TUMOR Dr Mohammad.ali Department of Orthopedics. PATHOLOGY of NEOPLASM Abnormal mass of tissue Growth is autonomous, exceeds normal, & persist after cessation

TUMORTUMOR

Dr Mohammad.aliDr Mohammad.ali

Department of OrthopedicsDepartment of Orthopedics

PATHOLOGY of NEOPLASMPATHOLOGY of NEOPLASM

Abnormal mass of tissueAbnormal mass of tissue

Growth is autonomous, exceeds Growth is autonomous, exceeds normal, & persist after cessation of normal, & persist after cessation of stimulistimuli

Benign vs. MalignantBenign vs. Malignant– Differentiation & AnaplasiaDifferentiation & Anaplasia– Growth rateGrowth rate– Local invasionLocal invasion– Ability to metastasizeAbility to metastasize

HISTOPATHOLOGYHISTOPATHOLOGY

AnaplasiaAnaplasia– Hallmark of Hallmark of

malignancymalignancy

– PleomorphismPleomorphism– HyperchromatismHyperchromatism– Nuclear cytoplasmic Nuclear cytoplasmic

ratio of 1:1ratio of 1:1– Abundant mitosesAbundant mitoses– Tumor giant cellsTumor giant cells

BENIGN more commonBENIGN more commonMALIGNANT (SARCOMAS) are rareMALIGNANT (SARCOMAS) are rare

Differentiation of the lesionDifferentiation of the lesion– Fibrous tissueFibrous tissue– FibrohistiocyticFibrohistiocytic– Adipose tissueAdipose tissue– Muscle tissueMuscle tissue– Lymph vesselsLymph vessels– Synovial tissueSynovial tissue– Peripheral nervesPeripheral nerves– Cartilage and Bone Forming tissueCartilage and Bone Forming tissue– Pluripotential MesenchymePluripotential Mesenchyme– Blood VesselsBlood Vessels– Uncertain HistogenesisUncertain Histogenesis

DIAGNOSTIC CLUESDIAGNOSTIC CLUES

Size Size A mass that is small (< 5 cm in its A mass that is small (< 5 cm in its greatest dimension) is unlikely to be greatest dimension) is unlikely to be malignant, while a mass that is > 5 cm malignant, while a mass that is > 5 cm has at least a 20%has at least a 20% chance of being a chance of being a soft tissue sarcomasoft tissue sarcoma

Determined by physical examination if Determined by physical examination if the lesion is subcutaneous and easily the lesion is subcutaneous and easily palpable, or by ultrasound, CT or MRIpalpable, or by ultrasound, CT or MRI


Superficial or deep?Superficial or deep? Superficial lesions are more likely to be Superficial lesions are more likely to be benign and, when malignant, may have benign and, when malignant, may have a better prognosis than deep lesionsa better prognosis than deep lesions

The depth is best determined by The depth is best determined by physical exam, ultrasound or MRIphysical exam, ultrasound or MRI

The thigh and buttocks are the 2 most common sites of sarcomas. Any large deep mass in the thigh or buttocks should be considered at high risk for being a malignant lesion


Cystic or solidCystic or solid Most cystic lesions are inflammatory or Most cystic lesions are inflammatory or benign lesions, such as ganglion cysts or benign lesions, such as ganglion cysts or soft tissue abscessessoft tissue abscesses

If the lesion is solid, it could represent If the lesion is solid, it could represent either a benign or malignant neoplasmeither a benign or malignant neoplasm

Attempt TransilluminationAttempt Transillumination

If deep, ultrasound or MR scan will If deep, ultrasound or MR scan will determine thisdetermine this


Length of symptomsLength of symptoms Rapidly increased in size over 2 months is Rapidly increased in size over 2 months is more likely to be a sarcoma than the lesion more likely to be a sarcoma than the lesion that has slowly enlarged over a 20-year that has slowly enlarged over a 20-year periodperiod

A mass that increases and decreases in size A mass that increases and decreases in size is usually a cystic lesionis usually a cystic lesion

Caution should be taken with masses that Caution should be taken with masses that have been present for a long time. Soft have been present for a long time. Soft tissue sarcomas occasionally present with a tissue sarcomas occasionally present with a history of many years duration up to 30 history of many years duration up to 30 yearsyears

TUMOUR WORKUPTUMOUR WORKUPHistory (age, sex, site and past history) History (age, sex, site and past history) Clinical examinationClinical examination– Thyroid Thyroid – Breasts Breasts – Chest Chest – Liver Liver – Kidney Kidney – Rectal (prostate & rectal tumors) Rectal (prostate & rectal tumors)

Bloods Bloods – FBC (leukaemic cells etc) & ESR (often elevated) FBC (leukaemic cells etc) & ESR (often elevated) – Biochemistry (Ca++, PO4, liver enzymes and Alkaline Biochemistry (Ca++, PO4, liver enzymes and Alkaline

Phosphatase) -> mets Phosphatase) -> mets – Acid Phosphatase (prostate and increased with Acid Phosphatase (prostate and increased with

metastatic deposits) metastatic deposits) – Thyroid function tests Thyroid function tests – PSA PSA – Serum Protein Electrophoresis (Myeloma) Serum Protein Electrophoresis (Myeloma)

TUMOUR WORKUP (cont)TUMOUR WORKUP (cont)

Urinalysis Urinalysis Urine Bence-Jones (myeloma) Urine Bence-Jones (myeloma) CXR CXR Abdominal ultrasound Abdominal ultrasound Bone scan -> other sites Bone scan -> other sites MRI -> soft tissue extent and association with MRI -> soft tissue extent and association with nerves and vessels nerves and vessels CT of lesion and chest (-> staging) CT of lesion and chest (-> staging) Angiography -> tumor blood supply and Angiography -> tumor blood supply and relationship to major vessels relationship to major vessels BiopsyBiopsy

