ORIGINAL ARTICLE
Childhood Ewing Sarcoma of the Orbit Ahmad S. Alfaar, MBBCh, MSc,*wz Manal Zamzam, MD,y Badr Abdalla, MBBCh,w Ranin Magdi, BPSc, MSc,* and Naglaa El-Kinaai, MD8
Summary: In the span of the last 48 years, only 33 cases of children with orbital Ewing sarcoma have been reported. This study is to present 3 cases that were admitted to Children’s Cancer Hospital Egypt 57357, during the period from 2009 to 2013. We have 2 cases treated using the hospital standard Ewing sarcoma treatment protocol, to completion, whereas the third discontinued treatment. All tumors have confirmed CD99 positivity, although translocation (11;22) was positive in 1 patient and negative in the third. With earlier diagnosis and adequate surgical resection and integration of chemotherapy and radiotherapy 1 patient survived for about 4 years, whereas the other 2 cases died due to disease progression or recurrence. Key Words: Ewing sarcoma, childhood cancer, orbital tumors
(J Pediatr Hematol Oncol 2015;37:433–437)
E
wing sarcoma is one of the neuroectodermal tumors. It usually affects skeletal long bones, vertebrae, and the pelvis. Survival has increased dramatically from 10% to 70% recently with the advancement of integrated management. Orbital presentation of Ewing sarcoma in children is extremely rare. Our aim was to review the cases diagnosed as Ewing sarcoma of the orbit from the cancer registry of Children’s Cancer Hospital Egypt 57357 (CCHE) between July 2007 and July 2013.
MATERIALS AND METHODS Cancer registry of CCHE was queried for Ewing sarcoma patients affecting orbital region. We have reviewed the files of these patients to ensure that history taking and proper examination were done for all cases.
RESULTS Of 202 patients diagnosed with Ewing sarcoma at CCHE, only 3 patients were affected by orbital disease.
Case 1 This is a 11-month-old boy who presented with left eye gradual-onset progressive proptosis (over 1 mo). Magnetic resonance imaging (MRI) showed osteolytic destruction with periosteal reaction in the posterior aspect of the left orbital roof with a dumbbell soft-tissue mass (4 3 2 cm) having intraorbital (extraconal) and intracranial extension Received for publication June 30, 2014; accepted March 26, 2015. From the Departments of *Research; yPediatric Oncology; 8Pathology, Children’s Cancer Hospital; wCairo University Hospitals, Faculty of Medicine, Cairo University, Cairo, Egypt; and zFaculty of Medicine, Ulm University, Ulm, Germany. The authors declare no conflict of interest. Reprints: Ahmad S. Alfaar, MSc, MD, MBBCh, 1 Sekket Al-Imam Street, SayedaZainab, Cairo 11441, Egypt (e-mail: ahmadsfar@ gmail.com). Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
J Pediatr Hematol Oncol
with proptosis and mild indentation on the left frontal lobe. The adjacent fat planes appeared clear, suggesting metastatic neuroblastoma. Skeletal survey showed no additional mass sites. The patient had an umbilical hernia and patent foramen ovale with a minimal left to right shunt. Biopsy revealed a malignant round cell tumor with hyperchromatic nuclei and scanty cytoplasm-infiltrating bone trabeculae with positive reaction to CD99 and negative to desmin, synaptophysin, and CD45 (leukocyte common antigen [LCA]), confirming the diagnosis of Ewing sarcoma and primitive neuroectodermal tumor (PNET). Fluorescence in situ hybridization (FISH) failed to yield signal because of referred status of the slides. Chemotherapy was planned for 54 weeks. It included cycles of ifosfamide, etoposide, vincristine, cyclophosphamide, adriamycin, and actinomycin. Radiotherapy was conducted with a total dose of 5580 cGy divided over 31 fractions (180 cGy/fraction). Ophthalmological followup examination revealed corneal opacity, recurrent keratitis, and a dry eye with a visual acuity of left eye of 0.2. Ocular examination showed early posterior subcapsular cataract with a normal fundus. After 2 years of follow-up, the patient had facial asymmetry with no detectable recurrence clinically and radiologically (Fig. 1).
