الفهرس 
Primary Malignant Bone TumorsOnly 14 pages are availabe for public view
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Abstract
A retrospective study of the record of 103 patients at pediatric age group and adolescence with a pathology proven diagnosis of primary malignant bone tumor presented to Ain Shams University Hospitals and Nasser Institute Hospital for research and treatment from April 1999 to April 2004. According to the available recorded data:
The Epidemiological data regarding Age, Sex, Residence, Height percentile at diagnosis, Parental occupation, history of exposure to alkalyting agents, History of exposure to radiotherapy, Presence of bone deformities, Presence of genitourinary abnormalities, Family history of Retinoblastoma and History of Li-Fraumeni syndrome.
The Clinical data regarding site of primary tumor, size of primary tumor, main presenting symptom, delay at diagnosis, misdiagnosis, histopathological diagnosis, basis of pathological diagnosis, extension of the tumor to adjacent joint , affection of neurovascular bundle, staging(metastatic or non metastatic at diagnosis),treatment protocol, modality of local control, tumor necrosis, complications on treatment, delay during treatment and outcome. All available data were statistically analyzed.
Non metastatic cases of osteosarcoma represented 38.83% of the studied cases, metastatic cases of osteosarcoma represented 9.71% of the studied cases, Non metastatic cases of Ewing’s sarcoma represented 37.86% of the studied cases, metastatic cases of Ewing’s sarcoma represented 13.6% of the studied cases .
The mean age for non metastatic cases of osteosarcoma was14.6±2.2 year, 14.2±1.5 year for metastatic cases of osteosarcoma, 13.41±2 for non metastatic cases of Ewing’s sarcoma and 14.64±1.8 year for metastatic cases of Ewing’s sarcoma.
Female to male ratio was 1.1: 1 and 1.5: 1 for non metastatic cases of osteosarcoma and metastatic cases of osteosarcoma respectively. Female to male ratio of 1:1.43 and 1:1.33 for non metastatic cases of Ewing’s sarcoma and metastatic cases of Ewing’s sarcoma respectively.
Non significant statistical correlation between sex & various age groups for non metastatic cases of osteosarcoma as well as for metastatic. Increased incidence for males and females at age 13 - 18 years old (P<0.303, P<0.096, P<0.078and P<0.074 were reported). Non significant statistical correlation between sex & various age groups for non metastatic cases of Ewing’s sarcoma as well as for metastatic. Increased incidence at age 13 - 18 years old (P<0.08, P<0.09, P<0.078 and P <0.069). 60% of non metastatic cases of Osteosarcoma were resident at rural areas versus 40% of metastatic cases, whereas43.59% of non metastatic cases of Ewing’s sarcoma were resident at rural areas versus57.14 % of metastatic cases of Ewing’s sarcoma.
There was a statistically significant correlation between increased height percentile at time of presentation and increased risk for non metastatic as well as metastatic osteosarcoma (P<0.0491 and P<0.0394 respectively). There is a statistically significant correlation between increased height percentile at time of presentation and increased risk for non metastatic as well as metastatic Ewing’s sarcoma (P<0.044and P<0.049respectively).
Agriculture related work does not correlate with increasing incidence of osteosarcoma and Ewing’s sarcoma in our study.
One case of non-metastatic osteosarcoma of jaw had history of Li- fraumeni syndrome and one case of non-metastatic osteosarcoma had multiple congenital deformities (osteogenesis imperfecta).were reported in the present study.
Femur was the most common affected primary site for non metastatic (47.5 % of cases) as well as metastatic cases (80% of cases) of osteosarcoma. Ilium was the most common affected primary site for non metastatic cases of Ewing’s sarcoma (23.077% of cases) as well as for metastatic cases of Ewing’s sarcoma (35.71% of cases).
Swelling was the main presenting symptom of non metastatic cases of osteosarcoma and Ewing’s sarcoma, while pain was the main presenting symptom of metastatic cases of osteosarcoma and Ewing’s sarcoma.
