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A 76-year-old patient was admitted with right-sided lower abdominal pain. CT scan of the abdomen demonstrated a fatty mass arising around the bowel in the left iliac fossa causing local distortion of the vasculature.
His clinical condition rapidly deteriorated with worsening abdominal signs necessitating an exploratory laparotomy. A segment of perforated small bowel associated with mesenteric mass was excised and the patient died 3 days later.
The histology demonstrated a well-differentiated liposarcoma. Liposarcomas are the most common soft tissue sarcomas in adults but occurrence in the gastrointestinal tract is extremely rare. The clinical presentation of these tumours is frequently non-specific and commonest symptoms are generalised, intermittent abdominal pain.
Surgical resection with clear margins is the treatment of choice for primary liposarcomas. They are moderately radiosensitive and chemotherapy is non-effective.
Although gastrointestinal liposarcomas have been previously reported, this is the first reported case of a primary liposarcoma associated with a small bowel perforation.
The case reported here is the first case of a primary liposarcoma of the small bowel presenting with spontaneous perforation of the bowel.
A 76-year-old man was admitted with a 2-day history of acute onset right-sided lower abdominal pain. It was a constant and gradually worsening type of pain. There was no history of nausea, vomiting, fever, loss of appetite or loss of weight, or disturbance of bowel function.
He had a background history of left-sided pleural effusion, chronic renal failure, hypertension, chronic anaemia and two recent cerebrovascular events.
On examination he was haemodynamically stable with mild pallor (haemoglobin 10.1 g/dl, mean corpuscular volume 83, mean corpuscular haemoglobin concentration 313). He was mildly tender in the right iliac fossa with no signs of localised peritonism or palpable masses. On auscultation, the bowel sounds were present with normal intensity. The inflammatory markers (C reactive protein) were normal with slightly raised leucocytes (13.4 × 109). During the first 24 h in hospital, his haemoglobin level dropped further to 7 g/dl requiring blood transfusion.
Plain abdominal x-ray and erect chest x-ray were unremarkable. Given his background of anaemia and right-sided abdominal pain, contrast enhanced CT scan showed the appearances of a possible internal hernia/volvulus or small bowel tumour. CT scan was performed because the patient was not peritonitic and had major morbidity, which made laparotomy very high risk. This demonstrated a fatty mass arising around the bowel in the left iliac fossa causing local distortion of the vasculature. Emergency mesenteric angiography was arranged to investigate the rapidly falling haemoglobin and the plan was to attempt embolisation of the presumed bleeding point to avoid the high-risk laparotomy. This revealed considerable architectural distortion of ileal arcades with normal vascularity (figures 1 and and22).
Perforated appendix, acute gastrointestinal bleeding and intestinal obstruction.
Twenty-four hours since the angiographic embolisation, an emergency exploratory laparotomy was performed. A segment of perforated small bowel (ileum) associated with a 20 cm mesenteric mass was excised. There was bleeding into the small bowel, but the tumour had perforated and there was diffuse fatty tumour material within the peritoneal cavity and a large mass, which was the liposarcoma that had burst its capsule.
Postoperatively, the patient was managed in the intensive care unit where he died 3 days later of multi-organ failure, principally respiratory failure.
The histological sections show small bowel with an attached encapsulated cellular tumour composed of adipocytes of different sizes with several blood vessels and scattered inflammatory cells. Although classical lipoblasts are not seen, there were enlarged, slightly hyperchromatic nuclei within the tumour. The adipocytes were stained with Oil Red O and, immunohistochemically, the tumour cells/lipoblasts were positive for S-100 protein.
The histology of the small bowel lesion demonstrated a well-differentiated liposarcoma (table 1).
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.
Competing interests None.
Patient consent Obtained.