Pannu Manjinder Kaur, Ehrsam Jonas Peter, Adamenko Olga Meier, Inci Ilhan, Schöb Othmar Markus
Medical School, University of Nicosia, Nicosia, Cyprus.
School of Medicine, University of Zurich, Zurich, Switzerland.
Open Respir Med J. 2025 Feb 18;19:e18743064348696. doi: 10.2174/0118743064348696250107092627. eCollection 2025.
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.
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.
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.
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.
卡斯特曼病（CD）是一种非常罕见的B细胞淋巴增殖性疾病，其特征为淋巴结组织异常肿大。它可表现为单中心型（UCD）或多中心型，前者常出现在胸腔内区域，尽管其在肺部出现并不常见。
我们报告了一例42岁女性接受11厘米肺内UCD切除术的病例。此外，我们对肺内UCD的人口统计学、临床表现、诊断和治疗方法进行了系统综述。
我们的综述确定了35例有记录的肺内UCD病例，包括我们的病例。平均年龄为34岁，女性占比57.7%。肿瘤大小从1.5厘米到11厘米不等，我们的病例是最大的。在24例有报告病史的病例中，58.3%无症状，而41.7%有非特异性症状，如咳嗽、胸痛或发热（如我们的病例）。24例可进行组织学分析，其中83.3%被鉴定为透明血管型。所有尝试通过小针穿刺活检或新鲜冷冻样本活检均失败，需要进行切除以进行诊断和治疗。由于血管丰富、位置精细且缺乏诊断，45.7%的病例进行了肺叶切除术或全肺切除术。在11例有随访报告的病例中，平均3年未观察到疾病复发。
我们的系统综述强调了UCD在肺部的罕见性。肺内UCD的人口统计学与该疾病的总体特征相符。手术切除对诊断和治疗都至关重要。这些肿瘤显著的血管丰富性和肺部位置带来了挑战，需要术前的认识和预防措施。
