Bill_Chang_Oncology

Common Presentations of Childhood Cancer : 

Common Presentations of Childhood Cancer Bill Chang, MD, PhD Linda Stork, MD Division of Peds Heme/Onc/BMT OHSU February 2006

First Take Home point : 

First Take Home point Childhood Cancer is a rare disease…

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• 1 in 750 20-year-olds alive in the U.S. today is a survivor of childhood cancer. • One in every 330 Americans develops cancer before age 20. BUT…

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* Over 3,000 children die from cancer yearly in the U.S. • More than from AIDS, asthma, diabetes, and cystic fibrosis combined Earlier diagnosis and referral can impact outcome More Statistics

Second Take Home points… : 

Second Take Home points… 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

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Brain Tumors Childhood Cancers Other Lymphoma Neuroblastoma Soft tissue sarcomas Kidney tumors Bone tumors Retino- blastoma Leukemia

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Specific Signs and Symptoms Depend on * type of cancer * site(s) of disease * age of patient

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Cancer in Younger Children Neuroblastoma Retinoblastoma Wilms tumor Age (yrs) at diagnosis Average annual rate per million SEER data, 2002

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Cancer in Children and Adolescents Age (yrs) at diagnosis SEER data, 2002 Average annual rate per million ALL AML Lymphoma Brain Tumors

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Cancer in Children and Adolescents Average annual rate per million Hepatic Bone Soft tissue Germ cell Age (yrs) at diagnosis SEER data, 2002

Leukemias : 

Leukemias

Presentations of Lymphoblastic Leukemia : 

# 1 4 y/o fever x 4 days ear pain = L otitis, began Amoxicillin T = 104 + chills next day ? urgent care again PE: L TM purulent drainage bilat. cervical, L axillary, L inguinal LNs? spleen 5 cm ?, liver 3 cm ? multiple bruises extremities ? Test Presentations of Lymphoblastic Leukemia

CBC : 

CBC WBC = 33K 8% Neut, 92% lymphocytes Hb = 6.5 PLT = 40K

Peripheral smear show lymphoBLASTS : 

Peripheral smear show lymphoBLASTS

Bone marrow : 

Bone marrow

Leukemia: Signs and Symptoms : 

Leukemia: Signs and Symptoms Bone marrow infiltration Anemia Pallor, lethargy Dyspnea, ?murmur ? Platelets Bleeding, petechiae, purpura Neutropenia Fevers and infections Bone pain Limp, ? walking, irritability

Leukemia: Signs and Symptoms : 

Leukemia: Signs and Symptoms Extramedullary spread Lymphadenopathy Hepatosplenomegaly Orthopnea, cough mediastinal mass tracheal compression Facial nerve palsy Testicular enlargement Skin lesions Gingival hypertrophy • Fever of malignancy

CBC and Differential : 

CBC and Differential ? WBC - 50%; nl or ? WBC – 50% + blasts on smear in 80% ? 2 Cytopenias - 95% ? Hgb - 80% ? Plts – 90% ? % Neutrophils – 90% 1 Cytopenia - 4% Normal CBC and diff – 1% Very helpful in the diagnosis ALL

CNS Tumors : 

CNS Tumors

Presentation : 

Presentation 23mo female with ataxia X 1month NB NB Emesis, in am X 2weeks Vomiting am Seen by PCP for GERD without improvement No Fevers Increasing vomiting Test

MRI : 

MRI

MRI : 

MRI

Brain Tumors of Childhood : 

Brain Tumors of Childhood Heterogeneous * Cell of origin: glial, neural, other, combination * Location: ? posterior fossa: 50% ? supratentorial: 50% * Clinical presentation: ? location ? age

Brain Tumors of Childhood : 

Brain Tumors of Childhood Infratentorial 50% esp < 6 y/o Supratentorial 50% esp > 8 y/o

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Nonlocalizing Signs of Brain Tumors Increased intracranial pressure (ICP) Obstructed CSF flow and hydrocephalus Child is often asymptomatic until critical threshold reached Medulloblastoma Can grow very large before detection

Increased Intracranial Pressure (ICP) : 

Headaches, progressively worsening Vomiting (morning) Irritability Papilledema rare < 2 y/o - head can expand “Double vision” with 6th nerve palsy Head tilt Bulging fontanel (infant) Increased Intracranial Pressure (ICP) In a young child with ? brain tumor: Measure head circumference and observe gait

Supratentorial Tumors : 

Supratentorial Tumors Signs depend on location and age like in adults; in addition: Younger child: Developmental delay or loss of milestones Older child: Deteriorating school performance Personality changes Endocrinopathies: DI, hypothyroidism, precocious puberty

Lymphomas : 

Lymphomas

Presentation : 

Presentation 16yo female with cough X 3 weeks Fevers for 2 months Wt loss X 2 months Exam noted to have supraclavicular LAD Test

Childhood Lymphomas : 

Childhood Lymphomas Signs and Symptoms depend on: Lymphoma subtype Hodgkin’s Disease (HD) Nonhodgkin’s Lymphoma (NHL) * Burkitt’s * Lymphoblastic * Anaplastic Large Cell Location

Presentation of Hodgkin’s Disease : 

Presentation of Hodgkin’s Disease Age: adolescents >> young child Painless lymphadenopathy Progresses over weeks ? months Location Cervical/supraclavicular ? LNS unilateral or bilateral Mediastinum ± hilum LNs below diaphragm and spleen Liver, lung, bone marrow

Presentation of Hodgkin’s Disease : 

Presentation of Hodgkin’s Disease Systemic symptoms Fevers Night sweats Weight loss Pruritus = Oncologic Emergency Superior Mediastinal Syndrome (SMS) Orthopnea, SOB, stridor, hypoxia Tracheal Bronchial Cardiac

What is the Test to get? : 

What is the Test to get?

