Ewing's sarcoma
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Transcript of Ewing's sarcoma
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Ewings Tumor
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History:1928 James Ewing described a rare lesion
Diaphysis of long bonesChildhoodFebrile attacks,anorexia, weight loss &
anaemiaRapidly involving other parts of skeletonRadiosensitive Histologically –endothelial elements in marrow
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Willis – All these tumors were not Ewing’s butNeuroblastomasReticulum cell sarcomasMetastatic tumors
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Ewing’s SarcomaEpidemiology:
4th. m.c. primary malignancy of bone2nd. m.c. in age < 30 yrs.Incidence < 1/ million / year9% of primary malignancies of
bone in the Mayo Clinic series.
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Age – Wide age range from infants to elderly m.c. 5 – 15 yrs.
Male slightly more than females.Very rare in Africans
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Location:M.c. in
metaphysis of long bones ( often extending into diaphysis),
Flat bones of shoulder and pelvis.Rarely in spine or small hand &
foot bones.Long bones
Tibia > fibula > humerus > femur.
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Spread –Whole of the skeleton.Regional lymph nodes.
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Clinical features:Pain – Universal complaint
Insidious in onset, long standing, intermittent attacks, responding to analgesics.
Often H/O preceding trauma.Slow growing tumor
in relation to the shaft of long bones.
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Delay in diagnosis Av. 34 weeksPatient delay – 15 weeksPhysician delay 19 weeks – Importance of
Xrays & rpt. X rays to compare.During attacks of pain tumor size may
enlarge visibly.
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Other clinical features:Fever, erythema & swelling ~ OsteomyelitisSkin – not involved unless surgical
exploration done.Pathological # seldom.Later stage – multiple deposits in skull, ribs,
sternum, pelvis & other long bones – cachexia & sec. Anaemia.
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Vertebral involvement – severe root pain or paralysis
Death – Metastatic involvement of lungs.
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Radiological appearance:X-rays:
Classically Destructive lesions,
diaphysis long bones, onion peel appearance.
Reality Metaphysis long bones frequently
extending into diaphysis.
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Diffuse rarefaction towards center of shaft extending to considerable area peripherally.
Flat bones:Non-specific destructive lesions & large
adjacent soft tissue mass.
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Early stage:Condensation
LaterReactive irritation – Onion skin layers
Last stageGross tumor formation, destruction of
bone, pathological #s.
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MRI:Taken of entire bone involvedExtent of diseased boneSoft tissue involvement
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Other Investigations:Blood Ix:
WBC Leukocytosis, ESR CRP LDH ~ suggestive of infective cause.
FNAC – resembles Pus.
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Base line X ray & CT chest –m.c. site of metastasis
Bone ScanBone marrow aspiration – routine
R/O diffuse systemic diseaseUSG abdomen
liver & spleen.
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Pathology:Begins in - Marrow (Mid shaft)Gross –
Color - Greyish-whiteAreas of necrosis & hemorrhage with cyst
formation.Lamellae destruction.
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Medulla Haversian canals Surface.Sub periosteal compensatory layers of new
bone are deposited only to be destroyed Onion Skin appearance.
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Histology:Microscopic features –
non specific.Intensely cellularCells –
Usually one typeBlue,Small, round &
polyhedralArrangement –
solid cords or sheets.
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Intercellular substance – minimalNecrosisRemaining cells arrange around the central
blood vessel – perithelioma.Nuclei – prominentMitosis - frequent
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Rossette arrangement with central fibril (Neuroblastoma)
Pseudorosette (without fibril) more common.
Despite bone destructionGiant cells/ osteoclasts are not found
Nor new boneExcept sub periosteal
deposits.
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Vessels & lymphatics Tumor emboli tumor spread.
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Cytogenetics & Immunohistochemistry:To differentiate from other small blue cell
tumors.m.c. translocations in >90% cases t(11;22)
(q24;q12) diagnostic of Ewing’s.Other diagnostic translocations include
t(21;22)(q22;q12) &t(7;22)(p22;p12)
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IHCStaining for MIC2 gene products – SpecificPAS + usually (high intracellular glycogen).Reticulin – ( c.f. lymphomas)
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Histological D/DLymphomas – PAS -, Reticulin +, Embryonal rhabdomyosarcoma – desmin ,
myoglobin, muscle specific actins +Hemangeopericytomas – Factor VIII +Small cell metastatic carcinomas & Melanomas
– Cytokeratin +
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Other D/D:Chronic OsteomyelitisMetastatic NeuroblastomaHistiocytosisRarely Osteolytic Osteosarcoma
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Treatment:Adjunct or Neoadjunct Chemotherapy or both
– for distant metastasis.Local Treatment: (Controversial)Highly radiosensitiveWide resection
Decreases local recurrence to <10%Increases overall survival
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Large, central, unresectable – RadiationSmall, more accessible lesions – SurgeryChoice is individual based.Repeat Staging studies
after neoadjunct chemotherapy.Repeat X rays - ossificationRepeat MRI - soft tissue mass.
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At this stage – If Lesions can be resected with wide margins &
an acceptable functional deficit – SURGERY.If not – RADIATION.
Radiation:Marginal Resection orContaminated wide resection.
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Treatment Plan:After long D/W patients & parentsInclude expected function after amputation,
limb salvage & radiationInherent short & long term risks with each
option.Disease relapse – and its prognosis.
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Survival rates :Long term survival rates reported from most
studies – 60-70%Before the use of chemotherapy – 10 %
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Prognostic factors:Location & Size of primary lesion.Older age of presentation & male gender.Distant metastasis – worst prognostic factor
with 20% long term survival even with aggressive therapy and surgery.
Disease relapseTime to relapse
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Histological grade is of no significance as ALL EWING’S SARCOMAS ARE CONSIDERED TO BE HIGH GRADE.
Fever, anemia, WBC, ESR, LDH indicate extensive disease so worse prognosis.
Aberrant p53 expression & histological response to chemotherapy.