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Childhood visual pathway and hypothalamic glioma

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General Information
Cellular Classification
Stage Information
Treatment Option Overview
Untreated Childhood Visual Pathway And Hypothalamic Glioma
Recurrent Childhood Visual Pathway And Hypothalamic Glioma

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Information from PDQ -- for Health Professionals

GENERAL INFORMATION

This treatment information summary on childhood visual pathway and hypothalamic glioma is an overview of diagnosis, classification, patient treatment, and prognosis. The National Cancer Institute created the PDQ database to increase the availability of new treatment information and its use in treating patients. Information and references from the most recently published literature are included after review by pediatric oncology specialists.

Primary brain tumors are a diverse group of diseases that together constitute the most common solid tumor of childhood. Brain tumors are classified according to histology, but tumor location and extent of spread are important factors that affect treatment and prognosis. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of mitotic activity are increasingly used in the tumor diagnosis and classification.

Approximately 50% of brain tumors in children are infratentorial, with three fourths of these located in the cerebellum or fourth ventricle. Common infratentorial (posterior fossa) tumors include the following:

1. cerebellar astrocytoma (usually pilocytic but also fibrillary and
high-grade)

2. medulloblastoma (primitive neuroectodermal tumor)

3. ependymoma (low-grade or anaplastic)

4. brain stem glioma (often diagnosed neuroradiographically without biopsy;
may be high-grade or low-grade)

5. atypical teratoid

Supratentorial tumors include those tumors that occur in the sellar or suprasellar region and/or other areas of the cerebrum. Sellar/suprasellar tumors comprise approximately 20% of childhood brain tumors and include the following:

1. craniopharyngioma

2. diencephalic (chiasm, hypothalamic, and/or thalamic) gliomas generally of
low grade

3. germ cell tumors (germinoma or nongerminomatous)

Other tumors that occur supratentorially include the following:

1. low-grade astrocytoma or glioma (grade 1 or grade 2)

2. high-grade or malignant astrocytoma (anaplastic astrocytomas, glioblastomas
multiforme (grade 3 or grade 4))

3. mixed glioma (low-grade or high-grade)

4. oligodendroglioma (low-grade or high-grade)

5. primitive neuroectodermal tumor (cerebral neuroblastoma)

6. ependymoma (low-grade or anaplastic)

7. meningioma

8. choroid plexus tumors (papilloma and carcinoma)

9. pineal parenchymal tumors (pineoblastoma, pineocytoma, or mixed pineal
parenchymal tumor)

10. neuronal and mixed neuronal glial tumor (ganglioglioma, desmoplastic
infantile ganglioglioma, dysembryoplastic neuroepithelial tumor)

11. metastasis (rare) from extra neural malignancies

Important general concepts that should be understood by those caring for a child who has a brain tumor include the following:

1. Selection of an appropriate therapy can only occur if the correct diagnosis
is made and the stage of the disease is accurately determined.

2. Children with primary brain tumors represent a major therapy challenge that,
for optimal results, requires the coordinated efforts of pediatric specialists in fields such as neurosurgery, neurology, rehabilitation, neuropathology, radiation oncology, medical oncology, neuroradiology, endocrinology, and psychology, who have special expertise in the care of patients with these diseases.[1-3]

3. More than one half of children diagnosed with brain tumors will survive 5
years from diagnosis. In some subgroups of patients, an even higher rate of survival and cure is possible. Each child's treatment should be approached with curative intent, and the possible long-term sequelae of the disease and its treatment should be considered before therapy is begun.

4. For the majority of childhood brain tumors, the optimal treatment regimen
has not been determined. Children who have brain tumors should be considered for enrollment in clinical trials when an appropriate study is available. Such clinical trials are being carried out by institutions and cooperative groups.

5. Guidelines for pediatric cancer centers and their role in the treatment of
pediatric patients with cancer have been outlined by the American Academy of Pediatrics.[4]

6. The cause of the vast majority of childhood brain tumors remains
unknown.[5,6]

This summary discusses the treatment of visual pathway and hypothalamic gliomas.

Information about ongoing clinical trials is available from the NCI (Http: //cancer.gov/clinical_trials/).

