Primary Small Bowel Liposarcoma (Atypical Lipomatous Tumour) with Myogenic Differentiation

J Patel,T A Macculloch,R Deb,W Speake

doi:10.1155/2010/807981

Abstract

Primary small intestinal liposarcomas originating in the small bowel are uncommon with a generally poor prognosis due to the advanced stage at the time of diagnosis. We describe a case of primary small bowel dedifferentiated liposarcoma presenting as a solid mass in the right iliac fossa. The current case is unusual as the tumour seemingly originated from the bowel and the well-differentiated component was seen extensively infiltrating the bowel wall including the small bowel submucosa.

Highlights

Primary small intestinal malignant mesenchymal tumours are uncommon, and liposarcomas originating in the small bowel are extraordinarily rare [1]
Radiological, and pathologic findings of a primary small bowel dedifferentiated-type liposarcoma with divergent myogenic differentiation
It was subsequently recognised that atypical lipomas and WDLPS have no metastatic potential without dedifferentiation and the nomenclature of these tumours was standardised in the WHO 2002 classification of soft tissue tumours

Summary

Introduction

Primary small intestinal malignant mesenchymal tumours are uncommon, and liposarcomas originating in the small bowel are extraordinarily rare [1]. The early clinical symptoms of these malignancies are nonspecific and for this reason the disease is often diagnosed at an advanced stage. The prognosis of these lesions is generally poor owing to the diffusion of the disease at the time of diagnosis. Small bowel neoplasms are preoperatively identified only in 27–72% of cases and the percentage of surgical removal is from 65–80% in clinical literature [2]. Radiological, and pathologic findings of a primary small bowel dedifferentiated-type liposarcoma with divergent myogenic differentiation

Case Report

Discussion

Findings

Conclusion