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.

Keywords: Castleman disease; Disorder; Intrapulmonary castleman disease; Lymph node tissue; Thoracic surgery; Unicentric castleman disease.

Fig. (1)

CT scan of the Unicentric Castleman tumor A) hemi-encircling the left upper lobe bronchus in coronal view, B) the inflow into the upper- and lower pulmonary vein in coronal view, C) the attachment and nutritive branch from the pars interlobaris of the pulmonary artery in axial view, D) the broad attachment in the bifurcation of the upper and lower lobe bronchus in axial view, E) the location with neighboring vessels and bronchi in sagittal view. F) PET-CT scan showing slight metabolic activity in the tumor.

Fig. (2)

Unicentric castleman tumor during robotic assisted thoracic surgery A) view on interlobar fissure, B) resection from upper lobe, C) resection from lower lobe, D) resecting branches draining into the lower lobe veins, E) view on the pars interlobaris of the pulmonary artery in the opened interlobar fissure. F) Resected histological specimen.

Fig. (3)

Histological slides of the resected unicentric Castleman tumor of the hyaline vascular type: A) overview (HE x40), B) a typical hyalinized vessel radially penetrating the germinal center (lollipop sign) (HE x100), C) lymphoid follicles with atrophic germinal centers showing proliferation of follicular dendritic cells (HE x100).

Fig. (4)

PRISMA diagram investigating resources on unicentric Castleman disease. A total of 318 articles were retrieved from PubMed. During the initial screening process, 237 articles which did not meet the inclusion criteria were excluded, leaving 81 articles included. A second screening process resulted in 11 and 44 articles being excluded, leaving 26 full length articles. During the final phase of evaluation, a total of four extra articles were discovered from reference lists and Google Scholar, resulting in 30 pulmonary UCD articles and 34 cases.