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PubMed1991

[Liposarcoma of the infratemporal fossa. Apropos of a case].

The authors report about one female patient with a liposarcoma of the right infratemporal fossa. Such tumors are rare, making up 10 to 12% of all soft-tissue sarcomas. Their occurrence in a cervical site is rare, unlike that in the inguinal region, the retroperitoneum or the lower limbs. Their diagnosis is mainly established by the histopathological study. Computed tomography and magnetic resonance imaging are particularly useful to choose the surgical approach. The technique chosen for this infratemporal tumor was a transmandibular cervical approach. Evolution was favorable, and the patient has currently had no reoccurrence after two years.

Adult↗
PubMed1992

DNA ploidy and karyotype in recurrent and metastatic soft tissue sarcomas.

To study mechanisms involved in evolution of soft tissue sarcomas, we compared DNA ploidy and karyotypes at different stages of their disease in two patients with myxoid liposarcomas (MLS), one with a fibrosarcoma (FS), and two with rhabdomyosarcomas (RMS). None of the MLS samples revealed clearcut histologic changes in later samples as compared to their primaries, and the DNA ploidy in all samples was diploid. In one patient karyotypes at four different times during the 19 yr of his disease all revealed a t(11;12) (p15;q13), but additional clonal chromosomal abnormalities occurred only in later recurrences. In another patient the karyotypes obtained in the 26th and 28th yr of his disease were similar and included the t(12;16) (q13;p11), characteristic of MLS. A comparison with karyotypes of six other MLS patients at different disease stages suggests that the presence of a t(12;16) may correlate with less aggressive clinical behavior. The histology of the FS remained low-grade and the DNA ploidy diploid. The karyotype, however, showed evolution. In both MLS and FS, chromosomal changes thus seem to be a more sensitive marker for tumor progression than histologic changes or DNA ploidy. In one embryonal RMS, karyotypes obtained 7 and 11 yr after the primary diagnosis were different but clearly had a common "progenitor." In one alveolar RMS, the primary and the synchronous lung and lymph node metastases all revealed a t(2;13). The findings in RMS suggest that polyploidization is an early event in tumor evolution, especially in the alveolar subtype, which may be followed by additional chromosomal changes. In addition, DNA ploidy was measured in eight other RMSs. Among the RMSs the embryonal subtype was characterized by DNA aneuploidy, whereas three of the alveolar cases were in the tetraploid range and one was peridiploid. In local recurrences and in metastases changes in DNA index were observed in half the cases.

Adolescent↗
PubMed1982

The role of chemotherapy as an adjuvant to surgery in the initial treatment of primary soft tissue sarcomas in adults.

A prospective study was carried out to determine the results of chemotherapy as an adjuvant to surgery in the initial treatment of adult primary soft tissue sarcomas. The results were compared with those in a group of patients with similar histologic types of primary sarcoma treated by surgery alone. The chemotherapy regimen consisted of adriamycin, 60 mg/m2 intravenously on Day 1, and DTIC, 250 mg/m2 on Days 1 through 5. The cycle was repeated every 22 days. The total dose of adriamycin was 500 mg/m2; the DTIC was continued for 1 year. The adjuvant chemotherapy group consisted of 113 patients (group one) and the concurrent surgical resection group consisted of 144 patients (group two). In group one, 53 tumors were T1 and 60 tumors were T2; 67 of the tumors were grade 3 and 46 were grade 4. In group two, 65 had T1 and 79 had T2 tumors; 82 tumors were grade 3 and 62 were grade 4. The anatomic location and histologic types were similar in both groups. Seventy-seven (77%) of the 113 patients in group one lived disease free for 2 years, compared with 59% of the 144 patients treated by resection alone. Of the patients eligible for 5-year survival analysis, 74% were disease free in the adjuvant chemotherapy group, compared to 50% in the surgical group. The incidence of local recurrence was about the same in both groups. From a histologic standpoint, malignant fibrous histiocytomas and myogenic sarcomas appeared to benefit most with use of the use of the two-drug regimen used here, and the least favorable response was in liposarcomas and fibrosarcomas. In other histologic types, the role of adjuvant chemotherapy needs further clarification.

