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A. Major Causes

  1. Renal
    1. Multicystic kidney - most common; unilateral
    2. Polycystic kidney - bilateral (Juvenile Form, that is, recessive)
    3. Neuroblastoma
    4. Wilms' Tumor
  2. Lymphoma - especially Burkitt's

B. Neuroblastoma [7]

  1. Sympathetic ganglion (adrenal or sympathetic chain) derived tumor
  2. Derived from neural crest cells
  3. Most common extracranial solid tumor of childhood
  4. Behavior
    1. Spontaneous regression does occur in ~5% (usually disseminated, Stage IVS)
    2. Loss of heterozygosity (LOH) or loss of chromosome 1p36 is predictor of unfavorable outcome [1,3]
    3. In addition, N-myc amplification and duplication of 17q were unfavorable [1]
    4. N-myc and (separately) MRP are independent, strong risk factors for poor outcome [2]
    5. Unbalanced chromosome 11q LOH associated with poor progression free survival [3]
    6. Any gain of function of chromosome arm 17q is highly unfavorable to outcome [4]
    7. N-myc amplification correlates with multidrug resistance protein (MRP) levels [2]
  5. Symptoms
    1. Gatrointestinal: abdominal pain, distension, vomiting, diarrhea
    2. Local tumor growth can cause specific symptoms due to mass effects
    3. May present as nasal mass [8]
  6. Radiographic Evaluation
    1. Plain Radiographs: calcification occurs in ~50%
    2. On intravenous pyelogram (IVP), see displaced / deformed kidney
    3. Computerized Tomographic / MRI scanning - define extent of tumor pre-operatively
  7. Therapy [5]
    1. Multi-modality therapy is generally used
    2. Extensive, often difficult surgical resection
    3. Chemotherapy
    4. Radiation therapy
    5. Intensive chemotherapy + radiotherapy with bone marrow transplantation
    6. Addition of 13-cis-retinoic acid to high risk patients improves survival [5]
  8. Survival
    1. Thoracic Tumors 90%
    2. Abdominal Tumors 25%

C. Wilms' Tumor [6]

  1. Tumor of fetal kidney tissue
  2. Abdominal Pain, fever, hematuria, mass
  3. IVP shows distortion and displacement of pelvises and calices
  4. Survival
    1. Non-metastatic - 90%
    2. Metastatic - 40-50%
  5. Surgery, chemotherapy, and radiation are used

References

  1. Caron H, van Sluis P, de Kraker J, et al. 1996. NEJM. 334(4):225 abstract
  2. Norris MD, Bordow SB, Marshall GM, et al. 1996. NEJM. 334(4):231 abstract
  3. Attiyeh EF, London WB, mosse YP, et al. 2005. NEJM. 353(21):2243 abstract
  4. Bown N, Cotterill S, Lastowska M, et al. 1999. NEJM. 340(25):1954 abstract
  5. Matthay KK, Villablanca JG, Seeger RC, et al. 1999. NEJM. 341(16):1165 abstract
  6. Coppes MJ, Haber DA, Grundy PE. 1994. NEJM. 331(9):586 abstract
  7. Maris JM, Hogarty MD, Bagatell R, Cohn SL. 2007. Lancet. 369(9579):2106 abstract
  8. Cunningham MJ, Lin DT, Curry WT Jr., et al. 2007. NEJM. 356(26):2721 (Case Record) abstract