Case 2 This is a 12-year-old male patient who complained of a 20-day progressive headache. Initial computed tomography (CT) and MRI scans showed an ill-defined left greater sphenoid wing soft-tissue mass with intracranial and extracranial extension invading left lateral wall of the sphenoid bone and intrasphenoidal sinus including intrasellar soft tissue displacing the infundibulum to the contralateral side measuring about 5.5 5 cm. Bone scan and bone marrow biopsy were both free. Left temporal craniotomy was done for biopsy. Immunostaining showed positive reaction to CD99, but FISH study was negative. The patient was put on the hospital’s Ewing sarcoma treatment protocol. During the first week, the patient suffered from a severe headache and decreased visual acuity of the left eye. During third week, the headache decreased and the visual acuity improved. At this point the patient started radiotherapy. After week 15, MRI showed a 50% reduction of the initial mass size. After week 21, CT showed a new increase in mass size, which progressed further as evident by further radiologic examinations. This coincided with worsening headaches and vomiting. Two courses of salvage ICE (ifosfamide, carboplatin, and etoposide) were given which decreased the headache along with the left eye proptosis. Unfortunately, the disease mass increased further, and the patient suffered from severe pain. The patient was referred to palliative care for 2 weeks and passed away on February 2013.
Volume 37, Number 6, August 2015
www.jpho-online.com |
Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
433
Alfaar et al
J Pediatr Hematol Oncol
Volume 37, Number 6, August 2015
FIGURE 1. Computed tomography (CT) imaging of case. A, Initial CT of case 1 with osteolytic destruction with periosteal reaction is noted in the posterior aspect of the left orbital roof with a dumbbell soft-tissue mass (43 2 cm) having intraorbital (extraconal) and intracranial extension with proptosis and mild indentation on the left frontal lobe. B, Follow-up magnetic resonance imaging 5 months after initial CT.
Case 3 This is a 2.5-year-old male patient who presented with a right orbital mass of a gradual onset and progressive course over 5 months. The patient suffered from a limitation of eye movement and inability to open the right eye. Radiologic studies showed a large extracranial soft-tissue mass in the supero-medial aspect of right globe without lid extension. The orbital roof was seen destructed with
intracranial extension displacing eyeball infero-laterally. Skeletal survey showed no distant osseous deposits. Bone marrow biopsy was normal. Excisional biopsy revealed a malignant round cell tumor having round open-face nuclei with indistinct nucleoli and scanty cytoplasm. There was a strong positive reaction to CD99, and a negative reaction to desmin and LCA. FISH study was positive. It must be noted that parents were cousins (positive consanguinity).
434 | www.jpho-online.com Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved. Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
J Pediatr Hematol Oncol
Volume 37, Number 6, August 2015
Childhood Ewing Sarcoma of the Orbit
TABLE 1. Reported Cases of Primary Ewing Sarcoma/PNET of the Orbit
Sex/Age
Presentation
Bone Invasion
Initial Extension
Treatment
Follow-up
(1965) Alvarez-Berdecia et al3 (1979) Howard and Lund4 (1985) Wilson et al5 (1988) Woodruff et al6 (1988) Arora et al7 (1993)—1
M/ < 1 M/6
No accessible details Proptosis; headache
+ +
Anterior cranial fossa
Excision, RT Excision, RT, CT
14 mo, died 7 mo, alive
M/14
+
Sinuses; cribriform plate
Excision, RT, CTh
12 mo, alive
+ +
Intracranial space Sinuses; cranial fossa
Excision, RT, CTh Biopsy, RT, CTh
45 mo, alive 9 mo, alive
Excision
NS, NS
