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P46 Pediatrics
Oncology Toronto Notes 2019
• non-Hodgkinlymphoma
■ generally categorized into lymphoblastic, diffuse large B cell, Burkitt’s lymphoma, and anaplastic
large cell
■ rapidly growing tumour with distant metastases (unlike adult non-Hodgkin lymphoma)
■ signs and symptoms related to disease site: most commonly abdomen, chest (mediastinal mass),
head and neck region
Management
• Hodgkinlymphoma
■ combination chemotherapy and radiation
■ aimed at limiting cumulative doses of anthracyclines (toxic to heart) and alkylators (risk of second
malignancy, infertility) and limiting dose and field of radiation
■ increasing role for use of PET scanning to assess early disease response and plan therapy
• non-Hodgkinlymphoma
■ combinationchemotherapy
■ no added benefit of radiation in pediatric protocols
Prognosis
• Hodgkinlymphoma:>90%5yrsurvival
• non-Hodgkinlymphoma:75-90%5yrsurvival
Brain Tumours
• seeNeurosurgery,NS38
Wilms’ Tumour (Nephroblastoma)
Epidemiology
• usuallydiagnosedbetween2-5yr;M=F
■ most common primary renal neoplasm of childhood
■ 5-10% of cases both kidneys are affected (simultaneously or in sequence)
Differential Diagnosis
• hydronephrosis,polycystickidneydisease,renalcellcarcinoma,neuroblastoma
Clinical Presentation
• 80%presentwithasymptomatic,unilateralabdominalmass
• mayalsopresentwithHTN,grosshematuria,abdominalpain,vomiting
• mayhavepulmonarymetastasesattimeofdiagnosis(respiratorysymptoms)
Associated Congenital Abnormalities
• WAGRsyndrome(Wilms’tumour,Aniridia,Genitalanomalies,mentalRetardation)with11p13deletion • Beckwith-Wiedemannsyndrome:
■ characterized by enlargement of body organs (especially tongue), hemihypertrophy, renal medullary cysts, and adrenal cytomegaly
■ also at increased risk for developing hepatoblastoma, and less commonly adrenocortical tumours, neuroblastomas, and rhabdomyosarcomas
• Denys-Drashsyndrome:characterizedbygonadaldysgenesisandnephropathyleadingtorenalfailure
Management
• staging±nephrectomy
• chemotherapy,radiationforhigherstages
Prognosis
• 90%long-termsurvival
Neuroblastoma
Epidemiology
• mostcommoncanceroccurringinfirstyearoflife
• neuralcrestcelltumourarisingfromsympathetictissues(neuroblasts)
Clinical Presentation
• canoriginatefromanysiteinsympatheticnervoussystem,presentingasmassinneck,chest,or abdomen (most common site is adrenal gland)
• signsandsymptomsofdiseasevarywithlocationoftumour ■ thoracic: dyspnea, Horner’s syndrome
■ abdomen: palpable mass
■ spinal cord compression
Constitutional symptoms = fever, chills, night sweats, unexplained weight loss