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Ewing sarcoma: a case report

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

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Ewing sarcoma: a case report

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  1. Faculty of Medical Science, Stip and Clinic of Oncology and Radiotherapy, Skopje R.Macedonia Ewing sarcoma: a case report D-r Marija Karakolevska - Ilova

  2. 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 • Biopsiofemoris lat. dex. - histophatologycal • - immunohistohemical Ewing sarcoma - PNET

  3. 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.

  4. 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

  5. Treatment: • Brain metastases: 3D conformal radiotherapy delivery with TTD=48Gy ( 24fr/2Gy ) local on the tumor site bad • Antiedematous 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 5 • Sceleton scan : pathological accumulation in right femur, right temporal bone and right sixth rib

  6. Bisphosphonate therapy was added • 3D conformal RT locally on tumefaction of the right parietal bone with TTD=39Gy • After 2 months : cephalea - recidivretrobulbaris( 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

  7. 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 ).

  8. 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

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