IMAGING OF SOFT TISSUE IMAGING OF SOFT TISSUE TUMORTUMOR

Plain radiographPlain radiograph– Soft tissue shadow, isodense with muscleSoft tissue shadow, isodense with muscle– Specific featuresSpecific features

Phlebolith within a hemangiomaPhlebolith within a hemangioma

Cartilaginous juxta-articular masses in synovial Cartilaginous juxta-articular masses in synovial osteochondromatosisosteochondromatosis

Mature peripheral calcification in myositis Mature peripheral calcification in myositis ossificansossificans

Central calcification in extraosseous Central calcification in extraosseous osteosarcomaosteosarcoma

Amorphous calcium deposit in tumoral Amorphous calcium deposit in tumoral calcinosiscalcinosis

– Bony involvementBony involvement


UltrasoundUltrasound– Rapid inexpensive testRapid inexpensive test– Differentiate solid from cysticDifferentiate solid from cystic– Determine sizeDetermine size– Can suggest sarcoma featuresCan suggest sarcoma features

As many soft tissue sarcoma present as As many soft tissue sarcoma present as hypoechoic mass than the echogenic pattern seen hypoechoic mass than the echogenic pattern seen in benign lesion, with exception of liposarcomain benign lesion, with exception of liposarcoma

– Delineate areas of distinctly solid portion of Delineate areas of distinctly solid portion of a a mass, a great help for biopsya a mass, a great help for biopsy

– Aid in percutaneous needle biopsyAid in percutaneous needle biopsy– Study of vascular supply by Color DopplerStudy of vascular supply by Color Doppler


CT scanCT scan– Detecting calcification or ossification within Detecting calcification or ossification within

the lesionthe lesion– Evaluate lung metastasesEvaluate lung metastases– CT guided needle biopsyCT guided needle biopsy

ArteriographyArteriography– Less frequently usedLess frequently used– Mapping of lesions in difficult anatomy Mapping of lesions in difficult anatomy

locationlocation– Identifying normal anatomic variants before Identifying normal anatomic variants before

surgerysurgery– Preoperatively, to embolize hypervascular Preoperatively, to embolize hypervascular

lesionslesions


Bone scanBone scan– Technetium Tc 99m bone scanTechnetium Tc 99m bone scan– Detects area of rapid bone turnoverDetects area of rapid bone turnover– Sensitive but not very specific, Sensitive but not very specific,

abnormal scan should be further abnormal scan should be further studied with plain radiograph, CT and studied with plain radiograph, CT and MRIMRI

– Part of stagingPart of staging


MRIMRI– Replace role of CT and arteriographyReplace role of CT and arteriography– AdvantagesAdvantages

superior soft tissue contrastsuperior soft tissue contrast multiplanar imagingmultiplanar imaging no ionizing radiation & the need for iodinated contrast no ionizing radiation & the need for iodinated contrast agentagent no artifact problemno artifact problem

– General RolesGeneral RolesAccurate preoperative stagingAccurate preoperative stagingRestriction of differential diagnosisRestriction of differential diagnosisAssistance with biopsy placementAssistance with biopsy placementMonitoring response to neoadjuvant chemotherapy or Monitoring response to neoadjuvant chemotherapy or radiotherapyradiotherapyIdentification of residual or recurrent tumor during Identification of residual or recurrent tumor during postoperative periodpostoperative period


MRI (cont)MRI (cont)– Conventional T1 weighted and T2 Conventional T1 weighted and T2

weightedweighted– T1 weighted differentiate the T1 weighted differentiate the

hyperintense fatty tissue with hyperintense fatty tissue with hypointense tumorhypointense tumor

– T2 weighted with or without fat T2 weighted with or without fat suppression is the most appropriate suppression is the most appropriate sequence; tumor will be hyperintensesequence; tumor will be hyperintense

STAGING SYSTEMSTAGING SYSTEM

Prognostic variablesPrognostic variables– Histologic gradeHistologic grade– Tumor sizeTumor size– Tumor depthTumor depth– Compartment statusCompartment status– MetastasesMetastases


SURGICAL STAGING SYSTEM SURGICAL STAGING SYSTEM (SSS)(SSS)– Enneking staging systemEnneking staging system

American Joint Committee on American Joint Committee on Cancer (AJCC)Cancer (AJCC)– Cancer Staging Manual 5Cancer Staging Manual 5thth Edition Edition

Memorial Sloan Kettering Cancer Memorial Sloan Kettering Cancer Center (MSK)Center (MSK)


SURGICAL STAGING SYSTEM SURGICAL STAGING SYSTEM (Enneking)(Enneking)

StageStage Stage Stage DescriptionDescription

Prognostic FactorsPrognostic Factors

IA Low GradeIA Low Grade

IntracompartmentalIntracompartmentalG1 T1 M0G1 T1 M0 G1 Low GradeG1 Low Grade

G2 High GradeG2 High Grade

T1 T1 IntracompartmentalIntracompartmental

T2 T2 ExtracompartmentalExtracompartmental

M1 Any regional orM1 Any regional or

systemic systemic metastasismetastasis

IB Low GradeIB Low Grade

ExtracompartmentalExtracompartmentalG1 T2 M0G1 T2 M0

IIA High GradeIIA High Grade

IntracompartmentalIntracompartmentalG2 T1 M0G2 T1 M0

IIB High GradeIIB High Grade

ExtracompartmentalExtracompartmentalG2 T2 M0G2 T2 M0

III Any MetastasesIII Any Metastases Any G, Any T, Any G, Any T, M1M1

Prognostic FactorsPrognostic FactorsHistologic Grade Histologic Grade (assessment of biological aggressiveness) (assessment of biological aggressiveness) – G0 G0

