title: Long-lasting activity of trabectedin in refractory uterine leiomyosarcoma: a case report.
abstract: BACKGROUND
  Leiomyosarcoma (LMS) is an aggressive soft tissue sarcoma derived from smooth muscle cells typically of uterine, gastrointestinal or soft tissue origin. The prognosis for this tumor is poor, with survival rates among the lowest of all soft tissue sarcomas. Surgery is the best approach for localized disease. The principal role of chemotherapy is prevalently in the treatment of metastatic disease. Trabectedin, a promising new DNA-damaging agent with a mechanism of action that differs from that of traditional alkylating agents, has been approved in Europe for the treatment of patients with advanced soft tissue sarcoma after failure of anthracyclines and ifosfamide,
  
  
  METHODS
  We report the case of a 53-year-old woman with metastatic well differentiated uterine leiomyosarcoma refractory to multiple treatments who underwent 22 cycles of trabectedin over 30 months, obtaining a partial response according to RECIST (Response Evaluation Criteria in Solid Tumors) criteria, with good tolerability, and maintaining the response for 10 months after trebectedin withdrawal.
  
  
  CONCLUSIONS
  This very prolonged response, which persisted after drug discontinuation, suggests that trabectedin exerts an oncostatic effect rather than the cytotoxic one produced by other chemotherapeutic agents. Our experience also raises the question of the best way to evaluate trabectedin efficacy.
authors.affiliation: Osteoncology and Rare Tumors Center, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy. alberto.bongiovanni@irst.emr.it.
authors.forename: Alberto
authors.initials: A
authors.lastname: Bongiovanni
authors.affiliation: Osteoncology and Rare Tumors Center, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy. nada.riva@irst.emr.it.
authors.forename: Nada
authors.initials: N
authors.lastname: Riva
authors.affiliation: Osteoncology and Rare Tumors Center, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy. marianna.ricci@irst.emr.it.
authors.forename: Marianna
authors.initials: M
authors.lastname: Ricci
authors.affiliation: Osteoncology and Rare Tumors Center, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy. laura.mercatali@irst.emr.it.
authors.forename: Laura
authors.initials: L
authors.lastname: Mercatali
authors.affiliation: Osteoncology and Rare Tumors Center, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy. chiara.liverani@irst.emr.it.
authors.forename: Chiara
authors.initials: C
authors.lastname: Liverani
authors.affiliation: Osteoncology and Rare Tumors Center, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy. federico.lamanna@irst.emr.it.
authors.forename: Federico
authors.initials: F
authors.lastname: La Manna
authors.affiliation: Osteoncology and Rare Tumors Center, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy. alessandro.devita@irst.emr.it.
authors.forename: Alessandro
authors.initials: A
authors.lastname: De Vita
authors.affiliation: Unit of Surgery and Advanced Oncologic Therapies, Morgagni-Pierantoni Hospital, Forlì, Italy. d.cavaliere@ausl.fo.it.
authors.forename: Davide
authors.initials: D
authors.lastname: Cavaliere
authors.affiliation: Pathology Unit, Morgagni-Pierantoni Hospital, Forlì, Italy. f.pieri@ausl.fo.it.
authors.forename: Federica
authors.initials: F
authors.lastname: Pieri
authors.affiliation: Radiology Unit, IRST IRCCS, Meldola, Italy. devil.oboldi@irst.emr.it.
authors.forename: Devil
authors.initials: D
authors.lastname: Oboldi
authors.affiliation: Unit of Surgery and Advanced Oncologic Therapies, Morgagni-Pierantoni Hospital, Forlì, Italy. gmverdecchia@gmail.com.
authors.forename: Giorgio Maria
authors.initials: GM
authors.lastname: Verdecchia
authors.affiliation: Osteoncology and Rare Tumors Center, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy. dino.amadori@irst.emr.it.
authors.forename: Dino
authors.initials: D
authors.lastname: Amadori
authors.affiliation: Osteoncology and Rare Tumors Center, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy. toni.ibrahim@irst.emr.it.
authors.forename: Toni
authors.initials: T
authors.lastname: Ibrahim
mesh_terms: D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use*; D004149:Dioxoles / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007890:Leiomyosarcoma / Q000188:drug therapy*; D008875:Middle Aged; D044005:Tetrahydroisoquinolines / Q000627:therapeutic use*; D000077606:Trabectedin; D016896:Treatment Outcome; D014594:Uterine Neoplasms / Q000188:drug therapy*
chemical_list: D018906:Antineoplastic Agents, Alkylating; D004149:Dioxoles; D044005:Tetrahydroisoquinolines; D000077606:Trabectedin