Dr sumer yadavDr sumer yadav

RETROPERITONEUM SPACERETROPERITONEUM SPACE LARGE POTENTIAL SPACE BOUNDED LARGE POTENTIAL SPACE BOUNDED

ANTERIORLY BY THE POSTERIOR ANTERIORLY BY THE POSTERIOR PERITONEUM.PERITONEUM.

POSTERIORLY BY THE SPINE AND POSTERIORLY BY THE SPINE AND BACK MUSCLESBACK MUSCLES

SUPERIORLY BY THE DIAPHRAGMSUPERIORLY BY THE DIAPHRAGM INFERIORLY BY THE LEVATORSINFERIORLY BY THE LEVATORS LATERALLY BY THE FLANK MUSCLES LATERALLY BY THE FLANK MUSCLES

AT THE LEVEL OF THE ANTERIOR AT THE LEVEL OF THE ANTERIOR SUPERIOR SPINE OF THE ILIAC SUPERIOR SPINE OF THE ILIAC CREST TO THE TIP OF THE 12CREST TO THE TIP OF THE 12THTH RIB. RIB.

INTRODUCTIONINTRODUCTION 15% OF ALL SOFT TISSUE 15% OF ALL SOFT TISSUE

SARCOMAS AND ONE THIRD OF SARCOMAS AND ONE THIRD OF MALIGNANT RETROPERITONEAL MALIGNANT RETROPERITONEAL TUMORS.TUMORS.

MANAGEMENT CHALLENGE MANAGEMENT CHALLENGE BECAUSE OF THEIR FREQUENT BECAUSE OF THEIR FREQUENT

LATE PRESENTATIONLATE PRESENTATION LACK OF SPECIFIC SIGN & LACK OF SPECIFIC SIGN &

SYMPTOMSSYMPTOMS PROXIMITY TO VITAL STRUCTUREPROXIMITY TO VITAL STRUCTURE LARGE SIZELARGE SIZE

INTRODUCTION (Cont.)INTRODUCTION (Cont.)SYNDROME ASSOCIATED WITH R.P. SYNDROME ASSOCIATED WITH R.P. SARCOMA :SARCOMA :

GARDNER SYN.GARDNER SYN. FAMILIAL RETINOBLASTOMAFAMILIAL RETINOBLASTOMA LI- FRAUMANI SYNLI- FRAUMANI SYN NEUROFIBROMATOSISNEUROFIBROMATOSIS

CARCINOGENS ASSOCIATED :CARCINOGENS ASSOCIATED : VINYL CHLORIDEVINYL CHLORIDE THORIUM DIOXIDETHORIUM DIOXIDE RADIATIONRADIATION

PRESENTATIONPRESENTATIONPATIENTS COMPLAIN WITH PATIENTS COMPLAIN WITH ABDOMINAL MASSABDOMINAL MASSBACK PAINBACK PAINWEIGHT LOSSWEIGHT LOSSLOWER EXTREMITY SENSORY LOWER EXTREMITY SENSORY

CHANGESCHANGESURINARY FREQUENCYURINARY FREQUENCYINTESTINAL OBS. INTESTINAL OBS.

PRESENTATIONPRESENTATIONTHE SIZE R.S. IS MORE THAN THE SIZE R.S. IS MORE THAN

10 cm. IN MOST REPORTED 10 cm. IN MOST REPORTED CASESCASES

EQUAL GENDER EQUAL GENDER DISTRIBUTION.DISTRIBUTION.

THE MEAN AGE AT PRIMARY THE MEAN AGE AT PRIMARY PRESENTATION IS BETWEEN PRESENTATION IS BETWEEN 49 AND 55 YEARS 49 AND 55 YEARS

DISTRIBUTION OF HISTOLOGIC SUBTYPES DISTRIBUTION OF HISTOLOGIC SUBTYPES OF RETROPERITONEAL SARCOMAOF RETROPERITONEAL SARCOMA

Liposarcoma58%

Hemangiopericytoma3%

Others12%

MFH4%

MPNST3%

Leiomyosarcoma20%

Liposarcoma Hemangiopericytoma Others MFH MPNST Leiomyosarcoma

HISTOLOGIC GRADEHISTOLOGIC GRADE IS OF MORE PROGNOSTIC IS OF MORE PROGNOSTIC

SIGNIFICANCE THAN THE CELL SIGNIFICANCE THAN THE CELL OF ORIGIN.OF ORIGIN.

