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PubMed1998

[Uterine sarcoma. Analytic study of 37 cases].

The lack of uniformity in the nomenclature of the uterine sarcomas, it have contributed to a variety and variability of classifications. Fortunately the sarcomas of uterus are rare. The incidence of this tumor is of 3-5% of all the uterine cancers or of 1.7/100,000 women of 20 years or more. The clinical presentation of these tumors is diverse could come bleed uterine abnormal, abdominal pain, pelvic mass, discharge or cervix prominent mass. Clinical discoveries associated with exist the sarcomas how they are the obesity and high blood pressure in a 30% of the patients it are also observed antecedents of pelvic radiation in a 5-10% of the cases. The genomic alterations that is reported the chromosomes in the literature is associated with 1, 7 11 playing an important paper in the initiation or progression of the sarcomas. We was carried out a retrospective analysis of 37 cases of uterine sarcoma managed in the National Institute of Cancerology at one time of 5 years. Being that the leiomiosarcomas comes in the 51.3% of the cases, followed by the stromal sarcoma, bleed uterine abnormal it was the clinical fact of high importance, detecting these patients in Ia and IIa stadiums predominantly. We observed an increment in the incidence of the uterine sarcoma in patients of 40 years or more. 17 patients were managed exclusively with surgery, 17 patients with surgery and radiotherapy and 5 patients with surgery and chemotherapy (2 patients were managed with surgery + radiotherapy + chemotherapy). The index of failure was from the 45.1% to two years in general form, coming metastasic illness in lung, liver and breast mainly. In conclusion, the adjuvant radiotherapy and chemotherapy to the hysterectomy doesn't increase the index of survivor in the several subtype of uterine sarcomas.

Combined Modality Therapy↗
PubMed1988

Ins(10;19) in an endometrial stromal sarcoma.

A 37-year-old woman with a history of bilateral mesenchymal cystic hamartomata of the lung was subsequently found to have low-grade endometrial stromal sarcoma. Cytogenetic analysis of the uterine tumor revealed, in all metaphases, an unusual derivative chromosome generated by the insertion of chromosome 19 into chromosome 10 at the level of its centromere [ins(10;19)(p11;p13q13)].

Adult↗
PubMed2003

Fine-needle aspiration of synovial sarcoma: criteria for diagnosis: retrospective reexamination of 37 cases, including ancillary diagnostics. A Scandinavian Sarcoma Group study.

The cytologic criteria of synovial sarcoma in fine-needle aspirates were defined by a retrospective examination of 37 primary tumors. Irrespective of subtype, a typical pattern at low power was found, provided the yield was rich. The typical pattern was a mixture of dispersed cells with the presence of striped nuclei and cell-tight tumor tissue fragments with irregular borders. Often a branching network of vessels was present in the fragments, imitating a true vascular tumor. Except in poorly differentiated synovial sarcomas, the tumor cells were, small to medium in size, with rounded, ovoid, or fusiform bland nuclei with inconspicuous nucleoli. In the biphasic variant, small glandular- or acinar-like structures were present, although not in all cases. In the poorly differentiated type, however, the cellular pleomorphism was marked with the presence of cells with irregular nuclei and rhabdomyoblast-like cells, corresponding to the pleomorphic variant. The Ewing's sarcoma-like and the atypical spindle cell variants of poorly differentiated synovial sarcoma were not diagnosed in the material. An unequivocal diagnosis of sarcoma is possible when the yield is rich. However, ancillary diagnostics are necessary for a correct diagnosis, to avoid important pitfalls, such as other sarcomas with bland tumor cells and vessel-rich tumor fragments, in particular, solitary fibrous tumor and true hemangiopericytoma. Electron microscopic and/or molecular genetic analyses were better diagnostic adjuncts than immunocytochemistry.

Biopsy, Fine-Needle↗
PubMed1989

[Clinical treatment of 37 cases of soft tissue sarcomas in the past 6 years].