Adult↗
PubMed2001

The association between tumour progression and vascularity in myxofibrosarcoma and myxoid/round cell liposarcoma.

Angiogenesis is an important factor in the morphological progression and metastasis of many solid tumours. We studied two homogeneous series of myxofibrosarcoma (MFS) and myxoid/round liposarcoma (MRLS), characterised by distinct vascular patterns and correlated the intratumoral microvessel density (IMD) with morphologic progression in both types of sarcoma. In our study, 43 cases of MFS and 42 cases of MRLS were graded according to established diagnostic criteria. For evaluation of IMD, representative sections were stained immunohistochemically for CD31. After selection of "neovascular hot spots", IMD was calculated by measuring the endothelial surface within twenty 200x fields in relation to the total analysed area. In addition to the correlation of IMD with histological grades of malignancy, a correlation of IMD with the inflammatory infiltrate in MFS was done. To determine whether vascular endothelial growth factor (VEGF) and its receptors, KDR and flt-1, may play a role in the progression of both types of sarcomas, we used mRNA in situ hybridisation (ISH) to study VEGF, KDR and flt-1 expression in selected cases. In addition, the expression of thrombospondin-1, which has been reported to inhibit angiogenesis, and of collagen type I was studied using mRNA ISH. Cases of MFS varied histologically from hypocellular, mainly myxoid, neoplasms (low-grade malignant, 18 cases) to intermediate-grade malignant lesions with increased cellularity and mitotic activity (13 cases), and high-grade malignant cases with marked pleomorphism, high proliferative activity and areas of necrosis in many cases (12 cases). Cases of purely low-grade myxoid liposarcoma (16 cases) were characterised by low-cellularity, mucin pooling and plexiform vasculature. In combined MRLS, these hypocellular areas were admixed with hypercellular, round cell areas (5-80% of the analysed tumour area; 23 cases), and in round cell liposarcoma (three cases) rounded tumour cells predominated (>80% of the analysed tumour area). The average IMD in intermediate and high-grade malignant MFS (4.03 and 4.09, respectively) was significantly higher than in low-grade malignant MFS (2.73). Correlation of vascularity with the inflammatory infiltrate in MFS showed increased IMD only in cases with abundant neutrophils; most of these cases were high-grade malignant neoplasms. In contrast, no statistical correlation between morphological progression and IMD was seen in myxoid liposarcoma (6.08), MRLS (6.57) and round cell liposarcoma (4.07). VEGF mRNA was expressed by tumour cells in all histological grades of MFS and MRLS. VEGF receptor mRNA was weakly expressed by endothelia of newly formed blood vessels in both entities. Interestingly, tumour cells of all analysed cases of MFS strongly expressed collagen type I and thrombospondin-1, while these proteins were not detected in tumour cells of MRLS. In conclusion, morphologic tumour progression in MFS is associated with increased IMD, whereas, in MRLS, no such correlation is seen. Whereas VEGF and VEGF receptor mRNA were expressed in both entities, a characteristic expression profile of collagen type I and thrombospondin-1 in MFS emerged. Further studies are necessary to correlate vascularity and clinical course in MFS and MRLS.

Adolescent↗
PubMed1999

[Malignant phyllodes tumor with liposarcomatous differentiation. Description of a clinical case].

