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Childhood Central Nervous System Embryonal Tumors Treatment (PDQ®)

Health Professional Version
Last Modified: 10/29/2014

Treatment of Newly Diagnosed Pineoblastoma

Children Older Than 3 Years
        Standard treatment options
        Treatment options under clinical evaluation
Children Aged 3 Years and Younger
Current Clinical Trials



Children Older Than 3 Years

Standard treatment options

Standard treatment options for children older than 3 years with newly diagnosed pineoblastoma include the following:

  1. Surgery.
  2. Adjuvant therapy.
    • Radiation therapy.
    • Chemotherapy.
Surgery

Surgery is usually the initial treatment for pineoblastoma for diagnosis. Total and near-total resection is infrequently obtained in pineoblastomas and the impact of the degree of resection on outcome is unknown.[1,2]

Adjuvant therapy

The usual postsurgical treatment for pineoblastomas begins with radiation therapy, although some trials have utilized preradiation chemotherapy. The total dose of radiation therapy to the tumor site is 54 Gy to 55.8 Gy using conventional fractionation.[1,2]

Adjuvant radiation therapy and chemotherapy
  1. Craniospinal irradiation with doses ranging between 23.4 Gy and 36 Gy are also recommended because of the propensity of this tumor to disseminate throughout the subarachnoid space.[1,2]

  2. Chemotherapy is usually utilized in the same way as outlined for high-risk medulloblastomas in children with nondisseminated disease at the time of diagnosis.
    • Five-year disease-free survival is approximately 50%.[1-3]

  3. For patients with disseminated disease at the time of diagnosis, survival is considerably poorer.[1,2]

Treatment options under clinical evaluation

For patients with pineoblastoma, a variety of different treatment approaches are under evaluation, including the use of higher doses of chemotherapy following radiation supported by peripheral stem cell rescue and the use of chemotherapy during radiation.

The following is an example of a national and/or institutional clinical trial that is currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.

  1. COG-ACNS0332 (NCT00392327) (Chemotherapy and Radiation Therapy in Treating Young Patients With Newly Diagnosed, Previously Untreated, High-Risk Medulloblastoma or Supratentorial Primitive Neuroectodermal Tumor): This Children's Oncology Group phase III trial for children older than 3 years is evaluating the efficacy of adding carboplatin to radiation therapy with vincristine, followed by maintenance chemotherapy with conventional adjuvant chemotherapy and isotretinoin.
Children Aged 3 Years and Younger

Biopsy is usually performed to diagnose pineoblastoma.

Children aged 3 years and younger with pineoblastoma are usually treated initially with chemotherapy in the hope of delaying, if not obviating, the need for radiation therapy.[4] In children responding to chemotherapy, the timing and amount of radiation therapy required following chemotherapy is unclear. The addition of craniospinal irradiation to chemotherapy-based regimens may successfully treat some children but with anticipated neurodevelopmental decline.[5][Level of evidence: 2A]

High-dose, marrow-ablative chemotherapy with autologous bone marrow rescue or peripheral stem cell rescue has been used with some success in young children.[6][Level of evidence: 2Di]

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with untreated childhood pineoblastoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References
  1. Jakacki RI, Zeltzer PM, Boyett JM, et al.: Survival and prognostic factors following radiation and/or chemotherapy for primitive neuroectodermal tumors of the pineal region in infants and children: a report of the Childrens Cancer Group. J Clin Oncol 13 (6): 1377-83, 1995.  [PUBMED Abstract]

  2. Timmermann B, Kortmann RD, Kühl J, et al.: Role of radiotherapy in the treatment of supratentorial primitive neuroectodermal tumors in childhood: results of the prospective German brain tumor trials HIT 88/89 and 91. J Clin Oncol 20 (3): 842-9, 2002.  [PUBMED Abstract]

  3. Gururangan S, McLaughlin C, Quinn J, et al.: High-dose chemotherapy with autologous stem-cell rescue in children and adults with newly diagnosed pineoblastomas. J Clin Oncol 21 (11): 2187-91, 2003.  [PUBMED Abstract]

  4. Mason WP, Grovas A, Halpern S, et al.: Intensive chemotherapy and bone marrow rescue for young children with newly diagnosed malignant brain tumors. J Clin Oncol 16 (1): 210-21, 1998.  [PUBMED Abstract]

  5. Friedrich C, von Bueren AO, von Hoff K, et al.: Treatment of young children with CNS-primitive neuroectodermal tumors/pineoblastomas in the prospective multicenter trial HIT 2000 using different chemotherapy regimens and radiotherapy. Neuro Oncol 15 (2): 224-34, 2013.  [PUBMED Abstract]

  6. Fangusaro J, Finlay J, Sposto R, et al.: Intensive chemotherapy followed by consolidative myeloablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) in young children with newly diagnosed supratentorial primitive neuroectodermal tumors (sPNETs): report of the Head Start I and II experience. Pediatr Blood Cancer 50 (2): 312-8, 2008.  [PUBMED Abstract]