Outcomes and prognostic variables of extracranial germ cell tumors in children and adolescents treated over a decade: A developing world perspective

Abstract

Background:The purpose of this single-center study was to analyze the outcomes of extracranial germ cell tumors (GCTs) in children treated on a multimodality regimen. Methods: Retrospective study of children (<18 years) with a histopathologically confirmed diagnosis of extracranial GCT over a period of 10 years (January 2009 to December 2018) treated on a uniform institution-based protocol consisting of both cisplatin-and carboplatin-based regimens. All completely excised teratomas and stage I gonadal tumors received no further therapy (low risk [LR]); stage IV ovarian, stage III-IV extragonadal GCTs received six cycles of chemotherapy (high risk [HR]), and the remaining received four cycles of chemotherapy (intermediate risk [IR]).Results: A total of 297 children were treated with a female:male ratio of 1.72:1 and median age of 4 years. Forty-three children had pure teratomas. Gonadal GCTs (N = 180) were more common than extragonadal GCTs (N = 117) with ovary as primary site in 128 children (43%) and sacrococcygeal site being the commonest extragonadal location (N = 41; 14%). LR, IR, and HR disease were noted in 60 (20.2%), 125 (42%), and 112 (37.8%) patients, respectively. Three-fourths of ovarian tumors and half of testicular tumors operated prior to presentation needed upstaging. Forty-one patients relapsed and 43 children expired (disease-related: 33; toxic deaths: 9; unknown: 1). The 5-year event-free survival (EFS)/overall survival (OS) of malignant GCT (n = 254) was 72.50%/82.70%, respectively, with gonadal site (p = .001), LR and IR (p = .001) and nonmetastatic disease (p = .001) being favorable prognostic variables. Conclusions:The LR and IR GCTs in our cohort had an excellent outcome. A significant proportion of IR gonadal GCTs can be spared of systemic chemotherapy by adhering to strict surgical guidelines.

Outcomes of relapsed/refractory extracranial germ cell tumors treated on conventional salvage chemotherapy without stem cell rescue: Experience from a tertiary cancer center

“…This could be because of a higher proportion of gonadal rel/ref‐GCTs in this cohort, which is known to be associated with a better outcome. Of note, we noted a similar observation in the cohort of pediatric GCTs at primary presentation treated at our center 6 …”

“…Of note, we noted a similar observation in the cohort of pediatric GCTs at primary presentation treated at our center. 6 Similar to other groups, we also noted the relatively poor outcome in ref-GCTs when compared to rel-GCTs. 2,12,18 In our cohort, ref-GCTs represented a resistant and "difficult-to-treat" subgroup of pediatric GCTs and need more intensive therapy than conventional SC.…”

“…Risk at primary presentation was stratified as all completely excised teratomas and stage I gonadal tumors being low risk (LR); stage IV ovarian, stage III–IV extragonadal GCTs as high risk (HR), and the remaining as intermediate risk (IR). Treatment protocol comprised of LR patients being treated with surgery alone, and IR patients and HR GCTs receiving four and six cycles of platinum‐based chemotherapy, respectively 6 . All rel‐GCTs and ref‐GCTs occurring during this time were included (Figure 1).…”

“…Treatment protocol comprised of LR patients being treated with surgery alone, and IR patients and HR GCTs receiving four and six cycles of platinum-based chemotherapy, respectively. 6 All rel-GCTs and ref-GCTs occurring during this time were included (Figure 1). Apart from these, children presenting to our institution with relapse/progression after receiving their primary chemotherapy elsewhere were also included (Figure 1).…”

Extracranial germ cell tumours in children and adolescents: Results from the French TGM13 protocol

“…As already reported in the paediatric 6,7,15 and adult literature on GCTs, 8 extragonadal tumours have a higher risk of progression. In the current series, the 5‐year EFS was inferior in the IR2 group (extragonadal tumours treated with VBP) compared with the other risk groups.…”