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POEM Group Online Case Discussion

Discussion on the case of ASA with mesoblastic nephroma recurrence, diagnostic findings, treatment history, and management options to be considered. Includes detailed disease history, investigations, and treatment plan.

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POEM Group Online Case Discussion

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  1. POEM Group Online Case Discussion Date: 5 Nov. 2013 Case # 1: CD006 ASA

  2. Case # 1: • Referred by Physician: Salma Hadad& team • Referring Hospital: CWTH • Patient Initials: ASA • Nationality: Iraqi • Gender: male • DOB: 12Oct. 2006 Age: 7years • Diagnosis: Mesoblasticnephroma • Date of Diagnosis: 20/7/2013 • FirstConsultation

  3. Briefing • Recurrence of Mesoblastic nephroma after 3 months from nephrectomy in a 7-year old boy

  4. Disease History • ASA presented with sudden onset of hematuria before 6 mo., abdominal U/S & GUE suggested renal stone and treated conservatively. • after few days; 2nd attack of hematuria with same U/S & GUE finding--treatment given 2 mo., during which he was well, once he finished Rx., the same symptom recurred again. • 15/7/2013 abdominal U/S showed left renal mass. • 16/7/2013 left nephrectomy; • Biopsy--mesoblastic nephroma • He was kept on regular follow up by his surgeon.

  5. Disease History • 21/10/2013, he was referred to CWTH with colicky abdominal pain & change in bowel habit; U/S revealed recurrence at site of operation. • O/E: Normal apart from 8 cm scar over left groin of previous surgical operation, localized tenderness over the left iliac fossa. • Blood pressure 120/90 mm-Hg > 90th percentile for age

  6. Disease History Investigations: • 2/6/2013, excretory urography.... normal. • After contrast.... left kidney shows delayed persistent nephrogram with non- visualizing of its PCS or ureter, normal right kidney. • 10/6/2013, abdominal U/S... left kidney normal in size, shows moderate hydronephrosis with low cortical thickness 10mm, lower pole calyces, pelvis and upper 2/3 of left ureter occupy thick turbid urine with normal lower third. • 12/6/2013, abdominal U/S... bilateral course crystals more evident in the left, normal other organs.

  7. Disease History • 6/7/2013, CT abdomen.... left kidney shows moderate -severe dilatation of PCS with dilated left ureter down to the urinary bladder with reduced paranchymal thickness. • 14/7/2013, GUE.... turbid, alkaline reaction, albumin ++++, pus +, RBC ..full field, amorphous phosphate..+. • 20/7/2013 left kindny biopsy: • microscopically: section showed cellular proliferation of atypical spindle cells with frequent mitotic figures alternating with foci of hemorrhage and necrosis, originating from renal pelvis and confined to renal capsule, ureteric surgical margin are tumor free.... picture of mesoblastic nephroma. • 15/10/2013 abdominal U/S... two masses 41x34mm adjacent to spleen & 33x24mm left lower abdomen hypo-echoic mass, left nephrectomy, normal other organs.

  8. Disease History • 20/10/2013, abdominal U/S... left kidney not seen, with two masses defined round soft tissue echogenicity masses noted at the region of left kidney; first 4.3x4.3 cm and the second 3.8x4.3cm, both with marked arterial vascularity on Doppler (recurrence or remnant of tumor), normal other organs. • 21/10/2013 B.urea 21mg/dl, s. creatinine 0.9mg/dl, S.Ca 7.8, uric acid 9.8. • 23/10/2013 CT abdomen: heterogenous enlarged mass 5.4x5.6cm at left kidney, another mass 4.1x3.8 over left iliac region?? enlarged L.N., normal other organs. • 23/10/2013 SR.. cellular type of mesoblastic nephroma. • Treatment: hydration and xyloric (because of high uric acid)

  9. Questions 1. What is the next step in the management? 2. 3. 4.

  10. POEM Group Online Case Discussion Date: Case # 2: CD007 (NHN )

  11. Case # 2: CD007 • Referred by Physician: Dr.Salma /Dr. Mazin • Referring Hospital: CWTH • Patient Initials: N H N • Nationality: Iraqi • Gender: female • DOB: 15/3/2013 Age: 7 months • Diagnosis: neuroblastoma • Date of Diagnosis: 7/10/2013 • First Consultation / Follow Up (first consultation)

  12. Briefing • Seven-month old girl with retroperitoneal mass, liver secondaries and nephrocalcinosis, incisional biopsy revealed neuroblastoma, no N-MYC study. • Started treatment with less toxic protocol • What is the most appropriate protocol for treatment? what actions needed for nephrocalcinosis? How can we stage her?

