Surgical resection is the mainstay of treatment for
retroperitoneal liposarcoma (RPLS). Herein, we describe a case of dedifferentiated RPLS successfully treated with an extended surgical approach with
adjuvant chemotherapy. A 61-year-old male was referred to our hospital with a chief complaint of chest tightness. Abdominal computed tomography revealed a large retroperitoneal
tumor, 11 cm in diameter, extensively invading the surrounding organs: the celiac axis, the splenic artery, the pancreatic body and tail, the lesser curvature of the stomach and the left adrenal gland. Endoscopic ultrasound-guided fine-needle aspiration biopsy confirmed
dedifferentiated liposarcoma, suggesting aggressive
tumor biology. We performed total
gastrectomy combined with distal
pancreatectomy with celiac axis and left adrenal gland resection with a curative intent. The postoperative course was almost uneventful. As the pathological findings indicated a positive margin with a
well-differentiated liposarcoma component, we added
adjuvant chemotherapy with four cycles of
doxorubicin and
ifosfamide (AI). Five years after primary surgery, regular follow-up CT demonstrated a pulmonary hilar lymph node enlargement and a
tumor at paraesophageal locations. After downsizing
chemotherapy with
eribulin followed by
pazopanib, he underwent partial
esophagectomy with dissection of the paraesophageal
tumor. The pathological findings indicated recurrence of
dedifferentiated liposarcoma with a
tumor-free
surgical margin. He is currently alive without any evidence of recurrence almost 7 years after the first surgery and 15 months after the second surgery. The long-term survival gained in this patient indicates that extended resections and
adjuvant chemotherapy could prolong survival in patients even with RPLS with dedifferentiated
tumor histology.