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NEPHROBLASTOMA and NEUROBLASTOMA

NEPHROBLASTOMA and NEUROBLASTOMA

WILMS,TUMOUR (NEPHROBLASTOMA)

 

  1. mean age at diagnosis 3-3 1/2 years, M=F
  2. 5% of all childhood cancers 1/3 hereditary and 2/3 sporadic
  3. associated with a number of congenital abnormalities:  sporadic
    anridia (often with 11p13 deletion), hemihypertrophy, genitourinary
    abnormalities

presentation

  1. 80% with asymptomatic abdominal mass
  2. hypertension, hematuria

 

differential diagnosis: 

 

  1. Hydronephrosis
  2. polycystic kidney
  3. renal cell
  4. carcinoma
  5. neuroblastoma
  6. lymphoma

 

Management

 

  1. nephrectomy
  2. staging
  3. chemotherapy (pre- or post-op)
  4. radiation generally good prognosis


NEUROBLASTOMA

 

  1. neural crest cell tumour arising from sympathetic tissues of the
    adrenal medulla (45%) or the sympathetic chain (25% retroperitoneal, 20% posterior mediastinal, 4% pelvis, 4% neck)
  2. most common malignancy in infancy, median age of onset 20 months

Presentation

 

  1. abdominal mass (most common), neck mass, chest mass (may be incidental finding on chest x-ray)
  2. direct extension: spinal cord compression, Horner syndrome blueberry muffin skin nodules
  3. metastases:periorbital ecchymosis, bone pain, hepatomegaly, blueberry muffin skin nodules
  4. paraneoplastic: hypertension, diarrhea (VIP secretion), opsoclonus, myoclonus

Diagnosis and Staging

 

  1. LFTs, renal function tests, serum ferritin
  2. VMA, HVA
  3. urine CT scan chest, abdomen
  4. bone scan bone marrow exam - for neuroblastoma cells in "rosettes"
  5. tissue biopsy


Management

 

  1. surgery, radiation, chemotherapy +/-
  2. bone marrow transplantation

Good Prognostic Factors 

 

  1. < 1 year old 
  2. female
  3. primary site - posterior mediastinum and neck
  4. stage I, II, IVS disease 
  5. low serum ferritin
  6. VMA/HVA ratio > 1
  7. aneuploidy no N-myc oncogene amplification

NEPHROBLASTOMA and NEUROBLASTOMA NEPHROBLASTOMA and NEUROBLASTOMA Reviewed by Radiology Madeeasy on September 05, 2010 Rating: 5
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