{"id":7473,"date":"2026-06-20T04:08:00","date_gmt":"2026-06-20T04:08:00","guid":{"rendered":"https:\/\/gothi.gov.eg\/?page_id=7473"},"modified":"2026-06-20T04:11:19","modified_gmt":"2026-06-20T04:11:19","slug":"%d8%a7%d9%88%d8%b1%d8%a7%d9%85-%d8%a3%d8%b7%d9%81%d8%a7%d9%84","status":"publish","type":"page","link":"https:\/\/gothi.gov.eg\/?page_id=7473","title":{"rendered":"\u0627\u0648\u0631\u0627\u0645 \u0623\u0637\u0641\u0627\u0644"},"content":{"rendered":"\t\t
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  1. <\/li>\n<\/ol>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t
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    \u0627\u0648\u0631\u0627\u0645 \u0623\u0637\u0641\u0627\u0644\n<\/h2>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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    \n\n \n\n \n <\/path><\/svg> <\/span>\n\n \n <\/path><\/svg> <\/span>\n \n <\/span>\n \n \n Paediatric Burkitt lymphoma <\/span>\n\n <\/h5>\n\n
    \n
    \n
    \n
    \n
    “last update: 14 Oct 2024”\u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0\u00a0\u00a0<\/span>Download Guideline<\/u><\/strong><\/a><\/h5>\n<\/div>\n<\/div>\n<\/div>\n
    \n
    \n
    \n
    \u00a0<\/div>\n<\/div>\n<\/div>\n<\/div>\n
    \n

    – Executive Summary<\/h3>\n
    \n

    \u00a0<\/p>\n

    This guidance provides a data-supported approach to the diagnosis, risk stratification, treatment and follow up of paediatric patients diagnosed with Burkitt lymphoma.<\/span><\/p>\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n
    \n

    <\/a>Recommendations<\/span><\/b><\/p>\n<\/td>\n

    		\n

    Level Of recommendation<\/span><\/b><\/p>\n<\/td>\n<\/tr>\n

    \n

    1<\/u><\/b>–<\/span><\/u><\/b>Work up for newly diagnosed NHL<\/span><\/u><\/b><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Pathology specimen is recommended with the proper IHC.<\/span><\/p>\n<\/td>\n

    		\n

    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    We recommend whole body FDG- PET CT\u00a0<\/span>if available otherwise contrast enhanced CT neck, chest, abdomen and pelvis is recommended.<\/span><\/p>\n<\/td>\n

    		\n

    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Bilateral bone marrow aspiration and biopsy is recommended as well as CSF examination.<\/span><\/p>\n<\/td>\n

    		\n

    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    2-Treatment of clinical group A<\/span><\/u><\/b><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Two 21-day cycles COPAD are recommended.<\/span><\/p>\n<\/td>\n

    		\n

    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Response assessment is recommended to include imaging studies of primary tumour site.<\/span><\/p>\n<\/td>\n

    		\n

    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    <\/a>3-Treatment of clinical group B<\/span><\/u><\/b><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Multiagent chemotherapy is recommended starting with pre phase COP Followed by response assessment post COP<\/span><\/p>\n

    Then administer 2 induction courses COPADM3<\/sub>\u00a0and two consolidation courses\u00a0<\/span>CYM.<\/span><\/p>\n<\/td>\n

    		\n

    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Contrast enhanced\u00a0<\/span>CT neck, chest, abdomen and pelvis is recommended for response assessment after COP and<\/span><\/p>\n

    FDG- PET\/CT is recommended for assessment after<\/span><\/p>\n

    CYM I.<\/span><\/p>\n<\/td>\n

    		\n

    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Response assessment after CYM I:<\/span><\/p>\n

    \u00b7\u00a0<\/span>If in CR, then continuation of CYM II is recommended.<\/span><\/p>\n

    \u00b7\u00a0<\/span>If not in CR, biopsy is recommended. If biopsy is not feasible then continue as group B if PET\/CT is negative. Upgrade to group C if biopsy is viable or PET\/CT is positive.<\/span><\/p>\n<\/td>\n

    		\n

    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Rituximab addition to chemotherapy is recommended in all high-risk group B<\/span><\/p>\n<\/td>\n

