This Article Abstract Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Hilden, J. M. Articles by Biegel, J. A. Search for Related Content PubMed PubMed Citation Articles by Hilden, J. M. Articles by Biegel, J. A.

Central Nervous System Atypical Teratoid/Rhabdoid Tumor: Results of Therapy in Children Enrolled in a Registry

From the Department of Pediatric Hematology/Oncology, The Children’s Hospital, The Cleveland Clinic, Cleveland, OH; Department of Pathology/NeuroPathology, Johns Hopkins Hospital, Baltimore, MD; Department of Hematology/Oncology, Hassenfeld Children’s Center, New York University Medical Center, New York, NY; Division of Neurology Research, Department of Pathology, and Division of Genetics Children’s Hospital of Philadelphia, Philadelphia, PA; Division of Anatomic Pathology, Mayo Clinic, Rochester, MN; Department of Hematology/Oncology, Christiana Care Health Services/A.I. duPont Institute, Wilmington, DE

Address reprint requests to Joanne M. Hilden, MD, Department of Pediatric Hematology/Oncology, The Children’s Hospital, The Cleveland Clinic, 9500 Euclid Ave, Desk S20, Cleveland, OH 44195; e-mail: hildenj{at}ccf.org

	ABSTRACT

TOP ABSTRACT INTRODUCTION MATERIALS AND METHODS RESULTS DISCUSSION Authors’ Disclosures of... REFERENCES

 
PURPOSE: Atypical teratoid/rhabdoid tumor (AT/RT) of the CNS is an extremely rare and aggressive tumor of early childhood. The poor outcome with conventional infant brain tumor therapy has resulted in a lack of clear treatment guidelines. A registry has been established to create an outcomes database and to facilitate biology studies for this tumor.

MATERIALS AND METHODS: A standardized data sheet was provided to treating physicians listing the reports that were to be sent to the registry for abstraction. Follow-up information was sought twice yearly.

RESULTS: Information was complete for 42 patients. Median age at diagnosis was 24 months. Nine patients (21%) had disseminated disease at diagnosis. Sixteen tumors were infratentorial; 26 were supratentorial. Twenty patients (48%) received a primary complete resection. Primary therapy included chemotherapy in all patients, radiotherapy in 13 patients (31%), stem-cell rescue in 13 patients (31%), and intrathecal chemotherapy in 16 patients (38%). Recurrent or progressive disease was reported in nine and 19 patients, respectively. Twenty-seven patients (64%) are dead of disease (3 to 62 months from diagnosis) and one patient died of toxicity. Fourteen patients (33%) show no evidence of disease (9.5 to 96 months from diagnosis). The median survival is 16.75 months and the median event-free survival is 10 months.

CONCLUSION: Aggressive therapy has prolonged the natural history in a subset of children. Prospective multi-institutional and national clinical trials designed specifically for AT/RT are needed. Enrollment onto the AT/RT registry should be continued.

	INTRODUCTION

TOP ABSTRACT INTRODUCTION MATERIALS AND METHODS RESULTS DISCUSSION Authors’ Disclosures of... REFERENCES

 
Atypical teratoid/rhabdoid tumor (AT/RT) of the CNS is an extremely rare and aggressive tumor of early childhood.^1-11 The rarity and the poor outcome with conventional infant brain tumor therapy have resulted in a lack of clear treatment guidelines; therefore, multiple approaches have been undertaken. Two of the authors (J.M.H., J.A.B.) created a registry for these tumors, both to create an outcomes database that tracks patients and to facilitate biology studies.

Rhabdoid tumor was originally described as a variant of Wilms’ tumor with rhabdomyosarcomatous features.¹² Subsequently, the features differentiating AT/RT from Wilms’ tumor were clarified.^13,14 Reports of extrarenal rhabdoid tumors ensued with debate over a different or common cell of origin.¹⁵ The most frequent anatomic location for nonrenal rhabdoid tumor is the CNS. The designation of AT/RT refers to the complex histologic features that may be present in these tumors. The association of renal and CNS AT/RT¹⁶ was consistent with cytogenetic studies that demonstrated abnormalities of 22q11.2 in tumors from both sites.^17,18 Molecular genetic studies have led to the identification of a rhabdoid suppressor gene (INI1/hSNF5) at 22q11.2. Somatic mutations in this gene predispose children to the development of AT/RT.^19-24 While this represents tremendous progress, not all mutations have been elucidated, as 25% of cases studied have no identified mutation.²⁵ There are reports of adults with AT/RT in the literature,^26-29 although molecular genetic studies have not yet confirmed the involvement of INI1 in an adult.

