|Year : 2022 | Volume
| Issue : 1 | Page : 41-44
Inflammatory myofibroblastic tumor presenting as malignant intestinal obstruction: A rare case report
Zeeshan Nahid, Sadaf Abbas, Asfa Shams, Kafil Akhtar
Department of Pathology, Faculty of Medicine, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
|Date of Submission||01-Mar-2022|
|Date of Decision||05-Apr-2022|
|Date of Acceptance||05-Apr-2022|
|Date of Web Publication||15-Jun-2022|
Dr. Kafil Akhtar
Department of Pathology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Inflammatory myofibroblastic tumor (IMFT) is an unusual solid tumor of mesenchymal origin seen in children and young adults. It occurs primarily in the lungs with equal gender predisposition. It has been proposed that the development of IMFT occurs after trauma, surgery, or infection with Epstein‒Barr virus and human herpesvirus, related with reactive cytokine production. Because of its rarity, it is important to distinguish IMFT from other colonic tumors. We report a rare case of IMFT in a 50-year-old man who presented with features of intestinal obstruction.
Keywords: Histopathology, immunohistochemistry, inflammatory, myofibroblastic, solid, tumor
|How to cite this article:|
Nahid Z, Abbas S, Shams A, Akhtar K. Inflammatory myofibroblastic tumor presenting as malignant intestinal obstruction: A rare case report. Ann Oncol Res Ther 2022;2:41-4
|How to cite this URL:|
Nahid Z, Abbas S, Shams A, Akhtar K. Inflammatory myofibroblastic tumor presenting as malignant intestinal obstruction: A rare case report. Ann Oncol Res Ther [serial online] 2022 [cited 2022 Jun 25];2:41-4. Available from: http://www.aort.com/text.asp?2022/2/1/41/347560
| Introduction|| |
Inflammatory myofibroblastic tumor (IMFT) is an unusual solid tumor of mesenchymal origin, commonly documented in children and young adults. According to the World Health Organization in 2020, IMFT comes under the intermediate or rarely metastasizing fibroblastic or myofibroblastic tumors. It occurs primarily in the lung, and IMFTs in many organs including stomach, small intestine, large intestine, liver, mediastinum, retroperitoneum, and bladder have also been documented.,
IMFT has been synonymously referred to as inflammatory pseudotumor, pseudosarcomatous myofibroblastic lesion, pseudosarcomatous fibromyxoid lesion, or plasma cell granuloma. The clinical features depend on the site affected by inflammatory myofibroblastic tumor. The presenting complaints of patients with IMFT intestine are similar to those having obstruction due to any other pathological conditions., We report an uncommon case of IMFT in a 50-year-old man who presented with intestinal obstruction.
| Case Report|| |
A 50-year-old man presented with inability to pass stool for 3 days. He also complained of abdominal pain, vomiting, and anal bleeding after defecation for 2 weeks. It was not associated with fever or weight loss. On presentation, he was hemodynamically stable and hydrated. Physical examination revealed distended abdomen, tenderness and vague palpable mass in the right iliac fossa, and localized guarding.
Abdominal ultrasonography revealed circumferential thickening of the cecal wall further extending into ascending colon and ileocecal junction, resulting in the dilatation of proximal bowel loops. Few enlarged and subcentrimetric hypoechoic mesenteric lymph nodes were seen in the right iliac fossa region showing a loss of fatty hilum. On X-ray abdomen in the erect position, multiple dilated bowel loops with air-fluid levels were seen.
Contrast-enhanced computed tomography showed a circumferential enhancing thickening of the cecal wall further extending into ascending colon and ileocecal junction, causing luminal narrowing leading to obstruction of the proximal bowel loops. Few enlarged and subcentrimetric hypodense mesenteric lymph nodes were seen in the right iliac fossa region. The patient was planned for exploration, and on laparotomy, a malignant appearing thickening of the cecal mucosa was noted for a length of 5.0 cm in length. The malignant appearing intestine with adequate margins was resected.
Gross examination showed a right hemicolectomy intestinal segment of 29 cm in length. A diffuse circumferential thickening of the intestine was observed measuring 5 cm × 4 cm × 3.5 cm at a distance of 9 cm from the proximal resection margin. A perforation was identified at the thickened serosal surface. Seven lymph nodes were received of sizes ranging from 1 to 2 cm.
