Paediatric solid tumors Year 4 Paediatrics Notes OmpathStudy

Paediatric solid tumors Year 4 · Paediatrics Collection: 4TH YEAR ALL NOTES / PAEDS / PAEDS 4.3 / Munge / ONCOLOGY -- Slide 1 Paediatric Solid Tumors Dr Rose Munge Slide 2 Outline Introduction Types of paediatric solid tumors Management Specific Tumors Slide 3 Introduction Solid tumors in account for about 30% of all cancers seen in paediatrics Advances in diagnosis and treatment modalities have significantly improved mortality rates Future is bright with advances in molecular genetics Slide 4 Types of Solid Tumors Data from American cancer society Figures in brackets are local (KNH) Common types: Brain tumours 22.3% (3.8) \nNephroblastoma 5.6% (8.5) Rhabdomyosarcoma 3.1% (5.2) Neuroblastoma 7.3% (3.3) Ewing sarcoma 1.4%\nOsteogenic sarcoma 2.4% (1.3) Retinoblastoma 2.8% (5.7) Kaposi’ s sarcoma (1.5) Others e.g. Hepatic tumours, other renal tumours Slide 5 Approach to Management History Physical Examination Laboratory studies\nRadiological studies Slide 6 Retinoblastoma Retinoblastoma is the most common primary ocular malignancy of childhood. It is a small, round, blue cell tumor with rosette formation and may arise in any of the nucleated layers of the retina and exhibits various degrees of differentiation;\n It tends to outgrow its blood supply, resulting in necrosis and calcification. Slide 7 Retinoblastoma Retinoblastoma occurs at a rate of 3.7 cases/million in the United States, with no racial or gender predilection.\nWorldwide, the incidence of retinoblastoma is recorded to be about 11 cases per million children younger than 5 years. \nAbout 60% of cases are unilateral and nonhereditary while 15% and unilateral and hereditary, and 25% bilateral and hereditary.\nBilateral involvement is found in 42% of those presenting when younger than 1 yr of age but in only 21% of those presenting during 1 yr of age and is even less common at older ages of presentation. Slide 8 Endophytic tumors arise from the inner surface of the retina, grow into the vitreous, and tend to seed to other areas of the retina.\nExophytic tumors grow from the outer retinal layer and may produce retinal detachment. Hematogenous or lymphatic spread to more distant sites can occur with extension to the choroid or along the optic nerve beyond the lamina cribrosa.\nDiffuse infiltrating growth-This is a rare subtype comprising 1.5% of all retinoblastomas,characterized by a relatively flat infiltration of the retina by tumor cells but without a discrete tumor mass. The obvious white mass seen in typical retinoblastoma rarely occurs. It grows slowly compared with typical retinoblastoma. Slide 9 Pathogenesis The hereditary form is associated with a germline defect in the retinoblastoma gene (RB1) located on the long arm of chromosome 13.\nRB1 encodes a tumor suppressor protein. Familial cases are generally multifocal and bilateral, whereas nonfamilial cases tend to have unilateral, unifocal involvement.\nAccording to the "two-hit" model, two mutational events are required for tumor development. In the heritable form, one mutated RB gene is inherited through the germ line and a second mutation occurs subsequently in the somatic retinal cell.\nIn the noninherited form, both mutations occur in retinal cells. Slide 11 Clinical Manifestations Only about 10% of retinoblastomas are detected by routine ophthamological screening.\nRetinoblastoma classically presents with leukocoria, a white pupillary reflex, often first noticed when a red reflex is not present at routine newborn or well-child examination or in a flash photograph of the child.\nStrabismus is commonly the initial presenting complaint.\nOrbital inflammation, hyphema, or pupil irregularity occurs with advancing disease.\nPain is usually a feature if secondary glaucoma is present. Slide 13 Leukocoria - 60% Strabismus - 20% Secondary glaucoma Anterior segment invasion Orbital inflammation Orbital invasion Presentations of retinoblastoma Slide 15 Management of Retinoblastoma The diagnosis is established by characteristic ophthalmologic findings.\nEUA by an ophthalmologist is required to obtain a complete visualization of both eyes and facilitates photographing and mapping of the tumors.\nRetinal detachment or vitreous hemorrhage can complicate the evaluation.\nOrbital ultrasonography and CT or MRI are used to evaluate the extent of intraocular disease and extraocular spread.\nOccasionally, a pineal area tumor will be detected, a phenomenon known as trilateral retinoblastoma. Slide 16 MRI allows for better evaluation of optic nerve involvement.\n Bone scan, cerebrospinal fluid evaluation, and bone marrow evaluation are necessary only if indicated by other clinical, laboratory, or imaging studies.\n The differential diagnosis includes hyperplastic primary vitreous, Coats disease, cataract, visceral larva migrans, choroidal coloboma, and retinopathy of prematurity. Slide 17 Treatment The treatment is determined by the size and location of the tumor, with the primary goal being cure and a secondar