The Scandinavian Sarcoma Group Register 1986-2001.
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Bone Neoplasms↗
Biomedical subjects
O Berlin
Publications and source records attributed to O Berlin.
Monitoring referral and treatment in soft tissue sarcoma: study based on 1,851 patients from the Scandinavian Sarcoma Group Register.
This report is based on 1.851 adult patients with soft tissue sarcoma (STS) of the extremities or trunk wall diagnosed between 1986 and 1997 and reported from all tertiary referral centers in Norway and Sweden. The median age at diagnosis was 65 years and the male-to-female ratio was 1.1:1. One third of the tumors were subcutaneous, one third deep, intramuscular and one third deep, extramuscular. The median size was 7 (1-35) cm and 75% were high grade (III-IV). Metastases at presentation were diagnosed in 8% of the patients. Two thirds of STS patients were referred before surgery and the referral practices have improved during the study. The preoperative morphologic diagnosis was made with fine-needle aspiration cytology in 81%, core-needle biopsy in 9% and incisional biopsy in 10%. The frequency of amputations has decreased from 15% in 198688 to 9% in 1995-1997. A wide surgical margin was achieved in 77% of subcutaneous and 60% of deep-seated lesions. Overall, 24% of operated STS patients had adjuvant radiotherapy. The use of such therapy at sarcoma centers increased from 20% 1986-88 to 30% in 1995-97. Follow-up has been reported in 96% of the patients. The cumulative local recurrence rate was 0.20 at 5 years and 0.24 at 10 years. The 5-year metastasis-free survival rate was 0.70.
Adolescent↗
Local recurrence of deep-seated, high-grade, soft tissue sarcoma: 459 patients from the Scandinavian Sarcoma Group Register.
This study was based on 459 adult patients with deep, high-grade, soft tissue sarcoma of extremities or trunk wall reported to the Scandinavian Sarcoma Group Register (1986-1993). All patients had their definitive surgery for primary tumor at a sarcoma center. The median follow-up was 7.5 (3-12) years. 204 patients are still alive. 68 patients had amputations and 391 underwent limb-sparing surgery. Among 183 patients with intralesional or marginal margins after limb-sparing surgery, 65% had postoperative radiotherapy and 9% of the 198 patients with wide margins. The local recurrence rate after limb-sparing surgery was 26%. The rate with an intralesional or marginal margin was 39% without postoperative radiotherapy versus 24% when radiotherapy was given. It was 25% after a wide margin, and no recurrences were noted among the 10 patients with a compartmental surgical margin. Among patients with a wide margin, a subset fulfilling criteria for a myectomy was defined. The local recurrence rate was 26% among these 62 and there was no advantage of myectomy over other wide margins. More radical surgical margins would improve the local recurrence rate, but this can hardly be achieved in center-operated patients without increasing the amputation rate. Instead, increased use of radiotherapy in all patients with inadequate margins, and to a larger extent in those with wide margins will improve local control.
Adolescent↗
Surgical margins, local recurrence and metastasis in soft tissue sarcomas: 559 surgically-treated patients from the Scandinavian Sarcoma Group Register.
The prognostic importance of surgical margins on local recurrence rates and metastasis-free survival (MFS) was studied in 559 patients with soft tissue sarcoma of the extremities and trunk wall. The patients were all surgically treated, but received no adjuvant treatment. The median follow-up for the survivors was 7.4 (range: 0.1 - 12.5) years. Independent prognostic factors for MFS were analysed by Cox models. The overall 5-year MFS was 0.72 (95% confidence intervals (CI) 0.68 - 0.76). High histopathological malignancy grade (relative risk (RR) 3.0; 95% CI 1.5 - 6.3) and an inadequate surgical margin (RR 2.9; 95% CI 1.8 - 4.6) were independent risk factors for local recurrence. High histopathological malignancy grade and large tumour size (> 7 cm) were the most important risk factors for metastasis. Local recurrence was associated with an increased risk of metastasis (RR 4. 4; 95% CI 2.9-6.8), but an inadequate surgical margin was not a risk factor for metastasis (RR 1.1; 95% CI 0.8-1.7). This study confirms that, as regards metastasis, tumour-related risk factors (malignancy grade and tumour size) are more important risk factors than treatment-related factors. Local recurrence was associated with an increased metastasis rate, whereas inadequate surgical margin was a risk factor for local recurrence but not for metastasis. Hence, the proposed causal association between local recurrence and metastasis is doubtful, and if it exists is a weak association.
Adolescent↗
Consequences of local recurrence of soft tissue sarcoma: 205 patients from the Scandinavian Sarcoma Group Register.