References:

  1. Heideman RL, Packer RJ, Albright LA, et al.: Tumors of the central nervous system. In: Pizzo PA, Poplack DG, eds.: Principles and Practice of Pediatric Oncology. Philadelphia, Pa: Lippincott-Raven, 3rd ed., 1997, pp 633-697.
  2. Pollack IF: Brain tumors in children. New England Journal of Medicine 331(22): 1500-1507, 1994.
  3. Cohen ME, Duffman PK, eds: Brain Tumors in Children: Principles of Diagnosis and Treatment, 2nd ed. New York: Raven Press, 1994.
  4. Sanders J, Glader B, Cairo M, et al.: Guidelines for the pediatric cancer center and role of such centers in diagnosis and treatment. American Academy of Pediatrics Section Statement Section on Hematology/Oncology. Pediatrics 99(1): 139-141, 1997.
  5. Kuijten RR, Bunin GR: Risk factors for childhood brain tumors. Cancer Epidemiology, Biomarkers and Prevention 2(3): 277-288, 1993.
  6. Kuijten RR, Strom SS, Rorke LB, et al.: Family history of cancer and seizures in young children with brain tumors: a report from the Childrens Cancer Group (United States and Canada). Cancer Causes and Control 4(5): 455-464, 1993.

CELLULAR CLASSIFICATION

Childhood visual pathway and hypothalamic gliomas are usually low-grade (grades 1 and 2) astrocytomas. Both pilocytic astrocytomas and fibrillary astrocytomas may occur. Malignant gliomas of the visual pathway are rare. Visual pathway gliomas occur at an increased incidence in patients with neurofibromatosis type I; approximately 20% of all patients with neurofibromatosis type I will develop a visual pathway glioma. In these patients, the tumor may be found on screening evaluations when the child is asymptomatic or has apparent static neurologic and/or visual deficits. Pathologic confirmation is frequently not obtained in asymptomatic patients, and when biopsies have been performed, these tumors have been found to be predominantly pilocytic (grade 1) rather than fibrillary (grade 2) astrocytomas.[1-4]

Visual pathway and hypothalamic gliomas include astrocytic tumors of the optic nerve, optic chiasm, and optic tract. Due to the infiltrative nature of such lesions, separation of visual pathway and hypothalamic gliomas from thalamic tumors is often difficult and arbitrary.

References:

  1. Kleihues P, Cavenee WK, eds.: Pathology and Genetics of Tumours of the Nervous System. Lyon, France: International Agency for Research on Cancer, 2000.
  2. Listernick R, Darling C, Greenwald M, et al.: Optic pathway tumors in children: the effect of neurofibromatosis type 1 on clinical manifestations and natural history. Journal of Pediatrics 127(5): 718-722, 1995.
  3. Burger PC, Scheithauer BW: Tumors of the central nervous system. Washington DC: Armed Forces Institute of Pathology. 1994.
  4. Allen JC: Initial management of children with hypothalamic and thalamic tumors and the modifying role of neurofibromatosis-1. Pediatric Neurosurgery 32(3): 154-162, 2000.

STAGE INFORMATION

There is no universally accepted staging system for visual pathway and hypothalamic gliomas. They are low-grade astrocytomas that grow slowly and may occur anywhere along the optic tracts. Visual pathway gliomas are found with increased frequency in patients with neurofibromatosis. The major clinical symptoms are visual. In infants and young children, hypothalamic gliomas may result in the diencephalic syndrome, which is manifested by failure to thrive in an emaciated, seemingly euphoric child. Such children may have little in the way of other neurologic findings, but can have macrocephaly, intermittent lethargy, and visual impairment.[1] Tumors may also arise in the hypothalamus; in large infiltrating lesions, distinctions between optic and hypothalamic tumors are often artificial and of little clinical importance. In infants and young children, hypothalamic gliomas may result in the diencephalic syndrome, which is manifested by failure to thrive in an emaciated, seemingly euphoric child. Such children may have little in the way of other neurologic findings, but can have macrocephaly, intermittent lethargy, and visual impairment.[1] Because the location of these tumors makes a surgical approach difficult, biopsies are not always done. This is especially true in patients with neurofibromatosis type I.[2] Visual pathway and hypothalamic gliomas usually spread contiguously, although subarachnoid dissemination has been reported.[1,3] Evaluation should include neuro-ophthalmological testing to carefully monitor the patient for the visual effects of tumor progression that may not be evident on computed tomography or magnetic resonance imaging. Visual-evoked responses have not, as yet, been shown to be more sensitive than clinical examination in patients with visual pathway gliomas, even in young children.

