Management of Ewing Sarcoma Family of Tumors: A Short Description of a Rare Primitive Uterine pPNET and Literature Review

• Main Authors: Pisconti S, Della Vittoria Scarpati G, Buonerba C, Messinese S, Carella R, Di Marzo M, Di Lorenzo G, Lazzari G, Perri F, Solla R
• Format: Article
• Language: English
• Published: Dove Medical Press 2020-02-01
• Series: OncoTargets and Therapy
• Subjects: pnet; ppnet; ewing sarcoma; genetics
• Online Access: https://www.dovepress.com/management-of-ewing-sarcoma-family-of-tumors-a-short-description-of-a--peer-reviewed-article-OTT

Salvatore Pisconti,1 Giuseppina Della Vittoria Scarpati,2 Carlo Buonerba,3 Simona Messinese,1 Roberta Carella,1 Massimiliano Di Marzo,4 Giuseppe Di Lorenzo,3 Grazia Lazzari,5 Francesco Perri,6 Raffaele Solla7,8 1Medical Oncology Unit, POC, SS Annunziata, Taranto, Italy; 2Medical Oncology Unit, Pollena Trocchia Hospital, ASL NA3sud, Naples, Italy; 3Department of Clinical Medicine and Surgery, University Federico II of Naples, Naples, Italy; 4Department of Abdominal Surgery, National Tumor Institute of Naples IRCCS G. Pascale, Naples, Italy; 5Department of Radiation Oncology, POC, SS Annunziata, Taranto, Italy; 6Head and Neck Medical Oncology Unit, National Tumor Institute of Naples IRCCS G. Pascale, Naples, Italy; 7Department of Radiation Oncology, University of Naples Federico II, Naples, Italy; 8Italian National Research Council, Institute of Biostructure & Bioimaging, Naples, ItalyCorrespondence: Francesco PerriHead and Neck Medical Oncology Unit, National Tumor Institute of Naples IRCCS G. Pascale, Via Mariano Semmola, Naples 80131, ItalyTel [phone]Email [e-mail]: 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.Keywords: PNET, pPNET, Ewing Sarcoma, genetics