Morphological Features and Subtypes of Craniopharyngioma
Craniopharyngiomas may appear as cystic, solid, or mixed lesions, often containing fluid with high cholesterol content. They are classified into two major histological variants:Adamantinomatous Craniopharyngioma: Commonly affects pediatric populations. It frequently includes calcifications, cystic regions, and wet keratin, and may show infiltrative behavior.
Papillary Type: Seen predominantly in adults. It usually presents as a solid mass, generally lacks calcifications, and is considered less aggressive than the adamantinomatous variant.
Clinical Manifestations
Symptoms depend on the size and location of the tumor and how much it affects nearby structures:Visual Impairment: Involvement of the optic chiasm may result in visual field loss, diplopia, or decreased visual acuity.
Hormonal Imbalances: Disruption of the hypothalamic-pituitary axis can lead to growth hormone deficiency, gonadal failure, hypothyroidism, adrenal insufficiency, and diabetes insipidus.
Signs of Elevated Intracranial Pressure: Patients may report persistent headaches, nausea, or vomiting, often linked to obstructive hydrocephalus.
Neurocognitive and Emotional Changes: Especially in long-standing cases, patients may exhibit memory deficits, reduced attention span, or mood disturbances.
Diagnostic Methods
A comprehensive evaluation involves multiple modalities to assess anatomical, hormonal, and visual status:Neurological Examination: Focuses on detecting motor or sensory deficits, cognitive changes, and signs of raised intracranial pressure.
Magnetic Resonance Imaging (MRI): Craniopharyngioma MRI; provides high-resolution imaging for delineating tumor boundaries, involvement of optico-chiasmatic complex, and hypothalamic extension.
Computed Tomography (CT): In craniopharyngioma radiology; especially helpful in visualizing calcified components, commonly found in the adamantinomatous subtype.
Endocrine Evaluation: Blood tests are used to determine pituitary hormone levels and guide endocrine management.
Visual Field Testing: Perimetry helps identify early visual field defects and monitor progression.
Treatment Approaches
Management strategies are based on patient age, tumor characteristics, and potential risks to surrounding brain structures. A multidisciplinary approach is essential.1. Surgical Intervention
Microsurgical Resection: The aim is to remove the tumor as completely as possible while protecting critical structures such as the optic apparatus and hypothalamus.
Endoscopic Endonasal Surgery: A less invasive option that allows access through the nasal cavity. It is often suitable for tumors with a midline and sellar location. Complete resection may not always be achievable.
2. Radiation Therapy
Radiosurgery with Gamma Knife or CyberKnife: These stereotactic radiosurgery techniques deliver highly focused radiation in a single or few sessions, effectively targeting small or residual tumor portions while sparing surrounding healthy tissue.
Conventional Fractionated Radiotherapy: Often used following incomplete resection to control residual tumor tissue and reduce recurrence risk.
Proton Beam Therapy: Offers precise targeting with minimal exposure to healthy tissue, making it especially advantageous for younger patients and tumors close to critical areas like the optic pathway.
3. Endocrine Replacement Therapy
Long-term hormone monitoring is necessary in cases with pituitary dysfunction.
Replacement of thyroid hormones, steroids, sex hormones, and sometimes growth hormone is required to restore hormonal balance and support normal development and metabolism.