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Brain tumor in children

Common brain tumor in children

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WELCOME
TO
SEMINAR PRESENTATION
Dr. Sharmin Nahar
Dr. Olivia Akhter
Resident, Phase-B
Paediatric Haematology & Oncology,
BSMMU
• Purba Sarker, 2 yrs 7 months old immunized girl,
only issue of her non consanguineous parents
,admitted with h/o headache, vomiting and
convulsion for last 1 month. There is also left sided
hemiparesis.
• On physical examination, there is right sided upper
motor type of facial palsy and left sided hemiparesis
present.
• 4th Ventricular SOL having mass effect
resulting moderate obstructive
hydrocephalus.
• Possibilities are-
Ependymoma/Medulloblastoma.
Brain Tumor
INTRODUCTION
• Brain tumors are not a single kind of tumor, but include several different tumor types.
• As a group, these are the most common solid tumors in children less than 15 years of
age, and account for approximately 20% of all cancers.
• Most childhood brain tumors arise from glial cells .
• Most brain tumors tend NOT to “metastasize” or spread to distant areas or` to other
parts of the body outside the brain or spinal cord (CNS).
• The morbidity associated with CNS tumors and their therapy is significant in terms of
physical deficits as well as neuropsychological and neuroendocrine sequelae.
• The long-term morbidity of childhood CNS tumors likely exceeds that associated with
other pediatric malignancies.
INCIDENCE
The incidence ranges from approximately 0.3 to 2.9 cases per 100,000 live births
in different parts of the world.
Sex: The male predominance ( Ratio 1.2: 1)
(primarily explained by a disproportionate incidence of both medulloblastoma and
ependymoma in males. For other tumor types, the genders are equally affected. )
Greater percentage of whites than nonwhites are affected.

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Brain tumor in children

  • 1. WELCOME TO SEMINAR PRESENTATION Dr. Sharmin Nahar Dr. Olivia Akhter Resident, Phase-B Paediatric Haematology & Oncology, BSMMU
  • 2. • Purba Sarker, 2 yrs 7 months old immunized girl, only issue of her non consanguineous parents ,admitted with h/o headache, vomiting and convulsion for last 1 month. There is also left sided hemiparesis. • On physical examination, there is right sided upper motor type of facial palsy and left sided hemiparesis present.
  • 3. • 4th Ventricular SOL having mass effect resulting moderate obstructive hydrocephalus. • Possibilities are- Ependymoma/Medulloblastoma.
  • 5. INTRODUCTION • Brain tumors are not a single kind of tumor, but include several different tumor types. • As a group, these are the most common solid tumors in children less than 15 years of age, and account for approximately 20% of all cancers. • Most childhood brain tumors arise from glial cells . • Most brain tumors tend NOT to “metastasize” or spread to distant areas or` to other parts of the body outside the brain or spinal cord (CNS). • The morbidity associated with CNS tumors and their therapy is significant in terms of physical deficits as well as neuropsychological and neuroendocrine sequelae. • The long-term morbidity of childhood CNS tumors likely exceeds that associated with other pediatric malignancies.
  • 6. INCIDENCE The incidence ranges from approximately 0.3 to 2.9 cases per 100,000 live births in different parts of the world. Sex: The male predominance ( Ratio 1.2: 1) (primarily explained by a disproportionate incidence of both medulloblastoma and ependymoma in males. For other tumor types, the genders are equally affected. ) Greater percentage of whites than nonwhites are affected.
  • 7. Figure : Incidence of common central nervous system tumors in children.
  • 10. Other associated conditions: • Ionizing radiation : In recent decades, brain tumors have been reported after cranial irradiation for acute lymphoblastic leukemia. • Immunosuppression: Wiskott-Aldrich syndrome, Ataxia-telangiectasia. • Familial conditions: The children or siblings of persons with brain tumors may be at higher risk for developing brain tumors themselves. Reports of familial clustering of embryonal tumors, gliomas, and choroid plexus papillomas (CPPs) also exist. • Environmental exposure: Polyomavirus has been reported (e.g., JC and SV-40 viruses) related with certain types of pediatric brain tumors such as medulloblastoma, ependymoma, and choroid plexus tumors. • Other cancers : Hereditery Retinoblastoma is associated with pineoblastoma, glioma,meningioma.
  • 11. Brain Tumor Molecular Biology and Genetics
  • 13. Anatomic Distribution of brain tumor Supratentorial Lesions 1. Cerebral hemisphere: low- and high-grade glioma, ependymoma, meningioma. 2. Sella or chiasm: craniopharyngioma, pituitary adenoma, germ cell tumors, optic nerve glioma. 3. Pineal region: Pineoblastoma, pineocytoma, germ cell tumors Infratentorial Lesions 1.Posterior fossa: medulloblastoma, glioma (low more frequent than high-grade), ependymoma, meningioma. 2. Brain stem tumors: low- and high-grade glioma Ventricular Lesions 1. Choroid plexus papilloma, neurocytoma.
