Gabriella Ferrandina1, Cynthia Aristei2, Pietro Raimondo Biondetti3, Ferdinando Carlo Maria Cananzi4, Paolo Casali5, Francesca Ciccarone6, Nicoletta Colombo7, Alessandro Comandone8, Renzo Corvo'9, Pierandrea De Iaco10, Angelo Paolo Dei Tos11, Vittorio Donato12, Marco Fiore13, Angiolo Gadducci14, Alessandro Gronchi15, Stefano Guerriero16, Amato Infante17, Franco Odicino18, Tommaso Pirronti19, Vittorio Quagliuolo20, Roberta Sanfilippo20, Antonia Carla Testa1, Gian Franco Zannoni21, Giovanni Scambia22, Domenica Lorusso1. 1. Fondazione Policlinico Universitario A. Gemelli IRCCS, Gynecologic Oncology Unit, Roma, Italy; Universita' Cattolica Del Sacro Cuore, Roma, Italy. 2. Radiation Oncology Section, Perugia General Hospital, Perugia, Italy. 3. Department of Radiology, Ca' Granda IRCSS Maggiore Policlinico Hospital Foundation Trust, Milan, Italy. 4. Surgical Oncology Unit - Humanitas Clinical and Research Center, Rozzano, Italy. 5. Department of Cancer Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy; Department of Oncology and Hemato-Oncology, University of Milan, Milan, Italy. 6. Fondazione Policlinico Universitario A. Gemelli IRCCS, Gynecologic Oncology Unit, Roma, Italy. 7. Gynecologic Cancer Program, University of Milan-Bicocca and European Institute of Oncology, IRCCS, Milan, Italy. 8. Division of Medical Oncology, Humanitas Gradenigo Hospital, Turin, Italy; ASL Città di Torino, Turin, Italy. 9. Radiation Oncology Department, IRCCS Ospedale Policlinico San Martino, Genoa, Italy; Health Science Department (DISSAL), University of Genoa, Genoa, Italy. 10. Unit of Oncologic Gynecology, Department of Obstetrics and Gynecology, University of Bologna, Hospital of Bologna Sant'Orsola-Malpighi Polyclinic, Bologna, Italy. 11. Department of Pathology, Treviso General Hospital Treviso, Padova, Italy; University of Padua, Padova, Italy. 12. Radiation Oncology Division, Oncology and Specialty Medicine Department, San Camillo-Forlanini Hospital, Roma, Italy. 13. Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. 14. Department of Clinical and Experimental Medicine, Division of Gynecology and Obstetrics, University of Pisa, Pisa, Italy. 15. Chair Sarcoma Service, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. 16. Department of Obstetrics and Gynecology, University of Cagliari, Policlinico Universitario Duilio Casula, Monserrato, Cagliari, Italy. 17. UOC COVID-2, Department of Bioimaging and Radiological Sciences, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Italy. 18. Division of Obstetrics and Gynecology, ASST Spedali Civili di Brescia, Università degli Studi di Brescia, Italy. 19. UOC COVID-2, Department of Bioimaging and Radiological Sciences, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Italy; Catholic University of Sacred Hearth, Department of Radiology, Rome, Italy. 20. Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. 21. Fondazione Policlinico Universitario A. Gemelli IRCCS, Department of Woman, Child and Public Health Sciences, Gynecopathology and Breast Pathology Unit, Rome, Italy; Pathological Anatomy Institute, Catholic University of Sacred Hearth, Rome, Italy. 22. Fondazione Policlinico Universitario A. Gemelli IRCCS, Gynecologic Oncology Unit, Roma, Italy; Universita' Cattolica Del Sacro Cuore, Roma, Italy. Electronic address: giovanni.scambia@policlinicogemelli.it.
