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Correlapaedia - a Correlative Encyclopedia of Pediatric Imaging, Surgery, and Pathology
The patient was an 8 month old male who 6 weeks ago developed fevers and was noted to be anemic with a hemoglobin of 3. He was treated with antibiotics and supplemental iron. Two weeks ago he was noted to have developed an abdominal mass.
Clinical Physical Exam:
Large, firm, non tender abdominal mass was palpated which was causing respiratory compromise.
White blood cell count of 26 with a differential of 30% polys and no bands, and a hematocrit of 17.8.
Clinical Differential Diagnosis:
An abdominal film revealed a mass in the mid abdomen. This was better defined by CT and MRI, which demonstrated the mass to be homogeneous without an obvious organ of origin.
Images 3 and 4
Images 6 and 7
Imaging Differential Diagnosis:
Inflammatory myofibroblastic tumor. Less likely is neuroblastoma, rhabdomyosarcoma and teratoma which would be more heterogenous when this size.
The child was taken to the operating room for a Tru-Cut needle biopsy. Three days later the child was taken back to the operating room for definitive excision of the mass. A long transverse incision was made. The mass was intimately associated to the terminal ileum and cecum and there were a large number of collateral blood vessels. The tumor was resected en bloc with the terminal ileum and cecum. The ileum and proximal colon were primarily anastomosed.
Examination of the surgical specimen revealed a tan to pink, firm, irregular mass measuring 14 x 13.5 x 8 cm. The mass was well encapsulated. No hemorrhagic necrosis or cystic degeneration was seen. The mass was felt to be a cellular, low-grade fibrous lesion, with inflammatory myofibroblastic tumor being the favored diagnosis.
Inflammatory Myofibroblastic Tumor
Follow-up and Prognosis:
The patient had an uncomplicated post-operative course. At his one year follow-up exam, he was noted to have recurrent tumor on US and CT examinations of the abdomen (not provided). An exploratory laparotomy performed one year after the first surgery showed multiple sites of recurrent tumor, particularly in the region of the ileocolic anastomosis, where the tumor had been extremely infiltrating in nature as noted during the prior surgery. Multiple mesenteric nodes, some of which were hard and some of which were soft, were noted. A decision was made not to resect the extensively affected area of the small bowel, since it would leave the patient with a short gut.
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