  • 14. Proposed Modification of Revision of the World Health Organization Classification of Brain Tumors in Children ,1985 (Neuroepithelial Tumors Only) • I. Tumors of neuroepithelial • tissue • A. Glial tumors • 1. Astrocytic tumors • (a) Astrocytoma • (b) Anaplastic astrocytoma • 2. Oligodendroglial tumors • (a) Oligodendroglioma • (b) Anaplastic oligodendroglioma • 3. Ependymal tumors • (a) Ependymoma • (b) Anaplastic ependymoma 4. Choroid plexus tumors • (a) Choroid plexus papilloma • (b) Choroid plexus adenoma • (c) Choroid plexus carcinoma • 5. Mixed gliomas • (a) Oligoastrocytoma (b) Ependymoastrocytoma • (c) Oligoastroependymoma • (d) Gliofibroma • 6. Glioblastomatous tumors • (a) Glioblastoma multiforme • (b) Giant-cell glioblastoma • (c) Gliosarcoma • 7.Gliomatosis cerebri
  • 15. • B. Mixed glial-neuronal tumors • 1. Ganglioglioma • 2. Superficial cerebral astrocytoma–desmoplastic infantile ganglioglioma • 3. Pleomorphic xanthoastrocytoma • 4. Subependymal giant-cell tumor (of tuberous sclerosis) • 5. Anaplastic ganglioglioma • C. Neural tumors • 1. Gangliocytoma • 2. Neurocytoma • • D. Embryonal tumors • 1. Primitive neuroectodermal tumor (PNET) • 2. Medulloepithelioma • 3. Atypical teratoid-rhabdoid tumor • E. Pineal cell tumors • 1. Pineocytoma
  • 16. 2016
  • 20. • Symptoms and signs are related to the location, size and growth rate of tumor. • The most common presenting signs are increased ICP and focal neurological deficit. • If localizing signs (seizures, ataxia, visual field defects, cranial neuropathies, or corticospinal tract dysfunction) are predominant in the absence of increased ICP it is more probable that the tumor originates in parenchymal structures (cerebral hemispheres, brain stem, or cerebellum) Clinical features
  • 21. •General Signs and Symptoms of Intracranial Tumors • 1.Headache • 2.Vomitting • 3. Disturbances of gait and balance. • 4. Hemiparesis. • 5. Cranial nerve abnormalities. • 6. Impaired vision • a. Diplopia • b. Papilledema ( from increased ICP may present as intermittent blurred vision) • c. Parinaud syndrome
  • 22. 7. Mental disturbances – somnolence, irritability, personality or behavioral change, or change in school performance. 8. Seizures – usually focal. Tumors are found in 12% to 33% of children who undergo surgery for intractable seizures. 9. Endocrine abnormalities: Midline supratentorial tumors may cause endocrine abnormalities due to effects on the hypothalamus or pituitary 10.Visual field disturbances as a result of optic pathway involvement. 11. Cranial enlargement in infants (characteristic of increased ICP). 12. Diencephalic syndrome
  • 25. 1. Tissue biopsy for histopathology and cytogentics (confirmatory) 2. Primary Imaging: 1. Computed Tomography Scan 2. Magnetic resonance imaging (MRI) : • Superior to CT-Scan. • Contrast MRI should be used. • More preferable in the temporal lobe and posterior fossa.
  • 28. Magnetic Resonance Angiography: Pre-operative evaluation of the normal anatomic vasculature. Magnetic Resonance Spectroscopy: 1. Both diagnosing and follow-up investigations. 2. Distinguish between malignant tumors and areas of necrosis. Positron emission tomography (PET): Used to determine • Metabolic differences between normal and malignant cells. • Appropriate biopsy site in patients with multiple lesions, large homogeneous and heterogeneous lesions. • Recurrent tumor from necrosis, scar and edema in patients who have undergone radiation therapy and chemotherapy. (Astrocytomas and oligodendrogliomas are generally hypometabolic whereas anaplastic astrocytomas and glioblastoma multiforme are hypermetabolic)
  • 29. • Cerebrospinal Fluid Examination • Cell count with slide examination for cytology of tumor cells. • CSF α-fetoprotein (AFP). • CSF human chorionic gonadotropin (β-hCG). • Bone Marrow Aspiration and Bone Scan • These studies are indicated in medulloblastoma and high-grade ependymomas with evidence of cytopenias on the blood count because a small percentage of these patients have systemic metastases at the time of diagnosis.
