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PubMed1976

Lymphatic dissemination of bone and soft tissue sarcomas: a lymphographic investigation.

The series consisted of 132 patients, 61 with primary bone sarcomas and 71 with primary soft tissue sarcomas. The patients were all evaluated by lymphography. The investigation included both patients who had not yet been treated and patients with suspected or confirmed metastases. All tumour diagnoses were confirmed microscopically. The findings as regards dissemination were based on clinical examinations, laboratory tests, roentgen examinations and lymphographies. In some cases, lymph node biopsies and surgical observations were also used. A total of 151 lymphographies were performed and 281 follow-up films taken. Preoperative lymphography was performed using the technique introduced by Kinmonth. For postoperative lymphography on the stumps of amputated extremities, two simple but useful methods were developed, which are presented here. Changes in the lymphographic appearance of lymph node metastases, the occurrence of new metastases, and the results of treatment were assessed by survey films and repeat lymphography. The generally accepted criteria for metastasis were used as a basis for the analysis of the lymphographic findings. The results may be summarized as follows: 1. Incidence of lymphatic dissemination. Different sarcomas varied greatly in their clinical course, including the frequency of dissemination. The lymphatic involvement in the metastatic cases was as follows: Bone sarcomas: 16 out of 28 (Table 10); of these, 13 were to regional lymph nodes, 8 to distant nodes and 5 to both (Table 14). Soft tissue sarcomas: 24 out of 40 (Table 11). All 24 had metastases in regional nodes, and 8 in distant nodes as well (Table 15). The highest frequencies of lymphatic spread in the different metastasized tumours were found to be: Bone sarcomas: reticulosarcoma 100%, Ewing's sarcoma 50%, osteosarcoma 47%. Soft tissue sarcomas: rhabdomyosarcoma 100%, synovial sarcoma 80%, neurogenic sarcoma 78%, leiomyosarcoma 67%. 2. Time-relation between lymphatic and haematogenic dissemination; The tendency to metastasize first via the lymphatics or via the blood vessels varied. Half of the cases of Ewing's sarcoma and reticulosarcoma had evidence of lymphatic spread before blood-borne metastases were detected. In the osteosarcoma cases, however, lymphatic dissemination was always preceded by haematogenic spread (Table 12). In synovial sarcoma, rhabdomyosarcoma and neurogenic sarcoma, the first dissemination was more frequently lymphatic than haematogenic (Table 13). 3. Possible existence of special lymphographic features of sarcoma metastases. Only reticulosarcoma displayed special characteristics. The lymph node metastases of reticulosarcoma of bone had lymphographic appearances similar to those found in reticulosarcoma of soft tissue or lymph node origin (Fig. 12). The lymph node metastases of other primary bone and soft tissue sarcomas had no specific lymphographic features and were indistinguishable from carcinomatous metastases (Figs 7, 9, 13, 15, 18, 19, 20, 22, 23). 4...

Adolescent↗
PubMed2000

Treatment of myxoid liposarcoma in pregnancy.

Two cases of pregnancy associated with myxoid liposarcoma are presented. Both patients were treated with hyperthermoradiotherapy after the induced delivery of healthy infants and by surgical excision of the tumor 5 to 7 weeks after delivery. No local recurrence or distant metastasis occurred; the children have progressed normally for 4 and 5 years, respectively, after surgery. Because coexistence of pregnancy and sarcoma is rare, it is difficult for the clinician to develop an appropriate approach to the management of bone and soft tissue sarcoma during pregnancy. The literature was reviewed regarding the effect that pregnancy may have on the clinical behavior of sarcoma and the effect treatment for sarcoma may have on the fetus.

Adult↗
PubMed1985

Immunohistochemistry of soft tissue tumours: a review.

The diagnosis and classification of soft tissue sarcomas can pose difficult problems for the histopathologist. Many sarcomas are too poorly differentiated to exhibit morphological features specific enough to define their histogenesis. Using the immunoperoxidase technique with commercially available antisera as a routine adjunct to other diagnostic aids, it is possible to arrive at more accurate diagnoses on which treatment protocols can be based. In addition a better understanding of mesenchymal neoplasms and their origins can be obtained by functional immunohistochemical studies.

