Craniopharyngiomas are rare intracranial squamous epithelial tumors with a solid and/or cystic Cystic Fibrocystic Change structure that arise from the remnants of Rathke’s pouch along the pituitary Pituitary A small, unpaired gland situated in the sella turcica. It is connected to the hypothalamus by a short stalk which is called the infundibulum. Hormones: Overview and Types stalk, in the suprasellar region. Craniopharyngiomas are histologically benign Benign Fibroadenoma but tend to invade surrounding structures; thus, they should be treated as low-grade malignancies. Histologically, there are 2 types of tumors: adamantinomatous, which is more common in children; and papillary, which tends to occur in adults. Both these types can present with various symptoms depending on their location and extension Extension Examination of the Upper Limbs patterns. The symptoms include headaches, nausea Nausea An unpleasant sensation in the stomach usually accompanied by the urge to vomit. Common causes are early pregnancy, sea and motion sickness, emotional stress, intense pain, food poisoning, and various enteroviruses. Antiemetics, vomiting Vomiting The forcible expulsion of the contents of the stomach through the mouth. Hypokalemia, visual disturbances, endocrine dysfunction, and behavioral issues. Diagnosis is made by imaging and histology. Management typically involves surgical excision and radiation Radiation Emission or propagation of acoustic waves (sound), electromagnetic energy waves (such as light; radio waves; gamma rays; or x-rays), or a stream of subatomic particles (such as electrons; neutrons; protons; or alpha particles). Osteosarcoma therapy.
Last updated: Jun 4, 2022
Craniopharyngiomas are rare squamous epithelial tumors with a solid and/or cystic Cystic Fibrocystic Change structure, which arise from the remnants of Rathke’s pouch along the pituitary Pituitary A small, unpaired gland situated in the sella turcica. It is connected to the hypothalamus by a short stalk which is called the infundibulum. Hormones: Overview and Types stalk, in line from the nasopharynx Nasopharynx The top portion of the pharynx situated posterior to the nose and superior to the soft palate. The nasopharynx is the posterior extension of the nasal cavities and has a respiratory function. Pharynx: Anatomy to the diencephalon Diencephalon The paired caudal parts of the prosencephalon from which the thalamus; hypothalamus; epithalamus; and subthalamus are derived. Development of the Nervous System and Face (in the suprasellar region).
Craniopharyngiomas have benign Benign Fibroadenoma histology but malignant behavior, meaning that they tend to invade the surrounding structures and can shorten life expectancy Life expectancy Based on known statistical data, the number of years which any person of a given age may reasonably expected to live. Population Pyramids. Therefore, despite their benign Benign Fibroadenoma appearance on histology, they are considered low-grade malignancies.
|Neuroepithelial tumors in the CNS||
|Sellar region tumors|
|Primary CNS lymphoma Lymphoma A general term for various neoplastic diseases of the lymphoid tissue. Imaging of the Mediastinum||Primary CNS lymphoma Lymphoma A general term for various neoplastic diseases of the lymphoid tissue. Imaging of the Mediastinum|
|Metastasis Metastasis The transfer of a neoplasm from one organ or part of the body to another remote from the primary site. Grading, Staging, and Metastasis to the brain Brain The part of central nervous system that is contained within the skull (cranium). Arising from the neural tube, the embryonic brain is comprised of three major parts including prosencephalon (the forebrain); mesencephalon (the midbrain); and rhombencephalon (the hindbrain). The developed brain consists of cerebrum; cerebellum; and other structures in the brain stem. Nervous System: Anatomy, Structure, and Classification (5x more common than primary brain Brain The part of central nervous system that is contained within the skull (cranium). Arising from the neural tube, the embryonic brain is comprised of three major parts including prosencephalon (the forebrain); mesencephalon (the midbrain); and rhombencephalon (the hindbrain). The developed brain consists of cerebrum; cerebellum; and other structures in the brain stem. Nervous System: Anatomy, Structure, and Classification tumors)||Most commonly arising from:|
There are 2 primary types of craniopharyngiomas:
Craniopharyngiomas are squamous epithelial tumors that arise along the pituitary Pituitary A small, unpaired gland situated in the sella turcica. It is connected to the hypothalamus by a short stalk which is called the infundibulum. Hormones: Overview and Types stalk in the suprasellar region, adjacent to the optic chiasm Optic Chiasm The x-shaped structure formed by the meeting of the two optic nerves. At the optic chiasm the fibers from the medial part of each retina cross to project to the other side of the brain while the lateral retinal fibers continue on the same side. As a result each half of the brain receives information about the contralateral visual field from both eyes. The Visual Pathway and Related Disorders.