  • 32. Surgery • The purpose of neurosurgical intervention : • 1. To provide a tissue biopsy for histopathology and cytogenetics. • 2. To attain maximum tumor removal with fewest neurologic sequelae. • 3. To relieve associated increased ICP due to CSF obstruction. • A major limitation of extensive surgical resections is, that aggressive resections may increase the risk of immediate and long-term morbidity, particularly for tumors in functionally critical locations. • The ideal goal is a gross-total resection of tumor (likely leaving microscopic residual) as removal of a margin of normal tissue would cause devastating neurologic sequelae .
  • 33. Preoperative and Perioperative Considerations • Use of pre-operative Dexamethasone can significantly decrease peritumoral edema, thus decreasing focal symptoms and often eliminating the need for emergency surgery. • Patients with increased hydrocephalus, endoscopic or standard ventriculostomy can decrease ICP. • Tumor resection is safer when performed 1–2 days following reduction in edema and ICP by these means.
  • 34. •Postoperative Considerations • A complete or partial resection of a posterior fossa tumor, particularly a tumor in or around the cerebellar vermis, may be complicated by Posterior fossa syndrome, also known as “cerebellar mutism,” • It is characterized by mutism, ataxia, hemiparesis, cognitive impairment, behavioral changes, cranial nerve palsies, bulbar palsy, and tremor. • Occur more commonly with medulloblastoma than with other posterior fossa tumor
  • 35. Radiation Therapy Radiation therapy is a central component of curative as well as palliative therapy for a majority of children with CNS tumors. Indications for Radiation Therapy The indications for radiation therapy depend on tumor histology. Pretreatment histological diagnosis is required except in cases of diffusely infiltrating pontine gliomas and visual pathway gliomas. For ependymomas and many of the astroglial tumors, the use and timing of irradiation also depend on anatomic site of involvement and degree of resection.
  • 36. • Techniques in Radiation Therapy: • Conventional External-Beam Radiation Therapy • Three-Dimensional Conformal Radiation Therapy • Intensity-Modulated Radiation Therapy • Particle Beam Irradiation • Brachytherapy
  • 37. • Radiation Effects on the Brain (a) Acute reactions: • Occurring during treatment • Intra- or perilesional edema • White matter necrosis. (B) Subacute or early delayed reactions: • Occurs a few weeks to 2 months after irradiation • Constitutional symptoms (e.g. Lassitude, low-grade fever , alterations in recent memory) • Diffuse white matter changes. • Resolving within several weeks to a few months. (C)Late reactions: • Occurs several months to years after treatment • Focal radiation necrosis. • Leukoencephalopathy , neuropsychological effects. • Intellectual impairment, memory deficits in younger children. • Secondary neoplasms (both benign and malignant). • Late effects can be progressive, irreversible, and sometimes fatal
  • 38. Principles of Chemotherapy Tumors of the CNS may fail to respond to standard-dose chemotherapy as a result of limited drug exposure in the tumor tissue or drug resistance. • (a) Agents that have better penetration to BBB. Factors that influence CNS tumor penetration of an agent across the BBB include : • Physiochemical properties of the agent • The degree of protein binding • The affinity of the agent for carriers that facilitate transport of endogenous compounds into the CNS • (b) Administration of very high-dose systemic chemotherapy • (c) Regional chemotherapy approaches (e.g., intrathecal therapy, intra-arterial therapy, intratumoral therapy ).
  • 39. Differentiating Agent • Inhibitors of Histone Deacetylase (phenylbutyrate, valproic acid, and suberoylanilide hydroxamic acid (SAHA). Antiangiogenic therapy: • Bevacizumab ,SU5416 a small molecule inhibitor of vascular endothelial growth factor (VEGF),lenalidomide,cediranib. Immunotherapy • Cytokines (interleukins and interferons), Cancer vaccines. Gene Transfer • Herpes simplex virus thymidine kinase type 1 (HSV-Tk1) gene Novel Agents
  • 41. Astrocytoma • It is a type glial tumor arises from astrocytes. • Peaks between ages 5-6 and 12-13 years. • Astrocytomas are divided into 4 grades (depending on their cells' appearance under a microscope; the higher a tumor's grade number, the more severe it is) • Low-grade tumors (WHO I and II) • Grade I tumors–Pilocytic astrocytoma, Subependymal giant cell tumor. • Grade II tumors–Diffuse astrocytoma, Pleomorphic xanthoastrocytoma. • High-grade astrocytomas (WHO III and IV) • Grade III tumors–Anaplastic astrocytoma or anaplastic pleomorphic xanthoastrocytoma. • Grade IV tumors–Glioblastoma or Diffuse midline glioma.
  • 42. • Cerebellar astrocytomas are most prevalent • (15% to 25% )  Clinical features of astrocytomas depends on the site of CNS involved
  • 44. Microscopically, the tumor has bipolar cells with long “hairlike” (hence the name “pilocytic” from the Latin pilus, or hair) projections that form a fibrillary mesh.
