Pediatric Tumors Pamela Simon R.N., C.P.N.P, MSN Lucile Packard Children’s Hospital

Pediatric TumorsPediatric Tumors

Pamela Simon R.N., C.P.N.P, Pamela Simon R.N., C.P.N.P, MSNMSN

Lucile Packard Children’s HospitalLucile Packard Children’s Hospital

Distribution of Childhood CancerDistribution of Childhood Cancer

CNS18%

Liver1%

Other8%

Leukemia33%Retina

3%Germ Cell3%

Bone5%

Wilm's Tumor

6%

Hodgkin's5% NHL

3%

NBL8%

Soft Tissue Sarcoma

7%

Abdominal massesAbdominal masses

HistoryHistory SymptomsSymptoms

– abdominal discomfort, increased abdominal abdominal discomfort, increased abdominal size or assymptomaticsize or assymptomatic

– Presence of systemic symptomsPresence of systemic symptoms bone pain, limping, malaise, feverbone pain, limping, malaise, fever

– Other symptomsOther symptoms hematuriahematuria

Abdominal massesAbdominal masses

Physical findingsPhysical findings Presence of abdominal massPresence of abdominal mass

– upper abdomen or lower abdomenupper abdomen or lower abdomen

Other associated physical findingsOther associated physical findings– other masses: orbitalother masses: orbital– bruisesbruises– hypertensionhypertension– painpain

Abdominal MassAbdominal Mass

Differential diagnosis Differential diagnosis depends on locationdepends on location

Upper abdominal Upper abdominal massmass– NeuroblastomaNeuroblastoma– Wilm’s tumorWilm’s tumor– HepatoblastomaHepatoblastoma– RhabdomyosarcomaRhabdomyosarcoma– Germ cell tumorGerm cell tumor– LymphomaLymphoma

Abdominal MassAbdominal Mass

Differential diagnosis Differential diagnosis (cont)(cont)

Lower Lower abdominal/pelvic abdominal/pelvic massmass– NeuroblastomaNeuroblastoma– RhabdomyosarcomaRhabdomyosarcoma– Germ cell tumorGerm cell tumor– LymphomaLymphoma– Ewing’s sarcomaEwing’s sarcoma

Abdominal MassesAbdominal MassesLaboratory evaluationLaboratory evaluation Bone marrow aspirateBone marrow aspirate

– neuroblastoma, lymphoma, neuroblastoma, lymphoma, rhabdomyosarcoma or rhabdomyosarcoma or Ewing’s sarcoma Ewing’s sarcoma

CSF tapCSF tap– if lymphoma is a if lymphoma is a

considerationconsideration

Abdominal massesAbdominal massesRadiologic work-upRadiologic work-up Abdominal/pelvic ultrasoundAbdominal/pelvic ultrasound

– useful screening test: helps define location and useful screening test: helps define location and quality (solid or cystic)quality (solid or cystic)

– not useful to assess for retroperitoneal adenopathynot useful to assess for retroperitoneal adenopathy

Computed tomographyComputed tomography – location of masslocation of mass– presence of calcificationpresence of calcification– obstructive signs obstructive signs

Wilm’s TumorWilm’s Tumor

Accounts for 6% childhood malignanciesAccounts for 6% childhood malignancies Median age at diagnosis 3 yearsMedian age at diagnosis 3 years Metastasizes to lung and lymph nodesMetastasizes to lung and lymph nodes

– 70% patients present with localized disease70% patients present with localized disease

Wilm’s Tumor: Signs & SymptomsWilm’s Tumor: Signs & Symptoms

Most commonly Most commonly presents as painless presents as painless abdominal massabdominal mass

Hypertension (20-30%)Hypertension (20-30%) Pain (20-30%)Pain (20-30%) Hematuria (25%)Hematuria (25%)

Wilm’s Tumor: Staging SystemWilm’s Tumor: Staging System

StageStage DefinitionDefinition

II Tumor limited to kidney, completely excisedTumor limited to kidney, completely excised

IIII Tumor extends beyond the kidney, completely resected; Tumor extends beyond the kidney, completely resected; no residual tumor beyond resection marginsno residual tumor beyond resection margins

IIIIII Residual non-hematogenous tumor confined to abdomenResidual non-hematogenous tumor confined to abdomen

IVIV Hematogenous metastasesHematogenous metastases

VV Bilateral renal involvementBilateral renal involvement

Bilateral Wilm’sBilateral Wilm’s

Survival by Stage and HistologySurvival by Stage and Histology

Histology/stage # pts 2 yr-s* 4 yr-s*

FH/I 546 98 97

II 281 96 94

III 290 91 88

IV 126 88 82

UH/I 20 89 89

UH/II-IV 40 56 54* survival

Wilms Tumor: Prognosis & Future DirectionsWilms Tumor: Prognosis & Future Directions

Prognostic factors: stage and histologyPrognostic factors: stage and histology Future directionsFuture directions

– Minimize therapy for favorable histology Minimize therapy for favorable histology patientspatients

