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POEM Group Online Case Discussion

POEM Group Online Case Discussion. Date: January 7, 2013. Case # 1: CD009 (AAK). Case # 1: CD009. Referred by Physician: Oncology Team Referring Hospital:CWTH Patient Initials : A A K Nationality:Iraqi Gender: Male DOB:17 Feb. 2004 Age: 9y & 10 m Diagnosis: Hyper-eosinophilia

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POEM Group Online Case Discussion

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  1. POEM Group Online Case Discussion Date: January 7, 2013

  2. Case # 1: CD009 (AAK)

  3. Case # 1: CD009 • Referred by Physician: Oncology Team • Referring Hospital:CWTH • Patient Initials: A A K • Nationality:Iraqi • Gender: Male • DOB:17 Feb. 2004 Age: 9y & 10 m • Diagnosis: Hyper-eosinophilia • Date of Diagnosis: 20 June 2013 • First Consultation

  4. Briefing • Hypereosinophilia (fluctuating levels) • Hx of allergic rhinitis

  5. Disease History • A known case of allergic rhinitis and bronchitis • Presented to outpatient clinic on 20/6/2011 with pallor and a complaints of itching at night. • No other symptoms.

  6. Disease History Investigation: • 20/6/2011 • Hb 10.3g/dl, WBC 41,300/cmm (N.2%, L.15%, Eosinophils 82% ) • BUN28mg/dl, Cr0.6mg/dl, uric acid2.4, TSB 0.2mg/dl, SGOT 7. • HBs Ag negative, Anti HCV negative. • Smear : normochromic with slight anisopoikilocytosis. • CXR: normal • 21/6/2011 • BMA: • Megakaryocyte in good number • Granulopoisis active with maturation to segmented cells • Evident of prominence of eosinophilic stages which is of normal morphology, blast <1%---marrow eosinophilia.

  7. Disease History • Received Zentel (levamizol) 400mg and loratidine 5mg daily. • 7/7/2011 • CT chest: normal • Echocardio: normal • 9/7/2011 • FISH study for Philadelphia chromosome was undetectable • Hb 10.9g/dl, WBC 7,300/cmm (N.14%, L.56%, E 26%), ESR 33.

  8. Disease History • 13/7/2011 • Igq normal • IgE not done?? • 18/7/2011 • Hb 12.1g/dl, WBC 8,000/cmm (N. 47%, L.33%, E.16%), no complaints • 5/6/2012 • Normal examination except for signs of allergic rhinitis. • IgE 524 KUI/L (normal <150 KHI/L). • He lost follow up till 6/10/2013 • Hb 10 g/dl, WBC 17,800(N. 24%, L.18%, E.52%), Plt. 265, normal blood film.

  9. Disease History • Then he went to Iran in October 2013 • Investigations: • Hb 10.9 g/dl, WBC 7,300/cmm (N. 14%, L.56%, E.26%) • B.film: normochromic, microcytic, anisopoikilocytosis, many oval cell seen. • ANA---negative • IgA 128mg/dl (36-165), IgG 915mg/dl (650-1400), IgM 112mg/dl (40-230), IgE 452 (<39) • C3 133mg/dl (90-180), C4 20mg/dl (10-40) • C-ANCA negative. GUE: normal, GSE: normal. • He was advised to get Gleevec 200mg/day

  10. Disease History • 29-12-2013 seen again in outpatient clinic and was kept without treatment as he was asymptomatic

  11. Treatment Protocol • None except for levamizol

  12. Questions 1. Does he need further evaluation? 2. Any suggested management? 3. Does Imatinib has any role? 4. What is the prognosis?

  13. Case # 2: CD010 (NFW)

  14. Case # 2: CD010 • Referred by Physician: Dr. Mazin al jadiry • Referring Hospital: CWTH • Patient Initials: N.F.W. • Nationality: Iraqi • Gender: female • DOB: 23/12/2002 Age: 11 years • Diagnosis: Essential thrombocythemia • Date of Diagnosis: 1/9/2013 • First Consultation

  15. Briefing • Frequent attacks of abdominal pain and headache • Thrombocythemia. • JAK 2.... positive • Not responding to Hydroxyuria then good response to alpha interferon

  16. Disease History • Recurrent attacks of severe abdominal pain (stabbing in nature), associated with headache and dyspnea of 9 month duration • Hx of duodenal ulcer not responding to treatment • Hx of N/V, in the last 3 months (sometimes mixed with blood) along with abdominal pain and headache • OGD refused by the family. • P/E is irrelevant

  17. Disease History • Investigations: • 1/9/2013 • Hb 11.6g/dl, WBC 10.4 x10^9/L (N 61%, L31%, M8%), platelets 1508x10^9/L. • U/S abd.... mild splenomegaly. • 3/9/2013 • platelets 1612x10^9/L. • 12/10/2013 JAK 2.... positive.