  • 45. Management of Astrocytomas • Low-Grade Astrocytomas (WHO Grades I and II) • Surgical excision is the initial treatment. •Surgery : • Gross total resection is desirable. • Pilocytic astrocytomas - GTR in 90% of patients with posterior fossa tumors and a majority with hemispheric tumors. • Diffuse low-grade astrocytomas - less often completely resected. • Diencephalic tumors - GTR in less than 40% of cases. • If removal is complete, no further treatment is recommended.
  • 46. • Radiotherapy: • The role of radiation therapy in patients with low-grade gliomas depends on the anatomic location and tumor extent, age of the child, and the degree of resection • The ability to re-resect hemispheric lesions usually allows postponement of adjuvant therapy. • Radiotherapy is used when chemotherapy has failed in unresectable symptomatic tumors, especially in older patients. • Dosing is 5,000–5,500 cGy, depending on age, to the original tumor bed with a 2 cm margin.
  • 47. •Chemotherapy: • Younger than 5 years of age • Carboplatin and vincristine - • Induction--- weekly---12weeks • Maintenance ---6weeks---8 cycle • 5-year event-free survival (EFS) is 39 +/- 4% • Older than 5 years of age • Thioguanine ,Procarbazine, Lomustine ,Vincristine 6-week --8cycles 5-year event-free survival (EFS) is 52 +/- 5%
  • 48. •Prognosis: • The 5- and 10-year overall survival rates : Completely resected and treated with surgery alone: • Low-grade supratentorial astrocytomas are 76–100% and 69–100%, respectively. • In the posterior fossa these rates approach 100%. Partially resected , who are observed without treatment or who are treated with postoperative radiotherapy • 67–87% and 67–94%, respectively. • Recurrence: • Recurrent astrocytomas should be approached surgically when possible. If not completely resectable, chemotherapy and/or radiation should be given, depending on prior therapy received.
  • 49. High-Grade Astrocytomas (WHO Grades III and IV) • Surgery: • The main purpose is to reduce the tumor burden. • Complete surgical removal is rarely accomplished because of their infiltrative nature. • Patients who have total or near-total resection have longer survival than the patients who have partial resection. • Radiotherapy: • Postoperative irradiation increases survival in high-grade astrocytomas. • The dose is 5,000– 6,000 cGy in children over 5 years of age.
  • 50. Chemotherapy: • Surgery+ RT+Chemotherapy > Surgery+ RT • • Procarbazine (or prednisone) • Lomustine Every 6 weeks for 8 cycles • Vincristine • But without a reasonable resection, chemotherapy is ineffective. • Progression-free survival is approximately 33% at 5 years. Other studies been reported the use of Temozolamide. The addition of lomustine to temozolomide as adjuvant therapy was associated with significantly improved outcome compared with temozolomide alone.
  • 52. Medulloblastoma • Medulloblastoma is the most common childhood brain tumor , presenting about 20% of all childhood brain tumors • It is an embryonal tumor that occurs in the posterior fossa . • 80% of the cases presents before the age of 15 year. • Presentation - Vomiting and headache (80% of patients) & usually associated with obstruction of cerebrospinal CSF flow at the outlet of the third or fourth ventricle and secondary hydrocephalus.
  • 53. • Histopathologic sub-classification • Classic • Desmoplastic/nodular • Extensive nodularity • Large cell/anaplastic Patients with nodular tumors tend to have good outcomes, while those with large cell/anaplastic tumors have poorer outcomes.
  • 55. Neuroimaging Fig: Medulloblastoma MRI.A .showing T2w image of a heterogenous mixed cystic and solid mass extending from cerebellar vermis. B.Post contrast image showing enhancement of solid portion of the tumor
  • 56. Staging and Risk Stratification • Chang staging system for Medulloblastoma
  • 58. Treatment •Surgery: • Surgical excision is employed as the initial therapy with an objective of gross total resection or near total resection with less than 1.5 cm2 of residual tumor. • Disease-free survival improves for patients who have had a radical resection of the tumor.
  • 59. •Radiotherapy: • The standard dose of radiotherapy is 5,400–5,580 cGy to the area of the primary tumor, with 2,340 cGy given to the neuroaxis in average-risk medulloblastoma and 3600 cGy with high-risk disease. • • Use of intensity modulated radiation therapy (IMRT) to spare the cochlea is important in decreasing therapy-induced hearing loss. • Radiation is considered to be either omitted or postponed in this young age group.