Sarcoma of the breast are a rare group of neoplasms representing less than 5% of Soft Tissue Sarcomas (STS). Between 1980 and 1995 in the National Institute foe Cancer Research (IST) of Genoa, 2188 patients were submitted to surgery for breast cancer. Seven of them were found to be affected by sarcoma, confirmed by histologic diagnosis. All the patients were between 39 and 87 years-old. Surgical treatments were: wide excision (1 case), total mastectomy (2 cases), radical mastectomy following Halsted (4 cases). A case of a 53 year-old woman with a phyllodes tumor initially transforming in to liposarcoma is reported. The patient was submitted to surgery (total mastectomy) and nowadays is alive and free of disease after 29 months. All authors agree that the treatment for sarcoma of the breast is early and complete surgical excision of the mass. The role of chemotherapy and radiotherapy is still uncertain. Outcome is based on histologic type, degree of differentiation and tumor size.

Adult↗
PubMed1994

Sweet syndrome associated with liposarcoma: a case report.

A 36-year-old woman with a dedifferentiated liposarcoma is described who had suffered from Sweet syndrome in the past. From the literature a relationship between this syndrome and malignancy is known to exist. However, the combination of Sweet syndrome with a sarcoma has not previously been reported.

Abdominal Neoplasms↗
PubMedDate not supplied

The pathology of head and neck tumors: fibroadipose tissue and skeletal muscle, Part 8.

Benign and malignant tumors originating from mesenchymal cells destined to become lipoblasts and myoblasts affect the head and neck with contrasting frequencies. Lipomas and especially liposarcomas are unusual lesions above the clavicles but when found there behave in a biologic manner identical to that of their counter-parts at other anatomic sites. Myogenic tumors, on the other hand, have a predilection for the head and neck, and for rhabdomyosarcomas this predilection is accentuated in childhood. Combination therapy of rhabdomyosarcomas has obviated radical surgery as a method of treatment, and many sites in the head and neck have benefited prognostically by this treatment. Success, however, is dependent on clinical stage of disease, and rhabdomyosarcomas of the nasopharynx, paranasal sinuses, and middle ear remain more resistant to short-term cures because of the extent of the neoplasm. A review of the clinicopathologic aspects of granular-cell tumors and alveolar soft-part sarcomas is also presented because it has been suggested that these tumors have a myogenous origin.

Adult↗
PubMed1971

Human sarcomas in culture. Foci of altered cells and a common antigen; induction of foci and antigen in human fibroblast cultures by filtrates.

In a study of human sarcomas maintained in culture for periods up to two years, the following observations were made. The most prominent cell type in serially cultured osteosarcomas was fibroblastic in appearance. After 16-20 wk in culture some lines spontaneously developed foci of altered cells resembling the foci produced in monolayer cultures by oncogenic viruses. The presence of these foci in the sarcoma cultures was transient, and usually they did not reappear; but in one instance they recurred with a characteristic periodicity of several weeks. From one of the sarcoma lines, in which foci appeared after 5 months in culture, two subcultures were established from stored frozen cells and these both exhibited foci after approximately the same lapse of time. The same phenomenon has been seen with another line, suggesting that the time of appearance of foci is characteristic for particular sarcomas. Foci of similar type could sometimes be induced in monolayer cultures of human fibroblasts by filtered medium from cultured sarcomas; this bore no relation to the presence or absence of foci in the sarcoma cultures at the time the filtrate was prepared. Electron microscopy of the spontaneous and induced foci, and of the sarcoma cultures, revealed no demonstrable virus. 12 out of 15 sarcoma cultures contained an antigen (S) demonstrable by indirect immunofluorescence with human sera. It was not present in any of the original sarcoma specimens, nor in any culture lines other than sarcomas. At least 3-4 wk in culture appear to be required for its demonstration. The antigen was cytoplasmic, occurred in only a small proportion of the cells, and was unpredictably variable in its expression, even in the same culture line. It could be induced in monolayer cultures of human fibroblasts by filtrates of medium from sarcoma cultures. As with the foci, the induction of S antigen in indicator cultures was not dependent upon the expression of antigen in the sarcoma line from which the filtrates were obtained. There was no association between the presence of foci and of antigen, nor was there any apparent relation between the ability of filtrates to induce foci and their ability to induce antigen. 80% or more of the general population have S antibody, and the titer of antibody in patients with sarcoma is no higher than in normal subjects. Thus, as in the case of Burkitt's lymphoma antigen, it appears that most individuals have been exposed to S antigen. But unlike Burkitt's lymphoma, no relation has so far been established between any particular disease and a corresponding high titer or frequency of occurrence of S antibody. The occurrence of foci of altered cells and of a common antigen, and the transmission of these two characters to indicator cells by filtrates, are all suggestive of a virus specifically associated with human sarcomas, one to which the general population is widely exposed, as indicated by the prevalence of antibody.

