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Atypical extragonadal germ cell tumors
Mainak Deb1, Suravi Mohanty, Anuradha Ananthamurthy
1Department of Paediatric Surgery, St. John's Medical College Hospital, St. John's National Academy of Health Sciences, Bangalore, Karnataka, India.
Insights
Extragonadal germ cell tumors (GCTs) in children are rare and can present with unusual features. This study found a high event-free survival rate in pediatric patients treated for these tumors.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Tumorigenesis
Background:
- Extragonadal germ cell tumors (GCTs) are uncommon in children.
- These tumors can exhibit atypical clinical, radiological, and histological characteristics.
- Diagnosis and management present unique challenges.
Purpose of the Study:
- To review the experience with the diagnosis and management of pediatric extragonadal GCTs.
- To perform a subset analysis of GCTs with atypical features.
Main Methods:
- Retrospective chart review of pediatric patients diagnosed with extragonadal GCTs between 2000 and 2010.
- Analysis included patient demographics, tumor location, histology, treatment, and outcomes.
Main Results:
- Fifteen children (7 days to 15 years) were included, with tumors located in the retrobulbar area, neck, mediastinum, abdomen, and sacrococcygeal region.
- Five tumors had immature elements; two were malignant and received adjuvant chemotherapy.
- No mortality was observed, with 14 out of 15 patients achieving event-free survival at a median follow-up of 4.5 years.
Conclusions:
- Extragonadal GCTs in children are rare and can present with diagnostic and therapeutic dilemmas due to atypical features.
- Effective management, including surgery and chemotherapy when indicated, leads to favorable outcomes.
- Long-term surveillance is crucial, as exemplified by a late recurrence of an immature teratoma.
Aim:
To review the experience with the diagnosis and management of extragonadal germ cell tumors (GCT) with a subset analysis of those with atypical features.
Materials And Methods:
A retrospective chart review of patients of extragonadal germ cell tumors between 2000 and 2010 was carried out.
Results:
Fifteen children aged 7 days to 15 years (median, 1.5 years) were included. Three had an antenatal diagnosis (one sacrococcygeal, one retrobulbar, one retroperitoneal tumor) and were operated in the neonatal period. The locations were distributed between the retrobulbar area (1), anterior neck-thyroid gland (1), mediastinum (4), abdominothoracic extending through the esophageal hiatus (1), retroperitoneal (4) and sacrococcygeal (4). On histological examination, five harbored immature elements while two were malignant; the latter children received postexcision adjuvant chemotherapy. There was no mortality. At a median follow-up of 4.5 years (6 months to 8 years), 14/15 have had an event-free survival. One immature mediastinal teratoma that recurred locally 7.5 years after the initial operation was excised and adjuvant chemotherapy instituted.
Conclusions:
Extragonadal GCTs in children are uncommon and occasionally present with atypical clinical, radiological and histological features resulting in diagnostic and therapeutic dilemmas.
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