GRADING USES A COMPOSITE GRADING USES A COMPOSITE OF HISTOPATHOLOGIC OF HISTOPATHOLOGIC FEATURES THAT INCLUDES :FEATURES THAT INCLUDES : NEROSISNEROSIS CELLULARITYCELLULARITY PLEMORPHISMPLEMORPHISM MITOSISMITOSIS

DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS RETROPERITONEAL SARCOMARETROPERITONEAL SARCOMA LYMPHOMALYMPHOMA METASTATIC TESTICULAR CANCERMETASTATIC TESTICULAR CANCER ADRENAL TUMORSADRENAL TUMORS PANCREATIC TUMORSPANCREATIC TUMORS GASTROINTESTINAL STROMAL GASTROINTESTINAL STROMAL

TUMORSTUMORS RENAL CELL CARCINOMARENAL CELL CARCINOMA RETROPERITONEAL FIBROSISRETROPERITONEAL FIBROSIS

DIAGNOSTIC EVALUATIONDIAGNOSTIC EVALUATIONCT SCANNINGCT SCANNING SIZE OF THE TUMORSIZE OF THE TUMOR ANY ANATOMIC CHANGES SECONDARY ANY ANATOMIC CHANGES SECONDARY

TO ITS GROWTH ARE EASILY TO ITS GROWTH ARE EASILY VISUALIZEDVISUALIZED

TUMOR INVASION OF ADJACENT TUMOR INVASION OF ADJACENT ORGAN CAN ABE DEMONSTRATED OR ORGAN CAN ABE DEMONSTRATED OR SUGGESTEDSUGGESTED

LYMPHOMA – HOMGENEOUS AND LYMPHOMA – HOMGENEOUS AND ENVELOPS THE IVC & AORTAENVELOPS THE IVC & AORTA

SARCOMA – USUALLY HETROGENEOUS SARCOMA – USUALLY HETROGENEOUS

DIAGNOSTIC EVALUATION (Cont.)DIAGNOSTIC EVALUATION (Cont.)THE FUNCTIONAL STATE OF AT THE FUNCTIONAL STATE OF AT

LEAST ONE KIDNEY MUST BE LEAST ONE KIDNEY MUST BE DEMONSTRATED BY EITHER THE DEMONSTRATED BY EITHER THE CONTRAST C.T. SCAN OR AN CONTRAST C.T. SCAN OR AN EXCRETORY UROGRAM BECAUSE EXCRETORY UROGRAM BECAUSE THE EN BLOC RESECTION OF ONE THE EN BLOC RESECTION OF ONE KIDNEY IS OFTEN REQUIRED.KIDNEY IS OFTEN REQUIRED.

NEITHER C.T. SCAN NOR M.R.I. IS NEITHER C.T. SCAN NOR M.R.I. IS SUPERIOR IN ASSESSMENT OF R.P. SUPERIOR IN ASSESSMENT OF R.P. SARCOMA. SARCOMA.

DIAGNOSTIC EVALUATION (Cont.)DIAGNOSTIC EVALUATION (Cont.)

ARTERIOGRAPHYARTERIOGRAPHY FINDING SUGGESTIVE OF FINDING SUGGESTIVE OF

NEOPLASIA INCLUDES :NEOPLASIA INCLUDES :

1.1. NEOVASCULARITYNEOVASCULARITY

2.2. TUMOR BLUSHTUMOR BLUSH

3.3. VESSEL ENCASEMENTVESSEL ENCASEMENT

DIAGNOSTIC EVALUATION (Cont.)DIAGNOSTIC EVALUATION (Cont.) BECAUSE MALIGNANT FIBROUS BECAUSE MALIGNANT FIBROUS

HISTIOCYTOMA TENDS TO OCCUR IN HISTIOCYTOMA TENDS TO OCCUR IN THE RENAL AREA THE THE RENAL AREA THE DEMONSTRATION OF AN EXTRA DEMONSTRATION OF AN EXTRA RENAL ARTERIAL SUPPLY IS RENAL ARTERIAL SUPPLY IS HELPFUL IN DECIDING TO SAVE THE HELPFUL IN DECIDING TO SAVE THE KIDNEY.KIDNEY.

A DOMINANT LUMBER OR A DOMINANT LUMBER OR INTERCOSTAL ARTERIAL SUPPLY INTERCOSTAL ARTERIAL SUPPLY ADDS TO THE LIKELIHOOD THAT THE ADDS TO THE LIKELIHOOD THAT THE TUMOR HAS A RETROPERITONEAL TUMOR HAS A RETROPERITONEAL ORIGIN.ORIGIN.