Histological benign Histological benign Well differentiated and low cell to matrix ratioWell differentiated and low cell to matrix ratio

– G1 G1 Low grade malignant Low grade malignant Few mitoses, moderate differentiation and local spread Few mitoses, moderate differentiation and local spread onlyonlyHave low risk of metastasesHave low risk of metastases

– G2 G2 High grade malignancy High grade malignancy Frequent mitoses, poorly differentiatedFrequent mitoses, poorly differentiatedHigh risk of metastasesHigh risk of metastases

Features of aggressive tumors: Features of aggressive tumors: – Cellular atypia Cellular atypia – Frequent mitoses Frequent mitoses – Extensive necrosis Extensive necrosis – Significant vascularity Significant vascularity – Small amounts of immature matrix Small amounts of immature matrix


Low GradeLow Grade High GradeHigh Grade

Myxoid LiposarcomaMyxoid Liposarcoma

Lipoma-like Lipoma-like LiposarcomaLiposarcoma

Angiomatoid Angiomatoid Malignant Malignant

Fibrous Fibrous HistiocytomaHistiocytoma

Malignant Fibrous Malignant Fibrous

HistiocytomaHistiocytoma

Pleomorphic Pleomorphic LiposarcomaLiposarcoma

Synovial SarcomaSynovial Sarcoma

RhabdomyosarcomaRhabdomyosarcoma


SiteSite (anatomic setting of the lesion) (anatomic setting of the lesion)

– T0T0 Intracapsular Intracapsular

– T1T1 Intracompartmental Intracompartmental (e.g. cortical bone, joint capsule (e.g. cortical bone, joint capsule

or fascia)or fascia)

– T2T2 Extracompartmental Extracompartmental (spreads beyond 'fascial' plane (spreads beyond 'fascial' plane

without longitudinal without longitudinal containment)containment)

Prognostic FactorsPrognostic FactorsIntracompartmentIntracompartment ExtracompartmentExtracompartment

IntraosseousIntraosseous

IntraarticularIntraarticular

Intrafascial Intrafascial compartmentscompartments

Ray of Hand or FootRay of Hand or Foot

Posterior or Anterior LegPosterior or Anterior Leg

Ant, Med, Post ThighAnt, Med, Post Thigh

ButtocksButtocks

Volar or Dorsal ForearmVolar or Dorsal Forearm

Ant or Post ArmAnt or Post Arm

PericapsularPericapsular

Soft Tissue ExtensionSoft Tissue Extension

Deep Fascial Deep Fascial ExtensionExtension

Extrafascial Extrafascial Planes/Spaces Planes/Spaces (Neurovascular containing (Neurovascular containing spaces)spaces)

Mid & Hind FootMid & Hind Foot

Mid HandMid Hand

Popliteal FossaPopliteal Fossa

Groin-Femoral TriangleGroin-Femoral Triangle

Intra PelvicIntra Pelvic

Antecubital FossaAntecubital Fossa

AxillaAxilla

ParaspinalParaspinal

BIOPSY OF TUMORSBIOPSY OF TUMORS

Simple ? Low Risk Procedure ?Simple ? Low Risk Procedure ?

The planning of the biopsy is technically The planning of the biopsy is technically demanding demanding

All Biopsy carry extreme risk to patient’s All Biopsy carry extreme risk to patient’s limb and potentially to the patient’s lifelimb and potentially to the patient’s life

Poorly planned biopsy can affect Poorly planned biopsy can affect diagnostic accuracy and result in delay diagnostic accuracy and result in delay in diagnosis and treatmentin diagnosis and treatment


Planning the biopsyPlanning the biopsy– Basic understanding of Basic understanding of

diseases/tumors and an ability to diseases/tumors and an ability to generate differential diagnosisgenerate differential diagnosis

The differential diagnosis determines the The differential diagnosis determines the indications for biopsyindications for biopsy

– Knowledge of appropriate placement Knowledge of appropriate placement of limb salvage incisions for resection of limb salvage incisions for resection and reconstructionand reconstruction

– Access to experienced Access to experienced musculoskeletal pathologistmusculoskeletal pathologist


Type of BiopsyType of Biopsy– Closed Biopsy (Core Needle)Closed Biopsy (Core Needle)– Open BiopsyOpen Biopsy

Incisional BiopsyIncisional Biopsy

Excisional BiopsyExcisional Biopsy

Primary Wide ExcisionPrimary Wide Excision


ClosedClosed OpenOpen

Accuracy of 85%Accuracy of 85%

Attributed to small size of Attributed to small size of biopsy leading to sampling biopsy leading to sampling errorerror

Insufficient for electron Insufficient for electron microscopic, microscopic, immunohistochemical, and immunohistochemical, and molecular genetic testingmolecular genetic testing

Non diagnostic needle Non diagnostic needle biopsy results in delay in biopsy results in delay in diagnosis and the need for diagnosis and the need for subsequent formal open subsequent formal open biopsybiopsy

Accuracy of 96% Accuracy of 96%

Further minimizes risk of Further minimizes risk of misdirected treatment and misdirected treatment and its associated morbidityits associated morbidity

BIOPSY OF TUMORSBIOPSY OF TUMORSClosedClosed OpenOpen

Less invasiveLess invasive InvasiveInvasive

Requires only LA outside Requires only LA outside formal OTformal OT

Requires Formal Anesthetics Requires Formal Anesthetics Support and OTSupport and OT

Less soft tissue Less soft tissue contaminationcontamination

Needle tract contaminationNeedle tract contamination

Inappropriate incision Inappropriate incision placement & soft tissue placement & soft tissue contamination that cannot be contamination that cannot be incorporated into a limb incorporated into a limb salvage or amputation plansalvage or amputation plan