The early literature reflects a time course from diagnosis to death of about 12 months with standard therapy.^5,6,26 Subsequent reports document the use of more aggressive therapies (including surgery, chemotherapy with or without stem-cell support, intrathecal chemotherapy, and early radiotherapy), with some prolongation of the natural history of this tumor.^1-3,7,30-34

From these reports it is not possible to clarify the role of a specific treatment modality, as many different therapies were used. This fact was born out in a workshop cosponsored by the Pediatric Brain Tumor Foundation and the National Cancer Institute. Participants concluded that despite multiple reports and information that significant proportions of children younger than 3 years with malignant brain tumors have AT/RTs, effective treatment strategies for these tumors remain unclear and require further study. Until a nationwide protocol for patients with AT/RTs is available, the AT/RT registry should be maintained. Any information obtained from the registry combined with information from prospective studies will facilitate the development of optimal treatment approaches for children with these potentially lethal tumors.⁷

We report on the treatment delivered and the outcomes for 42 children enrolled on the registry thus far, for whom information is complete.

	MATERIALS AND METHODS

TOP ABSTRACT INTRODUCTION MATERIALS AND METHODS RESULTS DISCUSSION Authors’ Disclosures of... REFERENCES

 
Patients were referred to the registry by either the treating physician, an evaluating pathologist, or by a parent (through Internet information sources regarding AT/RT). In the case of parent referral, information was obtained from the treating physician. Institutional Review Board approval was obtained. Cases were classified as AT/RT if the initial diagnosis had been made historically according to criteria set forth by Drs Burger, Rorke, and Scheithauer.³⁵

A standardized data sheet was provided to treating physicians listing the reports that were to be sent to the registry for abstraction. The registry requires radiology reports, operative notes, pathology reports, cytogenetics reports, discharge summaries, and treatment records in addition to patient demographics. Registry personnel abstract the information; follow-up information on surviving patients is sought twice yearly through procedures compatible with privacy regulations. In addition, physicians are asked to consider sending archived or frozen tumor tissue for molecular genetic analysis to Dr Biegel in a separately consented study.

	RESULTS

TOP ABSTRACT INTRODUCTION MATERIALS AND METHODS RESULTS DISCUSSION Authors’ Disclosures of... REFERENCES

 
Overall
Information was complete for 42 patients; among these, the ratio of males to females was 2:1 (28 males:14 females). The age at diagnosis ranged from 1.5 to 118 months with a median of 24 months. Nine patients (21%) had disseminated disease at diagnosis. Sixteen (38%) tumors were infratentorial and 26 were supratentorial. Table 1 shows the age, tumor site, sex, and therapy delivered to 42 registry patients. Twenty patients (48%) received a primary complete resection. Primary therapy included chemotherapy in all patients, radiotherapy in 13 patients (31%), bone marrow or peripheral blood stem cell rescue in 13 patients (31%), and intrathecal chemotherapy in 16 patients (38%). Recurrent or progressive disease was reported in nine and 19 patients, respectively. Twenty-seven patients (64%) are dead of disease (median, 12 months [3 to 62 months from diagnosis]), and one patient died of toxicity at 5.5 months from diagnosis. The median overall survival is 16.75 months (range, 2.5 to 96 months), and the median event-free survival (EFS) in the group is 10 months (range, 1 to 96 months). Fourteen patients (33%) show no evidence of disease (median, 46 months [9.5 to 96 months from diagnosis]). Ten (24%) have been free of disease 24 months.

Chemotherapy
All 42 patients were treated with chemotherapy, although various regimens were used (Table 1). Eight children were treated according to Children’s Oncology Group (COG) 99703 (modified in four patients by inclusion of intrathecal therapy), and six were treated according to Children’s Cancer Group 9921 regimen A (modified in one patient with high dosemethotrexate). Both of these protocols use vincristine, etoposide, cisplatin, and cyclophosphamide. Seven patients were treated according to Intergroup Rhabdomyosarcoma Study III (vincristine, cisplatin, adriamycin, cyclophosphamide, and etoposide, and triple intrathecal therapy). The other children were treated with various regimens that included these same agents but also included high-dose methotrexate, dactinomycin, carboplatin, and/or carmustine.

The responsiveness of this tumor to chemotherapy was assessed in the children who had an incomplete resection. Twelve of 22 patients had a complete (six patients) or partial (six patients) response to chemotherapy.