INFERIOR INFERIOR VENA VENA

CAVAGRAMCAVAGRAM

DIAGNOSTIC EVALUATION (Cont.)DIAGNOSTIC EVALUATION (Cont.)PET SCANNINGPET SCANNING CURRENTLY NO DEFINED ROLE FOR CURRENTLY NO DEFINED ROLE FOR

POSITRON EMISSION TOMOGRAPHY POSITRON EMISSION TOMOGRAPHY SCANNING IN PRIMARY LEVEL SCANNING IN PRIMARY LEVEL RETROPERITONEAL SARCOMA.RETROPERITONEAL SARCOMA.

FLUORODEOXYGLUCOSE UPTAKE FLUORODEOXYGLUCOSE UPTAKE DOES CORRELATE WITH TUMOR DOES CORRELATE WITH TUMOR GRADE IN SOFT TISSUE SARCOMAGRADE IN SOFT TISSUE SARCOMA

NO DISCRIMINATING LOW-GRADE NO DISCRIMINATING LOW-GRADE TUMORS FROM BEING TUMORS TUMORS FROM BEING TUMORS

DIAGNOSTIC EVALUATION (Cont.)DIAGNOSTIC EVALUATION (Cont.)

FUTURE POTENTIAL USE :FUTURE POTENTIAL USE :DETECTION METASTATIC DETECTION METASTATIC

DISEASE DISEASE DETECTION LOCAL DETECTION LOCAL

RECURRENCE RECURRENCE DETECTION OF RESPONSE DETECTION OF RESPONSE

TO NEOADJUVANT THERAPYTO NEOADJUVANT THERAPY

BIOPSYBIOPSY HISTOLOGICAL DIAGNOSIS HISTOLOGICAL DIAGNOSIS

SHOULD BE SECURED BY :SHOULD BE SECURED BY :1.1. F.N.A.C.F.N.A.C.2.2. TRU-CUT BIOPSYTRU-CUT BIOPSY3.3. CT GUIDED CORE BIOPSYCT GUIDED CORE BIOPSY FOR SMALL MASSES THAT FOR SMALL MASSES THAT

CAN BE RESECTED EN BLOC CAN BE RESECTED EN BLOC PREOPERATIVE DIAGNOSIS PREOPERATIVE DIAGNOSIS LESS IMPORTANT LESS IMPORTANT

BIOPSY (Cont.)BIOPSY (Cont.) PRE-OPERATIVE BIOPSY IS FOR PRE-OPERATIVE BIOPSY IS FOR

THOSE PATIENTS WHO ARE INVOLVED THOSE PATIENTS WHO ARE INVOLVED IN NEOADJUVANT TREATMENT IN NEOADJUVANT TREATMENT PROTOCOLS OR THOSE PATIENTS IN PROTOCOLS OR THOSE PATIENTS IN WHOM SYSTEMIC THERAPY WILL BE WHOM SYSTEMIC THERAPY WILL BE PRIMARY TREATMENT MODALITY PRIMARY TREATMENT MODALITY BECAUSE OF :BECAUSE OF : THE PRESENCES OF METASTIC THE PRESENCES OF METASTIC

DISEASEDISEASE LOCALLY ADVANCED DISEASE LOCALLY ADVANCED DISEASE DIAGNOSIS OF LYMPHOMA DIAGNOSIS OF LYMPHOMA

STAGING SOFT TISSUE SARCOMASTAGING SOFT TISSUE SARCOMA

T1T1 TUMOR < 5 cmTUMOR < 5 cmT1aT1aSUPERFICIAL TUMORSUPERFICIAL TUMORT1bT1bDEEP TUMORDEEP TUMORT2T2 TUMOR > 5 cm IN TUMOR > 5 cm IN

GREATEST DIMENSIONGREATEST DIMENSIONT2aT2aSUPERFICIAL TUMORSUPERFICIAL TUMORT2bT2bDEEP TUMORDEEP TUMOR

STAGING SOFT TISSUE SARCOMA (Cont.)STAGING SOFT TISSUE SARCOMA (Cont.)REGIONAL NODES (N)REGIONAL NODES (N) NXNX REGIONAL LYMPH NODES REGIONAL LYMPH NODES

CANNOT BE ASSESSEDCANNOT BE ASSESSED N0N0 NO REGIONAL LYMPH NODE NO REGIONAL LYMPH NODE

METASTASISMETASTASIS N1N1 REGIONAL LYMPH NODE REGIONAL LYMPH NODE

METASTASISMETASTASISDISTANT METASTASES (M)DISTANT METASTASES (M) MXMX DISTANT METASTASIS DISTANT METASTASIS

CANNOTCANNOT BE ASSESSEDBE ASSESSED M0M0 NO DISTANT METASTASIS NO DISTANT METASTASIS M1M1 DISTANT METASTASISDISTANT METASTASIS

STAGING SOFT TISSUE SARCOMA STAGING SOFT TISSUE SARCOMA (Cont.)(Cont.)