Adenocarcinoma↗
PubMed2003

Histopathologic type: an independent prognostic factor in primary soft tissue sarcoma of the extremity?

BACKGROUND: We attempted to define the effect of tumor histotype on local recurrence, distant metastasis, and disease-specific survival in patients with surgically treated primary extremity sarcoma. METHODS: A total of 951 patients with primary, localized soft tissue extremity sarcoma were followed up prospectively. Patient- and tumor-related variables, including histopathologic type, were used to identify independent prognostic factors for the study end points of local recurrence, distant recurrence, and disease-specific survival. RESULTS: There were 137 local recurrences, and significant adverse prognostic factors for local recurrence were patient age >50 years, microscopically positive margins, and malignant peripheral nerve tumor. Adverse prognostic factors for distant recurrence (200 patients) were tumor size >5 cm, tumors beneath the investing fascia, high tumor grade, and leiomyosarcoma. Of the 199 patients who died of disease-related causes, patient age >50 years, tumors beneath the investing fascia, high tumor grade, microscopically positive margin, tumor size >5 cm, leiomyosarcoma, and malignant peripheral nerve tumor were adverse prognostic factors. CONCLUSIONS: These data suggest that differences in biological behavior may exist between sarcoma histotypes and deserve further study.

Adult↗
PubMed1988

Sarcomas of the breast: a clinico-pathological study of 67 patients with long term follow-up.

A national Danish series of 68 breast sarcomas from 66 women and one man (age 17-86 years) was investigated. Tumour sections from 22 stromal sarcomas (SS), 24 phyllodes tumors of malignant type (MCSP), seven phyllodes tumors of borderline type (BLCSP), four malignant fibrous histocytomas (MFH), eight liposarcomas, two angiosarcomas and one leiomyosarcoma were reviewed retrospectively, and all patients were traced with a minimum follow-up of 15 years. Tumor contours appeared to be the best prognostic factor in predicting the risk of metastatic spread. Stromal overgrowth of MCSP was considered less utilizable due to difficulties in distinguishing between MCSP with marked stromal overgrowth and SS, which we consider as a variety of MCSP and which only showed slightly increased death rates compared to MCSP (45% versus 38%). Both angiosarcomas and the leiomyosarcoma proved lethal, and the other sarcoma subtypes had a death frequency of about 50%, with the exception of BLCSP, neither of which proved lethal. All patients, who died from metastases, were dead within 5 years irrespective of treatment. No positive lymph nodes were identified at the time of primary treatment, supporting the findings of previous investigators. We therefore advocate simple mastectomy or local excision with a wide margin as sufficient treatment of breast sarcomas. The indications for adjuvant therapy is as yet unclarified.

Adolescent↗
PubMed2004

CA125 expression in epithelioid sarcoma.

