陳建廷¹、江恆杰^1,2、嚴孟意^1,2、陳俊吉^1,2、林介山²

¹員林基督教醫院 外科部 泌尿科

²彰化基督教醫院 外科部 泌尿科

Jian Ting Chen¹, Heng-Chieh Chiang^{1, 2}, Meng-Yi Yan ^{1, 2}, Chun-Chi Chen^{1, 2}, Jesun Lin^{1, 2}

¹Division of Urology, Department of Surgery, Yuanlin Christian Hospital, Changhua

²Division of Urology, Department of Surgery, Changhua Christian Hospital, Changhua

 

Purpose: Soft tissue sarcomas are very rare tumors; however, there are many histologic subtypes.

Among those subtypes, liposarcoma is one of the most common soft tissue sarcomas. Liposarcomas are generally located in the head, neck,trunk, mediastinum, upper and lower extremities, gastrointestinal tract, and retroperitoneum.They commonly occur in patients aged 40–60 years, and men and women are equally affected.We present a case of 48-year-old woman had a retroperitoneal liposarcoma encasing the right kidney which was successfully removed with kidney and there was no short term recurrence.

 

Case presentation: A 48-year-old female patient presented with progressive abdominal distension. Physical examination revealed a large, palpable mass and CT scan showed a big lobulated mass at right retroperitoneal region,about 24cm in maximal diameter, heterogeneous enhanced soft tissue with some fatty component, liposarcoma is most likely.The tumor had the characteristic appearance of a retroperitoneal sarcoma (RPS) and was not biopsied. Chest CT is performed and there was no lung metastasis noted. En-block resection of the tumor with concomitant right nephrectomy was performed. The specimen is 32x14x14 cm in size and near 7 kg in weight.Surgical pathology was consistent with R0 resection of FNCLCC grade 3 retroperitoneal dedifferentiated liposarcoma. Patient had an uneventful postoperative course and was discharged home without complications.CT scan was done 3 and 6 months after the operation, there was no recurrence noted.

 

Conclusions: Retroperitoneal sarcomas are rare tumors, having an annual incidence of 0.3 to 0.4 new cases per 100,000 occupants with liposarcoma being the most common variant. Complete R0 surgical 

resection is the cornerstone of therapy, offering 50–70 % 5-year survival. No studies have proven the efficacy of postoperative external beam radiation following complete gross resection.