Stage Grouping

Stage I

A (LOW GRADE, SMALL, SUPERFICIAL, DEEP) G1-2 T1a-b N0 M0

B (LOW GRADE, LARGE, SUPERFICIAL) G1-2 T2a N0 M0

STAGE II

A (LOW GRADE LARGE, DEEP) G1-2 T2b N0 M0

B (HIGH GRADE, SMALL, SUPERFICIAL, DEEP) G3-4 T1a-b N0 M0

C (HIGH GRADE, LARGE SUPERFICIAL) G3-4 T2a N0 M0

STAGE III

HIGH GRADE, LARGE, DEEP G3-4 T2b N0 M0

STAGE IV

ANY MATASTASIS ANY G ANY T N1 M0

ANY G ANY T N0 M1

ALGORITHM FOR MANAGEMENT OF ALGORITHM FOR MANAGEMENT OF RETROPERITONEAL SARCOMAS RETROPERITONEAL SARCOMAS

PRIMARY RESECTABLE RETROPERITONEAL SARCOMA

BIOPSYNEOADJUVANT TRIAL

RESECTION

FOLLOW CLINICALLY

RESECTION

FOLLOW CLINICALLY

MANAGEMENT OF LOCALLY ADVANCED & MANAGEMENT OF LOCALLY ADVANCED & METASTATIC DISEASEDISEASE

LOCALLY ADVANCED & METASTATIC RETROPERITONEAL SARCOMA

ASYMPTOMATIC

CLINICALOBSERVATION

± CHEMOTHERAPY± INVESTIGATION AGENTS

± RADIATION THERAPY

MECHANICAL SYMPTOMS

PALLIATIVE RESECTION

± CHEMOTHERAPY± INVESTIGATION AGENTS

± RADIATION THERAPY

CLINICALOBSERVATION

SURGICAL RESECTIONSURGICAL RESECTION REMAINS THE ONLY POTENTIALLY REMAINS THE ONLY POTENTIALLY

CURATIVE MODALITY IN PATIENTS CURATIVE MODALITY IN PATIENTS WIT RETROPERITONEAL SARCOMAWIT RETROPERITONEAL SARCOMA

PRIMARY NONMETASTATIC PRIMARY NONMETASTATIC RETROPERITONEAL SARCOMA RETROPERITONEAL SARCOMA RESECTABILITY RATES HAVE RESECTABILITY RATES HAVE RANGED FROM 59% TO 95%.RANGED FROM 59% TO 95%.

RESECTABILITY RATES NOT RESECTABILITY RATES NOT SIGNIFICANTLY ASSOCIATED WITH SIGNIFICANTLY ASSOCIATED WITH TUMOR SIZE , GRADE OR TUMOR SIZE , GRADE OR HISTOLOGIC TYPE. HISTOLOGIC TYPE.

SURGICAL RESECTION SURGICAL RESECTION (Cont.)(Cont.) THE MOST COMMON ORGAN THE MOST COMMON ORGAN

REQUIRING SIMULTANEOUS EN BLOC REQUIRING SIMULTANEOUS EN BLOC RESECTION ARE KIDNEY. ADRENAL, RESECTION ARE KIDNEY. ADRENAL, COLON, PANCREAS AND SPLEENCOLON, PANCREAS AND SPLEEN

REASONS FOR UNRESECTABILITY OR REASONS FOR UNRESECTABILITY OR INCOMPLETE RESECTION AT THE TIME INCOMPLETE RESECTION AT THE TIME OF EXPORATION INCLUDE VASCULAR OF EXPORATION INCLUDE VASCULAR INVOLVEMENT PERITONEAL, INVOLVEMENT PERITONEAL, METASTASIS AND MULTIFOCALITY METASTASIS AND MULTIFOCALITY

OPERATIVE CONSIDERATIONSOPERATIVE CONSIDERATIONS

ALL PATIENTS SHOULD ALL PATIENTS SHOULD UNDERGO A FULL BOWEL UNDERGO A FULL BOWEL PREPARATION BECAUSE PREPARATION BECAUSE A LIMITED RESECTION OF A LIMITED RESECTION OF THE COLON OR RECTUM THE COLON OR RECTUM IS COMMONLY REQUIREDIS COMMONLY REQUIRED

