Localized retroperitoneal Castleman’s disease: a case report and review of the literature

Journal of Medical Case Reports

Hermann Janßen²  Horst Kinkel⁴  Bernd Klosterhalfen³  Michael Flasshove¹  Susanne Herold⁵  Ilhan Demirci²  Sorin-Lucian Milea²  Rodrigo Aguilar-Rodriguez² 

[1] Department of Hematology and Internal Oncology, Hospital of Düren, Düren, Germany;Department of General, Visceral, Vascular and Thoracic Surgery, Hospital of Düren, Düren, Germany;The Institute of Pathology, Hospital of Düren, Düren, Germany;Department of Gastroenterology, Hepatology and Diabetology, Hospital of Düren, Düren, Germany;Department of Internal Medicine II, University of Gießen Lung Center, Gießen, Germany

关键词: Unicentric;    Surgery;    Hyaline vascular variant;    Castleman’s disease;   

Others  :  822547
DOI  :  10.1186/1752-1947-8-93

 received in 2013-10-02, accepted in 2014-02-11,  发布年份 2014

【 摘 要 】

Introduction

Castleman’s disease, also known as angiofollicular lymph node hyperplasia, is a rare disease with two known expansion types, unicentric and multicentric, which play a major role in determining therapy. We focus here on the unicentric type, which can be treated and cured by surgery. To date, approximately 1000 cases of Castleman’s disease have been reported in the literature.

Case presentation

A 50-year-old Caucasian woman presented to our Department of Hematology and Internal Oncology with increasing fatigue as her sole symptom. Diagnostic investigations including laboratory studies, ultrasound, computed tomography and magnetic resonance imaging were performed. These revealed an interaortocaval, retroperitoneal tumor mass in her upper abdomen as the only manifestation of the disease. No enlarged lymph nodes were detected. We conducted a laparotomy with radical extirpation of the tumor mass (10×9×5.7cm). Complete tumor resection with clear margins was achieved. A pathological analysis of the resected sample showed atypical lymphoid tissue of small to medium cells with some clearly visible nucleoli, enlarged sinusoidal vessels, pleomorphic calcifications and focally preserved germinal-center-like structures. Histological and immunohistochemical analysis confirmed the diagnosis of Castleman’s disease: staining for CD3, CD5, CD10, CD20, CD23, CD79 and Ki-67 was strongly positive in the germinal-center-like structures. Histological findings clearly showed the disease to be the hyaline vascular subtype. Staining for cyclin D1 and CD30 was negative. Expression of CD15 was positive in the enlarged sinusoidal vessels. A supplementary clonality analysis was without pathological findings. Tests for human immunodeficiency virus and human herpes virus 8 were negative and results from a bone marrow biopsy were normal. Our patient recovered well from surgery and was discharged from our hospital. To date, no recurrence of the disease has been detected.

Conclusion

Castleman’s disease is a rare disorder that remains a diagnostic challenge. Radical surgical resection is considered to be the gold standard for treating the unicentric variant of this disease.

【 授权许可】

   
2014 Aguilar-Rodriguez et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

Figure 2.

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