Management of Ewing Sarcoma Family of Tumors: A Short Description of a Rare Primitive Uterine pPNET and Literature Review
| Autor: | Salvatore Pisconti, Roberta Carella, Simona Messinese, Giuseppe Di Lorenzo, Raffaele Solla, Francesco Perri, Carlo Buonerba, Giuseppina Della Vittoria Scarpati, Massimiliano Di Marzo, Grazia Lazzari |
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| Rok vydání: | 2020 |
| Předmět: |
0301 basic medicine
Oncology medicine.medical_specialty business.industry Peripheral PNET medicine.disease 03 medical and health sciences Neuroectodermal Differentiation 030104 developmental biology 0302 clinical medicine 030220 oncology & carcinogenesis Internal medicine medicine Round cell Pharmacology (medical) Medical history Sarcoma Young adult business Pathological Rare disease |
| Zdroj: | OncoTargets and Therapy. 13:1179-1184 |
| ISSN: | 1178-6930 |
| Popis: | 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. 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). 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. 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. |
| Databáze: | OpenAIRE |
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