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Background: The quantitative accuracy of MRI in predicting the intraosseous extent of primary sarcoma of bone has not been definitively confirmed, although MRI is widely accepted as an accurate tool to plan limb salvage resections. Because inaccuracies in MRI determination of tumor extent could affect the ability of a tumor surgeon to achieve negative margins and avoid local recurrence, we thought it important to assess the accuracy of MR-determined tumor extent to the actual extent observed pathologically from resected specimens in pediatric patients treated for primary sarcomas of bone., Questions/purposes: (1) Does the quantitative pathologic bony margin correlate with that measured on preoperative MRI? (2) Are T1- or T2-weighted MRIs most accurate in determining a margin? (3) Is there a difference in predicting tumor extent between MRI obtained before or after neoadjuvant chemotherapy and which is most accurate?, Methods: We retrospectively studied a population of 211 potentially eligible patients who were treated with limb salvage surgery between August 1999 and July 2015 by a single surgeon at a single institution for primary sarcoma of bone. Of 131 patients (62%) with disease involving the femur or tibia, 107 (51%) were classified with Ewing's sarcoma or osteosarcoma. Records were available for review in our online database for 79 eligible patients (37%). Twenty-six patients (12%) were excluded because of insufficient or unavailable clinical or pathology data and 17 patients (8%) were excluded as a result of inadequate or incomplete MR imaging, leaving 55 eligible participants (26%) in the final cohort. The length of the resected specimen was superimposed on preresection MRI sequences to compare the margin measured by MRI with the margin measured by histopathology. Arithmetic mean differences and Pearson r correlations were used to assess quantitative accuracy (size of the margin)., Results: All MR imaging types were positively associated with final histopathologic margin. T1-weighted MRI after neoadjuvant chemotherapy and final histopathologic margin had the strongest positive correlation of all MR imaging and time point comparisons (r = 0.846, p < 0.001). Mean differences existed between the normal marrow margin on T1-weighted MRI before neoadjuvant chemotherapy (t = 8.363; mean, 18.883 mm; 95% confidence interval [CI], 14.327-23.441; p < 0.001), T2-weighted MRI before neoadjuvant chemotherapy (t = 8.194; mean, 17.204 mm; 95% CI, 12.970-21.439; p < 0.001), T1-weighted after neoadjuvant chemotherapy (t = 10.808; mean, 22.178 mm; 95% CI, 18.042-26.313; p < 0.001), T2-weighted after neoadjuvant chemotherapy (t = 10.702; mean, 20.778 mm; 95% CI, 16.865-24.691; p < 0.001), and the final histopathologic margin. T1-weighted MRI after neoadjuvant chemotherapy compared with the final histopathologic margin had the smallest mean difference in MRI-measured versus histopathologic margin size (mean, 5.9 mm; SD = 4.5 mm)., Conclusions: T1 MRI after neoadjuvant chemotherapy exhibited the strongest positive correlation and smallest mean difference compared with histopathologic margin. When planning surgical resections based on MRI obtained after neoadjuvant chemotherapy, for safety, one should account for a potential difference between the apparent margin of a tumor on an MRI and the actual pathologic margin of that tumor of up to 1 cm., Level of Evidence: Level III, diagnostic study.

Limb salvage is widely used but has different disadvantages. Attempting to establish a new limb conservative procedure, we treate patients with malignant bone tumor by intraoperative microwave heating on tumor bearing bone in place and neoadjvant chemotherapy. The tumors were localized at distal femur in 16, proximal tibia in 5, femur shaft in 2, and illia in 2. Pathological diagnosis showed osteosarcoma in 16 patents, parosteoal osteosarcoma in 4, chondrosarcoma in 3, and leiomyosarcoma in 2. The stage of tumor was II b in 18 patients, II a in 6, and III b in 3. Operative technique was wide resection for tumor in soft tissue, protecting the surrounding tissue from heat injury by copper net, and cheating tumor bone at 50 C for 15 minutes. Follow-up varied from 4 to 180 months (mean 57 months). The 5-, 10-years surviral rates were 70.19% and 54.83% respectively. Functional results of 21 patients were > 15 points by Enneking system. The advantages of the method and keeping the shape and continuity of the tumor bearing bone without osteotomy and internal fixation, controlling the time and temperature of heating treatment easily, benefiting for bone remodeling of heated bone. The indications, complications, and surgical procedure of this method were discussed in detail.