Arora et al7 (1993)—2 Tewari et al8 (1993) Singh et al9 (1994) Fiorillo et al10 (1996)
M/4 F/10 F/10 M/7
Excision Excision, RT, CTh Biopsy, RT, CTh Excision, RT, CTh
NS, NS 18 mo, alive 9 mo, alive 52 mo, alive
Lam et al11 (1999) Dutton et al12 (2000)—1 Dutton et al12 (2000)—2 Alyahya et al13 (2000) Wilson et al14 (2001)
M/2 M/2
Painful swelling over nasal bridge Proptosis Headache, visual loss Proptosis and diminution of vision Proptosis Painful proptosis Orbital swelling Painless orbital swelling Painless proptosis Ptosis and proptosis. Possibly metastatic Frontal bone swelling Pain and chemosis
F/17
Sen et al15 (2002) Bajaj et al16 (2003) Sharma et al17 (2005)
F/17 F/12 M/5
Romero et al18 (2006) Tamer et al19 (2007)
M/6
References (Year) Howard2
Bakhshi et al20 (2008)—1 Bakhshi et al20 (2008)—2 Bakhshi et al20 (2008)—3 Bakhshi et al20 (2008)—4 Bakhshi et al20 (2008)—5 Romero et al21 (2008) Das et al22 (2009)
F/7 M/6 F/13
M/7 F/5
M/10
Proptosis and vertical diplopia No accessible details No accessible details Proptosis, diminution of vision Proptosis
+ + NS
Cranial fossa; temporal fossa
+ +
Sinuses L1 vertebra
Biopsy, RT, CTh Excision, RT, CTh
24 mo, alive 17 mo, died
+
Lung, anterior cranial fossa
Excision, RT, CTh
12 mo, died
Excision, CTh
4 y, alive
Excision, CTh
7 y, alive
Excision, RT, CTh Excision, RT NS
5 y, alive 6 mo, alive NS, NS
Excision, CTh
7 y, died
Excision, RT
8 mo, alive
CT
Died during management 40 mo, alive
+ + +
Anterior cranial fossa, temporal fossa Liver Middle cranial fossa
M/ < 1
Squint, diplopia, and proptosis Proptosis
+
M/4
Proptosis
+
Sinonasal
RT, CTh
F/9
Proptosis
+
RT, CTh
M/12
Proptosis
+
Sinonasal, anterior cranial fossa –
Debulking, RT, CTh
F/13
Lower eyelid and cheek swelling Painless mass
+
Sinonasal
Exentration, RT, CTh
Excision, RT, CTh
Lost followup 17 mo, alive
Proptosis, diplopia, pain Proptosis Decreasing visual acuity and right proptosis Painless swelling Proptosis Epistaxis and eyelid swelling
Excision
6 mo, alive
RT, CTh Excision, RT, CTh
30 mo, alive 14 mo, died
F/ < 1 F/8
Kim et al23 (2009) Yang et al24 (2010)
M/2 M/6
Santra et al25 (2011) Nair et al26 (2011) Meccariello et al27 (2013)
M/12 F/13 M6
Klufas et al28 (2014)
M/6
Orbital mass
This study This study
M/ < 1 M/12
Proptosis Headache
+ +
This study
M/2.5
Proptosis, ptosis
+
+ + + +
Anterior cranial fossa
Infratemporal fossa, masseteric space Maxillary sinus Anterior cranial fossa Nasal cavity
Orbital roof Infratemporal and temporal fossa, greater wing of sphenoid Orbital roof
NS Excision, RT, CTh Endoscopic management, brachytherapy Excision, RTh, CTh Exentration, brachytherapy, CTh Excision, RT, CTh CTh, RT CT, RT
Died during management 20 mo, alive
NS, NS 3, alive NS, alive 37 mo, alive 48 mo, alive Died during management 15 mo, died
– indicates negative; +, positive; CTh, chemotherapy; NS, not specified; PNET, primitive neuroectodermal tumor; RT, radiotherapy.
Copyright
r
2015 Wolters Kluwer Health, Inc. All rights reserved.
www.jpho-online.com |
Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
435
J Pediatr Hematol Oncol
Alfaar et al
As per parents’ request, the patient was referred to a neighborhood cancer center. There he underwent chemotherapy and radiotherapy, and remained afterwards in complete remission for 15 months. Local recurrence occurred in form of proptosis and brain metastases evident by CT scan. He then received 2 cycles of ICE with no notable response. Partial response occurred (particularly with the brain metastases and proptosis) after a trial regimen of 3 cycles of irinotecan and timodal. Another 2 cycles did not result in additional response. The disease progressed and the patient was put on palliative care for 5 months that included chemotherapy and radiotherapy. He eventually passed away.