Radiotherapy
Thirteen patients (31%) received radiotherapy as part of their primary therapy. The radiation field was to the primary tumor bed for nine children and was craniospinal for four children. For these patients, the median survival is 48 months (range, 10 to 96 months); median EFS is 48 months (range, 7 to 96 months). The median time to initiation of radiotherapy is six months (range, 1 to 10 months) from diagnosis. Median age at diagnosis of radiated patients is 47 months (range, 5 to 78 months). Eight of these children remain free of disease (range, 19 to 96 months), and five have died of disease 7 to 62 months from diagnosis.

Intrathecal Chemotherapy
Sixteen patients (38%) received intrathecal chemotherapy as part of primary treatment with a median EFS of 16.5 months and median survival of 23 months. Nine are dead of disease (10 to 62 months) and seven show no evidence of disease (19 to 90 months from diagnosis). Thirteen received intrathecal triple treatment consisting of methotrexate, hydrocortisone, cytarabine; two received both intrathecal triple treatment and intrathecal thiotepa; three received intrathecal therapy using thiotepa; and two received intrathecal therapy using methotrexate.

High-Dose Chemotherapy With Stem-Cell Rescue
Stem-cell rescue (SCR) was used as part of primary therapy for 13 patients. Nine of the 13 patients had no evidence of disease (NED) going into SCR. Median event free survival is 10 months (range, 7 to 90 months) and median overall survival is 21.5 months (range, 5.5 to 90 months) from time of diagnosis.

For eight patients, this was administered as a single myeloablative regimen. Six of eight patients had NED before SCR. Five of these children are alive with NED at 9.5 to 90 months from diagnosis, and three died of disease 10 to 22 months from diagnosis.

SCR was done for five patients as three mini-transplants following the currently open infant brain tumor protocol, CCG-99703. On this protocol, the three rescued chemotherapy courses consist of carboplatin/thiotepa, and these follow three courses of vincristine/cisplatin/etoposide/cytoxan, during which stem cells are harvested by pheresis. Three of five were NED before SCR. Among these five patients, one is alive with no evidence of disease at 48 months from diagnosis and three died of disease 12 to 22 months from diagnosis. There was one toxic death.

Therapy in Patients With Prolonged Survival
Fourteen (33%) of the 42 children remain alive with no evidence of disease at the time of this writing. The median age of these children is 30 months at time of diagnosis; median EFS is 42 months (range, 11 to 90 months) from diagnosis. The therapy delivered to these children is outlined in Table 3. Ten of the 14 children had a complete resection at diagnosis. Radiation therapy was part of primary treatment for seven of the children and SCR was part of primary therapy for four of the children. Six of the 14 children had intrathecal chemotherapy; five of these six children also had primary radiotherapy.

Molecular Genetics
In a separately consented process, specimens from the resected tumors were sent to Dr Biegel for molecular analysis. Specimens were adequate for testing from 23 patients. Cytogenetic abnormality at 22q was detected in 11 of 23 patients. INI-1 mutation was found in 14 patients (Table 1).

	DISCUSSION

TOP ABSTRACT INTRODUCTION MATERIALS AND METHODS RESULTS DISCUSSION Authors’ Disclosures of... REFERENCES

 
Therapy remains quite varied for children diagnosed with CNS AT/RT, and it was for this reason that a database of patients with CNS AT/RT, their therapy, and their outcomes was developed. There is debate as to whether the criteria for diagnosis of AT/RT should include molecular or cytogenetic evidence of an abnormality at chromosome band 22q11. Further biologic and molecular genetic studies are needed to aid both in clearer diagnosis and in the hope of ultimately designing tumor-specific therapy. Future clinical trials should require pathology and testing for molecular genetic evidence of an INI1 gene alteration for patient entry, and should look at whether germline mutations have a negative impact on survival.

The early AT/RT literature reflected poor outcomes with conventional therapy.^5,6,26 This has led to the use of aggressive chemotherapy and some use of intrathecal chemotherapy.^2,3 Simultaneously, the use of high-dose chemotherapy and SCR for high-risk brain tumors has been reported.^31-34,36 COG has instituted the use of this modality in protocols for high-risk pediatric brain tumors.

The role of surgery has not been addressed in the literature. The EFS for the 20 patients who had a gross total resection is 14 months (range, 1.5 to 72 months) compared to the EFS of 9.25 months (range, 1 to 96 months) for the subset with a partial resection. We recommend that an aggressive surgical approach be undertaken, including second look surgery when possible, to achieve gross total resection.

