Pediatric Solid Tumors Kuliah S1

04/22/2304/22/23 04:4004:40 [email protected] [email protected] 11

Pediatric Solid TumorsPediatric Solid Tumors

Bambang SudarmantoHematology-oncology working groupKariadi Hospital SemarangIndonesia



Pediatric TumorsPediatric Tumors

Kariadi Hospital is referall hospital Central Kariadi Hospital is referall hospital Central Java and part of BorneoJava and part of Borneo

Class of patients: Jamkesmas (poor Class of patients: Jamkesmas (poor people), Askes (health insurance), privatepeople), Askes (health insurance), private

Many are treated in Private HospitalsMany are treated in Private Hospitalsmany hospitals do not treat children with many hospitals do not treat children with

cancercancer


Pediatric Solid Tumors (<14)Pediatric Solid Tumors (<14)

9,000 new cases, 1500 deaths of pediatric 9,000 new cases, 1500 deaths of pediatric cancers annually in the U.S.cancers annually in the U.S.

Cancer- 2nd leading cause of death in Cancer- 2nd leading cause of death in children (1children (1stst infections) infections)

1/3 deaths from leukemia1/3 deaths from leukemiaCNS tumors most frequent SOLID tumors CNS tumors most frequent SOLID tumors

in pediatric populationin pediatric population Indonesia data not viable (Semarang?)Indonesia data not viable (Semarang?)


Pediatric solid tumorsPediatric solid tumors

Childhood cancer Childhood cancer requires multidisciplinary treatment groupsrequires multidisciplinary treatment groups

emotional needs of patient/familyemotional needs of patient/familynutritional needsnutritional needscoordinated care- medical, surgical, oncology, coordinated care- medical, surgical, oncology,

nursing, social services, therapists, educators nursing, social services, therapists, educators


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%


Thyroglossal Duct Cyst

Infectious/Inflammatory

Neoplasms

Pediatric Neck Mass

Congenital Acquired

Branchial cleft cyst

Cystic hygroma

Dermoid cyst


Congenital MassesCongenital Masses

Thyroglossal duct Thyroglossal duct cystscysts

Branchial cleft cystsBranchial cleft cysts Lymphatic Lymphatic

malformationsmalformations HemangiomasHemangiomas TeratomasTeratomas Dermoid cystsDermoid cysts

LaryngocelesLaryngoceles Thymic cystsThymic cysts Vascular Vascular

malformationsmalformations SCM tumors of SCM tumors of

infancyinfancy Plunging ranulasPlunging ranulas


Acquired MassesAcquired Masses

InfectiousInfectiousBacterialBacterialViralViralFungalFungalSialadenitisSialadenitis

InflammatoryInflammatoryKawasakiKawasakiSinus HistiocytosisSinus HistiocytosisDrug-induced Drug-induced

lymphadenopathylymphadenopathySarcoidosisSarcoidosis


Abdominal masses


Abdominal massesAbdominal masses

HistoryHistorySymptomsSymptoms

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

Presence of systemic symptomsPresence of systemic symptomsbone pain, limping, malaise, feverbone pain, limping, malaise, fever

Other symptomsOther symptomshematuriahematuria



Physical findingsPhysical findingsPresence of abdominal massPresence of abdominal mass

upper abdomen or lower abdomenupper abdomen or lower abdomenOther associated physical findingsOther associated physical findings

other masses: orbitalother masses: orbitalbruisesbruiseshypertensionhypertensionpainpain


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 abdominal/pelvic Lower 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



Radiologic 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 malignanciesMedian age at diagnosis 3 yearsMedian age at diagnosis 3 yearsMetastasizes 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-Hypertension (20-30%)30%)

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



Diagnosis:Diagnosis:UltrasoundUltrasoundCTCT

Lymph node and lung metsLymph node and lung metsRight sided tumorsliver metsRight sided tumorsliver metsMets to bone and brain possible with some Mets to bone and brain possible with some

histologieshistologies


Wilm’s Tumor: Staging Wilm’s Tumor: Staging SystemSystem

StageStage DefinitionDefinition

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

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

IIIIII Residual non-hematogenous tumor confined to Residual non-hematogenous tumor confined to abdomenabdomen

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


Wilm’s tumorWilm’s tumor

Treatment:Treatment:Remove kidney- nephrectomyRemove kidney- nephrectomy

Lymph nodes biopsiedLymph nodes biopsiedCan spread also through bloodCan spread also through bloodTumor spill is possible- abdominal cavity is at riskTumor spill is possible- abdominal cavity is at risk

