[The possibilities of the use of radioactive microspheres in intra-arterial administration in treatment of neoplasms of the extremities].
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In this article, several areas in the pathology of tumors are discussed in which electron microscopy has contributed valuable diagnostic information but, at the same time, has not resolved all the questions regarding histogenesis, differentiation, and cell type.
Human myosarcomas, liposarcomas, meningosarcomas, glioblastomas and malignant schwannomas, their benign counterparts and normal cells from which these tumours derive, were examined for the expression of beta 2-microglobulin (beta 2m). Formalin fixed specimens from these tumours were studied by light microscopy employing the immunoperoxidase method with the use of antibodies directed towards beta 2m. The malignant tumours showed a broad spectrum from unstained to strongly stained tumours, most pronounced among myosarcomas. In addition, most stained tumours displayed a mosaic staining pattern in that unstained areas alternated with stained. There was a tendency towards increased staining for beta 2m in malignant compared to normal cells; this was also observed in the benign tumours although to a lesser degree. The results differ from most earlier studies, mainly of carcinomas which have shown a tendency towards down-regulation of MHC I molecules on the tumour cells. The results are discussed in relation to concepts of immune surveillance of tumours.
This report documents heterogeneous cell populations within high-grade soft tissue sarcomas in seven patients. The authors observed that, following chemoradiotherapy (five patients) or chemotherapy alone (two patients), surgically resected residual or recurrent sarcoma was found to be of low-grade histology. All chemotherapy was of Adriamycin (doxorubicin)-containing combinations. Seven other patients with regional recurrence after resection of high-grade sarcomas had high-grade recurrent tumors. Five of these patients had received no adjuvant therapy; two with extensive retroperitoneal liposarcomas and gross tumor-involved margins had received chemoradiotherapy. Follow-up ranged from 3 to 60 months (median, 12 months). These findings suggest that high-grade populations of sarcoma cells are more susceptible to chemoradiotherapy, as long as there is minimal residual disease. Low-grade clones of tumor cells may survive such therapy and present as clinical recurrence after initial treatment of low-grade or high-grade soft tissue sarcomas. Surgical resection is warranted for these recurrent lesions.
On account of the considerable variability of the clinical situation, treatment of soft tissue sarcoma requires an individually oriented multimodal approach. In the case of patients with superficial low-grade tumors measuring less than 5 cm in diameter, resection alone is usually adequate. In the event of medium-grade lesions, resection with negative margins, resection in combination with radiotherapy achieves excellent local control rates associated with an overall survival rate of almost 80%. In patients with high-grade sarcomas measuring more than 5 cm in diameter, local control can be achieved with resection and radiotherapy, although every second such patient develops metastases. For patients with local recurrence, further resection should be considered/performed. Radiotherapy is all the more effective, the smaller the postoperative tumor cell burden.
Morphological findings of 21 soft tissue sarcomas of adulthood following preoperative chemo- and/or radiotherapy including perfusion therapy and resection are presented. The therapy-induced changes included a spectrum ranging from total tumor regression up to still completely vital tumor (median of all cases: 30% vital tumor tissue). 7 of 21 sarcomas (33%) were evaluated as responders (grade I-III). So-called malignant fibrous histiocytoma (MFH) revealed a good tumor response to preoperatively administered therapy (regression grades I and II). Myxoid/round-cell liposarcoma exhibited regression grades of IV-V (i.e. 30% up to 95% vital tumor tissue, non-responders). Synovial sarcoma was characterized by regression grades III up to VI (i.e. up to 100% vital tumor tissue without any signs of regression, 83% non-responders). Completely vital tumor was evident in 2 synovial sarcomas despite preoperative tumor therapy. These findings hint towards differences in response of distinct sarcoma entities to preoperative chemo-/radiotherapy. A grading of therapy-induced tumor regression in adult soft tissue sarcomas may best refer to already established grading schemes (e.g. according to the grading scheme for osteosarcomas following chemotherapy of Salzer-Kuntschik). A report of sarcoma resection specimens should include the percentage of vital tumor tissue.
