« A Clear cell sarcoma » FN

25 year-old male January cm mass on the medial side of the right heel Early August cm clinical right inguinal mass Preoperative MRI (images unavailable) End August 2007 General hospital: surgical biopsy of the right heel mass Pathology (reviewed): clear cell sarcoma No molecular analysis FN

PET-CT FN 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 flap Induction 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 ??? September FN

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) FN January 2008

CR:

February 2008 RECIST PR: 26mm x 20mm Moderate vascularization after dynamic injection = sign of good response FN

Surgery of the residual tumor? Radiotherapy?

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

August 2008

December 2008 May 2009 November FN

February 2010 Thoracic Surgery FN Adjuvant treatment?

January years after ILP 15 mm nodule on the inguinal incision Re evolution on the heel

March FN

? MET INHIBITORS and ANTI ANGIOGENICS

Doxorubicin/Dacarbazine started in February 2011 Patient deceased in March 2011 (+ 4 years after diagnosis) March FN