Liposarcoma of the colon presenting as an endoluminal mass
© D'Annibale et al; licensee BioMed Central Ltd. 2009
Received: 18 June 2009
Accepted: 23 October 2009
Published: 23 October 2009
Liposarcoma is one of the most common soft tissue sarcoma of adult life, usually occurring in the retroperitoneum and the extremities. Primary liposarcoma of the colon is very rare. The optimal treatment has not been established due to the small number of cases reported. We report a case of primary liposarcoma of the colon presenting as a massive intraluminal lesion.
A 79-year-old woman presented with abdominal pain, progressive constipation and weight loss. A CT scan and a colonoscopy revealed an intraluminal mass in the transverse colon and multiple intraperitoneal lesions. The patient underwent surgical resection of the lesions. Pathologic examination was consistent with pleomorphic liposarcoma of the colon.
Although no guidelines are available for the management of liposarcoma of the colon, surgical resection should be performed when feasible. Our patient's overall survival was satisfactory in spite of the multiple negative prognostic factors.
Liposarcoma is one of the most common soft tissue sarcomas of adult life. Histological classification includes five main subtypes: well-differentiated, myxoid, round cell, pleomorphic and dedifferentiated [1, 2]. Liposarcoma usually occurs in deep soft tissues of extremities and retro peritoneum. A presentation primarily involving the gastrointestinal tract (GI) is unusual and liposarcoma of the colon is extremely rare. To our knowledge, only 8 cases have been reported to date [3–10]. We report a case of a primary locally advanced liposarcoma of the colon presenting as a massive intraluminal mass in the right colic angle.
Twenty eight months after surgery, the patient developed progressive dysphagia with radiological evidence of tumour progression. Thirty months after surgery she died for an acute cardiac event. No autopsy was performed.
Liposarcomas of the large intestine usually present with nonspecific gastrointestinal symptoms such as bleeding, obstruction, diarrhea, abdominal pain and weight loss. It is thus difficult to distinguish them preoperatively from other colon cancers. Primary colonic liposarcoma tends to occur in adults, with a peak incidence between 5th and 6th decades, and an equal sex distribution . WHO classification of liposarcomas recognizes five subtypes: well-differentiated, myxoid, round cell, pleomorphic and dedifferentiated. Pleomorphic liposarcoma is a high grade tumour containing a variable number of pleomorphic lipoblasts. It is aggressive, showing a high metastasis rate, with the lung representing the most common site of metastasis and a tumour associated mortality of 40% .
Review or reported cases of primary liposarcoma of the colon.
Wood and Morgenstern
7.5 × 8 × 12 cm, ileocecal valve
Died 2 yrs
Parks et al.
Abdominal discomfort, diarrhea, anemia, weight loss
6 × 4 × 5 cm, ascending colon
Magro et al
Abdominal pain, intussusception
5 cm, caecum
Alive 6 mo
Abdominal pain, hematochezia
7.5 × 5.5 × 5 cm, descending colon
Alive 2 yrs
Gutsu et al.
12 × 11 × 10 cm, ascending colon
Alive 1 yr
Shahidzadeh et al.
3.5 × 3 × 2.8 cm, hepatic flexure
Chaudhary et al.
Abdominal pain, altered bowel habits, anemia, intussusception
4.5 × 3 × 2 cm, descending colon
Alive 10 mo
Jarboui et al.
Abdominal pain, constipation, weight loss
7 × 6 cm, splenic flexure
Alive 10 mo
Abdominal pain, constipation, weight loss
5 × 5.2 × 4.7 cm, hepatic flexure
Died 2.5 yrs
The small number of cases reported has not allowed the determination of an appropriate diagnostic and therapeutic approach to this rare tumor. Complete surgical excision should be considered the gold standard. In our case, despite wide local excision was performed, the complete removal of the tumour was technically unfeasible due to the numerous extraluminal lipomatoid lesions. Our patient showed macroscopic evidence of residual disease and was thus candidate to further therapy. The role of chemotherapy in the management of liposarcoma has not been yet well established . Conversely, radiotherapy has shown to affect survival rates . Therefore, in consideration of the patient's age and clinical status, we decided to refer her to radiation therapy.
Prognosis for colonic liposarcoma is still difficult to predict. It may be affected by a variety of factors, including location, size, dissemination of the disease, as well as histological type [23, 26]. No single morphological factor can predict the clinical outcome reliably; however, tumour depth size, more than 20 mitosis in 10 HPF (High Power Field), and areas of tumour necrosis are usually associated with a worse clinical prognosis [26, 27]. In our case, despite evidence of multiple negative prognostic factors (dissemination of the disease in contiguous tissues, pleomorphic subtype and areas of necrosis), mitotic count was less than 10 mitosis in 10 HPF, which could justify the lower malignancy observed.
Only a few cases of primary liposarcoma of the colon have been reported and optimal treatment strategies have not been established yet. However, surgery should be performed whenever feasible. The choice of therapeutic options should be based both on the natural history of the disease and on the patient's individual clinical status. The collection of a larger number of cases is needed for the definition of guidelines for the management of this rare tumour.
Patient consent could not be obtained as the patient died. Written informed consent was obtained from the patient's son for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
We acknowledge Josuel Ora, MD for his contribution in literature review and critical revision.
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