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CASE REPORT
Year : 2022  |  Volume : 18  |  Issue : 7  |  Page : 2066-2069

Inflammatory myofibroblastic tumor from the greater omentum in children: A rare case report


1 Department of Pediatric Surgery, Shandong Provincial Hospital, Shandong University, Jinan, People's Republic of China
2 Department of Urology Surgery, Shandong Provincial Hospital, Shandong University, Jinan, People's Republic of China
3 Department of Dermatology, The First Affiliated Hospital of Shandong First Medical University, Jinan, People's Republic of China
4 Department of Pediatric Surgery, Shandong Provincial Hospital, Shandong University, Jinan, People's Republic of China; Post-Doctoral Research Station of Clinical Medicine, Liaocheng People's Hospital, Liaocheng, People's Republic of China

Date of Submission24-May-2022
Date of Decision14-Jul-2022
Date of Acceptance21-Jul-2022
Date of Web Publication11-Jan-2023

Correspondence Address:
Wei Liu
Department of Pediatric Surgery, Shandong Provincial Hospital, Shandong University, 324 Jingwu Street, Jinan 250021, Shandong
People's Republic of China
Xiaoqing Wang
Department of Pediatric Surgery, Shandong Provincial Hospital, Shandong University, 324 Jingwu Street, Jinan 250021, Shandong
People's Republic of China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcrt.jcrt_1089_22

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 > Abstract 


Inflammatory myofibroblastic tumor (IMT) prevalence is less than one in a million. Anaplastic lymphoma kinase (ALK)-positive IMT in the greater omentum and with a diameter greater than 8 cm is extremely rare. Here, we present a case and provide a brief literature review. A 4-year-old female was referred to our hospital with a 1-month history of intermittent fever. Computed tomography revealed a 6.4 × 5.5 × 6.5-cm lesion between the spleen and stomach. During the operation, we confirmed that the mass originated in the greater omentum and completely resected the mass, achieving a negative margin. The maximum cross-sectional area of the tumor after surgery was 8.3 × 7.5 cm. The immunohistochemistry result of this IMT was ALK (+), S100 (−), Ki-67+ (20%), Desmin (+), CD21 (−), CD35 (+), Vim (+), and SMA (+). The final pathology was IMT. No local recurrence or metastasis has been observed in the 8 months of follow-up.

Keywords: Children, greater omentum, IMT, Inflammatory myofibroblastic tumor


How to cite this article:
Song H, Zhang H, Zhang Y, Wang X, Liu W. Inflammatory myofibroblastic tumor from the greater omentum in children: A rare case report. J Can Res Ther 2022;18:2066-9

How to cite this URL:
Song H, Zhang H, Zhang Y, Wang X, Liu W. Inflammatory myofibroblastic tumor from the greater omentum in children: A rare case report. J Can Res Ther [serial online] 2022 [cited 2023 Jan 27];18:2066-9. Available from: https://www.cancerjournal.net/text.asp?2022/18/7/2066/367452

Authors Honghao Song and Huiyu Zhang contributed equally




Inflammatory myofibroblastic tumor (IMT) is a rare tumor;[1] its clinical presentation and auxiliary examinations lack clinical specificity, often leading to misdiagnosis. IMT originates in many parts of the body, but occurrence in the greater omentum is uncommon. Anaplastic lymphoma kinase (ALK)-positive IMT with a diameter greater than 8 cm is extremely rare. Here, we present a case with a literature review to improve the IMT diagnostic rate.


 > Case Report Top


Medical history

A 4-year-old female was referred to our hospital with a 1-month history of intermittent fever. She had a recurrent fever without inducement three to four times a day; temperatures ranged from 36.2°C to 39.2°C. No improvement was observed after 10 days of anti-inflammatory treatment. Physical examination revealed a left upper quadrant abdominal mass with unclear and tough margins.

Laboratory examination

Pre-operation: RBC 2.90 × 1012/L, HGB 67 g/L, PLT 738 × 109/L, CRP 137.90 mg/L, PCT 0.28 ng/mL, IL-6 256.20 pg/mL. No abnormalities were detected in human chorionic gonadotropin, alpha-fetoprotein, neuron-specific enolase, lactate dehydrogenase, 24-h urine catecholamine test, and blood aerobic culture. Sternal bone marrow puncture suggested proliferative anemia.

Image examination

Ultrasonography revealed a unilateral 6.2 × 4.9 × 6.2-cm lump in the left adrenal gland; the contralateral gland was normal. Computed tomography (CT) revealed a 6.4 × 5.5 × 6.5-cm lesion between the spleen and stomach. Enhanced CT detected an unclear boundary between the spleen and pancreatic tail, more closely related to the spleen [Figure 1]. Additional movie files 1 and 2 show this in more detail. Positron Emission tomography-CT revealed no systemic bone metastases.
Figure 1:CT images before the operation. (a) plain CT scan; (b-d) contrast-enhancement CT scan (a and b) axial plane; (c) coronal plane; (d) sagittal plane. Axial, coronal, and sagittal computed tomography (CT) scans with contrast show a large mass between the stomach and spleen

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Therapy and pathology

Concentrated red blood cells and plasma were given to correct anemia, and meropenem was administered as an anti-inflammatory treatment, but no obvious effects were observed. The mass was diagnosed as IMT by pathological tissue collected via ultrasound-guided puncture.

