Rare and aggressive: imaging insights of intraperitoneal and retroperitoneal rhabdomyosarcoma in children and young adults

Shreyosi Santra; Suryansh Arora

doi:10.18203/2349-3291.ijcp20254194

Authors

Shreyosi Santra Department of Radiodiagnosis, CMC, Vellore, Tamil Nadu, India
Suryansh Arora Department of Radiodiagnosis, Dayanand Medical College, Ludhiana, Punjab, India

DOI:

https://doi.org/10.18203/2349-3291.ijcp20254194

Keywords:

Rhabdomyosarcoma, Intra-peritoneal tumor, Retro-peritoneal tumor, Aggressive, Soft-tissue sarcoma, Paediatric oncology

Abstract

This case series presents two cases of rhabdomyosarcoma (RMS), a rare malignancy of mesenchymal origin that accounts for 5% of all paediatric cancers and is extremely rare to present in adults. It can develop in various sites, including the head and neck, thorax, liver, biliary tract, retroperitoneum, urinary bladder, vagina, extremities, and paratesticular locations. Herein we present a case series of intrabdominal RMS, comprising the first case, an intraperitoneal RMS in a 2-year-old male child, and the second case, a retroperitoneal RMS in a 22-year-old male. Intraperitoneal RMS is extremely rare. Intraperitoneal and retroperitoneal RMS are aggressive tumours and have a poor prognosis. Both cases were of the spindle cell type of RMS, which is the most uncommon type.

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References

Egas-Bejar D, Huh WW. Rhabdomyosarcoma in adolescent and young adult patients: current perspectives. Adolesc Health Med Ther. 2014;5:115-25. DOI: https://doi.org/10.2147/AHMT.S44582

Dasgupta R, Rodeberg DA. Update on rhabdomyosarcoma. Semin Pediatr Surg. 2012;21(1):68-78. DOI: https://doi.org/10.1053/j.sempedsurg.2011.10.007

Ferrari A, Dileo P, Casanova M, Bertulli R, Meazza C, Gandola L, et al. Rhabdomyosarcoma in adults. A retrospective analysis of 171 patients treated at a single institution. Cancer. 2003;98(3):571-80.

Wasti AT, Mandeville H, Gatz S, Chisholm JC. Rhabdomyosarcoma. Pediatr Child Health. 2018;28:157-63. DOI: https://doi.org/10.1016/j.paed.2018.03.001

Martínez-Martínez A, García-Espinosa J, Pons EP. Primary mesenteric rhabdomyosarcoma in paediatric patient. Radiography. 2016;22(1):e8-10. DOI: https://doi.org/10.1016/j.radi.2015.05.007

Dagher R, Helman L. Rhabdomyosarcoma: an overview. The Oncol. 1999;4(1):34-44. DOI: https://doi.org/10.1634/theoncologist.4-1-34

Xia SJ, Pressey JG, Barr FG. Molecular pathogenesis of rhabdomyosarcoma. Cancer Biol Ther. 2002;1(2):97-104. DOI: https://doi.org/10.4161/cbt.51

Furlong MA, Mentzel T, Fanburg-Smith JC. Pleomorphic rhabdomyosarcoma in adults: A clinicopathologic study of 38 cases with emphasis on morphologic variants and recent skeletal muscle-specific markers. Mod Pathol. 2001;14;595-603.

Jo VY, Fletcher CD. WHO classification of soft tissue tumours: an update based on the 2013 (4th) edition. Pathology. 2014;46(2):95-104. DOI: https://doi.org/10.1097/PAT.0000000000000050

Chung CJ, Fordham L, Little S, Rayder S, Nimkin K, Kleinman PK, et al. Intraperitoneal rhabdomyosarcoma in children: incidence and imaging characteristics on CT. AJR. Am J Roentgenol. 1998;170(5):1385-7. DOI: https://doi.org/10.2214/ajr.170.5.9574621

Rajiah P, Sinha R, Cuevas C, Dubinsky TJ, Bush WH Jr, Kolokythas O. Imaging of uncommon retroperitoneal masses. Radiographics. 2011;31(4):949-76. DOI: https://doi.org/10.1148/rg.314095132

McCarville MB. What MRI can tell us about neurogenic tumors and rhabdomyosarcoma. Pediatr Radiol. 2016;46:881-90. DOI: https://doi.org/10.1007/s00247-016-3572-4

Chung CJ, Bui V, Fordham LA, Hill J, Bulas D. Malignant intraperitoneal neoplasms of childhood. Pediatr Radiol. 1998;28:317-21. DOI: https://doi.org/10.1007/s002470050363

Chung EM, Biko DM, Arzamendi AM, Meldrum JT, Stocker JT. Solid tumors of the peritoneum, omentum, and mesentery in children: radiologic-pathologic correlation: from the radiologic pathology archives. Radiographics. 2015;35(2):521-46. DOI: https://doi.org/10.1148/rg.352140273

Furlong MA, Mentzel T, Fanburg-Smith JC. Pleomorphic rhabdomyosarcoma in adults: a clinicopathologic study of 38 cases with emphasis on morphologic variants and recent skeletal muscle-specific markers. Mod Pathol. 2001;14(6):595-603. DOI: https://doi.org/10.1038/modpathol.3880357

Ferrari A, Dileo P, Casanova M, Bertulli R, Meazza C, Gandola L, et al. Rhabdomyosarcoma in adults. A retrospective analysis of 171 patients treated at a single institution. Cancer. 2003;98(3):571-80. DOI: https://doi.org/10.1002/cncr.11550