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Retroperitoneal Castleman Disease

Retroperitoneal Pararenal Mass; Castleman Disease:A Case Report

Retroperitoneal Pararenal Kitle; Castleman Hastalığı:Olgu Sunumu

DOI: 10.4328/JCAM.1743 Received: 20.03.2013 Accepted: 28.03.2013 Printed: 01.04.2016 J Clin Anal Med 2016;7(suppl 2): 102-3Corresponding Author: Alper Özorak, Suleyman Demirel University Faculty of Medicine, Department of Urology 32050 Isparta, Turkey.T.: +90 2462119259 F.: +90 2462371762 E-Mail: alperozorak@yahoo.com

Özet

Castleman hastalığı lenfadenopati ile seyreden etyolojisi bilinmeyen lenfoproli-

feratif bozukluktur. Castleman Hastalığı lenfatik zincir boyunca herhangi bir yer-

de ortaya çıkabilmesine rağmen, mediasten (% 70) en sık bulunduğu lokalizasyon-

dur. Biz abdominal tomografide sağ böbrek renal hilus ile ilişkili homojen retrope-

ritoneal kitlesi olan 36 yaşındaki erkek hastanın olgu sunumunu yaptık. Bu kitleye

cerrahi rezeksiyon uygulandı ve rezeke edilen dokunun histopatolojik tanısı Castle-

man hastalığının hiyalin-vasküler tipi idi. Bu hastalık histolojik ve prognostik ola-

rak malign lenf nodu hiperplazisinden farklıdır. Castleman hastalığı nadir görülen

bir durum olmasına karşın, her zaman retroperitoneal tümörlerin ayırıcı tanısında

akılda tutulması gereken bir durumdur.

Anahtar Kelimeler

Lenf Nodu; Retroperiton; Bening Tümör

Abstract

Castleman’s disease is a heterogeneous group of lymphoproliferative disorders

with unknown etiology presenting with lymphadenopathy. Although Castleman’s

Disease may occur anywhere along the lymphatic chain, the mediastinum is the

most common location (70%). We represent 36-year-old male patient with ho-

mogeneous retroperitoneal mass that interrelated with renal hilum of the right

kidney in abdominal tomography. Surgical complete resection performed and

histopathological diagnosis of the resected tissue was hyaline-vascular type of

Castleman’s disease It is histologically and prognostically distinct from malignant

lymph-node hyperplasia. Although Castleman’s disease is rare condition, it should

always be kept in mind in the differential diagnosis of retroperitoneal tumors.

Keywords

Lymph Node; Retroperitoneum; Benign Tumor

Alper Özorak1, Ahmet Güzel1, Taylan Oksay1, Şirin Başpınar2, Zafer Sabuncuoğlu3

1Department of Urology, 2Department of Pathology, 3Department of General SurgerySüleyman Demirel University Faculty of Medicine, Isparta, Turkey

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IntroductionBenign retroperitoneal tumors are rare conditions, comprising only about 20% of all primary retroperitoneal neoplasm’s [1]. Castleman’s disease is a heterogeneous group of lymphoprolif-erative disorders with unknown etiology presenting with lymph-adenopathy. Although Castleman’s disease may occur anywhere along the lymphatic chain, the mediastinum is the most com-mon location (70%). Extrathoracic sites such as the neck, axilla, pelvis, and retroperitoneum have been reported less frequently [2]. It is histologically and prognostically distinct from malig-nant lymph-node hyperplasia. We present a rare case of unicen-tric Castleman’s disease of the hyaline-vascular type located at right retroperitoneal area contract with kidney vessels.