Histiocytoma, Benign Fibrous↗
PubMed1991

A prospective randomized trial of adjuvant chemotherapy with bolus versus continuous infusion of doxorubicin in patients with high-grade extremity soft tissue sarcoma and an analysis of prognostic factors.

A prospective randomized trial was conducted to compare the cardiotoxic and therapeutic effects of doxorubicin (60 mg/m2 every 3 to 4 weeks) administered by bolus or 72-hour continuous infusion as adjuvant chemotherapy in 82 eligible patients after resection of high-grade soft tissue sarcoma of the extremity or superficial trunk. Cardiac toxicity, defined as a 10% or greater decrease in left ventricular ejection fraction as assessed by radionuclide cineangiography, was evaluated in 69 patients. Cardiotoxicity was seen in 61% of patients in the bolus treatment arm with the median doxorubicin dose of 420 mg/m2. Among patients who received continuous infusion, 42% had cardiotoxicity with a median dose of 540 mg/m2. The rate of cardiotoxicity as a function of the cumulative dose of doxorubicin was significantly higher in the bolus treatment arm (P = 0.0017). Two patients in each group had clinical congestive heart failure, with one cardiac death occurring in each. There was a trend toward a lower rate of metastasis (P = 0.19) and a significantly lower rate of death of disease (P = 0.036) for patients treated with the bolus dose. Cox model analysis identified three unfavorable characteristics for the rate of developing a distant metastasis: blood transfusion within 24 hours of operation (P less than 0.00001), tumor deep to the fascia and 5 cm or more in size (P = 0.0043), and a histologic subtype other than liposarcoma (P = 0.0002). The unfavorable effect of continuous infusion was not selected in the model (P = 0.16). Adjuvant chemotherapy for patients with soft tissue sarcoma is investigational. Furthermore, the impact of perioperative blood transfusion merits further study.

Adult↗
PubMed1991

Retroperitoneal sarcomas: pre-operative assessment and surgical therapy.

The clinical and pathological records of 22 patients treated for retroperitoneal sarcomas were retrospectively studied. The initial symptoms were pain and swelling of the abdomen in 11 patients. All tumours had a diameter of more than 10 cm. The pathological diagnosis was in 16 cases liposarcoma or leiomyosarcoma. Fourteen tumours were graded as high and eight as low. Sixteen of the 22 operated patients had complete resection of the tumour. Eleven patients had recurrence after five to 42 months, nine of them local recurrence and two distant metastases. Eleven patients needed repeated radical or palliative resections. Additional therapy (pre- or post-operative radiation or chemotherapy) was given to 11 patients. Radical resection of primary and recurrent tumours seems to give the best results in patients with retroperitoneal sarcomas but debulking of the tumour improves the quality of life in selected patients.

Combined Modality Therapy↗
PubMed1999

Paratesticular sarcoma: failure patterns after definitive local therapy.

PURPOSE: We examine disease control and patterns of failure for patients with adult paratesticular sarcoma treated with wide repeat excision and postoperative radiation for close margins. MATERIALS AND METHODS: A retrospective analysis was performed on 14 patients with paratesticular sarcomas referred to 1 institution from 1988 to 1995. RESULTS: Median followup was 50 months (range 26 to 90). Pathology review revealed malignant fibrous histiocytoma in 5 cases and liposarcoma in 3. Tumor grade was high in 8 cases. Microscopic residual disease was identified after repeat excision in 3 of 11 completely excised cases (27%). Cause specific survival 5 years after diagnosis was 70%. Both patients with local failure after wide excision had undergone initial intralesional excision and 1 had also received postoperative irradiation. In 5 patients metastases developed in the liver, lung, nodes or multiple sites 0 to 60 months after diagnosis. CONCLUSIONS: Simple excision is inadequate treatment for paratesticular sarcoma, since wide repeat excision revealed microscopic residual disease in 27% of completely excised cases. The 2 local failures occurred in patients with a history of inadvertent intralesional surgery, which may be a risk factor for local relapse after wide repeat excision. Adjuvant radiation should be considered for these patients as well as those with narrow repeat resection margins. Systemic failure was frequent in patients with high grade tumors, who will require effective systemic adjuvant therapy.

