Malignant Rhabdoid Tumor Follow-up

  • Author: James I Geller, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Mar 9, 2012
 

Further Inpatient Care

  • Treatment for malignant rhabdoid tumor (MRT) requires frequent inpatient admissions to administer chemotherapy and to manage complications of treatment, such as febrile neutropenia.
  • The duration of therapy is approximately 6-12 months.
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Further Outpatient Care

  • The myelosuppressive effects of the chemotherapy used to treat malignant rhabdoid tumor necessitate frequent monitoring of blood counts on an outpatient basis.
  • In addition, serum electrolyte levels and renal function must be observed closely because patients have a single kidney and often receive nephrotoxic chemotherapeutic agents. Electrolyte supplementation is not uncommonly required.
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Inpatient & Outpatient Medications

  • Chemotherapy regimens for malignant rhabdoid tumor are immunosuppressive.
  • As such, prophylaxis for Pneumocystis carinii pneumonia (PCP) is recommended. Trimethoprim-sulfamethoxazole or pentamidine are the first choices for PCP prophylaxis.
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Transfer

  • Initial transfer to the care of a pediatric oncologist, preferably one at a center that participates in clinical trials, is recommended.
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Deterrence/Prevention

  • In most cases, the cause of malignant rhabdoid tumor is unknown; thus, no preventive measures can be prescribed.
  • With advancements in genetic testing and counseling, families, including neonates, with rhabdoid tumor predisposition can be identified.
    • Recommendations for surveillance of individuals found to carry a constitutional SMARCB1 mutation are not based on evidence.
    • Unclear penetrance, gonadal mosaicism, and risk of multiple primary tumors confound assessing an individual's cancer risk. However, infants and young children with germline SMARCB1 mutation develop the most aggressive forms of malignant rhabdoid tumor within the first two years of life; therefore, screening such infants with noninvasive radiological techniques might enable cancer detection at an earlier cancer stage, and an earlier diagnosis can be hypothesized to impact overall prognosis.
    • As such, a preliminary screening plan under consideration proposed to the COG Rhabdoid Tumor Working Group includes the use of serial abdominal and transcranial ultrasonography (monthly for 1 y) with more detailed MRI performed every 3 months during the first year of life, with continued surveillance into the second and third year of life.
    • The risk of cancer development in late childhood and beyond in affected patients with constitutional SMARCB1 mutations remains even less predictable, making it challenging to prescribe a screening plan at this time.
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Complications

  • Complications related to tumoral progression: Malignant rhabdoid tumors in the abdomen can rapidly progress, as can those at metastatic sites, including the lungs, liver, and brain. Malignant rhabdoid tumors can be associated with tumoral hemorrhage and organ failure.
  • Complications related to treatment
    • Hematologic complications: The major acute complication of chemotherapy for malignant rhabdoid tumors is myelosuppression, which places patients at risk for serious infections. Patients require frequent RBC and platelet transfusions.
    • Renal complications: Patients may have renal tubular dysfunction, with wasting of protein, phosphorous, bicarbonate, and other electrolytes if platinum drugs or ifosfamide are used. The long-term prevalence of renal failure is unknown because malignant rhabdoid tumors is rare and the survival rate is low. Renal failure is uncommon in patients with unilateral Wilms tumor; however, patients with malignant rhabdoid tumors are treated intensively and with additional nephrotoxic drugs.
    • Cardiac complications: Some treatment regimens for malignant rhabdoid tumors include anthracyclines, which can cause arrhythmias and congestive heart failure. Cardiac function should be monitored periodically.
    • Gonadal complications: Ifosfamide and cyclophosphamide are associated with a risk of infertility.
    • Secondary cancers: The risk of secondary cancers from chemotherapy and/or radiation, particularly in patients with a genetic rhabdoid cancer predisposition, remain unknown.
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Prognosis

  • The prognosis for children with malignant rhabdoid tumors remains fair to poor, depending on the stage of the tumor at presentation, the patient's age at diagnosis, and possibly the genetic background.
  • The hope is that new multi-institutional clinical trials will help in identifying novel therapies that improve the outcome of patients with this disease.
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Patient Education

  • Patients and families should be educated about malignant rhabdoid tumor and its aggressive biologic behavior.
  • Although families must be given hope for a cure, they must also be made aware of the unfavorable prognosis associated with malignant rhabdoid tumors. Families must also understand the risks of intensive chemotherapy and the signs and symptoms that require immediate medical attention.