Case ReportA 36-year-old male patient with one-month history of right flank pain and dyspepsia admitted our clinic. Physical exami-nation, routine hematologic, blood biochemistry and, urine analysis were normal. Abdominal x-ray was considered normal but abdominal ultrasonography demonstrated retroperitoneal mass, with 60 mm in size and with regular contour in the right anterior pararenal space. Then abdominal computed tomog-raphy performed and tomography demonstrated 62x60 mm sized homogeneous retroperitoneal mass that interrelated with renal hilum of the right kidney (Figure 1). Testicular examina-tion and testicular ultrasonography were normal. Through a midline abdominal incision, the mass was found near the hilum of the right kidney and it was in contract with the kidney ves-sels. We performed complete resection of the mass. Patient had no complication in the postoperative period. Histopathological diagnosis of the resected tissue was hyaline-vascular type of Castleman’s disease (Figure 2). Postoperative 1 year abdominal tomography was normal.

DiscussionCastleman’s disease was first reported in 1956 by Castleman et al. as a different mediastinal mass, easily confused with the thymoma [3]. Castleman’s disease is relatively rare and poorly understood lymphoproliferative disorder. Hypotheses for the pathogenesis of Castleman’s disease include infection, autoim-munity and dysregulated cytokine expression causing lymphoid proliferation [4]. Mediastinum (70%) is the main location of the disease however retroperitoneal location has been reported in 7%, with only 2% involving the pararenal region [2]. Castleman’s disease has no predilection for either sex and affects varying ages of patients but most patients are in the second and third decades of life [5]. Three basic histopathologic subtypes have been described: hyaline-vascular, plasma cell, and mixed vari-ant [6]. Two clinical entities have also been described: a uni-centric presentation with disease confined to a single anatomic lymph node, and a multicentric presentation characterized by generalized lymphadenopathy and a more aggressive clinical course [6]. Patients with localized hyalinevascular type are usu-ally asymptomatic, as in our case. Although CT is helpful for the diagnosis of Castleman’s Disease, the final diagnosis depends on pathologic examination. The standard therapy for localized, hyaline-vascular form of Castleman’s disease is surgical exci-sion, which is curative when resection is complete and en-block; the 5 years of survival is nearly 100%, and no recurrences have been reported [7].

In conclusion, an asymptomatic retroperitoneal mass in a young adult always raises the suspicion of a malignant tumor, but it is necessary to consider hyaline-vascular type Castleman’s Dis-ease prominently in the differential diagnosis.

Competing interestsThe authors declare that they have no competing interests.

References1. Bartkowski DP, Ferrigni RG. Castleman’s disease: an unusual retroperitoneal mass. J Urol 1988;139:118-202. Keller AR, Hochholzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer 1972; 29: 670-833. Castleman B, Iverson L, Menendez VP. Localized mediastinal lymph node hyper-plasia resembling thymoma. Cancer 1956;9:822–34. Van Den Berg M, Pawels P, Jakimowicz JJ, Creemers GJ: Hyaline vascular Castle-man’s disease: a case report and brief review of the literature. Neth J Med, 2002; 60: 444-4475. Palestro G, Turrini F, Pagano M, Chiusa L: Castleman’s disease. Adv Clin Path, 1999; 3: 11-226. Chronowski G, Ha C, Wilder R, Cabanillas F, Manning J, Cox J. Treatment of uni-centric and multicentric Castleman disease and the role of radiotherapy. Cancer 2001;92:670-6.7. Shahidi H, Myers JL, Kvale PA. Castleman’s disease. Mayo Clin Proc 1995;70:969-77.

How to cite this article:Özorak A, Güzel A, Oksay T, Başpınar Ş, Sabuncuoğlu Z. Retroperitoneal Pararenal Mass; Castleman Disease: A Case Report. J Clin Anal Med 2016;7(suppl 2): 102-3.

Figure 1. Abdominal tomography demonstrated 62x60 mm sized homogeneous retroperitoneal mass that interrelated with renal hilum of the right kidney

Figure 2. Regressed follicles consist of small germinal centers with marked vas-cular proliferation and hyaline material. The interfollicular stroma shows hyper-plastic vessels and mixed infiltrate of plasma cells, lymphocytes, eosinophils, im-munoblasts, and plasmacytoid monocytes (HEX100).

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