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GASTROINTESTINAL Table of Contents   
Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 503-504
Carcinoid tumor of the Ileum


Department of Radiodiagnosis, Dyanand Medical College & Hospital, Ludhiana - 141001, India

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Date of Submission20-May-2006
Date of Acceptance10-Aug-2006
 

Keywords: Carcinoid, Ileum, Contrast enhanced CT

How to cite this article:
Kalia V, Saggar K, Sandhu P, Ahluwalia A, Vibhuti. Carcinoid tumor of the Ileum. Indian J Radiol Imaging 2006;16:503-4

How to cite this URL:
Kalia V, Saggar K, Sandhu P, Ahluwalia A, Vibhuti. Carcinoid tumor of the Ileum. Indian J Radiol Imaging [serial online] 2006 [cited 2014 Oct 23];16:503-4. Available from: http://www.ijri.org/text.asp?2006/16/4/503/32256
Primary malignant tumors of the small intestine are uncommon and Gastrointestinal Carcinoid (Carcinoid Tumor) is the most common primary tumor of the small bowel accounting for more than 95% of all carcinoids. Clinically, most of these tumors are silent but they may present with pain, intestinal obstruction, weight loss, palpable abdominal mass, carcinoid syndrome or rarely by bowel perforation[1].

A 52 year old female presented with vomiting, constipation, loss of weight and appetite over the last six months. No history suggestive of carcinoid syndrome was present. No previous relevant medial history was present. Routine lab tests were normal. Physical examination revealed an ill-defined firm, non-tender mass in the right iliac fossa. Gastrointestinal Series showed moderately dilated bowel loops in the right lower quadrant with distortion and thickening of the mucosal folds and increase in the interbowel loop distance. There is kinking, rigidity, thickening and foreshortening of one of the ileal loop with buckling of its mesenteric border [Figure - 1]. Sonography revealed a hypoechoic mass in the right iliac fossa with thickening of the adjacent gut loops. Following this, a CT scan examination was obtained. It showed thickening of the small bowel loops apparently adherent to the focal soft tissue density mass in the mesentry which shows calcification. Radiating soft tissues strands are seen extending from the mass to the adjacent thickened bowel loops encasing the mesenteric vessels and producing typical "stellate pattern" [Figure - 2]. The patient underwent laprotomy with resection of the neoplasm in the midileum and end to end anastomosis Histopathological examination of the resected specimen showed it to be a carcinoid tumor of the small gut.


   Discussion Top


Gastrointestinal carcinoid is the most common primary tumor of the small bowel and mesentry. It accounts for more than 95% of all carcinoids and 1.5% of all gastrointestinal tumors. The tumor arises from the endochromaffin cells of Kulchitsky i.e. neural crest cells situated at the base of crypts of Lieberkuhn. Various sites of origin of this neoplasm are Appendix - 30-45%, Small bowel - 25-35% (duodenum 2%, jejunum 7%, ileum 91%, multiple sites 15-35%), Rectum 10-15%, Caecum - 5% and Stomach - 0.5%. Carcinoids can also rarely occur in pancreas, biliary tract, oesophagus and liver [1,2]. The sex prediction for the tumor is M:F : : 2:1. Most carcinoids occur in patients older than 50 years, however, appendiceal carcinoids occur in young patients in their second to fourth decade. Most patients are asymptomatic but symptoms can vary from pain, intestinal obstruction (19%), weight loss (16%), palpable mass (14%), intussusception, perforation or gastrointestinal hemorrhage (rare)[1],[3]. The primary tumor is small however it frequently evolves a fibrotic reaction with foreshortening and thickening of the mesentry[4]. The tumor also elaborate serotonin and other histamine like substances than can cause carcinoid syndrome which is characterized by abdominal cramps, diarrhoea, hepatomegaly, flushing etc.[1],[3]. Carcinoids also have the tendency to metastasize to lymph nodes, liver and rarely bone [3],[5].

The primary tumor is small, slow growing and is rarely demonstrated radiologically. The diagnosis is made when the tumor has spread through the bowel wall into the peritoneum and mesentry and is achieved by several complementary imaging techniques[6]. Plain abdominal skiagram is the initial screening modality and it may show curvilinear calcification. However, it is highly non-specific [1]. Barium studies (enteroclysis, follow through examination) may show intramural or intralumural filling defects in the distended ileum. There can also be narrowing of the lumen with stricture formation, thickening of the valvulae conniventes and increase in the interbowel loop distance due to wall thickening. The characteristic desmoplastic reaction can be seen as kinking, rigidity, matting, displacement, stretching, foreshortening and thickening of the bowel loops with buckling of their mesenteric border [2],[3],[5]. Kinking of the small bowel on barium studies is considered the hallmark of carcinoid [1]. Further, tumor spread to the root of mesentry may encase the vessels and produce infarction.