Leading to increase likelihood Leading to increase likelihood of the need for soft tissue of the need for soft tissue coverage and a higher risk for coverage and a higher risk for amputationamputation

Lower risk of complicationLower risk of complication Higher risk of perioperative Higher risk of perioperative complication; infection, complication; infection, haematoma and pathological haematoma and pathological ##

Useful in areas of difficult Useful in areas of difficult access, like spine and pelvis access, like spine and pelvis without risking significant without risking significant contaminationcontamination

Biopsy TechniqueBiopsy Technique

Incisional BiopsyIncisional Biopsy– Directly cutting into tumor to remove a sample Directly cutting into tumor to remove a sample

without excising lesionwithout excising lesion– The entire field is excised en bloc with the major The entire field is excised en bloc with the major

tumor mass at the time of definitive resectiontumor mass at the time of definitive resection

Excisional BiopsyExcisional Biopsy– Removing the entire lesion at the time of biopsyRemoving the entire lesion at the time of biopsy– Marginal excisionMarginal excision

Primary Wide ExcisionPrimary Wide Excision– Entire lesion is excised while cutting through normal Entire lesion is excised while cutting through normal

healthy tissue and leaving a margin of surrounding healthy tissue and leaving a margin of surrounding healthy tissue against the lesionhealthy tissue against the lesion

Biopsy TechniqueBiopsy TechniqueExcisional BiopsyExcisional Biopsy

IndicationIndication– To obtain a large sample sizeTo obtain a large sample size– For benign non aggressive diagnosis, is For benign non aggressive diagnosis, is

the treatment of the lesion in single the treatment of the lesion in single stagestage

Carry higher risk of extensive soft Carry higher risk of extensive soft tissue & important structures tissue & important structures contaminationcontamination

Not for lesion which is suspiciously Not for lesion which is suspiciously malignant or at high risk anatomical malignant or at high risk anatomical regionregion

Biopsy TechniqueBiopsy TechniquePrimary Wide Excision BiopsyPrimary Wide Excision Biopsy

IndicationIndication– High suspicious of malignancyHigh suspicious of malignancy– When the risk of contaminating When the risk of contaminating

major important structures with major important structures with another form of biopsy another form of biopsy outweighs the risks and outweighs the risks and functional and cosmetic deficits functional and cosmetic deficits of excising the lesions primarilyof excising the lesions primarily

Biopsy ProcedureBiopsy Procedure

Longitudinal IncisionLongitudinal IncisionLimb Salvage or Amputation incisionLimb Salvage or Amputation incisionSurgical Instrument handlingSurgical Instrument handlingAnybody fingers should not be placed Anybody fingers should not be placed directly into the wounddirectly into the woundSoiled spongesSoiled spongesThe surgical field should not be in The surgical field should not be in continuity with other surgical field continuity with other surgical field Tourniquet, no exsanguinationsTourniquet, no exsanguinations

Biopsy Procedure (cont)Biopsy Procedure (cont)

Direct approach, contaminating only one Direct approach, contaminating only one compartment, not through planescompartment, not through planesHemostasisHemostasisVessels, nerves and tendons should not be Vessels, nerves and tendons should not be exposedexposedBlunt retractors drapingBlunt retractors drapingBiopsies the peripheral portion with ellipse shaped Biopsies the peripheral portion with ellipse shaped cutcutCulturesCulturesVolume of 1 to 2 cm3 Volume of 1 to 2 cm3 Post biopsy hemostasisPost biopsy hemostasisClosure in layersClosure in layersSmall bite suturingSmall bite suturingLight pressure bandagingLight pressure bandagingImmobilization and protection of weight bearingImmobilization and protection of weight bearing

OPTION OF DEFINITIVE OPTION OF DEFINITIVE MANAGEMENTMANAGEMENT

CLINICAL AND RADIOLOGICAL DIAGNOSIS

BIOPSY OBSERVE

STAGING STUDIES

ELIMINATION OF PRIMARY TUMOR

SURGERY NON SURGERY

RADIOTHERAPY CHEMOTHERAPY

GENETIC

IMMUNOLOGIC

PROTEIN BASED

COMBINATION OF SURGERY AND NON SURGERY

PRINCIPLES OF TUMOR PRINCIPLES OF TUMOR SURGERYSURGERY

INTRALESIONALINTRALESIONAL– Incomplete excisions, either gross or microscopic tumor Incomplete excisions, either gross or microscopic tumor

remains ( MARGIN? )remains ( MARGIN? )

MARGINAL EXCISIONMARGINAL EXCISION– Resection at border between the tumor & the immediate Resection at border between the tumor & the immediate

adjacent tissues, leaving no tumor, as verified by both gross adjacent tissues, leaving no tumor, as verified by both gross and microscopic inspection ( SATELLITE LESION? )and microscopic inspection ( SATELLITE LESION? )

WIDE EXCISIONWIDE EXCISION– Excision through normal tissue that is not reactive or Excision through normal tissue that is not reactive or

edematous, as judged by preoperative MRI, intraoperative edematous, as judged by preoperative MRI, intraoperative gross inspection and microscopic sections ( SKIP LESION? )gross inspection and microscopic sections ( SKIP LESION? )

RADICAL EXCISIONRADICAL EXCISION– Wide excision based on anatomic barriers to tumor Wide excision based on anatomic barriers to tumor

infiltration ( METASTATIC LESION?)infiltration ( METASTATIC LESION?)