Chemo and RT- sometimes preop to reduce Chemo and RT- sometimes preop to reduce tumor spill tumor spill

RT late stages, residual dx, LN mets, RT late stages, residual dx, LN mets, bilateral dx, lung metsbilateral dx, lung mets



ChemoChemo Actinomycin-DActinomycin-D Added vincristine, Adriamycin- 80-100% cure ratesAdded vincristine, Adriamycin- 80-100% cure rates Mets = 70% cure rateMets = 70% cure rate


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

Prognostic factors: stage and histologyPrognostic factors: stage and histologyFuture 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


Pediatric Brain Tumors(CNS Tumor)


Brain tumors: pediatric vs. adultBrain tumors: pediatric vs. adult Adults: 70% of tumors are supratentorialAdults: 70% of tumors are supratentorial

meningiomameningioma pituitary adenomapituitary adenoma High grade astrocytomaHigh grade astrocytoma

Anaplastic astrocytoma (grade III)Anaplastic astrocytoma (grade III) Glioblastoma multiforme (grade IV astrocytoma)Glioblastoma multiforme (grade IV astrocytoma)

Pediatric: 70% in posterior fossaPediatric: 70% in posterior fossa pilocytic astrocytoma (cerebellar astrocytoma)pilocytic astrocytoma (cerebellar astrocytoma) medulloblastoma medulloblastoma


CNS TUMORSCNS TUMORSMost common solid tumors in childrenMost common solid tumors in children22ndnd 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 Leading cause of cancer death in childrenLeading cause of cancer death in childrenResult in more morbidity than any other Result in more morbidity than any other

malignancy in childhoodmalignancy in childhoodMetastatic brain cases are rareMetastatic brain cases are rare


CNS TUMORSCNS TUMORS

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

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

Astrocytoma Astrocytoma

- high grade- high grade

- low grade- low gradeMedulloblastomaMedulloblastomaBenign tumor : adenomaBenign tumor : adenoma



MedulloblastomasMedulloblastomas

25% of all childhood brain tumors25% of all childhood brain tumors Occurs in the posterior fossaOccurs in the posterior fossa

Arises in the midline of the cerebellum and spreads to the in the Arises in the midline of the cerebellum and spreads to the in the 4th ventricle and brain stem4th ventricle and brain stem

High propensity to spread through CSFHigh propensity to spread through CSF 2-12 yr old2-12 yr old

peaks at 5 yr oldpeaks at 5 yr old Rare in adultsRare in adults arises from primitive neuroepithelial cellsarises from primitive neuroepithelial cells histologically cells appear as small, round, blue cells histologically cells appear as small, round, blue cells

forming pseudorosettes. Purple friable tumorforming pseudorosettes. Purple friable tumor


medulloblastomasmedulloblastomas

invasion of the fourth ventricle causes sx invasion of the fourth ventricle causes sx CSF does not flow causing hydrocephalusCSF does not flow causing hydrocephalusheadaches early- morning vomit get progressively headaches early- morning vomit get progressively

worseworseAtaxiaAtaxiacranial nerve abnormalities from invasion of the cranial nerve abnormalities from invasion of the

brain stembrain stemStaging correlates with survivalStaging correlates with survivalCT/MRI, myelography of entire CNS necessary CT/MRI, myelography of entire CNS necessary


MedulloblastomasMedulloblastomas Can develop mets in boneCan develop mets in bone Neurosurgery is first treatmentNeurosurgery is first treatment

remove gross diseaseremove gross disease ventricular shunts are discouraged because of possible ventricular shunts are discouraged because of possible

seeding into peritoneal cavityseeding into peritoneal cavity

CSI- Craniospinal IrradiationCSI- Craniospinal Irradiation postop- RTpostop- RT lateral- opposed brain fieldslateral- opposed brain fields

cover retroorbitally through mid-cervical cordcover retroorbitally through mid-cervical cord The divergence of the spinal field determines the collimator The divergence of the spinal field determines the collimator

twisttwist


CSI- Craniospinal IrradiationCSI- Craniospinal Irradiation Depending on the length of the spine, two spinal fields Depending on the length of the spine, two spinal fields

may be necessarymay be necessary Whole spine is treated- down to S2 or S3Whole spine is treated- down to S2 or S3 Electrons are used many times to avoid treating Electrons are used many times to avoid treating

anterior structuresanterior structures Gaps (photons) between fields are featheredGaps (photons) between fields are feathered Some institutions are encouraging a small gap Some institutions are encouraging a small gap

between lateral brain fields and the upper spinal fieldbetween lateral brain fields and the upper spinal field See page 326 RedSee page 326 Red