Adult↗
PubMed2001

[Myxoid liposarcoma of the spermatic cord].

A case of myxoid paratesticular liposarcoma in a 68-year old patient is reported. The evolution was normal during the follow-up period (22 months). Scrotal liposarcoma is a rare tumour: less than 100 cases were reported in literature. The diagnosis is difficult, clinical and radiological signs are the same as those of any intrascrotal tumour. Orchidectomy is the usual treatment, prognosis depends on the histological features, it is usually better than that of other paratesticular sarcomas. It did not need any additional treatment.

Aged↗
PubMed1997

Cytogenetics and experimental models.

The use of cytogenetics has led to significant improvement in the diagnoses and classification of sarcomas. Many of the major sarcomas have been to have characteristic tumor-specific chromosomal translocations that are currently used in the diagnosis of these tumors. In the past year, a subset of Ewing's family of tumors and myxoid liposarcomas, which lack one of the characteristic translocations, were found to carry related translocations. New technologies such as a spectral karyotyping will likely increase out ability to identify additional tumor-specific translocations. The emergence of genetic alterations as prognostic factors, as illustrated by Ewing's family of tumors, osteosarcoma, and p53 expression in soft tissue sarcomas in general, is discussed. The review concludes with laboratory applications derived from either tumor cytogenetic or gene function abnormalities that are related to tumor-specific translocations. It is anticipated that advances in diagnosis, prognosis, and modeling will translate into future therapeutic advances.

Humans↗
PubMed1998

[Atypical sarcomas localized within a head and neck in children].

Seven children treated surgically because of non-rhabdomyosarcomatous soft tissue sarcoma (NRSTS) localized extrameningeally on the head and neck were presented. Three of the patients were operated on haemangiopericytoma, two--fibrosarcoma, one child--neurofibrosarcoma and one--liposarcoma. The pre- and postoperative TNM classification was employed as a staging system. The surgical resectability--R was used to establish tumour margins. Four patients (two with fibrosarcoma and two with haemangiopericytoma) survived free of disease. The influence of the complete surgical resection on the outcome of the head and neck extrameningeal NRSTS was proved.

Child↗
PubMed1983

Management of patients with soft-tissue tumors. Strategy developed at a regional oncology center.

Clinical and morphologic variables in soft-tissue sarcomas and lipomas were retrospectively analysed and compared in consecutive, unselected population-based series from southern Sweden 1964-1981. Among the sarcoma patients these variables and the treatment given were evaluated by multivariate analysis with regard to the clinical course. The results of aspiration cytology and its influence on the choice of surgical procedure were evaluated in two series of patients referred to the Orthopaedic Oncology Group (the Center) because of suspected malignant soft-tissue tumors. Changes in the surgical treatment over time was determined. The annual incidence of sarcoma was 1.4/10(5) with a 30 per cent male preponderance. The mean age was 58 years. The most common histologic types were malignant fibrous histiocytoma, liposarcoma and leiomyosarcoma. Twenty-three per cent were histologically classified as low-grade malignant (Grades I and II), 33 per cent as Grade III and 44 per cent as Grade IV tumors. Slightly more than one half of the tumors were deep and these had a median size of 8 cm compared to 4 cm for the superficial tumors. One third of the tumors were located in the thigh. The annual clinical incidence of solitary subcutaneous lipoma was estimated to 1/10(3). Four fifths of the lipomas were smaller than 5 cm and they were most common in the trunk, shoulder and upper arm. By comparing clinical data for benign tumors and sarcomas it was found that a tumor 5 cm or larger or a deep tumor is relatively more likely to be a sarcoma. Patients with tumors of that size and depth should be referred before surgery. The probability of a benign cytodiagnosis being correct was 0.97 while that of a malignant one was 0.85. For a cytodiagnostic report of sarcoma the probability of correct diagnosis was 0.84. In a prospective evaluation the planned surgery was changed by subsequent cytodiagnosis in one third of the patients. Negative prognostic variables as regards survival in the order of decreasing relative risks were: Malignancy Grades IV and III, pain, male sex, increasing age and tumor size, marginal surgery and extracompartmental tumor site. Marginal excision, extracompartmental tumor site and age exerted their effect only via local recurrence which had a stronger negative influence on survival than any of the other variables. A linear survival function based on the prognostic variables was related to the probability of survival and the patients could be stratified into subsets with significantly different survival.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMed1993

