TABLE 22-1: PRESENTING SIGNS AND SYMPTOMS OF PEDIATRIC MALIGNANCIES

TABLE 22-1

PRESENTING SIGNS AND SYMPTOMS OF PEDIATRIC MALIGNANCIES^1,3,5

Type of Malignancy	Risk Factors/Patient Characteristics	Signs/Symptoms	Initial Workup
Leukemia	ALL: white race, radiation exposure AML: familial monosomy 7 Both: NF type 1, Down syndrome	Limp, hepatomegaly, splenomegaly, petechiae/bruising, bone pain, anemia, thrombocytopenia	BMA, LP, laboratory studies including morphology and flow cytometry
Lymphoma	HD and Burkitt's lymphoma: EBV, more common in adolescence NHL: immunodeficiency, more common in infants and school-aged children	Night sweats; pruritus; stridor; persistent respiratory symptoms; GI bleeding; back pain; hepatomegaly; splenomegaly; abdominal, head, neck, or chest mass	Imaging (CT chest/abdomen/pelvis), bone scan, LP, BMA, ferritin/LDH/uric acid/ESR
Wilms’ tumor	1–5 yr old (peak at 2–3 yr) Associated with some congenital anomalies Aniridia, Beckwith Wiedemann	Hypertension, abdominal distention, hematuria	Imaging (CT chest/abdomen/pelvis), echocardiogram, abdominal ultrasound with Doppler
Neuroblastoma	Peak incidence 1–5 yr old	Emesis; diarrhea; hypertension; opsoclonus-myoclonus; periorbital ecchymoses; Horner syndrome; stridor; persistent respiratory symptoms; abdominal, head, neck or chest mass; limp; blue subcutaneous nodules	Imaging (CT/MRI for staging), BMA, echocardiogram, urine catecholamines (HVA/VMA)
CNS tumors	Optic glioma: NF type I, other genetic syndromes Astrocytoma: full age range Ependymoma and medulloblastoma: more common in infants, school-aged children Craniopharyngioma: peak incidence 8–10 yr old	Irritability, headache, emesis, seizure, cranial nerve palsies, visual changes, proptosis, ataxia	MRI of brain and spine, lumbar puncture (cytopathology)
Testicular tumors	Adolescent age Cryptorchidism	Abdominal pain or tenderness, scrotal swelling or mass	Imaging (CT chest/abdomen/pelvis), serum β-hCG, AFP, LDH, uric acid
Bone tumors	Osteosarcoma: previous treatment with alkylating agents or radiation therapy, Li Fraumeni, hereditary Retinoblastoma Ewing's sarcoma: Caucasian race Both: adolescent age	Limp, back pain, persistent limb pain, fracture	Imaging (CT chest, primary site), x-ray primary site, bone scan; bilateral BMA (Ewing's sarcoma)
Histiocytic disease	Familial disease: infancy	Polyuria, polydipsia, otorrhea, hepatomegaly, splenomegaly, cutaneous lesions, osteolytic lesions, pulmonary infiltrates, anemia, thrombocytopenia	LDH/ferritin/uric acid, skeletal survey, BMA, chest x-ray
Retinoblastoma	<5 yr old, genetic associations	Leukocoria, asymmetrical red reflex, orbital inflammation, hyphema, pupil irregularity	Brain MRI, lumbar puncture for CSF
Rhabdomyosarcoma	More common in children >5 yr and adolescents Li Fraumeni, NF type 1	Symptoms differ based on location of tumor but bone pain, anemia, thrombocytopenia, neutropenia and respiratory symptoms are common with metastatic disease	Laboratory studies including LFTs, imaging based on location of suspected disease (CT, MRI, ultrasound), chest x-ray and bone scan to evaluate for metastatic disease
Hepatoblastoma	Peak incidence <3 yr old Beckwith Wiedemann, FAP	Abdominal pain and distention, anorexia, fatigue, thrombocytosis	Imaging of abdomen, laboratory studies, β-hCG, AFP

AFP, Alpha fetoprotein; ALL, acute lymphocytic leukemia; AML, Acute myeloid leukemia; BMA, bone marrow aspirate; CNS, central nervous system; CSF, cerebrospinal fluid; CT, computed tomography; ESR, erythrocyte sedimentation rate; FAP, familial adenomatous polyposis; GI, gastrointestinal; HD, Hodgkin's disease; β-hCG, beta human chorionic gonadotropin; HVA/VMA, urine catecholamines; LDH, lactic dehydrogenase; LFT, liver function tests; LP, lumbar puncture; MRI, magnetic resonance imaging; NF, neurofibromatosis; NHL, non-Hodgkin's lymphoma.