The mean time between complaint and diagnosis was2.41±1.18 month for non metastatic osteosarcoma , 2.5±2.12 month for metastatic osteosarcoma, 2.46±1.9 month for non metastatic Ewing’s sarcoma and 1.57±1.5 month for metastatic Ewing’s sarcoma.
Conventional osteosarcoma represents 35% of non metastatic cases of osteosarcoma and 40% of metastatic cases of osteosarcoma. PNET constituted 20% of non metastatic cases of Ewing’s sarcoma and 7.14% of metastatic cases.
Four chemotherapy protocols were used for treating the studied patients with osteosarcoma either non metastatic or metastatic with statistical significant increase in the cure rate (70%) for the patients with non metastatic osteosarcoma who were submitted to regimen (A) using Ifosfamide + Adriamycin alternating with cisplatin + Adriamycin for 4 courses preoperative then 4to 6 courses postoperative according to tumor necrosis.(P<0.039)and no significant statistical correlation between treatment protocol and outcome for metastatic patients(p<0.2364).
Two chemotherapy protocols were used for treating the studied patients with Ewing’s sarcoma either non metastatic or metastatic with statistical significant increase in the cure rate (57.58%) for the patients with non metastatic Ewing’s sarcoma who were submitted to regimen (1) using Ifosfamide + etoposide alternating with Vincristine + Adriamycin + cyclophosphamide for 54 weeks, (course every 3 weeks)(P<0.022)and no significant statistical correlation between treatment protocol and outcome for metastatic patients.(P<0.065).
65% of non metastatic cases of osteosarcoma had limb salvage surgeries as local control. 60% of metastatic cases of osteosarcoma had amputation as local control, with non significant statistical correlation between modality of local control and DFS for non metastatic patients as well as metastatic patients (P value<0.005 at 3 years).
Radiotherapy was the most common type of local control for non metastatic cases of Ewing’s sarcoma (56.41%of cases). Followed by Radiotherapy & surgery in (35.9% of cases) then Surgery alone in (7.69% of cases) while 57.14%of metastatic cases of Ewing’s sarcoma had Radiotherapy, 28.57%Radiotherapy & surgery the remaining 14.29% had Surgery alone as local control with no significant statistical correlation between DFS and modality of local control for non metastatic patients as well as metastatic patients (P<0.07 at 3 years and P<0.06 at 3 years for both groups respectively).
60.53%of non metastatic cases of osteosarcoma had tumor necrosis less than 90% and 87.5%of metastatic cases of osteosarcoma had tumor necrosis less than 90%.
Progressive disease after response (either locally or distant metastasis) and complications of treatment were the common causes of death for non metastatic cases of osteosarcoma, Recurrence and Progressive disease from start were the common causes of death for non metastatic cases of Ewing’s sarcoma, while progressive disease from the start was the common cause of death for metastatic cases of osteosarcoma as well as for metastatic cases of Ewing’s sarcoma.
For dead cases of non metastatic osteosarcoma the mean time for death from time of diagnosis was 12.66±12.1month compared to 8.71±6.1month for metastatic cases of osteosarcoma. For dead cases of non metastatic Ewing’s sarcoma the mean time for death from time of diagnosis was 11.18±3.9month. Regarding metastatic cases of Ewing’s sarcoma it was 6.27± 5.06 month.
1year disease free survival for non metastatic cases of osteosarcoma was 51.72%. For metastatic cases of osteosarcoma 1year disease free survival was 0%. 1year disease free survival for non metastatic cases of Ewing’s sarcoma was 64.52%.while for metastatic cases of Ewing’s sarcoma it was 15.38%.
1year overall survival for non metastatic cases of osteosarcoma was 78.57%.while for metastatic cases of osteosarcoma it was 25%. For non metastatic cases of Ewing’s sarcoma it was75.54%and for metastatic cases of Ewing’s sarcoma it was 25.45%.