    		\n

    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    4-Treatment of clinical group C<\/span><\/u><\/b><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Multiagent chemotherapy should be initiated with pre phase (R-COP), followed by 2 induction courses (R-COPADM8<\/sub>), 2 consolidation courses (R-CYVE) and 2 maintenance courses (Sequences 1 and 2)<\/span><\/p>\n<\/td>\n

    		\n

    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Rituximab addition to chemotherapy is recommended for all group C patients<\/span><\/p>\n<\/td>\n

    		\n

    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    For Group C CNS disease: We recommend a total of 3 intrathecals in pre phase COP and high dose methotrexate after CYVE1<\/span><\/p>\n<\/td>\n

    		\n

    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    5-End of treatment evaluation<\/span><\/u><\/b><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    End of treatment evaluation should be done and if in CR then follow up is recommended. If not in CR, repeat biopsy from suspicious lesions, and if relapse is confirmed, start relapse protocol.<\/span><\/p>\n<\/td>\n

    		\n

    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    6-Treatment of relapse or refractory disease<\/span><\/u><\/b><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Combination chemotherapy is recommended with regimen:<\/span><\/p>\n

    (R-ICE) Rituximab, ifosfamide, carboplatin, etoposide and intrathecal chemotherapy<\/span><\/p>\n<\/td>\n

    		\n

    Strong Recommendation<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    7- Surveillance (follow up after end of treatment)<\/span><\/u><\/b><\/p>\n<\/td>\n

    		\u00a0<\/td>\n<\/tr>\n
    \n

    Routine scans are not recommended unless clinically suspicious. Monthly clinical examination is recommended for the first 3 years then annually.<\/span><\/u><\/b><\/p>\n<\/td>\n

    		\n

    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<\/div>\n<\/div> <\/div>\n\n <\/div>\n

    \n\n
    \n\n \n\n \n <\/path><\/svg> <\/span>\n\n \n <\/path><\/svg> <\/span>\n \n <\/span>\n \n \n Ewing Sarcoma <\/span>\n\n <\/h5>\n\n
    \n
    \n
    \n
    \n
    “last update: 14 Oct 2024”\u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0<\/span>Download Guideline<\/strong><\/u><\/a><\/h5>\n<\/div>\n<\/div>\n<\/div>\n
    \n
    \n
    \n
    \u00a0<\/div>\n<\/div>\n<\/div>\n<\/div>\n
    \n

    – Executive Summary<\/h3>\n
    \n

    \u00a0<\/p>\n

    This guidance provides a data-supported approach to the diagnosis, treatment, and follow up of paediatric patients diagnosed with Ewing sarcoma.<\/span><\/p>\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n
    \n

    Recommendations<\/span><\/b><\/p>\n

    \u00a0<\/span><\/b><\/p>\n

    \u00a0<\/span><\/b><\/p>\n<\/td>\n

    		\n

    Strength Of recommendation<\/span><\/b><\/p>\n<\/td>\n<\/tr>\n

    \n

    1-<\/span><\/u><\/b>Work up for newly diagnosed\u00a0<\/span><\/u><\/b>Ewing Sarcoma<\/span><\/u><\/b><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Image guided biopsy with IHC is recommended.<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Molecular studies are recommended as needed guided by expert opinion.<\/span><\/p>\n

    \u00a0<\/span><\/u><\/em><\/p>\n<\/td>\n

    		\n

    Conditional recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Contrast enhanced MRI of the primary site is recommended.<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    We recommend PET\/CT if available or CT chest and bone scan if PET\/CT is unavailable.<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Bone marrow biopsy is recommended if PET\/CT is unavailable or positive uptake of bone marrow in PET\/CT.<\/span><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    2-\u00a0First line therapy for non-metastatic primary tumour<\/u><\/em><\/u><\/span><\/b><\/p>\n

    (neoadjuvant\/adjuvant)<\/span><\/u><\/b><\/em><\/u><\/b><\/p>\n

    \u00a0<\/span><\/b><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Multiagent chemotherapy for at least 9 weeks prior to local therapy is recommended (interval compressed chemotherapy<\/u><\/b>).<\/span><\/p>\n

    \u00a0<\/span><\/p>\n

    All patients are recommended to continue adjuvant chemotherapy after local control till 28 weeks.<\/span><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n

    \u00a0<\/span><\/p>\n

    \u00a0<\/span><\/p>\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Preferred Regimen<\/span><\/u><\/em><\/u><\/p>\n

