Epithelioid inflammatory myofibroblastic sarcoma: the youngest case reported

Sajida Batool; Arvind Ahuja; Devender Singh Chauhan; Minakshi Bhardwaj; Atul Kumar Meena

doi:10.4322/acr.2021.288

Clinical Case Report

Epithelioid inflammatory myofibroblastic sarcoma: the youngest case reported

Sajida Batool; Arvind Ahuja; Devender Singh Chauhan; Minakshi Bhardwaj; Atul Kumar Meena

http://dx.doi.org/10.4322/acr.2021.288 Autops Case Rep, vol.11, e2021288, 2021

PDF

Downloads: 1

Abstract

Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of the inflammatory myofibroblastic tumor. It has an aggressive clinical course and a high rate of recurrence. EIMS primarily affects children and young adults. Hereby, we report this entity in a 4-month-old infant who presented with an abdominal mass. Imaging studies revealed a large hypodense mesentery-based lesion involving the right half and mid-region of the abdomen. The mass with an attached segment of the small bowel was excised in toto. Grossly, a large encapsulated tumor was identified arising from the mesentery of the small bowel. The histological examination showed a tumor consisting of epithelioid to spindle cells loosely arranged in a myxoid background with numerous blood vessels and lymphoplasmacytic inflammatory infiltrate. On immunohistochemistry, the tumor cells showed positivity for ALK1 (nuclear), desmin, SMA, CD68, and focal positivity for CD30. A final diagnosis of EIMS of the small intestine was rendered. To the best of our knowledge, this case is the youngest reported case in literature.

Keywords

Sarcoma, Epithelioid Cells, Intestine, Small, Mesentery, Anaplastic Lymphoma Kinase

References

1 Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F. World Health Organization Classification of Tumors of Soft Tissue and Bone. 4th ed. Lyon, France: IARC Press; 2013. p. 83-4.

2 Marino-Enriquez A, Wang W-L, Roy A, Lopez-Terrada D, Lazar AJF, Fletcher CDM, et al. Epithelioid inflammatory myofibroblastic sarcoma: an aggressive intra-abdominal variant of inflammatory myofibroblastic tumor with nuclear membrane or perinuclear ALK. Am J Surg Pathol. 2011;35(1):135-44. http://dx.doi.org/10.1097/PAS.0b013e318200cfd5. PMID: 21164297.

3 Du X, Gao Y, Zhao H, Li B, Xue W, Wang D. Clinicopathological analysis of epithelioid inflammatory myofibroblastic sarcoma. Oncology Letters. 2018;15(6):9317-26. http://dx.doi.org/10.3892/ol.2018.8530. PMID: 29805657.

4 Yu L, Liu J, Lao IW, Luo Z, Wang J. Epithelioid inflammatory myofibroblastic sarcoma: a clinicopathological, immunohistochemical and molecular cytogenetic analysis of five additional cases and review of the literature. Diagn Pathol. 2016;11(1):67. http://dx.doi.org/10.1186/s13000-016-0517-z. PMid:27460384.

5 Xu P, Shen P, Jin Y, Wang L, Wu W. Epithelioid inflammatory myofibroblastic sarcoma of stomach: diagnostic pitfalls and clinical characteristics. Int J Clin Exp Pathol. 2019;12(5):1738-44. PMid:31933992.

6 Garg R, Kaul S, Arora D, Kashyap V. Posttransplant epithelioid inflammatory myofibroblastic sarcoma: a case report. Indian J Pathol Microbiol. 2019;62(2):303. http://dx.doi.org/10.4103/IJPM.IJPM_284_17. PMID: 30971562.

7 Zhang S, Wang Z. A case report on epithelioid inflammatory myofibroblastic sarcoma in the abdominal cavity. Int J Clin Exp Pathol. 2019;12(10):3934-9. PMid:31933785.

8 John R, Goldblum SW, Weiss ALF. Enzinger and weiss’s soft tissue tumors. 6th ed. Philadelphia, USA: Elsevier Saunders; 2014. P. 304-10.

9 Cook JR, Dehner LP, Collins MH, et al. Anaplastic lymphoma kinase (ALK) expression in the inflammatory myofibroblastic tumor: a comparative immunohistochemical study. Am J Surg Pathol. 2001;25(11):1364-71. http://dx.doi.org/10.1097/00000478-200111000-00003. PMid:11684952.

10 Griffin CA, Hawkins AL, Dvorak C, Henkle C, Ellingham T, Perlman EJ. Recurrent involvement of 2p23 in inflammatory myofibroblastic tumors. Cancer Res. 1999;59(12):2776-80. PMID: 10383129.

11 Hallin M, Thway K. Epithelioid inflammatory myofibroblastic sarcoma. Int J Surg Pathol. 2019;27(1):69-71. http://dx.doi.org/10.1177/1066896918767557. PMid:29623737.

12 Lee J-C, Li C-F, Huang H-Y, et al. ALK oncoproteins in atypical inflammatory myofibroblastic tumours: novel RRBP1-ALK fusions in epithelioid inflammatory myofibroblastic sarcoma. J Pathol. 2017;241(3):316-23. http://dx.doi.org/10.1002/path.4836. PMid:27874193.

13 Li J, Yin W, Takeuchi K, Guan H, Huang Y, Chan JKC. Inflammatory myofibroblastic tumor with RANBP2 and ALK gene rearrangement: a report of two cases and literature review. Diagn Pathol. 2013;8(1):147. http://dx.doi.org/10.1186/1746-1596-8-147. PMid:24034896.

14 Fujiya M, Kohgo Y. ALK inhibition for the treatment of refractory epithelioid inflammatory myofibroblastic sarcoma. Intern Med. 2014;53(19):2177-8. http://dx.doi.org/10.2169/internalmedicine.53.3038. PMid:25274227.

Submitted date:
01/10/2021

Accepted date:
04/11/2021

Publication date:
05/25/2021

Epithelioid inflammatory myofibroblastic sarcoma: the youngest case reported

Sajida Batool; Arvind Ahuja; Devender Singh Chauhan; Minakshi Bhardwaj; Atul Kumar Meena

Abstract

Keywords

References

Links

Share

Autops Case Rep