د.عبد الرحمن عبد العزيزاستشاري الجراحة العامة وجراحة الاطفال
Leukemia is the most common childhood cancer Brain tumors are second most common Lymphomas are the third most common Then solid tumors outside the CNS Neuroblastoma - neural crest derived Wilms - renal tumors and syndromes Bone tumors Rhabdomyosarcoma - soft tissue sarcomas Sacrococcygeal teratoma
Brain Tumors
Childhood CancersOther
Lymphoma
Neuroblastoma
Soft tissue sarcomas
Kidney tumors
Bone tumors
Retino- blastoma
Leukemia
Definition and General Characteristics Uncontrolled proliferation of immature white blood cells with a different immunologicalsubtype which is lethal within 1–6 months without treatment The disorder starts in the bone marrow, where normal blood cells are replaced byleukemic cells Morphological, immunological, cytogenetic, biochemical, and molecular geneticfactors characterize the subtypes with various responses to treatment
Bone marrow infiltrationAnemiaPallor, lethargyDyspnea, murmur Platelets Bleeding, petechiae, purpura Neutropenia Fevers and infectionsBone pain Limp, walking, irritability
Extramedullary spread Lymphadenopathy Hepatosplenomegaly Orthopnea, cough mediastinal mass tracheal compression Facial nerve palsy Testicular enlargement Skin lesions Gingival hypertrophy
Fever of malignancy
Histogenesis: * Cell of origin: glial, neural, primitive, choroid, mixed * Location: posterior fossa: 70% supratentorial: 30% * Clinical presentation:locationagetype and grade of the tumor
Infratentorial 70% esp. < 6 y/o Supratentorial 30% esp. > 8 y/o
Symptoms may include: Increased intracranial pressure secondary to obstruction of CSF at aqueduct hydrocephalus (infants), headache, papilledema, vomiting seizures focal neurological deficits hormonal changes (pituitary adenoma) visual changes (diplopia, field defects) pressure on optic chiasmSigns and Symptoms depend on:Lymphoma subtypeHodgkin’s Disease (HD)Non Hodgkin’s Lymphoma (NHL)* Lymphoblastic * Burkitt’s* Large Cell lymphomaLocation
Age: adolescents >> young childPainless lymphadenopathyProgresses over weeks monthsLocation Cervical/supraclavicular LNSunilateral or bilateral Mediastinal ± hilumLNs below diaphragm and spleenLiver, lung, bone marrow 95%
Systemic symptoms Fevers Night sweats Weight loss Pruritus
“B” symptoms25% = Oncologic Emergencycompression
Superior Mediastinal Syndrome (SMS) Orthopnea, SOB, strider, hypoxia Tracheal Bronchial Cardiac
Presentation of Non Hodgkin's lymphoma Lymphoblastic lymphoma
B-cell origin > 5 y/o Abdominal mass Large mass + LNs Terminal ileum, Cecum or appendix Jaw Tumor lysis syndrome Uric acid, phosphorus, creatinine Treatment can precipitate renal failure= Oncologic Emergency
Most common:Burkitt’s lymphomaNeuroblastoma Wilms Tumor Other: Hepatoblastoma Rhabdomyosarcoma pelvic Ovarian germ cell tumors pelvic
Age 90% < 5 y/o; 50% < 2 y/o Occasional ultrasonography detection in utero Location: any neural crest tissue Adrenal Paraspinal sympathetic tissue Cervical, Thoracic, Pelvic Often metastatic at diagnosis Bone and/or bone marrow
Abdominal massOften crosses midlineLower extremity weakness Spinal cord compressionThoracicabdominalCervical, high thoracic massHorner’s syndromeMiosis, ptosis, anhydrosis
Signs of metastatic disease Irritability Bone pain Fever
Proptosis Bone lesions Periorbital ecchymosesPeriorbital Ecchymoses of Neuroblastoma
13 months old at diagnosis1 month into therapy
Paraneoplastic syndromesWatery diarrhea – Vasoactive Intestinal Peptide Urinary catecholamines VMA – 85% BP – 25%Renal compressionCatecholamine secretion
Abdominal mass Often asymptomatic Healthy appearing
Encapsulated mass2 days before dx
BP – 25% Mass enlarges toward pelvis Wilms tumor: Signs and Symptoms
Associated anomalies, syndromes – 15%WAGR syndromeWilms, aniridia, ambiguous genitalia, mental retardationdue to 11p13 deletion (WT-1)AniridiaAge – Adolescents > younger childrenSigns and symptomsBone pain, palpable mass, motionOften hx of sports injury (coincidental) Ewing Sarcoma All bones: Long: diaphyses Flat Pelvis Skull Ribs
Osteogenic Sarcoma Metaphyses of long bones: Distal femur Proximal tibia Proximal humerus Pelvis
Plain X-Rays are usually abnormal
Classic X-ray of Ewing: Moth-eaten lytic lesionClassic X-ray of O.S.: “Sunburst pattern” Periosteal reaction Soft tissue mass + calcium
Further radiographic evaluation may help with differential diagnosis of bone pain Bone scan MRI Chest CT scan Metastases 20%
Rhabdomyosarcoma – most commonAge Birth to > 20 y/o70% < 10 y/oSitesHead and neck – 40%Genitourinary – 20%Extremities – 20%Trunk – 10%Retroperitoneal – 10% Signs and symptoms depend on age and site
Head and neck Orbit Proptosis Periorbital swelling Parameningeal Cranial nerve palsies Hearing loss Chronic aural or sinus drainage
Rhabdomyosarcomas: Signs and Symptoms
GenitourinaryBladder and prostateHematuriaUrinary obstructionParatesticular Painless mass - testicleVagina and uterus Abdominal massVaginal massVaginal bleeding or discharge Rhabdomyosarcomas: Signs and Symptoms
Botryoid: grape-like
Rhabdomyosarcoma – other sites 6 week old
NewbornCan grow up at any site and any age
Sacrococcygeal teratoma Its a rare tumor, occurring in approximately 1 in 40,000 live births. They arise from the caudal end of the spine, usually protrudingfrom the inferior end of the infant’s spinal column and displacing the anus forwards. They are much more common in girls, with the female to male ratio being at least 3:1. It is generally agreed that sacrococcygeal teratoma is the result of continued multiplication of totipotent cells from Hensen’s node, which fail to involute at the end of embryonic life.