The most common solid tumor in children. Second most common childhood cancer after leukaemia . * Most (CNS) tumors in children and adolescents are primary tumors that originate in the CNS, include: - low-grade astrocytomas - embryonic neoplasms (medulloblastoma, ependymoma). * Most tumors are infratentiorial
Calcification: - Primary tumors : do not metastasize to organs outside the CNS . - Metastatic tumors : lungs, breast , melanoma. - Benign - Malignant
symptoms * Presenting symptoms vary depending on tumor location. - Headache : dull, slowly progressive, poorly localized. - Focal neurological deficits : cranial nerve palsies, seizures. - Neurocognitive disturbance : deficit in school and work. - Psychiatric symptoms : depression. - Obstructive hydrocephalus : macrocephaly. - Visual field disturbances.
Pilocytic astrocytoma *Brain tumor that originates from star-shaped cells called astrocytes — a kind of glial cell, which support and nourish neurons in the brain. An astrocytoma is a type of glioma. *The most common primary brain tumor of childhood *Most common benign tumor. *slow-growing, circumscribed, non-invasive tumor * location Cerebellum ,Cerebral hemispheres. *Clinical features : slow progression of symptoms Vomiting, Ataxia, Failure to thrive. *Associated conditions: neurofibromatosis type 1
Diagnosis * MRI findings: Well-demarcated cystic lesion Bright contrast-enhancing solid nodule in the wall of the cyst
*biopsy: - well differentiated neoplasms - comprised of spindle cells with hair-like glial processes that are associated with microcysts. - GFAP positive
*Treatment: Surgical resection *Prognosis: Favorable prognosis: high rates of long-term survival Curable with complete resection of the tumor Median survival: > 10 years
Medulloblastoma *Most common malignant pediatric brain. *derived from primitive neuroectodermal tissue. *highly malignant tumor ,undifferentiated and aggressive with poor prognosis. *Peak incedince : 1st decade *Associated conditions : Turcot syndrome
Clinical features *The most common location is the cerebellum. *Most tumors arise within the cerebellar vermis : truncal ataxia. *Invasion or compression of the 4th ventricle : noncommunicating hydrocephalus, features of raised intracranial pressure (e.g., papilledema, vomiting, headache). *Drop metastases to the spinal cord are common → paraplegia.
Treatment and prognosis *surgical resection. *Adjuvant therapy : -Children ≥ 3 years: chemotherapy and craniospinal radiotherapy. -Children < 3 years: chemotherapy. *5-year survival rate: 60–80%. *Poor prognostic factors : - Inadequate resection - Presence of drop metastases - HER2/neu mutation
Ependymoma *The third most common brain neoplasm in children. *It arise from ependymal cells of ventricular system . *Associated conditions: Neurofibromatosis type II. *The 4th ventricle is the most common location in children.
Clinical features *The 4th ventricle : non-communicating hydrocephalus, features of raised intracranial pressure (e.g., headache, vomiting, papilledema). *Weakness and seizures are less common with ependymomas. *Ependymomas in the posterior fossa frequently infiltrate the brain stem, and one third may project through the foramina to involve the medulla and upper spinal cord.
Diagnosis and treatment *Imaging : Intra-parenchymal tumor with calcifications and cystic components due to necrosis and/or hemorrhage. *Biopsy : -Perivascular pseudorosettes . -Rod-shaped bodies ( blepharoblasts ) near the nucleus. *Surgical resection *Adjuvant radiotherapy
prognosis *poor prognosis. *Overall 5-year survival rate: 65–90% *Relied on age, presence of metastatic disease, extent of resection, and the appearance of significant histologic anaplasia : < 1 y have a significantly worse prognosis. older than 5 years have better prognosis.
Craniopharyngioma *benign, slow growing *derived from remnant of Rathke pouch. *bimodal age distribution: 5-14, 50-75.
clinical features The tumor arises in the suprasellar region and can extend into the intrasellar region: -Compression of the ventromedial hypothalamic nucleus : hyperphagia and obesity -Compression of interventricular foramina and/or aqueduct : obstructive hydrocephalus -optic chiasm compression: bitemporal hemianopsia. -Pituitary stalk compression: endocrinopathies: Growth failure, pubertal delay, diabetes insipidus.
Diagnosis *Imaging -Suprasellar calcified cyst with a lobulated contour *Biopsy -Cholesterol crystals found in a motor oil-like fluid on gross examination -Histological variants *surgical resection *Adjuvant radiotherapy *In the case of hypopituitarism: hormone replacement therapy