OPERATIVE CONSIDERATIONSOPERATIVE CONSIDERATIONSMIDLINE INCISION IS USUALLY MIDLINE INCISION IS USUALLY

BEST FOR THE INITIAL BEST FOR THE INITIAL EXPLORATIONEXPLORATION

IF THE TUMOR IS IN THE IF THE TUMOR IS IN THE UPPER RETROPERITONEUM UPPER RETROPERITONEUM TOWARDS OR INVADING THE TOWARDS OR INVADING THE DIAPHRAGM, A DIAPHRAGM, A THORACOABDOMINAL THORACOABDOMINAL APPROACH MAY BE APPROACH MAY BE INDICATEDINDICATED

OPERATIVE CONSIDERATIONS (Conts.)OPERATIVE CONSIDERATIONS (Conts.)

THE ABDOMINAL PORTION THE ABDOMINAL PORTION OF THE INCISIONS IS OF THE INCISIONS IS OPENED FIRST FOR THE OPENED FIRST FOR THE EXPLORATION TO EXPLORATION TO DETERMINE DETERMINE RESECTABILITY AND A RESECTABILITY AND A CAREFUL SEARCH IS MADE CAREFUL SEARCH IS MADE FOR HEPATIC OR FOR HEPATIC OR PERITONEAL MATASTASES.PERITONEAL MATASTASES.

OPERATIVE CONSIDERATIONS (Conts.)OPERATIVE CONSIDERATIONS (Conts.)

THE FLANK APPROACH IS THE FLANK APPROACH IS LESS SATISFACTORY THAN LESS SATISFACTORY THAN AN ABDOMINAL INCISION IN AN ABDOMINAL INCISION IN ALLOWING THE SURGEON ALLOWING THE SURGEON TO PERFORM AN EN BLOC TO PERFORM AN EN BLOC RESECTION OF INVOLVED RESECTION OF INVOLVED ORGANS OR TO CONTROL ORGANS OR TO CONTROL THE MAJOR ARTERIES AND THE MAJOR ARTERIES AND VEINS SUPPLYING THE VEINS SUPPLYING THE TUMORTUMOR

OPERATIVE CONSIDERATIONS (Cont.)OPERATIVE CONSIDERATIONS (Cont.) INCISIONAL WEDGE BIOPSIES INCISIONAL WEDGE BIOPSIES

SHOULD BE OBTAINED ONLY FROM SHOULD BE OBTAINED ONLY FROM PATIENTS WHO HAVE OBVIOUSLY PATIENTS WHO HAVE OBVIOUSLY INOPERABLE DISEASE OR WHERE INOPERABLE DISEASE OR WHERE LYMPHOMA IS SUSPECTEDLYMPHOMA IS SUSPECTED

GREAT CARE MUST BE TAKEN TO GREAT CARE MUST BE TAKEN TO ISOLATE THE AREA OF BIOPSY AND ISOLATE THE AREA OF BIOPSY AND TO OBTAIN ABSOLUTE HEMOSTASISTO OBTAIN ABSOLUTE HEMOSTASIS

OPERATIVE CONSIDERATIONS (Cont.)OPERATIVE CONSIDERATIONS (Cont.)

LOCALIZED TUMOR :LOCALIZED TUMOR : THIS SHOULD BE THIS SHOULD BE

REMOVED EN BLOC WHICH REMOVED EN BLOC WHICH MAY INCLUDE AN EN BLOC MAY INCLUDE AN EN BLOC RESECTION OF INVOLVED RESECTION OF INVOLVED SURROUNDING ORGAN.SURROUNDING ORGAN.

THERE SHOULD BE 1 TO 2 THERE SHOULD BE 1 TO 2 cm OF NORMAL MARGIN.cm OF NORMAL MARGIN.

OPERATIVE CONSIDERATIONS (Cont.)OPERATIVE CONSIDERATIONS (Cont.)

