[Clinicopathologic features and prognosis of retroperitoneal unicentric Castleman disease: report of 38 patients].

Objective

To explore the clinical and pathological characteristics and prognosis of retroperitoneal unicentric Castleman disease (UCD).

Methods

This study employed a retrospective observational research design. The inclusion criteria were: (1) postoperative pathological confirmation of Castleman disease; (2) patients underwent surgical treatment; (3) preoperative imaging diagnosis of a retroperitoneal unicentric lesion; (4) complete clinicopath- ological data. Exclusion criteria: (1) received preoperative antitumor therapy; (2) had a history of other malignancies within the past 5 years. According to these criteria, clinicopathological and follow-up data of 38 patients with retroperitoneal UCD treated at Tianjin Medical University Cancer Hospital from January 2015 to August 2024 were collected. The clinicopathological characteristics and prognosis were analyzed.

Results

Among the 38 patients with retroperitoneal UCD, there were 12 males and 26 females. The mean age was (42.8±15.4) years (range, 18-70 years), and the mean maximum tumor diameter was (5.8±2.3) cm. The disease had no specific clinical manifestations, and 28 cases (73.7%) were detected during physical examination. Of the 38 patients, 35 (92.1%) underwent radical resection, while 3 (7.9%) received intracapsular piecemeal resection due to large, invasive tumor growth. Postoperative pathology revealed that 32 cases (84.2%) were of the hyaline vascular type, and 3 cases (7.9%) each were of the plasma cell type and mixed type, respectively. The median follow-up duration was 32.4 months (range, 3-78 months). Eight patients were lost to follow-up, 6 died of other diseases, and the remaining 24 patients had no recurrence during postoperative follow-up (including 3 patients who underwent piecemeal resection, with no tumor progression). Competing risk model analysis showed that the 5-year tumor-specific survival rate of patients with retroperitoneal UCD after surgical treatment was 100%. In the sensitivity analysis, the 5-year survival rate under the worst-case scenario was 75.0%.

Conclusions

Retroperitoneal unicentric Castleman disease has a low incidence and lacks specific clinical manifestations. Surgical treatment is the main therapeutic approach, and patients have a favorable prognosis.