  • 60. Chemotherapy: Average-risk disease • Surgery + radiotherapy (> 80% 5-year disease free survival). • The Children’s Oncology Group study utilizing craniospinal irradiation following surgery and adjuvant chemotherapy Lomustine Vincristine Every 6 weeks for 8 cycles Cisplatinum 5 year PFS rate is 89%
  • 61. High-risk disease: Surgery + Radiation + Adjuvant Chemotherapy For children with high-risk disease, primarily those with disseminated disease at the time of diagnosis, chemotherapy has been utilized prior to, during, and after radiotherapy SIOP trial, the use of preradiation chemotherapy (Vincristine, Etoposide, carboplatin and cyclophosphamide) resulted in OS rates at 3 and 5 years were 50.0% and 43.9% . Children's Cancer group , randomized phase III study of pre and post RT, VCP (VCR, lomustine, prednisone) over 8-in-1 therapy (VCR, methylprednisolone, lomustine , hydroxyurea, procarbazine , cisplatin, cyclophosphamide, and cytarabine). Five- year estimates of PFS were 63% ± 5% compared with 45% ± 5% for VCP and 8-in-1 chemotherapy, respectively. (The Seventh Edition of Principles and Practice of Pediatric Oncology (Philip A.pizzo, David G poplack), page 2785,286.)
  • 62. Molecular therapy : • Identification of activating pathways in some subgroups has provided an opportunity for the development of small molecule inhibitors as molecular- targeted therapy . • Ex: Vismodegib, an SMO inhibitor that inhibits the SHH pathway Relapse: • Studies have shown some improvement of survival in relapsed patients using high-dose carboplatin, thiotepa, and etoposide followed by autologous stem cell rescue , with salvage rates up to 30%.
  • 63. Presentation content • Ependymoma • Craniopharyngioma • Brain Stem Glioma • Optic Glioma • Intracranial Germ Cell Tumour.
  • 66. Demography • Ependymomas, which constitute approximately 10% of all primary CNS tumors in children. • Ninety percent of the tumors are intracranial, and up to two-thirds of these occur in the posterior fossa. • The highest incidence of ependymoma in children occurs in the first 7 years of life, with a second peak in the third to fifth decades of life. • Male-to female ratio of between 1.3 and 2.0.
  • 67. Classified into 4 groups by the WHO classification of tumours : • Myxopapillary ependyoma (Grade 1) • Subependymomas (Grade 1) • Ependymoma (Grade 2) • Anaplastic ependymoma (Grade 3)
  • 69. Imaging • CT scans of supratentorial ependymomas tumors reveal a heterogeneous mass with calcification and cyst formation.
  • 70. MRI • MRI is the diagnostic modality of choice in the workup and follow-up observation of intracranial neoplasms, including ependymoma. • MRI exploits the use of increased water content of many neoplasms. This water concentration shows up as increased signal on T2 weighted images and decreased signal on T1 images • MRI is used to monitor ongoing treatment and to search for recurrence. • Final diagnosis is achieved through biopsy with histopathologic analysis
  • 75. Pathology • A key histological feature is the perivascular pseudorosette, which is characterized by tumor cell processes converging on vessels, creating a perivascular fibrillary zone.
  • 76. Patterns of Spread • Ependymomas are locally invasive tumors. • Tumors arising in the posterior fossa frequently infiltrate the brainstem. • The incidence of spinal subarachnoid dissemination has been estimated to be 7% to 12%. • Systemic metastases are rare .
  • 77. Treatment Surgery: • Total removal of these tumors is difficult to accomplish, especially in tumors originating from the fourth ventricle. • Gross total tumor resection predicts a greatly improved outcome. • Patients with a subtotal resection should receive two courses of chemotherapy before potential second-look surgery.
  • 78. Radiotherapy: • The recurrence rate with surgery alone is extremely high • Postoperative radiotherapy results in a significant increase in survival. • Local disease without evidence of subarachnoid spread should receive local irradiation with a margin.
  • 79. Subependymoma : A gross total resection and observation. Myxopapillary ependymoma : A gross total resection with or without radiation. Ependymoma : After a gross total resection of ependydoma, limited field fractionated external beam radiotherapy(LFFEBRT) can be considered. Post operative LFFEBRT is recommended for subtotal resection of ependydoma
  • 80. Anaplastic ependymoma : After a gross total or subtotal resection postoperative LFFEBRT is recommended. • After the patient recovers from surgery, daily out patient radiotherapy should begin. • This is generally given for approximately 5-8 weeks. • Usual dose is 5400-6000 cGY per day, 5 days in a week.
  • 81. Chemotherapy: • Single- and multiagent chemotherapeutic regimens have been used in ependymoma therapy. • Infants with localized ependymoma who had a GTR followed by a carboplatin-based chemotherapy regiment had a 5-year progression-free survival of 57 ± 17%. • In infants, chemotherapy is used to postpone radiotherapy.