    VDC\/IE (Vincristine, doxorubicin and cyclophosphamide) alternating with (Ifosfamide and etoposide) every 2 weeks with GCSF for a total of 14 cycles.<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Restage after neoadjuvant therapy before local control<\/span><\/i><\/b><\/p>\n

    CT chest and contrast enhanced MRI of primary site are recommended.<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Local Control Therapy for stable\/improved disease following neoadjuvant therapy<\/span><\/b><\/em><\/b><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    We recommend wide surgical excision and adjuvant chemotherapy. Radiotherapy is recommended if positive surgical margins.<\/span><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Definitive radiotherapy and adjuvant chemotherapy are recommended for irresectable tumours.<\/span><\/p>\n

    \u00a0<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    3-\u00a0First line therapy for metastatic disease at initial presentation<\/em><\/span><\/u><\/b><\/p>\n

    \u00a0<\/span><\/u><\/b><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Multiagent chemotherapy for at least 9 weeks prior to local therapy is recommended (interval compressed chemotherapy<\/u><\/b>).<\/span><\/p>\n

    \u00a0<\/span><\/p>\n

    Preferred Regimen<\/span><\/u><\/em><\/u><\/p>\n

    VDC\/IE (Vincristine, doxorubicin and cyclophosphamide) alternating with (Ifosfamide and etoposide) every 2 weeks with GCSF for a total of 14 cycles.<\/span><\/p>\n

    \u00a0<\/span><\/p>\n

    All patients are recommended to continue adjuvant chemotherapy after local control till 28 weeks.<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n

    \u00a0<\/span><\/p>\n

    \u00a0<\/span><\/p>\n

    \u00a0<\/span><\/p>\n

    Strong recommendation<\/span><\/p>\n

    \u00a0<\/span><\/p>\n

    \u00a0<\/span><\/p>\n

    \u00a0<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Local control for metastatic disease<\/span><\/i><\/b><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    We recommend wide surgical excision and adjuvant chemotherapy. Radiotherapy is recommended if positive surgical margins.<\/span><\/p>\n

    \u00a0<\/span><\/s><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/s><\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Definitive radiotherapy and adjuvant chemotherapy are recommended for irresectable tumours.<\/span><\/p>\n

    \u00a0<\/span><\/s><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/s><\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Management of metastases<\/span><\/i><\/b><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    <\/a>For lung only metastases with partial response to neoadjuvant treatment, resection and whole lung irradiation<\/span>\u00a0are recommended.<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    For lung only metastases with complete response to neoadjuvant treatment, whole lung irradiation is recommended.<\/span><\/p>\n

    \u00a0<\/span><\/i><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    For bone metastases it is recommended to give radiotherapy to metastatic sites.<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    4- Radiotherapy<\/span><\/u><\/b><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Timing of RT<\/span><\/u><\/i><\/p>\n

    For patients receiving radiation therapy only it is recommended to be delivered at the beginning of week 13 concurrently with chemotherapy.<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    If post-operative radiotherapy is recommended, consider starting at week 15 concurrently with chemotherapy starting on day 1 of the cycle as soon as possible after surgery.<\/span><\/p>\n

    \u00a0<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Patients with recent cord compression are recommended to start emergency concurrent radiotherapy and chemotherapy starting from day 1 first cycle.<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Concurrent chemotherapeutic agents<\/span><\/u><\/i><\/p>\n

    Ifosfamide, etoposide, cyclophosphamide and vincristine should be given with radiotherapy. It is recommended to withhold doxorubicin with radiotherapy and re-institute after completion of radiation.<\/span><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    5- Treatment of recurrent\/relapsed Ewing Sarcoma<\/span><\/u><\/b><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Chemotherapy<\/span><\/u><\/i><\/p>\n

    Recommended chemotherapy combination<\/span><\/p>\n

    \u00b7\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0<\/span>Irinotecan and temozolomide\u00a0<\/span>in 21-day interval cycles, Or<\/span><\/p>\n

    \u00b7\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0<\/span>Ifosfamide, carboplatin and etoposide (if > 6 months).<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Surgery<\/span><\/u><\/i><\/p>\n

    Surgical resection of both local and metastatic sites (especially pulmonary) if feasible is recommended.<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    Radiotherapy<\/span><\/u><\/i><\/p>\n

    Radiation is recommended either definitive or postoperative.<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    6- Surveillance \u2013 Follow up – for Ewing Sarcoma patients<\/span><\/u><\/b><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    X-ray of the primary site is recommended every 4 months for the first 2 years and as clinically warranted.<\/span><\/p>\n

