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BACKGROUND: Retroperitoneal soft-tissue sarcoma is a very rare neoplasm, the most frequent histological subtype is liposarcoma
with up to 45% of all cases. Unspecific clinical presentation, late diagnosis and high local recurrence rate represent important problems in clinical practice. We present the case of an adult patient with an unusual large liposarcoma of the retroperitoneum analyzing diagnostic workup, surgical approach and therapeutic strategies.
CASE REPORT: A 68-years old female was admitted with weight gain (+12 kg) and increasing abdominal girth. Computed tomography scan imaging showed a retroperitoneal tumor with 40 cm maximum diameter. Biopsy revealed a myxoid liposarcoma. The interdisciplinary curative surgical treatment included preoperative ureteral splinting, en-bloc tumorexstirpation, ileocecal resection, right ureteral resection and vascular reconstruction of the Arteria iliaca communis. The postoperative course was uneventful. After sixteen months the patient developed multifocal local recurrence requiring extensive surgical resection of tumor and retroperitoneal fat (Figs. 3, 4). However, thirteen months later the tumor reappeared and the patient was assigned to palliative chemotherapy. The patient is still alive with stable tumor disease.
CONCLUSION: The removal of a huge retroperitoneal sarcoma is a significant challenge for the surgeon. Accurate planning, interdisciplinary treatment options, and radical surgery are essential. However, the recurrence risk is exceptionally high because of the enormous tumor dimensions and the big tumor surface, multimodal therapeutic approaches may improve the outcome in these patients.