Malignant Rhabdoid Tumor Workup

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

Laboratory Studies

  • Although malignant rhabdoid tumor (MRT) is definitively diagnosed by means of histologic analysis (see Histologic Findings below), laboratory studies can help in distinguishing a rhabdoid tumor of the kidney from a Wilms tumor.
  • The following tests may be helpful:
    • CBC count: Approximately 55% of patients with malignant rhabdoid tumor present with a hemoglobin level of less than 9 g/dL. Only 25% of patients with Wilms tumor are anemic at presentation.
    • Urinalysis: Microscopic hematuria is seen in 75% of patients with malignant rhabdoid tumor. Approximately 25% of patients with malignant rhabdoid tumors have proteinuria; this prevalence is similar to that of patients with Wilms tumors.
    • Serum calcium measurement: As many as 25% of patients with malignant rhabdoid tumor present with hypercalcemia. This finding is attributed to the ectopic production of parathyroid hormone-related protein by the tumor. Hypercalcemia is uncommon in Wilms tumor but is associated with congenital mesoblastic nephroma.
  • Liver function test results may be abnormal in infants and children with primary hepatic malignant rhabdoid tumor or in the case of hepatic metastases from a renal or soft tissue primary malignant rhabdoid tumor.
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Imaging Studies

  • No pathognomonic imaging feature aids in distinguishing malignant rhabdoid tumor from the other renal tumors of childhood. However, several features may raise the suspicion for malignant rhabdoid tumor.
  • The following imaging studies are suggested for the diagnosis and staging of malignant rhabdoid tumor:
    • Abdominal CT: Malignant rhabdoid tumor typically appears as a large, lobulated mass in the center or periphery of the kidney. The margins of the tumor may be sharply defined from the adjacent renal parenchyma, or they may be indistinct. Tumoral lobules are often separated by hypoattenuating areas of hemorrhage or necrosis.
      • Calcification, seen in about 10% of Wilms tumors and rarely seen in clear cell sarcoma or congenital mesoblastic nephroma, occurs frequently in malignant rhabdoid tumor. Malignant rhabdoid tumor–associated calcifications are often linear or curvilinear, and they may outline tumor lobules, as is shown in the image below. Nonenhanced CT scan demonstrates linear and curvilNonenhanced 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.
      • A peripheral, subcapsular, crescent-shaped fluid collection is often seen in association with malignant rhabdoid tumor, as is shown in the image below. Contrast-enhanced CT scan demonstrates a subcapsulContrast-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.
      • In one study, this finding was present in 15 of 21 patients (71%) with malignant rhabdoid tumor but was present in only 8 of 93 patients (9%) with Wilms tumors, 6 of 44 patients (14%) with congenital mesoblastic nephromas, and 3 of 12 patients (25%) with clear cell sarcomas. These subcapsular fluid collections may be due either to hemorrhage or tumor necrosis.
    • Chest CT: Lungs remain the most common site of metastatic disease from non-CNS malignant rhabdoid tumor; the lungs are involved in 83% of infants and children with metastatic malignant rhabdoid tumor at diagnosis. When present, lung disease tends to be bilateral and unresectable.
    • Abdominal ultrasonography: Tumoral invasion of the renal vein and/or the inferior vena cava is sometimes seen with malignant rhabdoid tumor and is best diagnosed with Doppler ultrasonography or magnetic resonance angiography.
    • MR, CT, FDG, and/or PET imaging of the brain: Imaging of the head is indicated to exclude the possibility of a synchronous primary or metastatic brain tumor.[11]
    • Bone Scan: Bone metastases are present in 5% of infants and children with metastatic malignant rhabdoid tumor at diagnosis. As such, the COG's current malignant rhabdoid tumor research protocol now requires bone scan at diagnosis. Whether all children with malignant rhabdoid tumor require a bone scan at diagnosis is unclear.
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Other Tests

  • The finding of germline perturbations in SMARCB1, assessed via genetic study of patient lymphocyte DNA, confirms a rhabdoid tumor predisposition and is estimated to occur in approximately 15-30% of patients with malignant rhabdoid tumor.
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Procedures

