|Year : 2014 | Volume
| Issue : 1 | Page : 56-58
Giant mesenteric inflammatory myofibroblastic tumor: A case report and review of the literature
Mohamed Salem Al-Saeed
Department of Surgery, College of Medicine and Medical Sciences, Taif University, Taif, Saudi Arabia
|Date of Web Publication||9-Apr-2014|
Mohamed Salem Al-Saeed
Department of Surgery, College of Medicine and Medical Sciences, Taif University, Taif 21947
Source of Support: None, Conflict of Interest: None
The report describes the case of a 38-year-old woman with a giant inflammatory myofibroblastic tumor of the mesentery. She presented with a painful epigastric mass. The patient noticed a gradual increase in the size of the mass and worsening pain accompanied by nausea and infrequent vomiting for two weeks prior to consultation. Physical examination revealed a large, firm, slightly tender mass occupying the epigastric region and left hypochondrium. A computed tomography scan showed a huge abdominal mass displacing the bowel loops and encasing the mesenteric vessels. The mass was completely resected along with the infiltrated parts of the small intestine. The final histopathological diagnosis was inflammatory myofibroblastic tumor (IMT) originating from the mesentery. Follow-up for two years revealed no recurrence. Once a diagnosis of IMT is confirmed, adequate surgical resection should be performed to avoid local recurrence.
Keywords: Inflammatory myofibroblastic tumor, mesenteric, Saudi female
|How to cite this article:|
Al-Saeed MS. Giant mesenteric inflammatory myofibroblastic tumor: A case report and review of the literature. Saudi J Health Sci 2014;3:56-8
| Introduction|| |
Inflammatory myofibroblastic tumors (IMTs) are rare neoplasms and they are benign in most of the cases. , They are commonly noted in the lung and upper respiratory tract, but an extra-pulmonary occurrence is very rare. ,, The tumor is formed of spindle cells in inflammatory stroma.  Most abdominal tumors are discovered incidentally during radiological investigations; however, some patients may present with pain, an abdominal mass, or obstructive manifestations.  Complete surgical resection is the primary treatment in such cases due to the risk of local recurrence. 
Herein, we present a patient with a giant mesenteric IMT who presented with a painful abdominal mass.
| Case Report|| |
A 38-year-old Saudi woman presented to our hospital in January 2012 with a painful upper abdominal mass that had been persisting for 3 months. The mass had a gradual onset with an epigastric origin. Within three months, its size increased and it extended to the left upper abdomen. Initially, the patient complained of a mild vague pain where that the intensity of the pain increased during two weeks prior to consultation. The pain became colicky and was associated with nausea and infrequent vomiting. There was no change in bowel habits, and no evidence of other gastrointestinal symptoms. A systemic examination did not yield any remarkable findings. The patient had undergone laparoscopic gastric banding seven years earlier, which was removed after a few weeks due to the development of complications. An open gastric bypass was performed during the same operative procedure. Examination indicated the presence of a huge abdominal mass occupying the epigastric region and left hypochondrium.
Tumor marker levels and laboratory findings were normal. Abdominal computed tomography (CT) scan [Figure 1] showed a huge abdominal soft tissue mass measuring 18 × 14 × 11.5 cm with hypodense foci of degeneration. In addition, the mass was displacing the bowel loops laterally and encasing the vessels. A CT guided Tru-cut needle biopsy specimen was obtained from the mass and histopathological examination indicated a spindle cell lesion. The initial pathological diagnosis was desmoid tumor. After counseling the patient and her relatives, a decision was made to resect the tumor.
|Figure 1: CT scan showing huge abdominal soft tissue mass with hypodense foci of degeneration and displacement of the bowel loops laterally with vascular encasement|
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During the operation, adhesiolysis was carefully performed to expose a 20 × 17 × 13 cm mass, [Figure 2]a and b, originating from the mesentery with peripheral infiltration of the nearby jejunal loops, including the Roux loop of the gastric bypass. The tumor was completely resected along with approximately six feet of the infiltrated jejunum, and the gastric bypass was reversed.
