A 50-year-old woman presents with several year history of increasing abdominal girth, early satiety and weight loss.
- No significant medical or surgical history.
- Physical Exam: presence of a large intraabdominal mass.
- CT imaging: a large retroperitoneal tumour (having both lipomatous and solid components), causing mass effect with displacement and compression of intraabdominal/ retroperitoneal organs.
- No evidence of pulmonary metastasis on CT chest portion.
- Biopsy of the higher grade/dense tumour – dedifferentiated liposarcoma with MDM2 amplification
- Albumin: 3.3g/mL, prelab 5.5mg/dL, CRP 94mg/L
Prognosis: 7-year OS 31% and 7-year DFS 10%
Initial Treatment
- Exploratory laparotomy and radical resection of tumor with right nephroureterectomy, partial colectomy, resection of psoas fascia.
- Postoperative gastroparesis requiring PEG placement for venting and period of TPN- which gradually resolved and G tube removed.
Surveillance
- CT imaging of chest, abdomen and pelvis at 3 months interval
Outcome
No evidence of disease for 2 years, however developed recurrence at pancreas head
What could be the optimal treatment option for this individual at this stage?
According to the current guidelines from ESMO (European Society for Medical Oncology) and TARPSWG (Transatlantic Retroperitoneal Sarcoma Working Group), for the management of retroperitoneal sarcomas, what is the recommended setting?