    \u00a0<\/span><\/i><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    CT chest every 4 months is the recommended chest imaging in the first 2 years. Chest X-ray is recommended for chest imaging in later years.<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n

    \n

    It is recommended to increase intervals of imaging of primary site and chest after 24 months and annually after 5 years (indefinitely).<\/span><\/p>\n

    \u00a0<\/span><\/p>\n<\/td>\n

    		\n

    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<\/div>\n<\/div> <\/div>\n\n <\/div>\n

    \n\n
    \n\n \n\n \n <\/path><\/svg> <\/span>\n\n \n <\/path><\/svg> <\/span>\n \n <\/span>\n \n \n Wilms\u2019 tumor <\/span>\n\n <\/h5>\n\n
    \n
    \n
    \n
    \n
    “last update: 12 March \u00a02025”\u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0\u00a0<\/span>Download Guideline<\/u><\/strong><\/a><\/h5>\n
    \u00a0<\/pre>\n<\/div>\n<\/div>\n<\/div>\n
    \n
    \n
    \n
    \u00a0<\/div>\n<\/div>\n<\/div>\n<\/div>\n
    \n
    – Executive Summary<\/h3>\n
    \n
    \u00a0<\/p>\n
    This guidance provides a data-supported approach to the diagnosis, risk stratification, treatment and follow up of paediatric patients diagnosed with Wilms\u2019 tumor.<\/span><\/p>\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n
    \n
    <\/a><\/a>Recommendations<\/span><\/b><\/p>\n<\/td>\n
    		\n
    Level Of Recommendations<\/span><\/b><\/p>\n<\/td>\n<\/tr>\n
    \n
    1<\/u><\/b>–<\/span><\/u><\/b>Work up for newly diagnosed Wilms\u2019 tumor<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend complete assessment for signs of associated syndromes including blood pressure measurement and urine analysis.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Contrast enhanced CT abdomen and pelvis or MRI is recommended (to assess bilaterality, evidence of tumor rupture and evidence of tumor thrombus extension into the renal vein or inferior vena cava, nephrogenic rests)<\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    CT chest is recommended to assess for metastasis.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    For stage IV WT, we recommend local staging to determine local therapy.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    2-Treatment of unilateral WT with no predisposition to develop bilateral WT<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend primary nephrectomy with regional LN sampling (5-10 nodes) for all patients, followed by adjuvant treatment according to stage and histology.<\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    If initially unresectable or resection is contraindicated, we recommend proceeding to chemotherapy without biopsy (either image guided core needle biopsy or open).<\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend either image guided core needle biopsies, or open biopsy to confirm WT pathology in the following conditions:<\/span><\/p>\n
    \u00b7\u00a0 \u00a0<\/span>Age < 1year or older than 10 years<\/span><\/p>\n
    \u00b7\u00a0 \u00a0<\/span>Uncertain renal origin<\/span><\/p>\n
    \u00b7\u00a0 \u00a0<\/span>Atypical metastases: bones (any age), central nervous system (any age), isolated pulmonary nodules < 2years.<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Elevated LDH > 3-4 folds<\/span><\/p>\n
    \u00b7\u00a0 \u00a0<\/span>Hypercalcaemia and age<4 years.<\/span><\/p>\n<\/td>\n
    		\n
    Strong recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Management of initially resected WT with no predisposition to develop bilateral WT<\/span><\/u><\/i><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend the following adjuvant treatment:<\/u><\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    1. Favorable histology WT:<\/span><\/b><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Regimen EE-4A for LR patients<\/span><\/p>\n
    \u00b7\u00a0<\/span>Regimen DD-4A for SR patients<\/span><\/p>\n
    \u00b7\u00a0 \u00a0<\/span>Regimen M for HR patients.<\/b><\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    2. Focal anaplastic WT:<\/span><\/b><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Stages I-III: Regimen DD4-A<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Stage IV: Regimen UH-HR<\/b><\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    3. Diffuse anaplastic WT:<\/span><\/b><\/p>\n
    \u00b7\u00a0 \u00a0<\/span>Stage I: Regimen DD4A.<\/span><\/p>\n
    \u00b7\u00a0 \u00a0<\/span>Stages III-IV: \u00a0Regimen UH-HR<\/b><\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Management of initially unresectable WT with no predisposition to develop bilateral WT<\/span><\/u><\/i><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend neoadjuvant treatment, regimen (DD-4A) for initially unresectable tumors.<\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend reassessment at week 6 by contrast enhanced CT chest, abdomen and pelvis.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend total nephrectomy and LN sampling at week 6, if feasible. If not feasible, we recommend continuing (DD-4A) till week 12, followed by reassessment and surgery.<\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend the following postoperative adjuvant treatment for initially unresectable WT:<\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    \u00b7\u00a0\u00a0<\/span>Continuing Regimen DD-4A for SR favorable histology WT, stages I-III focal anaplastic WT and stage I diffuse anaplastic WT.<\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    \u00b7\u00a0\u00a0<\/span>Switching to Regimen M for HR favorable histology WT.<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    \u00b7\u00a0\u00a0<\/span>Switching to Regimen I for blastemal predominant histology.<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    \u00b7\u00a0\u00a0<\/span>Switching to Regimen UH-HR for stage IV focal anaplastic WT and stages II-IV diffuse anaplastic WT.<\/span><\/p>\n<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n
