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Biochemical marker decline may not indicate clinical stability in intracranial growing teratoma syndromeTumor Markers Drop But Mass Grows: A Case Report

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Key Takeaway
Note that declining serum markers (AFP/beta-HCG) do not guarantee stability in patients with intracranial growing teratoma syndrome.

This case report details the management of 2 pediatric patients with suprasellar mixed germ cell tumors. The cases illustrate intracranial growing teratoma syndrome (iGTS), where chemotherapy led to a marked decline in serum alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (beta-HCG) levels in both cases. However, despite these biochemical improvements, radiologic tumor enlargement occurred in 2/2 cases.

The report notes that surgical resection revealed mature teratoma in both patients. A significant clinical finding is the dissociation between favorable biochemical markers and worsening local mass effects. The authors note that declining tumor markers should not be interpreted as reassurance when symptoms worsen or tumors enlarge.

Due to the small sample size of 2 cases, these findings have low generalizability but provide a specific clinical illustration of iGTS. The report suggests that early surgical intervention should be considered to relieve mass effect regardless of biochemical status.

How this fits prior evidence

This case report addresses a gap in clinical management by illustrating the dissociation between biochemical markers and radiologic progression in intracranial growing teratoma syndrome. While previous coverage noted that etoposide and capecitabine may improve extracranial disease control in pulmonary choriocarcinoma, this report highlights the specific risks of local mass effect despite systemic response in germ cell tumors.

A new case report describes two children with suprasellar mixed germ cell tumors, a type of brain tumor. They received chemotherapy with etoposide, cisplatin, and ifosfamide. During treatment, their blood tumor markers, alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (β-HCG), dropped dramatically. However, imaging showed that the tumors were actually getting bigger. This surprising pattern is known as intracranial growing teratoma syndrome (iGTS).

When the tumors were surgically removed, they turned out to be mature teratomas, which are non-cancerous but can still cause problems by pressing on the brain. Both children experienced permanent hypothalamic-pituitary dysfunction, a side effect of the tumor or its treatment. This condition can affect hormones, growth, and other vital functions.

The main lesson from this report is that falling tumor markers are not always a sign that the tumor is shrinking. In fact, if symptoms worsen or the tumor grows, doctors should consider early surgery to relieve pressure on the brain. This is a rare situation, and the report is based on only two cases, so it may not apply to everyone. Still, it highlights an important clinical clue for doctors treating children with these tumors.

For families and patients, this report underscores the need for careful monitoring during treatment. If you or a loved one are undergoing treatment for a brain tumor, always discuss any new or worsening symptoms with your healthcare team.

What this means for you:
In rare cases, tumor markers can drop even as the tumor grows, so imaging and symptoms matter.

Common questions

What is growing teratoma syndrome?

Growing teratoma syndrome is a rare condition where tumor markers in the blood go down, but the tumor itself gets bigger. In this case report, two children with brain germ cell tumors had this happen. The tumors turned out to be mature teratomas, which are non-cancerous but can still cause problems by pressing on the brain.

Why did the tumor markers drop if the tumor was growing?

The tumor markers, AFP and β-HCG, dropped because the chemotherapy killed the cancerous parts of the tumor. However, the tumor still grew because it contained mature teratoma tissue, which is not affected by chemotherapy. This is why doctors need to look at imaging and symptoms, not just blood tests.

What are the side effects of treatment in this case report?

Both children experienced permanent hypothalamic-pituitary dysfunction. This is a condition where the part of the brain that controls hormones is damaged, leading to problems with growth, puberty, and other body functions. It can be caused by the tumor itself or by treatments like surgery and radiation.

How many patients were in this case report?

This case report describes two pediatric patients. Because it is based on only two cases, the findings may not apply to everyone. It provides a specific example of growing teratoma syndrome, but more research is needed to understand how common this is and how to best treat it.

Study Details

Study typeSystematic review
EvidenceLevel 1
PublishedJul 2026
View Original Abstract ↓
BackgroundIntracranial 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 presentationWe 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.ResultsThese cases illustrate a clear dissociation between biochemical response and local mass enlargement, consistent with classic iGTS in both patients.ConclusionDeclining 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.
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