? COMPLETE REMOVAL AND RECURRANCE RATE? COMPLETE REMOVAL AND RECURRANCE RATE


TUMOR RESECTION STRATEGIESTUMOR RESECTION STRATEGIES

– COMPLETE RESECTIONCOMPLETE RESECTION for for CURECURE

– INCOMPLETE RESECTIONINCOMPLETE RESECTION with with CURECURE obtained from obtained from LOCAL ADJUVANT THERAPIESLOCAL ADJUVANT THERAPIES

– PARTIAL RESECTIONPARTIAL RESECTION without the expectation of without the expectation of achieving cure achieving cure (DEBULKING)(DEBULKING)

CURE means when no local or distant viable CURE means when no local or distant viable tumor cells remain after surgical resectiontumor cells remain after surgical resection

Tumor debulking is to eliminate a majority of the Tumor debulking is to eliminate a majority of the tumor with minimum of morbidity to prolong tumor with minimum of morbidity to prolong quality of life and to reduce or eliminate painquality of life and to reduce or eliminate pain


Meticulous attention to the Meticulous attention to the isolation of clean and contaminated isolation of clean and contaminated fields, instruments, and personnelfields, instruments, and personnel

Minimize perioperative exposure to Minimize perioperative exposure to pathogenspathogens– IV antibiotic, antibiotic cement, IV antibiotic, antibiotic cement,

antibiotic in irrigation mediumantibiotic in irrigation medium– Allograft and Prosthetic componentsAllograft and Prosthetic components


HIGH GRADE SARCOMAHIGH GRADE SARCOMA– The goal is to remove as much tumor to achieve The goal is to remove as much tumor to achieve

cure while removing the least amount of healthy cure while removing the least amount of healthy tissue as possible to preserve functiontissue as possible to preserve function

– AMPUTATION versus Limb Salvage SurgeryAMPUTATION versus Limb Salvage Surgery– No Significant Statistical Advantage in term of No Significant Statistical Advantage in term of

Local RecurrenceLocal RecurrenceFollowing amputationFollowing amputation 1 to 3%1 to 3%Following limb salvage surgeryFollowing limb salvage surgery <8%<8%

– Functional loss can be extreme in amputation Functional loss can be extreme in amputation groupgroup

– Revision surgery rates for complications and Revision surgery rates for complications and revisions are considerably lower for amputation revisions are considerably lower for amputation groupgroup


HIGH GRADE SARCOMAHIGH GRADE SARCOMA– Superficial sarcoma of the trunk have Superficial sarcoma of the trunk have

better prognosis than deep axially better prognosis than deep axially located tumorlocated tumor

– Deep axially located tumor like around Deep axially located tumor like around the spine and pelvisthe spine and pelvis

Usually present late and often large by the Usually present late and often large by the time the diagnosis is madetime the diagnosis is made

Do not routinely permit large volumes of Do not routinely permit large volumes of normal surrounding tissue to be removed normal surrounding tissue to be removed with the tumorwith the tumor


HIGH GRADE SARCOMAHIGH GRADE SARCOMA– The more inflammatory, fast growing, The more inflammatory, fast growing,

& infiltrative the tumor, the wider the & infiltrative the tumor, the wider the margin should be, & the more strongly margin should be, & the more strongly preoperative radiation or preoperative radiation or chemotherapy is indicatedchemotherapy is indicated

– Final consideration is the patient’s Final consideration is the patient’s personal wishes.personal wishes.


LOW GRADE SARCOMALOW GRADE SARCOMA– Generally treated similar to the high gradeGenerally treated similar to the high grade– Margins are typically smaller as they show;Margins are typically smaller as they show;

Smaller or absent reactive zone of inflammationSmaller or absent reactive zone of inflammationLimited infiltrationLimited infiltration

– However a wider margin of excision might However a wider margin of excision might be preferable it if would eliminate the need be preferable it if would eliminate the need for radiation therapyfor radiation therapy

– Cure is achieved through a carefully Cure is achieved through a carefully planned surgical excision without adjuvant planned surgical excision without adjuvant therapytherapy


DEFECT RECONSTRUCTIONDEFECT RECONSTRUCTION– The second part of surgical resectionThe second part of surgical resection– Planned preoperativelyPlanned preoperatively– Liaison with plastic surgeonLiaison with plastic surgeon

– Principles for Pure Soft Tissue Principles for Pure Soft Tissue Defect ReconDefect Recon

Preserve Limb ViabilityPreserve Limb Viability Restore FunctionRestore Function Achieve Skin ClosureAchieve Skin Closure


DEFECT RECONSTRUCTIONDEFECT RECONSTRUCTION– Principles of Wound ClosurePrinciples of Wound Closure

To have the most rapid biologic To have the most rapid biologic reconstitution of a viable skin barrier, reconstitution of a viable skin barrier, between the deep tissue and outside between the deep tissue and outside world world

Minimize ‘dead’ space in the deep tissue Minimize ‘dead’ space in the deep tissue layer which can harbor bacterial inoculumlayer which can harbor bacterial inoculum

– Radiation and Chemotherapy impair Radiation and Chemotherapy impair wound healingwound healing


DEFECT RECONSTRUCTIONDEFECT RECONSTRUCTION– Dynamic ReconstructionDynamic Reconstruction– Space Filling ReconstructionSpace Filling Reconstruction– Skin Barrier ReconstructionSkin Barrier Reconstruction


DEFECT RECONSTRUCTIONDEFECT RECONSTRUCTION– Dynamic ReconstructionDynamic Reconstruction

Dynamic function include stability, power, Dynamic function include stability, power, blood transport or nerve conductionblood transport or nerve conduction

Refers to the implant or transfer of tissuesRefers to the implant or transfer of tissues– Ligaments deficit in knee replaced with Ligaments deficit in knee replaced with

autograft, allograft or artificial replacementautograft, allograft or artificial replacement– Vessels Reconstruction to restore blood flowVessels Reconstruction to restore blood flow– Nerve Grafting for protective sensation and Nerve Grafting for protective sensation and

motor functionmotor function– Tendon or Muscle Transfer restore lost motor Tendon or Muscle Transfer restore lost motor

powerpower


DEFECT RECONSTRUCTIONDEFECT RECONSTRUCTION– Space Filling ReconstructionSpace Filling Reconstruction