The efficacy of individual agents or regimens cannot be judged, but most children received chemotherapy according to current infant recommendations with platinum-based and alkylator-based regimens. Twelve of 22 patients had a complete or partial response to chemotherapy. Clearly, this is a chemotherapy-responsive tumor.

The literature is not helpful regarding the role of radiotherapy for this tumor. As can be seen from Table 3, eight of the 14 children in the cohort with NED received radiation. Children who received radiotherapy were older (median age, 47 months) relative to the entire cohort (median age, 24 months). These data and those from other participants in the National Cancer Institute AT/RT Workshop⁷ led to the recommendation that involved-field radiotherapy be included in the developing COG protocol for CNS AT/RT. Radiation therapy will continue to be controversial, yet it may contribute to disease control and should be evaluated prospectively.

The report of the successful treatment of CNS AT/RT with therapy similar to that used for parameningeal rhabdomyosarcoma² led to treating physicians including intrathecal chemotherapy in the therapy of these tumors.^1,3,26 Intrathecal therapy should be considered for future study, and we currently recommend that physicians consider it, especially in those children with residual disease that will not receive radiotherapy.

The role of high-dose chemotherapy and SCR in high-risk childhood brain tumors is the subject of currently enrolling national pediatric clinical trials. Six of 13 patients who received SCR as part of the primary therapy are alive without evidence of disease. The role of high-dose therapy with SCR for these children, as well as its timing, should also be studied.

Prospective multi-institutional and national clinical trials designed specifically for AT/RT are needed. Along with this effort, enrollment onto the AT/RT registry should be continued. Treating physicians can access Institutional Review Board material and the AT/RT Registry Information at The Children’s Hospital at The Cleveland Clinic Web site: http://www.clevelandclinic.org/pediatrics/departments/cancer_program/tumorregistry/default.htm.

	Authors’ Disclosures of Potential Conflicts of Interest

TOP ABSTRACT INTRODUCTION MATERIALS AND METHODS RESULTS DISCUSSION Authors’ Disclosures of... REFERENCES

 
The authors indicated no potential conflicts of interest.

	Acknowledgment

 
It is relevant to state that both the parents of these children and treating physicians have been very appreciative of the opportunity to review the outcome data maintained in the registry. Indeed, the parents of these children are the best supporters and feel well served. It is in honor of their children that the AT/RT registry continues. The authors gratefully acknowledge the physicians and their CRAs who have taken time to put the AT/RT registry through their IRBs, and send reports and summaries on their patients. Heartfelt respect and gratitude is also extended to the children’s parents for urging their physicians to enroll their children. The authors feel obligated to let these teams know that the parents in turn feel they serve their children well in so doing, and they thank the doctors as well.

	NOTES

 
Supported by National Institutes of Health (CA46274) and the Children’s Oncology Group Brain Tumor Resource Laboratory (J.A.B).

Authors’ disclosures of potential conflicts of interest are found at the end of this article.

	REFERENCES

TOP ABSTRACT INTRODUCTION MATERIALS AND METHODS RESULTS DISCUSSION Authors’ Disclosures of... REFERENCES

 
1. Hilden JM, Watterson J, Longee DC, et al: Central nervous system atypical teratoid tumor/rhabdoid tumor: Response to intensive therapy and review of the literature. J Neurooncol 40:265-275, 1998[CrossRef][Medline]

2. Olson TA, Bayar E, Kosnik E, et al: Successful treatment of disseminated central nervous system malignant rhabdoid tumor. J Pediatr Hematol Oncol 17:71-75, 1995[Medline]

3. Weinblatt M, Kochen J: Rhabdoid tumor of the central nervous system (letter). Med Pediatr Oncol 20:258, 1992[Medline]

4. Lefkowitz IB, Rorke LB, Packer RJ, et al: Atypical teratoid tumor of infancy: Definition of an entity. Ann Neurol 22:448-449, 1987 (abstr 133)

5. Rorke LB, Packer RJ, Biegel JA: Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: Definition of an entity. J Neurosurg 85:56-65, 1996[Medline]

6. Burger PC, Yu IT, Tihan T, et al: Atypical teratoid/rhabdoid tumor of the central nervous system. A highly malignant tumor of infancy and childhood frequently mistaken for a medulloblastoma: A Pediatric Oncology Group Study. Am J Surg Pathol 22:1083-1092, 1998[CrossRef][Medline]

