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
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.
Available from: PubMed Central
- "The clinical manifestation of Castleman's disease includes unicenter and multicenter types, with unicenter type observed most frequently (81%). Characteristics of unicenter type Castleman's disease are as follows: (1) males and females have the same incidence, with a median age of 37 years; (2) hyaline vascular type is the major pathologic type, accounting for 92%; (3) most lesions are in the mediastinum, neck, axillary, and retroperitoneal lymph nodes, and other rare locations include the intra-orbital region, hepatic portal, subcutaneous region, parotid glands, kidney, and pelvic cavity–; (4) the disease progresses very slowly, intact surgical excision could cure the disease and partial excision could significantly relieve symptoms. However, postoperative adjuvant radiotherapy is recommended due to the possibility of relapse after partial excision,. "
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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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