To fills defects created by surgeryTo fills defects created by surgery

Not to restore function but to relieve Not to restore function but to relieve tension on the wound, obliterate dead tension on the wound, obliterate dead space and place healthy tissue in areas space and place healthy tissue in areas that will promote rapid healing and that will promote rapid healing and reduce infection riskreduce infection risk

Muscle or Myocutaneous Local, Free and Muscle or Myocutaneous Local, Free and Vascularized FlapVascularized Flap


DEFECT RECONSTRUCTIONDEFECT RECONSTRUCTION– Skin Barrier ReconstructionSkin Barrier Reconstruction

Precise wound closure is criticalPrecise wound closure is critical

Wound breakdown can result in deep infection & Wound breakdown can result in deep infection & subsequent limb loss, might prevent or delay the use of subsequent limb loss, might prevent or delay the use of chemotherapy or local radiation therapychemotherapy or local radiation therapy

Skin and subcutaneous fascial layer should be closed in Skin and subcutaneous fascial layer should be closed in layers, with precise matching of the anatomic layerslayers, with precise matching of the anatomic layers

Non braided suture & short tail to minimize foreign Non braided suture & short tail to minimize foreign bodybody

Small skin staplers with Antibiotic Ointment appliedSmall skin staplers with Antibiotic Ointment applied

Early & often changing of dressingEarly & often changing of dressing

If skin closure with minimal tension cannot be achieved If skin closure with minimal tension cannot be achieved – SSG of flapSSG of flap


DEFECT RECONSTRUCTIONDEFECT RECONSTRUCTION– Surgical drainsSurgical drains

Eliminate dead space-preventing accumulating of Eliminate dead space-preventing accumulating of fluid pocketsfluid pockets

Divert drainage from the skin incisionDivert drainage from the skin incision

Prevent formation and spread of haematoma Prevent formation and spread of haematoma along fascial planes-might carry tumor cellsalong fascial planes-might carry tumor cells

– Drain tracksDrain tracksShould not traversed uninvolved anatomic areasShould not traversed uninvolved anatomic areas

Should exit the skin in line with the incisionShould exit the skin in line with the incision

– Critical drains can be sutured to skin but Critical drains can be sutured to skin but keep the suture sites close by for easy keep the suture sites close by for easy excision with drain track if neededexcision with drain track if needed


INTRAOPERATIVE ADJUVANT THERAPIESINTRAOPERATIVE ADJUVANT THERAPIES– Tumor killing potentialTumor killing potential

H2O2 - tumoricidal abilityH2O2 - tumoricidal ability

Thermal killThermal kill– Tissue Heating with diathermy, heat gun, Tissue Heating with diathermy, heat gun,

cryosurgery using liquid nitrogencryosurgery using liquid nitrogenProduce necrosis of 1 to 10mmProduce necrosis of 1 to 10mm

– LasersLasers

Intraoperative RadiationIntraoperative Radiation– Advantage; Precise & Direct to tumor bed, & not Advantage; Precise & Direct to tumor bed, & not

associated with wound healing difficulty seen with associated with wound healing difficulty seen with preop, high dose radiation therapypreop, high dose radiation therapy

BrachytherapyBrachytherapy– Deliver local radiation through catheters implanted Deliver local radiation through catheters implanted

surgically after tumor excisionsurgically after tumor excision


THE YOUNG CHILDTHE YOUNG CHILD– Remarkable adaptive capabilities to Remarkable adaptive capabilities to

anatomic loss either by tumor itself or anatomic loss either by tumor itself or amputationamputation

– Preserving epiphyses through very closed Preserving epiphyses through very closed resection margins a few mm away from resection margins a few mm away from metaphyseal tumormetaphyseal tumor

– If the child survive from the sarcoma, the If the child survive from the sarcoma, the extremely long life span ahead raises the extremely long life span ahead raises the lifetime risk of developinglifetime risk of developing

Secondary radiation induced sarcomas and other Secondary radiation induced sarcomas and other radiation associated morbiditiesradiation associated morbiditiesSecondary chemotherapy induced cancersSecondary chemotherapy induced cancers


THE VERY OLDTHE VERY OLD– Most common; malignant fibrous Most common; malignant fibrous

histiocytomahistiocytoma– Problem; less cardiac reserve and Problem; less cardiac reserve and

overall poorer physiologic functionoverall poorer physiologic functionAdjuvant chemotherapy and high dose Adjuvant chemotherapy and high dose radiation therapy will cause more risk than radiation therapy will cause more risk than benefitbenefitMore radical resection with much wider More radical resection with much wider marginmargin

– Senile and Demented Patients, Medical Senile and Demented Patients, Medical disease which complicate wound healingdisease which complicate wound healing

Aim for NO TREATMENT or PALLIATIVEAim for NO TREATMENT or PALLIATIVE


METASTATIC SARCOMA AT METASTATIC SARCOMA AT PRESENTATIONPRESENTATION– Poor prognosisPoor prognosis– Survival depends on removal of primary Survival depends on removal of primary

tumor & eradication of metastatic disease tumor & eradication of metastatic disease through either surgical or chemotherapeutic through either surgical or chemotherapeutic modalities or combination of bothmodalities or combination of both

– CHEMOTHERAPY IS ESSENTIAL to eliminate CHEMOTHERAPY IS ESSENTIAL to eliminate the micrometastasesthe micrometastases

– Any surgical complication that can delay or Any surgical complication that can delay or prevent perioperative chemotherapy must be prevent perioperative chemotherapy must be avoidedavoided

