Malignant Rhabdoid Tumor Follow-up

Updated: Sep 27, 2021
  • Author: James I Geller, MD, FAAP; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Follow-up

Further Care

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.

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 often have a single kidney and receive nephrotoxic chemotherapeutic agents. Electrolyte supplementation is not uncommonly required.

Inpatient and 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.

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 MRT 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 MRT within the first two years of life; therefore, screening such infants might enable cancer detection at an earlier cancer stage, and an earlier diagnosis can be hypothesized to impact overall prognosis.

As such, the current screening plan for those with germline truncating SMARCB1 mutations includes the use of serial abdominal ultrasonography every 3 months to year 5 with consideration of a whole body MRI at age 5 years as well as MRI brain every 3 months to age 5 years. [47]

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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