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Treatment and outcome of Unicentric Castleman Disease: a retrospective analysis of 71 cases.

David BoutboulJehane FadlallahSylvain ChawkiClaire FieschiMarion MalphettesAntoine DossierLaurence GérardPierre MordantVéronique MeigninEric OksenhendlerLionel Galicier
Published in: British journal of haematology (2019)
We retrospectively analysed 71 cases of Unicentric Castleman disease, a rare, usually asymptomatic, benign lymphoproliferative disorder presenting as a unique nodal mass. Although surgery is considered as the gold standard therapy, only 38 patients (54%) underwent initial surgical resection and 95% were cured. An additional 9 patients had surgery after an attempt at medical reduction. Reduction therapy was used in 21 patients with a 55% response rate, but without evidence for an optimal regimen. Radiotherapy was limited to 8 patients because of associated toxicity. Watch and wait was considered in 13 asymptomatic patients and 11 of these remained stable for up to 17 years.
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