    \u00a0<\/span><\/p>\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n
    \n
    <\/a>3. Treatment of bilateral WT and unilateral WT with predisposition to develop bilateral WT:<\/span><\/u><\/b><\/u><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We do not recommend upfront nephrectomy either partial or radical.<\/span><\/p>\n
    \u00a0<\/span><\/b><\/p>\n<\/td>\n
    		\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We do not recommend upfront biopsy (either needle or open).\u00a0<\/span>\u00a0<\/span>If biopsied, a tumor is stage III for determination of chemotherapy regimen, but biopsy alone does not upstage a tumor to stage III for determining whether to give radiation.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend neoadjuvant treatment (VAD) for 6 weeks.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend reassessment with contrast enhanced CT chest, abdomen and pelvis at week (6) VAD.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend bilateral partial nephrectomy (one or both sides) at week 6, if feasible.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    If bilateral partial nephrectomy at week 6 is not feasible, assess for response:<\/span><\/u><\/b><\/p>\n
    \u00b7\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0<\/span>Partial response in both kidneys, we recommend continuing VAD regimen till week 12 then reassess for bilateral partial nephrectomy or total nephrectomy with LN sampling followed by adjuvant treatment based on higher risk histology.<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Less than partial response (<50% reduction of tumor size) in either kidney, we recommend either immediate surgery or bilateral open biopsies followed by adjuvant treatment based on higher risk histology, reimage at week 12 for definitive surgery.<\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend the following adjuvant treatment after surgery or biopsy in bilateral WT determined by the highest assigned stage\/histology of either kidney:<\/span><\/u><\/b><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    EE-4A<\/span><\/u>\u00a0regimen is recommended for<\/span><\/p>\n
    \u00b7\u00a0 \u00a0 \u00a0<\/span>Stage I – CR\u00a0<\/span>with<\/span>\u00a0no lesion detectable on imaging after preoperative chemotherapy at week 6, or<\/span><\/p>\n
    \u00b7\u00a0 \u00a0<\/span>Stage I-II favorable histology WT after complete resection or completely necrotic.<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    DD-4A regimen<\/span><\/u>\u00a0is recommended for<\/span><\/p>\n
    \u00b7\u00a0<\/span>Stage III-IV favorable histology WT, or<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Stage III-IV completely necrotic, or<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Stage I favorable histology WT with blastemal predominant histology, or<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Stage I-III focal anaplastic WT, or<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Stage I diffuse anaplastic WT<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Regimen I<\/span><\/u>\u00a0is recommended for<\/span><\/p>\n
    \u00b7\u00a0<\/span>Stage II-IV favorable histology WT with blastemal predominant histology.<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Regimen UH<\/span><\/u>\u00a0(start week 1) is recommended for<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Stage IV focal anaplastic WT, or<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Stages II-IV diffuse anaplastic WT<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    4. Management of extrapulmonary metastasis stage IV WT<\/span><\/u><\/b><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    \u00b7\u00a0\u00a0<\/span>We recommend upgrading to regimen M in stage IV favorable histology WT and irradiation to all metastatic sites post nephrectomy.<\/span><\/p>\n<\/td>\n
    		\n
    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    \u00b7\u00a0\u00a0We do not recommend liver irradiation in the following condition only:<\/p>\n
    Solitary liver metastasis, at presentation (before chemotherapy) completely resected and negative margins with nephrectomy.<\/p>\n<\/td>\n
    		\n
    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    \u00b7\u00a0\u00a0We recommend regimen UH for stage IV anaplastic histology (focal or diffuse) and irradiation to all metastatic sites<\/p>\n<\/td>\n
    		\n
    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    5. Radiotherapy (RT):<\/span><\/u><\/b><\/u><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    <\/a>Timing of RT<\/span><\/u><\/i><\/p>\n
