Desmoplastic small round cell tumor: clinical case and literature review-Reference-Cited by-同舟云学术

Desmoplastic small round cell tumor: clinical case and literature review

Published:2024-06-21 Issue:1 Volume:2 Page:45-56
ISSN:3034-1477
Container-title:Clinical Case in Oncology
language:
Short-container-title:jour

Author:

Natalenko K. E.¹^ORCID,Korkina A. A.²

Affiliation:

1. City Clinical Oncology Dispensary

2. St. Petersburg State University

Abstract

Desmoplastic small round cell tumour (DSRCT) is a very rare and aggressive malignant tumour first described in 1989 [1]. The incidence of DSRCT is approximately 0.2 cases per 1,000,000 population [2]. DSRCT usually occurs in adolescents and young males. DSRCT is characterised by high aggressiveness and despite comprehensive treatment, the prognosis in this disease remains extremely poor. Median progression-free survival (PFS) ranges from 4 to 21 months, overall survival (OS) ranges from 17 to 60 months, and only 10–20% of DSRCT patients cross the 5-year survival threshold [3]. Currently, there are no treatment protocols for DSRCT due to the rarity of the nosology. In this article, we present a clinical case of a patient with DSRCT with multiple peritoneal metastases treated with the VAC-IE regimen with a maximum treatment effect of partial regression.

Publisher

Association of Oncologist Real Clinical Practice OncoPractitioner

Reference41 articles.

1. Gerald W.L., Rosai J. Case 2 desmoplastic small cell tumor with divergent differentiation // Pediatric Pathology. 1989. Vol. 9, No. 2. P. 177–183.

2. De Pinieux G., Karanian M., le Loarer F. et al. Nationwide incidence of sarcomas and connective tissue tumors of intermediate malignancy over four years using an expert pathology review network // PLoS ONE. 2021. Vol. 16, No. 2. e0246958.

3. Honoré C., Amroun K., Vilcot L. et al. Abdominal desmoplastic small round cell tumor: multimodal treatment combining chemotherapy, surgery, and radiotherapy is the best option // Annals of Surgical Oncology. 2015. Vol. 22, No. 4. P. 1073–1079.

4. Thway K., Noujaim J., Zaidi S., Miah A.B., Benson C., Messiou C. et al. Desmoplastic Small Round Cell Tumor: Pathology, Genetics, and Potential Therapeutic Strategies // International Journal of Surgical Pathology. 2016. Vol. 24, No. 8. P. 672–684.

5. Sawyer J.R., Tryka A.F., Lewis J.M. A Novel Reciprocal Chromosome Translocation t(11;22)(p13;q12) in an Intraabdominal Desmoplastic Small Round-Cell Tumor // American Journal of Surgical Pathology. 1992. No. 16. P. 411–416.