Since 1981, 37 cases of soft tissue sarcomas have been treated in our clinic. There were 20 males and 17 females with an average age of 43.5 years old. Histopathological classifications were liposarcomas 9 cases, MFH 8 cases, synovial sarcoma 5 cases, rhabdomyosarcoma 3 cases etc. Twenty-four cases (65%) were located in lower extremity, 11 cases (30%) in upper extremity, and 2 cases (5%) in trunk. All cases were treated with surgical intervention. Postoperative radiation was undertaken in 4 cases and adjuvant chemotherapy in 12 cases, respectively. There were 28 cases (76%) of excisional surgery; i.e., so-called limb salvage operation, and 9 cases (24%) of amputation or disarticulation. In August 1987, 10 cases had died of disease (DOD) and 27 cases were alive, but 4 cases were alive with disease (AWD). Each of the DOD and AWD cases were in stage III (by Enneking's surgical staging system) at first arrival to our clinic, or multioperated cases or cases with rapid distant metastases soon after operation. In conclusion, it was emphasized that in order to improve the clinical results, the soft tissue sarcomas must be treated earlier and that more effective chemotherapeutical protocols should be developed in the future.

Adolescent↗
PubMed2002

Expression profiling of synovial sarcoma by cDNA microarrays: association of ERBB2, IGFBP2, and ELF3 with epithelial differentiation.

Synovial sarcoma is an aggressive spindle cell sarcoma with two major histological subtypes, biphasic and monophasic, defined respectively by the presence or absence of areas of glandular epithelial differentiation. It is characterized by a specific chromosomal translocation, t(X;18)(p11.2;q11.2), which juxtaposes the SYT gene on chromosome 18 to either the SSX1 or the SSX2 gene on chromosome X. The chimeric SYT-SSX products are thought to function as transcriptional proteins that deregulate gene expression, thereby providing a putative oncogenic stimulus. We investigated the pattern of gene expression in synovial sarcoma using cDNA microarrays containing 6548 sequence-verified human cDNAs. A tissue microarray containing 37 synovial sarcoma samples verified to bear the SYT-SSX fusion was constructed for complementary analyses. Gene expression analyses were performed on individual tumor samples; 14 synovial sarcomas, 4 malignant fibrous histiocytomas, and 1 fibrosarcoma. Statistical analysis showed a distinct expression profile for the group of synovial sarcomas as compared to the other soft tissue sarcomas, which included variably high expression of ERBB2, IGFBP2, and IGF2 in the synovial sarcomas. Immunohistochemical analysis of protein expression in tissue microarrays of 37 synovial sarcomas demonstrated strong expression of ERBB2 and IGFBP2 in the glandular epithelial component of biphasic tumors and in solid epithelioid areas of some monophasic tumors. Fluorescence in situ hybridization analysis indicated that the ERBB2 overexpression was not because of gene amplification. Differentially expressed genes were also found in a comparison of the expression profiles of the biphasic and monophasic histological subgroups of synovial sarcoma, notably several keratin genes, and ELF3, an epithelial-specific transcription factor gene. Finally, we also noted differential overexpression of several neural- or neuroectodermal-associated genes in synovial sarcomas relative to the comparison sarcoma group, including OLFM1, TLE2, CNTNAP1, and DRPLA. Our high-throughput studies of gene expression patterns, complemented by tissue microarray studies, confirm the distinctive expression profile of synovial sarcoma, provide leads for the study of glandular morphogenesis in this tumor, and identify a new potential therapeutic target, ERBB2, in a subset of cases.

Cell Differentiation↗
PubMed1990

Opportunistic agents in bronchoalveolar lavage in 99 HIV seropositive patients.

