Rare Unicentric Intrapulmonary Castleman Disease: A Systematic Review and Report of a Case.

Abstract

Objectives: Castleman disease (CD) is a very rare B-cell lymphoproliferative disorder marked by the abnormal enlargement of lymph node tissue. It can present as either unicentric (UCD) or multicentric, with the former often appearing in intrathoracic regions, although its presence within the lungs is uncommon.

Methods: We report the case of a 42-year-old woman who underwent resection of an 11 cm intrapulmonary UCD. Additionally, we conducted a systematic review of the demographics, clinical presentation, diagnosis, and treatment approaches for intrapulmonary UCD.

Results: Our review identified 35 documented cases of intrapulmonary UCD, including our case. The average age was 34 years, with a female predominance of 57.7%. Tumor sizes ranged from 1.5 to 11 cm, with our case being the largest. Of the 24 cases with reported anamnesis, 58.3% were asymptomatic, while 41.7% had nonspecific symptoms such as cough, chest pain, or fever (as in our case). Histological analysis was available for 24 cases, with 83.3% identified as the hyaline vascular type. Biopsies through small needle aspiration or fresh-frozen samples failed in all attempts, requiring resection for diagnosis and treatment. Due to high vascularity, delicate location, and lack of diagnosis, lobectomy or pneumonectomy was performed in 45.7% of cases. Among the 11 cases with reported follow-up, no disease recurrence was observed over an average of 3 years.

Conclusion: Our systematic review highlights the rarity of UCD in the lungs. The demographics of intrapulmonary UCD align with the general disease profile. Surgical removal is crucial for both diagnosis and treatment. The significant vascularity and pulmonary location of these tumors present challenges, requiring pre-operative awareness and precautions.