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Mesenteric inflammatory myofibroblastic tumor as a rare cause of small intestinal intussusception

Mesenteric inflammatory myofibroblastic tumor as a rare cause of small intestinal intussusception Inflammatory myofibroblastic tumor (IMT) is an uncommon, usually benign, mesenchymal tumor. IMT affects people of all ages, but it more commonly occurs in children and adolescents. Also, it has the potential to arise in any part of the body, though, it frequently develops in the lungs and mesentery. In this report, we discuss a rare clinical manifestation of mesenteric IMT presented as intussusception of the small intestine in a 7-year-old child. INTRODUCTION the definitive diagnosis of IMT is established on pathological examination. Inflammatory myofibroblastic tumor (IMT) is a rare borderline mesenchymal tumor, histologically made up of proliferated CASE REPORT fibroblasts and myofibroblasts admixed with various types of A 7-year-old child was hospitalized due to an inability to pass inflammatory cells. IMT was previously known as inflammatory feces and gas. The patient’s mother mentioned that he has pseudotumor, plasma cell granuloma, omental mesenteric suffered from diffuse abdominal pain, fever (unmeasured) and myxoid hamartoma and inflammatory fibrosarcoma, which vomiting for 3 days. reflects a heterogeneity in the clinicopathological findings of On physical examination, the patient’s general condition was these tumors [1]. In the last three decades, a subset of these stable; he was pale and was suffering from abdominal pain, lesions was proved to be neoplastic, and a clonal chromosomal yet he had no fever or chills. Abdominal examination revealed abnormality, especially of the anaplastic lymphoma kinase localized tenderness in the right iliac fossa. He had no other (ALK) gene on chromosome 2p23, was documented. ALK gene complaints, and his medical record was clear except for an rearrangement is more common in children and young adults orchiopexy 2 years ago. than in old patients and ranges from 33 to 67% [2–4]. IMTs have Plain abdominal radiography revealed signs that suggested a local recurrence rate of ∼10–25% and a low risk of metastasis, a small intestinal intussusception, and the ultrasound exami- ∼5% [2, 5]. People of any age can be affected by IMT, but it tends nation revealed further signs of intussusception (target sign), as to occur in children and young adults [2]. Its etiology remains well as signs of appendicitis. Laboratory studies showed elevated unknown, though it is indicated to occur secondary to viral white blood cells (WBC) count and c- reactive protein (CRP) level infection, trauma or surgery [6]. The tumor was first reported in (WBC = 15.7 × 109/L, Neutrophils = 84.9%, CRP = 16 mg/L). the lungs and was later described in other organs [1, 2]. Clinical Laparotomy was performed, and the patient’s abdomen was manifestations of IMTs vary depending on the size and location explored, revealing an intussusception ∼70 cm from the ileocecal of the tumor, but generally, there are no specific symptoms and Received: July 2, 2020. Revised: July 18, 2020. Accepted: July 24, 2020 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2020. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com 1 Downloaded from https://academic.oup.com/jscr/article/2020/9/rjaa322/5910255 by DeepDyve user on 29 September 2020 2 Z. Ballast et al. Figure 1: Exploration of the ileum revealed a focally depressed region where a small intraluminal mass was palpated. valve. During the correction of the intussusception, the surgeon noticed a segmental enlargement of the ileum with the mesen- tery being focally depressed and palpated there an intraluminal mass (Fig. 1). The enlarged intestinal segment and the appendix were resected. The resected segment measuring 3 × 1.5 × 2cmwas opened, revealing a firm, sessile polypoid mass measuring 2.5 × 2 × 1.5 cm. The cut-section of the tumor was whitish-grey with a