Download PDF
CC BY-NC-ND 4.0 · South Asian J Cancer
DOI: 10.1055/s-0044-1791946
Case Report

Spindle Cell Variant of Rhabdomyosarcoma: An Aggressive Clinical Case of the Oral Cavity

Mayadevi Poojari
1   Department of Radiation Oncology, Oncolife Cancer Centre, Talegaon Dabhade, Pune, Maharashtra, India
,
Irappa Madabhavi
2   Department of Medical and Pediatric Oncology and Hematology, J. N. Medical College and Research Institute, Belgaum, Karnataka, India
3   Department of Medical and Pediatric Oncology and Hematology, Kerudi Cancer Hospital, Bagalkot, Karnataka, India
4   Nanjappa Hospital, Davangere, Karnataka, India
› Author Affiliations
› Further Information
Also available at
   Permissions and Reprints

Abstract

Zoom Image
Mayadevi Poojari

Rhabdomyosarcoma (RMS) is a malignant, soft tissue neoplasm consisting of cells derived from the primitive mesenchyme that exhibit a profound tendency to myogenesis. Historically, the spindle cell and sclerosing variants were subcategorized under embryonal type RMS. Spindle cell/sclerosing RMS (S-ScRMS) was recently recognized in 2013 by the World Health Organization as a stand-alone entity. Current data support that certain S-ScRMS cases have a more aggressive clinical course with a reduction of long-term survival, and those found in the head and neck region often exhibit extensive local recurrence. Here, we highlight an aggressive clinical course of S-ScRMS by presenting a case of a 7-year-old male child who presented with complaints of swelling in left cheek since 15 to 20 days. It was associated with pain. He was diagnosed as S-ScRMS and received chemotherapy but the disease was aggressive and progressed on chemotherapy. He received radiation but still the disease was inoperable, hence was kept on supportive care.

Keywords

rhabdomyosarcoma - oral cavity - spindle cell - soft tissue neoplasm - tomotherapy


Publication History

Received: 02 August 2024

Accepted: 21 September 2024

Article published online:
06 November 2024

© 2024. MedIntel Services Pvt Ltd. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

 

  • References

  • 1 Parham DM, Barr FG, Montgomery E, Nascimento AF. Skeletal muscle tumors. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F. eds. WHO Classification of Tumours of Soft Tissue and Bone. 4th ed. Lyon:: IARC Press;; 2013: 123-135
    Search in Google Scholar
  • 2 Cavazzana AO, Schmidt D, Ninfo V. et al. Spindle cell rhabdomyosarcoma. A prognostically favorable variant of rhabdomyosarcoma. Am J Surg Pathol 1992; 16 (03) 229-235
    CrossrefPubMedSearch in Google Scholar
  • 3 Smith MH, Atherton D, Reith JD, Islam NM, Bhattacharyya I, Cohen DM. Rhabdomyosarcoma, spindle cell/sclerosing variant: a clinical and histopathological examination of this rare variant with three new cases from the oral cavity. Head Neck Pathol 2017; 11 (04) 494-500
    CrossrefPubMedSearch in Google Scholar
  • 4 Alaggio R, Zhang L, Sung YS. et al. A molecular study of pediatric spindle and sclerosing rhabdomyosarcoma: identification of novel and recurrent VGLL2-related fusions in infantile cases. Am J Surg Pathol 2016; 40 (02) 224-235
    CrossrefPubMedSearch in Google Scholar
  • 5 Turner JH, Richmon JD. Head and neck rhabdomyosarcoma: a critical analysis of population-based incidence and survival data. Otolaryngol Head Neck Surg 2011; 145 (06) 967-973
    CrossrefPubMedSearch in Google Scholar
  • 6 Mentzel T, Katenkamp D. Sclerosing, pseudovascular rhabdomyosarcoma in adults. Clinicopathological and immunohistochemical analysis of three cases. Virchows Arch 2000; 436 (04) 305-11
    CrossrefPubMedSearch in Google Scholar