A rare case of unicentric plasma cell type Castleman's disease in the mediastinum.

Thoracic Surgery Clinic II, Carol Davila University of Medicine and Pharmacy, Bucharest.
Pneumologia (Bucharest, Romania) 01/2010; 59(1):32-5.
Source: PubMed

ABSTRACT Castleman's disease is a very rare (and thus little-known) lymphoproliferative disorder characterized by lymph node follicular hyperplasia with abnormal interfollicular vascular growth. It can be classified into unicentric and multicentric variants according to its localization, and into three histological types: hyaline-vascular, plasma cell and mixed. We describe the case of a 51 year-old woman who presented with precordial chest pain. Chest X-Ray and computed tomography (CT) raised the suspicion of an aortic aneurysm, and the patient was sent to the Interventional Cardiology Clinic at the Military Emergency Clinical Center for Cardiovascular Diseases (CCUBCVA), where the diagnosis of mediastinal tumor was made by ultrasonography, coronarography and CT. The patient was transferred to our clinic, where a diagnostic mediastinoscopy proved histolopathologically inconclusive. A lateral thoracotomy with ideal tumorectomy was decided upon and carried out, and the diagnosis of plasma cell type Castleman's disease was established. This paper discusses the pathogenic mechanisms, symptoms, diagnosis and treatment of Castleman's disease.

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    ABSTRACT: Castleman's disease is a slowly progressive and rare lymphoproliferative disorder. Here, we report a 55-year-old woman with superior mediastinal Castleman's disease being misdiagnosed for a long term. We found a 4.3 cm mass localized in the superior mediastinum accompanied with severe clinical symptoms. The patient underwent an exploratory laparotomy, but the mass failed to be totally excised. Pathologic examination revealed a mediastinal mass of Castleman's disease. After radiotherapy of 30 Gy by 15 fractions, the patient no longer presented previous symptoms. At 3 months after radiotherapy of 60 Gy by 30 fractions, Computed tomography of the chest showed significantly smaller mass, indicating partial remission. Upon a 10-month follow-up, the patient was alive and free of symptoms.
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