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CC BY 4.0 · Indian Journal of Neurosurgery
DOI: 10.1055/s-0044-1790532
Brief Report

Desmoplastic Small Round Cell Tumor of the Spinal Canal: A Rare Presentation in a Female Pediatric Patient

Sana Ahuja 
1   Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
,
Shaivy Malik 
1   Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
,
Vivek Yadav
2   Department of Neurosurgery, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
,
Charanjeet Ahluwalia 
1   Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
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Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy primarily affecting young individuals, typically originating in the abdominal cavity. While spinal involvement by DSRCT is exceedingly rare, especially in pediatric patients, recognizing such atypical presentations is crucial for optimal management. Here, we present a case of DSRCT involving the spinal canal in a 3-year-old female child, representing one of the youngest documented instances in the literature. Initial symptoms included weakness, lower limb pain, and urinary incontinence. Magnetic resonance imaging revealed an enhancing soft tissue lesion in the spinal canal, prompting tumor excision and histopathological examination. The tumor exhibited characteristic features of DSRCT, including small round cells within a desmoplastic stroma. Immunohistochemistry confirmed the diagnosis, showing positivity for Wilms tumor 1, epithelial membrane antigen, desmin, and synaptophysin. This case highlights the challenges in diagnosing and managing rare tumors like spinal DSRCT, emphasizing the importance of a comprehensive approach to differential diagnosis and individualized treatment strategies.

Keywords

desmoplastic small round cell tumor - female pediatric - spinal canal

Patients' Consent

The authors certify that they have obtained all appropriate patients' consent forms.


Authors' Contribution

All authors equally contributed to the conceptualization and design of the study. S.A. provided the definition of intellectual content and performed the literature search. S.A., S.M., V.Y., and C.A. contributed to data acquisition. S.A. and C.A. were involved in data analysis, while C.A. acted as the guarantor for the study. S.A. led the manuscript preparation, with S.M., V.Y., and C.A. contributing to manuscript editing. Manuscript review was carried out by S.A., V.Y., and C.A.




Publication History

Article published online:
10 October 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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  • References

  • 1 Gerald WL, Rosai J. Desmoplastic small cell tumor with multi-phenotypic differentiation. Zentralbl Pathol 1993; 139 (02) 141-151
    PubMedSearch in Google Scholar
  • 2 Cao L, Ni J, Que R, Wu Z, Song Z. Desmoplastic small round cell tumor: a clinical, pathological, and immunohistochemical study of 18 Chinese cases. Int J Surg Pathol 2008; 16 (03) 257-262
    CrossrefPubMedSearch in Google Scholar
  • 3 Mello CA, Campos FAB, Santos TG. et al. Desmoplastic small round cell tumor: a review of main molecular abnormalities and emerging therapy. Cancers (Basel) 2021; 13 (03) 498
    CrossrefPubMedSearch in Google Scholar
  • 4 Yamauchi K, Owari M, Kimura K, Ishii T, Wakasa T, Yonekura T. Desmoplastic small round cell tumor: a case report and literature review. J Pediatr Surg Case Rep 2023; 96: 102691
    CrossrefPubMedSearch in Google Scholar
  • 5 Cacciotti C, Samji N, Cox S. et al. Desmoplastic small round cell tumor with ascending intraspinal metastasis at recurrence: case report and review of the literature. J Pediatr Hematol Oncol 2022; 44 (02) e561-e566
    CrossrefPubMedSearch in Google Scholar
  • 6 Ertoy Baydar D, Armutlu A, Aydin O, Dagdemir A, Yakupoglu YK. Desmoplastic small round cell tumor of the kidney: a case report. Diagn Pathol 2020; 15 (01) 95
    CrossrefPubMedSearch in Google Scholar