    Post nephrectomy (either upfront or delayed), RT should begin close to chemotherapy, preferably by Day 10 (surgery is Day 0), but no later than Day 14.<\/span><\/u><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Flank RT\u00a0(10.8 Gy at 1.8 Gy per fraction)<\/u>\u00a0is recommended in unilateral WT, bilateral WT and unilateral WT with predisposition to develop WT under the following conditions:<\/span><\/p>\n
    \u00b7\u00a0<\/span>Locally stage III favorable histology WT.<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Locally stage I-III focal anaplasia<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Locally stage I-II diffuse anaplasia<\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Flank RT\u00a0(19.8 Gy at 1.8 Gy per fraction)<\/u>\u00a0is recommended in unilateral WT, bilateral WT and unilateral WT with predisposition to develop WT under the following conditions:<\/span><\/p>\n
    \u00b7\u00a0<\/span>Locally stage III diffuse anaplastic histology WT.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    For bilateral WT and unilateral WT with predisposition to develop WT, we do not recommend flank RT in these conditions:<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Biopsy alone, however reported surgical tumor spill will require RT.<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Complete resection with negative surgical margins\/nodes.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Whole abdomen RT\u00a0(10.5 Gy at 1.5 Gy per fraction)\u00a0<\/u>is recommended in all patients with:<\/span><\/p>\n
    \u00b7\u00a0<\/span>Cytology positive ascites<\/span><\/p>\n
    \u00b7\u00a0<\/span>Preoperative rupture<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Diffuse abdominal surgical spillage (reported by surgeon)<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Peritoneal seeding, in case of diffuse peritoneal implants (21Gy in 1.5 fractions)<\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    WLI is not recommended in unilateral favorable histology WT with pulmonary metastasis showing RCR post week 6<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    WLI (at week 7)\u00a0(12 Gy at 1.5 Gy per fraction or 10.5 Gy at 1.5 Gy per fraction if <12 months)<\/u>\u00a0is recommended in patients with:<\/span><\/p>\n
    \u00b7\u00a0 \u00a0<\/span>Unilateral favorable histology WT with pulmonary metastasis showing slow incomplete response at week 6 (with or without surgical excision of residual metastases).<\/span><\/p>\n
    \u00b7\u00a0 \u00a0<\/span>Metastatic bilateral and unilateral WT with predisposition to develop bilateral WT.<\/span><\/p>\n
    \u00b7\u00a0 \u00a0<\/span>Pulmonary metastasis and other extra-thoracic metastases (such as liver, bone, or brain).<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>LN metastases in the hilum and\/or mediastinum, or cytology-positive pleural effusion regardless of response to chemotherapy.<\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend dactinomycin and doxorubicin reduction by 50% during or within 6 weeks of completing a course of whole lung or abdominal RT.<\/span><\/p>\n<\/td>\n
    		\n
    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    <\/a>6-Treatment of relapse or refractory disease<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend complete evaluation for both local and metastatic sites at relapse with contrast enhanced CT chest, abdomen and pelvis<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend biopsy from site of recurrence to confirm WT relapse.<\/span><\/p>\n<\/td>\n
    		\n
    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Standard risk<\/span><\/u><\/i><\/b><\/u><\/i><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend 2nd<\/sup>\u00a0line chemotherapy as first treatment using four drugs (combinations of alternating courses of doxorubicin and cyclophosphamide and carboplatin and etoposide)<\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend surgical resection with clear resection margins if feasible<\/span><\/p>\n<\/td>\n
    		\n
    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend flank RT or whole abdomen irradiation in case of peritoneal extension or ascites.<\/span><\/p>\n<\/td>\n
    		\n
    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    High and higher risk:<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend combination chemotherapy (ICE\/CCE) alternating with topotecan\/cyclophosphamide up to 10 cycles if feasible<\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend surgical resection and consolidation with RT if feasible for local and metastatic sites.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Autologous bone marrow transplantation is recommended for patients with chemo-sensitive relapse who are not candidates for RT consolidation.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    7- End of treatment evaluation<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend end of treatment evaluation by contrast enhanced CT chest, abdomen and pelvis to confirm CR before starting follow up.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendations<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    8- Surveillance (follow up after end of treatment)<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend clinical examination together with chest and abdominal imaging every 3 months for 2 years, then every 6 months for 5 years.<\/u><\/span><\/p>\n