From the Scandinavian Sarcoma Group Register, information on 1,224 surgically-treated patients with soft tissue sarcoma (STS) of the extremity or trunk wall, diagnosed between 1987 and 1995, was collected. 205 patients, one third of whom were referred to a center with a local recurrence, had a total of 284 local recurrences. This analysis describes the treatment for these local recurrences, complications and risk of further recurrences. 169 patients were surgically treated for their first local recurrence. An intralesional or marginal margin was achieved in 110 of these patients, 59 of whom were also given radiotherapy. 54 of the 169 patients had a second local recurrence. The second local recurrence rate was 0.50 if the first local recurrence had been treated with only surgery with a marginal margin, compared to 0.28 if treated with surgery with a marginal margin and radiotherapy or with a wide margin (p = 0.0008). In extremity STS, the crude amputation rate for local recurrences was 0.22 (31 of 142)-i.e., higher than for primary tumors 0.09 (96 of 1065) (p < 0.0001). A high local recurrence rate after treatment outside of sarcoma centers has earlier been shown. We conclude that the consequences of local recurrence in terms of morbidity and costs justifies referral of STS patients for multidisciplinary evaluation and multimodality treatment.
Adult↗
Synovial sarcoma: identification of low and high risk groups.
BACKGROUND: Synovial sarcoma, one of the most common soft tissue sarcomas that occur in adolescents and young adults, is generally viewed and treated as a high grade sarcoma. However, the authors' own experience and some previous studies have indicated that it has a wide spectrum of biologic behavior and that low and high risk subgroups of patients with synovial sarcoma can be identified. METHODS: A total of 121 consecutive patients with synovial sarcoma (including 66 males and 55 females ages 9-74 years), treated primarily or secondarily at 2 large referral centers for musculoskeletal tumors, were included in a statistical analysis conducted to identify independent prognostic factors. RESULTS: There were local recurrences in 38 patients (31%), usually after inadequate primary surgery outside the referral centers; 64 patients (54%) developed metastasis, primarily to the lungs. The estimated 5-, 10-, and 15-year survival rates were 60%, 50%, and 45%, respectively (the mean follow-up for surviving patients was 9.8 years, with a range of 1-30 years). In multivariate analysis, independent risk factors for local recurrence included larger tumor size and primary surgical resection outside the referral center. Independent risk factors for metastasis were older patient age, tumor with poor histologic differentiation, and tumor necrosis. For tumor-related death, the independent risk factors were older patient age, tumor with poor histologic differentiation, and larger tumor size. Local recurrence was associated with a 3.66-fold increased risk of tumor-related death. A low risk group (patient age <25 years, tumor size <5 cm, and no histologic evidence of poorly differentiated tumor) with 88% overall disease free survival was identified, as was a high risk group (patient age > or = 25 years, tumor size > or = 5 cm, and poorly differentiated tumor) with 18% overall disease free survival (P < 0.001). CONCLUSIONS: The identification of low and high risk synovial sarcoma patients indicates that synovial sarcomas are not uniformly high grade tumors. It also indicates that treatment strategies should be modified for these risk groups. Adequate primary surgery is essential to both local control and outcome for synovial sarcoma patients.
Adolescent↗
The Scandinavian Sarcoma Group Register.
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Adult↗
Clinical course in synovial sarcoma: a Scandinavian sarcoma group study of 104 patients.
We analyzed treatment and outcome in 104 Scandinavian patients with synovial sarcoma in the extremities or trunk wall, diagnosed between 1986 and 1994. Only surgically treated patients without metastases at diagnosis were included. Median follow-up of survivors was 6 (3-11) years. 34 patients developed metastases. The overall 5- and 7-year survival rates were 0.76 (95% CI 0.66-0.83) and 0.69 (0.58-0.78), respectively. Large tumor size and amputation were significantly associated with impaired metastasis-free survival. Patients with local recurrence had a higher risk of metastases following the local event. Local excision with inadequate margin was associated with a higher risk of local recurrence.
Adolescent↗
Development of a novel spontaneous metastasis model of human osteosarcoma transplanted orthotopically into bone of athymic mice.
There is a pressing need for in vivo models in which potential antitumor agents can be tested for their ability to inhibit the growth and metastatic spread of human sarcomas. A recent advance in this regard has been the development of a v-Ki-ras-oncogene-transformed human osteosarcoma cell line (KRIB) that efficiently colonizes the lungs of athymic nude mice when cells (1 x 10(5)) are administered by i.v. injection. In the present study, we have utilized this cell line to develop a spontaneous metastasis model in which a small number of tumor cells are injected into the tibial bones of athymic mice. When as few as 1000 KRIB cells are orthotopically implanted into the tibial bones of nude mice, bone tumors, which are radiographically and histologically similar to primary human osteosarcoma, develop within 4 weeks. Furthermore, as in the human disease, cells from these primary tumors subsequently seed the animals' lungs, resulting in reproducible and quantifiable pulmonary metastasis evident both upon gross inspection of the lungs and histologically 6 weeks after tumor inoculation. Surgical amputation of the tumor inoculation site up to 2 weeks after tumor injection prevents pulmonary metastasis, indicating that substantial local (tibial) growth and invasion of the primary tumor for at least 2 weeks is required for subsequent metastasis. Implantation of s.c. 5000 KRIB cells fails to produce local or metastatic tumors. We anticipate that this model will prove to be a powerful tool with which to study the mechanisms of human osteosarcoma growth and pulmonary metastasis, and to assess the efficacy of promising therapeutic agents.
