[On 3 cases of primary gastric neoplasms not frequently seen (2 reticulosarcomas and 1 fibrosarcoma)].
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The authors report 12 cases (8 men and 4 women) of sarcomatous degeneration in Paget's bone disease, with an average age of 72.3 years. Sarcomatous degeneration occurred often in polyostotic Paget's disease, and osteitis deformans was seen in 4 cases. Femur and pelvis were the most affected bones. Pain was a constant feature, whereas tumefaction and fracture were less common. Osteolytic lesions were more frequent than condensed or mixed lesions and radiological signs of malignancy were usually found. Seven cases were histologically classified as osteogenic sarcoma and 3 cases as fibrosarcoma. Electron microscopy was performed on 2 osteogenic sarcomas and in 1 case revealed microcylindrical inclusions in Pagetic osteoclasts and in multinucleated giant tumor cells, but none in mononucleated tumor cells. The average survival time for the patients in this study was only 4.5 months.
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Heart neoplasms are of increasing interest among clinicians and surgeons. A review of primary malignant cardiac tumors, secondary cardiac tumors and carcinoid heart tumors is presented, with special reference to their pathological and surgical aspects. Primary malignant heart tumors represent about 25% of all cardiac tumors, the great majority are sarcomas and the whole family of this group is described including angiosarcoma, rhabdomyosarcoma, fibrosarcoma, leiomyosarcoma, liposarcoma, neurogenic sarcoma, synovial sarcoma and osteosarcoma; mesothelioma, lymphoma, malignant teratoma and thymoma are also included. Metastatic heart tumors are 20-40 times more common than primary malignancies, their behavior and more relevant aspects in diagnostic and surgical therapy are mentioned. Carcinoid heart tumors represent a distinctive entity and are discussed individually.
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An alarmingly high rate of postirradiation sarcomas following treatment for retinoblastoma has been described in the literature. We present four new cases and report 57 others from the English literature. Osteogenic sarcoma was the predominant histologic type (58%), followed by fibrosarcoma (21%) and various other sarcomas (21%). The average latency period between irradiation and development of the second primary (sarcoma) was 12.4 years. Irrespective of irradiation, a genetic linkage between retinoblastoma and osteogenic sarcoma on the 13q14 chromosome is recognized. Through a pleiotropic effect of this same chromosome, a predisposition for other sarcomas may exist as well. Finally, a strong role for radiation induction is proposed for all of these postirradiation sarcomas. This is based on the increased number of sarcomas arising in the field of prior irradiation (sites uncharacteristic of spontaneously occurring primary sarcomas) and the prolonged latency periods.
Report on 5 cases of so called carcinosarcomas of the larynx and hypopharynx and review of literature. These polypoid tumors have a small epidermoid carcinomatous part and a major part with sarcoma like appearance. The sarcomatoid tissue resembles to fibrosarcomas or some undifferentiated pleomorphic sarcoma. Observations of a gradual transition from the epithelial tumor cells to cells with a mesodermal appearance show that these tumors are varieties of squamous cell carcinomas (spindle cell or pleomorphic variety) and not "combination"--or "collision-tumors", "carcinosarcomas" or "seudosarcomas". These tumors appear in the larynx predominantly in man, at the same sites and in the same ageclasses as common squamous cell cancers and are not less malignant. The sarcomatoid tissue can arise at the beginning of the disease or at a later stage it can appear or disappear after operation or irradiation. The reason for this change of cellular structure is unknown.
A human fibrosarcoma cell line, HT-1080, and four new cell lines (HS-16, HS-28, HS-30, and HS-42) were established from untreated patients with mesenchymal chondrosarcoma, peripheral nerve sheath sarcoma, malignant hemangiopericytoma, and mixed mesodermal tumor, respectively, and were used for analysis of mechanisms of intrinsic resistance to methotrexate. All four new cell lines were resistant to methotrexate as determined by inhibition of thymidylate synthase in whole cells and by growth inhibition, as compared with HT-1080, a methotrexate sensitive cell line. Methotrexate uptake, level of dihydrofolate reductase, and inhibition of this enzyme by methotrexate in the four cell lines were comparable to HT-1080 cells. However, levels of long chain polyglutamates (glu3-5) of methotrexate achieved after a 24-h incubation with this drug were much lower in the four new cell lines as compared to the HT-1080 cell line (5- to 20-fold lower). The low levels of methotrexate polyglutamates formed is likely the major cause of intrinsic methotrexate resistance in these new sarcoma cell lines.
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From 1949 to 1974, 17 patients with sarcomas of the larynx were treated at the Mayo Clinic. Histologically, the sarcomas were classified as fibrosarcoma (six tumors), chondrosarcoma (seven tumors), rhabdomyosarcoma (three tumors), and osteosarcoma (one tumor). Patients with chondrosarcomas tended to differ from patients with other sarcomas in respect to origin of the sarcoma and length of survival free of disease.
Morphological transformation of NIH/3T3 cells by transfection with DNA has been used to identify transforming sequences in human tumours. Transforming activity has been reported for DNAs isolated from bladder, mammary, colon and lung carcinomas, neuroblastoma, lymphoid and myeloid tumours. Each of these tissues seems to contain different transforming sequences except for the colon and lung tumours where the same sequence seems to be involved. We now report that in two different human sarcoma cell lines, a fibrosarcoma and an embryonal rhabdomyosarcoma, the DNAs have transforming activity. The transforming gene is the same in both sarcomas but differs from the activated sequences detected in other tumours. We have also found that the transforming gene has no detectable homology to eight retrovirus oncogenes tested.