This paper presents 5 patients with repeated recurrence of osteosarcoma (RROS). The primary focus of 3 patients were in the distal portion of femur, and 2 patients were in the proximal portion of tibia. Three patients, whose chest X ray film were negative, were treated by amputation and chemotherapy. Two patients had isolated metastatic focus 1.5 cm in diameter in lung, were treated by amputation after 1 week of chemotherapy and then treated by lobectomy after 2 weeks of chemotherapy. After operation, the chemotherapy was carried out for 3 courses of treatment. The roentgenogram of chest and affected limb were taken once every two months. There were metastatic focuses found in the lung of 1 patient and in the distal portion of femur of 2 patients. One patient was operated on for 4 times. Up to now, 3 patients have been living for 5 years and 2 patients for 6 years after operation.

The authors present 15 cases of discrepancy of the lower limbs > 5 cm, in children in whom the histological diagnosis was Ewing's sarcoma or osteosarcoma, who were treated using polychemotherapy or radiotherapy or both in some cases and limb-salvage surgery. Ten of these patients required compensation for discrepancy by limb lengthening. In three patients, a second lengthening had to be carried out. A shortening of the contralateral femur was performed in two cases, and in another two, a femur-tibia epiphysiodesis was done. The average lengthening achieved was 8.1 cm with a healing index of 41 days/cm. Leg-length discrepancy in the treatment of sarcomas of the musculoskeletal system is an important sequela, especially when treating children. Compensation for this can be obtained by lengthening healthy bone, with a complication rate similar to that of other etiologies. The difference compensated depends on the biological capacity of bone regeneration of the individual and, therefore, is a definitive solution.

From August 1990 to June 1993, 30 patients with osteosarcoma of lower extremities were treated with chemotherapy by hyperthermic regional isolated perfusion. There were 19 male and 11 female with a mean age of 21 (15-28) years. All of the tumors were located in the lower limbs: 20 on the femora, 9 on the tibiae and 1 on the fibula. Chemotherapy was going on for 60 minutes during hyperthermic regional isolated perfusion. Temperature was kept at 42 degrees C in deep soft tissue around the tumor during perfusion. The results showed that local edema of the limbs were reduced observably, tumors were shrunken and hardened after perfusion. Perimeter of the limbs were decreased and mobility of the limbs increased. Pathological examination indicated that all of the tumors responded well to the chemotherapy by perfusion and 90%-95% of the osteosarcoma cells were destroyed. Two cases were complicated with compression syndrome, and 1 with renal failure. The authors would suggest that hyperthermic regional isolated perfusion is an effective chemotherapeutic method in management of malignant tumors of limbs.

The paper presents results of treatment of 16 patients operated on because of giant cell tumor. Frequent local recurrence after curettage makes the authors to suggest more common use of the thermal or chemical destruction of the neoplastic cells to achieve oncologic sterility.

The clinicopathologic features of osteosarcoma in 12 children younger than 16 years of age treated at The Children's Hospital and Dana-Farber Cancer Institute, Boston, during a 70-year time period are presented. Only one of six children treated before 1972 is a long-term survivor. Four of six children (67%) treated after 1972 are disease-free with an average follow-up of 8.8 years. The year 1972 marked the onset of use of effective chemotherapy in osteosarcoma, namely, high-dose methotrexate and leucovorin rescue. It would appear that the pathologic features and behavior of osteosarcoma in young children is similar to that of osteosarcoma in older children and adolescents. A combination of complete (wide) surgical resection or amputation and aggressive chemotherapy offers the best chance of long-term survival.