Fusion of CHOP to a novel RNA-binding protein in human myxoid liposarcoma.

Human myxoid liposarcomas contain a characteristic chromosomal translocation, t(12;16)(q13;p11), that is associated with a structural rearrangement of the gene encoding CHOP, a growth arrest and DNA-damage inducible member of the C/EBP family of transcription factors residing on 12q13.1. Using a CHOP-specific complementary probe and antiserum we report here the presence of an abnormal CHOP transcript and protein in these tumours. Cloning of the translocation-associated CHOP gene product revealed a fusion between CHOP and a gene provisionally named TLS (translocated in liposarcoma). TLS is a novel nuclear RNA-binding protein with extensive sequence similarity to EWS, the product of a gene commonly translocated in Ewing's sarcoma. In TLS-CHOP the RNA-binding domain of TLS is replaced by the DNA-binding and leucine zipper dimerization domain of CHOP. Targeting of a conserved effector domain of RNA-binding proteins to DNA may play a role in tumour formation.

Amino Acid Sequence↗
PubMed1995

Intra-operative radiotherapy in soft tissue sarcomas.

We treated 31 soft tissue sarcoma bearing patients with intraoperative radiation therapy (IORT) with ages ranging from 26 to 71: first curative intent treatment, 16 patients; and recurrent tumors, 15 patients. The tumor site was the pelvis and the retroperitoneal spaces in 13 patients and the limbs or the trunk in 18 patients. The histological type was: malignant histiocytofibroma, 14 patients; liposarcomas, 10 patients; malignant schwanoma, 1 patient; leiomyosarcoma, 2 patients; hemangiopericytoma, 1 patient; embryonic rhabdomyosarcoma, 2 patients; and synovialosarcoma, 1 patient. All the patients were diagnosed without any distant metastatic evolution at the moment of the treatment. All the patients except one underwent a complete surgical excision without any gross residual disease and received an intraoperative radiation single dose of 10 Gy in one case, 12.5 Gy in one case, 13 Gy in one case, 15 Gy in 17 cases, 18 Gy in three cases, 20 Gy in seven cases and 25 Gy in one case. Thereafter the treatment was completed by a postoperative X-ray dose of 45-50 Gy in 4.5-5 weeks for 16 patients. Local control (LC) was obtained in 27 out of 31 patients (87%), with a minimal follow-up duration of 2 years. Eleven out of 31 patients died: seven with local control (one from an intercurrent disease, six from distant metastasis) and four with local failure inside the IORT fields. Twenty patients are alive with no evolutive disease in 19 cases and with a distant metastasis in one case.(ABSTRACT TRUNCATED AT 250 WORDS)

Abdominal Neoplasms↗
PubMed1998

Logistic regression analysis of myxoid sarcomas: a cytologic study.

