Twenty-five consecutive patients with chordoma, chondrosarcoma, malignant lymphoma, myeloma, osteosarcoma, malignant fibrous histiocytoma, malignant schwannoma, fibrosarcoma, synovial sarcoma, rhabdomyosarcoma, liposarcoma, undifferentiated sarcoma in the pelvis were treated. Ten local recurrences (48%) occurred among twenty-one patients who had local tumor resected. Eight distant metastases (42%) occurred among the nineteen patients with localized disease at presentation. The ten-year overall survival was 32%. Patients with pelvic bone and soft tissue sarcoma continue to have a poor prognosis with standard treatment. In addition to a significant risk of local failure, these patients are at high risk for systemic relapse. Preoperative and/or postoperative intraarterial chemotherapy was useful for local tumor control in patients with pelvic sarcomas.
Limb-saving surgery plays an increasingly important role in the treatment of malignant tumors of the extremities.This type of surgery may pose special problems in longterm survivors. The different possibilities of reconstruction after a tumor resection and repercussions in growth are discussed.Case 1. In this ten year old girl with osteosarcoma of the left distal femur, wide excision of the tumor site and a total knee replacement were done in September 1983, Two years postoperatively she presented late hematogenous infection of total knee replacement. Three times of surgery, debridement only, remove of prosthesis and two stage revision, wase done. In September 1989 rotationplasty were done because of uncontrolled infection. Case 2. In this twentytwo year old female with malignant fibrous histiocytoma of the left proximal tibia, wide excision of the tumor site and a total knee replacement were done in December 1983. Four years postoperatively she presented patellar tendon rupture, In July 1988 repair of the tendon with the use dacron ligament prosthesis to reattatch the tendon to the tibial component were done. To date, they had been followed for seven years with good function of limb, but without any indication of either local recurrence or metastases.The role of recostruction by means of a rotation plasty versus other methods of recostruction, and the repair of the tendon using ligament prosthesis and autotendon to reattatch the tendon to the tibial component is specially cosidered.
Clinicopathologic analyses and therapeutic results of ten low-grade chondrosarcomas (Grade I) are presented. Seven patients were managed with a wide en bloc resection, two patients with an en bloc resection, and one with curettage. The duration of follow-up ranged from two to eleven years, with a mean of six years. Two chondrosarcomas recurred locally, a repeat excision was done, and finally the patients died eleven years after initial surgery. Radiographic findings of low-grade chondrosarcomas characteristically demonstrated an expanded and destroyed cortex. All ten tumors were studied immunohistochemically by using α1-antitrypsin (AT), α1-antichymotrypsin (ACT), and S-100. The results indicate that AT and ACT may not be useful indicators of malignancy. Diagnosis based not only on histologic but also radiographic findings may be needed in low-grade chondrosarcoma.
The histogenesis of malignant fibrous histiocytoma (MFH) is controversial. To elucidate the cellular origin and characteristics of this neoplasm, the authors analyzed cell lines grown from 17 patients (15 soft tissue MFH and 2 bone MFH) by using light and electron microscopy, immunocytochemistry, enzyme cytochemistry, and functional tests for receptors for the Fc portion of immunoglobulin (Fc receptors) and immunophagocytosis. Each culture exhibited a storiform/pleomorphic pattern with mixed cellular populations consisting of spindle cells, polygonal cells, and bizarre giant cells; these morphologic features corresponded to the histologio characteristics of the primary tumors. The cells in each MFH line displayed histiocytic functional markers such as lysosomal enzymes, Fc receptors, and immunophagocytosis. However, these cells differed from monocyte-derived macrophages (histiocytes) in immunoreactivity; the MFH cells expressed a mesenchymal antigen (FU3) distributed among perivascular cells and fibroblasts but demonstrated no positive reactions with Leu-M1 (CD15) and Leu-M3 (CD14), which recognize the cells of the monocyte-macrophage lineage. In conclusion, these findings suggest that MFH is not a tumor of true histiocytes but of facultative histiocytes showing mesenchymal differentiation in vitro. Chromosomal analysis performed in one MFH line demonstrated abnormal karyotypes; the modal chromosome number was 58, with 5 marker chromosomes.
Ipsilateral distal femoral fracture after postoperative femoral neck fracture is a rare complication and treatment is often difficult. We treated three cases in which retrograde supracondylar intramedullary nails were used for internal fixation. The patients consisted of three females with ages ranging from 75 to 89. Two patients underwent hemiarthroplasty and another underwent Gamma nailing. According to AO classification, they were classified into A1 (2 cases) and C1 (1 case), respectively. All patients tended to fall down easily after postoperative femoral neck fracture.Consequently all fractures healed within four months. The average knee range of motion was —10 to 93 degrees. Clinical results were evaluated according to Neer's criteria. All cases were ranked as unsatisfactory. In generally, walking ability tended to deteriorate because multiple fractures occurred ipsilatelly. One case developed secondary femoral fracture at the mid shaft postoperatively, because of jamming between the distal tip of the cement and proximal tip of the intramedullay nail.Use of retrograde supracondylar intramedullary nail is effective for this type of fracture, but we have to be careful about the length of the intramedullary nail before surgical planning. As rigid fixation is difficult for the osteoporotic bone, post operative treatment is also important.
We describe the evaluation of intra-arterial chemotherapy response for bone and soft-tissue high grade sarcoma. Subjects included 41 with 22 bone and 19 with soft-tissue sarcoma. Surgical stage was I-B (1), II-B (36) and III (4). Pre-operative intra-arterial chemotherapy was performed on 17 patients, pre- and post-operative on 11, and post-operative on 13. Limb salvage procedures were performed on 32 patients (78%) and primary amputation on 9. Over all disease free survival at 10 year follow-up was 54% (bone tumors) and 61% (soft-tissue tumor). 17 patients had CDF, 5 patients NED, 3 patients AWD and 16 patients DOD at a median follow-up period of 31 months (6-202). The prognosis was correlated with the evaluation of change in tumor size, pain and imaging findings, but did not correlate with the change in histopathology and serum alkalinephosphatase.
A seventeen-years-old girl who was diagnosed as suffering from tethered conus syndrome accompanied by lipoma in the lesion of spina bifida was investigated and treated in our institution.This syndrome is rarely seen and usually diagnosed in childhood. In this case, diagnosis was made by computerised tomography, physical examination, plane X-p and myelography.Computerised tomography was of great importance for the evaluation of invasion and also for confirmation of the level of the location of conus in the spinal canal.Radical excision was necessary for this lipoma, although many authors state it is difficult to perform such an excision redically in most cases. Decompression of the spinal cord and releasing the fibrous band are essential for the treatment of this syndrome.This paper reports a rare case of tethered conus syndrome acompanied by lipoma in the lesion of spina bifida, which was succesfully treated surgically.
Maffucci's syndrome is a congenital, non-hereditary mesodermal dysplasia manifested by multiple enchondromas and hemangiomas. It is associated with diverse secondary musculoskeletal deformities. A high proportion of the enchondromas undergo malignant degeneration.We report one case of Maffucci's syndrome in a 17-year-old girl.
We described a case of chondrosarcoma involving the proximal end of the humerus— which was treated by the total shoulder joint replacement (Michael Reese type) after the wide resection.The case was a 43 years old man who was admitted to our hospital with pathologic fracture of the right proximal humerus in April 1983. This case was treated with intra-arterial high-dose driamycin pre- and post-operatively and total shoulder joint replacement after wide resection of the primary tumor.Histological finding of the tumor showed chodrosarcoma of Grade III (Evans). Satisfactory result was obtained at one year after the operation.
