[Results of treatment of 170 sarcomas of the bones and soft parts].
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OBJECTIVE: To determine if chemotherapy offers a survival benefit to patients with large, high-grade, primary extremity liposarcoma. SUMMARY BACKGROUND DATA: The impact of chemotherapy on the survival of patients with primary extremity soft tissue sarcoma is controversial and its effect on individual histologic subtypes is not defined. PATIENT AND METHODS: Two prospectively collected sarcoma databases were used to identify all patients with >5 cm, high-grade, primary extremity liposarcoma that underwent surgical treatment of cure from 1975 to 2003 (n = 245). Clinical, pathologic and treatment variables were analyzed for disease-specific survival (DSS), distant recurrence-free survival (DRFS) and local recurrence-free survival (LRFS). RESULTS: Sixty-three (26%) patients were treated with ifosfamide based chemotherapy (IF), 83 (34%) with doxorubicin based chemotherapy (DOX) and 99 (40%) received no chemotherapy (NoC). To assess the impact of DOX, a contemporary cohort analysis of patients treated from 1975 to 1990 was performed. The 5 year DSS of the DOX treated patients was 64% (53%-74%) compared with 56% (51%-79%) for the NoC patients (log-rank P value = 0.28). To assess the impact of IF, a contemporary cohort analysis of patients treated from 1990 to 2003 was performed. The 5 year DSS of the IF treated patients was 92% (84%-100%) compared with 65% (51%-79%) for the NoC patients (log-rank P value = 0.0003). Independent prognostic factors for improved DSS were smaller size (HR = 0.7, P = 0.01), myxoid/round cell histologic subtype (HR = 0.3, P = 0.03) and treatment with IF (HR = 0.3, P = 0.01). The five-year DRFS of the IF treated patients was 81% (70%-92%) compared with 63% (50%-76%) for the NoC patients (log-rank P value = 0.02). The 5 year LRFS of the IF treated patients was 86% (76%-96%) compared with 87% (77%-97%) for the NoC patients (log-rank P value = 0.99). CONCLUSIONS: In patients with large, high-grade, primary extremity liposarcoma; DOX is not associated with improved DSS and IF is associated with an improved DSS. Treatment with IF should be considered in patients with high-risk primary extremity liposarcoma.
The ultrastructure of cytoplasmic filamentous material in cells of different human sarcomas was studied and several types of it are distinguished. The first type is specific and can be used as a differential diagnostic marker of tissue appurtenance of neoplasias: in fibroasarcoma cells--a uniform layer of thin filaments located near the plasmic membrane; in rhabdomyosarcomas--thick sheets of long filaments; in leiomyosarcomas--bundles of filaments with focal consolidation; in liposarcomas--loosely packed scarce layers of thin filaments of cylindrical shape; in malignant synoviomas--rod-shaped bundles of thin filaments, endotheliomas--thick single bundles of predominantly perinuclear location. Two types of nonspecific filamentous apparatus in cells of these neoplasias were distinguished: widelooped loose filamentous network and thick circular sheets. The problems of genesis and formation of filamentous apparatus of different types in tumor cells are discussed.
Postradiation sarcoma is a rare late complication of external radiotherapy. We herein present two cases with this disease. A 54-year-old man had undergone a lobectomy and chest wall resection for Pancoast type lung cancer 7 years previously. He had undergone irradiation with a total dose of 50 Gy. Computed tomography (CT) demonstrated a tumorous expansion of the right lateral thoracic wall. A pathological examination confirmed a diagnosis of osteosarcoma. A 60-year-old woman had undergone a resection of the lateral chest wall mass, which was diagnosed to be Hodgkin's disease in 1991. Chemotherapy was given postoperatively. A tumorous lesion arose again and irradiation was performed with a total dose of 110 Gy. In 2000, two tumors appeared in the irradiation field. A pathological examination showed a sarcoma with divergent differentiation. In 2003, a tumor recurred and was diagnosed to be a liposarcoma. Patients who have received radiotherapy should therefore be followed up while taking into consideration the possible development of postradiation sarcoma.
Liposarcoma is among the most common adult histotypes of soft tissue sarcoma. It usually arises from the deep soft tissue of the extremities or the retroperitoneum. Despite the high content of fat in the orbit, both benign and malignant tumors of adipose tissue are extremely rare at this site; reports describing liposarcoma metastatic to the orbit are rare. Our report is a new case and the appropriate methods for diagnosis and treatment of this unusual metastatic site in this type of cancer are discussed.
Common histologic variants of liposarcoma are readily recognized. Histologic classification might be difficult, however, when liposarcoma demonstrates cartilaginous differentiation. Although this phenomenon has previously been noted, it has not, to our knowledge, been reported as a specific pathologic entity. All three patients in the present study were men, ages 37, 42, and 63 years. Each presented with a solitary, enlarging mass of the thigh that was surgically excised. The tumors ranged in size from 8 to 13 cm. Microscopically, each lesion displayed characteristic features of myxoid liposarcoma; in addition, all possessed discrete, rounded foci of mature-appearing hyaline cartilage. One case displayed small foci showing chondrocyte atypia. No other patterns of mesenchymal differentiation were present. All patients received postoperative radiation therapy. No recurrences or metastases have been identified (mean duration, 39 mo). Myxoid liposarcomas with cartilaginous differentiation are of importance because they might be misdiagnosed as malignant mesenchymoma. The latter, if not qualified as to histologic grade, might be presumed to be a biologically more aggressive lesion. In addition, these lesions must be distinguished from two benign processes: chondroid lipoma and extraskeletal chondroma with lipoblast-like cells. Additional studies of this uncommon variant of liposarcoma will be necessary to document further its status as a low-grade sarcoma.