7. Packer RJ, Biegel JA, Blaney S, et al: Atypical teratoid/rhabdoid tumor of the central nervous system: Report on workshop. J Pediatr Hematol Oncol 24:337-342, 2002[CrossRef][Medline]

8. Guler E, Varan A, Soylemezoglu F, et al: Extraneural metastasis in a child with atypical teratoid rhabdoid tumor of the central nervous system. J Neurooncol 54:53-56, 2001[CrossRef][Medline]

9. Korones DN, Meyers SP, Rubio A, et al: A 4-year-old girl with a ventriculoperitoneal shunt metastasis of a central nervous system atypical teratoid/rhabdoid tumor. Med Pediatr Oncol 32:389-391, 1999[CrossRef][Medline]

10. Proust F, Laquerriere A, Constantin B, et al: Simultaneous presentation of atypical teratoid/rhabdoid tumor in siblings. J Neurooncol 43:63-70, 1999[CrossRef][Medline]

11. Reinhardt D, Behnke-Mursch J, Weiss E, et al: Rhabdoid tumors of the central nervous system. Childs Nerv Syst 16:228-234, 2000[CrossRef][Medline]

12. Beckwith JB, Palmer NF: Histopathology and prognosis of Wilms’ tumors: Results from the First National Wilms’ Tumor Study. Cancer 41:1937-1948, 1978[CrossRef][Medline]

13. Haas JE, Palmer NF, Weinberg AG, et al: Ultrastructure of malignant rhabdoid tumor of the kidney. A distinctive renal tumor of children. Hum Pathol 12:646-657, 1981[Medline]

14. Weeks DA, Beckwith JB, Mierau GW, et al: Rhabdoid tumor of kidney. A report of 111 cases from the National Wilms’ Tumor Study Pathology Center. Am J Surg Pathol 13:439-458, 1989[Medline]

15. Parham DM, Weeks DA, Beckwith JB: The clinicopathologic spectrum of putative extrarenal rhabdoid tumors. An analysis of 42 cases studied with immunohistochemistry or electron microscopy. Am J Surg Pathol 18:1019-1029, 1994

16. Bonnin JM, Rubinstein LJ, Palmer NF, et al: The association of embryonal tumors originating in the kidney and in the brain. A report of seven cases. Cancer 54:2137-2146, 1984[CrossRef][Medline]

17. Biegel JA, Rorke LB, Packer RJ, et al: Monosomy 22 in rhabdoid or atypical tumors of the brain. J Neurosurg 73:710-714, 1990[Medline]

18. Biegel JA, Allen CS, Kawasaki K, et al: Narrowing the critical region for a rhabdoid tumor locus in 22q11. Genes Chromosomes Cancer 16:94-105, 1996[CrossRef][Medline]

19. Biegel JA: Cytogenetics and molecular genetics of childhood brain tumors. Neuro-oncol 1:139-151, 1999[Abstract]

20. Biegel JA, Fogelgren B, Zhou JY, et al: Mutations of the INI1 rhabdoid tumor suppressor gene in medulloblastomas and primitive neuroectodermal tumors of the central nervous system. Clin Cancer Res 6:2759-2763, 2000[Abstract/Free Full Text]

21. Biegel JA, Fogelgren B, Wainwright LM, et al: Germline INI1 mutation in a patient with a central nervous system atypical teratoid tumor and renal rhabdoid tumor. Genes Chromosomes Cancer 28:31-37, 2000[CrossRef][Medline]

22. Biegel JA, Kalpana G, Knudsen ES, et al: The Role of INI1 and the SWI/SNF complex in the development of rhabdoid tumors: Meeting summary from the Workshop on Childhood Atypical Teratoid/Rhabdoid Tumors. Cancer Res 62:323-328, 2002[Abstract]

23. Versteege I, Sevenet N, Lange J, et al: Truncating mutations of hSNF5/INI1 in aggressive pediatric cancer. Nature 394:203-206, 1998[CrossRef][Medline]

24. Biegel JA, Zhou J-Y, Rorke LB, et al: Germ-line and acquired mutations of INI1 in atypical teratoid and rhabdoid tumors. Cancer Res 59:74-79, 1999[Abstract/Free Full Text]

25. Biegel J, Tan L, Zhang F, et al: Alterations of the hSNF5/INI1 gene in central nervous system atypical teratoid/rhabdoid tumors and renal and extrarenal rhabdoid tumors. Clin Cancer Res 8:3461-3467, 2002[Abstract/Free Full Text]