  • 82. • There is some evidence that low dose metronomic chemotherapy (i.e. oral etoposide at 50 mg/m2/day for 21 or 28 days) can be effective in treating relapsed disease. • Optimal length of therapy is not yet determined.
  • 83. Prognosis • The overall prognosis for ependymomas is dependent on the initial extent of resection, presence or absence of anaplasia and gender. • Patients with total resection who receive radiation have a 5-year survival of 67–86% compared to 22–47% 5-year event-free survival for patients who receive radiation after subtotal resection. • Patients with anaplasia have significantly worse event-free survival.
  • 85. • Craniopharyngiomas account for between 6% and 9% of all primary CNS tumors in children. • These lesions exhibit a bimodal age distribution, with one peak during childhood at approximately 8 to 10 years of age and a second peak in middle age. • This tumor rarely occurs in infants. • No gender predilection. Craniopharyngiomas
  • 86. Site: • Pituitary stalk and hypothalamus • Sella turcica or third ventricle
  • 87. Pathology and Patterns of Spread • Craniopharyngiomas in children arise predominantly from pharyngeal cell rests left from the embryonic hypophyseal-pharyngeal duct . • Grossly, these tumors are, lobulated masses with both solid and cystic components. • The cyst contents may range from gelatinous to viscous oily fluid rich in cholesterol crystals.
  • 88. • Rupture of a cyst into the CSF may cause an intense chemical meningitis. • Calcification is frequently apparent. • Craniopharyngioma is a histologically benign tumor composed of well-differentiated tissue • It may have a malignant clinical course because of its location and its propensity to infiltrate surrounding normal structures.
  • 89. Clinical Presentation • Short stature • Symptoms of increased ICP • Delayed puberty • Vision loss, and neurobehavioral abnormalities • Hydrocephalus (50%) • Because of slow tumor growth, papilledema is less common than optic pallor. • Visual field defects -homonymous hemianopsia and bitemporal hemianopsia being the most frequent defects encountered.(80 to 90%) • Visual deterioration(25% ) • Diabetes insipidus ( fewer than 10% of children )
  • 90. Evaluation • The CT scan characteristically demonstrates a partially cystic, low-density, contrast- enhancing lesion with calcification. • MRI defines the solid and cystic nature of the tumor, its extent, and its relation to adjacent structures . • CT and MRI, often show an enlarged or distorted sella with suprasellar tumor calcification.
  • 92. Treatment Surgery: • Craniopharyngiomas should be completely removed if possible. • Complete excision is possible in 60–90% of cases. • • However, controversy exists over whether subtotal resection followed by radiation will produce less morbidity and be an equally effective strategy. • Complete tumor removal is most easily accomplished in cystic tumors and is most difficult in solid or mixed tumors larger than 3 cm in size.
  • 93. Radiotherapy: • In tumors in which conservative surgery consisting only of drainage or subtotal removal is performed, the addition of radiotherapy reduces the local recurrence rate and improves long-term survival. • For children older than 5 years, 5,000–5,500 cGy are given. • In children less than 5 years of age the dose may be reduced to 4,000–4,500 cGy. • For patients with complete resections, radiation may be reserved for those who relapse.
  • 94. Chemotherapy: • Chemotherapy has no established role in the treatment of craniopharyngiomas. • An anecdotal response to a vincristine, BCNU, and procarbazine combination has been described in one patient. • Intracystic administration of bleomycin has also led to response and significant second remissions in some patients with recurrent disease but concerns have been raised regarding local toxicity. • Interferon-alpha 2a systemically shrank the lesion in 25% of patients treated, primarily those with cystic lesions.
  • 95. Prognostic Considerations • Patients with totally excised tumors have had considerably better survival rates. • Several studies have shown that the combination of subtotal resection and radiation therapy achieves survival results that rival those obtained with attempted GTR • Tumor size probably is not an independent variable but rather is related to the ease and extent of resection. • • Patients with purely cystic lesions appear to survive longer than those with solid or mixed solid and cystic tumors. • Children older than 5 years seem to have a better prognosis than do younger patients.
  • 96. Prognosis: • The long-term survival of patients treated with radical and total removal is 80– 90% at 5 years and 81% at 10 years. • The 5-year recurrence-free survival after subtotal removal is approximately 50%. • Survival after partial removal and radiation therapy is 62–84%.
  • 98. Demography • Brain stem gliomas comprise 15–20% of all childhood CNS tumors. • The median age at presentation is 6–7 years of age. • The male-to-female ratio is near unity. • Brainstem tumors are noted to be increasingly frequent among patients with NF1.
  • 99. Classification Categorized by location Tectal glioma focal midbrain tumor Focal intrinsic pontine glioma doral/exophytic glioma diffuse intrinsic pontine glioma Focal medullary glioma cervicomedullary glioma
  • 100. Categorized by pathology Low-grade- Focal, Diffuse, Fibrillary-type, (WHO grade I,II ) High-grade - Anaplastic astrocytoma (WHO grade III) Glioblastoma multiforme (WHO grade IV).