TUMOR SHOULD BE TUMOR SHOULD BE REMOVED ALONG WITH REMOVED ALONG WITH THIS PSEUDOCAPSULETHIS PSEUDOCAPSULE

FIXATION IS NOT A SIGN OF FIXATION IS NOT A SIGN OF UNRESECTABILITY UNLESS UNRESECTABILITY UNLESS THERE IS EXTENSIVE THERE IS EXTENSIVE INVOLVEMENT OF INVOLVEMENT OF IRREPLACEABLE OR IRREPLACEABLE OR UNREMOVABLE STRUCTURESUNREMOVABLE STRUCTURES

A A SEGMENT SEGMENT OF SMALL OF SMALL

BOWEL BOWEL AND AND

COLON COLON ADHERENT ADHERENT

TO THE TO THE LEFT LEFT

RETROPERRETROPER- ITONEAL - ITONEAL SARCOMASARCOMA

MANAGEMENT OF THE KIDNEYMANAGEMENT OF THE KIDNEY NEPHRECTOMY IS FREQUENTLY NEPHRECTOMY IS FREQUENTLY

PERFORMED AT THE TIME OF PERFORMED AT THE TIME OF RESECTION OF LARGE RESECTION OF LARGE RETROPERITONEAL SARCOMAS. RETROPERITONEAL SARCOMAS.

DIRECT RENAL, RENAL CAPSULE OR DIRECT RENAL, RENAL CAPSULE OR RENAL VASCULAR INVASION BY RENAL VASCULAR INVASION BY TUMOR OCCURS IN LESS THAN 30%TUMOR OCCURS IN LESS THAN 30%

MORE COMMONLY IN 70% OF CASES, MORE COMMONLY IN 70% OF CASES, THE TUMOR WILL ENCASE OR BE THE TUMOR WILL ENCASE OR BE ADHERENT TO THE KIDNEY WITHOUT ADHERENT TO THE KIDNEY WITHOUT HISTOLOGICAL INVASION. HISTOLOGICAL INVASION.

MANAGEMENT OF THE MANAGEMENT OF THE INFERIOR VENA CAVAINFERIOR VENA CAVA

RESECTION OF THE INFERIOR RESECTION OF THE INFERIOR VENA CAVA SHOULD BE VENA CAVA SHOULD BE UNDERTAKEN IN SELECTED UNDERTAKEN IN SELECTED PATIENTS WHEN COMPLETE PATIENTS WHEN COMPLETE GROSS RESECTION OF TUMOR GROSS RESECTION OF TUMOR IS LIMITED BY INVOLVEMENT IS LIMITED BY INVOLVEMENT OF THE INFERIOR VENA CAVA.OF THE INFERIOR VENA CAVA.

REPAIR OF REPAIR OF INFERIOR VENA CAVAINFERIOR VENA CAVA

PRIMARY REPAIRPRIMARY REPAIRAUTOLOGOUS PATCH REPAIR AUTOLOGOUS PATCH REPAIR AUTOLOGOUS VEIN REPAIR AUTOLOGOUS VEIN REPAIR PROSTHETIC TUBE GRAFTING PROSTHETIC TUBE GRAFTING LIGATION OF INFERIOR VENA LIGATION OF INFERIOR VENA

CAVA CAVA

ROLE OF INCOMPLETE RESECTIONROLE OF INCOMPLETE RESECTION

INCOMPLETE GROSS RESECTION INCOMPLETE GROSS RESECTION OR DEBULKING IS NOT OR DEBULKING IS NOT ADVOCATED BECAUSE IT HAS NOT ADVOCATED BECAUSE IT HAS NOT BEEN ASSOCIATED WITH BEEN ASSOCIATED WITH IMPROVED SURVIVAL.IMPROVED SURVIVAL.

DELIBERATE PARTIAL RESECTION DELIBERATE PARTIAL RESECTION OF MOST RETROPERITONEAL OF MOST RETROPERITONEAL SARCOMA SHOULD LIMITED TO SARCOMA SHOULD LIMITED TO RELIEF OF INTESTINAL RELIEF OF INTESTINAL OBSTRUCTIONOBSTRUCTION

RADIATION THERAPYRADIATION THERAPYTREATMENT OPTIONSTREATMENT OPTIONSE.B.R.T.E.B.R.T.

PREOPERATIVEPREOPERATIVE POSTOPERATIVEPOSTOPERATIVE

I.O.R.T. (10 – 15 Gy)I.O.R.T. (10 – 15 Gy)BRACHYTHERAPYBRACHYTHERAPYE.B.R.T. + I.O.R.T. OR E.B.R.T. + I.O.R.T. OR

BRACHYTHERAPY MORE EFFECTIVEBRACHYTHERAPY MORE EFFECTIVE

RADIATION THERAPY (Cont.)RADIATION THERAPY (Cont.)THE HIGH DOSE REQUIRED THE HIGH DOSE REQUIRED

AROUND 60 Gy. AROUND 60 Gy. EBRT HAVE LIMITED ROLE EBRT HAVE LIMITED ROLE

BECAUSE OF LOW TOXICITY BECAUSE OF LOW TOXICITY THRESHOLD OF SURROUNDING THRESHOLD OF SURROUNDING TISSUE.TISSUE.

