SOFT TISSUE TUMORS SOFT TISSUE TUMORS

Early diagnosisEarly diagnosis

SOFT TISSUE TUMORS SOFT TISSUE TUMORS

Early diagnosisEarly diagnosis

Nicolas SANS

Hôpital Universitaire Purpan - Toulouse - FRANCE

This is not a muscular tear…

RHADOMYOSARCOMA

This is not a popliteal cyst…

LYMPHOMA

This is not an intramuscular hematoma …

ANY MUSCULAR LESION WHICH DOES NOT EVOLVE BETWEEN 2 CONTROLS HAS TO MAKE EVOKE A TUMOR

ANGIOSARCOMA

EPIDEMIOLOGYEPIDEMIOLOGYEPIDEMIOLOGYEPIDEMIOLOGY

BENIGN TUMORS 300 /100 000

MALIGNANT TUMORS 3 /100 000

Kransdorf et Murphey, 1997

Soft Tissue Sarcomas

2000 new cases

per year in France

NATURAL HISTORYNATURAL HISTORYNATURAL HISTORYNATURAL HISTORY

Centrifugal

Longitudinal

Fibro-vascular reaction

NATURAL HISTORYNATURAL HISTORYNATURAL HISTORYNATURAL HISTORY

Centrifugal

Longitudinal

Fibro-vascular reaction

Capsule (B)

Pseudo capsule (M)

PHYSICAL SIGNSPHYSICAL SIGNSPHYSICAL SIGNSPHYSICAL SIGNS

deep mass, often little painful

duration of the symptoms ?

recent increase of volume ?

diameter > 5 cm

PROGNOSTIC FACTORSPROGNOSTIC FACTORSPROGNOSTIC FACTORSPROGNOSTIC FACTORS

• Age > 50 ansAge > 50 ans

• Male (Male (±)±)

• Location : head, neck, chestLocation : head, neck, chest

• Histological gradeHistological grade

• Histological type (±)Histological type (±)

• SURGICAL MARGINSSURGICAL MARGINS

• Age > 50 ansAge > 50 ans

• Male (Male (±)±)

• Location : head, neck, chestLocation : head, neck, chest

• Histological gradeHistological grade

• Histological type (±)Histological type (±)

• SURGICAL MARGINSSURGICAL MARGINS

The PROGNOSTIC depends on the The PROGNOSTIC depends on the initial surgical treatmentinitial surgical treatment

The PROGNOSTIC depends on the The PROGNOSTIC depends on the initial surgical treatmentinitial surgical treatment

R0R0

all tumour tissue was macroscopically all tumour tissue was macroscopically removed with microscopically clear marginsremoved with microscopically clear margins

recurrence 10% for 5 yearsrecurrence 10% for 5 years

R1R1

microscopic residual disease or with close microscopic residual disease or with close margins (less than 1 mm)margins (less than 1 mm)

recurrence 50% for 5 yearsrecurrence 50% for 5 years

R2R2

macroscopic residual diseasemacroscopic residual disease

recurrence 90% for 5 yearsrecurrence 90% for 5 years

R0R0

all tumour tissue was macroscopically all tumour tissue was macroscopically removed with microscopically clear marginsremoved with microscopically clear margins

recurrence 10% for 5 yearsrecurrence 10% for 5 years

R1R1

microscopic residual disease or with close microscopic residual disease or with close margins (less than 1 mm)margins (less than 1 mm)

recurrence 50% for 5 yearsrecurrence 50% for 5 years

R2R2

macroscopic residual diseasemacroscopic residual disease

recurrence 90% for 5 yearsrecurrence 90% for 5 years

Post operative Post operative

irradiation can’t irradiation can’t

improve an improve an

incorrect surgeryincorrect surgery

Muscular fascia

Enthesis

Cartilage

Cortical bone

Periost

Muscular fascia

Enthesis

Cartilage

Cortical bone

Periost

Anderson MW et al. AJR 1999

COMPARTMENTAL ANATOMY

UNI

PLURI

MEDICAL IMAGINGMEDICAL IMAGINGMEDICAL IMAGINGMEDICAL IMAGING

GOALS GOALS GOALS GOALS

1.1. To define the most To define the most sensitivesensitive technique in the technique in the

detection of the masses of soft tissuesdetection of the masses of soft tissues

2.2. To estimate the most To estimate the most specificspecific technique as for technique as for

the differentiation between a benign and the differentiation between a benign and

