後腹腔肉瘤 ─ 台北榮總之經驗
林冠榮1、魏子鈞1,2,3、張延驊1,2,3、林登龍1,2,3、陳光國1,2,3
台北榮民總醫院 泌尿部1；國立陽明大學醫學院 泌尿學科2書田泌尿科學研究中心3
Retroperitoneal sarcoma: a single institute experience with literature review
Kuan-Jung Lin1, Tzu-Chun Wei1,2,3, Yen-Hua Chang1,2,3, Alex Tong-Long Lin1,2,3, Kuang-Kuo Chen1,2,3
Department of Urology, Taipei Veterans General Hospital1; School of Medicine2 and Shu-Tien Urological Institute3, National Yang-Ming University, Taiwan
Purpose:
Soft tissue sarcoma (STS) accounts for less than one percent of all malignant tumors in adults. About 10–15% of adult STS are located in the retroperitoneum. Liposarcoma is the most common variant,more than 50%. Retroperitoneal sarcoma is a rare tumor and the surgical intervention is quite challenging due to its size, deepth and easy recurrence. We retrospectively reviewed patients with retroperitoneal sarcoma in our hospital.
Materials and Methods:
From 2010 to 2015, there are 34 patients with retroperitoneal sarcoma in Taipei Veteran General Hospital. We retrospectively reviewed their clinical data, stage, pathological features, treatment, recurrence rate, progression free and overall survival rate.
Results:
Among 34 patients , 24 are males and 10 females. The mean age of the patients was 62.4 ± 10.6 (range 42-86). The mean tumor size was 19.0 ± 7.4 cm (range 5-38). As for histological subtypes, 7 were well‑differentiated liposarcoma (20.6%), 23 were dedifferentiated liposarcoma (67.6%), 2 were round cells sarcoma (5.9%) and 2 were leiomyosarcoma (5.9%). All except one patient had tumor larger than 5 cm (T2b). One patient had lymphadenopathy and 3 had lung metastases at the time of diagnosis. Complete resections (R0) were of 44.1%, microscopic incomplete (R1) 41.2%, and grossly incomplete (R2) 14.7%. Twenty patients recieved combined resection of adjacent organs because of tumor adherence, mainly including kidney and colon. Median follow-up duration was 23.6 months. Twenty patients (58.8%) had local recurrence and seven patients (20.6%) had distant lung metastases. The mortality rate was 11.8%.. The median progression free survival (PFS) and overall survival (OS) were 9.1 and 23.6 months.
Conclusion:
Retroperitoneal sarcoma is a rare disease with high recurrence rate. Complete surgical resection is currently the standard treatment and combined resection of adjacent organs may be necessary. Patients who have dedifferentiated liposarcoma, incomplete resection with sparing of involved organs, or further necessity of chemotherapy tended to be more poorly prognostic.