Craniopharyngioma & Other Pediatric Brain Tumors (Non-PNET, Non-Glioma)

What are these tumors and how are they categorized?

This is an “umbrella” of diagnoses—from benign tumors with outstanding long-term control to more aggressive entities that require combined-modality treatment.

This section primarily includes the following groups:

• Intracranial germ cell tumors (GCTs): most commonly arise in the pineal region and/or the suprasellar area. They are broadly divided into germinoma and non-germinomatous germ cell tumors (NGGCTs).
• Craniopharyngioma: a benign (WHO grade I) but technically challenging tumor of the pituitary/hypothalamic region, often impacting vision and the endocrine system.
• Neuronal tumors: ganglioglioma, gangliocytoma, DNET (dysembryoplastic neuroepithelial tumor), central neurocytoma— frequently low-grade tumors that may be associated with epilepsy.
• Choroid plexus tumors: choroid plexus papilloma (CPP, typically benign) and choroid plexus carcinoma (CPC, malignant), intraventricular tumors that can be highly vascular.
• Rarer tumors: meningioma, pituitary adenoma, primary CNS lymphoma, and hemangioblastoma (often associated with von Hippel–Lindau syndrome).

A shared principle across these entities is the need for precise diagnosis, specialized pediatric neurosurgical expertise, and close collaboration with pediatric oncology, endocrinology, neuro-ophthalmology, and supportive care teams.

How common are they and at what ages do they occur?

Less frequent than gliomas, yet collectively they represent a meaningful proportion of pediatric brain tumors.

Intracranial germ cell tumors account for under ~4% of pediatric brain tumors and occur most often in older children and adolescents. Craniopharyngiomas represent roughly 5–10% of primary pediatric brain tumors, with a peak incidence between ages 5–15.

Neuronal tumors (such as DNET and ganglioglioma) commonly present in children and young adults with focal epilepsy, while choroid plexus papillomas are more often seen in infants and very young children (< 3 years). The other entities (meningioma, pituitary adenoma, lymphoma, hemangioblastoma) are rarer in childhood, but timely recognition remains critical.

What are the typical symptoms?

Symptoms depend on tumor location—hydrocephalus, hormonal disturbances, visual changes, and seizures are common patterns.

Germ cell tumors (pineal / suprasellar region):

• signs of raised intracranial pressure (headache, vomiting, drowsiness),
• eye movement abnormalities (Parinaud syndrome—difficulty looking upward, convergence-retraction),
• endocrine disturbances such as diabetes insipidus, precocious or delayed puberty, and growth issues.

Craniopharyngioma:

• persistent headaches and visual symptoms (blurred vision, visual field loss),
• excessive thirst/urination, short stature, pubertal disturbance,
• rapid or unexplained weight gain.

Neuronal tumors (DNET, ganglioglioma, central neurocytoma):

• focal seizures, sometimes resistant to medication,
• with central neurocytoma: symptoms of hydrocephalus due to obstruction near the foramen of Monro.

Choroid plexus tumors:

• rapid increase in head circumference in infants,
• hydrocephalus with irritability, vomiting, and lethargy.

Rarer tumors (meningioma, pituitary adenoma, primary CNS lymphoma, hemangioblastoma) may cause headaches, progressive neurological deficits, or hormonal symptoms. Persistent headache with vomiting, new seizures, or visual changes in a child warrants prompt medical evaluation.

How is the diagnosis made?

Diagnosis is based on MRI, targeted laboratory testing, and—often—tissue confirmation via biopsy or surgical resection.

Initial evaluation commonly includes:

• Brain MRI with contrast to define location, size, and relationship to critical structures (hypothalamus, optic pathways, ventricles, major vessels).
• Spine MRI when there is concern for CSF dissemination (especially with NGGCT or CPC).
• Endocrine work-up and ophthalmologic assessment for tumors involving the pituitary/hypothalamus or suprasellar region.
• For germ cell tumors: measurement of AFP and β-hCG in serum and/or CSF, which can guide classification (germinoma vs NGGCT).

Definitive diagnosis is established with histopathology from biopsy or resection. Surgical planning may also address hydrocephalus—for example, endoscopic third ventriculostomy (ETV) in select pineal region tumors.

How are they classified—and what do tumor markers mean?

In certain tumors—especially germ cell tumors—serum/CSF markers play a central role in treatment planning and prognosis.

Intracranial germ cell tumors (GCTs) are broadly divided into:

• Germinoma: typically does not produce AFP; β-hCG may be mildly elevated. Germinomas are highly radiosensitive and often have excellent outcomes.
• Non-germinomatous germ cell tumors (NGGCTs): may include yolk sac tumor, choriocarcinoma, embryonal carcinoma, or mixed tumors, often producing AFP and/or β-hCG. They are generally more aggressive and frequently require combined surgery, chemotherapy, and broader-field radiotherapy.

