1. SOFT TISSUE TUMORS Early diagnosis Nicolas SANS Hôpital Universitaire Purpan - Toulouse - FRANCE

2. This is not a muscular tear… RHADOMYOSARCOMA

3. This is not a popliteal cyst… LYMPHOMA

4. This is not an intramuscular hematoma …

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

6. EPIDEMIOLOGY BENIGN TUMORS 300 /100 000 MALIGNANT TUMORS 3 /100 000 Kransdorf et Murphey, 1997 Soft Tissue Sarcomas 2000 new cases per year in France

7. CAT The rarity and the anatomo-clinical heterogeneousness returns the difficult treatment The rarity and the anatomo-clinical heterogeneousness returns the difficult treatment The vital and functional future is strongly dependent on the initial treatment of the tumor The vital and functional future is strongly dependent on the initial treatment of the tumor A multidisciplinary approach is necessary and, it, from the diagnostic time to the post therapeutic time A multidisciplinary approach is necessary and, it, from the diagnostic time to the post therapeutic time

8. NATURAL HISTORY Centrifugal Longitudinal Fibro-vascular reaction

9. NATURAL HISTORY Centrifugal Longitudinal Fibro-vascular reaction Capsule (B) Pseudo capsule (M)

10. PHYSICAL SIGNS deep mass, often little painful duration of the symptoms ? recent increase of volume ? diameter > 5 cm

11. PROGNOSTIC FACTORS Age > 50 ans Age > 50 ans Male (±) Male (±) Location : head, neck, chest Location : head, neck, chest Histological grade Histological grade Histological type (±) Histological type (±) SURGICAL MARGINS SURGICAL MARGINS

12. The PROGNOSTIC depends on the initial surgical treatment R0 all tumour tissue was macroscopically removed with microscopically clear margins recurrence 10% for 5 years R1 microscopic residual disease or with close margins (less than 1 mm) recurrence 50% for 5 years R2 macroscopic residual disease recurrence 90% for 5 years Post operative irradiation can’t improve an incorrect surgery

13. Muscular fascia Enthesis Cartilage Cortical bone Periost Anderson MW et al. AJR 1999 COMPARTMENTAL ANATOMY UNI PLURI

14. MEDICAL IMAGING

15. GOALS 1.To define the most sensitive technique in the detection of the masses of soft tissues 2.To estimate the most specific technique as for the differentiation between a benign and malignant tumor 3.To appreciate the operability and participate in the therapeutic planification 4.To approach the histological nature

16. INITIAL DIAGNOSIS In few cases images are pathognomonic

17. Elastofibroma

18. Courtesy D Godefroy Fibrolipoma of the median nerve

19. PLAIN RADIOGRAPHS Frequently unrewarding Frequently unrewarding

20. PLAIN RADIOGRAPHS Sometimes evokes the diagnosis Sometimes evokes the diagnosis

21. SONOGRAPHY cystic vs solid lesions cystic vs solid lesions calficiations calficiations to eliminate an hematoma to eliminate an hematoma

22. CT LipomaOssificans myositis

23. MRI Morphological 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

24. Superficial : « benign » If size < 3 cm Deep : « malignant » If size > 5 cm Depth & Size

25. Sarcoma well defined margins

26. Poor defined margins T Hematoma

27. Desmoid tumor Poor defined margins

28. Vascular and/or nervous contact

29. Surgical planification

30. Crossing a FasciaExtra compartmental

31. Crossing a Fascia Fibromatosis Vascular tumor Nervous tumor

32. NOT WITHOUT FAT SAT !!! Synovialosarcoma Gielen, JCAT 2003

33. NOT WITHOUT FAT !!! T1 Fat Sat Gado

34. NOT WITHOUT FAT !!! T1 Fat Sat Gado

35. IN FAVOR OF MALIGNANCY Heterogeneous T1 Heterogeneous T1 T1 homogeneous → T2 heterogeneous T1 homogeneous → T2 heterogeneous Low signal intensity of the septa on T2 Low signal intensity of the septa on T2 Necrosis represents more than 50 % of the lesion Necrosis represents more than 50 % of the lesion Hermann et al. BJR 1992; 65:14-20

