« A Clear cell sarcoma »

2007-11383 FN

25 year-old male

January 2007 3cm mass on the medial side of the right heel

Early August 20073 cm clinical right inguinal massPreoperative MRI (images unavailable)

End August 2007General hospital: surgical biopsy of the right

heel massPathology (reviewed): clear cell sarcomaNo molecular analysis

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PET-CT

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September 2007 Clinical residual mass (4 cm) on the right

heel with skin infiltration Right inguinal mass (3 cm) Post surgical biopsy PET CT Chest CT: No lung metastases

 Amputation?Heel surgery with free flapInduction chemotherapy?ILP?Inguinal lymph node dissection?Radiotherapy?

What do you propose?

Sarcoma board• Metastatic disease (inguinal lymph nodes)• Conservative surgery not feasible

Upfront systemic treatment• Doxorubicin/Dacarbazine - 3 cycles

→ Stable disease (Mass 4 cm heel + Right 3 cm inguinal mass , no lung metastasis)

What next ???

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Isolated limb perfusion

• Melphalan 80 mg and TNF alpha 1 mg

• 1h (38°Celsius)

• + Inguinal lymph node dissection with sartorius

muscular flap

Pathological analysis

• Lymph nodes metastases of clear cell sarcoma

(2 N+ / 9)

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CR:

February 2008

RECIST PR: 26mm x 20mmModerate vascularization after dynamic injection = sign of good

response

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Surgery of the residual tumor?Radiotherapy?

March 2008Radiotherapy (45 Gy on the heel, 65 Gy on the inguinal region)Ended in May 2008 RECIST CR

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August 2008

August 2008 December 2008 May 2009

November 2009

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February 2010 Thoracic Surgery

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• Adjuvant treatment?

January 2011 + 3 years after ILP15 mm nodule on the inguinal incision Re evolution on the heel 

March 2011 2007-11383 FN

? MET INHIBITORS and ANTI ANGIOGENICS

Doxorubicin/Dacarbazine started in February 2011

Patient deceased in March 2011 (+ 4 years after diagnosis)

March 2011 2007-11383 FN