Intraabdominal and ganglionic desmoplastic small round cell tumor: a case series.
Case report
Chemotherapy
Desmoplastic small round cell
Radiotherapy
Surgery
Journal
Journal of medical case reports
ISSN: 1752-1947
Titre abrégé: J Med Case Rep
Pays: England
ID NLM: 101293382
Informations de publication
Date de publication:
12 Oct 2021
12 Oct 2021
Historique:
received:
13
05
2021
accepted:
02
09
2021
entrez:
12
10
2021
pubmed:
13
10
2021
medline:
14
10
2021
Statut:
epublish
Résumé
Desmoplastic small round cell tumor is a rare malignancy with poor prognosis, affecting young male patients. It frequently presents as a large abdominal mass with widespread peritoneal involvement at diagnosis. In late stages, metastases may be present. We retrospectively reviewed patient characteristics, presenting symptoms, tumor pathology, treatment, and outcome of four patients with desmoplastic small round cell tumor at our institution. The first three cases reported are 32-, 17-, and 30-year-old North African males with intraabdominal desmoplastic small round cell tumor treated by surgery, chemotherapy, and radiation therapy with different follow-ups. The final case is a 16-year-old North African male with ganglionic desmoplastic small round cell tumor but no evidence of a tissue mass. He underwent two lines of chemotherapy with no response. The patient was lost after 2 years of follow-up. In all cases, desmoplastic small round cell tumor was confirmed by presence of t(11,22) (p13,q12) translocation. Treatment of desmoplastic small round cell tumor is based on multidisciplinary therapy. Despite high-dose chemotherapy, extensive surgical resection, and radiotherapy, desmoplastic small round cell tumor remains lethal.
Identifiants
pubmed: 34635162
doi: 10.1186/s13256-021-03094-9
pii: 10.1186/s13256-021-03094-9
pmc: PMC8507229
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
500Informations de copyright
© 2021. The Author(s).
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