1

Common Presentations of Common Presentations of Childhood CancerChildhood Cancer

First Take Home point

Childhood Cancer is a rare disease…

New Cases of Cancer in the U.S. in 2003

105 000125,000130,000140000

105,000

12,500

020000400006000080000

100000120000

Breast Lung Prostate ChildhoodCancers

Cancer Type

Num

ber

•• One in every 330 Americans One in every 330 Americans develops cancer before age 20. develops cancer before age 20.

BUT…BUT…

•• 1 in 750 201 in 750 20--yearyear--olds alive in the olds alive in the U.S. today is a survivor of U.S. today is a survivor of childhood cancer.childhood cancer.

** Over 3,000 children die from Over 3,000 children die from cancer yearly in the U.S.cancer yearly in the U.S.

• • More than from AIDS, asthma, More than from AIDS, asthma, diabetes and cystic fibrosis combineddiabetes and cystic fibrosis combined

More StatisticsMore Statistics

diabetes, and cystic fibrosis combineddiabetes, and cystic fibrosis combined

Earlier diagnosis and referral Earlier diagnosis and referral can impact outcomecan impact outcome

Second Take Home points…Leukemia is the most common childhood cancerBrain tumors are second most commonLymphomas are the third most commonLymphomas are the third most commonThen solid tumors outside the CNS

Neuroblastoma - neural crest derivedWilms - renal tumors and syndromesBone tumorsRhabdomyosarcoma - soft tissue sarcomas

Brain Brain TumorsTumors

Childhood CancersChildhood Cancers

Retino-

LeukemiaLeukemia

OtherOther

LymphomaLymphoma NeuroblastomaNeuroblastomaSoft tissue sarcomas

Kidney tumors

Bone tumors

Retino-blastoma

2

Specific Signs and Symptoms

Depend on* f* type of cancer* site(s) of disease* age of patient

Cancer in Younger Children

NeuroblastomaRetinoblastomaWilms tumor

Average annual rate per million

Age (yrs) at diagnosis

SEER data, 2002

Cancer in Children and Adolescents

Average annual rate per million

ALLAMLLymphomaBrain Tumors

Age (yrs) at diagnosis

SEER data, 2002

Cancer in Children and Adolescents

Average annual rate per million

HepaticBoneSoft tissueGerm cell

Age (yrs) at diagnosis

SEER data, 2002

LeukemiasLeukemias

# 1 4 y/o fever x 4 daysear pain = L otitis, began AmoxicillinT = 104 + chills next day → urgent care again

Presentations of Lymphoblastic Presentations of Lymphoblastic LeukemiaLeukemia

PE: L TM purulent drainagebilat. cervical, L axillary, L inguinal LNs↑spleen 5 cm ↓, liver 3 cm ↓multiple bruises extremities

→ Test

3

CBC

WBC = 33K8% Neut, 92% lymphocytes

Hb = 6.5PLT = 40K

Peripheral smear show lymphoBLASTS

Bone marrowLeukemia: Signs and SymptomsLeukemia: Signs and Symptoms

Bone marrow infiltration

AnemiaPallor, lethargyDyspnea ↑murmurDyspnea, ↑murmur

↓ PlateletsBleeding, petechiae, purpura

NeutropeniaFevers and infections

Bone painLimp, ↓ walking, irritability

Leukemia: Signs and SymptomsLeukemia: Signs and Symptoms

Extramedullary spreadLymphadenopathyHepatosplenomegalyOrthopnea, cough

mediastinal mass tracheal compression

Facial nerve palsyTesticular enlargement Skin lesionsGingival hypertrophy

Monoblastic leukemia

• Fever of malignancy

CBC and DifferentialCBC and Differential

↑ WBC - 50%; nl or ↓ WBC – 50%+ blasts on smear in 80%

≥ 2 Cytopenias - 95%

Very helpful in the diagnosis ALL

≥ 2 Cytopenias - 95%↓ Hgb - 80% ↓ Plts – 90% ↓ % Neutrophils – 90%

1 Cytopenia - 4%Normal CBC and diff – 1%

4

CNS TumorsCNS Tumors

Presentation

23mo female with ataxia X 1monthNB NB Emesis, in am X 2weeksVomiting amS b PCP f GERD ith t i tSeen by PCP for GERD without improvementNo FeversIncreasing vomiting

Test

MRI MRI

Brain Tumors of ChildhoodBrain Tumors of Childhood

Heterogeneous * Cell of origin:

glial, neural, other, combination* Location:* Location:

• posterior fossa: 50%• supratentorial: 50%

* Clinical presentation:• location• age

Brain Tumors of ChildhoodBrain Tumors of Childhood

Infratentorial50%esp < 6 y/o

Supratentorial50% esp > 8 y/o

5

Nonlocalizing Signs of Brain TumorsNonlocalizing Signs of Brain Tumors

Increased intracranial pressure (ICP)

Obstructed CSF flowand hydrocephalus

Child is often asymptomaticChild is often asymptomaticuntil critical thresholdreached

Medulloblastoma

Can grow very large before detection

Headaches, progressively worsening Vomiting (morning)IrritabilityPapilledema

Increased Intracranial Pressure Increased Intracranial Pressure (ICP)(ICP)

Papilledemarare < 2 y/o - head can expand

“Double vision” with 6th nerve palsyHead tilt Bulging fontanel (infant)In a young child with ? brain tumor:In a young child with ? brain tumor:

Measure head circumference and observe gaitMeasure head circumference and observe gait

Supratentorial TumorsSupratentorial Tumors

Signs depend on location and agelike in adults; in addition:

Younger child:

Developmental delay or loss of milestonesDevelopmental delay or loss of milestones

Older child:Deteriorating school performancePersonality changes

Endocrinopathies:DI, hypothyroidism, precocious puberty

LymphomasLymphomas

Presentation

16yo female with cough X 3 weeksFevers for 2 monthsWt loss X 2 months

Exam noted to have supraclavicular LAD

Test

Childhood LymphomasChildhood Lymphomas

Signs and Symptoms depend on:

Lymphoma subtypeHodgkin’s Disease (HD)N h d ki ’ L h (NHL)Nonhodgkin’s Lymphoma (NHL)

* Burkitt’s * Lymphoblastic * Anaplastic Large Cell

Location

6

Presentation of Hodgkin’s Presentation of Hodgkin’s DiseaseDisease

Age: adolescents >> young childPainless lymphadenopathy

Progresses over weeks → months

LocationLocation Cervical/supraclavicular ↑ LNS

unilateral or bilateral Mediastinum ± hilum

LNs below diaphragm and spleenLiver, lung, bone marrow

95%

Presentation of Hodgkin’s Disease

Systemic symptoms FeversNight sweats Weight loss P it

“B” symptoms25%

Pruritus

= Oncologic Emergency= Oncologic Emergencycompression

• Superior Mediastinal Syndrome (SMS)– Orthopnea, SOB, stridor, hypoxia

•Tracheal

•Bronchial

•Cardiac

What is the Test to get?HD in 16 y/o girlHD in 16 y/o girl↑↑ left cervical LNs, left cervical LNs, 40 # wt losscough, cough, nono orthopneaorthopnea

HD in 9 y/o boycough, fever, night sweatsPruritus shins, + orthopnea

Superior Mediastinal Syndrome (SMS)Superior Mediastinal Syndrome (SMS)= = Oncologic EmergencyOncologic Emergency

HD – 9 y/o CT scan with SMS

Ant. mediastinal masscompressing trachea; Pleural effusion

Pericardial effusion with tamponade

Superior Vena Cava (SVC) SyndromeSuperior Vena Cava (SVC) Syndrome in in 10 y/o with 10 y/o with Lymphoblastic LymphomaLymphoblastic Lymphoma

Facial swelling, plethora, cyanosis, � neck veins

Mediastinal mass: tracheal and SVC compression

7

Lymphoblastic Lymphoma (TLymphoblastic Lymphoma (T--cell, thymus)cell, thymus)

• rapid onset • rapid responseSame boy 1 week after initial treatment

Burkitt’s Burkitt’s Lymphoma Lymphoma

B-cell origin> 5 y/oAbdominal mass

Large mass + � LNsCecum or appendix

NasopharynxTumor lysis syndrome

� Uric acid, phosphorus, creatinineTreatment can precipitate renal failure

= Oncologic Emergency= Oncologic Emergency

Other Abdominal Tumors

Malignant Abdominal Masses Malignant Abdominal Masses Most common:

Burkitt’s lymphomaNeuroblastoma Wilms Tumor

Other:• Hepatoblastoma• Rhabdomyosarcoma

–pelvic

• Ovarian germ cell tumors– pelvic

NeuroblastomaNeuroblastoma

Age90% < 5 y/o; 50% < 2 y/o Occasional USG detection in utero

Location: any neural crest tissue AdrenalParaspinal sympathetic tissue

Cervical, Thoracic, PelvicOften metastatic at diagnosis

Bone and/or bone marrow> 1 y/o: 70%

Neuroblastoma: Signs and SymptomsAbdominal mass

Often crosses midlineLower extremity weakness

Spinal cord compressionTh iThoracicabdominal

Cervical, high thoracic massHorner’s syndrome

Miosis, ptosis, anhydrosis

8

Neuroblastoma: Signs and Neuroblastoma: Signs and SymptomsSymptomsSigns of metastatic disease