References:

  1. Perilongo G, Carollo C, Salviati L, et al.: Diencephalic syndrome and disseminated juvenile pilocytic astrocytomas of the hypothalamic-optic chiasm region. Cancer 80(1): 142-146, 1997.
  2. Pollack IF, Mulvihill JJ: Special issues in the management of gliomas in children with neurofibromatosis 1. Journal of Neuro-Oncology 28(2-3), 257-268, 1996.
  3. Mamelak AN, Prados MD, Obana WG, et al.: Treatment options and prognosis for multicentric juvenile pilocytic astrocytoma. Journal of Neurosurgery 81(1): 24-30, 1994.

TREATMENT OPTION OVERVIEW

Many of the improvements in survival in childhood cancer have been made as a result of clinical trials that have attempted to improve on the best available, accepted therapy. Clinical trials in pediatrics are designed to compare new therapy with therapy that is currently accepted as standard. This comparison may be done in a randomized study of two treatment arms or by evaluating a single new treatment and comparing the results with those previously obtained with existing therapy.

Because of the relative rarity of cancer in children, all patients with brain tumors should be considered for entry into a clinical trial. To determine and implement optimum treatment, treatment planning by a multidisciplinary team of cancer specialists who have experience treating childhood brain tumors is required. Radiation therapy of pediatric brain tumors is technically very demanding and should be carried out in centers with experience in that area in order to ensure optimal results.

Debilitating effects on growth and neurologic development have frequently been observed following radiation therapy, especially in younger children.[1-3] There are also other less common complications of radiation therapy including cerebrovascular accidents.[4] For this reason, the role of chemotherapy in allowing a delay in the administration of radiation therapy is under study, and preliminary results suggest that chemotherapy can be used to delay, and sometimes obviate, the need for radiation therapy in children with benign and malignant lesions.[5] Long-term management of these patients is complex and requires a multidisciplinary approach.

The designations in PDQ that treatments are "standard" or "under clinical evaluation" are not to be used as a basis for reimbursement determinations.

References:

  1. Packer RJ, Sutton LN, Atkins TE, et al.: A prospective study of cognitive function in children receiving whole-brain radiotherapy and chemotherapy: 2-year results. Journal of Neurosurgery 70(5): 707-713, 1989.
  2. Johnson DL, McCabe MA, Nicholson HS, et al.: Quality of long-term survival in young children with medulloblastoma. Journal of Neurosurgery 80(6): 1004-1010, 1994.
  3. Packer RJ, Sutton LN, Goldwein JW, et al.: Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma. Journal of Neurosurgery 74(3): 433-440, 1991.
  4. Bowers DC, Mulne AF, Reisch JS, et al.: Nonperioperative strokes in children with central nervous system tumors. Cancer 94(4): 1094-1101, 2002.
  5. Duffner PK, Horowitz ME, Krischer JP, et al.: Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors. New England Journal of Medicine 328(24): 1725-1731, 1993.

UNTREATED CHILDHOOD VISUAL PATHWAY AND HYPOTHALAMIC GLIOMA

Treatment options should be considered not only to improve survival but also to stabilize visual function. Children with isolated optic nerve tumors have a better prognosis than those with lesions that involve the chiasm or that extend along the visual pathway.[1-3] Children with neurofibromatosis also have a better prognosis, especially when the tumor is found in asymptomatic patients at the time of screening.[1] Observation is an option for patients with neurofibromatosis or nonprogressive masses.[1,2,4] Spontaneous regressions have been reported.[5] For children with isolated optic nerve lesions and progressive symptoms, complete surgical resection or local radiation therapy may result in prolonged progression-free survival.[1]

Radiation therapy results in long-term disease control for the majority of children with chiasmatic and posterior pathway chiasmatic gliomas but may also result in substantial intellectual and endocrinologic sequelae and possibly in an increased risk of secondary tumors.[1,6] An alternative to immediate radiation therapy is subtotal surgical resection, but it is unclear how many patients will have stable disease and for how long.[1]

Chemotherapy may result in objective tumor shrinkage and will delay the need for radiation therapy in the majority of patients. Chemotherapy may be particularly appropriate for patients with neurofibromatosis and for infants in whom the sequelae of radiation therapy are most likely. Chemotherapy has been shown to shrink tumors in children with hypothalamic gliomas and the diencephalic syndrome, resulting in weight gain in those who respond to treatment.[7] However, it is unclear how long such a response will endure and whether radiation therapy can ultimately be obviated in any child with progressive symptomatology.[4,8]

References:

  1. Jenkin D, Angyalfi S, Becker L, et al.: Optic glioma in children: surveillance, resection, or irradiation? International Journal of Radiation Oncology, Biology, Physics 25(2): 215-225, 1993.
  2. Kovalic JJ, Grigsby PW, Shepard MJ, et al.: Radiation therapy for gliomas of the optic nerve and chiasm. International Journal of Radiation Oncology, Biology, Physics 18(4): 927-932, 1990.
  3. Tao ML, Barnes PD, Billett AL, et al.: Childhood optic chiasm gliomas: radiographic response following radiotherapy and long-term clinical outcome. International Journal of Radiation Oncology, Biology, Physics 39(3): 579-587, 1997.
  4. Packer RJ, Ater J, Allen J, et al.: Carboplatin and vincristine chemotherapy for children with newly diagnosed progressive low-grade gliomas. Journal of Neurosurgery 86(5): 747-754, 1997.
  5. Schmandt SM, Packer RJ, Vezina LG, et al.: Spontaneous regression of low-grade astrocytomas in childhood. Pediatric Neurosurgery 32(3): 132-136, 2000.
  6. Wisoff JH, Abbott R, Epstein F: Surgical management of exophytic chiasmatic-hypothalamic tumors of childhood. Journal of Neurosurgery 73(4): 661-667, 1990.
  7. Gropman AL, Packer RJ, Nicholson HS, et al.: Treatment of diencephalic syndrome with chemotherapy: growth, tumor response, and long term control. Cancer 83(1): 166-172, 1998.
  8. Prados MD, Edwards MS, Rabbitt J, et al.: Treatment of pediatric low-grade gliomas with a nitrosourea-based multiagent chemotherapy regimen. Journal of Neuro-Oncology 32(3): 235-241, 1997.

RECURRENT CHILDHOOD VISUAL PATHWAY AND HYPOTHALAMIC GLIOMA

Recurrence may take place in both benign and malignant childhood visual pathway and hypothalamic gliomas and may develop many years after initial treatment. Recurrent disease is usually at the primary tumor site, although widely disseminated disease to other intracranial sites and to the spinal leptomeninges has been documented. At the time of recurrence, a complete evaluation to determine the extent of the relapse is indicated. Biopsy or surgical resection may be necessary for confirmation of relapse because other entities, such as secondary tumor and treatment-related brain necrosis, may be clinically indistinguishable from tumor recurrence. The need for surgical intervention must be individualized on the basis of the initial tumor type, the length of time between initial treatment and the reappearance of the mass lesion, and the clinical picture.

If patients have not received radiation therapy, local radiation therapy is the usual treatment. However, in patients treated with surgery alone whose disease progresses, chemotherapy and radiation therapy are options. If recurrence takes place after irradiation, chemotherapy should be considered. Chemotherapy may result in relatively long-term disease control.[1] Entry into studies of novel therapeutic approaches should be considered for patients with recurrent brain tumors.[2-4] Information about ongoing clinical trials is available from the NCI (Http: //cancer.gov/clinical_trials/).

References:

  1. Packer RJ, Lange B, Ater J, et al.: Carboplatin and vincristine for recurrent and newly diagnosed low-grade gliomas of childhood. Journal of Clinical Oncology 11(5): 850-856, 1993.
  2. Chamberlain MC, Grafe MR: Recurrent chiasmatic-hypothalamic glioma treated with oral etoposide. Journal of Clinical Oncology 13(8): 2072-2076, 1995.
  3. Gaynon PS, Ettinger LJ, Baum ES, et al.: Carboplatin in childhood brain tumors: a Children's Cancer Study Group phase II trial. Cancer 66(12): 2465-2469, 1990.
  4. Gentet JC, Doz F, Bouffet E, et al.: Carboplatin and VP 16 in medulloblastoma: a phase II study of the French Society of Pediatric Oncology (SFOP). Medical and Pediatric Oncology 23(5): 422-427, 1994.
Date Last Modified: 11/2002

This information from PDQ is reviewed regularly by members of the PDQ Editorial Boards. If you have specific comments on the content of this information, direct them to: PDQ Editorial Board, CIPS/NCI, 6116 Executive Boulevard, Suite 3002B, MSC-8321, 20892-8321, fax: 301-480-8105. * * The PDQ database also contains listings of clinical trial protocols and directories of organizations and physicians who treat cancer patients, but this information is not available through CancerMail. For more information on accessing PDQ, consult the CancerMail Contents List.
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