Genetic counseling

  • Genetic counseling is highly recommended for all malignant rhabdoid tumor affected families.
  • The incidence of germline deletions or missense mutations of SMARCB1 in infants and children with malignant rhabdoid tumor approximates 15-30%. Families with more than one affected child have been reported; in 2 families, evidence of germline mosaicism was suggested because neither parent had a mutation in their own peripheral blood. The incidence and age distribution of cancer in individuals with inherited SMARCB1 mutations has not been formally studied, but adults without cancer have been shown to transmit the abnormal allele in at least 3 families, and individuals with germline perturbations of SMARCB1 are predisposed to malignant rhabdoid tumors of the kidney, soft tissues, and brain and may, in fact, present with more than one primary tumor.
  • Accumulating evidence suggests that individuals with a confirmed malignant rhabdoid tumor should be evaluated for SMARCB1 expression in the tumor. Direct evaluation of the tumor by karyotyping, fluorescence in situ hybridization (FISH), or genomic microarray, with high-density single nucleotide polymorphism-based oligonucleotide arrays and multiplex ligation-dependent probe amplification as necessary, should be pursued to detect the mechanisms for biallelic silencing of SMARCB1 expression. Direct sequencing of SMARCB1 for missense mutations is recommended if abnormalities are not seen in both alleles. Evaluation of peripheral blood should follow tumor analysis. The finding of a chromosomal abnormality involving 22q or SMARCB1 missense mutation in the germline of an affected individual would then be followed by testing both parents. Because sibling recurrence is known to occur, testing of siblings, particularly those younger than 5 years, should be considered, even if bothparents are healthy.
  • Surveillance of individuals found to carry a constitutional SMARCB1 mutation for the development of CNS or abdominal malignant rhabdoid tumor may be advisable (see Deterrence/Prevention).
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Contributor Information and Disclosures
Author

James I Geller, MD  Associate Professor of Clinical Pediatrics, Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center

James I Geller, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Clinical Oncology, American Society of Pediatric Hematology/Oncology, and Children's Oncology Group

Disclosure: Nothing to disclose.

Coauthor(s)

Nancy D. Leslie  MD, Professor of Clinical Pediatrics, Cincinnati Children's Hospital

Nancy D. Leslie is a member of the following medical societies: American College of Medical Genetics, American Society of Human Genetics, Society for Inherited Metabolic Disorders, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Hong Yin, MD  Assistant Professor, Department of Pathology and Laboratory Medicine, University of Cincinnati School of Medicine; Staff Pathologist, Department of Pathology, Cincinnati Children's Hospital

Hong Yin, MD is a member of the following medical societies: American Medical Association, Children's Oncology Group, College of American Pathologists, Society for Pediatric Pathology, and United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Specialty Editor Board

Stephan A Grupp, MD, PhD  Director, Stem Cell Biology Program, Department of Pediatrics, Division of Oncology, Children's Hospital of Philadelphia; Associate Professor of Pediatrics, University of Pennsylvania School of Medicine

Stephan A Grupp, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Steven K Bergstrom, MD  Department of Pediatrics, Division of Hematology-Oncology, Kaiser Permanente Medical Center of Oakland

Steven K Bergstrom, MD is a member of the following medical societies: Alpha Omega Alpha, American Society of Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and International Society for Experimental Hematology

Disclosure: Nothing to disclose.

Helen SI Chan, MBBS, FRCP(C), FAAP  Associate Senior Scientist, Research Institute; Professor, Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto Faculty of Medicine, Canada

Helen SI Chan, MBBS, FRCP(C), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Jeffrey Dome, MD, to the original writing and development of this article.

