1. POEM Group Online Case Discussion Date: March 4, 2014
4 New Cases:
CD018: Infantile Fibrosarcoma (Iraq)
CD019: Bilateral WT (Iraq)
CD020: ALL (Iraq)
CD021: RMS (Lebanon)
3 Follow Up Cases:
Case1: Mesoblastic Nephroma (Iraq)
Case2: Neuroblastoma (Iraq)
Case3: Orbital Mass (Iraq)

2. POEM Group Online Case Discussion
Date: 4/3/2014
Case # 1:CD018 (Yousif AL)

3. Case # 1: CD018 Referred by Physician: Oncology Team
Referring Hospital: CWTH
Patient Initials: Yousif AL
Nationality: Iraqi
Gender: male
DOB: 24/11/2013 Age: 3 months
Diagnosis: infantile fibrosarcoma
Date of Diagnosis: 30/1/2014
First Consultation

4. Disease History
Small superficial mass at right elbow, noticed by the family by age of 10 days.
MRI & U/S showed round mass 36x 32 mm, subcutaneous, with peripheral pattern of enhancement, the humerus look intact with normal joint space, no obvious soft tissue invasion
Underwent Excisional biopsy on 28/12/2013.

5. Disease History
14/1/2014 right arm U/S (after surgery): lower third show presence of 2.6x1.8x3mm size hypoechoic triangular heterogeneous mass with mild vascularity at site of operation.
21/1/2014 CT chest... normal, BM normal
21/1/2014 biopsy showed infantile fibrosarcoma- intermediate grade, negative for S-100, bcl-2, CD99, cytokeratin and EMA markers
2/2/2014 Rt. arm U/S: 2.9x19.9x23.9mm irregular heterogeneous mass noted in the distal right forearm in close relation to brachial a R/O recurrence.

6. Imaging

7. Imaging

8. Disease History
4/2/2014 started on EpSSG NRSTS 2005 Protocol, with Doxorubicin for 2 days and Ifosfamide for 3 days.

9. Questions
Any advice about the management regarding choice of the protocol, modification of the doses, and the need for radiotherapy.

10. POEM Group Online Case Discussion
Date: 4/3/2014
Case # 2: CD019 (Helen S.Y.)

11. Case # 2: CD019 Referred by Physician: Oncology Team
Referring Hospital: CWTH
Patient Initials: Helen S.Y.
Nationality: Iraqi
Gender: female
DOB: 16/9/2011 Age:3 years and 2 months
Diagnosis: Bilateral WT
Date of Diagnosis: 25/3/2012
First Consultation

12. Disease History
Bilateral Wilms tumor diagnosed at 6 months by incisional biopsy
U/S:
11.5cmx7cm mass with cystic degeneration in the Rt. kidney.
7.8x5.8cm Left renal mass with cystic degeneration
SIOP WT/pre-operative/ localized disease Stage III.
Rt. renal mass biopsy showed WT of favourable histology.

13. Disease History
Evaluation after 5 weeks chemo: well defined lobulated mass 6x4.5cm in lower pole of Rt. kidney, and 2x1.5cm in upper Lt. kidney.
After 2 month Abdominal CT scan:
well-defined mass at lower pole of Rt. kidney measuring 4.5x4cm shows less enhancement than renal parenchyma.
Hypo-dense mass at the lower pole of the Lt. kidney measuring 1.2x1.5cm

14. Disease History
CT chest and Abdomen (after 20 weeks chemo): clear lung fields, bilateral hypoechoic masses over lower pole of both kidneys, smaller size than previous CT.
30 Dec finished chemotherapy.
April Rt. partial nephrectomy
June Histopathology showed Teratoid Wilms Tumor.

15. Disease History
July 2013 Rome slide review: biphasic nephroblastoma (Wilms tumor) mainly blastemal. C/W squamous predominant teratoid Wilms tumor.
Jan.2014 abdominal U/S:
Rt. kidney is 6.2x4.1cm normal in texture ,
left kidney is 7.8x3.4 cm showing lower pole 17x13x15mm hypoechoic mass.
The surgeon ask if there is any possibility for further chemotherapy.
14/1/2014 CT abdomen: Recurrence or residue of prior WT of right kidney.

16. Imaging

17. Questions
What is the suggested further management? is there any role for chemotherapy?

18. POEM Group Online Case Discussion
Date: 4/3/2014
Case # 3: CD020 (Tammar HG)

19. Case # 3: CD020 Referred by Physician: Oncology Team
Referring Hospital: CWTH
Patient Initials: Tammar HG
Nationality: Iraqi
Gender: male
DOB: 6/2/ Age: 9 years
Diagnosis: ALL
Date of Diagnosis: 2/4/2012
First Consultation

20. Disease History 9y old, boy, diagnosed with ALL In April 2012
Treated as per MRC-UK ALL 2003/ SR with three drugs regimen
At week 70 of maintenance, he developed left lower thigh swelling, preceded by few weeks history of pain.

