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[Sarcomas associated with Paget's disease].

The authors report 12 cases of Paget's disease with sarcomatous degeneration that were observed among 311 patients with Paget's disease hospitalized in the Montpellier Rheumatology Clinic. They compare their experience with information they were able to extract from the literature in French and English. The frequency of such degeneration in cases of Paget's disease is difficult to determine but did not appear to exceed 1%. Degeneration rarely occurred before the age of 50 years, affected men twice as frequently as women, and occurred particularly in cases of diffuse Paget's disease, mainly in the femur or the humerus; the rachis was rarely affected. Pain was the main symptom, was practically constant, was remarkable because of its permanence and its intensity. Tumefaction was frequently seen. Pathological fractures were seen in almost a third of the patients with sarcomas of the long bones. Radiculo-medullary compression characterized the rare cases with involvement of the vertebral column or the sacrum. Radiography showed rupture of cortical layers with invasion of the soft parts without any periostal reaction. Histological investigation confirmed the diagnosis by demonstrating one of the three types of osteogenic sarcoma: osteosarcomas were the most frequent. Less frequently giant-cell sarcomas or reticulosarcomas were found. The affected patients nearly always died, survival at five years being rare. Treatment, amputation or radiotherapy, was disappointing.

Aged↗

[Our experience with endoprosthesis in surgery for bone tumors].

Endoprosthesis is a method allowing a valuable substitution of bone defects (articular ends of long tubular bones, both articular ends of the bones making the articulation, whole long tubular bones with articulations). The employment of endoprostheses is expedient for the substitution of defects of the breast bone, pelvis, hand bones as well. Endoprosthesis in combination with preoperative radiotherapy and post-operative modern chemotherapy allows preserving the extremities in certain patients with osteogenic sarcoma, chondrosarcoma, reticulosarcoma, chondrosarcoma and solitary plasmacytoma. The experience with 204 operations of endoprosthesis of bones is presented.

Bone Neoplasms↗

[Orthopedic rehabilitation of children after combined treatment of malignant bone tumors].

The report is based on the data concerning 27 children who were given radiotherapy and chemotherapy for osteogenic sarcomas (14), Ewing's sarcomas and reticulosarcoma (10) and malignant giant cell tumours (2). The reconstructive conservative treatment (7 patients) were referred for prevention of contractures and fractures. The orthopaedic surgical correction of the appearing false joints, deformations and defects of the bones (20 patients) was performed 4 to 5 years after the termination of the primary treatment. During the reconstructive period 3 patients with malignant giant cell tumours and osteogenic sarcoma died because of metastases. The authors have made a conclusion about the advantage of extrafocal compression and distraction osteosynthesis over other methods of fixation and correction of deformations in children after the treatment of malignant bone tumours.

Adolescent↗

[Multipotent primary bone sarcoma].

The results of clinical and roentgen-morphological studies on 17 cases of multipotent primary sarcoma of the bone are presented. The difficulties of the tumor recognition are due to the fact that it occurs rarely, has no specific clinical-roentgenological picture and shows marked tissue polymorphism. Most frequent are differentiations of the type of osteogenic sarcoma, chondrosarcoma, reticulosarcoma with simultaneous combination with areas having the structure of Ewing's tumor, or extensive fields of undifferentiated primitive tumor cells. Further studies are necessary to decide whether or not this tumor is an independent form among other known skeletal neoplasias.

Adolescent↗

Radiotherapy of malignant lymphomas.

Radiotherapy of non-Hodgkin's lymphomas situated in the central nervous system is basically similar to the treatment applied to any other part of the body. There are two requirements: the first is the prophylactic irradiation of the CNS in a systemic disease of the reticuloendothelial system mainly in acute lymphatic leukemia; the second is the treatment of solitary tumors in the CNS. Radiotherapy can improve dramatically the results obtained with intrathecal chemotherapy, particularly in leukemic involvement of the CNS in childhood. The fractionated single radiation dose should be increased slowly and total doses of 2 500 rd should not be exceeded. Prophylactic radiotherapy of the CNS in acute leukemia has to include the entire subarachnoid space. It is therefore necessary to irradiate the entire skull and spinal cord. This treatment schedule and its results will be discussed. There is a tendency to sue prophylactic radiotherapy in other systemic diseases of the RES as well. The indications and contraindications for this will be discussed. The methods and results of treatment of focal lesions with regard to the morphologic tumor pattern are discussed. Rare primary tumors of the CNS which have the histological features of lymphoma-sarcoma or reticulosarcoma have the best prognosis as far as radiotherapy is concerned.

Brain Neoplasms↗