DISCUSSION Ewing sarcoma is reported to be the second most common type of primary bone tumor in the United States and Europe, accounting for nearly 25% to 34% of all malignant bone tumors, and 15% of primary bone sarcomas.1 It can arise in any bone, but it mainly affects pelvic, and long bones of the leg. Tumors that arise in the hands, feet, and head are very rare and comprise
Childhood Ewing Sarcoma of the Orbit Ahmad S. Alfaar, MBBCh, MSc,*wz Manal Zamzam, MD,y Badr Abdalla, MBBCh,w Ranin Magdi, BPSc, MSc,* and Naglaa El-Kinaai, MD8
Summary: In the span of the last 48 years, only 33 cases of children with orbital Ewing sarcoma have been reported. This study is to present 3 cases that were admitted to Children’s Cancer Hospital Egypt 57357, during the period from 2009 to 2013. We have 2 cases treated using the hospital standard Ewing sarcoma treatment protocol, to completion, whereas the third discontinued treatment. All tumors have confirmed CD99 positivity, although translocation (11;22) was positive in 1 patient and negative in the third. With earlier diagnosis and adequate surgical resection and integration of chemotherapy and radiotherapy 1 patient survived for about 4 years, whereas the other 2 cases died due to disease progression or recurrence. Key Words: Ewing sarcoma, childhood cancer, orbital tumors
(J Pediatr Hematol Oncol 2015;37:433–437)
E
wing sarcoma is one of the neuroectodermal tumors. It usually affects skeletal long bones, vertebrae, and the pelvis. Survival has increased dramatically from 10% to 70% recently with the advancement of integrated management. Orbital presentation of Ewing sarcoma in children is extremely rare. Our aim was to review the cases diagnosed as Ewing sarcoma of the orbit from the cancer registry of Children’s Cancer Hospital Egypt 57357 (CCHE) between July 2007 and July 2013.
MATERIALS AND METHODS Cancer registry of CCHE was queried for Ewing sarcoma patients affecting orbital region. We have reviewed the files of these patients to ensure that history taking and proper examination were done for all cases.
RESULTS Of 202 patients diagnosed with Ewing sarcoma at CCHE, only 3 patients were affected by orbital disease.
Case 1 This is a 11-month-old boy who presented with left eye gradual-onset progressive proptosis (over 1 mo). Magnetic resonance imaging (MRI) showed osteolytic destruction with periosteal reaction in the posterior aspect of the left orbital roof with a dumbbell soft-tissue mass (4 3 2 cm) having intraorbital (extraconal) and intracranial extension Received for publication June 30, 2014; accepted March 26, 2015. From the Departments of *Research; yPediatric Oncology; 8Pathology, Children’s Cancer Hospital; wCairo University Hospitals, Faculty of Medicine, Cairo University, Cairo, Egypt; and zFaculty of Medicine, Ulm University, Ulm, Germany. The authors declare no conflict of interest. Reprints: Ahmad S. Alfaar, MSc, MD, MBBCh, 1 Sekket Al-Imam Street, SayedaZainab, Cairo 11441, Egypt (e-mail: ahmadsfar@ gmail.com). Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
J Pediatr Hematol Oncol
with proptosis and mild indentation on the left frontal lobe. The adjacent fat planes appeared clear, suggesting metastatic neuroblastoma. Skeletal survey showed no additional mass sites. The patient had an umbilical hernia and patent foramen ovale with a minimal left to right shunt. Biopsy revealed a malignant round cell tumor with hyperchromatic nuclei and scanty cytoplasm-infiltrating bone trabeculae with positive reaction to CD99 and negative to desmin, synaptophysin, and CD45 (leukocyte common antigen [LCA]), confirming the diagnosis of Ewing sarcoma and primitive neuroectodermal tumor (PNET). Fluorescence in situ hybridization (FISH) failed to yield signal because of referred status of the slides. Chemotherapy was planned for 54 weeks. It included cycles of ifosfamide, etoposide, vincristine, cyclophosphamide, adriamycin, and actinomycin. Radiotherapy was conducted with a total dose of 5580 cGy divided over 31 fractions (180 cGy/fraction). Ophthalmological followup examination revealed corneal opacity, recurrent keratitis, and a dry eye with a visual acuity of left eye of 0.2. Ocular examination showed early posterior subcapsular cataract with a normal fundus. After 2 years of follow-up, the patient had facial asymmetry with no detectable recurrence clinically and radiologically (Fig. 1).
