Jejunal and Multiple Mesenteric Calcifying Fibrous Pseudotumor Induced Jejunojejunal Intussusception

Abstract

We describe here a patient with an unusual presentation--mesenteric calcifying fibrous pseudotumor in association with jejunojejunal intussusception. This 25-year-old woman came to our emergency department with severe abdominal distension and intermittent epigastric cramping pain. She was found to have rebound tenderness and an ill-defined mass palpable in her lower abdomen. Abdominal computed tomography revealed an inhomogeneous soft tissue mass with target sign and intussusception of small intestine was suspected. Emergency laparotomy showed jejunojejunal intussusception involving ~30cm of jejunum with multiple tumors on the mesentery surface extending over the whole bowel mesenteric and peritoneal surfaces. One of the mesenteric tumors formed the leading point of the intussusception. Segmental resection of jejunum with primary anastomosis was done. Some larger tumors were resected but most of the other tumors were left in place because they were small and numerous and total resection was impossible. Pathology confirmed that the lesions were benign mesenteric calcifying fibrous pseudotumors. In such patients, surgery might be curative. If a large segment of the bowel is affected by calcifying fibrous pseudotumor, smaller pseudotumors might be left in place because these tend to be asymptomatic. But they might become the leading points of recurrent intussusception.

Multiple calcifying fibrous tumors in the small intestine and the mesentery

“…However, it may be difficult to clean up minor tumors of <2 mm in size at multiple sites and consequently these tumors will be left untreated, especially when the patient is without any discomfort. 4 Sleigh et al suggested using repeated CT to monitor the tumor in the pleural or the mediastinal location. 14 In our case, we used gastroscopy to monitor CFT in the GI tract, because endoscopy is more efficient and provides clearer demonstration than CT for this purpose.…”

Calcifying Fibrous Tumor of the Gastrointestinal Tract

“…The most commonly involved sites are the stomach [3][4][5][6][7][8][9][10][11][12][13][14][15][16] and small intestine. 3,4,6,14,[17][18][19][20][21][22][23][24][25] Three examples in the large intestine exist 14,17,26 and a single case in the esophagus. 27 Patient age ranges from 5 to 77 years, with a mean of 40.8 years; no sex predilection is evident.…”

“…Multiple lesions were documented in 11 of 43 patients, many of which had concurrent extra-GI involvement, with the most common site being the mesentery. 14,17,19,20,[22][23][24][25] One author reported GI CFTs occurring in siblings. 19 Although most are discovered incidentally, CFTs in the GI tract can present in unique ways.…”

“…17,19,24 Two other small intestinal cases presented with intussusception. 20,21 Some simply manifest as abdominal or epigastric pain.…”

“…6 While multifocal CFTs have been described, there have been no documented cases of metastatic disease. 14,17,19,20,[22][23][24][25] CONCLUSION Calcifying fibrous tumor has been predominantly thought of as a tumor of the deep soft tissues. However, a thorough review of the literature reveals 43 cases of this tumor involving the GI tract, indicating that it may not be as rare in this location as previously thought.…”