  13. Disease progress • Since third month of age; changing in stool pattern followed by screaming few days later, abdominal U/S revealed an abdominal mass. 19/9/2013 Incisional biopsy--Neuroblastoma. • O/E: no dysmorphic features, 10 cm abd. Scar, no palpable organs. Investigations: • 31/8/2013 Abd. U/S: • left suprarenal mass 7x5cm heterogeneous of irregular outline and calcifications is seen. It crosses the midline engulfing abd. aorta, involves left kidney which shows multiple stones of different sizes (3mm average). • Moderate hepatomegaly diffusely infiltrated by a large no. of hypo echoic masses of different sizes (metastasis), normal spleen • Right kidney is normal in size with multiple stones (2mm in average), scattered all over its PCS..... neuroblastoma stage IV-S, engulfing abdominal aorta and left kidney.

  14. Disease progress • 1/9/2013, Hb 9.5g/dl, WBCc 13.8x10^9/L, platelets 515x10^9/L. • 2/9/2013, bilateral tibial BMA.... cellular reactive marrow smear, no evidence of neoplastic processes. • 9/9/2013, CT abdomen... large heterogeneous abdominal mass about 7.2x5.7cm in left lumbar region with numerous hepatic cysts? Bilateral nephrocalcinosis, no bony secondaries. • 30/9/2013 Left side suprarenal mass incision biopsy: picture compatible with undifferentiated neuroblastoma (stroma poor). • 7/10/2013, blood urea 22mg/dl, serum creatinine 0.7mg/dl, serum Ca+ 11.2mEq/l, uric acid 7.1mg/dl.

  15. Disease progress • 7/10/2013, abdominal U/S... left side retroperitoneal lesion about 78x45x39mm, heterogeneous texture, prominent vascular flow pattern, pushing left kidney superolaterally, extending to midline around engulfing aorta, picture of advanced neuroblastoma, liver mildly enlarged with multiple hypo-echoic nodular lesions scattered at both lobes ( secondaries), left kidney looks normal , pushed and compressed superolaterally in position, with few stones, largest 3mm, normal right kidney. • 7/10/2013, skeletal survey... negative.

  16. Treatment Protocol • 9/10/2013, started on Favorable risk neuroblastoma protocol( Doxorubicin 1mg/kg, and cyclophosphamide 30 mg/kg)+ post hydration every three weeks for a total of 5 courses.

  17. Initial Imaging (skull x-ray ,7-10-2013)

  18. (CT abdomen , 9-9-2013)

  19. (CT abdomen , 9-9-2013)

  20. (CT abdomen , 9-9-2013)

  21. Questions 1. any suggested treatment for bilateral nephrocalcinosis and high uric acid level? 2. is she stage IV (as we consider her) or Stage IVs? 3. 4.

  22. POEM Group Online Case Discussion Date: Case # 3: CD008 (RRA)

  23. Case # 3: CD008 • Referred by Physician: Salma and Team • Referring Hospital: CWTH • Patient Initials: RRA • Nationality: Iraqi • Gender: Female • DOB: 5/August/2010 Age: 3y&2mo • Diagnosis: BilateralRetinoblastoma • Date of Diagnosis: 01-Oct-2012 • FirstConsultation

  24. Briefing • Orbital mass diagnosed in Iraq in October 2012, • Inoculation in Turkey in April 2013, • CSF cytospin was and continued to be positive after 7 courses of chemotherapy--- • what is the next step in management?

  25. Disease History • 3-year old female presented with a 9 mo Hx. of whitish spot over the center of her right eye, an ophthalmologist suggested CT orbit which showed right eye mass. • After > 6 months, inoculation of right eye done in Turkey on 29/3/2013. Then returned to Iraq to complete the treatment. • 4/5/2013, she was seen in CWTH • O/E: fleshy small swelling from right eye orbit, no visible abnormality in the left eye.

  26. Disease History Turkey investigations: • 26/3/2013, Bone scan.... normal. • 26/3/2013, orbital MRI ..... bilateral retinoblastoma. • 30/3/2013, right eye inoculation..... retinoblastoma with optic nerve involvement. • April/2013, CSF cytospin ..... positive. Baghdad investigations: • 30/4/2013 orbital U/S... right side 3.2cm size soft tissue mass, normal left side. • 30/4/2013, CXR & abdominal U/S... normal. • 4/5/2013, LFT & RFT.... normal. BMA..... normal.

  27. Treatment Protocol • 5/5/2013, first JOE. • 15/5/2013, BMB.... normal. • 10/6/2013, second JOE, CSF analysis.... normal. • 16/6/2013, brain MRI.... normal. • 7/7 /2103, third JOE. • 13/8/2013, fourth JOE. • 14/8/2013, CSF analysis. cells > 150 lymphocytes, protein 1000 g/dl. • 17/8/2013, she developed fit > 1 hour, started on anticonvulsant (for a short period) . • 3/9/2013, fifth JOE. • 24/9/2013, sixth JOE. • 20/10/2013, 7th. JOE, CSF Cytospin.... positive. • 24/10/2013, upon discharge she developed fit (status epileptics), treated with anticonvulsant, and now she is well.

  28. Questions 1. what is the next step in treatment of persistence CNS involvement? 2. 3. 4.

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