We have reviewed 16 cases of soft tissue sarcomas of the upper limb treated with wide excision of the tumour and immediate soft tissue reconstruction by means of a microvascular flap. 9 patients were males, 7 females; age ranged from 12 to 74 years. All the patients had been referred to us after previous failed or inadequate excisions. All the tumours were high-grade malignancies in an extracompartmental location (stade IIB according to Enneking). Histopathologic diagnosis was: synovial-sarcoma in 11 cases; fibrosarcoma in 2 cases; malignant fibrous histiocytoma in 1 case, soft tissue osteosarcoma in 1 case, epitheloid sarcoma in 1 case. The site of the tumour was the hand in 2 cases, the wrist in 6 cases, the forearm in 5 cases and the elbow in 3 cases. The free flaps used for soft tissue reconstructions were the latissimus dorsi in 6 cases, the dorsalis pedis in 2 cases, the lateral arm flap in 2 cases, the serratus in 1 case, the gracilis in 1 case. The island flaps were the radial forearm flap in 1 case, the posterior interosseous flap in 1 case, the cubital flap in 1 case. 14 cases required immediate associated reconstructive procedures such as tendon transfers or grafts (5 cases), vascularized or non vascularized bone grafts (4 cases), articular allografts (1 case), ligament reconstruction (1 case), nerve grafts (2 cases). Brachytherapy was associated to microsurgical reconstruction in the last 6 cases of this group. The catheters were charged with the radioisotope 5 days after surgery. No complications were observed.(ABSTRACT TRUNCATED AT 250 WORDS)
Spindle cell sarcomas often present the surgical pathologist with a considerable diagnostic challenge. Malignant peripheral nerve sheath tumor, leiomyosarcoma, fibrosarcoma, and monophasic synovial sarcoma may all appear similar histologically. The application of ancillary diagnostic modalities, such as immunohistochemistry and electron microscopy, may be helpful in the differentiation of these tumors, but in cases in which these adjunctive techniques fail to demonstrate any more definitive evidence of differentiation, tumor categorization may remain difficult. Cytogenetic and molecular genetic characterization of tumors have provided the basis for the application of molecular assays as the newest components of the diagnostic armamentarium. Because the chromosomal translocation t(X;18) has been observed repeatedly in many synovial sarcomas, it has been heralded as a diagnostic hallmark of synovial sarcoma. To formally test the specificity of this translocation for the diagnosis of synovial sarcoma, RNA extracted from formalin-fixed, paraffin-embedded tissue from a variety of soft tissue and spindle cell tumors was evaluated for the presence of t(X;18) by reverse transcriptase-polymerase chain reaction. Although 85% of the synovial sarcomas studied demonstrated t(X;18), 75% of the malignant peripheral nerve sheath tumors in our cohort also demonstrated this translocation. We conclude that the translocation t(X;18) is not specific to synovial sarcoma and discuss the implications of the demonstration of t(X;18) in a majority of malignant peripheral nerve sheath tumors.
Spindle cell sarcomas often present the surgical pathologist with a considerable diagnostic challenge. Malignant peripheral nerve sheath tumor, leiomyosarcoma, fibrosarcoma, and monophasic synovial sarcoma may all appear similar histologically. The application of ancillary diagnostic modalities, such as immunohistochemistry and electron microscopy, may be helpful in the differentiation of these tumors, but in cases in which these adjunctive techniques fail to demonstrate any more definitive evidence of differentiation, tumor categorization may remain difficult. Cytogenetic and molecular genetic characterization of tumors have provided the basis for the application of molecular assays as the newest components of the diagnostic armamentarium. Because the chromosomal translocation t(X;18) has been observed repeatedly in many synovial sarcomas, it has been heralded as a diagnostic hallmark of synovial sarcoma. To formally test the specificity of this translocation for the diagnosis of synovial sarcoma, RNA extracted from formalin-fixed, paraffin-embedded tissue from a variety of soft tissue and spindle cell tumors was evaluated for the presence of t(X;18) by reverse transcriptase-polymerase chain reaction. Although 85% of the synovial sarcomas studied demonstrated t(X;18), 75% of the malignant peripheral nerve sheath tumors in our cohort also demonstrated this translocation. We conclude that the translocation t(X;18) is not specific to synovial sarcoma and discuss the implications of the demonstration of t(X;18) in a majority of malignant peripheral nerve sheath tumors.
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A 26-year-old woman was operated on for a bulky tumor in the sacral region; she died of massive local tumor recurrence and pulmonary metastases 3 months later. Most of the original tumor showed a highly cellular spindle-cell sarcoma compatible with a fibrosarcoma of a high grade of malignancy. In a few small areas of the tumor, a chordoma-like pattern surrounded by growth of spindle-cell sarcoma was found. The spindle-cell component exhibited vimentin positivity in all tumor cells, but many cells were also cytokeratin-positive. The chordoma-like areas showed cytokeratin in all tumor cells. The chordoma-like areas, but not the spindle-cell areas also were positive for epithelial membrane antigen and S-100 protein. This case indicates that the sarcomatous change associated with chordoma may contain keratins as a sign of epithelial differentiation, and may thus represent sarcomatous transformation of chordoma cells, rather than a coincidental soft-tissue sarcoma or collision tumor.