21. Disease History
X-ray of left thigh: an ill-defined osteolytic lesion seen involving the distal femoral metaphysis with wide zone of periosteal reaction
CT scan of left femur: soft tissue mass 4x3.7cm, in anterior aspect of lower part of left thigh with bony destruction and cortical erosion, sign of secondary infiltration.
BMA & BMB... normal.
Orthopedic surgeon advised for follow up only.

22. Initial Imaging
Caption: (local x-ray, 11/10/2013)

23. Disease History
14/1/2014 left knee MRI: well defined destructive lesion at the lower diaphysis of left femur, Onion peel periosteal reaction with cortical sclerosis
Feb.2014 biopsy and slide review: Osteosarcoma, fibrohistiocytic variant.
18/Feb./2014, received week 80 maintenance 100%

24. Initial Imaging

25. Imaging
18/2/2014
18/2/2014

26. Questions
what is the next step in the management?

27. POEM Group Online Case Discussion
Date: 4/3/2014
Case # 4: CD021 (Tahani F)

28. Case # 4: CD021 Referred by Physician: Dr. Raya Saab
Referring Hospital: AUBMC-CCCL
Patient Initials: T.H.
Nationality: Lebanese
Gender: F
DOB: 12/07/ Age: 21
Diagnosis: RMS
Date of Diagnosis:
First Consultation / Follow Up (First Consultation)

29. Disease History
T.F. is a 21 y.old, girl, initially diagnosed with ALL at 4 years of age (1996), received full treatment course (3 years)
In october 2008, was diagnosed with alveolar RMS of left temporalis muscle (non metastatic)
Started on chemotherapy as per ARST-0531 protocol (VAC based), followed by surgery and radiotherapy
Ended treatment in October 2009

30. Disease History
August 2010 (after 1 year), had relapse in the left parotid gland (FNA)
Underwent surgical resection, followed by 2nd line chemotherapy (Cyclo/Doxo and I/E) 10 cycles and radiotherapy
Ended treatment in july 2011

31. Disease history

32. Disease History
In March 2012 (after 18month) patient developed 3d relapse anterior to the left sternocleidomastoid at the level of the hyoid bone
Underwent surgery followed by radiotherapy and 3d line chemotherapy Cyclo/Topo and Vcr/ Irinotecan 8 cycles
Ended in October 2012

33. Disease history

34. Disease History
December 2013, presented with Small lymph node anterior to the left sternocleidomastoid muscle
FNA was consistent with recurrent RMS

35. Disease history

36. Treatment Protocol Localized ARMS Oct 2008
1st line : VAC (Oct 2008-Oct 2009)
Regional Lymph Node Localized recurrence Aug 2010
2nd line : Cyclo/Doxo and I/E; 8 cycles (Aug July 2011)
Regional Lymph node localized recurrence March 2012
3rd line : Cyclo/Topo and Irino/VCR; 10 cycles (March 2012-Oct 2012)
Regional Lymph node recurrence January 2014; undergoing metastatic workup.

37. Questions
1. If localized disease: Suggestions about further management ?

38. Follow Up Cases

39. Case#1: Abass SA
Presented on: 29/10/2013 Brief History: Recurrence of Mesoblastic nephroma after 3 months from nephrectomy in a 7-year old boy.
Questions:
What is the next step in the management?
Answers after Group Discussion:
Do biopsy then 2 courses of chemotherapy. Then evaluation, surgery plus RTP
Update:
Received 4 courses of Ewing sarcoma like protocol ( VCR, cyclophosphamide , and doxorubicin on D1-D2 repeated every 3 weeks).
After 4 course, CT abdomen with contrast on 9/1/2014: cystic like lesion 3.2 x 2.9 cm in left renal region, normal right kidney, localized.
26/1/2014, admitted to receive 13 weeks ( fifth course).

40. Case#2: Noor HN Presented on: 28/10/2013
Brief History: 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.
Questions: any suggested treatment for bilateral nephrocalcinosis and high uric acid level? Is she stage IV (as we consider her) or Stage IVs?
Answers after Group Discussion:
This is a stage IV (high risk). Suggested treatment is high-risk protocol.

41. Case#2: Noor HN Case Update:
In October had been already started on Favorable risk neuroblastoma protocol (Doxorubicin 1mg/kg, and cyclophosphamide 30 mg/kg) every three weeks planned for a total of 5 courses.
8/11/2013, Rome university review: poorly differentiated neuroblastoma (Schwannian stroma poor).
31/12/2013, finished 5 courses.
21/1/2014,last evaluation by U/S abdomen and CXR ( result was normal).
26/1/2014, admitted to receive course no 1 of cycle 1 of high risk group neuroblastoma protocol (VCR, CYCLO, DOX Alternating with VP16 & carboplatin).

42. Case#3: Ruqaia RA Presented on: 30-10-2013
Brief History: 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.
Questions: what is the next step in treatment of persistence CNS involvement?
Answers after Group Discussion: Radiation and palliative care
Case Update: 24/10/2013, upon discharge she developed fit (status epileptics), treated with anticonvulsant, but unfortunately the patient did nor respond to treatment and died.