Abstract

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Liposarcoma is a rare and primary malignancy developed from mesenchymal tissu. We report the case of a 65-year-old woman who had a complete excision of a 40 cm retroperitoneal myxoid liposarcoma. THE FOLLOW-UP in 6 months has not objectified local or remote recurrence,with the literature data, we review the pathological, therapeutic and prognostic aspects of this tumor.

Introduction

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The retro peritoneal liposarcomas are rare mesenchymal tumors, they represent 7.5 to 25% of all soft tissue sarcomas and 1 to 2% of genitourinary malignancies. They are primitive and are independent of the retroperitoneal space. Their diagnosis has largely benefited from advances in imaging, however, they continue to acknowledge the difficulties of orders diagnostic, therapeutic and above all scalable.

Case Report(s)

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MQM, aged 65, had diabetes and hypertension poorly followed, who had submitted four months before admission left low back pain radiating to the left hypochondrium with a progressive increase in abdominal volume while operating in the context of conservation status general. The examination showed a mass range of the left hypochondrium to the left flank of 20/15cm, of hard consistency, fixed relative to the two plans, painless, giving the contact lumbar. The ultrasound and CT abdominopelvic objectified have a huge heterogeneous tumor tissue of 22cm in diameter start taking the lower pole of left kidney associated with significant renal lipomatosis perished.(illustration 1,2) The patient was operated. A midline incision straddles the umbilicus with open posterior parietal peritoneum, allowing en bloc resection of the left kidney with a tumor of 40x35x18 cm and weighing 7 kg.(illustration 3) Histological examination showed a myxoid liposarcoma grade I (FLNCC) infiltrating the adjacent renal parenchyma.(illustration 4)

Discussion

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Liposarcomas are rare mesenchymal tumors occupying frequently the soft tissues of the extremities. They sit in 12-40% of cases in the retroperitoneal space [1, 2, 12, 17]. However, recent studies have shown that undifferentiated forms frequently sat in the retroperitoneal space that in the soft tissues of the ends [16]. The age of predilection LSRP of between 40 and 60 [1, 2, 10, 11, 12], but they can be observed at any age [1, 2], with a slight predominance Women [1]. Diagnosis is often delayed LSRP see difficult because o their location and deep posterior, which explains the appearance extensive at the momment diagnosis [2, 3, 5, 12]. The clinical developers are often late, variable and non specific [1, 4, 12]. They are dominated by abdominal pain that is present in 50 to 88% in diffuse type of gravity, sometimes intense, often seat thoracolumbar or pelvic [1, 3, 6, 7, 13].The gastrointestinal symptoms in type of vomiting, gastrointestinal bleeding, bowel dysfunction, are observed in 60% of cases [1]. Other Signs can be found: neurological side to invasion or compression of the lumbosacral plexus or its branches, urinary (6-11% of cases) [1],  or venous compression (10-11% of cases) [13], the condition is often retained, but can be altered with fatigue and weight loss in the very forms advanced [2, 15]. Ultrasound, CT and magnetic imaging résonnace (MRI) examinations are the key to the exploration of retroperitoneum [16]. CT seems to be the best diagnostic tool to indicate the location retroperitoneal mass, to judge its extirpabilité and predict operational difficulties. MRI can identify the characteristics of tumor, detect a possible vascular invasion indicating the extent of the thrombus [1, 8, 12]. It also allows early detection of recurrences thus constituting an excellent way of monitoring postoperative [12, 16].
The histology, the diagnosis depends on identification of lipoblaste [17]. These tumors are classified according to the predominance in five tissue types [17, 19]:
1. The well-differentiated liposarcoma composed of fat cells
mature.
2. The myxoid liposarcoma composed of spindle cells in a myxoid matrix, abundant.
3. The round cell liposarcoma composed of homogeneous layers cells round, oval or sometimes fusiform relatively regular.
4. The pleomorphic liposarcoma cell-rich nuclei with very atypical,nucleoli and often in mitosis.
5. The undifferentiated liposarcoma associated areas of liposarcoma well differentiated and poorly differentiated other.
The treatment is surgical. It consists of a wide excision of the tumor [1,14,18] sometimes taking organs Neighbourhood: kidney, colon, stomach, duodenum, small intestine, pancreas[2]. Some authors propose a study to extemporaneous to ensure the integrity of the limits of resection [2]. The incision transperitoneal is the most utitilisée, and by site of tumor, it can be a way thoracoabdominal, laparotomy median or a transverse way [1]. Complementary therapies are of limited value because their use has not shown any survival benefit or decrease the rate of metastasis [2, 14]. Radiotherapy may be used preoperatively for tumors unresectable or postoperative recurrences between two [1, 8] Chemotherapy can be used in the treatment of metastatic tumors and recurrent in the adjuvant or neoadjuvant, however the overall results remain disappointing [14]. The prognosis of LSRP depends strongly on the degree of differentiation and quality of excision, it is marked by a tendency to recurrence and a rapidly fatal [11]. Recurrences are increasingly early, they occur within 2 to 5 years after excision, The survival rate at 5 years is greater than 75% in histological type myxoid and well differentiated, approximately half the cases develop recurrences local and very rarely with distant metastases. On the other hand, the survival rate at 5 years does not exceed 20% for liposarcomas round cell, pleomorphic and undifferentiated [14].

Conclusion

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Although very rare, retroperitoneal liposarcoma remains one of the most common mesenchymal sarcomas. The diagnosis is often delayed because of clinical polymorphism and evolution in low noise of the tumor. The working diagnosis is mainly based on imaging. Surgery is the treatment of choice. The evolution is marked by the risk of local recurrence. The prognosis is unfortunate, but it can be improved by early diagnosis and complete surgical excision

References

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Source(s) of Funding

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None

Competing Interests

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No competing interests

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