Synovial sarcomas usually metastasize after >5 years: a multicenter retrospective analysis with minimum follow-up of 10 years for survivors.

BACKGROUND: Synovial sarcoma (SS) is a malignant soft tissue sarcoma with a poor prognosis because of late local recurrence and distant metastases. To our knowledge, no studies have minimum follow-up of 10 years that evaluate long-term outcomes for survivors. PATIENTS AND METHODS: Data on 62 patients who had been treated for SS from 1968 to 1999 were studied retrospectively in a multicenter study. Mean follow-up of living patients was 17.2 years and of dead patients 7.7 years. RESULTS: Mean age at diagnosis was 35.4 years (range 6-82 years). Overall survival was 38.7%. The 5-year survival was 74.2%; 10-year survival was 61.2%; and 15-year survival was 46.5%. Fifteen patients (24%) died of disease after 10 years of follow-up. Local recurrence occurred after a mean of 3.6 years (range 0.5-14.9 years) and metastases at a mean of 5.7 years (range 0.5-16.3 years). Only four patients were treated technically correctly with a planned biopsy followed by a wide resection or amputation. Factors associated with significantly worse prognosis included larger tumor size, metastases at the time of diagnosis, high-grade histology, trunk-related disease, and lack of wide resection as primary surgical treatment. CONCLUSIONS: In SS, metastases develop late with high mortality. Patients with SS should be followed for >10 years.

[Giant cell tumor of bone with rapid malignant course]. / Riesenzelltumor des Knochens mit rapid malignem Verlauf.

The case of a 28-year-old male patient with a locally aggressive lesion of the distal tibia is presented. Following the diagnosis of giant cell tumor of bone (GCT) on biopsy and curettage, a rapid malignant course was observed with recurrence 2.5 months later. Multiple metastases appeared 6 months after initial presentation. Following initial chemotherapy according to the COSS protocol and later with carboplatin and VP-16, therapy was changed to Adriamycin and later gemcitabine due to progressive disease. Good palliation was achieved, and the patient felt well with less shortness of breath on exertion and was ambulatory with walking aids. The malignant nature of the tumor was not detected in the initial pathologic examinations. Review of the pathologic material provided histologic clues permitting the diagnosis of a primary malignant GCT with a fibrohistiocytic/fibrosarcomatous component. Malignancy in a giant cell tumor is a much debated diagnostic dilemma when a frank sarcomatous component is lacking. Cytologic atypias and flame-like tufts of infiltration of soft tissue are important clues. Surgical treatment should be commensurate. Monotherapy with Adriamycin or gemcitabine can be considered in order to inhibit the disease progression.

Zürich experience with preoperative, high dose methotrexate-containing chemotherapy in patients with extremity osteosarcomas (OSA).

From 1979 to 1990, 37 patients with extremity osteosarcomas, 22 of them males and 15 females, median age 19 years, received pre- and postoperative chemotherapy. The period of observation, calculated from after the primary operation, ranged from 1-114 months, median 25 months. After preoperative chemotherapy, 9 (24%) underwent a primary amputation, in 28 (76%) a limb salvage procedure was possible, 4/29 (14%) later developed local recurrences, metastases were diagnosed in 8/9 amputees and 5/28 after limb-sparing surgery. The five-year disease-free and overall survivals after amputation are 11% and 33%, respectively, compared to 68% and 75%, respectively (p = 0.001 and 0.018, respectively, for long rank). Results of histologic assessment after preoperative chemotherapy are of significant prognostic impact. The earlier prognostic groups of 0%-49% necrosis versus 50%-100% necrosis were statistically no longer suitable for distinguishing useful prognostic groups. In this second analysis, patients with 0%-79% necrosis versus 80%-100% necrosis had 5-year disease-free survivals of 81% versus 44% (p = 0.032) and 5-year overall survivals of 88% In summary, 24% of our patients with extremity osteosarcomas, most of them with large primaries close to joints, had to undergo primary amputation and had only a 33% 5-yr-survival, whereas limb salvage procedures with pre-and postoperative chemotherapy were associated with a 5-yr survival of 75% and thus had no adverse impact. Careful selection of patients for successful management of osteosarcomas is important; necrosis of 80% and more after preoperative chemotherapy is a prerequisite for a favourable outcome.

[Value of punch and puncture drill biopsy in the diagnosis of tuberculous spondylitis]. / Die Wertigkeit der Stanz- und Punktionsbohrbiopsie in der Diagnostik der Spondylitis-Tbc.

Thanks to a specially designed guide instrumentarium, punch and drill aspiration biopsy for the vertebrae and intervertebral spaces have become routine diagnostic procedures which any patient can be expected to tolerate. With the authors' puncture technique material can be obtained from a single vertebra at various heights or simultaneously from different segments with the drilling cannula. For an experienced osteopathologist histologic assessment of the tissue cylinders obtained or of aspirated material presents no problems. Percutaneous removal of samples by this method avoids an open diagnostic intervention with its considerably greater attendant risks, or enables a therapeutic intervention to be prepared with greater precision. The authors report on their own experience of drill aspiration biopsy in 35 fully documented patients and discuss considerations regarding indication and confirmation of the diagnosis.

