Abstract:

Objective: To characterize the radiological features, diagnostic process, clinical manifestations, treatment strategies, and prognostic outcomes of pulmonary Castleman disease (PCD), with the aim of reducing misdiagnosis and missed diagnosis. Methods: We conducted a retrospective analysis of the diagnostic workup, therapeutic interventions, and follow-up of the first documented case of PCD at Fuzhou Pulmonary Hospital, Fujian Province. Additionally, we performed a systematic literature search using the terms “Castleman’s disease” and “pulmonary” across the Wanfang, China National Knowledge Infrastructure (CNKI), and PubMed databases, covering the period from January 1994 to January 2024. A total of 48 eligible articles were identified, encompassing 239 reported cases of PCD. Results: A 42-year-old male patient presented with a one-year history of chronic cough and sputum production and was admitted to Fuzhou Pulmonary Hospital, Fujian Province, on April 23, 2019. He had previously been misdiagnosed with pulmonary tuberculosis at an outside institution. Following a comprehensive diagnostic evaluation—including bronchoscopy, cervical lymph node biopsy, percutaneous lung puncture, and multiple pathological consultations—the patient was ultimately diagnosed with plasma cell type PCD. He received standardized targeted therapy, which led to symptomatic improvement and clinical stabilization. Through literature screening, 239 csases of PCD were identified. Including the present case, a total of 240 patients were analyzed. Based on imaging characteristics and the feasibility of surgical resection, these cases were categorized into two subtypes: isolated PCD (IPCD) and diffuse PCD (DPCD). Of the 240 cases, 108 were classified as IPCD and 132 as DPCD, with both subtypes predominantly affecting young and middle-aged adults. IPCD was largely asymptomatic, with 68 patients (62.96%) presenting no clinical symptoms. In contrast, DPCD was associated with a broader spectrum of clinical manifestations, including cough (63 cases, 47.73%), fever and night sweats (44 cases, 33.33%), dyspnea (56 cases, 42.42%), and fatigue (54 cases, 40.91%). Histopathological analysis revealed that the majority of IPCD cases exhibited the hyaline vascular variant (77 cases, 79.39%), whereas the plasma cell variant was predominant in DPCD (75 cases, 56.82%). Pulmonary function tests were performed in four patients with DPCD, all of whom demonstrated mixed ventilatory defects and impaired diffusion capacity. Surgical resection was the primary treatment modality for IPCD and was associated with favorable outcomes. In contrast, DPCD was primarily managed with chemotherapy and targeted therapies, and exhibited a poorer prognosis, with seven reported deaths. Conclusion: PCD is an uncommon and often underrecognized lymphoproliferative disorder that presents diagnostic and therapeutic challenges. Multidisciplinary collaboration is essential for accurate diagnosis and optimal management. Imaging-based classification distinguishes two clinical subtypes—IPCD and DPCD—with DPCD presenting more frequent and severe symptoms, distinct pathological features, and poorer outcomes. This classification framework enhances clinical recognition and may guide therapeutic decision-making; however, further validation through prospective clinical studies is warranted.

Key words: Lung diseases, Pulmonary Castleman disease, Disease attributes, Diagnosis, differential, Review literature as topic