whorled appearance (Fig. 2). Microscopic examination of the H&E-stained sections revealed the proliferation of interlacing bundles of spindle cells containing large elongated and vacuolated nuclei with prominent nucleoli. Sparse mitotic figures were observed. The vascularized stroma contained a mix of inflammatory cells (lymphocytes, plasmocytes, neutrophils Figure 2: Gross section of the tumor reveals whitish-grey surface with whorled and eosinophils) (Fig. 3). appearance. The spindle cell proliferation extended through all layers of the intestinal wall and projected as a polypoid mass into the lumen of the ileum and was covered by an ulcerated mucosa. Immunohistochemically, the spindle cells were positive for ALK1 and vimentin, with weak positivity for smooth muscle actin (SMA). C-kit, CD34, Desmin, epithelial membrane antigen (EMA) and Cytokeratin (CK) were all negative in the tumor cells (Fig. 4). Finally, IMT was diagnosed based on the immunohisto- chemical results and routine microscopic findings. The stained section of the appendix vermiformis showed mild acute inflam- mation with intraluminal Enterobius vermicularis. DISCUSSION IMT is a rare mesenchymal tumor that usually presents as firm, well-circumscribed and multi-nodular masses ranging in size from 0.4 to 36 cm. The tumor could be polypoid, sessile or pedunculated, and is covered with either eroded or ulcerated mucosa [2]. The tumor in our 7-year-old patient had presented Figure 3: (A) Microscopic findings of IMT. (B, C and D) Fibroblasts and myofibrob- lasts intermixed with different types of inflammatory cells on a background of as an intraluminal polypoid mass. abundant blood vessels. The tumor cells containing large elongated and vacuo- Intestinal IMTs lack specific clinical manifestations, and they lated nuclei with prominent nucleoli. (Hematoxylineosin, original magnification are usually accompanied by weight loss, malaise and a variety ×40 [A] ×100 [B, C] ×200 [D]). of laboratory abnormalities [7]. Several other atypical presenta- tions have been reported in the literature such as portal venous thrombosis, intussusceptions and anemia [2]. The intussuscep- tumors and differentiating them from other tumorous or tumor- tion of the small intestine was the primary presentation in like lesions [2, 5]. In our case, the tumor cells were positive for our patient. Microscopically, the main proliferating cells in IMTs ALK and vimentin with weak positivity for SMA. CD117, CD34, are myofibroblasts and fibroblasts, and immunohistochemically, Desmin, EMA and CK were negative in the tumor cells. they show variable positivity for SMA, and vimentin with nega- In addition to other soft tissue tumors that can arise in tivity for CD34, Desmin, S100 and CD117 [8]. The abnormalities the mesentery and intestinal wall, the differential diagnoses of the ALK gene lead to the expression of ALK1 and p80 in include other lesions that present as intestinal polyps. All epithe- the spindle cell components, which can aid in diagnosing these lial tumors and hamartomatous lesions are easily excluded as Downloaded from https://academic.oup.com/jscr/article/2020/9/rjaa322/5910255 by DeepDyve user on 29 September 2020 Mesenteric inflammatory myofibroblastic tumor 3 CONCLUSION Diagnosing mesenteric IMT as a cause of intestinal intussus- ceptions can be very challenging due to the rarity of the tumor and the unusual presentation. The pathological examination remains the essential tool for accurate diagnosis of IMT in all cases. REFERENCES 1. Yi E, Aubry M-C. Pulmonary pseudoneoplasms. Arch Pathol Lab Med 2010;134:417–26. 2. Chaudhary P. Mesenteric inflammatory myofibroblastic tumors. Ann Gastroenterol 2015;28:49–54. 