Microscopic examination of thickened intestinal segment showed spindle cell proliferation with inflammatory cell infiltrate, comprising lymphocytes, plasma cells, and eosinophils. The spindled cells showed no atypia, with vesicular chromatin and prominent nucleoli, in a loose myxoid stroma [Figure 1] and [Figure 2]. On the basis of histomorphological features, the differential diagnosis was fibromatosis and IMFT. Further immunohistochemistry was applied, which showed cytoplasmic positivity of smooth muscle actin in the myofibroblastic spindle cells [Figure 3]. According to the French Federation of Cancer Centers Sarcoma Group, histological Grade 1 was given on the basis of tumor differentiation, mitosis, and necrosis. Resection margins were uninvolved. No mitosis, necrosis, or lymphovascular invasion was identified. All the lymph nodes received were uninvolved and showed reactive follicular hyperplasia. These findings were consistent with the diagnosis of IMFT. The postoperative condition was uneventful, and our case was discharged after a week. He has been free of complications after 2 months of follow-up.
|Figure 1: Microscopic examination of thickened intestinal segment showed spindle cell proliferation with inflammatory cell infiltrate, comprising lymphocytes, plasma cells, and eosinophils in a loosely arranged myxoid stroma. Hematoxylin and Eosin ×10|
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|Figure 2: The spindled cells showed no atypia, with vesicular chromatin and prominent nucleoli. Hematoxylin and Eosin ×40|
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|Figure 3: On immunohistochemistry, smooth muscle actin showed cytoplasmic positivity in the myofibroblastic spindle cells. IHC SMA ×10|
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| Discussion|| |
IMFT was first described in the lungs in 1937 and since then has been reported in the mesentery, stomach, small intestine, large intestine, liver, mediastinum, retroperitoneum, and bladder., Lung is the most common site of IMFTs and mesentery and omentum among the extrapulmonary sites, with 43.0% of the cases., Although it is common in children and young adults (mean age, approximately 10 years), recently, a broad age range has been documented. There is no difference in incidence between females and males., Some reports show a female predilection for IMFT.
IMFT has a very unclear etiology. It has been proposed that development of IMFT occurs after trauma, surgery or Epstein Barr virus and human herpes virus infection with reactive cytokine production., These lesions may possess chromosomal aberrations resulting in monoclonality which may frequently demonstrate locally aggressive behavior. It has also been observed that a chromosomal rearrangement involving 2p23, the site of the Anaplastic Lymphoma kinase (ALK) gene, is present in a subset of these tumors. Thus, findings have recently shown that chromosomal abnormalities which are present in IMFT may be suggestive of its clonal origin and not just only a reactive process. Hence, IMFT should be considered as a true neoplasm.
Similar to our case report, several IMFTs in the colon have been confirmed in other reports., Broad differential diagnosis of IMFT includes malignancy and submucosal tumor from endoscopic findings. Radiologically differential diagnosis of abdominal IMFT includes any chronic infective, inflammatory, and neoplastic processes such as solitary fibrous tumor, fibromatoses (desmoid tumor), inflammatory bowel disease, and adenocarcinoma intestine.
Histomorphologically, inflammatory fibroid polyp, fibromatoses (desmoid tumor), gastrointestinal stromal tumors (GIST), leiomyoma, leiomyosarcoma, and schwannoma have very similar pathological findings with IMFT., Microscopically, inflammatory fibroid polyp is submucosal and consists of small proliferating blood vessels, spindle cells, and inflammatory cells (particularly eosinophils). Fibromatoses are composed of parallel fascicles of bland-looking spindled or stellate cells in a collagenous stroma. Fibromatoses often have mitotic activity, but cytological atypia is absent. GIST stains for CD34 and CD117 (c-kit). Leiomyoma and leiomyosarcoma stain positively for desmin and actin and negatively for CD117 and CD34. Schwannoma stains strongly for S-100 (nuclear and cytoplasmic) and is CD117 negative., Almost all IMFTs show strong diffuse cytoplasmic positivity for vimentin. Reactivity for smooth muscle actin and muscle-specific actin varies from a focal to a diffuse pattern in the spindle cell cytoplasm, and desmin is identified in many cases. Approximately 50.0% of IMFTs show immunohistochemical cytoplasmic positivity for ALK, however, it is not specific for IMFT.
Because of IMFTs rarity, it is important to distinguish IMFT from other colonic tumors. IMFT cannot be diagnosed solely on preoperative biopsy. Surgical resection is the treatment of choice. Due to its intermediate/rarely metastasizing ability, we have to understand IMFT accurately, and the diagnosis should be carefully made so as to prevent overdiagnosis and unnecessary treatment.
The treatment modalities include chemotherapy, radiation treatment, nonsteroidal anti-inflammatory drug, steroid, cyclosporin-A, and ceritinib, but surgical resection is considered the treatment of choice., Most IMFTs behave in a benign manner after surgical resection. Rarely, it may recur or may metastasize or have malignant transformations. Thus, regular follow-up is necessary even though surgical resection is being done.,
| Conclusions|| |
Although IMFTs are common in children and young adults, it can present in older age in an unusual location of the intestine, with features of intestinal obstruction. IMFT should be kept in the differentials of spindle cell neoplasms and strictly followed up due to its intermediate/rarely metastasizing tendency.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]