OBJECTIVE: There has been no report on useful immunohistological markers for epithelioid sarcoma (ES) so far. The purpose of this study is to evaluate the positivity and specificity of CA125 as a marker for the correct diagnosis of ES. METHODS: This study was performed in 11 patients with ES (nine men and two women; distal type: 10 cases; proximal type: one case), 78 patients with other soft tissue tumors and nine with benign granulomas. The other soft tissue tumors consisted of six synovial sarcomas, six clear cell sarcomas, eight leiomyosarcomas, six rhabdomyosarcomas, five malignant peripheral nerve sheath tumors, ten malignant fibrous histiocytomas, 17 desmoid tumors, 14 liposarcomas, six squamous cell carcinomas (cutaneous SCC of the distal extremities), two rheumatoid nodules and seven foreign body granulomas. Immunohistochemical analysis for CA125 was performed for these 89 soft tissue tumors and nine granulomas using a labeled streptavidin biotin method. Immunohistochemical analysis of epithelial membrane antigen, cytokeratin, carcinoembrionic antigen, vimentin and CD34 was performed only for the 11 ES patients. RESULTS: CA125 was strongly expressed in 10 out of the 11 ES patients. EMA, cytokeratin and vimentin were also positive in all the cases. CEA was positive in two of the 11 patients. Immunohistochemical study in six ES patients showed expression of CD 34. The other 78 soft tissue tumors and nine granulomas did not express CA125. CONCLUSION: This study clearly revealed the specificity and positivity of CA125 in ES. These data indicate that CA125 may be a useful tumor marker for diagnosing ES.

Adolescent↗
PubMedDate not supplied

Cardiac encasement by metastatic myxoid liposarcoma.

Isolated cardiac metastasis from a primary liposarcoma of noncardiac origin is a rare occurrence. A patient who presented with biventricular failure and constrictive hemodynamics years after successful resection of a primary liposarcoma of the thigh is described. Extensive cardiac encasement by tumor was suspected on diagnostic imaging. Hemodynamic instability and multiorgan failure necessitated urgent exploratory sternotomy. The patient died intraoperatively. Extensive metastatic sarcoma limited to the heart was confirmed during surgical procedure. This case suggests that in clinical and pathological investigation of a cardiac mass, knowledge of previous extracardiac involvement with soft tissue sarcoma is essential.

Fatal Outcome↗
PubMed1980

A light and electron microscopic study with comments on their relation to malignant fibrous histiocytoma and angiosarcoma.

Analysis of 365 cases of malignant soft tissue tumors revealed 83 cases of liposarcoma. The ages of the patients were distributed between 18 and 86 years with a mean average of 54.3 years. Approximately 50% of the cases were located in the lower extremity. Histologically, they consisted of 5 types; well differentiated (17 cases), myxoid (49 cases), round cell (3 cases), pleomorphic (11 cases), and mixed (3 cases). The appearance of lipoblasts of either the signet-ring type or mulberry (multivacuolated) type with displaced nuclei was the common characteristic feature for all types. Electron microscopically, lipid droplets were found inside of endoplasmic reticulum in lipoblasts, and abundant glycogen granules were seen in the cells containing only few lipid droplets. The lipoblasts were usually located close to the vascular wall in the interstitium, and findings suggesting an intimate relation between pericytes and lipoblasts were encountered. Although the differential diagnosis of malignant fibrous histiocytoma and liposarcoma was made possible by the appearance of lipoblasts in the latter, storiform pattern and histiocyte-like cells sometimes appeared in poorly differentiated liposarcoma. This tendency was also found in angiosarcoma. A close follow-up of recurrent cases revealed that there are actually cases of liposarcoma with malignant fibrous histiocytoma-like pattern and angiosarcoma with malignant fibrous histiocytoma-like pattern. It was considered that malignant fibrous histiocytoma-like figures can appear in various poorly differentiated sarcomas.

Adult↗
PubMed1973

The induction of tumours in the guinea-pig with methylcholanthrene and diethylnitrosamine and their propagation in vivo and in vitro.