CSI -Craniospinal IrradiationCSI -Craniospinal Irradiation

Immobilization importantImmobilization importantWhat position?What position?Anesthesia may be necessaryAnesthesia may be necessary

RT Doses:RT Doses:1.5 Gy day to 36 Gy1.5 Gy day to 36 GyBoost to posterior fossa area- 54 Gy @ 1.8 Boost to posterior fossa area- 54 Gy @ 1.8

daydayChemo investigationChemo investigation


RetinoblastomasRetinoblastomas

Most common intraocular tumor in childrenMost common intraocular tumor in children200 U.S. annually (Indonesia: no data) 200 U.S. annually (Indonesia: no data) Most occur 6 mos-4yrs oldMost occur 6 mos-4yrs oldHereditary risk factors- 10% of casesHereditary risk factors- 10% of cases

Can be bilateral- 35% casesCan be bilateral- 35% casesRarely curativeRarely curative



Discovered by light reflexDiscovered by light reflexWhite vs. redWhite vs. red

Ophthalmologist examOphthalmologist examNo biopsyNo biopsy

Avoid possibility of vitreous seedingAvoid possibility of vitreous seedingCT: detect extraocular extensionCT: detect extraocular extension



CSF diseaseCSF diseaseAssessed by lumbar punctureAssessed by lumbar puncture

Bone marrow may be biopsedBone marrow may be biopsedStaging systems developedStaging systems developed

Preferred treatments outlined Preferred treatments outlined Predict success ratesPredict success ratesMost commonMost common

Reese-EllsworthReese-Ellsworth



Treatment TechniquesTreatment TechniquesPhotocoagulationPhotocoagulationCryosurgeryCryosurgery

Small tumors not near optic disc and maculaSmall tumors not near optic disc and macula

EnucleationEnucleationUnilateral diseaseUnilateral diseaseSacrifices globeSacrifices globe



Treatment Techniques:Treatment Techniques:SurgerySurgeryRadiation ImplantsRadiation Implants

RTRT Inoperable or bilateral dxInoperable or bilateral dxSpare lens and corneaSpare lens and corneaTreat retinaTreat retina



Treatment Method: if disseminated dxTreatment Method: if disseminated dxChemo Chemo

Good response expectedGood response expectedVAC- Vincristine, Adriamycin, cyclophosphamideVAC- Vincristine, Adriamycin, cyclophosphamideOPEC- vincristine, cisplatin, etoposide, OPEC- vincristine, cisplatin, etoposide,

cyclophosphamidecyclophosphamide



Late Effects of TreatmentLate Effects of TreatmentEnucleationEnucleation

Several prosthetics will be needed as child growsSeveral prosthetics will be needed as child grows

RTRTFacial growth affectedFacial growth affectedSmall orbitsSmall orbitsBlind spotsBlind spotsRadiation retinitis, and dry eyesRadiation retinitis, and dry eyes2nd malignancy2nd malignancy


Neuroblastomas

500 cases annually U.S.500 cases annually U.S.2nd most common pediatric solid tumor-2nd most common pediatric solid tumor-

after brain tumorsafter brain tumorsAge range- newborns-several yrs oldAge range- newborns-several yrs oldMedian age 2 yrs oldMedian age 2 yrs old


NeuroblastomasNeuroblastomas

Occur mainly in the abdomenOccur mainly in the abdomen Originate in adrenal gland or paraspinal gangliaOriginate in adrenal gland or paraspinal ganglia

Lethargic, ill appearing child under 2 with Lethargic, ill appearing child under 2 with abdominal mass is commonabdominal mass is common

Spinal cord invasion will promote neurological sxSpinal cord invasion will promote neurological sx Horner’s syndrome present with upper Horner’s syndrome present with upper

sympathetic ganglia involvementsympathetic ganglia involvement Flushing, diarrheaFlushing, diarrhea



Many children present with metsMany children present with mets Children older than 18 months have 70% Children older than 18 months have 70%

chance of developing metschance of developing mets CT- chest, abd (US)CT- chest, abd (US)

Lungs and liver assessedLungs and liver assessed MRI spineMRI spine Bone marrow biopsyBone marrow biopsy Bone scanBone scan



