CLINICO-EPIDEMIOLOGICAL CHARACTERISTICS AND OUTCOME OF PEDIATRIC BONE TUMORS: A FIFTEEN YEARS EGYPTIAN EXPERIENCE

Abstract

SUMMARY 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