3. Cessna MH, Zhou H, Sanger WG, Perkins SL, Tripp S, Pickering D, et al. Expression of ALK1 and p80 in inflammatory myofi- broblastic tumor and its mesenchymal mimics: a study of 135 Figure 4: (A) Positivity of ALK1 in the tumor cells. (B) Vimentin is diffusely positive cases. Mod Pathol 2002;15:931–8. in the tumor cells. (C) Negativity of CD34 in the tumor cells. (D) Tumor cells are 4. Snyder CS, Dell ’Aquila M, Haghighi P, Baergen RN, Suh YK, Yi negative for CD117 (Immunohistochemistry, original magnification ×100 [A, C, D] ES. Clonal changes in inflammatory pseudotumor of the lung: ×200 [B]). a case report. Cancer 1995;76:1545–9. 5. Karnak I, Senocak ME, Ciftci AO, Caglar ˘ M, Bingöl-Kologlu ˘ M, Tanyel FC, et al. Inflammatory myofibroblastic tumor in chil- they arise from mucosal membranes, unlike IMTs. A constel- dren: diagnosis and treatment. J Pediatr Surg 2001;36:908–12. lation of clinical and microscopic findings in addition to the 6. Groenveld RL, Raber MH, Oosterhof-Berktas R, Eijken E, Klaase results of immunohistochemical stains aid in ruling out the JM. Abdominal inflammatory myofibroblastic tumor. Case Rep other mesenchymal tumors and tumor-like lesions, such as Gastroenterol 2014;8:67–71. gastrointestinal stromal tumors, mesenteric fibromatosis and 7. Appak YÇ, Sahin GE, Ayhan S, Taneli C, Kasırga E. Inflam- sclerosing mesenteritis [2]. matory myofibroblastic tumor of the colon with an unusual In most cases, complete surgical resection is adequate; presentation of intestinal intussusception. European J Pediatr however, in cases where the tumor has locally recurred or Surg Rep 2014;2:54–7. metastasized, other treatment modalities might be used such 8. Miettinen M, Sobin LH, Sarlomo-Rikala M. Immunohisto- as chemotherapy, radiation treatment, nonsteroidal anti- chemical Spectrum of GISTs at different sites and their dif- inflammatory drugs, steroid and cyclosporin-A [2, 5]. After ferential diagnosis with a reference to CD117 (KIT). Mod Pathol 2 years of the surgery, our patient is in well condition without 2000;13:1134–42. any complaints. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Surgical Case Reports Oxford University Press

Mesenteric inflammatory myofibroblastic tumor as a rare cause of small intestinal intussusception

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Oxford University Press
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Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2020.
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2042-8812
DOI
10.1093/jscr/rjaa322
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Abstract

Inflammatory myofibroblastic tumor (IMT) is an uncommon, usually benign, mesenchymal tumor. IMT affects people of all ages, but it more commonly occurs in children and adolescents. Also, it has the potential to arise in any part of the body, though, it frequently develops in the lungs and mesentery. In this report, we discuss a rare clinical manifestation of mesenteric IMT presented as intussusception of the small intestine in a 7-year-old child. INTRODUCTION the definitive diagnosis of IMT is established on pathological examination. Inflammatory myofibroblastic tumor (IMT) is a rare borderline mesenchymal tumor, histologically made up of proliferated CASE REPORT fibroblasts and myofibroblasts admixed with various types of A 7-year-old child was hospitalized due to an inability to pass inflammatory cells. IMT was previously known as inflammatory feces and gas. The patient’s mother mentioned that he has pseudotumor, plasma cell granuloma, omental mesenteric suffered from diffuse abdominal pain, fever (unmeasured) and myxoid hamartoma and inflammatory fibrosarcoma, which vomiting for 3 days. reflects a heterogeneity in the clinicopathological findings of On physical examination, the patient’s general condition was these tumors [1]. In the last three decades, a subset of these stable; he was pale and was suffering from abdominal pain, lesions was proved to be neoplastic, and a clonal chromosomal yet he had no fever or chills. Abdominal examination revealed abnormality, especially of the anaplastic lymphoma kinase localized tenderness in the right iliac fossa. He had no other (ALK) gene on chromosome 2p23, was documented. ALK gene complaints, and his medical