  18. Disease History • 14/10/2013 • Platelets 1489x10^9/L. • FISH for Philadelphia chromosome.... negative. • 20/10/2013 • Platelets 1578x10^9/L.

  19. Disease History • 22/10/2013 BMA: • megakaryocytes are hyper-plastic with frequent giant hyperploidy megakaryocytes • erythropoeisis and granulopoiesis are normal • blasts <1%, overall findings shows the possibility of Essential Thrombocythemia, familial thrombocytosis. • 23/10/2013, BMB: • Hypercellular marrow tissue, with increase number of megakaryocytes, some large megakaryocytes are seen.

  20. Treatment: • 15/10/2013 • Hydroxyurea, a dose of 15mg/kg for about one month. • 17/11/2013 • Hb 12g/dl, WBCc 11.6 x10^9/L, platelets 1151x10^9/L • Dose of hydroxyurea was increased to 30mg/kg for about three weeks.

  21. Disease History • 1/12/2013 platelets 831x10^9/L. • 15/12/2013 she was presented with history of headache and abdominal pain, she was admitted to hospital. • 17/12/2013 Doppler U/S, MRA- MRV....normal. • CBC for the mother (to exclude any familial cause)..... normal. • started on pegelated alfa interferon a dose of 3mg/kg, weekly. • 26/12/2013 platelets 545x10^9/L.

  22. Treatment Protocol • 15/10/2013, Hydroxyurea, (15mg/kg) for about one month. • 17/12/2013 Pegelated alfa interferon a dose of 3mg/kg, weekly.

  23. Questions 1.Any treatment guideline for the control and maintenance of normal platelets 2. what is the prognosis?

  24. Case # 3: CD011 (OHH)

  25. Case # 3: CD011 • Referred by Physician: Oncology team • Referring Hospital: CWTH • Patient Initials:OHH • Nationality:Iraqi • Gender: female • DOB: 1 July 2006 Age: 7 years & 5 months • Diagnosis: Wilms tumor • Date of Diagnosis: 25 June 2013 • First Consultation

  26. Briefing • Wilms tumor • Bilateral Foot drop post 8 doses of VCR.

  27. Disease History • Right sided abdominal mass • U/S. Rt. kidney markedly enlarged in size with very large upper pole mass 11 cm diameter, renal vein not visualized but IVC patent. • CT abdomen. large right renal mass (nephroblastoma), normal other organs, patent main renal vein and IVC. • Underwent radical right nephrectomy

  28. Pathology • WILMS TUMOUR with no capsular invasion. • Renal pelvis, renal vein and ureter are tumor free.....

  29. Disease History • Past medical history: • At age of 4 years, she developed tonic clonic seizure (brain MRI done at that time.... SOL? ischemia?), • One month later MRI brain repeated and was normal. negative family history.

  30. Disease History • Microphthalmia, soft abdomen, with an oblique scar from right flank to the right upper hypochondrial region about 15cm in length. • Growth parameter: Wt: 25 kg and Ht.:124 cm (both at 75th percentile), OFC 51cm. • Blood pressure at time of admission 100/70 mm-Hg.

  31. Disease History • 7/7/2013 • CT chest: single small nodular opacity 5x6 mm in the posterior-lateral periphery of right lung • CT abdomen..... normal • 8/7/2013 • slide review: Wilms Tumor, FH, show blastema and primitive tubules.

  32. Treatment Protocol • 11/7/2013, started on SIOP WT 2001, IR STAGE III, with 3 drug regimen ( VCR, adriamycin, Actinomycin). • 15/9/2013 (8th week), she became unable to walk; the reflexes were normal, hypotonia, power grade 3 (peripheral neuropathy) • VCR was stopped.

  33. Treatment Protocol • EMG & NCS.... sensory motor poly-radiculoneuropathy. • VCR was omitted (as she still has bilateral foot drop), only receiving actinomycin and adriamycin according to the protocol.

