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POEM Group Online Case Discussion
CD 001 Mahdi H.
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Case # 1: M.H. Referred by: Dr. Mazin Aljadiry, Children Welfare Teaching Hospital Patient Initials: M. H. Nationality: Iraqi Gender: M DOB: Age: 5years and 6 months Diagnosis: Ewing sarcoma Date of Diagnosis: First Consultation
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Briefing Swelling in the back, incisional biopsy showed Ewing sarcoma of 10th rib, staged as II, received chemotherapy and sent to Turkey where he underwent another local surgical intervention and the biopsy revealed Ewing sarcoma with free margins, his PET/CT scan was normal. They suggested to have more aggressive chemotherapy protocol and no need for radiotherapy.
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Disease History Upper back swelling of 5-month duration noticed accidently during bathing by the mother, the family neglected the condition for about two months, when the swelling became larger the family sought medical advice, for which incisional biopsy was taken in March 2012 The diagnosis was Ewing Sarcoma, he was referred to CWTH/oncology unit on 10-April-2012. O/E: there is a transverse incisional scar at the back to the Rt. side 8cm. chest wall U/S: well defined irregular outline solid heterogeneous mass 33x15mm is seen in the Rt. lower intercostal space
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Initial Imaging N.A April chest CT scan (post operation): clear both lungs field, osteolytic lesion at posterior part of Rt. 10th rib, no soft tissue mass. Bone scan??
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Disease History th rib mass biopsy (143): Gross: single piece of tissue 33x17x12 mm ill-defined congested tumor involving the medulla and cortex of rib and soft tissue around the bone, all taken. Histology: Ewing's sarcoma stage IA, IHC study indicated to confirm the diagnosis. back mass biopsy (143) slide review: fragment tissue including skeletal muscles and bone trabeculae densely and diffusely infiltrated by sheets of malignant small round blue cells with high mitotic figures, haemorrhage, multifocal necrosis, picture consistent with Ewing's sarcoma/PNET. BMA and biopsy: cellular marrow, no evidence of neoplastic process.
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Treatment Protocol He received 1st VIDE of EURO-EWING 99 protocol. received 2nd VIDE. post 2nd VIDE: O/E: subcutaneous mass about 2x2cm movable Rt. posterior chest wall Abd U/S: normal, chest wall U/S: 19x7mm (smaller than in previous U/S) heterogeneous hypoechoic mass with sinus tract to the skin located subcutaneously.. received 3rd VIDE. received 6th VIDE. received 1st VAI (consolidation phase).
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Follow-up Imaging chest CT scan: the remnant of 10th rib looks deformed and expanded, no focal SOL obviously detected.
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Treatment Protocol Radiotherapy was not possible in our country, so he continued receiving chemotherapy. received 2nd VAI. chest CT scan: evidence of old united fracture at Rt. 10th rib with thickening and structural changes, clear both lungs field. received 3rd VAI. received 4th VAI.
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Follow-up imaging chest CT scan: evidence of old united fracture at Rt. 10th rib with thickening and structural changes, clear both lungs field.
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Treatment Protocol Rome Histo-pathological evaluation on 16/11/2012:Muscle and bone fragments infiltrated by aggregates of densely packed round cells with uniform round to oval nucleus, finely dispersed granular chromatin and scanty cytoplasm. The neoplastic cells markedly express CD99 at cell membrane level and NSE. The histologic and IHC features suggest a diagnosis of Ewing sarcoma/peripheral neuroectodermal tumor (EWS/PNET). , received 5th VAI , received 6th VAI , received 7th VAI , finished the 8th VAI
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Treatment Protocol Then he was kept on regular follow-up at outpatient clinic. In July 2013, he was sent by the government to ACIBADEM center in Turkey, where PET-CT done and it was free of disease. In Turkey he underwent surgery as local treatment on ; The pathology was consistent with Ewing Sarcoma/ PNET, Surgical margins were negative, There was no necrosis observed in the specimen, so local radiotherapy was not done and referred back again to us to receive chemotherapy as they said it seems it wasn’t enough U/S of right chest wall normal. admitted to receive another VAI while waiting the result of teleconsultation.
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Questions Was the staging appropriate? Did he receive proper protocol?
What is the suggested further step in the management?
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POEM Group Online Case Discussion
CD 002 Ameer A.
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Case # 2: A.A. Referred by: Dr. Mazin Aljadiry, Children Welfare Teaching Hospital Patient Name: A. A. Nationality: Iraqi Gender: M DOB: 7-April-1999 Age: 14 years and 4 months Diagnosis: Hodgkin’s Lymphoma Date of Diagnosis: First Consultation
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Disease History He presented to oncology unit in June 2011 with history of left axillary swelling for the last 6 months, discovered suddenly by his family, increased in size gradually then got swelling of the left side of the chest, no fever, and no night sweat.