– Identify biologic factors predictive of outcomeIdentify biologic factors predictive of outcome– Intensify therapy for patients with unfavorable Intensify therapy for patients with unfavorable

histologyhistology

Osteosarcoma: DistributionOsteosarcoma: Distribution by Age and Site by Age and Site

Clinical presentationClinical presentation Pain and swelling usually lasting 3-4 Pain and swelling usually lasting 3-4

months months – Duration: sometimes > 6 monthsDuration: sometimes > 6 months

Occurs around metaphysis of long Occurs around metaphysis of long bonesbones– Most common primary sites: distal femur, Most common primary sites: distal femur,

proximal tibia and proximal humerusproximal tibia and proximal humerus– 50-70% around the knee joint50-70% around the knee joint

Metastases: ~15-20% patientsMetastases: ~15-20% patients– Sites: lung and other bonesSites: lung and other bones

Most Common Metastatic SitesMost Common Metastatic Sites

Diagnostic workupDiagnostic workup

Imaging Work-up: Plain Imaging Work-up: Plain X ray, MRI of primaryX ray, MRI of primary

Metastatic workupMetastatic workup– Chest CT Chest CT – Bone scanBone scan– PET scanPET scan

BiopsyBiopsy

Imaging Evaluation Imaging Evaluation

Metastatic Work-Up: Chest X-RayMetastatic Work-Up: Chest X-Ray

Metastatic Work-Up: Chest CTMetastatic Work-Up: Chest CT

Metastatic Work-Up: Bone ScanMetastatic Work-Up: Bone Scan

PathologyPathology

Intramedullary high-grade Intramedullary high-grade sarcoma sarcoma – Malignant mesenchymal Malignant mesenchymal

cells producing osteoid cells producing osteoid

The World Health The World Health Organization (WHO) Organization (WHO) recognizes three major recognizes three major subtypes: based on subtypes: based on matrix: matrix: – OsteoblasticOsteoblastic– ChondroblasticChondroblastic– fibroblasticfibroblastic

Osteosarcoma: PathologyOsteosarcoma: Pathology

Osteosarcoma: TherapyOsteosarcoma: Therapy

Historically, 2-year Historically, 2-year survival < 20% for survival < 20% for patients treated with patients treated with surgery and/or surgery and/or radiotherapyradiotherapy

Uncontrolled trials of Uncontrolled trials of adjuvant chemotherapy adjuvant chemotherapy resulted in EFS of 45-resulted in EFS of 45-60% suggesting 60% suggesting chemotherapy improved chemotherapy improved outcomeoutcome

Multi-Institutional Osteosarcoma Multi-Institutional Osteosarcoma Study:Study: Design Design

BIOPSY

SURGERY

RANDOMIZE

Adjuvant Chemotherapy

No Adjuvant Chemotherapy

Osteosarcoma: TreatmentOsteosarcoma: Treatment

Current therapy: multi-Current therapy: multi-agent chemotherapy agent chemotherapy usually including usually including cisplatin, doxorubicin cisplatin, doxorubicin and methotrexate.and methotrexate.

Complete surgical Complete surgical resection for local resection for local control.control.

Osteosarcoma TreatmentOsteosarcoma Treatment

Types of surgical techniquesTypes of surgical techniques

– AmputationAmputation– RotationplastyRotationplasty– ““growing’ prosthesisgrowing’ prosthesis– Total knee titanium replacementTotal knee titanium replacement

AmputationAmputation

RotationplastyRotationplasty

RotationplastyRotationplasty

Total knee titanium Total knee titanium replacementreplacement

(endoprosthesis)(endoprosthesis)

Prognostic Factors: MetastasesPrognostic Factors: Metastases

20

40

60

80

100

1 2 3 4 5

pre-chemo

SURVIVAL OF PATIENTS WITH LOCALIZED OSTEOSARCOMA

years

1980’s- combination chemo

1960’s – single agentchemo

2004 - chemo regimensmultiple intensified

% s

urv

ival

Osteosarcoma: Outcome Modern Osteosarcoma: Outcome Modern TrialsTrials

Osteosarcoma: OutcomeOsteosarcoma: Outcome

Reached a plateau in outcome for Reached a plateau in outcome for osteosarcoma patientsosteosarcoma patients– Further improvement will require large Further improvement will require large

cooperative studies: International collaborationcooperative studies: International collaboration– EURAMOS: EURAMOS:

North American Children’s Oncology Group (COG) North American Children’s Oncology Group (COG) German Austrian Swiss Cooperative German Austrian Swiss Cooperative

Osteosarcoma Study Group (COSS)Osteosarcoma Study Group (COSS) European Osteosarcoma Intergroup (EOI) European Osteosarcoma Intergroup (EOI) Scandinavian Sarcoma Group (SSG) Scandinavian Sarcoma Group (SSG)

Osteosarcoma: ConclusionsOsteosarcoma: Conclusions

We have made significant progress in the We have made significant progress in the treatment of osteosarcomatreatment of osteosarcoma

