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Malignant Rhabdoid Tumor Follow-up

  • Author: James I Geller, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
 
Updated: Dec 05, 2014
 

Further Outpatient Care

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  • 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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Further Inpatient Care

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  • 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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Inpatient & Outpatient Medications

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  • 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

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  • 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

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  • 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

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  • 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

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  • 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

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  • 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

See the list below:

  • 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 Pediatric Hematology/Oncology, Children's Oncology Group, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Coauthor(s)

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

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

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, College of American Pathologists, United States and Canadian Academy of Pathology, Children's Oncology Group, Society for Pediatric Pathology

Disclosure: Nothing to disclose.

Specialty Editor Board

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, Children's Oncology Group, American Society of Clinical Oncology, International Society for Experimental Hematology, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

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

Disclosure: Nothing to disclose.

Additional Contributors

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, Society for Pediatric Research, American Society for Blood and Marrow Transplantation, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Acknowledgements

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.

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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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