EBRT ASSOCIATED WITH DELAY IN EBRT ASSOCIATED WITH DELAY IN TIME OF LOCAL RECURRENCE TIME OF LOCAL RECURRENCE BUT NO IMPROVEMENT IN BUT NO IMPROVEMENT IN SURVIVAL.SURVIVAL.

CHEMOTHERAPYCHEMOTHERAPY NO PROVEN ROLE FOR ADJUVANT NO PROVEN ROLE FOR ADJUVANT

CHEMOTHERAPY IN COMPLETELY CHEMOTHERAPY IN COMPLETELY RESECTED R.P. SARCOMA.RESECTED R.P. SARCOMA.

CHEMOTHERAPY MAY BE USED IN :CHEMOTHERAPY MAY BE USED IN :

1.1. LOCALLY UNRESECTABLE DISEASELOCALLY UNRESECTABLE DISEASE

2.2. METASTIC R.P. SARCOMAMETASTIC R.P. SARCOMA

3.3. PATIENT UNDERGOES PATIENT UNDERGOES NEOADJUVANT TRAIL NEOADJUVANT TRAIL

MANAGEMENT OF LOCAL MANAGEMENT OF LOCAL RECURRENCE RECURRENCE

LOCAL RECURRENCE OCCUR IN LOCAL RECURRENCE OCCUR IN 41% OF PATIENTS IN FIVE YEARS41% OF PATIENTS IN FIVE YEARS

LOCAL RECURRENCE IS PRIMARY LOCAL RECURRENCE IS PRIMARY CAUSE OF DISEASE SPECIFIC CAUSE OF DISEASE SPECIFIC MORTALITY.MORTALITY.

COMPLETE SURGICAL RESECTION COMPLETE SURGICAL RESECTION IS MOST EFFECTIVE THERAPY IS MOST EFFECTIVE THERAPY FOR LOCAL RECURRENCE FOR LOCAL RECURRENCE

WHEN TO OPERATE? WHEN TO OPERATE?

PATIENTS WITH FIRST PATIENTS WITH FIRST LOCAL RECURRENCE LOCAL RECURRENCE SHOULD BE CONSIDER FOR SHOULD BE CONSIDER FOR REEXPLORATION.REEXPLORATION.

COMPLETE RESECTABILITY COMPLETE RESECTABILITY RATE AFTER FIRST RATE AFTER FIRST RECURRENCE IS 54 – 82%.RECURRENCE IS 54 – 82%.

WHEN TO OPERATE? WHEN TO OPERATE? (Cont.)(Cont.)

IN PATIENTS WITH SHORT IN PATIENTS WITH SHORT DISEASE FREE INTERVAL A DISEASE FREE INTERVAL A PERIOD OF OBSERVATION PERIOD OF OBSERVATION SHOULD BE FOLLOWED SHOULD BE FOLLOWED BEFORE OPERATION TO BEFORE OPERATION TO EXCLUDE THE EXCLUDE THE DEVELOPMENT OF DEVELOPMENT OF DISSEMINATED DIS. DISSEMINATED DIS.

DISTANT METASTASISDISTANT METASTASISMOST COMMON SITE FOR MOST COMMON SITE FOR

DISTANT METASTASIS IS LUNG & DISTANT METASTASIS IS LUNG & LIVER.LIVER.

RESECTION OF DISTANT RESECTION OF DISTANT METASTASIS RESECTED TO THE METASTASIS RESECTED TO THE PATIENTS IN WHOM A COMPLETE PATIENTS IN WHOM A COMPLETE RESECTION CAN BE RESECTION CAN BE PERFORMED. PERFORMED.

SURVIVAL & PREDICTORS OF SURVIVAL & PREDICTORS OF OUTCOMEOUTCOME

FACTOR ASSOCIATED WITH POOR FACTOR ASSOCIATED WITH POOR SURVIVAL.SURVIVAL.

1.1. INCOMPLETE GROSS RESECTIONINCOMPLETE GROSS RESECTION

2.2. UNRESECTABILITYUNRESECTABILITY

3.3. HIGH GRADEHIGH GRADE FACTOR ASSOCIATED WITH LOCAL FACTOR ASSOCIATED WITH LOCAL

RECURRENCERECURRENCE

1.1. HIGH GRADEHIGH GRADE

2.2. LIPOSARCOMA HISTOLOGYLIPOSARCOMA HISTOLOGY

SURVIVAL & PREDICTORS OF SURVIVAL & PREDICTORS OF OUTCOME OUTCOME (Cont.)(Cont.)

