Intrapulmonary unicentric Castleman's Disease: A clinical analysis of 8 patients

Castleman's disease (CD) is a rare, chronic, lymphoproliferative disorder, increasingly recognized as a clonal neoplasm of lymph node stromal cells that can affect multiple organs and tissues. Intrapulmonary unicentric Castleman's disease (UCD) represents an exceptionally uncommon subtype, primarily involving the lung parenchyma and hilum, with only a few pathologically confirmed cases reported to date. This study aimed to characterize the clinical, radiological, and pathological features of intrapulmonary UCD and to summarize diagnostic and therapeutic experiences.

We retrospectively analyzed eight patients diagnosed with intrapulmonary UCD. Clinical manifestations, laboratory data, radiologic and bronchoscopic findings, histopathological characteristics, treatment approaches, and follow-up outcomes were systematically reviewed.

No patient exhibited specific clinical symptoms or distinctive laboratory abnormalities. Chest computed tomography (CT) served as the optimal imaging modality, consistently revealing solitary, well-circumscribed, soft-tissue masses with smooth lobulated contours. Definitive diagnosis relied on histopathological examination. All patients underwent complete surgical resection, followed by regular postoperative surveillance with chest CT. During follow-up, no local recurrence or disease progression was detected.

Intrapulmonary UCD is an exceedingly rare form of Castleman's disease that can be easily misdiagnosed as primary lung tumors, lymphoma, granulomatous disease, or tuberculosis due to its nonspecific clinical and radiological features. Complete surgical excision remains the mainstay of treatment and is associated with an excellent prognosis following complete resection.