References

  1. Haas JE, Palmer NF, Weinberg AG, Beckwith JB. Ultrastructure of malignant rhabdoid tumor of the kidney. A distinctive renal tumor of children. Hum Pathol. Jul 1981;12(7):646-57. [Medline].

  2. Roberts CW, Biegel JA. The role of SMARCB1/INI1 in development of rhabdoid tumor. Cancer Biol Ther. Mar 2009;8:412-6. [Medline].

  3. Jackson EM, Sievert AJ, Gai X, et al. Genomic analysis using high-density single nucleotide polymorphism-based oligonucleotide arrays and multiplex ligation-dependent probe amplification provides a comprehensive analysis of INI1/SMARCB1 in malignant rhabdoid tumors. Clin Cancer Res. Mar 2009;15:1923-30. [Medline].

  4. Kohashi K, Oda Y, Yamamoto H, et al. SMARCB1/INI1 protein expression in round cell soft tissue sarcomas associated with chromosomal translocations involving EWS: a special reference to SMARCB1/INI1 negative variant extraskeletal myxoid chondrosarcoma. Am J Surg Pathol. Aug 2008;32:1168-74. [Medline].

  5. Cheng JX, Tretiakova M, Gong C, Mandal S, Krausz T, Taxy JB. Renal medullary carcinoma: rhabdoid features and the absence of INI1 expression as markers of aggressive behavior. Mod Pathol. Jun 2008;21:647-52. [Medline].

  6. Kreiger PA, Judkins AR, Russo PA, et al. Loss of INI1 expression defines a unique subset of pediatric undifferentiated soft tissue sarcomas. Mod Pathol. Jan 2009;22:142-50. [Medline].

  7. Trobaugh-Lotrario AD, Tomlinson GE, Finegold MJ, Gore L, Feusner JH. Small cell undifferentiated variant of hepatoblastoma: adverse clinical and molecular features similar to rhabdoid tumors. Pediatr Blood Cancer. Mar 2009;52:328-34. [Medline].

  8. Russo P, Biegel JA. SMARCB1/INI1 alterations and hepatoblastoma: another extrarenal rhabdoid tumor revealed?. Pediatr Blood Cancer. Mar 2009;52:312-3. [Medline].

  9. Reinhard H, Reinert J, Beier R, et al. Rhabdoid tumors in children: prognostic factors in 70 patients diagnosed in Germany. Oncol Rep. Mar 2008;19:819-23. [Medline].

  10. Chung BY, Ahn IS, Cho SI, Kim HO, Kim KH, Park CW, et al. Primary malignant rhabdoid melanoma. Ann Dermatol. Oct 2011;23:S155-9. [Medline]. [Full Text].

  11. Mestre-Fusco A, Trampal C, Intriago B, Wessling H, Fuertes J, Suárez-Piñera M, et al. Assessment of rhabdoid brain tumor by F-18 FDG PET, C-11 methionine PET and MRI. Clin Nucl Med. Feb 2012;37(2):e33-5. [Medline].

  12. Hilden JM, Meerbaum S, Burger P, et al. Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry. J Clin Oncol. Jul 15 2004;22(14):2877-84. [Medline].

  13. Tekautz TM, Fuller CE, Blaney S, 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. Mar 1 2005;23(7):1491-9. [Medline].

  14. Koga Y, Matsuzaki A, Suminoe A, et al. Long-term survival after autologous peripheral blood stem cell transplantation in two patients with malignant rhabdoid tumor of the kidney. Pediatr Blood Cancer. Jul 2009;52:888-90. [Medline].

  15. Agarwala S. Primary Malignant Liver Tumors in Children. Indian J Pediatr. Mar 1 2012;[Medline].

  16. Agrons GA, Kingsman KD, Wagner BJ, Sotelo-Avila C. Rhabdoid tumor of the kidney in children: a comparative study of 21 cases. AJR Am J Roentgenol. Feb 1997;168(2):447-51. [Medline].

  17. Amar AM, Tomlinson G, Green DM, et al. Clinical presentation of rhabdoid tumors of the kidney. J Pediatr Hematol Oncol. Feb 2001;23(2):105-8. [Medline].

