Case Report: Intracranial growing teratoma syndrome in two children with suprasellar mixed germ cell tumors: implications for the timing of surgery.
This report describes two children with suprasellar mixed germ cell tumors who developed symptomatic tumor enlargement despite falling AFP and β-HCG during chemotherapy, with resection demonstrating mature teratoma consistent with intracranial growing teratoma syndrome.
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This report describes two children with suprasellar mixed germ cell tumors who developed symptomatic tumor enlargement despite falling AFP and β-HCG during chemotherapy, with resection demonstrating mature teratoma consistent with intracranial growing teratoma syndrome.
Research significance
The cases provide clinical and histopathologic evidence that falling tumor markers can coexist with enlarging mature teratoma; they support, but do not establish, the hypothesis that prompt surgical evaluation and selected early resection during clinical or radiologic progression may relieve mass effect and possibly limit morbidity.
Source abstract
BACKGROUND: Intracranial growing teratoma syndrome (iGTS) is characterized by paradoxical tumor enlargement despite normalization of tumor markers during treatment of germ cell tumors. Its recognition is critical, particularly in the suprasellar region where mass effect can lead to significant morbidity. CASE PRESENTATION: We report two pediatric patients with suprasellar mixed germ cell tumors treated with alternating etoposide-cisplatin and ifosfamide-etoposide chemotherapy. In both cases, serum alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (β-HCG) levels declined markedly during treatment; however, clinical deterioration and radiologic tumor enlargement occurred. Surgical resection revealed mature teratoma in both cases, confirming classic iGTS after histopathologic review. Gross total resection was achieved in both cases, followed by completion of chemotherapy and craniospinal irradiation. At follow-up, both patients remained in remission but developed permanent hypothalamic-pituitary dysfunction. RESULTS: These cases illustrate a clear dissociation between biochemical response and local mass enlargement, consistent with classic iGTS in both patients. CONCLUSION: Declining tumor markers should not be interpreted as reassurance in the presence of worsening symptoms or tumor enlargement. Early surgical intervention should be considered in selected patients to relieve mass effect and potentially reduce long-term morbidity.