IrritabilityWeight lossBone painFeverFever

– Proptosis

– Bone lesions

– Periorbital

ecchymoses

More Periorbital EcchymosesMore Periorbital Ecchymosesof Neuroblastomaof Neuroblastoma

13 months old at diagnosis

1 month intotherapy

Same patient: Same patient: 5 years later

12 years later Neuroblastoma: Signs and Neuroblastoma: Signs and SymptomsSymptoms

Paraneoplastic syndromesWatery diarrhea – Vasoactive Intestinal PeptideOpsoclonus-myoclonus, cerebellar ataxia

Cross-reacting antibodies↑↑ Urinary catecholamines

VMA/HVA – 85%↑ BP – 25%

Renal compressionCatecholamine secretion

Wilms tumor: Signs and Wilms tumor: Signs and SymptomsSymptomsAbdominal mass

Often asymptomaticHealthy appearing

Encapsulatedmass

2 daysbeforedx

- BP – 25%

Mass enlarges toward pelvis

Wilms tumor: Signs and SymptomsWilms tumor: Signs and Symptoms

9

Signs and Symptoms of Signs and Symptoms of Wilms tumorWilms tumorAssociated anomalies, syndromes – 15%

HemihypertrophyAniridia

WAGR syndromeWilms, aniridia, ambiguous genitalia, retardation

Signs and Symptoms of Signs and Symptoms of Wilms tumorWilms tumorMore anomalies, syndromes

GU anomalies Denys-Drash syndrome

GU anomalies and renal failureBeckwith-Wiedemann syndrome

Umbilical hernia

Hemihypertrophy

Macro-glossia

Hypogly-cemia

Presentation

6 yo male with abd mass2week hx of abdomenal distensionOtherwise, healthy

Test

CT scan - Stage III Wilms Tumor

VS. another presentation3yo male with 1 month of feversIrritable, not walking X 4days with bone pain and feversPalePale

Test

CT scan - Stage IV Neuroblastoma

10

Bone tumorsBone tumors

Bone Tumors in ChildhoodBone Tumors in Childhood

Age – Adolescents > younger childrenSigns and symptoms

Bone pain, � palpable mass, � � motionOften hx of sports injury (coincidental)

Ewing SarcomaAll bones:Long: diaphysesFlatPelvisSkullRibs

Osteogenic SarcomaMetaphyses of long bones:

Distal femurProximal tibiaProximal humerusPelvis

Presentation of Bone TumorsPresentation of Bone TumorsPlain X-Rays are usually abnormal

Classic X-rayof Ewing:g

Moth-eatenlytic lesion

Classic X-ray of O.S.:“Sunburst pattern”Periosteal reactionSoft tissue mass + calcium

Presentation of Bone TumorsPresentation of Bone TumorsFurther radiographic evaluation may help

with differential diagnosis of bone pain Bone scanMRIChest CT scanChest CT scan

Metastases 20%

Pelvic Ewings sarcoma

Soft tissue sarcomasSoft tissue sarcomas

Presentation of Soft Tissue SarcomasPresentation of Soft Tissue Sarcomas

Rhabdomyosarcoma – most commonAge

Birth to > 20 y/o70% < 10 y/o Signs and Signs and

tty

SitesHead and neck – 40%Genitourinary – 20%Extremities – 20%Trunk – 10%Retroperitoneal – 10%

symptomssymptomsdepend on depend on age and siteage and site

11

Head and neckOrbit

ProptosisPeriorbital swelling

Rhabdomyosarcomas: Signs and SymptomsRhabdomyosarcomas: Signs and Symptoms

Parameningeal Cranial nerve palsiesHearing lossChronic aural or

sinus drainage

Same patient:

S/P radiationand chemo 3 months off Rx:

eye lashes regrown

GenitourinaryBladder and prostate

HematuriaUrinary obstruction

Rhabdomyosarcomas: Signs and Rhabdomyosarcomas: Signs and SymptomsSymptoms

Paratesticular Painless mass - ↑ testicle

Vagina and uterus Abdominal massVaginal massVaginal bleeding or discharge

Botryoid:grape-like

Rhabdomyosarcoma Rhabdomyosarcoma –– other sitesother sites

6 week old NewbornCan show up at any site and any age

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

Concluding RemarksConcluding Remarks

childhood cancer.

• Children should be followed throughout adulthood for potential late effects oftherapy and second malignancies.