  18. 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. May 2000;28(1):31-7. [Medline].

  19. Biegel JA, Zhou JY, Rorke LB, et al. Germ-line and acquired mutations of INI1 in atypical teratoid and rhabdoid tumors. Cancer Res. Jan 1 1999;59(1):74-9. [Medline].

  20. Bruch LA, Hill DA, Cai DX, et al. A role for fluorescence in situ hybridization detection of chromosome 22q dosage in distinguishing atypical teratoid/rhabdoid tumors from medulloblastoma/central primitive neuroectodermal tumors. Hum Pathol. Feb 2001;32(2):156-62. [Medline].

  21. 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 medulloblastoma: a Pediatric Oncology Group study. Am J Surg Pathol. Sep 1998;22(9):1083-92. [Medline].

  22. Chai J, Charboneau AL, Betz BL, Weissman BE. Loss of the hSNF5 gene concomitantly inactivates p21CIP/WAF1 and p16INK4a activity associated with replicative senescence in A204 rhabdoid tumor cells. Cancer Res. Nov 15 2005;65(22):10192-8. [Medline].

  23. Chai J, Lu X, Godfrey V, et al. Tumor-specific cooperation of retinoblastoma protein family and Snf5 inactivation. Cancer Res. Apr 2007;67:3002-9. [Medline].

  24. Chi SN, Zimmerman MA, Yao X, et al. Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. J Clin Oncol. Jan 2009;27:385-9. [Medline].

  25. Chung CJ, Lorenzo R, Rayder S, et al. Rhabdoid tumors of the kidney in children: CT findings. AJR Am J Roentgenol. Mar 1995;164(3):697-700. [Medline].

  26. D'Angio GJ, Breslow N, Beckwith JB, et al. Treatment of Wilms' tumor. Results of the Third National Wilms' Tumor Study. Cancer. Jul 15 1989;64(2):349-60. [Medline].

  27. Fruhwald MC, Hasselblatt M, Wirth S, et al. Non-linkage of familial rhabdoid tumors to SMARCB1 implies a second locus for the rhabdoid tumor predisposition syndrome. Pediatr Blood Cancer. Sep 2006;47(3):273-8. [Medline].

  28. Fujisawa H, Misaki K, Takabatake Y, et al. Cyclin D1 is overexpressed in atypical teratoid/rhabdoid tumor with hSNF5/INI1 gene inactivation. J Neurooncol. Jun 2005;73(2):117-24. [Medline].

  29. Gururangan S, Bowman LC, Parham DM, et al. Primary extracranial rhabdoid tumors. Clinicopathologic features and response to ifosfamide. Cancer. Apr 15 1993;71(8):2653-9. [Medline].

  30. Han TI, Kim MJ, Yoon HK, et al. Rhabdoid tumour of the kidney: imaging findings. Pediatr Radiol. Apr 2001;31(4):233-7. [Medline].

  31. Hoot AC, Russo P, Judkins AR, et al. Immunohistochemical analysis of hSNF5/INI1 distinguishes renal and extra-renal malignant rhabdoid tumors from other pediatric soft tissue tumors. Am J Surg Pathol. Nov 2004;28(11):1485-91. [Medline].

  32. Isakoff MS, Sansam CG, Tamayo P, et al. Inactivation of the Snf5 tumor suppressor stimulates cell cycle progression and cooperates with p53 loss in oncogenic transformation. Proc Natl Acad Sci U S A. Dec 6 2005;102(49):17745-50. [Medline].

  33. Jafri SZ, Freeman JL, Rosenberg BF, et al. Clinical and imaging features of rhabdoid tumor of the kidney. Urol Radiol. 1991;13(2):94-7. [Medline].

  34. Janson K, Nedzi LA, David O, et al. Predisposition to atypical teratoid/rhabdoid tumor due to an inherited INI1 mutation. Pediatr Blood Cancer. Sep 2006;47(3):279-84. [Medline].

  35. Kordes U, Gesk S, Fruhwald MC, et al. Clinical and molecular features in patients with atypical teratoid rhabdoid tumor or malignant rhabdoid tumor. Genes Chromosomes Cancer. Feb 2010;49(2):176-81. [Medline].

