Retroperitoneal Unicentric Castleman Disease: Surgery is Curative

Authors

DOI:

https://doi.org/10.34635/rpc.1140

Keywords:

Castleman Disease/diagnostic imaging, Castleman Disease/surgery

Abstract

Castleman disease (CD) comprises a group of rare nonclonal lymphoproliferative disorders. Unicentric Castleman disease (UCD) typically presents as isolated lymphadenopathy with progressive enlargement and an indolent course. Most patients are asymptomatic or may present symptoms related to a localized mass effect on organ function. Multicentric Castleman disease (MCD) affects various lymph node stations and leads to systemic constitutional symptoms and systemic cytokine dysregulation, resulting in laboratory abnormalities, hepatosplenomegaly, and complex organ dysfunction.
We report a case of retroperitoneal UCD. Imaging studies showed a solitary, solid, right pararenal lesion causing anterior deviation of the inferior vena cava and renal pedicle. Complete surgical resection was performed, and histopathologic analyses confirmed the diagnosis of the hyaline-vascular subtype of UCD.
Patients with UCD generally have an excellent prognosis following curative surgical resection. However, recurrence may occur, and long-term follow-up is advised.

Downloads

Download data is not yet available.

References

Castleman B, Towne VW. Case records of the Massachusetts General Hospital; weekly clinicopathological exercises; Case No. 40351. N Engl J Med. 1954;251:396-400. doi: 10.1056/NEJM195409022511008

Bonekamp D, Horton KM, Hruban RH, Fishman EK. Castleman disease: the great mimic. Radiographics. 2011;31:1793-807. doi: 10.1148/rg.316115502

Hoffmann C, Hentrich M, Tiemann M, Rosenwald A, Weber F, Willenbacher W, et al. Recent Advances in Castleman Disease. Oncol Res Treat. 2022;45:693-704. doi: 10.1159/000526640

Carrion DM, Alvarez-Maestro M, Gómez Rivas J, Brygadyr Y, García-Fernandez E, Martínez-Piñeiro L. Challenging Diagnosis of a Solitary Retroperitoneal Mass: A Case Report of Castleman’s Disease and Review of the Literature. Urol Int. 2019;103:245-8. doi: 10.1159/000493511

Wong RS. Unicentric Castleman Disease. Hematol Oncol Clin North Am. 2018;32:65-73. doi: 10.1016/j.hoc.2017.09.006

Wu D, Lim MS, Jaffe ES. Pathology of Castleman Disease. Hematol Oncol Clin North Am. 2018;32:37-52. doi: 10.1016/j.hoc.2017.09.004

Lomas OC, Streetly M, Pratt G, Caver J, Royston D, Schey S, et al. The management of Castleman disease. Br J Haematol. 2021;195:328-37. doi: 10.1111/bjh.17688

Gündüz E, Özdemir N, Bakansy S, Karkus S. A Rare Lymphopro-liferative Disease: Castleman Disease. Turk J Haematol. 2021;38:314-20. doi: 10.4274/tjh.galenos.2021.2021.0440

Talat N, Belgaunkar AP, Schulte KM. Surgery in Castleman’s disease: a systematic review of 404 published cases. Ann Surg. 2012;255:677-84. doi: 10.1097/SLA.0b013e318249dcdc

Xu J, Zhou BO, Cao HL, Wang BO, Yan S, Zheng SS. Surgical management of isolated retroperitoneal Castleman’s disease: A case report. Oncol Lett. 2016;11:2123-6. doi: 10.3892/ol.2016.4177

Bourboul D, Fedallah J, Chawki S, Fieschi C, Malphettes M, Dossier A, et al. Treatment and outcome of Unicentric Castleman Disease: a retrospective analysis of 71 cases. Br J Haematol. 2019;186:269-73. doi: 10.1111/bjh.15921

Downloads

Published

2025-12-26

Issue

Section

Clinical Case