Five hundred and sixty-five patients with soft-tissue sarcoma were admitted to Memorial Sloan-Kettering Cancer Center from July 1, 1982 to July 1, 1985. Clinical and pathologic factors were analyzed to identify initial characteristics and to determine factors associated with prognosis and the development of metastasis. In decreasing order of frequency, the most common sites of primary tumor were the extremity (51.1%), retroperitoneum/viscera (28.8%), trunk (15.9%), head and neck (3.7%) and breast (0.4%). The most common histologic types were liposarcoma (21.4%), malignant fibrous histiocytoma (20.2%), leiomyosarcoma (20.0%), fibrosarcoma (11.0%), and tendosynovial sarcoma (9.6%). One hundred and twenty-eight patients (22.7%) had metastases in a median follow-up period of 19 months. Metastasis was more common in patients with retroperitoneal and visceral sarcomas than in patients with extremity sarcomas. Patients with leiomyosarcomas were more likely to develop metastases than those with other histologic types. Fibrosarcoma and liposarcomas were the least likely to develop metastases in this time frame. Tumors greater than 5 cm in diameter occurred more frequently in patients with metastases. Actuarial survival was related to the site of primary tumor and the histologic type and grade of malignancy. Multivariate analysis indicated that patients with low-grade sarcomas, extremity sarcomas, and fibrosarcomas and patients without metastatic disease demonstrated significantly improved survival. In patients with metastases, disease-free interval (less than 365 or greater than or equal to 365 days) was significantly related to overall survival.(ABSTRACT TRUNCATED AT 250 WORDS)
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Solid tumours in man are characterized by acquired genetic rearrangements that, in most cases, can be detected by cytogenetic methods as clonal chromosomal abnormalities. Whereas primary abnormalities contribute to the establishment of the tumour and often are seen as solitary changes, secondary aberrations accrue during clonal evolution. Both abnormalities are nonrandom in distribution. Some primary abnormalities are so characteristic as to be virtually pathognomonic for particular types of solid tumours, eg, t (11;22)(q24;q12) in Ewing's sarcoma, t (9,22)(q22;q12) in extraskeletal myxoid chondrosarcoma, t (X;18)(p11;q11) in synovial sarcoma, and t (12;16)(q13;p11) in myxoid liposarcoma. To these purely cytogenetic data implicating specific genetic changes in carcinogenesis may now be added a growing evidence of molecular specificity emerging from recombinant DNA-studies. It appears that both currently known classes of directly cancer-relevant genes, the dominant oncogenes and the recessive tumour suppressor genes, are located at precisely those genomic sites that are visibly involved in neoplasia-associated chromosomal rearrangements. The importance of cytogenetic characterization of solid tumors is thus twofold. First, the recurrent aberrations provide insight into the pathogenetic mechanisms that are operative. They pinpoint areas of the human genome that carry genes or regulatory sequences whose function is disrupted in neoplastic cells. Second, even before the long-term goal of a more fundamental understanding of the neoplastic process is reached, the cytogenetic aberrations have direct clinical importance. The finding of an acquired clonal chromosomal abnormality identifies the presence of a neoplastic disease, and the specific type of aberration may reveal the true nature of the tumor and thus improve the diagnostic precision.
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Anaplastic large cell (Ki-1) lymphomas are a recently described subtype of non-Hodgkin's lymphoma. A single case of the sarcomatoid variant has recently been reported. Below we report the fine needle aspiration findings in an additional case of the sarcomatoid variant of Ki-1 lymphoma. The diagnosis was made only in retrospect with the aid of immunostains because the cytologic findings were more in keeping with a sarcoma. Striking vascular structures in the smears suggested angiosarcoma or liposarcoma. Additional features were pleomorphic cells with eccentric nuclei and abundant, tapering cytoplasm; spindle cells; multinucleation; cytoplasmic vacuolization; and prominent nucleoli. The smear pattern was composed of dispersed cells with some cohesive groups within an acute inflammatory background with a virtual absence of lymphoglandular bodies. These findings, atypical of lymphoma, broaden the spectrum of possible cytologic findings in lymphoid malignancies and highlight the possible utility of immunostaining. The distinction of lymphoma from sarcoma or carcinoma is of therapeutic importance.
