CC BY-NC-ND 4.0 · Laryngorhinootologie 2022; 101(S 02): S218
DOI: 10.1055/s-0042-1746659
Poster
Head-Neck-Oncology: Rare tumors

Prevalence of idiopathic multicentric Morbus Castleman in patients suffering from cervical lymphadenopathy

Michael Krokenberger
1   Klinikum rechts der Isar der Technischen Universität München, Hals-Nasen-Ohrenklinik und Poliklinik München
,
Kristina Schwamborn
2   Klinikum rechts der Isar der Technischen Universität München, Institut für Allgemeine Pathologie und Pathologische Anatomie München
,
Ulrich Straßen
1   Klinikum rechts der Isar der Technischen Universität München, Hals-Nasen-Ohrenklinik und Poliklinik München
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    Background Idiopathic multicentric Morbus Castleman (iMCD) is a very rare disease with a broad spectrum of symptoms and a complex histopathological diagnosis. An expert meeting in 2017 by Fajgenbaum et al. defined new criteria for the diagnosis for iMCD. Estimations for the prevalence in the German population do not exist yet. As lymphadenopathy often occurs primarily in the head and neck region patients are frequently referred to the otorhinolaryngology department for further differential diagnosis and biopsy.

    Methods The aim of this retrospective study is to determine the prevalence of iMCD in patients, who underwent a lymph node removal in our clinic between 2010 and 2020. Data was analysed descriptively while diagnosis of mono- and multicentric M. Castleman disease were made utilizing the international diagnostic criteria proposed by Fajgenbaum et al.

    Results 714 patients suffering of cervical lymphadenopathy were included into the study. In 75 patients matching the clinical criteria for the diagnosis of iMCD histopathologic samples were re-analysed by a histopathologist. 15 cases (2,1%) matched the major criterion defined by Fajgenbaum, 1 case (0,1%) matched for both major and minimum required minor criteria.

    Discussion The new criteria from Fajgenbaum et al. offer an objective tool for the diagnosis of iMCD. Even if the histopathological major criterion often fits for the extirpated lymph nodes,≥2 enlarged lymph node stations and complementary minor criteria were often missing for the final diagnosis.

    Conclusions In our data after utilization of the new criteria 2,1% of patients could be diagnosed with monocentric and 0,1% with idiopathic multicentric M. Castleman. Clinicians should keep in mind this differential diagnosis as effective therapies are available.


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    Conflict of Interest

    Der Erstautor weist auf folgenden Interessenkonflikt hin Educational Grant der Firma EUSA Pharma

    Publication History

    Article published online:
    24 May 2022

    © 2022. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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