We report a case of systemic intraosseous lipomatosis involving the proximal femur, both ends of the tibia, and the tarsal and metatarsal bones. The lesions progressed during a five-year follow-up with a pathological fracture of the tibial plateau. CT scans were characteristic and helpful in diagnosis but MR imaging added little information. Intraosseous lipomatosis is a hamartomatous malformation due to hyperplasia of adipose tissue, and is fundamentally different from solitary benign intraosseous lipoma. Management involves reconstruction of any pathological fracture. Large progressive lesions should be treated by curettage and grafting in an attempt to prevent such fractures.

Between March 1983 and September 1988, 22 patients with non-metastatic malignant fibrous histiocytoma MFH of bone of the extremities were treated with two regimens of neo-adjuvant chemotherapy successively activated. Preoperatively, the patients received moderate doses of methotrexate and cisplatinum-Regimen 1- or high dose methotrexate, cisplatinum and adriamycin-Regimen 2. Cisplatinum was delivered intraarteriously, the other drugs intravenously. Limb salvage surgery was performed in 20 patients, and 2 patients were amputated. The surgical margins were adequate (radical or wide) in 18 cases and inadequate (marginal) in 4. The histologic response to chemotherapy was good (90% or more tumor necrosis) in 8 patients. In both regimens postoperative chemotherapy was tailored according to the grade of necrosis determined by preoperative treatment on the primary tumor. At an average follow-up of 40 months (15-70), 15 patients (68%) remained continuously disease-free and 7 relapsed with metastases. No local recurrences were observed. Regimen 2 was slightly more effective than Regimen 1 in terms of good histologic response (5/10 vs 1/12) and continuous disease-free survival (8/10 vs 7/127). The results demonstrate that, as in osteosarcoma, in non-metastatic malignant fibrous histiocytoma of bone in the extremities a high percentage of patients can be cured with neoadjuvant chemotherapy and that in most of them limb sparing surgery is possible and safe.

Multifactorial analysis of patients with osteosarcoma of the distal femur and proximal tibia, recorded in the Swedish Cancer Registry during 1958 through 1968, disclosed a 5-year survival of 15.1% for femoral osteosarcomas and 38.1% for tibial tumours. The prognosis was better in adults than in children and better in males than in females. Tumour size, soft tissue involvement, the presence of pathological fracture and the duration of symptoms before treatment influenced the prognosis. The best treatment for the tibial lesions was high amputation alone, whilst for the femoral tumours primary ablative surgery was not superior to combined high-dose radiotherapy and delayed amputation. The main cause for the higher survival rate for tibial neoplasms seemed to be the fact that they were less advanced on admission than those of the distal femur. The findings emphasize the importance of early diagnosis and treatment for improved survival.

The authors describe the theoretical principles and the experimental and clinical investigations that form the basis for the technique of selective hyperthermic perfusion of the limbs, with or without the addition of antiblastic agents. Neoplastic cells are much more sensitive to heat than normal cells, undergoing necrosis at a temperature of 42.5 to 43 degrees C. The authors discuss the histological appearances and the clinical results obtained in the treatment of 29 cases of malignant neoplasms of the limbs in which antiblastic hyperthermic perfusion was carried out one month before surgical removal of the neoplasm. The long term results show that antiblastic hyperthermic perfusion significantly reduces the risk of local recurrences in cases of resection and in some cases enables amputations rather than disarticulations to be performed. This type of treatment is particularly indicated in the highly undifferentiated and aggressive forms such as osteosarcoma, malignant fibrous histiocytoma, adamantinoma, fibrosarcoma, giant-cell sarcoma, Ewing's tumour, and synovioma.

Three cases of osteoid osteoma and one of benign osteoblastoma in children are described. The main complaint was severe pain which was worse at night; it was relieved by aspirin or other analgesics. The diagnosis was made on clinical and radiological grounds and was confirmed on histological examination of the central nidus removed at operation. The pain was relieved in the patients with osteoid osteoma, and it was very much less after operative removal of the benign osteoblastoma. Both conditions are probably variations of the same disease process, depending on the anatomical site and the type of bone affected.