record was clear except for an rearrangement is more common in children and young adults orchiopexy 2 years ago. than in old patients and ranges from 33 to 67% [2–4]. IMTs have Plain abdominal radiography revealed signs that suggested a local recurrence rate of ∼10–25% and a low risk of metastasis, a small intestinal intussusception, and the ultrasound exami- ∼5% [2, 5]. People of any age can be affected by IMT, but it tends nation revealed further signs of intussusception (target sign), as to occur in children and young adults [2]. Its etiology remains well as signs of appendicitis. Laboratory studies showed elevated unknown, though it is indicated to occur secondary to viral white blood cells (WBC) count and c- reactive protein (CRP) level infection, trauma or surgery [6]. The tumor was first reported in (WBC = 15.7 × 109/L, Neutrophils = 84.9%, CRP = 16 mg/L). the lungs and was later described in other organs [1, 2]. Clinical Laparotomy was performed, and the patient’s abdomen was manifestations of IMTs vary depending on the size and location explored, revealing an intussusception ∼70 cm from the ileocecal of the tumor, but generally, there are no specific symptoms and Received: July 2, 2020. Revised: July 18, 2020. Accepted: July 24, 2020 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2020. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com 1 Downloaded from https://academic.oup.com/jscr/article/2020/9/rjaa322/5910255 by DeepDyve user on 29 September 2020 2 Z. Ballast et al. Figure 1: Exploration of the ileum revealed a focally depressed region where a small intraluminal mass was palpated. valve. During the correction of the intussusception, the surgeon noticed a segmental enlargement of the ileum with the mesen- tery being focally depressed and palpated there an intraluminal mass (Fig. 1). The enlarged intestinal segment and the appendix were resected. The resected segment measuring 3 × 1.5 × 2cmwas opened, revealing a firm, sessile polypoid mass measuring 2.5 × 2 × 1.5 cm. The cut-section of the tumor was whitish-grey with a whorled appearance (Fig. 2). Microscopic examination of the H&E-stained sections revealed the proliferation of interlacing bundles of spindle cells containing large elongated and vacuolated nuclei with prominent nucleoli. Sparse mitotic figures were observed. The vascularized stroma contained a mix of inflammatory cells (lymphocytes, plasmocytes, neutrophils Figure 2: Gross section of the tumor reveals whitish-grey surface with whorled and eosinophils) (Fig. 3). appearance. The spindle cell proliferation extended through all layers of the intestinal wall and projected as a polypoid mass into the lumen of the ileum and was covered by an ulcerated mucosa. Immunohistochemically, the spindle cells were positive for ALK1 and vimentin, with weak positivity for smooth muscle actin (SMA). C-kit, CD34, Desmin, epithelial membrane antigen (EMA) and Cytokeratin (CK) were all negative in the tumor cells (Fig. 4). Finally, IMT was diagnosed based on the immunohisto- chemical results and routine microscopic findings. The stained section of the appendix vermiformis showed mild acute inflam- mation with intraluminal Enterobius vermicularis. DISCUSSION IMT is a rare mesenchymal tumor that usually presents as firm, well-circumscribed and multi-nodular masses ranging in size from 0.4 to 36 cm. The tumor could be polypoid, sessile or pedunculated, and is covered with either eroded or ulcerated mucosa [2]. The tumor in our 7-year-old patient had presented Figure 3: (A) Microscopic findings of IMT. (B, C and D) Fibroblasts and myofibrob- lasts intermixed with different types of inflammatory cells on a background of as an intraluminal polypoid mass. abundant blood vessels. The tumor cells containing large elongated and vacuo- Intestinal IMTs lack specific clinical manifestations, and they lated nuclei with prominent nucleoli. (Hematoxylineosin, original magnification are usually accompanied by weight loss, malaise and a variety ×40 [A] ×100 [B, C] ×200 [D]). of laboratory abnormalities [7]. Several other atypical presenta- tions have been reported in the literature such as portal venous thrombosis, intussusceptions and anemia [2]. The intussuscep- tumors and differentiating them from other tumorous or tumor- tion of the small intestine was the primary presentation in like lesions [2, 5]. In our case, the tumor cells were positive for our patient. Microscopically, the main proliferating cells in IMTs ALK and vimentin with weak positivity for SMA. CD117, CD34, are myofibroblasts and fibroblasts, and immunohistochemically, Desmin, EMA and CK were negative in the tumor cells. they show variable positivity for SMA, and vimentin with nega- In addition to other soft tissue tumors that can arise in tivity for CD34, Desmin, S100 and CD117 [8]. The abnormalities the mesentery and intestinal wall, the differential diagnoses of the ALK gene lead to the expression of ALK1 and p80 in include other lesions that present as intestinal polyps. All epithe- the spindle cell components, which can aid in diagnosing these lial tumors and hamartomatous lesions are easily excluded as Downloaded from https://academic.oup.com/jscr/article/2020/9/rjaa322/5910255 by DeepDyve user on 29 September 2020 Mesenteric inflammatory myofibroblastic tumor 3 CONCLUSION Diagnosing mesenteric IMT as a cause of intestinal intussus- ceptions can be very challenging due to the rarity of the tumor and the unusual presentation. The pathological examination remains the essential tool for accurate diagnosis of IMT in all cases. REFERENCES 1. Yi E, Aubry M-C. Pulmonary pseudoneoplasms. Arch Pathol Lab Med 2010;134:417–26. 2. Chaudhary P. Mesenteric inflammatory myofibroblastic tumors. Ann Gastroenterol 2015;28:49–54. 3. Cessna MH, Zhou H, Sanger WG, Perkins SL, Tripp S, Pickering D, et al. Expression of ALK1 and p80 in inflammatory myofi- broblastic tumor and its mesenchymal mimics: a study of 135 Figure 4: (A) Positivity of ALK1 in the tumor cells. (B) Vimentin is diffusely positive cases. Mod Pathol 2002;15:931–8. in the tumor cells. (C) Negativity of CD34 in the tumor cells. (D) Tumor cells are 4. Snyder CS, Dell ’Aquila M, Haghighi P, Baergen RN, Suh YK, Yi negative for CD117 (Immunohistochemistry, original magnification ×100 [A, C, D] ES. Clonal changes in inflammatory pseudotumor of the lung: ×200 [B]). a case report. Cancer 1995;76:1545–9. 5. Karnak I, Senocak ME, Ciftci AO, Caglar ˘ M, Bingöl-Kologlu ˘ M, Tanyel FC, et al. Inflammatory myofibroblastic tumor in chil- they arise from mucosal membranes, unlike IMTs. A constel- dren: diagnosis and treatment. J Pediatr Surg 2001;36:908–12. lation of clinical and microscopic findings in addition to the 6. Groenveld RL, Raber MH, Oosterhof-Berktas R, Eijken E, Klaase results of immunohistochemical stains aid in ruling out the JM. Abdominal inflammatory myofibroblastic tumor. Case Rep other mesenchymal tumors and tumor-like lesions, such as Gastroenterol 2014;8:67–71. gastrointestinal stromal tumors, mesenteric fibromatosis and 7. Appak YÇ, Sahin GE, Ayhan S, Taneli C, Kasırga E. Inflam- sclerosing mesenteritis [2]. matory myofibroblastic tumor of the colon with an unusual In most cases, complete surgical resection is adequate; presentation of intestinal intussusception. European J Pediatr however, in cases where the tumor has locally recurred or Surg Rep 2014;2:54–7. metastasized, other treatment modalities might be used such 8. Miettinen M, Sobin LH, Sarlomo-Rikala M. Immunohisto- as chemotherapy, radiation treatment, nonsteroidal anti- chemical Spectrum of GISTs at different sites and their dif- inflammatory drugs, steroid and cyclosporin-A [2, 5]. After ferential diagnosis with a reference to CD117 (KIT). Mod Pathol 2 years of the surgery, our patient is in well condition without 2000;13:1134–42. any complaints.

Journal

Journal of Surgical Case ReportsOxford University Press

Published: Sep 1, 2020

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