Case 2 This is a 12-year-old male patient who complained of a 20-day progressive headache. Initial computed tomography (CT) and MRI scans showed an ill-defined left greater sphenoid wing soft-tissue mass with intracranial and extracranial extension invading left lateral wall of the sphenoid bone and intrasphenoidal sinus including intrasellar soft tissue displacing the infundibulum to the contralateral side measuring about 5.5 5 cm. Bone scan and bone marrow biopsy were both free. Left temporal craniotomy was done for biopsy. Immunostaining showed positive reaction to CD99, but FISH study was negative. The patient was put on the hospital’s Ewing sarcoma treatment protocol. During the first week, the patient suffered from a severe headache and decreased visual acuity of the left eye. During third week, the headache decreased and the visual acuity improved. At this point the patient started radiotherapy. After week 15, MRI showed a 50% reduction of the initial mass size. After week 21, CT showed a new increase in mass size, which progressed further as evident by further radiologic examinations. This coincided with worsening headaches and vomiting. Two courses of salvage ICE (ifosfamide, carboplatin, and etoposide) were given which decreased the headache along with the left eye proptosis. Unfortunately, the disease mass increased further, and the patient suffered from severe pain. The patient was referred to palliative care for 2 weeks and passed away on February 2013.
Volume 37, Number 6, August 2015
www.jpho-online.com |
Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
433
Alfaar et al
J Pediatr Hematol Oncol
Volume 37, Number 6, August 2015
FIGURE 1. Computed tomography (CT) imaging of case. A, Initial CT of case 1 with osteolytic destruction with periosteal reaction is noted in the posterior aspect of the left orbital roof with a dumbbell soft-tissue mass (43 2 cm) having intraorbital (extraconal) and intracranial extension with proptosis and mild indentation on the left frontal lobe. B, Follow-up magnetic resonance imaging 5 months after initial CT.
Case 3 This is a 2.5-year-old male patient who presented with a right orbital mass of a gradual onset and progressive course over 5 months. The patient suffered from a limitation of eye movement and inability to open the right eye. Radiologic studies showed a large extracranial soft-tissue mass in the supero-medial aspect of right globe without lid extension. The orbital roof was seen destructed with
intracranial extension displacing eyeball infero-laterally. Skeletal survey showed no distant osseous deposits. Bone marrow biopsy was normal. Excisional biopsy revealed a malignant round cell tumor having round open-face nuclei with indistinct nucleoli and scanty cytoplasm. There was a strong positive reaction to CD99, and a negative reaction to desmin and LCA. FISH study was positive. It must be noted that parents were cousins (positive consanguinity).
434 | www.jpho-online.com Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved. Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
J Pediatr Hematol Oncol
Volume 37, Number 6, August 2015
Childhood Ewing Sarcoma of the Orbit
TABLE 1. Reported Cases of Primary Ewing Sarcoma/PNET of the Orbit
Sex/Age
Presentation
Bone Invasion
Initial Extension
Treatment
Follow-up
(1965) Alvarez-Berdecia et al3 (1979) Howard and Lund4 (1985) Wilson et al5 (1988) Woodruff et al6 (1988) Arora et al7 (1993)—1
M/ < 1 M/6
No accessible details Proptosis; headache
+ +
Anterior cranial fossa
Excision, RT Excision, RT, CT
14 mo, died 7 mo, alive
M/14
+
Sinuses; cribriform plate
Excision, RT, CTh
12 mo, alive
+ +
Intracranial space Sinuses; cranial fossa
Excision, RT, CTh Biopsy, RT, CTh
45 mo, alive 9 mo, alive
Excision
NS, NS
Arora et al7 (1993)—2 Tewari et al8 (1993) Singh et al9 (1994) Fiorillo et al10 (1996)
M/4 F/10 F/10 M/7
Excision Excision, RT, CTh Biopsy, RT, CTh Excision, RT, CTh
NS, NS 18 mo, alive 9 mo, alive 52 mo, alive