    (Chest x-ray and abdominal US may be used in place of cross-sectional imaging with CT chest and abdomen with IV contrast or MRI).<\/span><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    9- Screening recommendations predisposed patients to develop bilateral WT<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend renal US every 3 months until 7 years (ie, all of year 6).<\/span><\/p>\n<\/td>\n
    		\n
    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<\/div>\n<\/div>                    <\/div>\n\n                <\/div>\n                            
    \n\n                    
    \n\n                                                    \n\n                                \n                                    <\/path><\/svg>                                <\/span>\n\n                                                                    \n                                        <\/path><\/svg>                                    <\/span>\n                                \n                            <\/span>\n                        \n                                                \n                                                        Retinoblastoma                        <\/span>\n\n                    <\/h5>\n\n                    
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    \u00a0<\/div>\n<\/div>\n<\/div>\n<\/div>\n
    \n
    – Executive Summary<\/h3>\n
    \n
    \u00a0<\/p>\n
    This guidance provides a data-supported approach to the diagnosis, treatment and follow up of paediatric patients diagnosed with retinoblastoma.<\/span><\/p>\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n
    \n
    <\/a>Recommendations<\/span><\/b><\/p>\n<\/td>\n
    		\n
    Level Of recommendation<\/span><\/b><\/p>\n<\/td>\n<\/tr>\n
    \n
    1<\/u><\/b>–<\/span><\/u><\/b>Work up for newly diagnosed retinoblastoma<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend bilateral examination under anaesthesia and full ophthalmology assessment for all patients suspected to have retinoblastoma.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend MRI of brain and orbits with IV contrast prior to therapy in all patients.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    For all tumors stage II and above – according to IRSS- \u00a0we recommend metastatic work up: Bilateral bone marrow biopsies, bone scan, CSF cytology and whole spine MRI<\/span><\/p>\n<\/td>\n
    		\n
    Strong recommendation<\/p>\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Pathology is not recommended for initial confirmation of diagnosis prior to therapy.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend family counseling for all children with RB.<\/p>\n<\/td>\n
    		\n
    Strong recommendation<\/p>\n<\/td>\n<\/tr>\n
    \n
    2- Treatment of Unilateral Intraocular Retinoblastoma<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Surgery \u00a0<\/span><\/u><\/b><\/p>\n
    We recommend enucleation for:<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>IIRC Group E<\/span><\/p>\n
    \u00b7\u00a0\u00a0<\/span>Failed\u00a0<\/span>eye\u00a0salvage with local treatment and systemic chemotherapy in groups C and D<\/span><\/p>\n
    \u00b7\u00a0<\/span>Unilateral advanced intraocular group D disease with no hope of useful vision.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Optic nerve resection margin is recommended to be at least 10 mm<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Histopathological assessment and staging of<\/span>\u00a0the\u00a0enucleated eye is recommended for all patients.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    <\/a>Treatment of Group A<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend local treatment for all group A eyes.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Treatment of Group B<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend both local treatment and systemic\u00a0<\/span>IV three drug chemotherapy (Vincristine-Carboplatin-Etoposide) for a total of 6 cycles with 21-28 days interval in between cycles.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Treatment of Groups C and D\u00a0<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend both local treatment and systemic\u00a0<\/span>IV three drug chemotherapy (Vincristine-Carboplatin-Etoposide) for a total of 6 cycles with 21-28 days interval in between cycles.\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend routine EUA with every\u00a0<\/span>1 cycle\u00a0and if progressive disease we recommend enucleation and histopathology examination.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    If NO high risk features in pathology, we do not recommend further treatment after enucleation.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    If\u00a0 high risk\u00a0<\/span>features are present in pathology, we recommend giving total 6 cycles of systemic\u00a0<\/span>IV three drug chemotherapy (Vincristine-Carboplatin-Etoposide) with 21-28 days interval in between cycles.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend starting systemic chemotherapy whithin 35 days of enucleation for high risk\u00a0<\/span>features.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Treatment of Group E<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend enucleation upfront for all group E eyes<\/p>\n<\/td>\n
