Treatment of unicentric Castleman disease with rituximab, bendamustine and dexamethasone reduced the volume of the inoperable expansive lesion in the upper mediastinum and enabled its radical removal

Czech version

Authors: T. Horváth ¹; L. Frola ²; Z. Adam ³; P. Fabian ⁴; Z. Řehák ⁵; R. Koukalová ⁵; P. Opletal ⁶; T. Ostřížek ⁷; K. Hudáček ⁸; P. Žuffa ⁹; M. Krejčí ³; Z. Král ³; L. Pour ³
Authors‘ workplace: Chirurgická klinika LF MU a FN Brno ¹; Ústav patologie, LF MU a FN Brno ²; Interní hematologická a onkologická klinika LF MU a FN Brno ³; Oddělení onkologické patologie, MOÚ Brno ⁴; Oddělení nukleární medicíny, MOÚ Brno 6 Oddělení radiologie, MOÚ Brno ⁵; Centrum kardiovaskulární a transplantační chirurgie, Brno ⁷; Klinika anesteziologie, resuscitace a intenzivní medicíny LF MU a FN Brno ⁸; Chirurgické oddělení, KN Tomáše Bati, Zlín ⁹
Published in: Klin Onkol 2025; 38(2): 132-143
Category: Case Reports
doi: https://doi.org/10.48095/ccko2025132

Overview

Background: Castleman disease (CD) is a historical name derived from the name of the surgeon who first described it. It is used for lesions or foci of the character of non-malignant lymphoproliferative activity. According to the extent of the affliction, it is divided into two basic forms, the unicentric form (UCD) and the multicentric form of Castleman disease, where UCD is formed by the hyaline vascular type of CD. Observation: The first symptom in the described case of UCD was pain radiating to the left upper limb, especially when moving. MRI of the cervical spine revealed pathological expansion on the border between the neck and the upper mediastinum, more on the left. Targeted biopsy showed Castleman disease, hyaline vascular type. According to PET/CT imaging with fluorodeoxyglucose (FDG-PET/CT), it was the only pathological lesion in the body. The size of the tumour resistance did not allow safe resection, so the only solution was to administer adjuvant treatment. The patient started treatment with rituximab 850 mg on day 1 of a 28-day cycle, cyclophosphamide 600 mg on days 1 and 15 and dexamethasone 20 mg, also on days 1 and 15 of a 28-day cycle. Due to individual intolerance of cyclophosphamide in the first cycle, the administration of this drug was discontinued, and from the third cycle onwards, instead of cyclophosphamide, she received bendamustine at a total dose of 100 mg on days 1 and 15. Results: FDG-PET/CT imaging after 9 cycles of treatment showed a marked reduction in the infiltrate size and a decrease in the rate of FDG accumulation. This allowed the team of thoracic and cardiac surgeons to completely remove it down to healthy tissue. Conclusion: The treatment of choice for UCD is surgical removal. If the location or size of the lesion does not allow radical surgery, it is possible to achieve reduction by the mentioned drug treatment. In the case described, the combination of rituximab, bendamustine and dexamethasone reduced the size of the lesion, which allowed its complete resection.

Keywords:

rituximab – dexamethasone – bendamustine – unicentric Castleman disease

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