  • Tumor tissue sampling is required to make the diagnosis of malignant rhabdoid tumor and assists in defining the genetic background.
  • Bone marrow aspiration and biopsy are not routinely necessary in the workup of malignant rhabdoid tumor because malignant rhabdoid tumor rarely metastasizes to the bone marrow.
  • Lumbar puncture is not routinely indicated unless a CNS tumor is diagnosed.
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Histologic Findings

  • On gross examination, MRTs are heterogeneous, bulky, lobulated, friable, solid, gray-tan masses with areas of necrosis and hemorrhage.
  • On microscopic examination, malignant rhabdoid tumors are characterized by sheets or solid trabeculae of large tumor cells with vesicular chromatin, nuclei with prominent cherry-red nucleoli, moderate amounts of eccentric eosinophilic cytoplasm, and a distinctive, globoid, hyaline pink intracytoplasmic inclusion, as is shown in the image below. Histology of malignant rhabdoid tumors (MRTs). ThiHistology 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).
  • Mitoses are frequent and necrosis is common. A subset of tumors may be composed predominantly of primitive undifferentiated small round blue cells, but, upon closer inspection, small foci of cells with diagnostic cytologic features can be identified. Other patterns described as sclerosing (including chondroid), epithelioid, spindled, lymphomatoid or histiocytoid, and vascular may coexist with the classic pattern. Unlike a Wilms tumor, malignant rhabdoid tumor of the kidney typically has an infiltrative border with the surrounding nonneoplastic cortex and renal medulla.
  • The most useful ultrastructural finding is a large whorl of intermediate filaments in the cytoplasm with a diameter of 8-10 nm, correlating with the rhabdoid inclusions seen with light microscopy. Dilated rough endoplasmic reticulum, rudimentary cell junctions, and cytoplasmic tonofilamentlike bundles are other characteristic features. The cells do not have external lamina or evidence of myogenic differentiation.
  • On immunohistochemical examination, the tumor cells are polyphenotypic with consistent staining for vimentin, and most are positive for epithelial membrane antigen and/or cytokeratin. Positivity for glial fibrillary acidic protein, neuron-specific enolase, smooth muscle actin, desmin, CD99, and other markers has been seen in malignant rhabdoid tumor. Malignant rhabdoid tumor lacks INI1 immunohistochemical staining, as is seen in the image below, whereas most other tumors have detectable INI1 protein. INI1 immunohistochemistry stain shows diffuse lossINI1 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).
  • Loss of INI1 protein expression correlates closely with biallelic SMARCB1 gene perturbation, assessed via molecular genetic testing. Therefore, INI1 immunohistochemical studies can be used in conjunction with other studies to confirm the histologic diagnosis of malignant rhabdoid tumor.
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Staging

  • In North America, malignant rhabdoid tumors are staged according to the staging system of the NWTS Group, which the COG has modified.
  • The COG staging system is as follows:
    • Stage I: Tumor is limited to the kidney and completely excised. The renal capsule is intact. The tumor is not ruptured or sampled for biopsy before it is removed. (Fine-needle aspiration is excluded from this restriction.) The vessels of the renal sinus are not involved. No evidence suggests tumor at or beyond the margins of resection.
    • Stage II: The tumor extended beyond the kidney, but it was completely excised. The tumor may regionally extend into the renal sinus or penetrate the renal capsule. Blood vessels outside the renal sinus may contain tumor, but the tumor must be removed en bloc with the tumor. No evidence of tumor at or beyond the margins of resection is present.
    • Stage III: Residual nonhematogenous tumor is confined to the abdomen. Any of the following may occur: (1) Tumor involves abdominal lymph nodes. (2) The tumor has penetrated the peritoneal surface. (3) Tumor implants are found on the peritoneal surface. (4) Gross or microscopic tumor remains after surgery. (5) The tumor is not completely resectable because of local infiltration of vital structures. (6) Tumoral spillage occurs before or during surgery. (7) Tumor biopsy was performed before resection.
    • Stage IV: Hematogenous metastases or lymph node metastases are present outside the abdominal and/or pelvic cavity.
    • Stage V: Tumors are bilateral.
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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.

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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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