@article {8051, title = {Mature and immature teratoma: A report from the second Italian pediatric study.}, journal = {Pediatr Blood Cancer}, volume = {62}, year = {2015}, month = {2015 Jul}, pages = {1202-8}, abstract = {

BACKGROUND: Teratomas demonstrate a benign clinical behavior, however they may recur with malignant components or as teratoma, and in a small group of patients prognosis could be fatal. After the first Italian study, we collected cases of teratoma, alongside the protocol for malignant germ cell tumors.

PROCEDURE: Patients with teratoma were collected from 2004 to 2014. Teratomas were classified according to the WHO classifications, as mature and immature. Patients with pathological aFP and/or bHCG, and those with a malignant germ cell component were not included.

RESULTS: The study enrolled 219 patients (150 mature, 69 immature teratomas) with a median age at diagnosis of 42 months. The primary sites involved were: 118 gonadal and 101 extragonadal teratomas. Two females with ovarian teratoma had a positive family history. Complete and incomplete surgeries were performed in 85\% and 9\% of cases. Seventeen events occurred: six females had a second metachronous tumor (5 contralateral ovarian teratoma, 1 adrenal neuroblastoma) and 11 teratomas relapsed/progressed (3 mature, 8 immature teratomas). Two patients died, one of progressive immature teratoma and one of surgical complications. At a median follow up of 68 months, the event-free, relapse-free, and overall survival rates were 90.6\%, 94.3\%, 98.6\%, respectively.

CONCLUSIONS: Teratomas show a good prognosis, especially the mature ones: surgery and follow-up remain the standard approach. Incomplete surgery in immature teratoma is the group at greatest risk of relapse. Bilateral ovarian tumors are a possibility, and the rare family predisposition to ovarian mature teratoma warrants further analyses.

}, keywords = {Adolescent, Adult, Child, Child, Preschool, Female, Follow-Up Studies, Humans, Incidence, Infant, Infant, Newborn, Italy, Male, Neoplasm Grading, Neoplasm Recurrence, Local, Neoplasm Staging, Neoplasms, Second Primary, Neuroblastoma, Ovarian Neoplasms, Prognosis, Prospective Studies, Survival Rate, Teratoma, Testicular Neoplasms, Young Adult}, issn = {1545-5017}, doi = {10.1002/pbc.25423}, author = {Terenziani, Monica and D{\textquoteright}Angelo, Paolo and Inserra, Alessandro and Boldrini, Renata and Bisogno, Gianni and Babbo, Gian Luca and Conte, Massimo and Dall{\textquoteright} Igna, Patrizia and De Pasquale, Maria Debora and Indolfi, Paolo and Piva, Luigi and Riccipetitoni, Giovanna and Siracusa, Fortunato and Spreafico, Filippo and Tamaro, Paolo and Cecchetto, Giovanni} }