FACTOR ASSOCIATED WITH FACTOR ASSOCIATED WITH DISTANT METASTASIS :-DISTANT METASTASIS :-

1.1. INCOMPLETE RESECTION INCOMPLETE RESECTION

2.2. HIGH GRADEHIGH GRADE LIPOSARCOMA ASSOCIATED LIPOSARCOMA ASSOCIATED

WITH REDUCED RISK OF WITH REDUCED RISK OF DISTANT MATASTASIS DISTANT MATASTASIS

FOLLOW - UPFOLLOW - UP GOAL OF FOLLOW – UP IS TO DETECT GOAL OF FOLLOW – UP IS TO DETECT

CURABLE RECURRENT OR METASTATIC CURABLE RECURRENT OR METASTATIC DISEASE.DISEASE.

PATIENTS ARE EVALUATED CLINICALLY PATIENTS ARE EVALUATED CLINICALLY EVERY 4 MONTHS FOR 3 YEARS AND EVERY 4 MONTHS FOR 3 YEARS AND EVERY 6 MONTHS THERE AFTER.EVERY 6 MONTHS THERE AFTER.

CT SCAN ARE PERFORMED IN CT SCAN ARE PERFORMED IN PATIENTS IN WHOM OPERATION IS PATIENTS IN WHOM OPERATION IS CONSIDERED AT 6-12 MONTH INTERVAL CONSIDERED AT 6-12 MONTH INTERVAL

CONCLUSIONCONCLUSIONRETROPERITONEAL SARCOMA RETROPERITONEAL SARCOMA

ARE RARE.ARE RARE.THEY USUALLY REACH A LARGE THEY USUALLY REACH A LARGE

SIZE BEFORE PRESENTATION.SIZE BEFORE PRESENTATION.LIPOSARCOMAS IS MOST LIPOSARCOMAS IS MOST

COMMON.COMMON.CT SCAN IS THE MOST CT SCAN IS THE MOST

IMPORTANT IN PLANNING IMPORTANT IN PLANNING RESECTION.RESECTION.

CONCLUSION CONCLUSION (Cont.)(Cont.)AN ABDOMINAL APPROACH AS AN ABDOMINAL APPROACH AS

USUALLY ADVISEDUSUALLY ADVISEDCURABLE LESION SHOULD BE CURABLE LESION SHOULD BE

REMOVED RADICALLY AND NOT REMOVED RADICALLY AND NOT REMOVED FROM THEIR REMOVED FROM THEIR PSEUDOCAPSULEPSEUDOCAPSULE

50% OF TUMOR ARE 50% OF TUMOR ARE RESECTABLE AND 75% REQUIRE RESECTABLE AND 75% REQUIRE RESECTION OF ADJACENT RESECTION OF ADJACENT ORGANS. ORGANS.

CONCLUSION CONCLUSION (Cont.)(Cont.)SURVIVAL DEPENDENT UPON SURVIVAL DEPENDENT UPON

GRADE OF TUMOR AND STAGE.GRADE OF TUMOR AND STAGE.80% OF PATIENTS SUFFER 80% OF PATIENTS SUFFER

RECURRENCE.RECURRENCE.ADJUVANT CHEMOTHERAPY ADJUVANT CHEMOTHERAPY

HAS NO ROLE OUTSIDE HAS NO ROLE OUTSIDE CLINICAL TRIALCLINICAL TRIAL

CONCLUSION CONCLUSION (Cont.)(Cont.)RADIATION: NO PROVEN BENEFIT RADIATION: NO PROVEN BENEFIT

BUT DATA SUGGESTS THAT BUT DATA SUGGESTS THAT LOCAL CONTROL IS IMPROVED LOCAL CONTROL IS IMPROVED WITH RADIATION. WITH RADIATION.

THERE IS A PROBLEM OF DOSE-THERE IS A PROBLEM OF DOSE-RELATED TOXICITY. RELATED TOXICITY.

COMBINED EXTERNAL BEAM COMBINED EXTERNAL BEAM RADIATION THERAPY AND BOOST RADIATION THERAPY AND BOOST APPEARS TO BE SUPERIOR FOR APPEARS TO BE SUPERIOR FOR RESPONSE. RESPONSE.