More Than Meets the Eye? A Cautionary Tale of Malignant Ectomesenchymoma Treated as Low-risk Orbital Rhabdomyosarcoma.
Journal Article (Journal Article)
Malignant ectomesenchymoma (MEM) is a rare multiphenotypic tumor comprised of mesenchymal and neuroectodermal components. MEM is typically diagnosed in infants and younger children and outcomes are variable. The current approach for treating MEM includes targeting the more aggressive mesenchymal component of the tumor, which is often rhabdomyosarcoma. Here, we describe a case of an orbital tumor initially diagnosed and treated as low-risk rhabdomyosarcoma. Local failure prompting a second biopsy revealed neuronal differentiation consistent with a diagnosis of MEM. Intensifying therapy and local radiotherapy led to a long-term cure. This case offers a cautionary tale that while outcomes for MEM were similar to matched rhabdomyosarcoma cohorts when treated on conventional Intergroup Rhabdomyosarcoma Study Group (IRSG) III/IV protocols, treating MEM using a decreased intensity low-risk rhabdomyosarcoma regimen may not be sufficient.
Full Text
Duke Authors
Cited Authors
- Rashid, T; Bagatell, R; Pawel, B; Bentley, RC; Kreissman, SG; Deel, MD
Published Date
- August 1, 2021
Published In
Volume / Issue
- 43 / 6
Start / End Page
- e854 - e858
PubMed ID
- 32769567
Electronic International Standard Serial Number (EISSN)
- 1536-3678
Digital Object Identifier (DOI)
- 10.1097/MPH.0000000000001901
Language
- eng
Conference Location
- United States