Liposarcomas are the most common soft tissue sarcomas in adults accounting for approximately 16–18% of all soft tissue neoplasms.1
Liposarcomas occurring in the gastrointestinal tract are extremely rare. They are among the least common malignant tumours and have been reported to occur in the stomach, jejunum, ileo-caecal valve and retro-peritoneum. Most of these tumours arise de novo and are believed to take origin from the undifferentiated perivascular mesenchyme.2
The clinical presentation of these tumours is frequently non-specific. The commonest symptoms are generalised, intermittent abdominal pain and bleeding usually as frank haemorrhage, maelena or occult blood in the faeces.3
WHO classifies these tumours into four categories: well differentiated, myxoid/round cell, pleomorphic and dedifferentiated liposarcomas.4
The myxoid and the well-differentiated types are the commonest and the latter has the best prognosis; the 5-year survival rate ranges between 75% and 100%.5
The myxoid liposarcomas with associated round cell component are said to have a substantially higher risk of metastasis.6
Overall metastatic rates for liposarcoma have been reported to range from 37–50%.7
Surgical resection with clear margins whenever possible is the treatment of choice for primary liposarcomas.8 9
They are moderately radiosensitive with no evidence that chemotherapy is effective. Neither treatment has been proven to be of benefit in increasing long-term survival10
; however, this is such a rare tumour that it is difficult to accurately estimate treatment effect.
There is only a handful of literature published on liposarcomas associated with intestinal perforation. In 1984, the title of Orzechowski and Wlodarczyk's paper in the Medline database indicated that liposarcoma of the intestinal mesentery caused peritonitis. The article was written in Polish and no abstract available. Horiguchi et al
described a case of liposarcoma with peritonitis due to jejunal perforation. It reported the suspected cause of the perforation was adhesions due to a previous operation and was not due to direct infiltration of tumour cells because tumour cells were not observed at the perforation site.11
We believe this is the first reported case of primary liposarcoma associated with a small bowel perforation (confirmed with the histological findings) and represents an important learning point. In retrospect, this patient was clinically very high-risk for anaesthesia and was septic with rapidly falling haemoglobin. Also the presence of a mesenteric mass with distortion of the vasculature caused some uncertainty leading to further investigation.
If not for the above reasons, an earlier laparotomy may have been associated with a more favourable outcome.
- Although, the liposarcomas occurring in the gastrointestinal tract are extremely rare they can cause intestinal perforation.
- The clinical presentation of these tumours is frequently non-specific.
- This unique case represents an important learning point for all clinicians and pathologists, particularly in this age of increased access to cross-sectional imaging. The simple lesson here is to treat the patient not the scan.