1. Solid Tumors Forum POEM Group Case Discussions Solid Tumors Forum Date: May 27, 2014 www.Cure4Kids.org

2. CASE # 1: CD027 IRAQ

3. Children Welfare Teaching Hospital Presenter: Oncology Team Consultant Physician: Salma Hadad Presentation Date: May 27, 2014 POEM Case#: CD027 Patient Initials: Hussein FS Age: 17 year old Gender: Male Time to diagnosis from initial presenting symptoms: 4 years Date of Diagnosis: January 2009 Previously Presented Date (if applicable): None

4. Initial Presentation Presenting Complaints/ History of Illness : In 2002, the patient had a cervical mass at the age of 5 years Mass biopsy showed a benign disease Then, cervical and chest wall masses appeared in 2004 Hussein was treated in Iran and Iraq over 5 years with four different diagnoses, and he underwent a total of 4 surgical interventions Relevant Past / Family History: Negative

5. Initial Presentation Four different types of diagnosis: 1.Plexiform neurofibromatosis 2.Spindle cell sarcoma 3.Peripheral nerve sheath tumor 4.Malignant fibrous histiocytoma In January 2009, the diagnosis of Dermatofibrosarcoma protuberans was made after taking a second opinion from Rome, where the tissue sample was sent The patient then received Gleevec with good response to treatment

6. Initial Presentation Physical Findings: - Single café au lait spot < 5mm in the abdomen - Surgical scar at the left upper cervical region - Large disfiguring scar occupying the left cervical region - Surgical scar and skin discoloration secondary to radiotherapy over the left shoulder Baseline Labs:

7. Diagnostic Work-up

8. In Jan 30, 2009, pathology showed tissue fragments infiltrated by a soft tissue tumor, and composed of a uniform population of spindle cells arranged in a distinct storiform pattern around inconspicuous vessels. They also showed frequent mitotic figures. Other parts of the tumor showed myxoid areas where the storiform pattern became less distinct. Neoplastic cells were characterized by marked expression of CD34. They didn’t not express CD31, S100 protein, CD68, desmin, EMA, cytokeratin, and smooth muscle actin. 10% of the neoplastic cells showed proliferative activity as evaluated by Ki67 immunostaining. The histologic and immunohistochemical features were consistent with dermatofibrosarcoma protuberans

9. Final Diagnosis Dermatofibrosarcoma protuberans

10. Treatment Course Overall Course: On Feb 8, 2009: Gleevec 400mg/day started, with good response On May 4, 2010: Gleevec was discontinued On Feb 5, 2013, a soft tissue mass measuring ~ 2x3cm appeared at the left side of the neck, with cervical lymph node enlargement Neck U/S was done and showed multiple bilateral LAP seen along the neck major vessels, largest one seen in the left side measuring 17.2x4.6mm; Also, there is a superficial mass of medium echogenicity, measuring 22x10.6 mm, seen on the left side of the neck above the submandibular lymph node, and showing vascularity in its periphery; Overall picture was suggestive of keloid or disease recurrence

11. Treatment Course On Feb 19, 2013, Gleevec was restartd at 400 mg daily On May 21, 2013, U/S neck showed a left sided 21 mm superficial mass, together with thickening of the muscle bundle, associated with the presence of a 21 mm ovoid shaped lesion, with hilar mass lymphadenopathy On Jan 23, 2014, U/S neck showed a well defined homogeneous solid lesion at the left submandibular region, relatively avascular on colour Doppler, measuring 35*11 mm, with reactive nearby LN 13.3*6 mm On Feb 25, 2014, patient still had left cervical soft tissue swelling ~ 4x5cm, non-tender, soft in consistency, without other abnormal physical findings

12. Question? Is there any suggested treatment option other than Gleevec, given that the mass is still of the same size and causing embarrassment to both the patient and the family?

13. Patient HFS Early in 2004 In March 2009: After 5 years of misdiagnosis

14. Feb 3, 2009

17. March 8, 2009

20. Nov 10, 2009: 8 months post treatment

23. Feb 25, 2014: One year after resuming Gleevec

28. CASE # 2: CD028 IRAQ

29. Children Welfare Teaching Hospital Presenter: Oncology Team Consultant Physician: Salma Hadad Presentation Date: May 27, 2014 POEM Case#: CD028 Patient Initials: Deyar MK Age: 4 year old Gender: Male Time to diagnosis from initial presenting symptoms: 3 months Date of Diagnosis: April 6, 2014 Previously Presented Date (if applicable): None

30. Initial Presentation Presenting Complaints/ History of Illness: January 2014: - Left cervical swelling of 2 weeks duration - No associated fever or weight loss - Not responding to antibiotic therapy Relevant Past Medical/ Family History: Negative

31. Initial Presentation Physical Findings: - Enlarged left cervical lymph node, measuring ~ 2 X 3 cm - No associated organomegaly Baseline Lab and Imaging Studies: Jan 2, 2014: Hgb 11 g/dl, WBC 7800 /cmm (N 39%, L 51%), Plts 239000 /cmm; Blood film inspection normal; CXR clear Jan 20, 2014: Abdominal U/S revealed multiple right para-iliac LNs, largest measuring about 13.3x7.4 mm Jan 20, 2014: U/S Neck showed multiple bilateral small LNs noted, largest on the right measuring about 10.1x6 mm, and on the left measuring about 15x4.1 mm

