit dem Anspruch an wissenschaftliches Publizieren auf internationalem Niveau zu verbinden. Durch ihren zentralen Platz im Verlagsprogramm stellte die RöFo die Basis für das heute umfassende und erfolgreiche Radiologie-Medienangebot im Georg Thieme Verlag. Besonders eng verbunden ist die RöFo mit der Geschichte der Röntgengesellschaften in Deutschland und Österreich. Sie ist offizielles Organ von DRG und ÖRG und die Mitglieder der Fachgesellschaften erhalten die Zeitschrift im Rahmen ihrer Mitgliedschaft. Mit ihrem wissenschaftlichen Kernteil und dem eigenen Mitteilungsteil der Fachgesellschaften bietet die RöFo Monat für Monat ein Forum für den Austausch von Inhalten und Botschaften der radiologischen Community im deutschsprachigen Raum.
期刊最新文献
[Thoracic penetrating aortic ulcer: conventional radiography as a valuable diagnostic tool]. Large language models (LLMs) in radiology exams for medical students: Performance and consequences. Position Paper of the German Society for Interventional Radiology and Minimally Invasive Therapy (DeGIR) and the German Roentgen Society (DRG) on Structural and Professional Requirements in Interventional Oncology. Assessment of Efficacy and Complications of Endovascular Interventions for Critical Limb Ischemia in Germany: A Nationwide Study. Improving the use of LLMs in radiology through prompt engineering: from precision prompts to zero-shot learning.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1