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[Localized Castleman disease with paraneoplastic pemphigus and pulmonary involvement: clinical features and
Hongyue Sun1, Rengui Wang, Huaiyou Bin
1Department of Radiology, First Hospital, Peking University, Beijing 100034, China.
Objective:
To investigate the clinical, histopathologic and CT features of localized Castleman disease complicated with paraneoplastic pemphigus (PNP) and pulmonary involvement.
Methods:
The clinical, laboratory, histopathologic records and data of computed tomographic (CT) images of 4 patients of Castleman disease with PNP were reviewed.
Results:
All of the four patients presented with PNP which was confirmed by pathological and immunological studies and also developed lung abnormalities, including bronchiolitis obliterans (n = 3), lung abscess (n = 1) and multiple lung infarction (n = 1), 2 to 5 months after the onset of PNP. Castleman's tumor, which was found incidentally during routine examination, manifested as a solitary and huge mass (5 to 14 cm in size) which markedly enhanced homogeneously on CT in the retroperitoneum (n = 3) and mediastinum (n = 1). Histologically, three cases were of hyaline-vascular type and the other one was of mixed type. Complete remission of PNP occurred 2 weeks to 8 months after surgical resection of the tumor. The condition of two patients with bronchiolitis obliterans failed to be improved. They had been presenting dyspnea and irritable cough 3 to 15 months after surgery.
Conclusion:
PNP and lung abnormalities are rare and severe complications of localized Castleman's disease. Early diagnosis and surgery are very important for prognosis.