Craniopharyngioma is considered WHO grade I, yet clinical behavior is largely driven by location and adherence to the hypothalamus and optic apparatus. Two histologic subtypes are recognized: adamantinomatous (common in children, often cystic/calcified) and papillary (more common in adults).

Neuronal tumors & choroid plexus tumors: DNETs and gangliogliomas are typically low-grade with excellent long-term control after complete resection. Choroid plexus papilloma (CPP) is generally benign, while choroid plexus carcinoma (CPC) is malignant and usually requires aggressive multimodality therapy.

What are the main treatment options?

Treatment is individualized by tumor type, age, location, and overall health—often combining surgery, radiotherapy, and chemotherapy.

Care is ideally coordinated through a multidisciplinary tumor board including pediatric neurosurgery, pediatric oncology, radiation oncology, endocrinology, neuro-ophthalmology, neuroradiology, and rehabilitation/support services.

Core treatment “pillars” may include:

• Surgery for decompression, maximal safe resection, and/or tissue diagnosis.
• Radiotherapy—central for germinoma; also used for residual/recurrent craniopharyngioma, CPC, and select additional tumor types.
• Chemotherapy—often essential for NGGCTs, CPC, primary CNS lymphoma, and selected rare entities.
• Endocrine and neuropsychological support—sometimes long-term or lifelong, especially for tumors affecting the hypothalamic-pituitary axis.

When is surgery alone sufficient?

For several of these tumors, complete resection—performed safely in an experienced center—can be effectively curative.

• Germinoma: surgery is typically limited to biopsy and/or hydrocephalus treatment; definitive therapy is radiotherapy ± chemotherapy.
• NGGCTs: aim for maximal safe resection plus chemotherapy and appropriate radiotherapy.
• DNET, ganglioglioma, gangliocytoma, central neurocytoma, CPP: gross total resection is often definitive, and adjuvant radiotherapy is frequently unnecessary.
• Craniopharyngioma: the goal is maximal safe tumor control while protecting hypothalamus and optic pathways; adjuvant radiotherapy is often considered for residual disease.

What complications and long-term effects can occur?

Both the tumor and its treatment can affect vision, endocrine function, cognition, and development—making long-term follow-up essential.

Examples by tumor category include:

• Germ cell tumors: hypothalamic-pituitary dysfunction, neurocognitive effects (particularly after radiotherapy), and the need for surveillance for CSF dissemination.
• Craniopharyngioma: frequent permanent panhypopituitarism, visual impairment, weight/metabolic dysregulation, and the need for lifelong endocrine follow-up.
• Neuronal tumors: appropriate resection may lead to seizure freedom, yet continued monitoring for seizure recurrence and medication adjustments may be needed.
• Choroid plexus tumors: potential for significant intraoperative bleeding; in malignant cases, risk of CSF spread.

What is the prognosis?

Many of these tumors have very high cure rates; others require more intensive and prolonged therapy.

• Germinoma: long-term survival often exceeds 90% with modern treatment approaches.
• NGGCTs: overall prognosis is less favorable than germinoma but has improved with combined therapy; outcome depends on histology, response to treatment, and extent of disease.
• Craniopharyngioma: high likelihood of durable tumor control, but often with substantial long-term morbidity.
• DNET, ganglioglioma, CPP, central neurocytoma: excellent outcomes after complete resection, with low recurrence rates.

Prognosis is individualized and depends on tumor type, extent of resection, presence of dissemination, and—in selected cases—underlying genetic syndromes. At Neuroknife, we take time to explain what each factor means for your child’s long-term outlook in clear, practical terms.

How is follow-up organized?

Follow-up often spans years and may include MRI surveillance, endocrine monitoring, and neuropsychological support.

Typical follow-up may include:

• scheduled brain MRI (and spine MRI when indicated),
• endocrine surveillance (thyroid, cortisol, growth hormone, sex hormones) and optimization of replacement therapy,
• ophthalmologic follow-up for acuity and visual fields when optic pathways are involved,
• neuropsychological assessment and support for school and social reintegration.

Practical considerations for parents and children

Beyond treatment, day-to-day life, school needs, and emotional wellbeing are equally important for the child and family.

At Neuroknife, we place special emphasis on:

• clear, honest communication about the diagnosis, treatment options, and realistic expectations,
• coordination with school services and learning support when needed,
• practical guidance for medications, hormone therapy, and warning signs families should monitor,
• psychological support for the child and family, when desired.