malignant tumormalignant tumor

3.3. To appreciate the To appreciate the operabilityoperability and participate in and participate in

the therapeutic planification the therapeutic planification

4.4. To approach the histological nature To approach the histological nature

INITIAL DIAGNOSISINITIAL DIAGNOSISINITIAL DIAGNOSISINITIAL DIAGNOSIS

In few cases images are In few cases images are

pathognomonicpathognomonic

Elastofibroma

Courtesy D Godefroy

Fibrolipoma of the median nerve

PLAIN RADIOGRAPHSPLAIN RADIOGRAPHSPLAIN RADIOGRAPHSPLAIN RADIOGRAPHS

Frequently Frequently

unrewardingunrewarding

PLAIN RADIOGRAPHSPLAIN RADIOGRAPHSPLAIN RADIOGRAPHSPLAIN RADIOGRAPHS

Sometimes evokes Sometimes evokes

the diagnosis the diagnosis

SONOGRAPHYSONOGRAPHYSONOGRAPHYSONOGRAPHY

cystic cystic vsvs solid lesions solid lesions

calficiationscalficiations

to eliminate an hematomato eliminate an hematoma

MRIMRIMRIMRI

Morphological Analysis - Signal analysisMorphological Analysis - Signal analysis

1. Multiplanar study (axial +++)

2. T1 weighted - T2 weighted

1. Pre and post Gadolinium injection

2. With and without fat saturation

3. Dynamic study

4. MRA

Superficial : « benign »

If size < 3 cm

Deep : « malignant »

If size > 5 cm

Depth & Size

Sarcoma

well defined margins

Poor defined margins

T

Hematoma

Desmoid tumor

Poor defined margins

Vascular and/or nervous contact

Surgical planification

Crossing a Fascia Extra compartmental

Crossing a Fascia

Fibromatosis Vascular tumorNervous tumor

NOT WITHOUT FAT SAT !!!

Synovialosarcoma

Gielen, JCAT 2003

NOT WITHOUT FAT !!!

T1 Fat Sat Gado

NOT WITHOUT FAT !!!

T1 Fat Sat Gado

SIGNAL ANALYSISSIGNAL ANALYSIS

Heterogeneous or hyperintense on T1Heterogeneous or hyperintense on T1

Synovialosarcoma

Se +++ Sp ---

Liposarcoma

Leiomyosarcoma

T1 T2

Homogeneous signal on T1 Heterogeneous on T2Homogeneous signal on T1 Heterogeneous on T2

Se = 72-80% Sp = 87-91%

Liposarcoma

Low signal intensity of the septa on T2Low signal intensity of the septa on T2

T2 T1 Fat Sat Gado

Fast and prolonged enhancementFast and prolonged enhancement

Necrosis > 50%Necrosis > 50%

MRIMRIMRIMRI

1.1. Lesion of more than 50 mm in diameterLesion of more than 50 mm in diameter

2.2. Deep localizationDeep localization

3.3. Irregular or lobulated marginsIrregular or lobulated margins

4.4. Irregular or tick septa Irregular or tick septa

5.5. Heterogeneous signal on T1 and T2Heterogeneous signal on T1 and T2

6.6. Low signal intensity of the septa on T2Low signal intensity of the septa on T2

7.7. Fast and prolonged enhancementFast and prolonged enhancement

8.8. Necrosis more than > 50%Necrosis more than > 50%

1.1. Lesion of more than 50 mm in diameterLesion of more than 50 mm in diameter

2.2. Deep localizationDeep localization

3.3. Irregular or lobulated marginsIrregular or lobulated margins

4.4. Irregular or tick septa Irregular or tick septa

5.5. Heterogeneous signal on T1 and T2Heterogeneous signal on T1 and T2

6.6. Low signal intensity of the septa on T2Low signal intensity of the septa on T2

7.7. Fast and prolonged enhancementFast and prolonged enhancement

8.8. Necrosis more than > 50%Necrosis more than > 50%

KRANSDORF, 2000; DESCHEPPER, 2000; VARMA, 1999;CEUGNART,2002

MORPHOLOGY

SIGNAL

PATHOLOGYPATHOLOGYPATHOLOGYPATHOLOGY

PATHOLOGYPATHOLOGYPATHOLOGYPATHOLOGY

1.1. To differentiate begnin or malignant tumorTo differentiate begnin or malignant tumor

2.2. To confirm that it is indeed a conjunctival tumor To confirm that it is indeed a conjunctival tumor

(vs lymphoma, metastasis…)(vs lymphoma, metastasis…)

3.3. Define the type of surgery which must be Define the type of surgery which must be

realized realized (enucleation for conjunctival tumor, extended (enucleation for conjunctival tumor, extended

resection for sarcoma)resection for sarcoma)

