Document Type : Case Report(s)


1 Department of Hematology and Oncology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran

2 Department of Internal Medicine, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran

3 Department of Pathology, Ghaem Hospital, Mashhad University of Medical Sciences, Mashhad, Iran


Epithelioid rhabdomyosarcoma is an uncommon tumor. It is found mainly in somatic soft tissues like parotid, urinary bladder, trunk, neck, and limbs. Only two cases of this tumor in gastrointestinal tract (liver and gastro esophageal junction) has been reported to date; thus, we herein reported an unusual site of this tumor in the pylorus part of the stomach. In the present paper, we studied a 67-year-old male subject with complaint of dyspepsia, belching, and reflux for about 4 years. He had also consumed herbal drugs to relieve the pain for several years. In the endoscopy procedure, there was a large tumor at the distal part of the gaster. Furthermore, excisional biopsy was performed and the histological specimens showed tumor necrosis with high mitotic cell counts, large vesicles, eosinophilic cytoplasm of epithelial cells, and round nuclei. Immunohistochemistry staining was positive for Myogenin, Desmin, MyoD1 (30% ratio), and Ki67 (70 % ratio). Computed tomography scan images showed several metastases to peritoneum and lungs. After initiating the treatment, the adverse effects of chemotherapy persuade us to discontinue the regimen. Finally, the patient died due to pulmonary embolism. To conclude, primary epithelioid rhabdomyosarcoma is a very rare lethal cancer that may be found as gastrointestinal cancers. Hence, it requires further diagnostic investigations in order for the best treatment to be found.


Sajad Ataei Azimi (Google Scholar)


How to cite this article:

Ataei Azimi S, Qoorchi Moheb Seraj F, Jafarian AH, ShariatMaghani SS, Shamsabadi E. Gastric epithelioid rhabdomyosarcoma: a case report. Middle East J Cancer. 2023;14(3):463-8. doi: 10.30476/mejc.2023.95087.1755.

  1. Agaram NP. Evolving classification of rhabdomyosarcoma. Histopathology. 2022;80(1):98-108. doi: 10.1111/his.14449.
  2. WHO Classification of Tumours Editorial Board. WHO classification of tumours: soft tissue and bone tumours. 5th ed. Lyon: International Agency for Research on Cancer; 2020.
  3. Nakamura H, Koyanagi Y, Kitamura M, Kukita Y. Primary epithelioid rhabdomyosarcoma of the liver: Case report of an unusual histological variant. Int J Surg Pathol. 2022;30(3):331-4. doi: 10.1177/10668969211049832.
  4. Valério E, Almeida GC, Neotti T, Nascimento AG, Bezerra SM, Costa FD. Epithelioid rhabdomyosarcoma: Report of a cutaneous case and literature review of a recently described variant of rhabdomyosarcoma. Am J Dermatopathol. 2020;42(4):275-9. doi:10.1097/DAD.0000000000001560.
  5. Wang Y, Guo P, Zhang Z, Jiang R, Li ZJDP. Primary epithelioid rhabdomyosarcoma of the stomach: a case report and review of literature. Diagn Pathol. 2019;14(1):137. doi:10.1186/s13000-019-0917-y.
  6. Fan R, Parham DM, Wang LL. An integrative morphologic and molecular approach for diagnosis and subclassification of rhabdomyosarcoma. Arch Pathol Lab Med. 2022;146(8):953-9. doi: 10.5858/arpa.2021-0183-RA.
  7. Seidal T, Kindblom LG, Angervall L. Rhabdomyosarcoma in middle-aged and elderly individuals. APMIS. 1989;97(3):236-48. doi: 10.1111/j.1699-0463.1989.tb00783.x.
  8. Bowe SN, Ozer E, Bridge JA, Brooks JS, Iwenofu OH. Primary intranodal epithelioid rhabdomyosarcoma. Am J Clin Pathol. 2011;136(4):587-92. doi: 10.1309/AJCPRQES4NFDQ0XC.
  9. Zin A, Bertorelle R, Dall'Igna P, Manzitti C, Gambini C, Bisogno G, et al. Epithelioid rhabdomyosarcoma: a clinicopathologic and molecular study. Am J Surg Pathol. 2014;38(2):273-8. doi: 10.1097/PAS.0000000000000105.
  10. Fox KR, Moussa SM, Mitre RJ, Zidar BL, Raves JJ. Clinical and pathologic features of primary gastric rhabdomyosarcoma. Cancer. 1990;66(4):772-8. doi: 10.1002/1097-0142(19900815)66:4<772::aid-cncr2820660429>;2-r.
  11. Yu L, Lao IW, Wang J. Epithelioid rhabdomyosarcoma: a clinicopathological study of seven additional cases supporting a distinctive variant with aggressive biological behaviour. Pathology. 2015;47(7):667-72. doi: 10.1097/PAT.0000000000000321.
  12. Schizas D, Tomara N, Katsaros I, Sakellariou S, Machairas N, Paspala A, et al. Primary gastric melanoma in adult population: a systematic review of the literature. ANZ J Surg. 2021;91(3):269-75. doi: 10.1111/ans.16160.
  13. Malipatel R, Patil M, Pritilata Rout P, Correa M, Devarbhavi H. Primary gastric lymphoma: Clinicopathological profile. Euroasian J Hepatogastroenterol. 2018;8(1):6-10. doi: 10.5005/jp-journals-10018-1250.
  14. Köseoğlu H, Duzenli T, Sezikli M. Gastric neuroendocrine neoplasms: A review. World J Clin Cases. 2021;9(27):7973-85. doi: 10.12998/wjcc.v9.i27.7973.
  15. Gonzalez RS, Messing S, Tu X, McMahon LA, Whitney-Miller CL. Immunohistochemistry as a surrogate for molecular subtyping of gastric adenocarcinoma. Hum Pathol. 2016;56:16-21. doi: 10.1016/j.humpath.2016.06.003.
  16. Wang X, Mori I, Tang W, Utsunomiya H, Nakamura M, Nakamura Y, et al. Gastrointestinal stromal tumors: clinicopathological study of Chinese cases. Pathol Int. 2001;51(9):701-6. doi: 10.1046/j.1440-1827.2001.01260.x.
  17. Eguía-Aguilar P, López-Martínez B, Retana-Contreras C, Perezpeña-Diazconti M. Alveolar rhabdomyosarcoma: origin and prognostic implications of molecular findings. Bol Med Hosp Infant Mex. 2016;73(6):405-10. doi: 10.1016/j.bmhimx.2016.09.001.
  18. Cummings NP, Morris HG, Strunk RC. Failure of children with asthma to respond to daily aspirin therapy. J Allergy Clin Immunol. 1983;71(2):245-9. doi: 10.1016/0091-6749(83)90106-9.
  19. Agaram NP, LaQuaglia MP, Alaggio R, Zhang L, Fujisawa Y, Ladanyi M, et al. MYOD1-mutant spindle cell and sclerosing rhabdomyosarcoma: an aggressive subtype irrespective of age. A reappraisal for molecular classification and risk stratification. Mod Pathol. 2019;32(1):27-36. doi: 10.1038/s41379-018-0120-9.
  20. Deb PQ, Chokshi RJ, Li S, Suster DI. Pleomorphic rhabdomyosarcoma: A systematic review with outcome analysis and report of a rare abdominal wall lesion. Int J Surg Pathol. 2022:10668969221105622. doi: 10.1177/10668969221105622.