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Soft-tissue sarcomas of the retroperitoneum constitute a heterogeneous group of tumors with varying histology, potential for complete resection, and propensity for recurrent disease-making the development of effective treatment difficult and challenging. A retrospective review of 23 patients with retroperitoneal sarcomas from 1985 through 1998 was performed to assess the biological behavior and clinical outcomes and to identify factors that may influence prognosis and optimize treatment strategy. Liposarcomas were the most common pathology (61%); 79 per cent of these were of low grade. Leiomyosarcomas were the next most common pathology (30%); 43 per cent of these were of low grade. Low-grade sarcomas overall accounted for 62 per cent of the total group. Low-grade tumors independent of histologic type exhibited good prognosis for long-term survival with a median survival of 44 months. In contrast, intermediate- or high-grade tumors were associated with a median survival of only 9 months (P < 0.02). On the other hand, tumor histologic type independent of grade did not have a significant survival difference. Complete tumor resection was possible in 21 of 23 patients, which gives an overall resectability rate of 91 per cent. Eight patients (36%) remain disease-free after initial surgical treatment. However, local recurrence was common; this occurred in 11 of 22 patients (50%). Local recurrence, however, did not preclude long-term survival. Surgical resection of recurrent disease was done in nine patients with a median survival of 91 months (range 24-150 months). Three patients had as many as three operations for recurrent disease. With subsequent recurrences there was a decrease in interval from approximately 4 years to 2 years, and 33 per cent of these patients developed tumor dedifferentiation to high grade. An aggressive surgical approach with reoperation can produce prolonged survival in patients with low-grade retroperitoneal sarcoma.
In relation with other laryngeal neoplasms, sarcomas are uncommon tumours. With the object to put in knowledge one chondrosarcoma and one liposarcoma located in the larynx seen in our hospital in a small period of time, we decided to do this publication, discussing the differential clinical aspects of these laryngeal cancers.
Multifocal presentation, defined as the presence of tumor at two or more anatomically separate sites, before the manifestation of disease in sites where sarcomas usually metastasize (e.g., lungs) occurs in about 1% of extremity soft tissue sarcomas (STSs). Debate still persists whether multifocal STSs represent an unusual pattern of metastasis or multiple separate primary tumors. Among STSs with multifocal presentation, myxoid liposarcoma is the predominant histological type. This subtype of liposarcoma contains the specific t(12;16) chromosomal translocation, which results in rearrangement of the TLS and CHOP genes that is clone specific at the DNA level. We, therefore, sought to address the question of clonality by molecular analysis in six patients who presented with either synchronous or metachronous multifocal myxoid liposarcoma. In all six cases, adequate frozen tumor was available for DNA extraction from at least two distinct anatomical sites. Southern blot analysis using CHOP, TLS, and EWS cDNA probes was performed on genomic DNA. Five cases contained a TLS-CHOP rearrangement, and one case had the variant EWS-CHOP fusion (seen in <5% of cases). The size of the rearranged CHOP fragment differed among the six patients, as expected, but was identical in all anatomically separate tumor samples from each patient. Likewise, the sizes of the rearranged bands observed with either the TLS or EWS probes supported the monoclonality of all cases. Our results confirm the monoclonal origin of multifocal myxoid liposarcoma, establishing the metastatic nature of distant soft tissue lesions in these cases. It remains unclear whether this unusual pattern of metastasis represents an intrinsic property of this subset of myxoid liposarcoma or merely a rare chance occurrence. The clinical outcomes observed in this small series suggest that the prognosis of multifocal myxoid liposarcoma is poor, regardless of its often bland or "low-grade" histological appearance.
UNLABELLED: Primary soft tissue sarcomas of the chest wall are uncommon, and data concerning treatment and results are sparse. Most studies have categorized these tumors as truncal sarcomas and inferred a poor prognosis. To assess the results of surgical treatment, we reviewed our 40-year experience. METHODS: Records of 189 patients admitted to our institution from 1948 to 1988 were reviewed. Pathologic material was available for review in the 149 cases (79%) that comprise this report. Survival was calculated by the Kaplan-Meier method, with comparisons by log-rank analysis and significance defined as p less than 0.05. RESULTS: Ages ranged from 3 weeks to 86 years (median, 38 years); the ratio of male to female patients was 2:1. The initial complaint was mass or pain in 97% of the cases. Ninety sarcomas (60%) were high grade and 59 (40%) were low grade. Histologic types were as follows: desmoid tumor (n = 32, 21%); liposarcoma (n = 23, 15%); rhabdomyosarcoma (n = 18, 12%); fibrosarcoma (n = 17, 11%); embryonal rhabdomyosarcoma (n = 14, 9%); malignant peripheral nerve tumor (n = 13, 9%); malignant fibrous histiocytoma (n = 11, 7%); spindle cell sarcoma (n = 4, 3%); tenosynovial sarcoma (n = 3, 3%); hemangiopericytoma (n = 3, 3%); alveolar soft part sarcoma (n = 3, 3%); and other types (n = 12, 9%). Resection was the primary treatment in 140 cases (94%). Local recurrence developed in 27%. Metastases occurred in 52 (35%) of the cases (metachronous in 42, synchronous in 10) and were more common in patients with high-grade disease (46/90, 51%) than in those with low-grade disease (6/59, 10%). Overall 5-year survival was 66%. Five-year survival rate for those with high-grade sarcomas (49%) was significantly lower than that for low-grade sarcomas (90%, p less than 0.0001). Tumor size and age of patient were not prognostic. CONCLUSIONS: Survival of patients with primary soft tissue sarcomas of the chest wall after resection is similar to that of patients with sarcomas of the extremities. Resection alone provides acceptable survival (90% at 5 years) for those with low-grade sarcomas, but adjuvant treatment should be considered for those with high-grade sarcomas.