    		\n
    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    If NO high risk features in pathology, we do not recommend further treatment after enucleation<\/span><\/p>\n<\/td>\n
    		\n
    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    If\u00a0 high risk\u00a0<\/span>features are present in pathology, we recommend giving total 6 cycles of systemic\u00a0<\/span>IV three drug chemotherapy (Vincristine-Carboplatin-Etoposide) with 21-28 days interval in between cycles.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend starting systemic chemotherapy whithin 35 days of enucleation for high risk histology.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    3-Treatment of Bilateral Intraocular Retinoblastoma<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend dictating treatment protocol by the most advanced eye.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend upfront enucleation of the most advanced eye followed by histopathology. If the better eye is group A, B or C, we recommend enucleation of group D eyes.<\/span><\/p>\n
    If both eyes are group D we recommend chemoreduction and focal therapy for both eyes.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend chemoreduction for groups B, C and D eyes as well as local therapy similar to unilateral intraocular treatment protocols.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    4- Treatment of Extraocular Retinoblastoma \u00a0<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend systemic chemotherapy followed by radiation therapy for IRSS stages II-III orbital and regional lymph node involvement.<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    For stage IV metastatic eyes, we recommend systemic chemotherapy, followed by\u00a0<\/span>high dose chemotherapy and stem cell rescue.<\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    Recommended systemic chemotherapy regimen includes 4 cycles of 4 drug regimen (VCR-CDDP-CPM-ETOP)<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    5- Surveillance<\/span><\/u><\/b><\/p>\n<\/td>\n
    		\n
    \u00a0<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend EUA every 3<\/span>-4\u00a0weeks\u00a0<\/span>for patients receiving active anti-tumor treatment till\u00a0the end of therapy<\/span>.<\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend 1-2 months EUA starting at the end of therapy and continuing for 24 months thereafter for patients who present with unilateral disease and are younger than 2 years of age at presentation or bilateral disease of any age.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend life long follow up and counseling for bilateral RB patients and patients with positive family history of RB as well as sibling screening.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    For sibling screening we recommend EUA at intervals:<\/p>\n
    \u2022\u00a0\u00a0Every 2 weeks since birth till 8 weeks of age,<\/p>\n
    \u2022\u00a0\u00a0then monthly till 1 year of age,<\/p>\n
    \u2022\u00a0\u00a0then every 3 months till 3 years of age,<\/p>\n
    \u2022\u00a0\u00a0then every 6 months till 7 years of age<\/p>\n<\/td>\n
    		\n
    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    For unilateral retinoblastoma patients who are older than 2 years of age we recommend extending ophthalmology examination intervals post therapy, reaching 6 monthly by age of 5 and annually thereafter.<\/span><\/p>\n<\/td>\n
    		\n
    Strong recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n
    \n
    We recommend that RB survivors treated with chemotherapy or EBRT , follow up at oncology clinic every 3 months then for longer intervals as clinically warranted.<\/span><\/p>\n<\/td>\n
    		\n
    Strong Recommendation<\/span><\/p>\n<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<\/div>\n<\/div>                    <\/div>\n\n                <\/div>\n                    <\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t","protected":false},"excerpt":{"rendered":"
    \u0627\u0648\u0631\u0627\u0645 \u0623\u0637\u0641\u0627\u0644 Paediatric Burkitt lymphoma “last update: 14 Oct 2024”\u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 […]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-7473","page","type-page","status-publish","hentry"],"aioseo_notices":[],"_links":{"self":[{"href":"https:\/\/gothi.gov.eg\/index.php?rest_route=\/wp\/v2\/pages\/7473","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/gothi.gov.eg\/index.php?rest_route=\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/gothi.gov.eg\/index.php?rest_route=\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/gothi.gov.eg\/index.php?rest_route=\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/gothi.gov.eg\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=7473"}],"version-history":[{"count":4,"href":"https:\/\/gothi.gov.eg\/index.php?rest_route=\/wp\/v2\/pages\/7473\/revisions"}],"predecessor-version":[{"id":7480,"href":"https:\/\/gothi.gov.eg\/index.php?rest_route=\/wp\/v2\/pages\/7473\/revisions\/7480"}],"wp:attachment":[{"href":"https:\/\/gothi.gov.eg\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=7473"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}