Saudi Journal of Gastroenterology
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CASE REPORT Table of Contents   
Year : 2005  |  Volume : 11  |  Issue : 3  |  Page : 170-172
Leiomyosarcoma of the small intestine presenting as intussusception


Department of Surgery, King Khalid University Hospital, Riyadh, Saudi Arabia

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Date of Submission02-Nov-2003
Date of Acceptance16-Jul-2005
 

How to cite this article:
Noureldine OH, Bokhari AA, El-Tinay OF. Leiomyosarcoma of the small intestine presenting as intussusception. Saudi J Gastroenterol 2005;11:170-2

How to cite this URL:
Noureldine OH, Bokhari AA, El-Tinay OF. Leiomyosarcoma of the small intestine presenting as intussusception. Saudi J Gastroenterol [serial online] 2005 [cited 2014 Dec 22];11:170-2. Available from: http://www.saudijgastro.com/text.asp?2005/11/3/170/33321


Tumors of the small intestine are relatively rare, and both their diagnosis and treatment challenge the gastrointestinal surgeon. Although the small intestine constitutes 75% of the length of the gastrointestinal tract, only 1-2% of gastrointestinal malignancies occur in this segment and 10 20% are leiomyosarcoma [1],[2],[3],[4] . This infrequent, occurrence compared with malignancies of the stomach or colon, is accompanied by non-specific clinical symptoms and inconclusive or incorrectly interpreted diagnostic tests. The consequences are significant delay in the presentation and diagnosis and therefore finding an advanced, incurable disease at operation. Wide surgical resection of early lesions is the only potentially curative treatment, but it is possible in only a minority of patients [4],[5] . Adult intussusception is associated with definable pathological lesion or lead point in 70-90% of the cases [4],[5] and usually presents with symptoms mimicking partial bowel obstruction. Preoperative diagnosis is often difficult to establish. Computed tomography has proven useful in establishing the diagnosis especially if combined with small bowel series; however, diagnosis is often established at surgery.

We present a case of leiomyosarcoma of small bowel presenting late with intussusception and liver metastasis.


   Case Report Top


A 63-year-old male presented to the emergency room of King Khalid University Hospital, Riyadh with upper abdominal pain, distention, vomiting and rectal bleeding for 4 days prior to admission. The patient was treated for nonspecific abdominal pain six months prior to current admission. He was known hypertensive on medication. At presentation, his general condition was poor, pale, dehydrated and tachycardic 11Ob/min, blood pressure 100/60. No peripheral lymphadenopathy. Cardiovascular examination was normal, chest examination revealed bilateral crepitations and abdominal examination showed distended abdomen with exaggerated bowel sounds and reducible paraumbilical hernia. No masses or organomegaly could be felt. Per rectal examination showed melena, no masses. After initial resuscitation, the patient was admitted for further investigation.

At presentation his hemoglobin was low (6.1gm %), the ESR was high (60), white blood cell count (12.6g/dl), liver function tests were normal, urea and electrolyte and amylase were normal. Upper gastrointestinal endoscopy was normal. Colonoscopy revealed no source of bleeding in large bowel but blood noticed coming from small bowel. Labeled RBC scan revealed bleeding from upper jejunum. Computed tomography of the abdomen showed a bowel within bowel diagnostic of intussusception at the jejunal level, with possible liver metastasis, no ascites and no intraabdominal lymphadenopathy [Figure - 1].

Chest computed tomography showed right pretracheal and subcarinal lymph nodes with minimal pleural effusion. Based on the above findings the patient was taken up for exploratory laparotomy two weeks after admission.

Operative findings revealed an intussuscepted jejunum loop, 15 cm distal to the duodeno jejunal flexure with congested outer surface and areas of dimpling. The inner surface showed a large fungating mass measuring 1 1 x 6 x 3 cm and another polypoid mass measuring 3 x 1.5 x 3 cm with tumor deposits in the serosal surface. Liver and spleen were normal. There was no ascites or mesenteric lymph nodes. The involved segment was resected with end to end anastomosis using GIA 50.

The postoperative course was uneventful apart from wound infection that was cultured and treated with daily dressing and oral antibiotics according to culture and sensitivity. Pathological examination of the specimen revealed two mass lesions measuring 11 x 6 x 3 cm and the other mass measure 3 x 1.5 x 3 cm located in the muscularis propria exhibiting smooth muscle differentiation.

The malignant cells were positive for vimentin and smooth muscle actin immunostains. Other immunostains were negative (cytokeratin, CD34, CD45, CD 117, desmin and S100 protein). The tumor involves the serosal layer with vascular invasion present. The proximal and distal margins are free of tumor and no lymph nodes.

