Faculty of Medical Science, Stip and Clinic of Oncology and Radiotherapy,

Skopje R.Macedonia

Ewing sarcoma: a case report

D-r Marija Karakolevska - Ilova

MATERIAL AND METHODS

• 17 year old patient

• Pain and swelling of the right femoral region for 4 weeks with intermittent temperature (02.05.2007year)

• Laboratory – normal

• CT on right femoral bone: changed sceletal structure in distal part of right femoral bone with soft tissue substrate in deep musculature without involvement of peripheral musculature.• Sceleton scan: pathological accumulation of the lower and middle third of the right femur, great trochanter of the right femur and right parietal bone.• FNAB – classification group 5 - cytomorphologycal• Biopsio femoris lat. dex. - histophatologycal - immunohistohemical

Ewing sarcoma - PNET

Initial treatment with:

-Radyotherapy : 3D conformal delivery treatment with TCT (1,25Mev), depth = 6sm, field: 10x30 sm, TTD=50Gy (25fr/2Gy)(11.06.2007 – 13.07.2007 ) followed by

- Chemotherapy :IV courses with : Cyclophosphamide 1000mg 1-3 d, Doxorubicin 30mg 1-3d, Etoposid 200 mg 1-3d, MESNA 4000mg 1-3 d. followed by II courses of Cyclophosphamide 500mg 1-3 d, Etoposid 200mg 1-3 and Vinkristine 2mg -Blood support is achieved with GSF and antifungal drug after each course.

- At the end of chemotherapy the surgeon was consulted for reevaluation for surgery , and no surgery was performed.

Follow up:

2008:Stable disease. ECOG PS =0, X-rays of the lung : normal, ultrasound of abdomen: normal, laboratory: normal ( every 3 months )

2009: Stable disease. ECOG PS =0, X-rays of the lung: normal, ultrasound of abdomen: normal, laboratory: normal ( every 3 months )

2010: ECOG PS=0, X-rays of the lung: normal, ultrasound of abdomen: normal, laboratory: normal ( every 3 months ) until 1.10.2010 when the pacient came with:

- Cephalea- Vomiting and fatigue- Left side facial pain- Egzophtalmus on the left eye

Examinations : CT of brain: Exspansive mass in middle fossa witch is compromasing the temporal lobe and left frontal lobe. It penetrates into the left orbit with dislocation of the eye bulge anteriorly. Scan sceleton(Tc99m): Recidiv localis

Treatment:

Brain metastases: 3D conformal radiotherapy delivery with TTD=48Gy ( 24fr/2Gy ) local on the tumor site badAntiedematous therapy

followed by

Chemotherapy: I-III courses with Cyclophosphamide 500mg 1-3d, Etoposid 200mg 1-3, Vincristine 2mg, IV-V course continued with Doxorubicin 30mg 1-3d, VI-VII course with Ifosfamid 4000mg , Vepesid 150mg , Mesna 6000mg.

During the treatment and 6 months after CT on brain was performed : no sings of local relapse were found ( 15.11.2011)

( 1.12.2011) – Tumefaction on the right parietal bone – 3-4sm . FNAB – classification group 5Sceleton scan : pathological accumulation in right femur, right temporal bone and right sixth rib

Bisphosphonate therapy was added3D conformal RT locally on tumefaction of the right parietal bone with TTD=39Gy

After 2 months : cephalea - recidiv retrobulbaris ( 28.02.2012 )The patient was treated with palliative chemotherapy with Docetaxel 120 mg ( VI courses )4 months after :

-Egzophtalmus on the left eye - LDH : 889; 913

Treatment : Whole brain irradiation TTD=30Gy with Temozolomide.

At the moment the patient is with deteriorated general condition, with paresis of the left lower limb

DISCUSSION

- Ewing's sarcoma has a propensity to metastasize to the lung, bone and bone marrow. This tumor can also involve the CNS with a relatively low incidence – 1-8%.

- The literature suggests the incidence may be increasing with the increase in use of chemotherapy and with patients living longer.

- Between 20-25% of patients are diagnosed with metastatic disease.

- Studies investigating CNS involvement of Ewing's sarcoma have reported that spread through direct extension from the skull is more frequent than hematogenous spread.

- Prognostic factors that confer poorer outcome are: serum LDH level, axial localization or older age ( > 15 years), size and volume, incomplete or no surgery for local therapy and time of relaps ( < 24 months ).

LDH elevated at time of CNS relapse – LDH has to be evaluated during the whole treatment.

Patients with metastases and recurrent disease still fare poorly, with 5-year survival rates of 20% witch highlights the need for novel chemotherapeutic agents, bisphosphonates and targeted therapy