WILMS,TUMOUR (NEPHROBLASTOMA) mean age at diagnosis 3-3 1/2 years, M=F 5% of all childhood cancers 1/3 hereditary and 2/3...
WILMS,TUMOUR (NEPHROBLASTOMA)
- mean age at diagnosis 3-3 1/2 years, M=F
- 5% of all childhood cancers 1/3 hereditary and 2/3 sporadic
- associated with a number of congenital abnormalities: sporadic
anridia (often with 11p13 deletion), hemihypertrophy, genitourinary
abnormalities
presentation
- 80% with asymptomatic abdominal mass
- hypertension, hematuria
differential diagnosis:
- Hydronephrosis
- polycystic kidney
- renal cell
- carcinoma
- neuroblastoma
- lymphoma
Management
- nephrectomy
- staging
- chemotherapy (pre- or post-op)
- radiation generally good prognosis
NEUROBLASTOMA
- 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) - most common malignancy in infancy, median age of onset 20 months
Presentation
- abdominal mass (most common), neck mass, chest mass (may be incidental finding on chest x-ray)
- direct extension: spinal cord compression, Horner syndrome
- metastases:periorbital ecchymosis, bone pain, hepatomegaly, blueberry muffin skin nodules
- paraneoplastic: hypertension, diarrhea (VIP secretion), opsoclonus, myoclonus
Diagnosis and Staging
- LFTs, renal function tests, serum ferritin
- VMA, HVA
- urine CT scan chest, abdomen
- bone scan bone marrow exam - for neuroblastoma cells in "rosettes"
- tissue biopsy
Management
- surgery, radiation, chemotherapy +/-
- bone marrow transplantation
Good Prognostic Factors
- < 1 year old
- female
- primary site - posterior mediastinum and neck
- stage I, II, IVS disease
- low serum ferritin
- VMA/HVA ratio > 1
- aneuploidy no N-myc oncogene amplification