Therapy has reached a plateau and further Therapy has reached a plateau and further improvements will require large number of improvements will require large number of patientspatients– International collaboration: significant barriers International collaboration: significant barriers

but offers the best chance of increasing the but offers the best chance of increasing the number of patients availablenumber of patients available

– Biologic studies: essential for continued Biologic studies: essential for continued progressprogress

CNS TUMORSCNS TUMORS


Most common solid tumors in childrenMost common solid tumors in children 22ndnd most frequent (16.6% of all childhood most frequent (16.6% of all childhood

malignancies)malignancies) Incidence has increased over the past 2 Incidence has increased over the past 2

decades decades Males > females, white> African AmericanMales > females, white> African American


Signs & SymptomsSigns & Symptoms (related to the location, histologic grade of (related to the location, histologic grade of

tumor & age of child)tumor & age of child)GeneralGeneral-Headache-Headache-Seizures-Seizures-Mental status changes-Mental status changes-Increased intracranial Pressure (ICP)-Increased intracranial Pressure (ICP)


Signs & Symptoms-Posterior FossaSigns & Symptoms-Posterior Fossa-Cerebellum--Cerebellum-

-nausea, vomiting, headache, papilledema, -nausea, vomiting, headache, papilledema, clumsy walk, double vision, dizzynessclumsy walk, double vision, dizzyness

-Brainstem--Brainstem- -vomiting, cranial nerve palsies, headache, -vomiting, cranial nerve palsies, headache,

head tilt, personality changes, hearing losshead tilt, personality changes, hearing loss


S&S-Cerebral HemisphereS&S-Cerebral Hemisphere– Frontal lobe-one-sided paralysis, memory loss, Frontal lobe-one-sided paralysis, memory loss,

mental changes, urinary changesmental changes, urinary changes– Occipital lobe-visual changes, seizuresOccipital lobe-visual changes, seizures– Parietal lobe-Language disturbances, seizures, Parietal lobe-Language disturbances, seizures,

loss of reading, math loss of reading, math – Temporal lobe-seizures, unable to recognize Temporal lobe-seizures, unable to recognize

sounds, visual impairments sounds, visual impairments


S&S-Midline TumorsS&S-Midline Tumors– Headache, nausea/vomiting, papilledema, Headache, nausea/vomiting, papilledema,

visual loss or abnormal eye movements, visual loss or abnormal eye movements, precocious puberty, diabetes insipidusprecocious puberty, diabetes insipidus

(impairment of hypothalmic/pituitary fx)(impairment of hypothalmic/pituitary fx)


S&S- Spinal Cord-depends on locationS&S- Spinal Cord-depends on location– Thoracic-chest painThoracic-chest pain– Cervical or lumbar-neck, arm, back, leg Cervical or lumbar-neck, arm, back, leg

weakness, muscle spasms & wasting, altered weakness, muscle spasms & wasting, altered bowel, bladder functionbowel, bladder function

– Progression of symptoms can result in paralysisProgression of symptoms can result in paralysis


Age & developmental stage are important factors Age & developmental stage are important factors in re: to symptomsin re: to symptoms

-infants-delay or loss of dev. Milestones-infants-delay or loss of dev. Milestones

-school age-personality changes, decline in -school age-personality changes, decline in school performance, change in handwritingschool performance, change in handwriting


Diagnostic EvaluationDiagnostic Evaluation– MRI head-preferredMRI head-preferred

Add spine if requiredAdd spine if required

– CT headCT head– Lumbar punctureLumbar puncture– Lab work for “tumor markers” for germ cell Lab work for “tumor markers” for germ cell

tumors-AFP & B-hCGtumors-AFP & B-hCG– Pet Scan Pet Scan


TREATMENTTREATMENT– SurgerySurgery

Most extensive resection feasibleMost extensive resection feasible

--Radiation Therapy--Radiation Therapy

--Chemotherapy--Chemotherapy


PrognosisPrognosis– Varies greatly depending on type of tumor, Varies greatly depending on type of tumor,

resectability, resectability,


AstrocytomaAstrocytoma– Occur at any age & various areas of brainOccur at any age & various areas of brain– Graded according to anaplasiaGraded according to anaplasia– Low grade cerebellar-pilocytic-85-95 % 5 year Low grade cerebellar-pilocytic-85-95 % 5 year

survival ratesurvival rate– Low grade cerebral-90% 5 year survival rateLow grade cerebral-90% 5 year survival rate– -High grade astrocytoma-29% for anaplastic -High grade astrocytoma-29% for anaplastic

astrocytoma & 18% -glioblastoma multiformeastrocytoma & 18% -glioblastoma multiforme


Medulloblastoma (PNET)Medulloblastoma (PNET)– 20-25%of all brain tumors20-25%of all brain tumors– Small, round blue cell tumor-fast growingSmall, round blue cell tumor-fast growing– 30% metastasis30% metastasis– Surgery, radiation & chemotherapySurgery, radiation & chemotherapy– 5 yr survival rates 80%5 yr survival rates 80%

Documents

Pediatric Tumors Pamela Simon R.N., C.P.N.P, MSN Lucile Packard Children’s Hospital