[Thoracic involvement of Castleman's disease: report of 10 cases].

ABSTRACT

OBJECTIVE: To improve the recognition of thoracic involvement of Castleman' s disease. METHODS: Ten patients with thoracic involvement of Castleman' s disease were retrospectively studied by review of the clinical manifestations, radiology, pathology, differential diagnosis, therapy and prognosis. RESULTS: The 10 patients were all females. Five of the patients, aged from 29 to 49 years, presented with multicentric lesions, manifested by interstitial pneumonia and mediastinal lymphadenopathy on radiology, as well as extrathoracic involvement including peripheral lymphadenopathy, and multiple organ impairment. The pathology was the plasma cell type. Treatment with corticosteroids and chemotherapy achieved partial remission in 4 cases, but 1 died of cardiac and respiratory failure. Localized lesions were found in the other 5 patients, aged from 13 to 49 years. No specific manifestations were revealed in these patients. The major radiological sign was right mediastinal masses, 6-9 cm in diameter, uniformly enhanced by contrast radiology. The pathology revealed vascular hyaline degeneration. Early surgical resection achieved good prognosis. The 5 patients were all alive. CONCLUSIONS: Diagnosis of thoracic involvement of Castleman' s disease is not easy. Early, repeated and multiple biopsy of lymph nodes is recommended, especially when the disease is multicentric or when the lymph nodes are massive. Synchronous enhancement with the vessels on contrast radiology is suggestive of the diagnosis.