  • 101. Biology • There was differential expression of IL-13Rα2 protein in brainstem tumors versus normal brain. This suggests that the IL-13Rα2 receptor is a potential therapeutic target.
  • 102. Presentation • Upper brainstem tumors tend to present with cerebellar findings and hydrocephalus . • Lower brainstem tumors tend to present with multiple lower cranial nerve deficits and long tract finding.
  • 103. Signs and symptoms : • Gait disturbance • Headache • Nausea/vomiting • Cranial nerve deficits: diplopia, facial asymmetry • Distal motor weakness in 30% . • Papilledema in 50% • Hydrocephalus in 60%, usually due to aqueductal obstruction • Failure to thrive (especially in age ≤ 2 yrs)
  • 104. Evaluation MRI • The diagnostic test of choice. • MRI evaluates status of ventricles, gives optimal assessment of tumor (CT is poor in the posterior fossa) and detects exophytic component. • T1WI: almost all are hypointense, homogeneous (excluding cysts). • T2WI: increased signal, homogeneous (excluding cysts). Gadolinium enhancement is highly variable
  • 108. Treatment • The choice of treatment depends largely on whether the tumor is a diffusely infiltrative brainstem glioma or a focal brainstem tumor. • Even among focal lesions, the site of the tumor affects selection of therapy.
  • 109. • For focal tumors (nontectal), complete resection may be safe and may not require any further therapy. • Partial resection of exophytic tumors will establish the diagnosis and reduce the obstructing mass within the fourth ventricle. • If hydrocephalus is present, a CSF diversion should be performed.
  • 110. Surgery • Surgical resection is not usually possible because of the proximity to vital structures, limited room for expansion and swelling and possible damage to medullary structures. • There is no apparent benefit from a surgical biopsy when the imaging and clinical picture are indicative of a diffuse infiltrating pontine glioma.
  • 111. Radiotherapy • Limited field irradiation is standard palliative care in patients with infiltrative pontine gliomas. • A tumor dose of approximately 5,400 cGy is standard. • The treatment field should include the extent of the defined tumor and a 2-cm margin around the tumor. • In irradiating these tumors, precaution should be taken to minimize brain stem edema, especially in patients who have not been shunted.
  • 112. • The use of high-dose steroids is valuable during treatment and may be required throughout the treatment period. • Children with diffusely infiltrating pontine gliomas often initially respond to radiation therapy but progressive disease is usually seen within 8–12 months. • Timing of radiation for partially resected focal lesions is unclear. • It is not known whether immediate adjuvant therapy is superior to observation and therapy only at the time of progression.
  • 113. Chemotherapy • The use of combination chemotherapy after radiotherapy has not improved the disease-free survival in brain stem tumors. • With focal brainstem gliomas, weekly carboplatin with vincristine has shown activity in a very limited number of patients younger than 5 years. • Recent trials have evaluated the addition of a diverse array of molecularly targeted agents (e.g., imatinib and gefitinib)or antiangiogenic agents (e.g., thalidomide,bevacizumab)) for children with newly diagnosed brainstem gliomas.
  • 114. Prognosis • Prognosis depends principally on the tumor type . • Patients with diffusely infiltrative gliomas fare dismally, regardless of therapy. In most series, median survival is less than 1 year, and survival rates at 2 years are lower than 10% to 20%. • In contrast, the prognosis for patients with focal brainstem tumors is relatively good if the tumor is accessible. Survival for these patients is reported to be between 50% and 100%.
  • 115. • Among patients with NF1, brainstem gliomas, whether diffuse or focal, display a generally indolent biologic behavior. • The tumor may stabilize in size or regress without intervention and survival approximates 90% at 5 years.
  • 117. Demography • Optic pathway tumors (OPTs) are low-grade glial tumors. • OPTs generally arise in the optic nerves, chiasm, and hypothalamus. • They comprise approximately 3% to 5% of pediatric intracranial tumors. • Nearly two thirds of OPTs are diagnosed in the first 5 years of life. • Seventy-five percent of OPTs will become symptomatic in the first decade of life, and 90% will become so before the age of 20.
  • 118. • Boys and girls are affected equally. • OPTs are prevalent in patients with NF1. • Unilateral or bilateral optic nerve involvement alone is seen almost exclusively in patients with NF1 • chiasmal involvement is significantly more common in patients without NF1.
  • 119. Pathology and Patterns of Spread • Histologically, OPTs are usually low-grade astrocytoma; the majority are pilocytic . • Although these tumors usually are confined to the structures of the visual pathways and extending contiguity along them , they may extend also into the frontal lobes, hypothalamus, thalamus, and other midline structures. • Tumor growth is slow • Malignant degeneration is rare.