  36. Madigan CE, Armenian SH, Malogolowkin MH, Mascarenhas L. Extracranial malignant rhabdoid tumors in childhood: the Childrens Hospital Los Angeles experience. Cancer. Nov 2007;110:2061-6. [Medline].

  37. McKenna ES, Sansam CG, Cho YJ, et al. Loss of the epigenetic tumor suppressor SNF5 leads to cancer without genomic instability. Mol Cell Biol. Oct 2008;28:5223-33. [Medline].

  38. 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. [published erratum appears in Am J Surg Pathol. 1995;19(4):488-9.]. Am J Surg Pathol. Oct 1994;18(10):1010-29. [Medline].

  39. Puri DR, Meyers PA, Kraus DH, Laquaglia MP, Wexler LH, Wolden SL. Radiotherapy in the multimodal treatment of extrarenal extracranial malignant rhabdoid tumors. Pediatr Blood Cancer. Jan 2008;50:167-9. [Medline].

  40. Reddy AT. Atypical teratoid/rhabdoid tumors of the central nervous system. J Neurooncol. Dec 2005;75(3):309-13. [Medline].

  41. Roberts CW, Galusha SA, McMenamin ME, et al. Haploinsufficiency of Snf5 (integrase interactor 1) predisposes to malignant rhabdoid tumors in mice. Proc Natl Acad Sci U S A. Dec 5 2000;97(25):13796-800. [Medline].

  42. Roberts CW, Leroux MM, Fleming MD, Orkin SH. Highly penetrant, rapid tumorigenesis through conditional inversion of the tumor suppressor gene Snf5. Cancer Cell. Nov 2002;2(5):415-25. [Medline].

  43. Rorke LB, Packer RJ, Biegel JA. Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: definition of an entity. J Neurosurg. Jul 1996;85(1):56-65. [Medline].

  44. Rousseau-Merck MF, Fiette L, Klochendler-Yeivin A, et al. Chromosome mechanisms and INI1 inactivation in human and mouse rhabdoid tumors. Cancer Genet Cytogenet. Mar 2005;157(2):127-33. [Medline].

  45. Rousseau-Merck MF, Versteege I, Legrand I, et al. hSNF5/INI1 inactivation is mainly associated with homozygous deletions and mitotic recombinations in rhabdoid tumors. Cancer Res. Jul 1 1999;59(13):3152-6. [Medline].

  46. Sigauke E, Rakheja D, Maddox DL, et al. Absence of expression of SMARCB1/INI1 in malignant rhabdoid tumors of the central nervous system, kidneys and soft tissue: an immunohistochemical study with implications for diagnosis. Mod Pathol. May 2006;19(5):717-25. [Medline].

  47. Sisler CL, Siegel MJ. Malignant rhabdoid tumor of the kidney: radiologic features. Radiology. Jul 1989;172(1):211-2. [Medline].

  48. [Best Evidence] Tomlinson GE, Breslow NE, Dome J, et al. Rhabdoid tumor of the kidney in the National Wilms' Tumor Study: age at diagnosis as a prognostic factor. J Clin Oncol. Oct 20 2005;23(30):7641-5. [Medline].

  49. Tsikitis M, Zhang Z, Edelman W, et al. Genetic ablation of Cyclin D1 abrogates genesis of rhabdoid tumors resulting from Ini1 loss. Proc Natl Acad Sci U S A. Aug 23 2005;102(34):12129-34. [Medline].

  50. Versteege I, Sevenet N, Lange J, et al. Truncating mutations of hSNF5/INI1 in aggressive paediatric cancer. Nature. Jul 9 1998;394(6689):203-6. [Medline].

  51. Wagner L, Hill DA, Fuller C, et al. Treatment of metastatic rhabdoid tumor of the kidney. J Pediatr Hematol Oncol. Jun-Jul 2002;24(5):385-8. [Medline].