Lam et al11 (1999) Dutton et al12 (2000)—1 Dutton et al12 (2000)—2 Alyahya et al13 (2000) Wilson et al14 (2001)
M/2 M/2
Painful swelling over nasal bridge Proptosis Headache, visual loss Proptosis and diminution of vision Proptosis Painful proptosis Orbital swelling Painless orbital swelling Painless proptosis Ptosis and proptosis. Possibly metastatic Frontal bone swelling Pain and chemosis
F/17
Sen et al15 (2002) Bajaj et al16 (2003) Sharma et al17 (2005)
F/17 F/12 M/5
Romero et al18 (2006) Tamer et al19 (2007)
M/6
References (Year) Howard2
Bakhshi et al20 (2008)—1 Bakhshi et al20 (2008)—2 Bakhshi et al20 (2008)—3 Bakhshi et al20 (2008)—4 Bakhshi et al20 (2008)—5 Romero et al21 (2008) Das et al22 (2009)
F/7 M/6 F/13
M/7 F/5
M/10
Proptosis and vertical diplopia No accessible details No accessible details Proptosis, diminution of vision Proptosis
+ + NS
Cranial fossa; temporal fossa
+ +
Sinuses L1 vertebra
Biopsy, RT, CTh Excision, RT, CTh
24 mo, alive 17 mo, died
+
Lung, anterior cranial fossa
Excision, RT, CTh
12 mo, died
Excision, CTh
4 y, alive
Excision, CTh
7 y, alive
Excision, RT, CTh Excision, RT NS
5 y, alive 6 mo, alive NS, NS
Excision, CTh
7 y, died
Excision, RT
8 mo, alive
CT
Died during management 40 mo, alive
+ + +
Anterior cranial fossa, temporal fossa Liver Middle cranial fossa
M/ < 1
Squint, diplopia, and proptosis Proptosis
+
M/4
Proptosis
+
Sinonasal
RT, CTh
F/9
Proptosis
+
RT, CTh
M/12
Proptosis
+
Sinonasal, anterior cranial fossa –
Debulking, RT, CTh
F/13
Lower eyelid and cheek swelling Painless mass
+
Sinonasal
Exentration, RT, CTh
Excision, RT, CTh
Lost followup 17 mo, alive
Proptosis, diplopia, pain Proptosis Decreasing visual acuity and right proptosis Painless swelling Proptosis Epistaxis and eyelid swelling
Excision
6 mo, alive
RT, CTh Excision, RT, CTh
30 mo, alive 14 mo, died
F/ < 1 F/8
Kim et al23 (2009) Yang et al24 (2010)
M/2 M/6
Santra et al25 (2011) Nair et al26 (2011) Meccariello et al27 (2013)
M/12 F/13 M6
Klufas et al28 (2014)
M/6
Orbital mass
This study This study
M/ < 1 M/12
Proptosis Headache
+ +
This study
M/2.5
Proptosis, ptosis
+
+ + + +
Anterior cranial fossa
Infratemporal fossa, masseteric space Maxillary sinus Anterior cranial fossa Nasal cavity
Orbital roof Infratemporal and temporal fossa, greater wing of sphenoid Orbital roof
NS Excision, RT, CTh Endoscopic management, brachytherapy Excision, RTh, CTh Exentration, brachytherapy, CTh Excision, RT, CTh CTh, RT CT, RT
Died during management 20 mo, alive
NS, NS 3, alive NS, alive 37 mo, alive 48 mo, alive Died during management 15 mo, died
– indicates negative; +, positive; CTh, chemotherapy; NS, not specified; PNET, primitive neuroectodermal tumor; RT, radiotherapy.
Copyright
r
2015 Wolters Kluwer Health, Inc. All rights reserved.
www.jpho-online.com |
Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
435
J Pediatr Hematol Oncol
Alfaar et al
As per parents’ request, the patient was referred to a neighborhood cancer center. There he underwent chemotherapy and radiotherapy, and remained afterwards in complete remission for 15 months. Local recurrence occurred in form of proptosis and brain metastases evident by CT scan. He then received 2 cycles of ICE with no notable response. Partial response occurred (particularly with the brain metastases and proptosis) after a trial regimen of 3 cycles of irinotecan and timodal. Another 2 cycles did not result in additional response. The disease progressed and the patient was put on palliative care for 5 months that included chemotherapy and radiotherapy. He eventually passed away.
DISCUSSION Ewing sarcoma is reported to be the second most common type of primary bone tumor in the United States and Europe, accounting for nearly 25% to 34% of all malignant bone tumors, and 15% of primary bone sarcomas.1 It can arise in any bone, but it mainly affects pelvic, and long bones of the leg. Tumors that arise in the hands, feet, and head are very rare and comprise