– Limb salvage has NO ROLELimb salvage has NO ROLE


‘‘UNRESECTABLE’ TUMORUNRESECTABLE’ TUMOR– Tumor involves major vessels, nerves or other Tumor involves major vessels, nerves or other

critical structurescritical structures– Means unacceptable morbidityMeans unacceptable morbidity– Truly unresectable situation is death as result.Truly unresectable situation is death as result.– In cases with unacceptable morbidity like In cases with unacceptable morbidity like

resection of tumor including the sciatic nerve, resection of tumor including the sciatic nerve, producing insensate lower limbproducing insensate lower limb

Amputation is better choice OR Amputation is better choice OR Limb Salvage surgery with the idea of tiring and skill Limb Salvage surgery with the idea of tiring and skill demanding nerve grafting or reconstruction, in mind demanding nerve grafting or reconstruction, in mind OR if complication arises, amputation at a very high OR if complication arises, amputation at a very high level is unavoidablelevel is unavoidable

– Excellent candidate for preoperative Excellent candidate for preoperative chemotherapy, radiation therapy or more chemotherapy, radiation therapy or more aggressive techniques like isolated limb aggressive techniques like isolated limb perfusion to gain tumor size regression.perfusion to gain tumor size regression.

PRINCIPLES OF CHEMOTHERAPYPRINCIPLES OF CHEMOTHERAPY

Action of Chemo AgentsAction of Chemo Agents– Damage DNADamage DNA

Alkylating agents, platinum compounds, Alkylating agents, platinum compounds, anthracyclines, epipodophyllotoxinsanthracyclines, epipodophyllotoxins

– Deplete the cellular building blocks Deplete the cellular building blocks required for replicationrequired for replication

Antifolates, 5-fluoropyrimidines, cytidine Antifolates, 5-fluoropyrimidines, cytidine analogsanalogs

– Interfere with microtubule function Interfere with microtubule function required for mitosisrequired for mitosis

Vinca alkaloids, taxanesVinca alkaloids, taxanes


Chemo Agents are cytotoxic to tumor Chemo Agents are cytotoxic to tumor cells through induction of apoptosiscells through induction of apoptosis

Selectivity is due to the fact that rapidly Selectivity is due to the fact that rapidly dividing tumor did not have sufficient dividing tumor did not have sufficient time to repair the chemotherapy time to repair the chemotherapy induced damageinduced damage

Combination chemotherapyCombination chemotherapy– Antitumor effect will be cumulative whereas Antitumor effect will be cumulative whereas

the toxicity will notthe toxicity will not– Avoid resistantAvoid resistant– Effective for different phases of cell cycleEffective for different phases of cell cycle


IndicationIndication– Induction chemotherapy (Neoadjuvant or Preoperative)Induction chemotherapy (Neoadjuvant or Preoperative)

Decrease tumor sizeDecrease tumor sizeIn tumor that are capable of metastasesIn tumor that are capable of metastases

(Standard of care for osteosarcoma and Ewing’s (Standard of care for osteosarcoma and Ewing’s sarcoma)sarcoma)

– Malignant musculoskeletal tumor with systemic Malignant musculoskeletal tumor with systemic metastasesmetastases

– Chemosensitive neoplasmChemosensitive neoplasmOsteosarcomaOsteosarcomaEwing’s sarcomaEwing’s sarcomaRhabdomyosarcomaRhabdomyosarcomaSynovial sarcomaSynovial sarcomaMalignant Fibrous HistiocytomaMalignant Fibrous Histiocytoma

– For chemosensitive benign tumors which is multiply For chemosensitive benign tumors which is multiply recurrent or impinge on vital structuresrecurrent or impinge on vital structures


Chemoresistant tumorsChemoresistant tumors– Gastrointestinal stromal tumorsGastrointestinal stromal tumors– ChondrosarcomaChondrosarcoma– Alveolar soft part sarcomaAlveolar soft part sarcoma


Side EffectsSide Effects– Stunting of growthStunting of growth– Osteoporosis Osteoporosis – AVN AVN – Cisplatinum Cisplatinum -> Nephrotoxicity and -> Nephrotoxicity and

hearing loss hearing loss – Adriamycin Adriamycin -> Cardiotoxicity -> Cardiotoxicity – Vincristine Vincristine -> Neurotoxicity -> Neurotoxicity – Chemotherapeutic induced malignancy Chemotherapeutic induced malignancy

usually blood forming eg leukaemias but usually blood forming eg leukaemias but also may -> Ca bladder or skin (particularly also may -> Ca bladder or skin (particularly associated with cyclophosphamide)associated with cyclophosphamide)

PRINCIPLES OF PRINCIPLES OF RADIOTHERAPYRADIOTHERAPY

Radioactive focused local treatmentRadioactive focused local treatment

Using high energy photon beam Using high energy photon beam produced by a linear acceleratorproduced by a linear accelerator

Others; gamma rays, electron beams, Others; gamma rays, electron beams, radiation from brachytherapy, beams radiation from brachytherapy, beams from heavy particle like protons and from heavy particle like protons and neutronsneutrons

Mechanism of actionMechanism of action– Oxygenation of water molecules within the Oxygenation of water molecules within the

cells, producing free radicals that interact cells, producing free radicals that interact with DNA to cause strand break, eventually with DNA to cause strand break, eventually results in cell death results in cell death


Radiation unitsRadiation units– Grays (Gy), a unit of absorbed dose Grays (Gy), a unit of absorbed dose

(1Gy=1J/kg)(1Gy=1J/kg)– In the past;In the past; radsrads

1 rad = 1 centigray (cGy) or 1/100 of a Gy1 rad = 1 centigray (cGy) or 1/100 of a Gy

Radiotherapy is delivered in small doses Radiotherapy is delivered in small doses or fractions over many daysor fractions over many days– Allows a large total dose of radiation to be Allows a large total dose of radiation to be

delivered without exceeding the tolerance delivered without exceeding the tolerance levels of surrounding normal tissuelevels of surrounding normal tissue