Animals↗
The combined tensor fasciae latae/rectus femoris musculocutaneous flap: a possibility for major soft tissue reconstruction in the groin, hip, gluteal, perineal, and lower abdominal regions.
A 57-year-old man, with a long-lasting multiple hereditary cartilaginous exostoses, presented with a progressive tumor growth in the left iliac wing and in the gluteus maximus muscle. An open biopsy revealed a secondary chondrosarcoma, which had developed from an osteochondroma. A wide surgical resection, sparing the inferior limb, was the option for treatment. The fairly constant and reliable vascular anatomy of the lateral circumflex femoral artery, as confirmed by 27 previous anatomical dissections, gave us the opportunity to repair the wide postoperative defect by means of a single, very large flap, combining the vascular territories of the tensor fasciae latae and rectus femoris musculocutaneous flaps. The postoperative period was uneventful, and 1 year after surgery the man had no sign of local recurrence or metastases, and the flap was fully viable without sign of local complication. A functional evaluation was performed on a Kin-Com II dynamometer, showing major impairment of the limb that had undergone surgery. Despite the severe functional disturbance, the man prefers the impaired status to an amputation after a hemipelvectomy.
Abdominal Neoplasms↗
Surgery for soft tissue sarcoma in the extremities. A multivariate analysis of the 6-26-year prognosis in 137 patients.
In 137 patients with soft tissue sarcomas in the extremities, the influence of patient and tumor characteristics and surgical procedures on prognosis was studied using a multivariate statistical analysis. The minimum follow-up time was 6 years, and no patient was lost to follow-up. Eighty-nine patients were referred with the tumor intact (primary series), while 48 were referred after biopsy or with local recurrence after previous surgery (secondary series). The frequency of amputation was 15 percent in the primary series and 48 percent in the secondary series. A local, function-preserving operation with a wide margin was performed in 37 patients without biopsy (clinical diagnosis alone). Local control of the disease was obtained in all but one of these patients without any adjunctive treatment. Independent, unfavorable factors concerning local recurrence were advancing age, open biopsy, and marginal surgery. Independent, unfavorable factors concerning survival were advancing age, increasing histologic malignancy grade, and ablative surgery. A tumor-related death was observed in 0, 29, 47, and 67 percent of the patients with tumors of malignancy grades I, II, III, and IV, respectively.
Adolescent↗
Surgical margin in soft tissue sarcoma. The Scandinavian Sarcoma Group experience.
Two-hundred and forty adult patients with a high-grade soft tissue sarcoma were treated surgically in 18 hospitals participating in the Scandinavian Sarcoma Group Protocol I. The patients were randomized to either postoperative doxorubicin or control; patients whose surgical margin was judged marginal also received radiotherapy. The outcome after different surgical margins was analyzed in 185 tumors of Grades III or IV in the extremities. The total cumulative local tumor control was 91 percent (168 of 185) after a median of 47 months. The cumulative local control rates in the surgical groups were: compartmental or wide amputation--37/37 (100 percent), compartmental local excision--23/24 (96 percent), wide local excision--77/84 (92 percent), marginal excision and radiotherapy--19/21 (90 percent), and marginal excision alone (reevaluated margin)--12/19 (63 percent, significantly lower than others). The risk of local recurrence was 13 times higher after marginal than after compartmental surgery (P = 0.02) and 3 times higher if the tumor was larger than 10 cm (P = 0.05). The treatment with doxorubicin did not influence the risk of local recurrence. The survival rates did not differ significantly in the groups.
Combined Modality Therapy↗
Primary leiomyosarcoma of bone. A clinical, radiographic, pathologic-anatomic, and prognostic study of 16 cases.