|Figure 2: (a) The tumor delivered from the abdominal incision (b) The tumor after resection, note the infi ltrated nearby jejunal loops|
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The final histopathological report showed that the tumor originated from the mesentery and composed of myofibroblastic spindle cells with an inflammatory infiltrate of plasma cells, lymphocytes, histiocytes, and neutrophils in a sclerotic stroma with foci of myxoid tissues [Figure 3]a. Immunohistochemical staining indicated positive results for anaplastic lymphoma kinase (ALK) [Figure 3]b and vimentin [Figure 3]c, and negative results for CD117. There were no tumor deposits in the mesenteric lymph nodes. A definitive diagnosis of mesenteric IMT was established.
|Figure 3: (a) Showing the spindle cell tumor in inflammatory stroma (H and E x 400) (b) Showing the spindle cells are positive for ALK (x 200) (c) Showing the spindle cells are positive for vimentin (x 200)|
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The postoperative course was uneventful, and the patient was discharged after ten days without any complications. She has not exhibited any evidence of recurrence after two years of follow-up.
| Discussion|| |
The nature of IMTs has been controversial with regard to whether it represents a tumor or inflammation, and whether it is benign or malignant in nature.  Various synonyms have been used for its description such as inflammatory pseudotumor or plasma cell granuloma. ,,,,,, Recently, cytogenetic studies have indicated that IMT is a true tumor, based on the abnormalities in the anaplastic lymphoma kinase gene on chromosome 2p23 with DNA aneuploidy. ,
The occurrence of IMT at extra-pulmonary sites has a higher incidence in adult females,  as in the present case. Moreover, 43% of extra-pulmonary IMTs commonly originate the mesentery of the distal ileum and transverse colon. ,,,, In this case, the tumor originated from the mesentery of the proximal jejunum, which is an extremely rare site. 
Extra-pulmonary IMTs are usually discovered incidentally; occasionally, they present as an abdominal mass with associated pressure symptoms, such as abdominal pain and vomiting. ,,, The current patient presented with a painful abdominal mass, which was tender, and associated with nausea and vomiting.
Some reports correlated the development of IMT with the oncogenic viruses and post-surgery process. ,, Leon et al.,  described the case of an IMT of the gastric remnant in a woman who had earlier undergone a gastrectomy. The present case had a history of gastric surgeries and the tumor was found in close proximity to the site of the gastric bypass, surrounded by marked adhesions and had infiltrated the Roux loop. Thus, prior gastric surgery could be considered as a possible risk factor for tumor development.
A definitive preoperative diagnosis is usually difficult as there are no specific radiological features of the tumor. , In the present case, the preoperative diagnosis based on the CT-guided Tru-cut needle biopsy findings was desmoid tumor, and the diagnosis of IMT was established only after operative resection. The tumor was primarily formed of spindled or epithelioid cells in sclerotic or/and myxoid stroma with round cell infiltrates, mainly consisting of plasma cells and lymphocytes, moreover, the tumor is usually positive for vimentin and ALK but, negative for CD117 which differentiate it from gastrointestinal stromal tumors. , The same findings were detected in the current case.
Surgery is the primary treatment for IMTs, as complete tumor removal minimizes the risk of local recurrence and the prognosis is usually good. , For irresectable tumors, chemotherapy and radiotherapy are usually beneficial and there are recent reports of promising results with anti-inflammatory agents and targeted therapy using anti-tumor necrosis factor-α binding antibodies. , The current patient was successfully treated with complete resection of the tumor along with the infiltrated jejunal loops, and no recurrence was noted after two years postoperatively.
Although the diagnosis of IMT may be challenging and confusing, once a diagnosis is established, adequate surgical resection should be performed to avoid local recurrence. The postsurgical prognosis is usually good in such cases.
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[Figure 1], [Figure 2], [Figure 3]