The objective of this study was to identify key diagnostic cytologic criteria for the most common myxoid sarcomas studied by fine-needle aspiration cytology. We reviewed 27 myxoid malignant fibrous histiocytomas, 8 chordomas, 16 chondrosarcomas, and 12 myxoid liposarcomas in which both cytologic specimens and final histopathologic diagnoses were available. All specimens were coded as to the presence or absence of the following variables: high cellularity, low cellularity, tissue fragments, epithelial fragments, pale/loose ground substance, dense ground substance, chondroid fragments, large amount of myxoid material, small amount of myxoid material, capillary vessel networks, pleomorphism, binucleate cells, multinucleate cells, physaliphorous cells, cells in lacunae, signet ring cells, lipoblasts, fibroblast-like cells, histiocyte-like cells, stellate cells, long filamentous cells, short spindle cells, osteoclastic giant cells, nuclei with pointed ends, nuclei with cigar-shaped ends, fish-hook nuclei, round/ovoid nuclei, naked nuclei, large nucleoli, small nucleoli, mitotic figures, abnormal mitotic figures, intracytoplasmic hemosiderin deposits, background cells, fat, cytoplasmic vacuoles, and pleomorphic giant cells. A logistic regression analysis was performed to identify the variables predictive of myxoid malignant fibrous histiocytoma, chordoma, myxoid chondrosarcoma, and myxoid liposarcoma. The statistical analysis selected pleomorphic giant cells and the presence of fibroblast-like cells as most predictive of malignant fibrous histiocytoma, physaliphorous cells as most closely associated with chordoma, chondroid fragments as most predictive of chondrosarcoma, and lipoblasts as most predictive of liposarcoma. While myxoid lesions have many overlapping cytologic features, key criteria including the presence of lipoblasts, physaliphorous cells, chondroid fragments, and pleomorphic giant cells are useful in subclassifying these neoplasms.

Adipocytes↗
PubMed1990

Negative scintigraphy with positive magnetic resonance imaging in bone metastases.

We report three patients with known primary tumor in whom radionuclide skeletal imaging for metastatic disease was normal with or without clinical symptomatology referable to this area. Magnetic resonance imaging (MRI) of the spine demonstrated focal areas of abnormal signal intensity in the vertebral bodies of these patients. In all three patients, biopsy confirmed metastatic disease. All the patients received radiation or chemotherapy depending upon the etiology. These preliminary data suggest that MR imaging may be useful in evaluating patients with known primary tumor in whom clinical suspicion persists despite a negative radionuclide bone scan.

Bone Marrow↗
PubMedDate not supplied

Carbon-11 choline positron emission tomography in musculoskeletal tumors: comparison with fluorine-18 fluorodeoxyglucose positron emission tomography.

PURPOSE: Recently, a new positron emission tomography (PET) tracer, carbon-11 choline, has been introduced in oncology investigations, but the role of choline PET in musculoskeletal tumor evaluation has not been previously examined. This is the first trial to investigate the utility of choline PET in evaluating musculoskeletal tumors in comparison with fluorine-18 fluoro-2-deoxy-D-glucose (FDG) PET. METHOD: Thirty-three patients were examined with both choline PET and FDG PET, of which standardized uptake values (SUVs) were used for evaluation of the lesions. To decide the appropriate cutoff value and compare the two PET studies, receiver operating characteristic curve analysis was used. The binomial test was used for comparison of sensitivities between choline PET and FDG PET. RESULTS: A significant correlation (r = 0.537, P = 0.0013) between choline and FDG SUVs was noted for all lesions (n = 33). Mean SUVs for malignant tumors were significantly higher than those for benign lesions in both choline PET and FDG PET. Using a cutoff value of 2.7 for choline SUVs, the sensitivity for correctly diagnosing malignancy was 92.3% (12/13) with a specificity of 90.0% (18/20), resulting in an accuracy rate of 90.9%. With use of a cutoff value of 3.3 for SUVs in FDG PET, the sensitivity was 84.6% (11/13) with a specificity of 80.0% (16/20), resulting in an accuracy rate of 81.8%. The receiver operating characteristic curves of two analyses showed that the mean area under the curve value of choline PET (0.9577 +/- 0.041) was significantly greater (P = 0.0488) than that of FDG PET (0.8192 +/- 0.0806). There was no significant difference in sensitivity and specificity between choline PET and FDG PET analysis using either the binomial test (P = 0.4531) or McNemar test (P = 0.371). CONCLUSION: Choline PET analysis may not be inferior to FDG PET analysis for differentiating malignant from benign musculoskeletal tumors. The advantages of choline PET were shorter examination time and little retention in the bladder; therefore, this modality may be useful for preoperative planning for musculoskeletal tumors, especially for lesions around the hip joints.

Adolescent↗