Two main hypotheses have been proposed to explain the etiology of craniopharyngiomas:
Craniopharyngiomas are slow-growing tumors; therefore, there is an insidious development of symptoms usually after the tumor Tumor Inflammation attains a diameter > 3 cm. The onset of symptoms is usually 1‒2 years after the tumor Tumor Inflammation develops.
Visual disturbances are seen in about 40%‒65% of patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship and include:
Some type of endocrine dysfunction is common in craniopharyngiomas, and is seen in about 65%‒90% of patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship. Deficiencies in growth hormone (GH), gonadotropins, thyroid-stimulating hormone Thyroid-stimulating hormone A glycoprotein hormone secreted by the adenohypophysis. Thyrotropin stimulates thyroid gland by increasing the iodide transport, synthesis and release of thyroid hormones (thyroxine and triiodothyronine). Thyroid Hormones (TSH), and adrenocorticotropic hormone Adrenocorticotropic hormone An anterior pituitary hormone that stimulates the adrenal cortex and its production of corticosteroids. Acth is a 39-amino acid polypeptide of which the n-terminal 24-amino acid segment is identical in all species and contains the adrenocorticotropic activity. Upon further tissue-specific processing, acth can yield alpha-msh and corticotropin-like intermediate lobe peptide (clip). Adrenal Hormones (ACTH) can all be seen.
Cognitive dysfunction and behavioral issues may be due to impact on the thalamus Thalamus The thalamus is a large, ovoid structure in the dorsal part of the diencephalon that is located between the cerebral cortex and midbrain. It consists of several interconnected nuclei of grey matter separated by the laminae of white matter. The thalamus is the main conductor of information that passes between the cerebral cortex and the periphery, spinal cord, or brain stem. Thalamus: Anatomy, hypothalamus Hypothalamus The hypothalamus is a collection of various nuclei within the diencephalon in the center of the brain. The hypothalamus plays a vital role in endocrine regulation as the primary regulator of the pituitary gland, and it is the major point of integration between the central nervous and endocrine systems. Hypothalamus, and frontal Frontal The bone that forms the frontal aspect of the skull. Its flat part forms the forehead, articulating inferiorly with the nasal bone and the cheek bone on each side of the face. Skull: Anatomy lobes.
Increased intracranial pressure Increased Intracranial Pressure Normal intracranial pressure (ICP) is defined as < 15 mm Hg, whereas pathologically increased ICP is any pressure ≥ 20 mm Hg. Increased ICP may result from several etiologies, including trauma, intracranial hemorrhage, mass lesions, cerebral edema, increased CSF production, and decreased CSF absorption. Increased Intracranial Pressure (ICP) may occur due to obstructive hydrocephalus Obstructive Hydrocephalus Hydrocephalus in Children, and the signs/symptoms may include:
Diagnosis of craniopharyngiomas should be a multidisciplinary process, including the involvement of specialists in endocrinology, neurology, neuro-ophthalmology, and neurosurgery Neurosurgery Neurosurgery is a specialized field focused on the surgical management of pathologies of the brain, spine, spinal cord, and peripheral nerves. General neurosurgery includes cases of trauma and emergencies. There are a number of specialized neurosurgical practices, including oncologic neurosurgery, spinal neurosurgery, and pediatric neurosurgery. Neurosurgery.
The main approach to the diagnosis of craniopharyngiomas is by using radiological imaging, specifically MRI and CT, which reveal a sellar/suprasellar calcified cyst (note: calcification is rare with papillary type). The tumors may have both solid and cystic Cystic Fibrocystic Change components.
The following should be completed if not done at initial presentation:
Surgery is indicated in almost all cases, but there are 2 primary management options:
While surgical management is typically required, there are additional options available to reduce cyst size/ mass Mass Three-dimensional lesion that occupies a space within the breast Imaging of the Breast effect, including:
The conditions listed below should be considered in the differential diagnosis for craniopharyngiomas. Most cases will present similarly and can be differentiated based on differences in imaging and histology.
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