  34. Questions 1.What is the next step in management of VCR side effects 2. Any alternative to vincristine? 3. Is there any role for radiotherapy in this case?

  35. Follow Up on Previously Discussed Cases

  36. Case#1: CD001 M.H • Presented on: 3/9/2013 • Swelling in the back, incisional biopsy showed Ewing sarcoma of 10th rib, received chemotherapy • sent to Turkey for another local control that showed Ewing sarcoma with free margins • PET/CT scan was normal. • More aggressive chemotherapy was recommended and no need for radiotherapy.

  37. Case#1: CD001 M.H • Questions: • Was the staging appropriate? • Did he receive proper protocol? • What is the suggested further step in the management? • Answers after Group Discussion: • Needs radiotherapy. • No more chemotherapy.

  38. Case#1: CD001 M.H • Case Update: • 10-10-2013---superficial U/S evidence of well hypo echoic nodule within the surgical scar at posterior chest wall region about 7*6.5 mm. • 7-11-2013--- 8 mm hypoechoic area at surgical scar. • 5-12-2013---8*5 mm at surgical site. • Still waiting for radiotherapy to be sponsored by Iraqi MOH

  39. Case#2:CD002 A.A • Hodgkin’s lymphoma (Left axillary), nodular lymphocytic rich type • Started COPP/ABV first cycle 100%, • 6/12/2011 he finished his treatment. • Has persistent axillary lymphadenopathy

  40. Case#2:CD002 A.A • 13/3/2012 did PET (Turkey) hypermetabolic lymph nodes are seen only in the portal and epigastric area (biggest is 1.4cm), • Laparoscopy was done and lymph node biopsy showed reactive process. • He was kept under observation for about 17 months with the same clinical and axillary ultrasound finding.

  41. Case#2:CD002 A.A • 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 • Answers after Group Discussion: • Observe and follow up with PET scan in the meantime

  42. Case#2:CD002 A.A • Case Update: • 19/12/2013 • 23 month off treatment, no complaint , normal examination. • CXR…normal • U/S… left side (axillary) 23mm soft tissue mass L.N?? Associated with multiple size each 3mm lymph nodes and < 8mm right cervical lymph node , not significant. • Waiting PET to be sponsored by MOH.

  43. Case#3: CD004 ASA • Presented on: 1/10/2013 • Brief History: • Abdominal masses retroperitoneal, with multiple peritoneal L.N 2ndary deposit • 19-June-2013 Mass biopsy C/W undifferentiated Neuroblastoma. Startded on NB protocol • IHC results on 10-7-2013 PPNET. • 24-7-2013 shifted to Euro Ewing protocol then Rome review on 17-9-2013: C/W desmoplastic small round cell tumor (DSRCT).

  44. Case#3: CD004 ASA • Questions: • What is the next step in the management of DSRCT? • Answers after Group Discussion: • This is a high risk patient and chances of cure are extremely low. • Options include aggressive P6 protocol, followed by surgery, high-dose chemo with SCT, and radiotherapy. • A third option is palliation and focus on quality of life. • Another option is to continue as per Ewing protocol and give radiotherapy at week 12.

  45. Case#3: CD004 ASA • Case Update: • We continued as Ewing protocol and on 3/12/2013 he received 6th VIDE, Still waiting for his evaluation in the next visit.

  46. Case#4: CD003 AAA • Presented on: 1/10/2013 • Brief History: • small buccal mass discovered at one year of age totaly excised • Pathology showed Embryonal RMS. • After 5 wks on RMS protocol, Rome 2nd opinion on 30-8-2013 showed Triton tumor. • So small tumor < 5cm, completely excised, no L.N. involvement and no residual tumor after surgery. As the response of MPNST to chemotherapy is questionable; is it enough to have surgery with close follow up after stopping chemotherapy.

  47. Case#4: CD003 AAA • Questions: • What is the next step in the management? • Answers after Group Discussion: • It is unlikely that margins are indeed negative, therefore this is not a low-risk tumor. • Evaluate residual disease by MRI or CT scan, evaluate lymph node for involvement. • If residual disease or positive lymph node, should treat with chemotherapy followed by local control .

  48. Case#4: CD003 AAA • Case Update: • No L.N. in the excised specimen available for evaluation. • Shifted to Non Soft Tissue Sarcoma protocol. • 1/12/2013 received 7th week (3rd block) on NSTS protocol.

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