Giant dedifferentiated liposarcoma of small bowel mesentery: a case report.

Susanta Meher,Tushar Subhadarshan Mishra,Satyajit Rath,Pritinanda Mishra,Susama Patra,Prakash Kumar Sasmal

doi:10.1186/s12957-016-1007-1

Susanta Meher, Tushar Subhadarshan Mishra + Show 4 more

Open Access

https://doi.org/10.1186/s12957-016-1007-1

Copy DOI

Abstract

BackgroundDedifferentiated liposarcoma is an uncommon variant of liposarcoma, with poor prognosis and higher preponderance to local recurrence. Only nine cases of dedifferentiated liposarcoma of small bowel mesentery have been reported till now. This is a case of giant dedifferentiated liposarcoma of the small bowel mesentery, weighing nearly 9 kg (19.8 lbs), with synchronous lesions in the extraperitoneal space, which is the first such case to be reported.Case presentationWe report a case of a 62-year-old man, who presented with a huge abdominal mass occupying nearly the entire abdomen. A contrast enhanced computed tomography of abdomen and pelvis revealed a large, poorly enhancing, heterogeneous, lobulated mass of size 27 × 16 cm, displacing the bowel loops peripherally. At laparotomy, a large mass arising from the small bowel mesentery was found. In addition, many other smaller synchronous lesions were studded in the entire small bowel mesentery and a couple more in the extraperitoneal space. A palliative excision of the giant mass along with the adjacent small bowel was done. The other smaller swellings were not causing any mass effect and were left behind as they were numerous, virtually ruling out any possibility of a curative excision. The histopathological examination suggested the diagnosis of dedifferentiated liposarcoma. On immunohistochemistry, S-100 was positive in the well-differentiated sarcomatous areas. The CD 117 and SMA were strongly negative ruling out the possibility of a gastrointestinal stromal tumour. The CD 34 however was positive in the tumour cells.ConclusionsDedifferentiated liposarcoma of the small bowel mesentery is rare. Involvement of nearly whole of the small bowel mesentery in the disease process virtually rules out the possibility of a curative resection, the mainstay of management. This report would add to the knowledge of this rare disease and the possible therapeutic problem that may be encountered in case of multifocal disease.

Highlights

ConclusionsDedifferentiated liposarcoma of the small bowel mesentery is rare
Dedifferentiated liposarcoma is an uncommon variant of liposarcoma, with poor prognosis and higher preponderance to local recurrence
Involvement of nearly whole of the small bowel mesentery in the disease process virtually rules out the possibility of a curative resection, the mainstay of management

Summary

Conclusions

Dedifferentiated liposarcoma is a variant of liposarcoma having worse prognosis and higher risk of local recurrence than the well-differentiated variety. Accurate histopathological diagnosis is essential for prognostication and closer follow-up. Complete surgical excision with negative margin is associated with overall survival and disease-free survival. DDLPS at multiple locations in the mesentery is a possibility which may exclude the chance of curative resection. Palliative resection upfront, of large offending masses, or upon recurrence will have the benefit by relieving pressure symptoms due to the tumour. The scope of adjuvant chemotherapy and radiotherapy is limited to recurrent, residual or poorly differentiated liposarcomas

Background

Findings

Discussion