Pediatric Cancers leukemia ALL Blineage T lineage AML

Pediatric Cancers

leukemia - ALL -- > B-lineage & T- lineage - AML - CML

Clinical manifestation - Pallor - Arthritis - Bleeding - Lymphadenopathy - Extremity pain - Lethargy & anorexia - Fever - Malaise

Physical Examination - Lymphadenopathy - Hepatosplenomegaly - Painless enlargement of testes T-cell ALL: - High WBC - Anterior mediastinal mass - Bulky disease ( CNS envolvement) AML: - Chloroma

Laboratory - Neutropenia - Anemia - Thrombocytopenia WBC : low, normal or high t( 12, 21) favorable prognosis t( 9, 22) and t( 4, 11) poor prognosis

Differential Diagnosis - Non malignant disease: infections: EBV, CMV, Pertussis, mycobacteria noninfectious : aplastic anemia, histiocytosis, juvenile rheumatoid arthritis, ITP - Malignant disease: malignant diagnosis can metastasis to BM: neuroblastoma, rhabdomyosarcoma, Ewing sarcoma transient myeloproliferative disorder

Transient myeloproliferative disorder: -in trisomy 21 - High WBC, anemia, thrombocytopenia, peripheral blasts Treatment: Supportive care

prognosis

Treatment CHEMOTHERAPY

Lymphoma has two major types: - Hodgkin Disease - Non Hodgkin Lymphoma( NHL) EBV plays a causal role in both

NHL: Are diffuse, highly malignant & show little differentiation NHL has 3 histologic subtypes: - Burkitt lymphoma - Lymphoblastic - large cell B-cell or T-cell

HD: PEAKS IN ADOLESCENT/ADULTS & AFTER 50 Y/O NHL : IN ASSOCIATION WITH CONGENITAL OR ACQUIRED IMMUNODEFICIENCY & AFTER ORGAN OR STEM CELL TRANSPLANTATION.

HD: Pathologic hallmark is Reed sternberg cell Histologic subtypes: - Lymphocyte predominant - Nodular sclerosis - Mixed cellularity - Lymphocyte depletion

Clinical manifestations -lymphadenopathy -Anemia -neutropenia Bleeding - B symptoms: • fever( > 38 c for 3 consecutive days) • Drenching night sweet • Unintentional weight loss of 10% or more within 6 months

Burkitt : - Abdominal (sporadic form, in America) jaw( endemic form, in Africa) SVC syndrome: Mediastinal & cervical nodes T-cell lymphoma Air way & superior vena cava obstruction

DIFFERENTIATION DIAGNOSIS: -LEUKEMIA, RHABDOMYOSARCOMA, NASOPHARYNGEAL CARCINOMA, GERM CELL TUMORS, THYMOMA - NONMALIGNANT : EBV INFECTION, BRANCHIAL CLEFT& THYROGLOSSAL DUCT CYSTS, CAT SCRATCH DISEASE, BACTERIAL OR VIRAL LYMPHADENITIS, MYCOBACTERIA, TOXOPLASMOSIS

TREATMENT : HD: COMBINATION OF CHEMOTHERAPY & LOW DOSE RADIATION THERAPY NHD: CHEMOTHERAPY

CNS Tumors - Primary tumors - Secondary tumors

Clinical Manifestation Increased intracranial pressure: lethargy, headache, vomiting( morning) Seizure Increase in head circumference Loss of visual activity or visual field defects Inability to abduct the eyes( 6 th nerve palsy) Other nerve palsy( involvement of Brainstem) Irritability, anorexia, poor school performance, loss of developmental milestone Galactorrhea, excessive growth, precocious puberty( pituitary) Ataxia, loss of coordination( cerebellar)

LABORATORY: MRI & CSF DIFFERENTIAL DIAGNOSIS OF CNS MASS: MALIGNANT TUMOR, BENIGN TUMOR, ARTERIOVENOUS MALFORMATION, ANEURYSM, BRAIN ABSCESS, CYSTICERCOSIS, GRANULOMATOSIS DISEASE (TUBERCULOSIS , SARCOID), INTRACRANIAL HEMORRHAGE, PSEUDOTUMOR CEREBRI, VASCULITIS & METASTATIC TUMOR.

Neuroblastoma NB derived from neural crest cells adrenal medulla & sympathetic nervous system. Median age at diagnosis is 20 months.

Clinical Manifestation Abdominal mass or pain Fever, weight loss Neck mass Abnormal eye movements, involuntary movements, ataxia, Lymphadenopathy Pancytopenia Hypercalcemia

Paraneoplastic syndrome: secretory diarrhea, profuse sweeting, opsomyoclonus( dancing eyes& dancing feet) Periorbital ecchymosis Stage 4 s: is associated with favorable outcome Infants < 1 y/o Small primary tumor Metastasis to: skin, liver, bone marrow,

Diagnosis Calcification within abdominal NB Vanillylmandelic acid( VMA) & Homovanillic acid (HVA) in urine biopsy of tumor CT scan Bone marrow aspiration & biopsy

DIFFERENTIAL DIAGNOSIS: WILMS TUMOR TREATMENT: - SURGERY - CHEMOTHERAPY - STEM CELL TRANSPLANTATION

Wilms Tumor of kidney WT 1 gene

Clinical Manifestation Abdominal mass Fever Abdominal pain Hypertension hematuria

DIFFERENTIAL DIAGNOSIS: - HYDRONEPHROSIS - POLYCYSTIC DISEASE - BENIGN RENAL TUMORS - OTHER MALIGNANT TUMORS: RENAL CELL CARCINOMA, NEUROBLASTOMA, LYMPHOMA, RETROPERITONEAL RHABDOMYOSARCOMA

TREATMENT: - NEPHRECTOMY - CHEMOTHERAPY - RADIATION THERAPY

Sarcoma Soft tissue sarcoma: Rhabdomyosarcoma, extraosseous Ewing sarcoma, fibrosarcoma, synovial sarcoma Bone cancers: osteosarcoma, Ewing sarcoma

Clinical Manifestation Rhabdomyosarcoma : Periorbital swelling, proptosis, ear discharge, dysphagia, chronic otitis media, cranial nerve involvement, urethral or vaginal mass, paratesticular swelling, hematuria, urinary frequency or retention

Osteosarcoma: distal femur, proximal tibia, proximal humerus Pain Palpable mass Lytic lesion, often with calcification in soft tissue surrounding the lesion

Ewing sarcoma: In any bone Most common: femur & pelvic Local pain & swelling Fever & weight loss