32. Diagnostic Workup Excisional cervical lymph node biopsy was done on March 6, 2014, and pathology findings were consistent with metastatic nasopharyngeal carcinoma Second opinion in KHCC on April 6, 2014, where pathology was read as metastatic mucoepidermoid carcinoma, low-grade CT brain done on May 6, 2014: Evidence of well-defined mass, measuring ~15X16 mm, seen at the base of tongue, causing narrowing of the left vacuole, with peripheral enhancement No associated lymphadenopathy

33. Diagnostic Workup –Plan to do CT chest –Maxillofacial surgeons were consulted; they said that the mass could be reached via a transmandibulotomy approach

34. CT Brain on May 6, 2014

35. Final Diagnosis Mucoepidermoid Carcinoma, Low-Grade

36. Treatment Overall Course: None Complications / Morbidities:

37. Questions to be Discussed What is the appropriate management in this case? Given the presence of the small para-aortic lymph nodes, should CT abdomen be done? Does surgery carry a major morbidity?

38. CASE # 3: CD029 IRAQ

39. Children Welfare Teaching Hospital Presenter: Oncology Team Consultant Physician: Salma Hadad Presentation Date: May 27, 2014 POEM Case#: CD029 Patient Initials: Zahraa S Age: 2 year old Gender: Female Time to diagnosis from initial presenting symptoms: One month Date of Diagnosis: June 19, 2013 Previously Presented Date (if applicable): None

40. Initial Presentation Presenting Complaints/ History of Illness: Abdominal distension U/S and CT abdomen revealed a right-sided renal mass Tru-cut biopsy was done Pathology read as Wilms tumor Relevant Past Medical/ Family History: Negative

41. Diagnostic Work-up Abdominal U/S done on June 11, 2013 showed a solid mass arising anteriorly form the right kidney, measuring 10.7x9.7 cm, crossing the midline to the left side, and displacing great vessels, aorta, and IVC CT chest done on June 19, 2013 was normal A Tru-cut biopsy was done on June 19, 2013; pathology findings were consistent with nephroblastoma, featuring primitive tubular and blastemal type

42. Diagnostic Work-up CT abdomen done on July 30, 2013 showed a large well defined mass arising from the right kidney, measuring 70x57x50 mm, with cystic necrosis and scattered calcifications; it is displacing the adjacent vessels and abdominal organs CT chest is normal Right nephrectomy was done on August 28, 2013 Pathology read as Wilms tumor with predominant primitive tubular structure, that is associated with scattered foci of blastema and cellular mesenchyma, with abundant necrosis and calcification, and focal mature squamous structure (related to chemotherapy effect)

43. Treatment Protocol On June 20, 2013, treatment was started as per SIOP Wilms Tumor 2001 protocol, for localized Stage III disease On August 6, 2013, patient finished 6 weeks of neoadjuvant chemotherapy On August 21, 2013, nephrectomy was done Treatment was continued as per SIOP Wilms Tumor 2001 protocol, for localized Stage III disease, since Sep 2, 2013 Finished 27 weeks chemotherapy on March 30, 2014 As recommended by the protocol, radiation therapy should be given at week 2-4; however, this wasn’t done due to the lack of facilities in Iraq

44. Question? Is it still indicated to give radiation therapy?

45. CASE # 4: CD030 ERBIL

46. Nanakaly Hospital for Blood Disease Presenter: Oncology Team Consultant Physician: Mouroge Al Ani Presentation Date: May 27, 2014 POEM Case#: CD030 Patient Initials: NA Age:10 year old Gender: Female Time to diagnosis from initial presenting symptoms: 2 years Date of Diagnosis: July 1, 2012 Previously Presented Date (if applicable): None

47. Initial Presentation Ten year old girl, previously healthy, had sudden-onset asymmetrical appearance of her abdomen, with no other associated signs or symptoms U/S abdomen showed a big cystic lesion in the right ovary, for which she underwent oophorectomy Although the pathology was read as malignant teratoma, the surgeon recommended to do a follow-up imaging every 6 months Patient currently presenting with another cystic mass lesion in the left ovary, found incidentally during a routine follow-up U/S Relevant Past Medical / Family History: None

48. Initial Presentation Physical Findings: Abdomen soft, non-tender, and not distended, with no palpable mass or cyst Baseline Lab Studies: CBC and ESR within normal range values Renal and liver function tests within normal range values Alfa-fetoprotein = 8 mcg/l HCG-B < 5 mcg

49. Diagnostic Work-up –CXR is clear –U/S abdomen showed a big cystic mass in the left ovary - CT chest is negative - CT abdomen showed a left ovarian cystic mass lesion measuring 58*49*61 mm, with associated small pelvic fluid collection

50. Diagnostic Work-up –Right ovarian histopathology showed Malignant Teratoma –Pathology findings were reviewed in three different labs - Aspiration cytology of the ovarian cyst was negative for malignant cells - There is a family concern regarding another Oophorectomy

51. Treatment Overall Course: Therapy not given yet Complications / Morbidities:

52. Questions? What is the appropriate management in this case? Shall the diagnosis of malignant teratoma be questioned, as it took 2 years without any given treatment for the disease recurrence to occur? Would fertility be affected?

53. Next Solid Tumor Forum: June 24, 2014 Next Leukemia/Lymphoma Forum: June 3, 2014