36. SIGNAL ANALYSIS

37. Heterogeneous or hyperintense on T1 Synovialosarcoma Se +++ Sp --- Liposarcoma

38. Leiomyosarcoma T1T2 Homogeneous signal on T1 Heterogeneous on T2 Se = 72-80% Sp = 87-91%

39. Liposarcoma Low signal intensity of the septa on T2

40. T2T1 Fat Sat Gado Fast and prolonged enhancement

41. Necrosis > 50%

42. MRI 1. Lesion of more than 50 mm in diameter 2. Deep localization 3. Irregular or lobulated margins 4. Irregular or tick septa 5. Heterogeneous signal on T1 and T2 6. Low signal intensity of the septa on T2 7. Fast and prolonged enhancement 8. Necrosis more than > 50% KRANSDORF, 2000; DESCHEPPER, 2000; VARMA, 1999;CEUGNART,2002 MORPHOLOGYMORPHOLOGY SIGNALSIGNAL

43. PATHOLOGY

44. PATHOLOGY 1.To differentiate begnin or malignant tumor 2.To confirm that it is indeed a conjunctival tumor (vs lymphoma, metastasis…) 3.Define the type of surgery which must be realized (enucleation for conjunctival tumor, extended resection for sarcoma) 4.To discuss a neoadjuvant treatment GOALS

45. PATHOLOGY 1.Microbiopsy 2.Biopsy excision 3.Surgical biopsy

46. PATHOLOGY Tissue sample 1. Formol fixation 2. Freezing - Cryosection molecular study X

47. PATHOLOGY Tissue sample 1. Formol fixation 2. Freezing - Cryosection molecular study X Pathologist !

48. PATHOLOGY Tissue sample 1. Formol fixation 2. Freezing - Cryosection molecular study X Pathologist !

49. BIOPSY 1.Perform the biopsy before the MRI 2.Compromise or complicate the later treatment by an unsuitable way What you should not make

50. BIOPSY 1.Perform the biopsy before the MRI 2.Compromise or complicate the later treatment by an unsuitable way 3.Obtain insufficient samples What you should not make

51. STAGING

52. CONCLUSION (1) The initial medical management of a soft tissue sarcoma is essential for the future of patient The initial medical management of a soft tissue sarcoma is essential for the future of patient Think of a sarcoma when : Think of a sarcoma when : Size more than 5 cm Size more than 5 cm Deep Deep Symptomatic lesion Symptomatic lesion

53. CONCLUSION (2) 1.MRI 2.Discuss the therapeutic plan before any surgical procedure 3.Biopsy Experimented pathologist Experimented pathologist Freezing Freezing 4.PHRC MULTIDISCIPLINARY CONCERTATION

54. Impact d’un Programme d’intervention de Santé publique ciblé sur la prise en charge initiale des SARcomes des tissus de l’adulte AquitaineLanguedoc-RoussillonLimousinMidi-Pyrénées Pays de Loire

55. CONSTATS Non conformité de la prise en charge initiale malgré la diffusion de recommandations nationales Non conformité de la prise en charge initiale malgré la diffusion de recommandations nationales Méconnaissance clinique et radiologique Méconnaissance clinique et radiologique Multiplicité des intervenants ; sites spécialisés ? Multiplicité des intervenants ; sites spécialisés ? PAYS SCANDINAVES (1989) : prise en charge spécialisée dans 80% des cas

56. OBJECTIFS Mise en place d’actions collectives pour améliorer la prise en charge des STM de l’adulte (diagnostic + bilan initial) Mise en place d’actions collectives pour améliorer la prise en charge des STM de l’adulte (diagnostic + bilan initial) Mesurer l’impact en terme de : Mesurer l’impact en terme de : proportion de prise en charge globale adéquate proportion de prise en charge globale adéquate survie survie Estimer l’incidence régionale des sarcomes en collaboration avec les registres départementaux des cancers des régions étudiées Estimer l’incidence régionale des sarcomes en collaboration avec les registres départementaux des cancers des régions étudiées