  52. Waldron PE, Rodgers BM, Kelly MD, Womer RB. Successful treatment of a patient with stage IV rhabdoid tumor of the kidney: case report and review. J Pediatr Hematol Oncol. Jan-Feb 1999;21(1):53-7. [Medline].

  53. Weeks DA, Beckwith JB, Mierau GW, Luckey DW. Rhabdoid tumor of kidney. A report of 111 cases from the National Wilms' Tumor Study Pathology Center. Am J Surg Pathol. Jun 1989;13(6):439-58. [Medline].

  54. Weeks DA, Beckwith JB, Mierau GW, Zuppan CW. Renal neoplasms mimicking rhabdoid tumor of kidney. A report from the National Wilms' Tumor Study Pathology Center. Am J Surg Pathol. Nov 1991;15(11):1042-54. [Medline].

  55. Winger DI, Buyuk A, Bohrer S, et al. Radiology-Pathology Conference: rhabdoid tumor of the kidney. Clin Imaging. Mar-Apr 2006;30(2):132-6. [Medline].

  56. Yamamoto M, Suzuki N, Hatakeyama N, et al. Treatment of stage IV malignant rhabdoid tumor of the kidney (MRTK) with ICE and VDCy: a case report. J Pediatr Hematol Oncol. May 2006;28:286-9. [Medline].

  57. Zhang ZK, Davies KP, Allen J, et al. Cell cycle arrest and repression of cyclin D1 transcription by INI1/hSNF5. Mol Cell Biol. Aug 2002;22(16):5975-88. [Medline].

 
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Nonenhanced CT scan demonstrates linear and curvilinear calcifications outlining tumor lobules in a malignant rhabdoid tumor (MRT) (arrows). A hypoattenuating fluid collection surrounds and separates the lobules. These imaging features are seen with MRT more often than with other childhood renal neoplasms.
Contrast-enhanced CT scan demonstrates a subcapsular fluid collection (arrow) and the lobulated nature of a malignant rhabdoid tumor (MRT). Subcapsular fluid collections are more common with MRTs than with the other renal neoplasms that occur in children.
Histology of malignant rhabdoid tumors (MRTs). This photomicrograph shows the typical large malignant cells with large, vesicular nuclei, prominent red nucleoli, and abundant eosinophilic cytoplasm. Many tumor cells have a distinct, pale, rhabdoid inclusion in the cytoplasm. (Hematoxylin and eosin stain, original magnification x400).
INI1 immunohistochemistry stain shows diffuse loss of INI1 expression in tumor nuclei, with appropriate staining of intratumoral endothelial cells serving as the internal control (original magnification x400).
Table 1. One Ifosfamide-Carboplatin-Etoposide regimen for Malignant Rhabdoid Tumor
DrugDosageRouteSchedule
CarboplatinTarget dose to the AUC of 6 mg/mL/min by using the Calvert equationIVDay 1
Etoposide3.3 mg/kg/dose or 100 mg/m2/doseIVDays 1, 2, and 3
Ifosfamide65 mg/kg/dose or 2 g/m2/doseIVDays 1, 2, and 3
Mesna16 mg/kg/dose or 500 mg/m2/doseIVStart immediately after and at 3 h, 6 h, and 9 h after ifosfamide
Filgrastim G-CSF5 mcg/kg/doseSCStart 24 h after chemotherapy and continue until ANC recovers
Table 2. One Vincristine-Doxorubicin-Cyclophosphamide Regimen for Malignant Rhabdoid Tumor
DrugDosageRouteSchedule
Vincristine0.05 mg/kg/dose or 1.5 mg/m2/dose; not to exceed 2 mg/doseIVDays 1, 8, and 15
Doxorubicin1.2 mg/kg/dose or 37.5 mg/m2/doseIVDays 1 and 2
Cyclophosphamide60 mg/kg/dose or 1.8 g/m2/doseIVDay 1
Mesna15 mg/kg/dose or 450 mg/m2/doseIVStart immediately after and at 3, 6, and 9 h after cyclophosphamide
Filgrastim G-CSF5 mcg/kg/doseSCStart 24 h after chemotherapy and continue until ANC recovers
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