26. Fisher BJ, Siddiqui J, Macdonald D, et al: Malignant rhabdoid tumor of the brain: An aggressive clinical entity. Can J Neurol Sci 23:257-263, 1996[Medline]

27. Horn M, Schlote W, Lerch KD, et al: Mallignant rhabdoid tumor: Primary intracranial manifestation in an adult. Acta Neuropathol 83:445-448, 1992[CrossRef][Medline]

28. Balaton AJ, Vaury P, Videgrain M: Paravertebral malignant rhabdoid tumor in an adult. A case report with immunocytochemical study. Pathol Res Pract 182:713-718, 1987[Medline]

29. Lutterbach J, Liegibel J, Koch D, et al: Atypical teratoid/rhabdoid tumors in adult patients: Case report and review of the literature. J Neurooncol 52:49-56, 2001[CrossRef][Medline]

30. Hilden JM, Biegel J, Rorke L, et al: Registry for central nervous system (CNS) atypical teratoid tumor/rhabdoid tumor (ATT/RT): Treatment and outcome in 18 cases. Proceedings of the 9th International Symposium on Pediatric Neuro-Oncology, 2000

31. Papadakis V, Dunkel IJ, Cramer LD, et al: High-dose carmustine, thiotepa and etoposide followed by autologous bone marrow rescue for the treatment of high risk central nervous system tumors. Bone Marrow Transplant 26:153-160, 2000[CrossRef][Medline]

32. 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 16:210-221, 1998[Abstract/Free Full Text]

33. Finlay JL: The role of high-dose chemotherapy and stem cell rescue in the treatment of malignant brain tumors: A reappraisal. Pediatr Transplant 1:87-95, 1999 (suppl 3)

34. Dunkel IJ, Finlay JL: High-dose chemotherapy with autologous stem cell rescue for brain tumors. Crit Rev Oncol Hematol 41:197-204, 2002[Medline]

35. Kleihues P, Louis DN, Scheithauer BW, et al: The WHO classification of tumors of the nervous system. J Neuropathol Exp Neurol 61:215-225, 2002[Medline]

36. Guruangan S, Dunkel IJ, Goldman S, et al: Myeloablative chemotherapy with autologous bone marrow rescue in young children with recurrent malignant brain tumors. J Clin Oncol 16:2486-2493, 1998[Abstract]

Submitted July 10, 2003; accepted January 15, 2004.

This article has been cited by other articles:

				I. F. Pollack Diagnostic and Therapeutic Stratification of Childhood Brain Tumors: Implications for Translational Research J Child Neurol, October 1, 2008; 23(10): 1179 - 1185. [Abstract] [PDF]

				K. Koral, L. Gargan, D. C. Bowers, B. Gimi, C. F. Timmons, B. Weprin, and N. K. Rollins Imaging Characteristics of Atypical Teratoid-Rhabdoid Tumor in Children Compared with Medulloblastoma Am. J. Roentgenol., March 1, 2008; 190(3): 809 - 814. [Abstract] [Full Text] [PDF]

				S.P. Meyers, Z.P. Khademian, J.A. Biegel, S.H. Chuang, D.N. Korones, and R.A. Zimmerman Primary intracranial atypical teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient outcomes. AJNR Am. J. Neuroradiol., May 1, 2006; 27(5): 962 - 971. [Abstract] [Full Text] [PDF]

				J. C. Allen, A. R. Judkins, M. K. Rosenblum, and J. A. Biegel Atypical teratoid/rhabdoid tumor evolving from an optic pathway ganglioglioma: Case study Neuro-oncol, January 1, 2006; 8(1): 79 - 82. [Abstract] [Full Text] [PDF]

				T. M. Tekautz, C. E. Fuller, S. Blaney, M. Fouladi, A. Broniscer, T. E. Merchant, M. Krasin, J. Dalton, G. Hale, L. E. Kun, et al. Atypical Teratoid/Rhabdoid Tumors (ATRT): Improved Survival in Children 3 Years of Age and Older With Radiation Therapy and High-Dose Alkylator-Based Chemotherapy J. Clin. Oncol., March 1, 2005; 23(7): 1491 - 1499. [Abstract] [Full Text] [PDF]

This Article Abstract Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Hilden, J. M. Articles by Biegel, J. A. Search for Related Content PubMed PubMed Citation Articles by Hilden, J. M. Articles by Biegel, J. A.