During a ten month period, 117 fibreoptic bronchoscopies and bronchoalveolar lavages (BAL) were performed in human immunodeficiency virus (HIV) infected patients suspected of having opportunistic pulmonary infections. The BAL were classified into 3 groups, according to clinical manifestations related to HIV infection at the time of fibreoptic bronchoscopy: pre-acquired immunodeficiency syndrome (AIDS) (n = 54), AIDS with Kaposi's sarcoma (n = 37), AIDS without Kaposi's sarcoma (n = 26). On chest X-ray, diffuse infiltrates were most common (54%), followed by normal X-rays (24%) and localized infiltrates (18%). Amongst the 117 BAL, 68 (58%) yielded at least one opportunistic agent. In 28 BAL performed for pulmonary signs or unexplained fever with normal chest X-rays, one or several opportunistic agents were isolated in 17 samples of BAL fluid. The most frequently identified opportunistic agents were Pneumocystis carinii (in 38% of BAL) and cytomegalovirus (35%); these were associated in 17% of BAL. There was no statistically significant difference in opportunistic agents among the 3 groups of BAL (pre-AIDS, AIDS with Kaposi's sarcoma, AIDS without Kaposi's sarcoma). In particular, cytomegalovirus was found in BAL with the same frequency in these 3 groups.

Acquired Immunodeficiency Syndrome↗
PubMed1984

[Treatment of Ewing's sarcoma with intensive initial chemotherapy. 1st evaluation of a French pediatric multicenter protocol].

From April, 1978 to December, 1982, 87 cases of Ewing's sarcoma were registered in 12 centres of paediatric oncology in France. The results of a therapeutic protocol consisting of intensive chemotherapy (vincristine, cyclophosphamide and actinomycin with or without adriamycin) prior to tumoral irradiation, followed by maintenance chemotherapy are reported after 3 years' follow-up from the beginning of treatment. In localized sarcomas, complete remission (66/70) persisted without relapse or metastasis in 54% of the cases (78% in sarcomas of long bones, and 37% in sarcomas of short bones). In non-metastatic sarcomas of the ribs, the first remission persisted in 7/14 patients (50%). All these patients had been initially treated with chemotherapy, radiotherapy and surgical excision. These preliminary results emphasize the value of co-operative therapeutic trials in a rare tumour, demonstrate the effectiveness of the initial chemotherapy used against Ewing's sarcoma and are in favour of a combined treatment, including systematic excision, for Ewing's sarcomas affecting the ribs.

Adolescent↗
PubMed2001

Keratin 1 expression in endothelia and mesenchymal tumors: an immunohistochemical analysis of normal and neoplastic tissues.

Keratin polypeptides of the nonhair type, numbered 1 through 20 in the Moll catalog, are selectively expressed in normal and neoplastic tissues. Keratin 1 (K1), the highest-molecular-weight keratin (67 kd), was generally considered specific for keratinizing squamous epithelia. However, recent studies have shown that it is an integral component of the multiprotein kininogen receptor of endothelial cells. A library of formalin-fixed, paraffin-embedded tissue samples was evaluated immunohistochemically (avidin-biotin peroxidase complex method) for K1 expression using a specific monoclonal antibody (Novocastra clone 34betaB4). The study group included a wide variety of normal tissues and 541 tumors of epithelial or mesenchymal derivation. The specificity of the antibody to K1 was verified in normal epithelial tissues, where the staining was essentially limited to the epidermis and Hassal corpuscles of the thymus and focally to other squamous epithelia. Among carcinomas, it was essentially limited to keratinizing squamous carcinomas. It was also regularly found in endothelial cells of normal capillaries, veins, and arteries. Capillary, cavernous, and venous hemangiomas often had endothelia with K1 positivity. Among the malignant vascular tumors, epithelioid hemangioendotheliomas were consistently positive (8 of 8). However, angiosarcomas had more variable expression (59 of 81 were positive), with well-differentiated tumors generally having greater reactivity than poorly differentiated examples. Mesenchymal tumors with K1 expression included schwannomas (10 of 16), epithelioid sarcomas (26 of 37), and synovial sarcomas (19 of 68). In the last 2 tumor types, K1 reactivity was detected in both epithelioid and spindled neoplastic populations. In addition to its specificity for keratinizing squamous epithelia, K1 can be immunohistochemically detected in normal vascular endothelial cells and a spectrum of vascular tumors. However, its expression in poorly differentiated vascular tumors is variable, suggesting that this marker is poorly conserved in highly transformed endothelia. The unexpected K1 immunoreactivity in nonvascular soft tissue tumors, such as synovial sarcoma, epithelioid sarcoma, and schwannomas, requires further study.

Adult↗