Abstract
BACKGROUND: Uterine sarcomas are very rare tumours with different histotypes, molecular features and clinical outcomes; therefore, it is difficult to carry out prospective clinical trials, and this often results in heterogeneous management of patients in the clinical practice. AIM: We planned to set up an Italian consensus conference on these diseases in order to provide recommendations on treatments and quality of care in our country. RESULTS: Early-stage uterine sarcomas are managed by hysterectomy + bilateral salpingo-oophorectomy according to menopausal status and histology; lymphadenectomy is not indicated in patients without bulky nodes, and morcellation must be avoided. The postoperative management is represented by observation, even though chemotherapy can be considered in some high-risk patients. In early-stage low-grade endometrial stromal sarcoma and adenosarcomas without sarcomatous overgrowth, hormonal adjuvant treatment can be offered based on hormone receptor expression. In selected cases, external beam radiotherapy ± brachytherapy can be considered to increase local control only. Patients with advanced disease involving the abdomen can be offered primary chemotherapy (or hormonal therapy in the case of low-grade endometrial stromal sarcoma and adenosarcoma without sarcomatous overgrowth), even if potentially resectable in the absence of residual disease in order to test the chemosensitivity (or hormonosensitivity); debulking surgery can be considered in patients with clinical and radiological response. Chemotherapy is based on anthracyclines ± ifosfamide or dacarbazine. Palliative radiotherapy can be offered for symptom control, and stereotactic radiotherapy can be used for up to five isolated metastatic lesions. CONCLUSIONS: Treatment of uterine sarcoma should be centralised at referral centres and managed in a multidisciplinary setting.
BACKGROUND: Uterine sarcomas are very rare tumours with different histotypes, molecular features and clinical outcomes; therefore, it is difficult to carry out prospective clinical trials, and this often results in heterogeneous management of patients in the clinical practice. AIM: We planned to set up an Italian consensus conference on these diseases in order to provide recommendations on treatments and quality of care in our country. RESULTS: Early-stage uterine sarcomas are managed by hysterectomy + bilateral salpingo-oophorectomy according to menopausal status and histology; lymphadenectomy is not indicated in patients without bulky nodes, and morcellation must be avoided. The postoperative management is represented by observation, even though chemotherapy can be considered in some high-risk patients. In early-stage low-grade endometrial stromal sarcoma and adenosarcomas without sarcomatous overgrowth, hormonal adjuvant treatment can be offered based on hormone receptor expression. In selected cases, external beam radiotherapy ± brachytherapy can be considered to increase local control only. Patients with advanced disease involving the abdomen can be offered primary chemotherapy (or hormonal therapy in the case of low-grade endometrial stromal sarcoma and adenosarcoma without sarcomatous overgrowth), even if potentially resectable in the absence of residual disease in order to test the chemosensitivity (or hormonosensitivity); debulking surgery can be considered in patients with clinical and radiological response. Chemotherapy is based on anthracyclines ± ifosfamide or dacarbazine. Palliative radiotherapy can be offered for symptom control, and stereotactic radiotherapy can be used for up to five isolated metastatic lesions. CONCLUSIONS: Treatment of uterine sarcoma should be centralised at referral centres and managed in a multidisciplinary setting.
Authors: Riccardo Di Fiore; Sherif Suleiman; Francesca Pentimalli; Sharon A O'Toole; John J O'Leary; Mark P Ward; Neil T Conlon; Maja Sabol; Petar Ozretić; Ayse Elif Erson-Bensan; Nicholas Reed; Antonio Giordano; C Simon Herrington; Jean Calleja-Agius Journal: Int J Mol Sci Date: 2021-04-07 Impact factor: 5.923
Authors: Fionnuala Crowley; Karen A Cadoo; Sarah Chiang; Diana L Mandelker; Raazi Bajwa; Alexia Iasonos; Qin C Zhou; Kathryn M Miller; Martee L Hensley; Roisin E O'Cearbhaill Journal: Gynecol Oncol Rep Date: 2022-04-06
Authors: Carlo Ronsini; Aniello Foresta; Matteo Giudice; Antonella Reino; Marco La Verde; Luigi Della Corte; Giuseppe Bifulco; Pasquale de Franciscis; Stefano Cianci; Vito Andrea Capozzi Journal: Medicina (Kaunas) Date: 2022-08-23 Impact factor: 2.948