Rare Diseases Symptoms Automatic Extraction

Experience with surgical treatment of retroperitoneal soft tissue sarcomas at a university hospital in Thailand.

[liposarcoma]

Retroperitoneal soft tissue sarcomas (RSTS) are rare malignant tumors with a distinguishing feature of slow growth in the silent retroperitoneal space. The patients usually present late with a large retroperitoneal mass surrounded by the major vascular structures and visceral organs rendering curative resection an extremely difficult and risky operation. The purpose of the present study was to demonstrate surgical experience and results of treatment of RSTS at King Chulalongkorn Memorial Hospital. Operative techniques of these complex surgical procedures were also described.A retrospective study was performed in patients who had RSTS and underwent surgical resection between June 2003 and November 2011 at King Chulalongkorn Memorial Hospital, Bangkok, Thailand. All patients were followed after the operations until death or last follow-up at the out-patient clinic in October 2012. Data collection included demographic data, details of operations, operative complications, neoadjuvant and adjuvant chemoradiation therapy, local recurrence, treatment of local recurrence, and 5-year overall survival rate. Factors associated with local recurrence were also examined.During the 9.4-year period, 18 patients entered into the present study. Fourteen (77.8%) were female and four (22.2%) were male. The age ranged from 44 to 80 years (median 53.5 years). Duration of symptoms ranged from one week to 24 months (median 3.5 months). The tumor size ranged from 10 to 48 cm (median 27 cm) in greatest dimension. All patients underwent preoperative CT scan. Preoperative core needle biopsy was performed in one patient. One patient had preoperative radiation therapy. Sixteen patients (88.9%) underwent complete gross resection (CGR) (R0 or R1 resection) and two (11.1%) had palliative resection (R2 resection). All patients who had CGR (n = 16) had one or more contiguous organ resection (kidney 87.5%, colon 50%, or adrenal gland 43.7%). The operative time ranged from 120 to 360 minutes (median 330 minutes). The operative blood transfusion ranged from 0 to 12 units (median 2.5 units). Postoperative bleeding complication requiring reoperation occurred in three patients (16.7%). One patient had postoperative uncomplicated pancreatic fistula. There was no perioperative mortality. The final pathological reports were liposarcoma in 15 patients (83.3%). Other histology were atypical lipomatous tumor malignant fibrous histiocytoma, and unspecified spindle cell tumor in one patient each. Two patients who had palliative resection died at six and 16 months after the operations. Local recurrence occurred in five patients who had CGR (31.3%). One of them died at 60 months after the operation. The median follow-up time in patients who underwent CGR was 39.5 months (range 12-114 months). The 5-year overall survival of the entire cohort was 73.5% (95% CI: 44.3-88.4%). The 5-year overall survival of patients who had CGR was 83.3% (95% CI: 53.5-98.5%). Univariate analysis of the tumor size, tumor grading, status of the surgical margins, and primary operation or re-resection revealed no statistical significance in patients who had CGR with and without local recurrence.Acceptable outcomes after complete surgical resection of the RSTS were achieved from this small but important case-series. The authors have demonstrated that CGR with concomitant resection of the contiguous organs can be safely performed in patients with large RSTS. Preoperative CT scan was invaluable for diagnosis and treatment plan. Preoperative core needle biopsy was not necessary when preoperative CT scan was diagnostic. Intention for curative resection should be attempted whenever possible to minimize chance of local recurrence and improve survival. Experience of the surgical team is an important factor for successful results when conducting these technically demanding operations.