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Nccn guidelines ewings – Nccn guidelines ewings – Presentation Transcript

  • NCCN Guidelines Ewing’s SarcomaDr. S. Moses ArunsinghBIR&O22.07.2011
  • A SHORT HISTORY Explained by Dr. James Ewing in 1921. He called it “endothelioma of bone”. Ewing’s most influential academic contribution was his 1919 cancer textbook, Neoplastic Disease: A Textbook on Tumors, of which he was the sole author.2
  •  Was recognized by Time magazine, which featured a sketch of his visage on the cover, calling him “Cancer Man Ewing”.  Ironically, the Cancer Man died of bladder cancer in 1943 at the age of 76, and at autopsy was also found to have low-grade prostate cancer.3
  •  Ewing tumor is the second most common primary tumor of bone in childhood.  Between 8 years of age and after 25 years of age.  24.7% - Pelvis 16.4% - Femur 16.7% - Below knee 12.1% - Ribs, 8.0% - Spine4 4.8% - Humerus
  • 20 – 25 % present with metastasis at diagnosis. Ewing’s Sarcoma Family of tumors: Ewing’s sarcoma (Bone –87%) Extraosseous Ewing’s sarcoma (8%) Peripheral PNET(5%) Askin’s tumor (PNET of chest wall).5
  •  More than 90% of patients have t(11;22) [or t(21;22)] involving the EWS gene on chromosome 22.  Chromosome 11 – FLI1 (Friend leukemia virus integration 1)  Chromosome 21 – ERS (ethylene response sensor)  The c-myc protooncogene is frequently expressed in Ewing’s.  CD 99 ( MIC2)6
  •  Primary site: Extremity primaries (particularly distal extremity)versus axial lesions (unfavorable). Volume of tumor: Small primary versus larger lesion (unfavorable). Botha maximum tumor size of approximately 8 cm has beenused, as well as a volume cutoff of 200 cm3, Extent of disease : Localized disease versus metastatic disease(unfavorable). Site of metastasis: Lung metastases versus bone/bone marrow(unfavorable) versus both bone and lung metastases (worst). Age, High LDH, High ESR, No Surgery Response to Induction Chemo. 7
  •  For patients with localized disease – 5 year OS 60 – 70 %  Lung and Pleural Mets Only – 30 %  Bone / Bone Marrow mets – 15%  Both - < 10 %8
  • WORK UP MRI +/- CT OF THE PRIMARY SITE CT CHEST PET SCAN +/- BONE SCAN BONE MARROW BIOPSY OR MRI SCREENING OF PELVIS AND SPINE CYTOGENETICS / MOLECULAR STUDY LDH9 FERTILITY CONSULTATION
  • Induction TherapyMulti agent Chemotherapy for at least 12 –24 weeks prior to local therapy Restage with: ( Localized Disease) • Chest Imaging • Local Imaging • Consider PET Scan or Bone Scan • FOR METASTATIC DISEASE REPEAT THE PREVIOUSLY ABNORMAL STUDY Assess Response to Induction Chemotherapy10
  • Local Control Therapy response to primary treatment Wide Local Stable Disease Following Excision Definitive RT and chemotherapy Preoperative RT Amputation11
  • Continue Chemotherapy CAT I Positive Followed by RT Margins Chemotherapy CAT IWide Local Excision And RT Negative Chemotherapy Margins 12
  • Chemotherapy Pre Operative WIDE +/- Additional RT EXCISION RT13
  • Post Consider RT operative based on Amputation Chemothera Marginal py Status14
  • RT or Progressive Surgery of Chemotherapy Disease after Primary for or Best Primary Local supportive care Treatment Palliation15
  • Surveillance Physical Exam, Local and Chest Imaging: • Every 2- 3 months • Increase interval after 24 months • Annually after 5 years indefinitely CBC and other lab studies as indicated Consider Bone Scan or Pet scan16
  • Relapse Early relapse – less than 2 years: Consider Changing Chemotherapy Late relapse – more than 2 years: Continue the previously used chemotherapy17
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  • Gross disease(after biopsy onlyor intralesionalresection)Treatment once a Initial Field 45 1.8 q.dday Boost field 10.8 1.8 q.d.Treatment twice a Initial field 36 1.2 b.i.d.day Boost field 19.2 1.2 b.i.d.After marginal 41.4 – 45 1.8 q.d.resection or poorhistologic responseat surgeryPreoperative 45 1.8 q.d.radiotherapy: 20
  • Chemotherapy First Line therapy:  VAC/IE (Vincristine, Adriamycin, Cyclophosphamide, Ifosphamide, Etoposide)  VAI (Vincristine, Adriamycin, Ifosphamide)  VIDE ( Vincristine, ifosphamide, Doxorubicin, Etoposide) Metastatic:  CVD  VAC/IE  VAI  VIDE21
  • TMH Guidelines22
  • Radiotherapy detail  Tailored portals for every patient.  Entire Medullary cavity need not be included in the RT portal.  Target volumes (GTV) mentioned are MRI based. Includes bone + soft tissue component.  Try and spare a strip of normal tissue for lymph drainage.  If disease involves non-infiltrating extension into pre-formed body cavities e.g. lung & pelvis, radiotherapy volume includes post induction volume with 2cm margin in order to reduce treatment related toxicity.  3D-CRT / IMRT wherever necessary.23
  • Post Operative RT Surgical Margins Radiotherapy Dose If Radiotherapy Dose If Necrosis 100% Necrosis <100% Negative No Radiotherapy 45 GY Marginal 45 Gy 50 GY resection/Close Microscopic Positive 45 Gy 50 GY (R1) Gross + Positive (R2) 50 Gy 50 GY24
  • Borderline Resectable All patients to receive radical RT doses. Patients to be evaluated for surgery at 6th week after completion of RT. PTV: Phase I : Pre-chemotherapy volume + 3cm. margin Phase II : Post-chemotherapy volume + 2cm. margin (There is no field reduction for bony component). TOTAL DOSE : Phase I : 45Gy / 25# / 5wks (@1.8Gy / fr.) Phase II : 1) If complete response after induction no further boost. 2) If >50% regression after induct 10.8Gy / 6# / 1wk 25 3) If <50% regression after inductio 14.4Gy / 8# /1.5wks (@1.8Gy /#
  • Unresectable Same as borderline resectable lesions26
  • Lung Bath (Whole Lung Irradiation) All patients with metastatic disease to the lungs at presentation receive whole lung irradiation (“Lung Bath”), even if complete remission of pulmonary metastatic disease has been achieved after chemotherapy. RT Target Volume : Bilateral Lung No Cardiac Shield Shield Bilateral Shoulder Dose : 12.6 Gy/ 7#/ 1week27
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