The final diagnosis of malignant high-grade leiomyosarcoma was made. Upon the suspicious of liver lesions preoperatively as there were no palpable liver lesions intraoperatively we proceeded for MRI as suggested by our oncologist which showed multiple bilobal liver metastases. Due to the evidence of metastatic disease the patient was not offered any chemotherapy and was kept on regular follow-up by our oncologist for palliative care till seven months post surgery the patient was readmitted with deterioration in his condition evident by ascites renal impairment and eventually to multiple organ failure and died after fourteen months of his diagnosis.


   Discussion Top


Primary neoplasm's of small bowel are rare and constitute 1-3% of GIT malignancies [1],[2],[3],[4] . The histological types are adenocarcinoma which constitute 30-50%, carcinoid tumors 28%, leiomyosarcomas 1020% and lymphoma 10-15%. The reasons for the malignant potential of the small bowel mucosa are unknown. The polyp to cancer sequence is not well established for simple tubular adenoma of small bowel except in periampullary region [6],[7]

The infrequency of small bowel tumors makes their natural history and factors affecting their growth poorly defined. The detection of neoplasm of small intestine is further complicated by their non-specific symptoms [2] ; the most common presentation of small bowel tumors is gastrointestinal bleeding and anemia. Abdominal pain and palpable mass is present in 5-50% of patients. Other symptoms include nausea, vomiting and weakness [4] . Intestinal obstruction is seen in less than 5% patients with smooth muscle tumor. Intussusception is a rare phenomenon in patients with leiomyosarcoma and tumors of ileal region presents with intussusception more often than jejunal location [4]

Review of English literature revealed only 2 cases of jejunal intussusception caused by leiomyosarcoma [4]

More recently, gastrointestinal mesenchymal tumors have been classified as a separate entity (from epithelial tumors) termed gastrointestinal stromal tumors (GIST). They arise from hollow viscus wall including smooth muscle, fibrous tissue, nerve vessels and fat. They have highly variable clinical course and a different malignant potential [8],[9] as well as staging system and prognostic factors [10],[11] , main treatment of small bowel tumours remains surgical excision.

Small bowel tumors are relatively sensitive to chemotherapy and resistant to radiotherapy.

In conclusion, the Jejunal leiomyosarcoma is a rare variety of malignant small bowel tumor with diverse presentation, heterogeneous behavior and high recurrence rate which pose difficulties in the diagnosis, classification and treatment strategies. Radical excision of the contained lesions fails due to progressive natural course of the disease.

 
   References Top

1.Ashley SW, Well SA. Tumors of small intestine. Semin Surg Oncol 1988; 15: 116-28.  Back to cited text no. 1    
2.Herbsman H, Weststein L, Rosen Y, etal. Tumors of small intestine. Curr Prob Surg 1980; 17: 121-8.  Back to cited text no. 2    
3.Coutesftides T, Shibata HR. Primary malignant tumors of small intestine. Dis Colon Rectum 1979; 22: 24-6.  Back to cited text no. 3    
4.Samaiya Atul, Deo SV, Thulkar S, et al. An unusual presentation of malignant jejunal tumor and a different management strategy. World J Surg Oncol 2005; 3:3.  Back to cited text no. 4    
5.Desai N, Wayne M, Taub P, Levitt M, Spiegel R, Kim U. Intussusception in adults. Mount Sinai J Med. 1999; 66: 336-40.  Back to cited text no. 5    
6.Williamson RCN, Welch CE, Malt RA. Adenocarcinoma and lymphoma of small intestine. Ann Surg 1983; 197-80.  Back to cited text no. 6    
7.Deck KB, Silverman H. Leiomyosarcoma of small intestine. Cancer 1979; 44: 323-5.  Back to cited text no. 7  [PUBMED]  
8.Lee JS. Epithelioid GIT tumors: Astudy of fifty-five cases. Surgery 1995; 118: 653-60.  Back to cited text no. 8    
9.Ludwing DJ. Gut stromal tumors and their clinical behavior. Am J Surg 1997; 173: 390-4.  Back to cited text no. 9    
10.Ng EH. Prognostic factors influencing survival in gastrointestinal lieomyosarcoma implications from surgical management and staging. Ann Surg 1992; 215: 68-77.  Back to cited text no. 10    
11.Copper PN. A flow cytometrics, clinical and histological study of stromal neoplasm of GIT. Am J Surgery Pathol 1992; 16: 163-70.  Back to cited text no. 11    

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Correspondence Address:
Osman Hassan Noureldine
Department of Surgery, King Khalid University Hospital, P.O Box 7805 Riyadh 11472
Saudi Arabia
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DOI: 10.4103/1319-3767.33321

PMID: 19861854

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