Mature and immature teratoma: A report from the second Italian pediatric study

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. Pediatr Blood Cancer 2015;62:1202–1208. © 2015 Wiley Periodicals, Inc.

Pediatric teratomas: Still fascinating

“…The compliance to guidelines was good as only four cases of 69 immature teratomas received initial chemotherapy at the discretion of the physician in charge. Contralateral metachronous teratomas developed with a delay of 11 to 84 months in 5/95 patients with ovarian teratomas . This is in line with a 66% ± 26% risk of metachronous teratomas published within 10 years .…”

“…Pure teratomas tell a different story: The strategy in adults may not apply because pediatric teratomas do not have similar biological significance and outcome. The second pediatric Italian study on mature and immature teratomas reported in this issue is a major contribution to this field . Based on guidelines the Italian registry reports for the second time an exhaustive collection of data, This suggests an active ongoing collaboration between pathologists and clinicians that should be acknowledged.…”

Gonadal germ cell tumors in children

“…Immature teratoma was the 2nd common type of malignant GCT, which was diagnosed in 7 patients. According to a grading system of immature teratoma introduced by Norris, modified by Gonzales-Crussi and others, [5] 4 were grade 1, 2 were grade 2, and 1 was grade 3. The pathology of malignant mixed GCT included dysgerminoma and yolk sac tumor.…”

“…AFP is also an early sign of relapse of malignant GCTs, and testing AFP levels may help to reduce the burden of follow-up. [5] In recent literature, the rate of benign lesions associate with the rise of tumor markers varies from 3.4% to 20.7%. [24,25] In our study, the patients with benign GCTs who had serum tumor markers detection had a normal level, except 1 patient with ovarian GCT had a slightly high level of AFP.…”

Tumours of the chest in children: A review

“…Adjuvant chemotherapy should be employed for immature teratomas. [11] The definitive diagnosis rests on histology after the lesion is removed, demonstrating features from multiple germ cell lines as shown in Fig. 4.…”