A contrary viewpoint concerning certain issues in soft-tissue sarcoma pathology is presented. Three issues emphasized are (1) the role of immunocytochemistry and electron microscopy in diagnosis, (2) the classification of liposarcomas and atypical lipomatous tumors, and (3) the validity and usefulness of histologic grading.
Lymph node metastases occur in an appreciable number of soft-tissue sarcomas. The histologic subtype plays a most important role. Synovial sarcoma, malignant fibrous histiocytoma, rhabdomyosarcoma, and angiosarcoma manifest relatively frequently such metastases, whereas fibrosarcoma, liposarcoma, and malignant schwannoma do so very infrequently. With the use of the newer radiologic techniques, it is expected that this feature will be more clearly defined and taken into account in planning the treatment. For histologic subtypes known frequently to manifest metastases to the regional lymph nodes, the latter should be considered for inclusion in the surgical and/or radiation treatment plan, particularly for primary tumors situated close to a nodal basin; and careful follow-up of these nodes should be practiced.
Eleven patients with advanced soft tissue sarcoma were treated with whole body hyperthermia (41.8 degrees C-43.0 degrees C) for 2 hours, doxorubicin (45 mg/m2) at the beginning of peak temperature and cyclophosphamide (1000 mg/m2) 6 hours after doxorubicin. Warming was accomplished with a nylon and vinyl mesh water perfused suit and heating blankets under barbiturate anesthesia. Thirty-five thermochemotherapy treatments were administered after an initial baseline euthermic course. There were two complete and two partial responses including three of three liposarcomas and one of two leiomyosarcomas, and there were two disease stabilizations . Morbidity included anasarca, nausea and vomiting, diarrhea, myalgias, mild surface burns, perioral herpes simplex, reversible neuropathy, hypotension, and cardiac arrythmias . Hyperglycemia and hypophosphatemia were found during heating, and normalized at 24 hours. Liver enzyme elevations occurred 24 hours after heating and normalized within 1 week. A uniform platelet decrease (mean, 107,000/microliter) was found at 24 hours. Thermochemotherapy was found to be a feasible approach for selected patients with advanced soft tissue sarcoma for the subset of liposarcomas and leiomyosarcomas.
BACKGROUND: Histologic grade is said to be the most important prognostic factor in adult soft tissue sarcomas (STS), but most grading systems have been tested in the overall sarcoma group and the predictive value of histologic grade needs to be assessed specifically for each of the histologic categories. METHODS: From 1980 to 1994, 1240 nonmetastatic patients were entered in the French STS database. The following parameters were studied: patient's age and gender, previous history, tumor location, size and depth, neurovascular or bone involvement (NBI), histologic type and subtype, and grade (the French Federation of Cancer Centers [FNCLCC] system). Median follow-up for the survivors was 88 months; only 5% of patients were lost to follow-up. The authors performed univariate and multivariate analyses for metastasis-free survival for the overall sarcoma group and for every main histologic type. RESULTS: In order of importance, parameters were respectively retained as independent predictors of metastasis as follows: grade, tumor size, NBI and tumor depth for the overall group, grade and NBI for malignant fibrous histiocytomas (n = 349), tumor size, histologic subtype and grade for liposarcomas (n = 188), NBI, grade and tumor size for leiomyosarcomas (n = 148), grade and NBI for synovial sarcomas (n = 125), grade for unclassified sarcomas (n = 140), and sarcomas of other types (n = 158). No parameter was significant for malignant schwannomas (n = 72) or for rhabdomyosarcomas (n = 60). CONCLUSION: In this study, histologic grade appeared as an independent predictor of metastasis development in the main histologic types of adult STS, with the exception of malignant schwannomas and rhabdomyosarcomas.
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