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HD in 16 y/o girl left cervical LNs, 40 # wt loss cough, no orthopnea HD in 9 y/o boy cough, fever, night sweats Pruritus shins, + orthopnea Superior Mediastinal Syndrome (SMS) = Oncologic Emergency

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HD – 9 y/o CT scan with SMS

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Superior Vena Cava (SVC) Syndrome in 10 y/o with Lymphoblastic Lymphoma Facial swelling, plethora, cyanosis, ? neck veins Mediastinal mass: tracheal and SVC compression

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Lymphoblastic Lymphoma (T-cell, thymus) • rapid onset • rapid response Same boy 1 week after initial treatment

Burkitt’s Lymphoma : 

Burkitt’s Lymphoma B-cell origin > 5 y/o Abdominal mass Large mass + ? LNs Cecum or appendix Nasopharynx Tumor lysis syndrome ? Uric acid, phosphorus, creatinine Treatment can precipitate renal failure = Oncologic Emergency

Other Abdominal Tumors : 

Other Abdominal Tumors

Malignant Abdominal Masses : 

Malignant Abdominal Masses Most common: Burkitt’s lymphoma Neuroblastoma Wilms Tumor Other: Hepatoblastoma Rhabdomyosarcoma pelvic Ovarian germ cell tumors pelvic

Neuroblastoma : 

Neuroblastoma Age 90% < 5 y/o; 50% < 2 y/o Occasional USG detection in utero Location: any neural crest tissue Adrenal Paraspinal sympathetic tissue Cervical, Thoracic, Pelvic Often metastatic at diagnosis Bone and/or bone marrow > 1 y/o: 70%

Neuroblastoma: Signs and Symptoms : 

Neuroblastoma: Signs and Symptoms Abdominal mass Often crosses midline Lower extremity weakness Spinal cord compression Thoracic abdominal Cervical, high thoracic mass Horner’s syndrome Miosis, ptosis, anhydrosis

Neuroblastoma: Signs and Symptoms : 

Neuroblastoma: Signs and Symptoms Signs of metastatic disease Irritability Weight loss Bone pain Fever Proptosis Bone lesions Periorbital ecchymoses

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More Periorbital Ecchymoses of Neuroblastoma

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Same patient:

Neuroblastoma: Signs and Symptoms : 

Neuroblastoma: Signs and Symptoms Paraneoplastic syndromes Watery diarrhea – Vasoactive Intestinal Peptide Opsoclonus-myoclonus, cerebellar ataxia Cross-reacting antibodies ? Urinary catecholamines VMA/HVA – 85% ? BP – 25% Renal compression Catecholamine secretion

Wilms tumor: Signs and Symptoms : 

Wilms tumor: Signs and Symptoms Abdominal mass Often asymptomatic Healthy appearing

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­ BP – 25% Mass enlarges toward pelvis Wilms tumor: Signs and Symptoms

Signs and Symptoms of Wilms tumor : 

Signs and Symptoms of Wilms tumor Associated anomalies, syndromes – 15% Hemihypertrophy Aniridia WAGR syndrome Wilms, aniridia, ambiguous genitalia, retardation

Signs and Symptoms of Wilms tumor : 

Signs and Symptoms of Wilms tumor More anomalies, syndromes GU anomalies Denys-Drash syndrome GU anomalies and renal failure Beckwith-Wiedemann syndrome

Presentation : 

Presentation 6 yo male with abd mass 2week hx of abdomenal distension Otherwise, healthy Test

CT scan - Stage III Wilms Tumor : 

CT scan - Stage III Wilms Tumor

VS. another presentation : 

VS. another presentation 3yo male with 1 month of fevers Irritable, not walking X 4days with bone pain and fevers Pale Test

CT scan - Stage IV Neuroblastoma : 

CT scan - Stage IV Neuroblastoma

Bone tumors : 

Bone tumors

Bone Tumors in Childhood : 

Bone Tumors in Childhood Age – Adolescents > younger children Signs and symptoms Bone pain, ? palpable mass, ? ? motion Often 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

Presentation of Bone Tumors : 

Presentation of Bone Tumors Plain X-Rays are usually abnormal Classic X-ray of Ewing: Moth-eaten lytic lesion Classic X-ray of O.S.: “Sunburst pattern” Periosteal reaction Soft tissue mass + calcium

Presentation of Bone Tumors : 

Presentation of Bone Tumors Further radiographic evaluation may help with differential diagnosis of bone pain Bone scan MRI Chest CT scan Metastases 20%

Soft tissue sarcomas : 

Soft tissue sarcomas

Presentation of Soft Tissue Sarcomas : 

Presentation of Soft Tissue Sarcomas Rhabdomyosarcoma – most common Age Birth to > 20 y/o 70% < 10 y/o Sites Head and neck – 40% Genitourinary – 20% Extremities – 20% Trunk – 10% Retroperitoneal – 10% Signs and symptoms depend on age and site

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Head and neck Orbit Proptosis Periorbital swelling Parameningeal Cranial nerve palsies Hearing loss Chronic aural or sinus drainage Rhabdomyosarcomas: Signs and Symptoms

Same patient: : 

Same patient:

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Genitourinary Bladder and prostate Hematuria Urinary obstruction Paratesticular Painless mass - ? testicle Vagina and uterus Abdominal mass Vaginal mass Vaginal bleeding or discharge Rhabdomyosarcomas: Signs and Symptoms

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Rhabdomyosarcoma – other sites Can show up at any site and any age

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Over 70% of children diagnosed with cancer will be cured of their disease. 1 in every 1000 young adults alive in the U.S. today is a survivor of childhood cancer. Children should be followed throughout adulthood for potential late effects of therapy and second malignancies. Concluding Remarks