الفهرس 
OSTEOSARCOMAOnly 14 pages are availabe for public view
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Abstract
T
he present study of the record of 37 patients at pediatric age group with a pathology proven diagnosis of primary bone tumor presented to Pediatric hematology/oncology department, Orthopedic surgery department and Nuclear medicine and Radiology department at Ain Shams University during period from January 1994 to July 2016.
The epidemiological data regarding age at first presentation, age at diagnosis, sex, residency, parent’s consanguinity, and family history of bone tumor or other malignancies were recorded.
The clinical data regarding main presenting symptoms, site of primary tumor, delay at diagnosis, laboratory workup, radiological workup, histopathological diagnosis, treatment protocol, modality of surgery, tumor necrosis, complication on treatment was recorded. Functional status by using Musculoskeletal Tumour Society Score (MSTS score) was assessed. All available data were statistical analysed.
They were 37 patients diagnosed as bone tumor from a total of 1581 patients in the twenty-two years (2.3%). 31 of cases presented after 2005 and represent 83.8% while on the other hand six cases presented before 2005 and represent 16.2%. 22 patients diagnosed as Ewing sarcoma with percentage of 59.5%. On the other hand, 15 patients diagnosed as osteosarcoma with percentage 40.5%.
The age for patients presented with osteosarcoma ranged from 2 – 17 years with a median 11 (4 – 14). For patients presented with Ewing sarcoma the age ranged from 2-15 years with median 5 (4-11).
Male to female ratio for patients presented with Ewing sarcoma was 1:2,1. male: female ratio for patients presented with osteosarcoma was 1.5:1.
There is positive statistical significance correlation between age of studied patients with median age 13(12-15) for osteosarcoma and 5(4-11) for Ewing sarcoma and occurrence of bone tumor (p-value 0.005).
Fifty % of our studied patients were resident at rural area (outside Cairo metropolitan). The other 50% were resident at Cairo metropolitan.
Two cases had positive family history, one of them her sister diagnosed with brain tumor, the other one her sister had bone tumor.
Femur was the most common site for Ewing sarcoma as well as osteosarcoma 30% and 46.6% respectively. Tibia was the second most common site for osteosarcoma 20%. On the other hand, chest wall was the second most common site for Ewing sarcoma (20%).
Swelling was the most common symptom of osteosarcoma representing 73.3% then followed by pain representing 66.6%. For patients presented with Ewing sarcoma, swelling and pain were the most common presenting symptoms as each one of them represent 27.8%.
The time between complaint and diagnosis was ranged from 9-1065 days with median (IQR) 90 (45-300) days for patients presented with osteosarcoma. For patients presented with Ewing sarcoma the time between complaint and diagnosis was ranged from 45-700 days with median (IQR) 90(60-180) days.
MRI for the involved part was the most accurate as it was diagnostic in all patients 100%. While CT for the involved part was diagnostic in 72%, the three patients who were not diagnosed by CT (CT was negative) when they do MRI was diagnosed.
Two cases only presented with metastasis at time of presentation and it represent 6.9%. One patient with left distal femur osteosarcoma presented with skip metastasis at left proximal femur and osteo-sclerotic bony lesions with small soft tissue component are seen at the costo-vertebral junction of left 4th and 12th ribs sclerotic soft tissue at the right 3rd rib. The other patient with left hemi-thorax Ewing sarcoma presented with lung metastasis.
Two chemotherapy protocols (POG #9354/CCG #7924 protocol VS combined therapy protocol) were used for treating the studied patients with Ewing sarcoma. three out of 12 who treated with regimen A had relapse (25%), and nine (75%) were relapse free, three (27.2%) died and eight (72.8%) were alive, one lost follow-up. Only three patients (20%) were treated with combined therapy protocol; one of them relapsed and died, the second is relapse free and alive, the third one lost follow-up. One of patient treated with regimen A presented with metastasis and none of those treated with combination therapy were. There are no statistical differences between two regimens as regard relapse (X2= 0.525, P= 0.469) nor survival (X2= 0.535, P= 0.475)
Two chemotherapy regimens based on (protocol CCG 7921) were used for treating the studied patients. nine patients treated with regimen A, two relapsed (22.2%) and seven were relapse free (77.8%), four died (44.4) and five still alive (55.6%). Two patients (15.4%) treated with regimen B; one of them (50%) relapsed and both are alive. Two patients refuse treatment, one died and one lost follow up, both was relapse free at time of assessment. There are no statistical differences between two regimens as regard relapse (X2=0.637, P=0.425) nor survival (X2=1.397, P=0.237)
Limb salvage was the most common type for local control with 59.26% followed by other type of local control with 33.3% as debulking surgery for tumor in paraspinal area and excision of mass present in chest wall. On the other hand, two patients only gone for amputation (7.4%) both of them diagnosed with osteosarcoma. The first one done amputation on 2009 due to aggressive behavior of the tumor, and the other patients done amputation on 2016 due to osteomyelitis after limb salvage operation.
Infection was the most common complication of treatment of Ewing sarcoma and osteosarcoma as it represents 50% and 53% respectively. While six patients only had febrile neutropenia distributed as one patient with osteosarcoma (6.6%) and five presented with Ewing sarcoma (22.7%).
The percentage of patients with osteosarcoma had tumor necrosis less than 90% was 83.3% and 16.7% had tumor necrosis more than 90%.
Complications of treatment and relapse were the most common cause of death for both osteosarcoma and Ewing sarcoma.
Fifteen patients had MSTS ranged from 50 to 87 (Good to Excellent) with Mean±SD (74.53 ± 10.39. Most of patients with osteosarcoma and Ewing sarcoma had MSTS >75 with percentage 55.6% and 66.6% respectively
There is significance statistical correlation between MSTS and outcome as for died patients the MSTS ranged from 50-65 (Good) and for the survived patients the MSTS ranged from 76-87 (Excellent) (P-value <0.001).
There was statistical significance difference in MSTS for patients gone for surgery. As it is higher in patients gone for limb salvage operation compared to amputation (p-value <0.001)