gms | German Medical Science

Introduction: Retroperitoneal sarcomas are rare neoplasms that often present with invasion of multiple viscera. The frequency of distant metastases is low whereas the major burden is loco-regional tumor progress. Liposarcoma is the most common primary retroperitoneal neoplasm. The most frequent symptoms include abdominal pain, ileus and a palpable abdominal mass. Radical resection currently represents the most effective treatment; the aggressiveness of surgery is individually planned and considers patients and tumor related factors. Margin-negative resection often mandates extirpation of multiple retroperitoneal and intra-abdominal viscera; nonetheless such operations are fraught with high rates of locoregional recurrence. Neo and or -adjuvant radio-chemotherapy may facilitate higher rates of mid- and longterm tumor control, reduce local recurrences and improve survival. The decision making to apply multimodal regimens is left for clinical judgement.

Material and methods: All documents required where available for detailed presentation of the case. Written case history, medical records, surgery-, CT-scan- and pathology reports where used for an adequate extraction of scientific information.

Results: A 46 years old female presented with a giant retroperitoneal liposarcoma, which extended from the diaphragm to the pelvis with an 360° encasement of the abdominal aorta. After interdisciplinary discussion of the case in our tumor board, decision was made to administer four cycles of neoadjuvant hyperthermic chemotherapy using doxorubicin & ifosamide followed, after reassessment by an extensive 12 hours multi-visceral resection including parts of the left diaphragm, pancreas body and tail, left kidney, descending colon, spleen and abdominal aorta and an additional radical retroperitoneal lymphadenectomy. The extracted sarcoma weighted 6,4kg and the imbedded dimensions from medial to lateral, cranial to caudal and ventral to dorsal where 37cm, 35cm and 17cm respectively. The postoperative follow up was without surgical complications. A port infection with candida albicans contributed to a delayed hospitalization of 34 days before discharge. The patient was fully mobilized and showed no signs of any neurological focal deficits.

Conclusion: Current data shows that well and dedifferentiated retroperitoneal liposarcomas are at diagnosis often multifocal involving multiple intra-peritoneal and retroperitoneal organs. Although reports showed that neoadjuvant therapy can significantly reduce tumor size and pre-existing symptoms, extensive surgery remains the mainstay of treatment for local tumor control with curative intention.