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Management of Ewing Sarcoma Family of Tumors: A Short Description of a Rare Primitive Uterine pPNET and Literature Review.
- Source :
-
OncoTargets and therapy [Onco Targets Ther] 2020 Feb 14; Vol. 13, pp. 1179-1184. Date of Electronic Publication: 2020 Feb 14 (Print Publication: 2020). - Publication Year :
- 2020
-
Abstract
- Purpose: To describe the outcome of a patient with a rare primitive uterine pPNET and to perform a review of the available data in literature, leading the clinicians to better face this rare disease.<br />Methods: We have rescued data regarding the multidisciplinary treatment of pPNET from the PUBMED database, highlighting also issues regarding the pathogenesis and the genetic landscape of the ESFTs (Ewing Sarcoma Family of Tumors).<br />Results: Ewing sarcoma and primitive neuroectodermal tumors (PNETs) are small round cell tumors presenting with different degrees of neuroectodermal differentiation. PNETs are further divided into central PNET and peripheral PNET (pPNET). Since pPNETs share the same genetic background of Ewing Sarcomas, they are considered to belong to the Ewing Sarcoma Family of Tumors (ESFTs). Multimodality treatment currently represents the best choice to offer to the affected patients.<br />Conclusion: Although pPNETs are generally diagnosed in children and young adults, an elderly woman aged 85 years came to our attention after a diagnosis of uterine pPNET. Her medical history is presented here, along with a literature review of the subject, highlighting the main biological, pathological and clinical features, with a hypothesis about the possible future therapeutic approaches for these rare malignancies.<br />Competing Interests: All authors declared no conflicts of interest in this work.<br /> (© 2020 Pisconti et al.)
Details
- Language :
- English
- ISSN :
- 1178-6930
- Volume :
- 13
- Database :
- MEDLINE
- Journal :
- OncoTargets and therapy
- Publication Type :
- Report
- Accession number :
- 32110037
- Full Text :
- https://doi.org/10.2147/OTT.S213233