PrognosisPrognosisDependent on age at presentationDependent on age at presentation< 1 yr old disease may regress if acute sx are < 1 yr old disease may regress if acute sx are

managedmanaged Treatment TechniquesTreatment Techniques

RT and chemo- low cureRT and chemo- low cureSurgery if no mets- high cure rateSurgery if no mets- high cure rateChemo and BMTChemo and BMTRT- palliationRT- palliation


LymphomaLymphoma


LymphomasLymphomas

33rdrd most common cancer in children. 60% are most common cancer in children. 60% are NHL; 40% Hodgkins disease NHL; 40% Hodgkins disease

Extranodal disease is common in children with Extranodal disease is common in children with 1/3 rd involving the mediastinum, abdomen or 1/3 rd involving the mediastinum, abdomen or head and neckhead and neck

Multi agent chemotherapy is the mainstay of Multi agent chemotherapy is the mainstay of treatment, surgery and radiotherapytreatment, surgery and radiotherapy

Anticipation and treatment of complications is Anticipation and treatment of complications is extremely important (lysis tumor syndrome, SVC extremely important (lysis tumor syndrome, SVC syndrome)syndrome)


Non Hodgkins lymphomaNon Hodgkins lymphoma

NHL result from malignant proliferation of NHL result from malignant proliferation of cells of lymphocytic ar histiocytic linage.cells of lymphocytic ar histiocytic linage.

Generally restricted to lymphoid tissue Generally restricted to lymphoid tissue such as lymphnodes and spleen such as lymphnodes and spleen

NHL categorized as low, intermediate and NHL categorized as low, intermediate and high grade on the base of their clinical high grade on the base of their clinical aggressiveness.aggressiveness.



Epidemiology :Epidemiology : peak is between 5-15 years median age 9 peak is between 5-15 years median age 9

years; male preponderance , 3:1years; male preponderance , 3:1 Increase risk include patient with immunologic Increase risk include patient with immunologic

defect (AIDS, etc), immunosuppresive therapy defect (AIDS, etc), immunosuppresive therapy (post transplant)(post transplant)

EBV is almost invariably associated with the EBV is almost invariably associated with the African Burkitt’s lymphomaAfrican Burkitt’s lymphoma



Clinical feature:Clinical feature: are varied depend on the location of the tumor, the are varied depend on the location of the tumor, the

extent of the disease (stage) and histologic subtypeextent of the disease (stage) and histologic subtype Extranodal involvement :Extranodal involvement : Mediastinum: may produce SVC syndromeMediastinum: may produce SVC syndrome Abdomen: ileocaecal region, appendix, colon and Abdomen: ileocaecal region, appendix, colon and

retroperitoneal area. Cayse pain, vomiting, retroperitoneal area. Cayse pain, vomiting, intussuception, ascites, obstructiveintussuception, ascites, obstructive

Head and neck: enlargement of cervical node, paritis, Head and neck: enlargement of cervical node, paritis, tonsilar hyperthropy; jaw swellingtonsilar hyperthropy; jaw swelling



Diagnosis and staging: Diagnosis and staging: History and physical examHistory and physical examBlood count, BMA and BiopsyBlood count, BMA and BiopsyLDH levelLDH levelEvaluation of liver test (bil, SGOT/SGPT; Evaluation of liver test (bil, SGOT/SGPT;

alkphospatase)alkphospatase)Chest radiography; USG; CT Scan abdomenChest radiography; USG; CT Scan abdomenAdequate surgical biopsyAdequate surgical biopsy



Staging:Staging:Stage I: a single tumor (extranodal) or single Stage I: a single tumor (extranodal) or single

anatomic areawith exclusion of mediastinum anatomic areawith exclusion of mediastinum and abdomenand abdomen

Stage II: stage I + two or more nodal area on Stage II: stage I + two or more nodal area on the same side of diaphragnathe same side of diaphragna

Satege III: stage II + all extensive node Satege III: stage II + all extensive node enlargementenlargement

Stage IV: stage III + with initial involvement of Stage IV: stage III + with initial involvement of CNS or bone marrow or both CNS or bone marrow or both



Principal therapy :Principal therapy :Multi agent chemotherapy : CHOPMulti agent chemotherapy : CHOP

Anticipation and treatment of complication:Anticipation and treatment of complication:Two complication : Two complication :