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Imaging 2/June/2011: left axilla and anterior chest wall U/S: well defined demarcated multiple matted hypoechoic masses, big one 34x44mm at anterolateral aspect of chest wall associated with central necrotizing like tissue content at subcutaneous in region and some deeply extended, no calcification (necrotizing lymphadenitis). 4/June/2011 Chest CT: multiple hypodense masses of the Lt axilla and chest wall, a big one 4*4 cm at antero lateral chest wall, partial enhancement with IVC, no bony lesion, normal lung and mediastinum.
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Disease History 5/June/2011 FNA from left axillary mass: cellular smear show mainly difuse monomorphic lymphocyte, some of them immature cell together with highly atypical large cell, picture highly suggestive of lymphoid neoplasia, biopsy is indicated. 16/June/2011 axillary lymph node biopsy : Gross: a nodule piece of tissue, matted together, the largest measure 3x2cm, the smaller 0.5cm homogenous firm cut section. Microscopy: three out of four axillary lymph nodes show hodgkin’s lymphoma, nodular lymphocytic rich type with numerous reed Sternberg cell. 19/6/2011…….. CXR…N.
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Disease History 23/6/2011 Slide review: totally effaced architecture by nodular pattern of growth composed of lymphocyte with large number of (R-S) cell with folded, multi lobed nucleus, prominent nucleus, prominent post capillary venules.--- Hodgkin’s lymphoma of nodular lymphcytic predominant type. 20/6/2011 s.LDH 230, Hb 12.6g/dl, WBC 6,100/cmm, Platelet 84,000/cmm, s.ferritin 22.6ng/ml, Blood urea 46, s.creatinine 0.5, TSB 0.1, S.Al phos 20, SGOT 36 23/6/2011 abdominal U/S: right mesenteric lymphadenopathy, the largest 1.7cm in length.
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Disease History 30/6/2011: started on COPP/ABV first cycle 100%
Chest wall U/S: left axillary multiple different sized heterogenous soft tissue measuring the largest 5.3cm size, it extended to the left upper chest wall. 29/6/2011 Hb 12.7g/dl, WBC 5,100/cmm, Platelet245,000/cmm. N.68% L.23% 3/7/2011 BMA; cellular marrow, no evidence of neoplastic process could be obtained from the material while BMB: no marrow tissue is seen in the available material. He was Staged as IIIB 30/6/2011: started on COPP/ABV first cycle 100%
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Follow-up imaging 26/7/2011: chest CT: multiple enlarged lymph nodes in the left axilla and left anterior chest wall with enlarged lymph nodes in the anterior mediastinum. In comparison with this previous study; there is good reduction in the size but still they are of remarkable size.
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Follow-up imaging 29/11/2011: he was due for the 2nd block of course no. 6, his CT chest showed; multiple left axillary lymph node enlargement, clear lungs, normal heart, no mediastinal lymph node enlargement, no pleural effusion and no bony infiltration.
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Treatment Protocol 6/12/2011 he finished his treatment (COPP/ABV x 6 doses) 18/12/2011 axillary lymph nodes: left sided 2.4x1.8cm size hypo-echoic mass detected while CXR and abdominal U/S were normal, he was kept under observation. 13/3/2012 He went to Turkey for PET scan and further management, PET showed no hypermetabolic activity in his bilateral cervical, axillary and mediastinal regions but a couple of hypermetabolic lymph nodes are seen on the portal and epigastric area (biggest is 1.4cm), accordingly laparoscopy was done and lymph node biopsy showed reactive process (in Turkey).
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Treatment Protocol He was kept under observation for about 17 months with the same clinical and axillary ultrasound finding. 15/8/2013 he has non-specific complaint, his father is worried about the persistent lymph nodes, he has palpable left axillary lymph nodes of around 2cm. his neck, chest and abdominal U/S were normal but axillary U/S showed evidence of bilateral enlarged lymph nodes seen in the axillary area, largest node in the left side measures 24x10mm, while largest one on the right side 19x8mm, all are oval in shape with detected hilum. His ESR 30mm/hr, s.LDH 213 U/L, s.copper 70micro/dl (normal).
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Follow-up imaging 22/8/2013 CT scan of neck, chest and abdomen: enlarged lymph node in the axilla, para-aortic and inguinal region with prominent thymic gland, largest axillary node measured 2.3cm.
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Questions Do we need to do lymph node biopsy or PET scan? His lymph nodes didn't disappear nor increased in size. Was stage III over staging without doing CT abdomen?
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