Metachronous multifocal myxoid liposarcoma involving the gastrointestinal tract. Management and literature review
A 69-year-old female presented with a 2-year history of a slowly enlarging right buttock mass. Her past medical history was unremarkable, except for hyperlipidemia. Clinical examination revealed a large, non-tender right buttock mass. The remainder of clinical examination was unremarkable. Computed tomography (CT) scan showed a mass measuring 16 × 15 × 14 cm with imaging features suggestive of a liposarcoma []. Full blood count, biochemical investigations, and tumor markers were all within the normal range. The patient underwent a complete resection of the mass. Histological examination showed a myxoid liposarcoma with clear surgical margins. Staging investigations including CT of the chest and abdomen were unremarkable. Since an excision with clear surgical margins had been performed, the patient was not given any adjuvant treatment.\nEighteen months later, the patient was readmitted complaining of abdominal fullness and dull abdominal pain. Physical examination revealed a large, non-tender mass occupying the left abdomen and a cutaneous mass on the upper inner left thigh. CT scan of the abdomen revealed a large retroperitoneal tumor measuring 22 × 12.2 × 17.5 cm with imaging features suggestive of a liposarcoma. In addition, a smaller tumor measuring 7 × 5 cm was detected in the ileal mesentery close to the ileocecal valve []. At exploratory laparotomy, the retroperitoneal tumor was resected with clear margins. Segmental ileal resection was performed along with sigmoid resection, because a second intraperitoneal tumor was found in the sigmoid mesentery. Wide resection was also performed of the subcutaneous left thigh tumor. Histological examination of all specimens revealed a myxoid liposarcoma with 10-15% round cell component. In addition, the histology of the subcutaneous thigh lesion revealed the presence of tumor emboli within small veins. The patient was given postoperative adjuvant chemotherapy with 6 courses of ifosfamide 5 g/m2 and doxorubicin 50 mg/m2. Follow-up investigations after completion of chemotherapy were negative for recurrent disease. However, 2 years later, on CT scans, multiple intraperitoneal and left retroperitoneal masses were detected despite the excellent physical condition of the patient. At this point, the multidisciplinary team suggested chemotherapy with 3 courses of ifosfamide 6 g/m2, followed by 6 courses of doxorubicin 25 mg/m2. Despite initial stabilization, progression of the disease was noted and the patient was started on new chemotherapy with 6 courses of liposomal doxorubicin with simultaneous administration of granulocyte colony-stimulating factor and erythropoietin. Unfortunately, although the size of the lesions was initially stabilized, the disease progressed again. Finally, on May 2011, a new chemotherapy regimen consisting of trabectedin was started. After the administration of 6 courses, a significant decrease of the lesions’ size was noted, and, thus, it was decided to administer 3 more courses. Presently, the patient remains in a stable partial remission of the disease and is scheduled for new investigations.