4.4. To discuss a neoadjuvant treatmentTo discuss a neoadjuvant treatment

GOALS

PATHOLOGYPATHOLOGYPATHOLOGYPATHOLOGY

1.1. MicrobiopsyMicrobiopsy

2.2. Biopsy excisionBiopsy excision

3.3. Surgical biopsySurgical biopsy

PATHOLOGYPATHOLOGYPATHOLOGYPATHOLOGY

Tissue sampleTissue sample

1.1. Formol fixationFormol fixation

2.2. Freezing - CryosectionFreezing - Cryosection

molecular studymolecular study

X

PATHOLOGYPATHOLOGYPATHOLOGYPATHOLOGY

Tissue sampleTissue sample

1.1. Formol fixationFormol fixation

2.2. Freezing - CryosectionFreezing - Cryosection

molecular studymolecular study

X

Pathologist !

PATHOLOGYPATHOLOGYPATHOLOGYPATHOLOGY

Tissue sampleTissue sample

1.1. Formol fixationFormol fixation

2.2. Freezing - CryosectionFreezing - Cryosection

molecular studymolecular study

X

Pathologist !

BIOPSYBIOPSYBIOPSYBIOPSY

1.1. Perform the biopsy before the Perform the biopsy before the

MRIMRI

2.2. Compromise or complicate the Compromise or complicate the

later treatment by an later treatment by an

unsuitable wayunsuitable way

What you should not make

BIOPSYBIOPSYBIOPSYBIOPSY

1.1. Perform the biopsy before the Perform the biopsy before the

MRIMRI

2.2. Compromise or complicate the Compromise or complicate the

later treatment by an later treatment by an

unsuitable wayunsuitable way

3.3. Obtain insufficient samplesObtain insufficient samples

What you should not make

STAGINGSTAGINGSTAGINGSTAGING

CONCLUSION (1)CONCLUSION (1)CONCLUSION (1)CONCLUSION (1)

The initial medical management of a soft tissue The initial medical management of a soft tissue

sarcoma is essential for the future of patientsarcoma is essential for the future of patient

Think of a sarcoma when :Think of a sarcoma when :

• Size more than 5 cmSize more than 5 cm

• DeepDeep

• Symptomatic lesionSymptomatic lesion

CONCLUSION (2)CONCLUSION (2)CONCLUSION (2)CONCLUSION (2)

1.1. MRI MRI

2.2. Discuss the therapeutic plan before any surgical Discuss the therapeutic plan before any surgical

procedureprocedure

3.3. BiopsyBiopsy

• Experimented pathologistExperimented pathologist

• FreezingFreezing

4.4. PHRC PHRC

MULTIDISCIPLINARY CONCERTATIONMULTIDISCIPLINARY CONCERTATION

Impact d’un Programme d’intervention de Santé Impact d’un Programme d’intervention de Santé

publique ciblé sur la prise en charge initiale des publique ciblé sur la prise en charge initiale des

SARcomes des tissus de l’adulteSARcomes des tissus de l’adulte

Impact d’un Programme d’intervention de Santé Impact d’un Programme d’intervention de Santé

publique ciblé sur la prise en charge initiale des publique ciblé sur la prise en charge initiale des

SARcomes des tissus de l’adulteSARcomes des tissus de l’adulte

AquitaineAquitaine

Languedoc-RoussillonLanguedoc-Roussillon

LimousinLimousin

Midi-PyrénéesMidi-Pyrénées

Pays de LoirePays de Loire

CONSTATSCONSTATSCONSTATSCONSTATS

NonNon conformitéconformité de la prise en de la prise en

charge initiale malgré la diffusion charge initiale malgré la diffusion

de recommandations nationalesde recommandations nationales

MéconnaissanceMéconnaissance clinique et clinique et

radiologiqueradiologique

MultiplicitéMultiplicité des intervenants ; sites des intervenants ; sites

spécialisés ?spécialisés ?

PAYS SCANDINAVES (1989) :

prise en charge spécialisée dans

80% des cas

OBJECTIFSOBJECTIFSOBJECTIFSOBJECTIFS

Mise en place d’Mise en place d’actions collectivesactions collectives pour améliorer la prise en pour améliorer la prise en

charge des STM de l’adulte (diagnostic + bilan initial)charge des STM de l’adulte (diagnostic + bilan initial)

Mesurer Mesurer l’impact l’impact en terme de :en terme de :

• proportion de prise en charge globale adéquateproportion de prise en charge globale adéquate

• surviesurvie

Estimer Estimer l’incidence régionalel’incidence régionale des sarcomes en collaboration des sarcomes en collaboration

avec les registres départementaux des cancers des régions avec les registres départementaux des cancers des régions

étudiéesétudiées