- Arising Space occupying tumor:- Arising Space occupying tumor:

intrathoracic complicationintrathoracic complicationpericardial effusionpericardial effusion

- Metabolic complication of chemotherapy:Metabolic complication of chemotherapy:

tumor lysis syndrometumor lysis syndrome

Management of relaps Management of relaps


Bone tumorsBone tumors

Benign and malignant tumorBenign and malignant tumorFracture pathologicFracture pathologicPlain radiography is lytic bone lesion; Plain radiography is lytic bone lesion;

cortical destructions soft tissue extension.cortical destructions soft tissue extension.Two most common cancers of bone : Two most common cancers of bone :

osteosarcoma and Ewing sarcoma and the osteosarcoma and Ewing sarcoma and the fascinating group of disorders known as fascinating group of disorders known as histiocytosis histiocytosis


OsteosarcomaOsteosarcoma

Epidemioloy and etiology:Epidemioloy and etiology:Most frequent adolescent and young adultsMost frequent adolescent and young adultsAnnual 600 new cases in USAAnnual 600 new cases in USACorrelated with radiation exposureCorrelated with radiation exposure Incidence increase among survival Incidence increase among survival

retinoblastoma (Rb) and p53 tumor supressor retinoblastoma (Rb) and p53 tumor supressor gen. gen.



Pathology:Pathology:Clinical history and radiographic appereance Clinical history and radiographic appereance

of bone lesionof bone lesionCorrect diagnosis requires of bone biopsyCorrect diagnosis requires of bone biopsy

Biology : Biology : Genetic alteration occur in the tumor Genetic alteration occur in the tumor

suppresor genes p53 and Rb to play suppresor genes p53 and Rb to play important role in tumorogenesisimportant role in tumorogenesis



Pathology:Pathology:Clinical history and radiographic appereance Clinical history and radiographic appereance

of bone lesionof bone lesionCorrect diagnosis requires of bone biopsyCorrect diagnosis requires of bone biopsy

Biology : Biology : Genetic alteration occur in the tumor Genetic alteration occur in the tumor

suppresor genes p53 and Rb to play suppresor genes p53 and Rb to play important role in tumorogenesisimportant role in tumorogenesis



Clinical presentation:Clinical presentation: Painful hard of the bonePainful hard of the bone History of trauma History of trauma Osteosarcoma can occur in any body but usually Osteosarcoma can occur in any body but usually

develop in metaphysis of long bone, common site is develop in metaphysis of long bone, common site is distal femur; proximal humerus, distal femur; proximal humerus,

Systemic symptom are fever, night sweats, weight Systemic symptom are fever, night sweats, weight loss.loss.

Metastatic disease: other bone; pulmonary; soft tissueMetastatic disease: other bone; pulmonary; soft tissue



Diagnosis and staging:Diagnosis and staging:Plain radiography lytic and/or sclerotic bone Plain radiography lytic and/or sclerotic bone

abnormalityabnormalityA “sunburst” appearance A “sunburst” appearance Cortical distruction and periosteal reactionCortical distruction and periosteal reactionMRI and biopsyMRI and biopsy



Treatment : local control of tumor and systemic Treatment : local control of tumor and systemic therapy to eradicated microscopic metastasis therapy to eradicated microscopic metastasis disease at distant sitesdisease at distant sites Preoperative chemotherapy : reduced tumor bulk; Preoperative chemotherapy : reduced tumor bulk;

prognostic implication; making surgical resection prognostic implication; making surgical resection easiereasier

HDX MTX; doxorubicin and cisplatinHDX MTX; doxorubicin and cisplatin Post operative chemotherapy 12 monthPost operative chemotherapy 12 month Non metastatic disease : 60 -65% to be cured Non metastatic disease : 60 -65% to be cured

Metastatic diseases indicated poor prognosisMetastatic diseases indicated poor prognosis


Ewing sarcomaEwing sarcoma

Ewing sarcoma is one of a group of Ewing sarcoma is one of a group of tumors referred to as the Ewing sarcoma tumors referred to as the Ewing sarcoma family tumors (ESFT): Ewing sarcoma of family tumors (ESFT): Ewing sarcoma of bone; soft tissue Ewing sarcoma and bone; soft tissue Ewing sarcoma and peripheral primitive neuro ectodermal peripheral primitive neuro ectodermal tumor (PNET)tumor (PNET)

Arise from share the same chromossomal Arise from share the same chromossomal abnormality abnormality


