[Localized Castleman disease with paraneoplastic pemphigus and pulmonary involvement: clinical features and histopathology].

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.