MLPS accounts for about 30% of all liposarcomas and about 10% of all soft tissue tumors. It often occurs in the deep soft tissues of the limbs, most commonly in the thighs, and rarely in the retroperitoneum and abdomen (2, 11, 12).Although MLPS is more common in people aged 50–65, liposarcoma is the predominant pathological type in children and adolescents (11, 13). Myxoid liposarcoma is the second most common subtype of liposarcoma (14). The risk of local recurrence is very low, even for larger tumors (more than 5 cm), the risk of recurrence within 3 years is 15% in the case of positive margins; A negative marginal status leads to a risk of recurrence of less than 10%. Unfortunately, 10% of myxoid liposarcoma patients go on to develop metastatic disease, in which case the 10-year overall survival rate is about 60% (15). However, the three patients whose tumors had metastasized to the pancreas, as summarized in Table 1, appeared to have low overall survival.
Table 1
Review of the treatment and outcome of pancreatic mucinous liposarcoma
Case | Age/ | Primary lesion | Metastatic | Transfer focus location | Size | Treatment | Adjuvant therapy | Outcome | Reference |
No. | Sex | (time) |
1 | 40/F | right thigh | 5 years | Pancreatic body | 14*9*7 cm3 | Medial pancreatectomy | NA | No recurrence (22 months) | 9 |
2 | 66/M | Left knee | 6 years | Pancreatic head | 3 cm | PD | Radiation therapy | No recurrence (6months) | 10 |
3 4 5 | 52/M 35/M 43/F | Knee Thigh Mesentery or neck | 26 months 0 months 9 or 7 years | Pancreatic head Pancreatic tail Pancreatic head | 2.5 cm 11 cm 10*9*8 cm3 | PPPD DP PD | NA CTx NA | Alvie (12months) Dead (16months) No recurrence (12months) | 24 24 Present case |
CT, Computed tomography; PD, pancreaticoduodenectomy; DP, distal pancreatectomy; M, male; F, female; CTx, adjuvant chemotherapy; PPPD, pylorus-preserving pancreaticoduodenectomy. |
MLPS had a tendency of bone metastasis, about 17%, and lung metastasis rate was 14%. Therapeutically, MLPS is more sensitive to cytotoxic chemotherapy drugs than other types of liposarcoma (16–18). Sarcomas preferenically metastasate through the blood rather than the lymphatic system, and lung and bone tissue are the most common sites of metastatic sarcomas (19). A review of the characteristics of metastatic sarcoma of the pancreas showed no lymph node metastasis in all eight cases of lymph node dissection, suggesting that metastatic sarcoma of the pancreas is more likely to occur through the blood than through the lymphatic system (20).We report and summarize 3 cases of myxoid liposarcoma with pancreatic metastasis. Two cases were metastatic to the pancreas from the extremities, and our case was considered to be metastatic from mesenteric or liposarcoma of the neck.
Since myxoid liposarcomas tend to grow slowly and lack symptoms, most patients do not complain of pain at the tumor site. Most of these tumors are large when they are diagnosed (21). The typical radiographic appearance of myxoid-round cell liposarcomas are to The shoe market myxoid matrix of The tumor which is non-septal in contrast studies (22). It is often difficult to determine the origin of such tumors by preoperative radiological examination. Therefore, the surgical procedure needs to be selected during the operation (18). Surgery is viewed as the mainstay treatment of myxoid liposarcomas. In some instances, surgery is combined with neoadjuvant or adjuvant radiation and/or chemotherapy (15). The optimal goal of surgery is to successfully remove the tumor with a function-sparing procedure that maintains a wide excision with negative margins. Radiation therapy has been reported to be effective in myxoid liposarcoma of the extremities (18). When sufficient resection margins cannot be ensured, postoperative irradiation can be performed to prevent recurrence (23). The survival status of different types of liposarcoma also seems to be different (24). However, myxomatous liposarcoma has been rarely reported so far, and we will continue to pay attention to the reports of this particular type of malignant tumor in the future. In our case, we suggested adjuvant therapy after surgery, but the patient refused. Also, unfortunately, the patient died a year later from unexplained gastrointestinal bleeding.
In conclusion, we report a rare case and review the literature to find that myxoid liposarcoma is at risk of metastasis to the pancreas in patients who may benefit from radical surgery.