Current Clinical Trials

Careful evaluation to fully determine the extent of disease must precede the treatment of childhood supratentorial primitive neuroectodermal tumors (SPNET) and pineoblastoma. Surgery should be an attempt at maximal tumor reduction. Postoperatively, studies should be conducted to determine if the patient has disseminated disease.[1] Risk criteria are outlined in the stage information section. Patients with extensive tumors should be considered at the highest risk of relapse and should be treated on protocols specifically designed for them.[2] Children with pineal primitive neuroectodermal tumors may have a more favorable prognosis when treated with surgery, radiation therapy, and chemotherapy than children with SPNETs.[2,3]

The usual postsurgical treatment is radiation therapy.[4] The suggested tumor dose is 54 Gy to 56 Gy using conventional fractionation. Craniospinal irradiation with 23.4 Gy to 36 Gy is also recommended because of the propensity of this tumor to disseminate through the subarachnoid space. Given the overall poorer prognosis for patients with extensive disease, the addition of chemotherapy before or after radiation therapy is being explored.[5,6] The role of chemotherapy to delay irradiation and its consequences is under clinical evaluation; preliminary results suggest that chemotherapy can be used to delay, modify, or, in selected cases, obviate the need for radiation therapy.[1]

Children younger than 3 years:

Some patients younger than 3 years with newly diagnosed SPNETs and pineoblastomas will respond at least partially to chemotherapy.[1-3,7] Some patients, especially those with minimal residual postoperative disease, may have a long-lasting response. For this reason, studies that use chemotherapy to delay, modify, or possibly obviate the need for radiation therapy have been undertaken. Results of such studies for young children with SPNET and pineoblastomas have been disappointing.[1,8] Although chemotherapy is being used to prevent neurologic damage caused by radiation therapy in very young patients, neurologic deficits may be present in children prior to the initiation of therapy, and progressive neurologic damage has been noted during therapy.[9]

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with untreated childhood supratentorial primitive neuroectodermal tumor ¹, childhood pineoblastoma ² and untreated childhood pineoblastoma ³. 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 ⁴.

References

1. 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. N Engl J Med 328 (24): 1725-31, 1993.  [PUBMED Abstract]
   
   
2. Cohen BH, Zeltzer PM, Boyett JM, et al.: Prognostic factors and treatment results for supratentorial primitive neuroectodermal tumors in children using radiation and chemotherapy: a Childrens Cancer Group randomized trial. J Clin Oncol 13 (7): 1687-96, 1995.  [PUBMED Abstract]
   
   
3. 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]
   
   
4. Disclafani A, Hudgins RJ, Edwards MS, et al.: Pineocytomas. Cancer 63 (2): 302-4, 1989.  [PUBMED Abstract]
   
   
5. Reddy AT, Janss AJ, Phillips PC, et al.: Outcome for children with supratentorial primitive neuroectodermal tumors treated with surgery, radiation, and chemotherapy. Cancer 88 (9): 2189-93, 2000.  [PUBMED Abstract]
   
   
6. Massimino M, Gandola L, Spreafico F, et al.: Supratentorial primitive neuroectodermal tumors (S-PNET) in children: A prospective experience with adjuvant intensive chemotherapy and hyperfractionated accelerated radiotherapy. Int J Radiat Oncol Biol Phys 64 (4): 1031-7, 2006.  [PUBMED Abstract]
   
   
7. Duffner PK, Cohen ME, Sanford RA, et al.: Lack of efficacy of postoperative chemotherapy and delayed radiation in very young children with pineoblastoma. Pediatric Oncology Group. Med Pediatr Oncol 25 (1): 38-44, 1995.  [PUBMED Abstract]
   
   
8. Timmermann B, Kortmann RD, Kühl J, et al.: Role of radiotherapy in supratentorial primitive neuroectodermal tumor in young children: results of the German HIT-SKK87 and HIT-SKK92 trials. J Clin Oncol 24 (10): 1554-60, 2006.  [PUBMED Abstract]
   
   
9. Mulhern RK, Horowitz ME, Kovnar EH, et al.: Neurodevelopmental status of infants and young children treated for brain tumors with preirradiation chemotherapy. J Clin Oncol 7 (11): 1660-6, 1989.  [PUBMED Abstract]