Sonography of the bowel can depict pseudokidney sign due to wall thickening, associated lymphadenopathy and liver metastasis. However, the findings are too nonspecific to offer a confident diagnosis [1]. Diagnosis can be made fairly confidently on computed tomography. CT is used for demonstrating the intestine, mesentry, lymph nodes and liver in a single examination and for staging the neoplasm as well as follow up after surgery or chemotherapy. It reveals a soft density mass with spiculated borders and radiating strands with or without calcification in 80% of cases [2]. The mass may be associated with thickened bowel loops apparently adherent to the mass and extending to the root of mesentry encasing the mesenteric vessels. Radiant soft tissue density strands can be seen due to tumor infiltration and fibrosis of the neurovascular bundles producing typical "stellate pattern". However, similar mesenteric masses can be seen in lymphoma, leukemia, metastatic carcinoma and sarcoma (especially ovary and leiomyosarcoma, retractile mesenteritis, kaposi's sarcoma and peritoneal mesothelioma. Bowel wall thickening associated with mesenteric changes can also be confused with crohn's disease. Other findings on CT can be lymphadenopathy (retroperitoneal, retrocrural) and metastasis commonly to the liver. Metastases are frequent from a midgut primary tumor and rare from appendiceal carcinoid. They also depend on size of the primary tumor: tumors smaller than one cm metastasize in two per cent of cases, tumor 1-2 cms metastasize in 50% and >2 cms are clinically silent but they may produce carcinoid syndrome. On CT, hepatic metastases are hypervascular enhancing lesions and may assume variety of patterns such as finely nodular, coarsely nodular, mixed single large mass and rarely, pseudocystic appearance probably due to ischemic necrosis [7]. Bone metastases are rare and most osseous metastases have been reported from carcinoid of the stomach and rectum. Mostly they are osteoblastic but rarely, lytic or mixed lesions occur [1]. Carcinoid tumors may also be associated with a high incidence of other primary malignant neoplasms e.g. in the colon, breast, pancreas, kidney, lip, vulva, cervix, larynx, rectosigmoid and histiocytic lymphoma [8].

Somatostatin receptor scintigraphy with Indium III Octreotide, Indium III pentetrotide and Iodine 123 meta iodobenzylguanidine is a sensitive and non-invasive technique for imaging carcinoid and its metastatic spread. FDG-PET is useful in poorly differentiated carcinoids and its main indication is in cases with negative scintigraphy. Recently, [Fluoro 18] -dopa PET has been used to image primary gastrointestinal carcinoid tumors and lymph node and organ metastases with promising results [1].

The primary objective of treatment in these patients is the control of symptoms resulting from carcinoid syndrome as the life span of the patients is long regardless of treatment. And when the disease becomes resistant to pharmacologic agonists, patients can usually benefit from hepatic artery embolization by polyvinlyalcohol, gelfoam or coils.

 
   References Top

1.Khan AN, Carcinoid. Gastrointestinal. emedicine, July, 2005.  Back to cited text no. 1    
2.Gould M, Johnson RJ. Computed Tomography of abdominal carcinoid tumor. British J. of Radiol. 1986; 59, 881-885.  Back to cited text no. 2    
3.Nolan DJ. The Small Intestine. In Grainger and Allison, eds. Diagnostic Radiology. 4th ed. London: Churchill Livingston, 2001: 1075-1099.  Back to cited text no. 3    
4.Siegel RS, Kuhns LR, Borlaza GS, McCormick, TL, Simmons JL. Computed tomography and Angiography in ileal carcinoid tumor and retractile mesenteritis. Radiology 1980; 134: 437-440.   Back to cited text no. 4    
5.Jeffree MA, Barter SJ, Hemingway AP, Nolan DJ. Primary carcinoid tumors of the ileum: the radiological appearances. Clin. Rad. 1984; 35: 451-455.  Back to cited text no. 5    
6.Piais D, Glazer HS, Levitt RG, Husband JE. Computed tomography of abdominal carcinoid tumors. Am. J. of Roentgenology, 1985; 143: 581-584.  Back to cited text no. 6    
7.Dent GA, Feldman JM. Pseudocystic liver metastases in patients with carcinoid tumors: Report of 3 cases. Am. J. Clin. Pathol. 1984; 275-279.  Back to cited text no. 7    
8.Barclay THC, Schapira DV. Malignant Tumors of the small intestine. Cancer 1983; 51: 878-887.  Back to cited text no. 8    

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V Kalia
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DOI: 10.4103/0971-3026.32256

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