Besides the malignant fibrous histiocytoma, liposarcoma is one of the most common soft-tissue sarcomas seen in adults. They are most often localized in the lower extremity, followed by sarcomas in the retroperitoneal space which differ from the former in clinical appearance, epidemiology, and prognosis. The prognosis is especially dependent on tumor histology which is mainly classified into four subtypes. The aim of the study was to investigate whether there are specific CT-features for each histologic subgroup, by analyzing the examination of 20 patients. The highest percentage in fat and the biggest tumors were seen with well-differentiated liposarcomas (n = 4), whereas myxoid ones (n = 6) showed the sharpest margins. Round-cell (n = 2) and pleomorphic (n = 5) and pleomorphic (n = 5) types showed mainly soft-tissue attenuation and could not be differentiated, either from each other or from other soft-tissue tumors. A clear identification of the histologic subgroup does not seem to be possible.
The present study was undertaken with the aim of evaluating the clinical and anatomopathological findings, emphasizing expression of the protein p53 as possible prognostic markers, in patients with breast sarcoma. p53 immunohistochemical expression was determined in archival paraffin embedded tissue blocks of 30 breast sarcoma patients, (19 fibrosarcomas, nine malignant fibrohistiocytomas and two liposarcomas) treated at the Hospital do Cancer AC Camargo, São Paulo, Brazil from 1955 to 1990. Immunopositivity was present in 50% of the cases. The survival of the patients was compared with the above parameters. Median follow up time was 113 months. The 5 years specific survival rates were 55.1% for patients with a positive expression of p53 contrariwise to 92.3% of specific survival found in p53 negative patients (p = 0.04). Positive expression of p53 was found in 3/4 (75%) of the patients with local recurrence and in 7/9 (77%) of patients with metastatic disease. No significant correlation between survival and clinicopathologic features (age, menopausal status, tumor size, stage and histological type), was found. A slight positive correlation between high grade and poor outcome was observed, 89% of the metastatic cases being classified as high grade (p = 0.02, by one sided Fisher's exact test). When we have compared, independently, survival probability curves between p53 positive/negative expression and each category of clinicopathologic features a worse prognosis was observed when p53 was positive in patients older than 50 years (p = 0.01), in tumors larger than 5 cm (p = 0.02), within the malignant fibrous histiocytoma subtype (p = 0.01) and in tumors classified as high grade (p = 0.07). In conclusion p53 expression seems to be a useful prognostic marker for this type of tumor.
Liposarcoma is one of the most common adult soft tissue sarcomas. It is best viewed as several related tumors rather than a common one, however. Support for this is derived from the fact that the various subtypes differ in their demographic, cytogenetic, and biologic properties. The pathologist, therefore, assumes a pivotal role in accurately classifying these lesions since this directly translates into grade and behavior. As the molecular events leading to these tumors are better understood, our approach to treating the various forms of liposarcoma may, likewise, differ.
99mTc-DTPA, a renal agent, has been noted to accumulate in tumors such as neurofibroma, uterine myoma, soft tissue sarcoma, hepatic hemangioma, leiomyosarcoma, and plasmacytoma. We report a case in which uptake of 99mTc-DTPA was demonstrated in a retroperitoneal liposarcoma.
Hyperthermia greater than or equal to 42 degrees C is tumoricidal in vitro and in many animal models, although such temperatures have only recently been achieved experimentally in some human cancers. A recently developed radio frequency device that provides safe hyperthermia to any depth without surface tissue injury now permits evaluation of the effects of hyperthermia on advanced human sarcomas. Twelve patients with large sarcomas located intraabdominally [7], in the chest wall [2], proximal extremity [2], and the neck [1], were evaluated in this study. Tumor types include liposarcoma [3], rhabdomyosarcoma [2], leiomyosarcoma [2], neurofibrosarcoma [2], and one each malignant mesothelioma, undifferentiated sarcoma, and osteosarcoma. Intratumor temperatures greater than or equal to 42 degrees C were observed in all tumors, with virtually no normal tissue injury. Selective tumor heating greater than or equal to 45 degrees C occurred in 9/12 (75%) and greater than or equal to 50 degrees C in 6/12 (50%). One to five weekly treatments greater than or equal to 50 degrees C and ten daily treatments greater than or equal to 45 degrees C resulted in significant tumor necrosis and pain relief in some patients. Hyperthermia of advanced sarcomas is possible with little host toxicity and may be of potential therapeutic benefit.
BACKGROUND: Soft tissue sarcomas are challenging to oncologists due to their unique character, the infrequency of their occurrence, and the difficulties in predicting outcomes. Advances in imaging, as well as improvements in surgical techniques and adjunctive treatment methods, have improved care for patients with these unusual disorders. METHODS: The various types of soft tissue tumors are defined, and the statistics for the Orthopaedic Oncology Group in relation to them are reviewed and compared with literature references. RESULTS: The overall survival rate for 1,220 tumors treated at our institute from June 1972 to June of 2001 was 72%, with a wide range. Patients with leiomyosarcomas, clear cell sarcomas, and malignant fibrous histiocytomas had a poorer survival rate, while those with fibrosarcomas, liposarcomas, and neurofibrosarcomas fared better. Outcome was affected by patient age, tumor anatomic site, tumor stage, and a history of recurrence. CONCLUSIONS: Competent imaging, predictive immunological and genetic studies, improved surgery, and newer methods of adjunctive and neoadjunctive treatment should result in improvements in outcomes for patients with these tumors.
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