Tumour induction with diethylnitrosamine (DEN) and methylcholanthrene (MCA) has been studied in 3 strains of guinea-pig. A DEN concentration of 80 μg/ml drinking water daily proved too toxic but reasonable survival was obtained with 20 μg/ml 3 times per week in Hartley guinea-pigs and a local inbred strain. Heston Strain 13 guinea-pigs were particularly susceptible to the toxic effects of the diethylnitrosamine. In all3strains, 100% of the animals which survived the early toxic effects subsequently developed hepatomata, the mean time being 15 months. Methylcholanthrene was less toxic but more erratic as a carcinogen, the incidence of tumours in Hartley guinea-pigs varying from 18 to 100% in different experiments, the mean time of tumour development being 10 months.Three transplantable hepatomata and 3 transplantable sarcomata have been developed. The hepatomata are all predominantly hepatocellular carcinomata and the sarcomata comprise two liposarcomata and a fibrosarcoma. Successful shortterm primary cultures of hepatomata, sarcomata and of normal liver tissues have been accomplished. Established cell lines in tissue culture have been developed from one cholangiocarcinoma from an outbred guinea-pig and one transplanted hepatocellular carcinoma from an inbred guinea-pig.

Animals↗
PubMed2000

Immunohistochemical spectrum of GISTs at different sites and their differential diagnosis with a reference to CD117 (KIT).

Gastrointestinal (GI) stromal tumor (GIST) is the designation for the major subset of GI mesenchymal tumors and encompasses most tumors previously classified as GI smooth muscle tumors. Although GISTs typically express CD117 (KIT), often express CD34, and sometimes express alpha-smooth muscle actin (SMA), the relative frequency of these markers has not been characterized in large series of GISTs of different sites, and the CD117 expression has not been fully characterized in intra-abdominal tumors. In this study, we immunohistochemically analyzed 292 GISTs throughout the GI tract, including omentum and mesentery, and compared the immunoreactivities with 211 other tumors that may enter in the differential diagnosis. GISTs were defined in this study as CD117-positive primary spindied or epithelioid mesenchymal tumors of the GI tract, omentum, or mesentery. The CD34 positivity of GISTs varied from 47% in small bowel to 96 to 100% in rectum and esophagus, whereas SMA expression showed the opposite patterns and was most frequent in the GISTs of small bowel (47%) and rarest in the GISTs of rectum and esophagus (10-13%). Desmin was seen only occasionally. S100 positivity was rare but was seen most frequently in small intestinal GISTs (15%). True leiomyomas from esophagus, muscularis mucosae of colorectum, and pericolic leiomyomas similar to uterine leiomyomas were negative for CD117 and CD34 and positive for SMA and desmin (46 of 46). Inflammatory fibroid polyps of stomach and small intestine were negative for CD117 but were often positive for CD34 (6 of 8) and variable for SMA (3 of 8). Inflammatory myofibroblastic tumors involving gastric or colonic wall were negative for CD117 but some showed CD117-positive endothelia. GI schwannomas were all negative for CD117 and positive for S100 protein (11 of 11). Extremely focal CD117 positivity was seen in the neoplastic cells of some retroperitoneal leiomyosarcomas and liposarcomas. Among other CD117-positive tumors were intestinal metastatic melanomas (8 of 11) and extraskeletal Ewing's sarcomas (5 of 11), two of which were abdominal. In conclusion, strong CD117 expression defines most primary GI mesenchymal tumors as GISTs, which show different patterns for CD34 and SMA in various parts of the GI tract. Some unrelated CD117-positive tumors (melanomas, Ewing's sarcomas) should not be confused with GISTs.

Actins↗
PubMed1975

Immunofluorescence of soft-tissue tumors with anti-smooth-muscle and anti-skeletal-muscle antibodies.

One hundred thirteen soft-tissue tumors were studied by immunofluorescence using anti-smooth-muscle (SMA) and anti-skeletal-muscle antibodies (MGA). Of the classic leiomyomas examined, 94% manifested bright fluorescence with SMA, whereas cellular leiomyomas and leiomyosarcomas failed to stain. Of the rhabdomyosarcomas studied, 21% showed fluorescent cross-striations or fluorescent granules after incubation with MGA. None of 48 control tumors fluoresced with either antibody. No tumor vessel seen showed smooth-muscle fluorescence in its walls. Although of little diagnostic value, immunofluorescence utilizing specific human antibodies is a valuable tool with which to study some of the antigenic components of these neoplasms.

Adolescent↗