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Pediatrics: Renal and Adrenal Oncology
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=== Diagnosis and Evaluation === ==== History and Physical Exam ==== * '''Clinical manifestations vary widely''' ** '''<span style="color:#ff0000">Most children have abdominal pain or a palpable mass''' *** '''Most primary tumors arise within the abdomen (65%)''' **** Physical examination often reveals a fixed, hard abdominal mass *** '''Pelvic neuroblastoma arising from the organ of Zuckerkandl account for 4% of tumors.''' **** Extrinsic compression of the bowel and bladder can produce symptoms of urinary retention and constipation. ** '''<span style="color:#ff0000">Other are identified because of manifestations of metastatic disease, including bone or joint pain and periorbital ecchymosis.''' *** '''Metastases are present in 70% of patients with neuroblastoma at diagnosis''' ** '''<span style="color:#ff0000">Symptoms produced by catecholamines''' *** '''<span style="color:#ff0000">Release may mimic those seen in pheochromocytoma: paroxysmal hypertension, palpitations, flushing, and headache''' *** '''Secretion of vasoactive intestinal peptide (VIP) can produce severe watery diarrhea and hypokalemia''' ==== Labs ==== * '''<span style="color:#ff0000">Urinary metabolites of catecholamines, vanillylmandelic acid (VMA) and homovanillic acid (HVA)''' **'''Increased levels are found in 90-95% of patients''' *** These metabolites can be monitored to detect tumor relapse and response to therapy * '''CBC''' **'''Anemia''' '''is found with widespread bone marrow involvement''' [[File:Neuroblastoma 103.jpg|alt=Axial CT scan of Neuroblastoma|thumb|500x500px|Axial CT scan of Neuroblastoma. Source: [[commons:File:Neuroblastoma_103.jpg|Wikipedia]]]] ==== Imaging ==== * '''<span style="color:#ff0000">CT or MRI''' ** '''Provide more information about the local extent of the primary tumors and vascular involvement''' ** '''Advantages of MRI over CT''' *** '''Evaluating intraspinal tumor extension, which is not uncommon in paravertebral lesions''' *** Demonstrating the relationship between the major vessels and the tumor ** '''<span style="color:#ff0000">CT findings''' **# '''<span style="color:#ff0000">Crossing midline''' **# '''<span style="color:#ff0000">Vascular encasement''' **# '''<span style="color:#ff0000">Intra-tumoral stippled calcifications''' **#* '''Note that egg-shell calcifications are found in adrenal hemorrhage, which may also present in the neonate as an abdominal mass''' *** '''<span style="color:#ff0000">These imaging characteristics are absent in Wilms tumor''' *'''Plain radiographs''' ** May demonstrate a '''calcified abdominal or posterior mediastinal mass''' * '''Radionuclide bone scan and metaiodobenzylguanidine (MIBG) scans for can be used staging''' ==== Other ==== * '''Bone marrow biopsies''' **'''Marrow biopsies add substantially to the detection of marrow involvement by tumor,''' compared with marrow aspirates alone. *** '''2 marrow aspirates and 2 biopsies are recommended'''
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