[Occurrence of pannus in arthrosis]. / Das Vorkommen von Pannus bei Arthrose.

Eight human femoral heads, surgically removed from patients with the clinical diagnosis: coxarthrosis or necrosis, were examined. In six of these we found severe arthrosis combined with synovitis and pannus formation. The pannus appeared as granulation tissue or fibrous connective tissue covering the surface of the cartilage or growing into it. In most cases the pannus showed a destructive appearance, although newly formed cartilage repair tissue was also observed. There was no indication for a pannus originating from the bone marrow. We therefore assume that we are dealing with a pannus of synovial origin. The possibility of a local transformation of chondrocytes into fibroblasts, is also discussed. The earlier reports concerning the occurrence of arthrosis in combination with pannus, are contradictory. We suggest that coxarthrosis can occur both with and without pannus. These two possibilities may represent different forms of arthrosis.

[The Zurich experience with preoperative high-dose methotrexate in osteosarcomas]. / Zürcher Erfahrungen mit präoperativem, hochdosiertem Methotrexat bei Osteosarkomen.

15 consecutive patients with osteosarcoma underwent preoperative chemotherapy with high dose methotrexate (HDMTX) containing regimens according to the T7 or T10 protocols of ROSEN, Preoperative chemotherapy was well tolerated and did not impair surgical procedures. 67% of the patients responded clinically with reduction of pain and tumor size. Histologic examination of the tumor after preoperative chemotherapy revealed extensive necrosis in 53% of patients. In a retrospective analysis, patients with extensive necrosis (group B) were compared with those with little or no necrosis (group A). Patients from group B had a longer relapse free and overall survival period than group A. In addition, patients of group A had significantly higher initial levels of alkaline phosphatase than group B. The incidence of a 2.5-fold increase of the transaminases 2-3 days after HDMTX was significantly greater in patients of group B compared to group A. In the absence of documented necrosis after chemotherapy according to the T7 or T10 protocols, further use of HDMTX is not indicated. New aspects on the treatment of osteosarcoma, derived from recent publications, are discussed.

Histomorphological investigations of coxa femoral ends following double-cup arthroplasty according to Freeman.

Of 20 shell prostheses inserted in cases of early coxarthrosis in our clinic during the period from mid-1976 to the end of 1978, three had to be removed. Morphological examinations, employing different techniques, were made of the coxa femoral ends which were removed. The foreign body reaction of the bone tissue beneath the cement within the prosthetic shell and the biomechanical response were investigated on the basis of macroscopic and microscopic structural analyses. Dependent upon the time elapsed, the exposed spongiosa surface was observed to have become remodeled to a thin cortical protective layer. Independent of time between insertion and removal of the prosthetic shell, a continuous wide layer, consisting of cellular and fibrous tissue, was formed between the bone and the cement (which remained intact) even after the formation of the above-mentioned cortical bone substrate.

[Differentialdiagnostic problems in fibrosarcoma of the foot (author's transl)]. / Differnetialdiagnostische Probleme beim Fibrosarkom am Fuss.

3 cases of fibrosarcoma of the foot are presented. Despite a history of pain up to several years the first radiological examination did not reveal any signs of a malignant tumor. X-rays taken 1/2 to one year later showed a localized osteolytic process. Then the diagnosis was established by biopsy. Consecutive x-rays and timing for a biopsy are discussed.

[Differential diagnosis of tumors: plasma cell osteomyelitis--Ewing's sarcoma (author's transl)]. / Zur Differentialdiagnose von Geschwulstkrankheiten: Plasmazelluläre Osteomyelitis--Ewing-Sarkom.

Radiographical changes of the left humerus, diaphysial, partly subcortical partly periostal suspect for Ewing's Tumor alarms the surrounding of a 15-year-old boy. By a rapid and exact examination as well as by an therapeutical punction the severe suspect of Ewing's Tumor could be eliminated. The tumore symptoms similarity of the plasmazellular Osteomyelitis is discussed.

Intraosseous ganglion (author's transl). / Das intraossäre Ganglion

The intraosseous ganglion must be always included in the differential diagnosis of cystic epiphysial bone lesions, which are localised in the vicinity of a joint, especially since this bone disease can be succesfully operated on. The preoperative diagnosis can be established with the help of conventional and tomographic x-rays examination. A communicating chanel, which is recognizable in tomographic cuts as a lucent line between the ganglion and a joint, is a pathognomic finding. The interpretation of the radiological symptoms can be difficult in those cases in which the lesions are not yet well developed or in which concomitant degenerative joint changes are present.