Ewing sarcomaEwing sarcoma

Epidemiology and etiologyEpidemiology and etiologyLess than 20 years at diagnosisLess than 20 years at diagnosisAnnual incidence 2.7 case permillionAnnual incidence 2.7 case permillionThe cause of ESFT is unknown, unrelated The cause of ESFT is unknown, unrelated

with radiationwith radiationPathology and biology:Pathology and biology:


Soft Tissue SarcomasSoft Tissue Sarcomas

Arise from mesenchymal tissueArise from mesenchymal tissueCan arise anywhere in bodyCan arise anywhere in bodyWide variety of histologiesWide variety of histologies

Most common 60 %Most common 60 %RhabdomyosarcomasRhabdomyosarcomasUndifferentiated Undifferentiated



Diagnosis and StagingDiagnosis and StagingDepends on area involvedDepends on area involvedH&N, genitourinary, extremities and trunkH&N, genitourinary, extremities and trunk

A large painless mass- extremityA large painless mass- extremityPainful orbitPainful orbit

In genitourinary areas- urinary problems In genitourinary areas- urinary problems (male)(male)

Exophytic mass vaginaExophytic mass vaginaMets to lung and bone marrow most commonMets to lung and bone marrow most common



StagingStagingPhysical examPhysical examUSUSCT or MRICT or MRIBM biopsy/Chest CT (mets)BM biopsy/Chest CT (mets)Stage and histology dictate txStage and histology dictate tx


StagingStaging

TreatmentTreatment SurgerySurgery

First choice is removalFirst choice is removal RTRT

Improves local control, spare organsImproves local control, spare organs Field covers entire muscular compartmentField covers entire muscular compartment 50.4 Gy 1.8 Gy fx50.4 Gy 1.8 Gy fx Shield growth platesShield growth plates Spare BM in pelvis if possibleSpare BM in pelvis if possible BrachyBrachy

Chemo- resected/nonresected tumorsChemo- resected/nonresected tumors Vincristine and actinomycin-DVincristine and actinomycin-D


Unusual Childhood TumorsUnusual Childhood Tumors

Germ Cell TumorsGerm Cell Tumors Can occur anywhere in the midline from CNS to Can occur anywhere in the midline from CNS to

ovaries and testesovaries and testes Sacrococcygeal teratomas often seen in newbornsSacrococcygeal teratomas often seen in newborns NonseminomasNonseminomas

Embryonal carcinomaEmbryonal carcinoma ChoriocarcinomaChoriocarcinoma Yolk-sac tumorsYolk-sac tumors These often produce AFP, HCG chemical markersThese often produce AFP, HCG chemical markers

Seminoma- dysgerminomaSeminoma- dysgerminoma



Liver tumorsLiver tumors Appear much like Wilm’s tumor or Appear much like Wilm’s tumor or

neuroblastomaneuroblastoma CT or US will differentiateCT or US will differentiate

Hepatoblastoma- under 2 yrs oldHepatoblastoma- under 2 yrs old Hepatocellular carcinoma- after 10 yrs old, multifocalHepatocellular carcinoma- after 10 yrs old, multifocal Surgery- primary txSurgery- primary tx

Chemo- often usedChemo- often used RT- rarely usedRT- rarely used Poor survival ratesPoor survival rates



Histiocytosis X syndromesHistiocytosis X syndromes Spectrum of diseaseSpectrum of disease Involves Langerhan’s cellsInvolves Langerhan’s cells

Miscellaneous TumorsMiscellaneous Tumors Nasopharyngeal angiofibromasNasopharyngeal angiofibromas Undifferentiated nasopharyngealUndifferentiated nasopharyngeal Thyroid tumors- benign and malignant, Iodine 131-Thyroid tumors- benign and malignant, Iodine 131-

ablationablation Keloids or fibromasKeloids or fibromas Kaposi’s sarcomaKaposi’s sarcoma


Pediatric Patients- Special Pediatric Patients- Special ConsiderationsConsiderations

Extra Time NeededExtra Time NeededAnesthesiaAnesthesiaPsychosocial needs of family- jobs/homePsychosocial needs of family- jobs/home

Understanding/compassionate therapistUnderstanding/compassionate therapist

Use of rewards- stickers/toysUse of rewards- stickers/toysProtocolsProtocols

Info sent for immediate reviewInfo sent for immediate reviewCareful adherence to guidelines importantCareful adherence to guidelines important


Thank for your attention

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Pediatric Solid Tumors Kuliah S1