We considered that the fever was caused by IMT, excluding the factor of infection. With the presumptive diagnosis of IMT arising from the abdomen, we confirmed that the mass originated in the greater omentum during the operation. The mass was completely resected, and a negative margin was achieved. The maximum cross-sectional area of the tumor after surgery was 8.3 × 7.5 cm [Figure 2]. The largest cross-sectional area in the postoperative pathology report was 8 × 7 cm. The final pathology was consistent with the puncturing pathology. During the 8 months of follow-up, no local recurrence or metastasis has been observed [Figure 3]. Additional movie files 3 and 4 show this in more detail.
Figure 2: Images during the operation and images of the tumor. (a) This image shows that the tumor is attached to the spleen, but not from the spleen (b-d) The maximum cross-sectional area of the tumor is 8.3 × 7.5 cm; the tumor is irregular in shape and tough in texture

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Figure 3: CT images after the operation. (a and b) axial plane (a) plain CT scan; (b) contrast-enhancement CT scan. Axial computed tomography (CT) scan with contrast 5 months after the operation shows no recurrence of the tumor at the original site

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 > Discussion Top


IMT is a rare tumor[1] first found in the lung[2] and has since been observed in the mesentery, omentum, gastrointestinal tract, urogenital tract, upper respiratory tract, and soft tissue.[3] Among these tumor sites, IMT in the greater omentum is extremely rare. IMT was defined by the World Health Organization (WHO) in 2002 and classified as an intermediate tumor in 2006.[4] In 2013, the WHO defined IMT as “consisting of differentiated myofibroblast and fibroblast, accompanied by plasma cells, lymphocytes, eosinophils, and other inflammatory cells,” and a few parts of IMT were classified as transferable tumors.[5]

IMT of abdominal parenchymal or empty viscera are typically smaller (diameter <5 cm), whereas retroperitoneal lesions are larger (diameter >5 cm). However, in this case, an IMT originating in the greater omentum was greater than 8 cm in diameter, suggesting that IMT is clinically variable and has no specific manifestations. Therefore, we reported this extremely rare clinical case.

IMT most commonly occurs in children,[1] and the clinical presentation depends on the tumor site.[6] Abdomen-originating IMT is characterized by abdominal mass with fever, growth retardation, loss of weight, anemia, thrombocythemia, erythrocyte sedimentation rate (ESR), and immunoglobulin elevation.[7] Some metrics (fever, growth, weight, anemia, and thrombocythemia) are consistent with our report.

No specific laboratory test results confirm the disease,[1] but interleukin-6 showed the greatest pre- to postoperative change (RBC 4.37 × 1012/L, HGB 119 g/L, PLT 512 × 109/L, CRP 38.20 mg/L, PCT 0.62 ng/mL, IL-6 21.48 pg/mL), suggesting that fever was related to interleukin-6.

Pathology is the gold standard for diagnosing IMT[8] and is mainly based on the following indicators: ALK (+), Vim (+), SMA (+), S100 (−), and spindle cells. ALK is related to IMT, and the sensitivity of ALK in children is higher than that in adults, which can be used as a diagnostic auxiliary indicator.[9] Thus, multidisciplinary investigation before surgery is necessary.[10]

Complete surgical resection with a negative margin is an independent risk factor affecting prognosis.[11] Cases with negative surgical margins had an 87% reduction in death as compared to those with macroscopic positive surgical margins.[12] Preoperative pathological diagnosis can prevent the expansion of surgical scope and excessive treatment. Small hidden lesions should be explored and resected during surgery to reduce the risk of recurrence. When the tumor closely adheres to the surrounding tissues and organs, and the boundary is not obvious, comprehensive treatment, including chemotherapy, anti-inflammatory, and ALK inhibitors (crizotinib[13]) should be combined. IMT treatment with ALK inhibitors is a recent research direction. Cyclooxygenase-2 inhibitors, which act via the down-regulation of vascular endothelial growth factor expression, direct inhibition of endothelial cell proliferation and activation, and anti-cytokine,[14],[15] are used to reduce the size of larger IMTs, enabling surgical resection. If both ALK and Ki-67 are positive, postoperative treatment should include radiotherapy and chemotherapy.[12]

IMT prognosis is dictated by the tumor size and location, complete resection, and adjuvant therapy. The recurrence rate of IMT ranges from 18% to 40%. The recurrence rate is 35% with multiple-organ involvement and 6% with single-organ involvement.[16] The relationship between Ki-67, pathological type, ALK, and prognosis remains controversial, and larger samples are needed to analyze the correlation between these factors and prognosis. With every 1 cm increase in diameter, the risk of IMT relapse increases by 60%.[12] Diameters greater than 8 cm are significantly associated with aggressive behavior.[17] So, for children with ALK- and Ki-67-positive IMT of the greater omentum with a diameter greater than 8 cm, close attention should be paid to follow-up.

