年輕病患罕見小腸繫膜脂肪肉瘤
陳怡璇1,2、闕光瞬1,2、阮雍順1,2
1高雄市立大同醫院、2高雄醫學大學附設中和醫院
A case of giant dedifferentiated small intestine mesenteric liposarcoma in young age.
Chen Yi-Hsuan, Kuang-Shun Chueh, Yung-Shun Juan
Department of Urology, Kaohsiung Municipal Ta-Tung Hospital, Kaohsiung, Taiwan
Department of Urology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan
Case description:
This is a 35-year-old female presented with green colored stool passage for 2-3 days, decreased appetite in recent 6 months, body weight loss 69kg to 57kg in 1 year with no diet control. She also complained of nausea when hungry, but improved after oral intake. Upon gastrointestinal outpatient department, physical exam showed palpable abdominal mass. Lab datas were within normal range, including tumor markers (CA-199, CA-125, CEA, α-FETO PROTEIN, β-HCG, LDH). Abdominal echo was also performed which showed a large heterogeneous mass. Further investigation was done, and abdominal CT showed favoring two huge retroperitoneal liposarcoma (17*19cm and 26*28cm) with mass effect (Figure1 and 2). Laparotomy tumor excision was arranged then and the tumor originating from jejunum mesentery was noted (figure 3 and 4). The pathology described dedifferentiated liposarcoma, histologic grade (FNCLCC) 2.
During PET-CT image follow-up 2 months later revealed recurrent tumor, and after surgical excision, the pathology report was well-differentiated liposarcoma, histologic grade (FNCLCC) 1(figure 5). She received 5 cycles of chemotherapy with Doxorubicin + Ifosfamide after operation. However, after 24 months from the second surgery, abdominal CT showed multiple recurrent tumors. Great omentectomy and segmental intestine resection were done owing to the operation finding showed multiple recurrent tumors over great omentum and small intestine mesentery (Figure 5 and 6). The pathology showed recurrent well-differentiated liposarcoma. The patient was currently under regular imaging follow up at our outpatient department.
Liposarcoma is the most common soft tissue sarcoma in adults, most(75%) originated from four limbs and retroperitoneum(20%). Primary mesenteric liposarcoma is an extremely rare, particularly dedifferentiated histological type. According to previous case reports, all of the patients with primary mesenteric liposarcoma were above 50 years old. This is the youngest case of mesenteric liposarcoma which had been reported.