Sixteen cases of primary leiomyosarcoma of bone are described. The patients, 11 males and 5 females, ranged in age from 9 to 74 years. The annual incidence of this tumor in Sweden was calculated to be 0.09 cases per million. This figure was obtained by reviewing a Swedish series of spindle cell sarcomas of bone of which one quarter (11/44) were diagnosed by us as primary leiomyosarcoma. The diagnosis was based on light- and electron-microscopic examinations using the same criteria as for leiomyosarcoma of soft tissues. Thirteen tumors were located in a long bone of an extremity (nine close to the knee joint) and three in the central skeleton. Radiographically, all the tumors presented as a purely osteolytic lesion, and three patients had sustained a pathologic fracture. In four of six cases angiography suggested malignancy by revealing hypervascularity, irregular tortuous vessels, and diffuse contrast opacification. Contrast-enhanced computed tomography, performed in two cases, showed hypervascular areas within the tumors. Scintigraphy showed a marked increase in radionuclide uptake in all five cases studied. The clinical behavior indicates that primary leiomyosarcoma of bone is highly malignant. Eight patients had died of the tumor and, of the eight patients who were alive at follow-up, two had metastases, and one had been operated on three times for a cutaneous metastasis, which had recurred locally twice. The remaining five patients had been continuously free of disease for 6.5 to 12.3 years.
Adolescent↗
Vascularization of peripheral neurilemomas: angiographic, computed tomographic, and histologic studies.
The vascularization of peripheral neurilemoma was studied in 17 patients, in eight by angiography and in 16 by computed tomography (CT) using contrast enhancement. Angiographically the degree of vascularity varied from scarcity to abundance of vessels. In two patients, arteries connected to the proximal and distal poles of the tumor could be identified as nutrient arteries of the affected nerve, and in three patients some of the veins draining the tumor followed the course of the nerve. All 16 tumors examined by CT showed lower density than skeletal muscle. They were well delineated and at least partially surrounded by adipose tissue. With contrast enhancement the density increased focally, reflecting the distribution of vessels in the tumors. Histologic examination showed that Antoni-A tissue was rich in small (below 15 micron) and medium-sized vessels, whereas large vessels (above 100 micron) were more frequently seen in Antoni-B tissue. Medium-sized and large vessels often tended to be arranged along the border between Antoni-A and Antoni-B tissues.
Adult↗
Extra-abdominal desmoid tumors.
A follow-up study of 53 patients with extra-abdominal desmoid tumor is presented. Fine-needle aspiration biopsy seemed to be valuable in the differentiation between a desmoid tumor and a truly malignant soft tissue tumor. The angiographic pictures did not differ from those of other soft tissue tumors, but valuable topographic information was obtained from the angiography in the preoperative planning. The overall local recurrence rate was 17/44, and 15 of the recurrences occurred within the first 2 years. Complete removal of the affected muscle or muscle group is recommended. Local control can be achieved by non-ablative surgery even after multiple recurrences. Only one out of 53 patients died of the tumor disease. Radiotherapy can be attempted in extensive tumors, and amputation should be reserved for tumors which have failed to respond to this treatment.
Adolescent↗
[How accurate is caries diagnosis?].
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Dental Caries↗
Leiomyosarcomas of venous origin in the extremities. A correlated clinical, roentgenologic, and morphologic study with diagnostic and surgical implications.
This article presents a correlated clinical, roentgenologic, and morphologic study of six cases of leiomyosarcoma of large veins in the extremities. Growth of the tumor within the lumen of the affected vein was demonstrated by phlebography in one case and by angiography and computed tomography in one case. In all six cases, such growth was demonstrated by dissection of the surgical specimen. In four cases, arteriography showed both neovascularity at the site of the lesion and compression of the artery accompanying the affected vein. The compression apparently was caused by entrapment of the artery within the same preformed fibrous sheath (conjunctiva vasorum) that enclosed the tumor and the vein from which it had arisen. The histologic diagnosis was based on the light microscopic appearance in all cases and was supported by electron microscopic findings in three cases by the demonstration of ultrastructural features of leiomyoblasts. All six tumors were light microscopically of high-grade malignancy. Five of the patients died of metastatic tumor disease and one is alive with lung metastases. The information obtained by a careful physical examination combined with phlebography, arteriography, and computed tomography may be helpful in the clinical diagnosis and the planning of adequate surgical treatment.
Adult↗
Primary leiomyosarcoma of bone: a study of five cases.
Five cases of leiomyosarcoma of bone are described. The patients, 3 men and 2 women, ranged in age from 24 to 74 years. Four of the five tumors were located in the distal half of the femur. Radiographically, all tumors presented as purely osteolytic lesions. Angiography, performed in 3 cases, revealed hypervascularity and the presence of irregular, tortuous vessels at the site of the lesion in 1, but not in 2 others. Histologically, the five tumors showed features characteristic of leiomyosarcomas. The most prominent features were blunt-ended, cigar-like, chromatin-rich nuclei in elongated acidophilic cells arranged in bundles which intersected each other at wide angles. The importance of trichrome stains, such as van Gieson stain, for th recognition of leiomyosarcomas is emphasized. Electron microscopic examination, performed in 3 cases, showed as the most prominent features spindle-shaped cells in parallel arrangement containing abundant myofilaments which elongated densities, pinocytotic vesicles, and basal lamina structures.
Adult↗