We report a case in which IMT occurred in the greater omentum with ALK-positive and a diameter > 8 cm. However, because of its clinical manifestations, laboratory tests and imaging have no obvious specificity, and the diagnosis of IMT mainly depends on pathology. Therefore, an accurate pathological diagnosis has to be obtained before surgery, and a negative margin has to be ensured during surgery. Close follow-up and collection of relevant cases should be conducted to explore prognostic factors.

Ethics approval and consent to participate

The present study was approved by the Medical Ethics Committee of Shandong Provincial Hospital.

Financial support and sponsorship

This work was supported by the grants from the Science and Technology Development Plan Project of Shandong Province, China (2014GSF118144, 2018GSF118209, 2019GSF108061), the Jinan Science and Technology Bureau (202019134), and the Shandong Provincial Natural Science Foundation (ZR2017MH091, ZR2020QH263).

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

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Meis JM, Enzinger FM. Inflammatory fibrosarcoma of the mesentery and retroperitoneum. A tumor closely simulating inflammatory pseudotumor. Am J Surg Pathol 1991;15:1146–56.  Back to cited text no. 6
    
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Coffin CM, Watterson J, Priest JR, Dehner LP. Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases. Am J Surg Pathol 1995;19:859–72.  Back to cited text no. 7
    
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Dua SG, Purandare N, Pramesh CS, Karimundackal G, Menon S, Shah S, et al. Fluoro-deoxy glucose-avid endobronchial inflammatory myofibroblastic tumor mimicking bronchial malignancy: Report of a case. J Cancer Res Ther 2011;7:340–3.  Back to cited text no. 8
    
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Coffin CM, Hornick JL, Fletcher CD. Inflammatory myofibroblastic tumor: Comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. Am J Surg Pathol 2007;31:509–20.  Back to cited text no. 9
    
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Yagci MA, Sezer A, Yeldan E, Coskun I, Usta U, Cicin I, et al. Inflammatory myofibroblastic tumor presenting as an abdominal wall mass in an adult patient. J Cancer Res Ther 2010;6:224–6.  Back to cited text no. 10
    
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Casanova M, Brennan B, Alaggio R, Kelsey A, Orbach D, van Noesel MM, et al. Inflammatory myofibroblastic tumor: The experience of the European pediatric Soft Tissue Sarcoma Study Group (EpSSG). Eur J Cancer 2020;127:123–9.  Back to cited text no. 11
    
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Ong HS, Ji T, Zhang CP, Li J, Wang LZ, Li RR, et al. Head and neck inflammatory myofibroblastic tumor (IMT): Evaluation of clinicopathologic and prognostic features. Oral Oncol 2012;48:141–8.  Back to cited text no. 12
    
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Haimes JD, Stewart CJR, Kudlow BA, Culver BP, Meng B, Koay E, et al. Uterine inflammatory myofibroblastic tumors frequently harbor ALK fusions with IGFBP5 and THBS1. Am J Surg Pathol 2017;41:773–80.  Back to cited text no. 13
    
14.
Applebaum H, Kieran MW, Cripe TP, Coffin CM, Collins MH, Kaipainen A, et al. The rationale for nonsteroidal anti-inflammatory drug therapy for inflammatory myofibroblastic tumors: A Children's Oncology Group study. J Pediatr Surg 2005;40:999–1003; discussion 1003.  Back to cited text no. 14
    
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Sun L, Tu L, Wang X, Zhu H, Mao J, Zhuo H, et al. Management of rectal inflammatory myofibroblastic tumor recurrence. J Cancer Res Ther 2014;10:425–7.  Back to cited text no. 15
    
16.
Bertocchini A, Lo Zupone C, Callea F, Gennari F, Serra A, Monti L, et al. Unresectable multifocal omental and peritoneal inflammatory myofibroblastic tumor in a child: Revisiting the role of adjuvant therapy. J Pediatr Surg 2011;46:e17–21.  Back to cited text no. 16
    
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Bennett JA, Nardi V, Rouzbahman M, Morales-Oyarvide V, Nielsen GP, Oliva E. Inflammatory myofibroblastic tumor of the uterus: A clinicopathological, immunohistochemical, and molecular analysis of 13 cases highlighting their broad morphologic spectrum. Mod Pathol 2017;30:1489–503.  Back to cited text no. 17
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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