– Standard fractionation; 180 to 200 cGy daily, Standard fractionation; 180 to 200 cGy daily, 5 days per week5 days per week


Indication in soft tissue sarcomaIndication in soft tissue sarcoma– Radiosensitive sarcoma, theoretically, well Radiosensitive sarcoma, theoretically, well

oxygenated tumorsoxygenated tumorsRhabdomyosarcoma Rhabdomyosarcoma PNET (extraosseous Ewing’s sarcoma)PNET (extraosseous Ewing’s sarcoma)

(definitive primary radiotherapy local control rate for (definitive primary radiotherapy local control rate for the above type of tumor is 80%)the above type of tumor is 80%)

(mandatory treatment for all patient)(mandatory treatment for all patient)

Other unresectable soft tissue tumor local control Other unresectable soft tissue tumor local control rate is in the range of 25 to 30%rate is in the range of 25 to 30%

Current standard for high grade resectable Current standard for high grade resectable extremity soft tissue sarcomas is limb salvage extremity soft tissue sarcomas is limb salvage surgery, when possible, in conjunction with surgery, when possible, in conjunction with radiotherapyradiotherapy


Indication in soft tissue sarcomaIndication in soft tissue sarcoma– Postoperative radiotherapyPostoperative radiotherapy

Decrease risk of local relapse for both high grade and Decrease risk of local relapse for both high grade and certain high risk low grade sarcoma.certain high risk low grade sarcoma.For low grade sarcoma, consider if having microscopic For low grade sarcoma, consider if having microscopic positive margin or of large tumor size (>5cm) positive margin or of large tumor size (>5cm)

– Preoperative radiotherapyPreoperative radiotherapyShrink tumor sizeShrink tumor sizeDecrease risk of tumor contaminationDecrease risk of tumor contaminationIntact well oxygenated tumor respond better than Intact well oxygenated tumor respond better than hypoxic tumor bedhypoxic tumor bedDisadvantagesDisadvantages

– Delay in surgeryDelay in surgery– Possible wound healing complicationPossible wound healing complication– Less information on tumor extent and pathologyLess information on tumor extent and pathology


Indication in soft tissue sarcomaIndication in soft tissue sarcoma– BrachytherapyBrachytherapy

Insertion of radiation sources into tissues after Insertion of radiation sources into tissues after tumor resection before closuretumor resection before closureRadioactive source (usually cesium or iodine) Radioactive source (usually cesium or iodine) placed inside the catheters on post op D5placed inside the catheters on post op D5Excellent postoperative treatment for extremity Excellent postoperative treatment for extremity soft tissue sarcoma (adjuvant brachytherapy local soft tissue sarcoma (adjuvant brachytherapy local control rate for high grade lesion is 89%)control rate for high grade lesion is 89%)

– Intraoperative RadiotherapyIntraoperative RadiotherapyElectron or orthovoltage beamElectron or orthovoltage beam74% local control rate for primary retroperitoneal 74% local control rate for primary retroperitoneal sarcomas when combined with external beam sarcomas when combined with external beam radiotherapyradiotherapy


Timing of radiotherapyTiming of radiotherapy

– Definitive radiotherapy for Definitive radiotherapy for rhabdomyosarcoma and Ewing’s rhabdomyosarcoma and Ewing’s sarcoma is generally integrated into sarcoma is generally integrated into the middle of a chemotherapy the middle of a chemotherapy programprogram

– Postoperatively, as soon as adequate Postoperatively, as soon as adequate wound healing is achieved, usually 3 wound healing is achieved, usually 3 to 6 weeksto 6 weeks


Side EffectsSide Effects– Acute side effects depend on the total Acute side effects depend on the total

dose of radiation and overall dose of radiation and overall treatment timetreatment time

– Late side effects depend on fraction Late side effects depend on fraction sizesize


Acute Side EffectsAcute Side Effects– begins after first 2 weeks and increase as begins after first 2 weeks and increase as

the treatment continuesthe treatment continues– Most common is fatigueMost common is fatigue– Majority develop erythema, and temporary Majority develop erythema, and temporary

desquamationdesquamation– Uncommon; wound dehiscence, suppresses Uncommon; wound dehiscence, suppresses

blood counts and other complications blood counts and other complications pertaining to anatomic site involvedpertaining to anatomic site involved

– At least one clinic visit per week to see At least one clinic visit per week to see radiation oncologist to assess and manage radiation oncologist to assess and manage potential side effectspotential side effects


Late Side EffectsLate Side Effects– Months to years after receiving radiotherapyMonths to years after receiving radiotherapy

Muscle fibrosis; stretching exercise Muscle fibrosis; stretching exercise Weaken Bones and cause Joint DysfunctionWeaken Bones and cause Joint DysfunctionEdema distal to irradiated site; full circumference Edema distal to irradiated site; full circumference of limb should never be treated to a high doseof limb should never be treated to a high dose

– More then 20 years after radiationMore then 20 years after radiationSecondary malignancy esp. in childrenSecondary malignancy esp. in childrenRate of secondary bone cancer after radiation Rate of secondary bone cancer after radiation treatment for all childhood cancers was less than treatment for all childhood cancers was less than 1%1%For those treated for Ewing’s sarcoma, the risk For those treated for Ewing’s sarcoma, the risk was 5.4%was 5.4%Others; bone growth arrest and iatrogenic scoliosis Others; bone growth arrest and iatrogenic scoliosis in childrenin children

THANK YOUTHANK YOU

Documents

TUMOR Dr Mohammad.ali Department of Orthopedics. PATHOLOGY of NEOPLASM Abnormal mass of tissue Growth is autonomous, exceeds normal, & persist after cessation