  • 120. Biology • As for low-grade gliomas occurring elsewhere in the CNS, activation of the RAS/RAF (MAPK) signaling cascade has been implicated as a critical event in the pathogenesis of both sporadic and NF1-associated optic pathway gliomas.
  • 121. Prognostic Considerations • The three factors that appear to have prognostic importance for the outcome of patients with OPTs include NF1 status, tumor location, and age. • The presence of NF1 generally is associated with more indolent disease, reflected in longer times to disease progression and higher rates of PFS and survival. • Tumors involving the chiasm and hypothalamus have a worse prognosis in most (but not all) • Youngest children, generally younger than 3 to 5 years, do worse than do their older counterparts.
  • 122. Clinical Features • Patients may present with decreased vision, proptosis, optic atrophy and papilledema. • In young children asymmetric nystagmus may be the presenting sign of a chiasmatic tumor. • Tumors that extend beyond the optic pathway may be associated with hypothalamic dysfunction.
  • 123. Treatment Surgery: • Biopsy may compromise vision • Surgery should be reserved for tumor extension into the optic canal or increasing visual compromise.
  • 124. Radiotherapy: • The role of radiotherapy in optic gliomas has not been completely established but it may have a beneficial role in preserving vision. • Radiation may be indicated in patients with progressive disease instead of surgery, especially for chiasmatic–hypothalamic lesions. • Patients with NF-1 may have exacerbation of moya moya, which one must consider before recommending radiation. • A local field to the tumor of 4,500–5,000 cGy over 5–6 weeks is usually recommended.
  • 125. Recommendations • Since optic glioma can run an indolent course, the decision for therapeutic intervention should be based on radiographic findings and visual assessment of visual acuity, visual fields and visual-evoked response.
  • 126. The following treatment plan is recommended: Evidence of optic nerve tumor and normal visual assessment: • No therapeutic intervention is recommended. • CT/MRI scan and visual assessments are performed every 6 months to monitor progression of disease. • Evidence of progression on visual assessment, with or without tumor extending posteriorly into the optic canal: A trial of chemotherapy is recommended in an attempt to preserve useful vision.
  • 128. Prognosis • Progression-free survival rates with carboplatin and vincristine are 68% at 3 years. Actinomycin D and vincristine ,4 year PFS is 62.5% but 7 years PFS is only about 33%.
  • 130. Demography • Primary intracranial germ cell tumors (GCT) comprise 1–3% of primary pediatric brain tumors. • The peak age is between 10 and 21 years of age.
  • 131. Multiple tumor types can be seen: 1.Germinomas 2.Teratomas and mixed germ cell tumors 3.Malignant endodermal sinus tumors 4. Embryonal cell carcinomas, 5. Choriocarcinomas and teratocarcinomas
  • 132. • In all but the germinomas, serum and cerebrospinal fluid alpha-fetoprotein and βHCG may be elevated. • MRI of the spine with Gd-DTPA should be performed, as leptomeningeal spread is relatively common. • The outcome of patients with pure germinoma is considerably better than with mixed germ cell tumors.
  • 133. Surgery: • Surgical biopsy is indicated in all germ cell tumors to make an appropriate diagnosis unless elevated serum or CSF tumor markers establish the diagnosis. • For patients with benign tumors such as teratomas or dermoids, surgery can be curative.
  • 134. Radiotherapy • Conventional radiotherapy for a CNS germinoma includes doses to the primary tumor of 5,000 cGy with 3,000 cGy craniospinal therapy. • Nongerminoma germ cell tumors (NGGCT) respond poorly to radiation therapy but the use of chemotherapy with radiation appears to improve survival substantially in preliminary studies.
  • 135. Chemotherapy In both germinomas and nongerminoma germ cell tumors: • Cycles of cisplatin 20 mg/m2/day IV days 1–5 with etoposide 100 mg/m2/day IV days 1–5 alone • OR Alternating with cyclophosphamide 1 g/m2/day for 2 days with vincristine 1.5 mg/m2/day weekly for 3 days has been used.
  • 136. • For high-risk disease doses of cisplatin and cyclophosphamide have been doubled or ifosfamide has been added to the cisplatin/etoposide above at 1.5 g/m2/day for days 1–5. • These approaches are generally combined with graded doses of radiation depending on initial risk and response.
  • 137. Prognosis • The germinomas have the best overall survival rate followed by teratomas and pineal parenchymal tumors. • Nongerminoma germ cell tumors such as embryonal carcinoma, endodermal and choriocarcinoma have a worse survival. • However, preliminary data from combined chemotherapy/radiation approaches demonstrate encouraging data indicating that survival may now approach 60–80%.