Aim

Purpose of this study was to review a single Institution experience and results of management of extraskeletal osteosarcoma (OGS), with emphasis on the role of combined treatment consisting of surgery and adjuvant chemotherapy.

Introduction: Extraskeletal osteosarcoma is a rare malignant tumor of the soft tissues. Overall, this malignancy has been associated with worse local control and overall survival rates than its skeletal counterpart despite multimodal approach. Purpose of this study was to review a single Institution experience and analyse results of management to identify factors affecting the outcome.

Methods: Retrospective study of 48 patients observed between 1966 and 2007 was undertaken. Of the total, 36 patients were admitted and managed at our Institution while 12 patients were sent for consultation and therefore not included in this study. Clinico-pathologic features and details of treatment of all 36 patients were reviewed and correlated with outcome. Updated follow-up was available in all patients.

Results: There were 21 males and 15 females, mean age was 53.6+/−19.3 years (range 14–84 yrs); 23 patients (63.9%) presented with localised disease while distant metastases were present in 13 patients (36.1%). Surgery consisted of a limb-salvage procedure in 25 patients (69.4%), amputation in 9 patients (25%) and 2 patients were considered inoperable (5.6%). Postoperative radiation therapy was given to 6 patients (16.7%) and multiagent chemotherapy administered to 19 patients (52.8%). At mean follow-up of 5.8 years, 23 patients had died of disease, expected 5 and 10 year overall survival rates were 41% and 31%. Tumor size and age at presentation were the most important predictors of survival while chemotherapy showed a trend towards improved survival in patients with localised disease.

Discussion and Conclusion: Extraskeletal osteosarcoma was associated with substantially worse prognosis than skeletal osteosarcoma despite multimodal management.

Introduction: Osteogenic Sarcoma is one of the most common malignant bone tumors in the younger population. The advances in chemotherapy in conjunction with surgery has improved the survival rates from less than 20% in 1970s to more than 70% in 1990s. Advanced imaging, better histopathological techniques, availability of bone banks and newer chemotherapeutic agents have made limb salvage surgery a viable option even in advanced stages of the disease. We reviewed the outcome and analysed the complications of patients with Osteogenic Sarcoma at our institution.

Materials and methods: The objective of our study was to evaluate our experience with the treatment of osteogenic sarcoma and to do a survival analysis. It was a retrospective study consisting of 20 patients who were treated between 1990–1998. Mean age was 17 years with equal distribution of males and females. Of all the patients, 18 had stage II b disease and 2 patients had stage III disease. The quetionnaire focused on the initial mode of presentation of the patients, their stage of disease, the type of neo-adjuvent chemotherapy used and the type of surgery they underwent

Results: Majority of the patients presented at least six months after the onset of symptoms with pain and swelling being the most common modes of presentation. Majority of our patients had open biopsies done outside our hospital and received non-uniform neo-adjuvant chemotherapy. Distal femur was involved in 60% of the cases followed by proximal and distal tibia. Limb salvage surgery was performed in 90% of the cases, while the rest had primary amputation due to the extent of the disease. Autoclaved bone, allografts, free fibular grafts and custom made prostheses were used to reconstruct the intercalary defects left by the resection of the tumor. Polyuria was our main early post operative complication followed by wound infection. Only one patient had a local recurrence and 7/20 had distant metastasis in our follow-up (mean 2.5 years). Mortality rate was 25% in our study.

Conclusion: Limb salvage surgery was performed quite successfully with only one local recurrence in all of our patients with stage II b disease. Most of our patients who developed late distant metastasis had a non-uniform and uncontrolled chemotherapy protocol which could have adversely affected our final outcome.

Introduction: Developmental dysplasia of the hip is a congenital anomaly that is best treated immediately after birth. The deformity, if not treated early, usually progresses with time and becomes more complicated to treat. Its treatment in an older child requires a complete understanding of the primary disease process as well as correction of the secondary anatomical deformities which have matured at that time. In this study, we present our experience with the treatment of this complex problem in older children.

Materials & Methods: All patients who presented to us with idiopathic DDH beyond 18 months of age at the Aga Khan University Hospital were included in the study. We operated on 20 patients with 25 hips from 1990 to 1998. There were 13 females and five patients with bilateral disease. The mean age was 48 months and the average follow-up was 13 months. Patients were classified according to the Tonnis class and the acetabular index and the central edge angle were measured both pre- and post-operatively. Functional evaluation was then done using MacKay’s scoring system.

Results: There were 7 (28%) hips in the Tonnis class II, 10 (40%) hips in Tonnis class III, and 8 (32%) hips in the Tonnis class IV at the time of presentation. We were able to achieve closed reduction in five (20%) hips, and satisfactory open reduction without any bony procedure was performed in four (16%) hips. The triple procedure of open reduction, femoral and innominate osteotomy was performed in 16 hips in 13 patients. The acetabular index improved from an average of 350 pre-operatively to an average of 180 post-operatively. The MacKay score was good to excellent in 22(88%) hips and we had a failure in 1(4%) hip joint. The Severin class was good to excellent in 21 (82%) patients at the time of final evaluation. There were 3 cases of posterior subluxation and 1 case each of avascular necrosis and myositis ossificans.

Conclusions: Tonnis class and age of the patients are important determinants of the final outcome. The triple procedure of open reduction, femoral and innominate osteotomy gives the best results in an older child with DDH.