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A 40-yr-old woman with alveolar soft-part sarcoma and multiple hypervascular cardiac tumors involving both left and right ventricles is reported. Papillary muscle dysfunction and mitral valve prolapse with mitral regurgitation were caused by the largest tumor in the left ventricle.
Augmenting immunogenicity by genetically modifying tumor cells to express costimulatory molecules has proven to be a promising therapeutic strategy in murine tumor models and is currently under investigation in human clinical trials for metastatic cancer. However, there are significant technical and logistic problems associated with implementing strategies requiring direct gene modification of primary tumor cells. In an effort to circumvent these problems, we are developing a strategy in which the costimulatory signal required for tumor-specific T lymphocyte activation is provided by a genetically modified human fibroblast (trans-costimulation). We have evaluated the efficiency of CD80- and CD86-mediated trans-costimulation in the activation of human CD8+ and CD4+ T lymphocytes in MHC class I- and class II-restricted lymphoproliferation reactions. Our studies demonstrate that the efficiency of CD80- or CD86-mediated trans-costimulation of purified human CD8+ and CD4+ T lymphocytes is comparable to cis-costimulation under defined conditions. Moreover, a dose-response relationship consistent with the predicted two-hit kinetics of the reaction was evident in trans-costimulation reactions in which the ratio of target cells expressing either signal 1 or signal 2 was varied incrementally from 1:10 to 10:1. Importantly, the level of cell-surface CD86 required for trans-costimulation is equivalent to that constitutively expressed by human peripheral blood monocytes. These results may have significant implications for the clinical implementation of this type of cancer immunotherapy and also raise questions about the possibility of trans-costimulating autoreactive T lymphocytes in vivo.
Lung metastasis is a common finding, either before, with or after discovery of the primary tumor. Histological examination of a lung formation of unknown origin can lead to diagnosis of a primary tumor. We report the case of a 28-year old woman who presented an apparently primary tumor in the lower left pulmonary lobe. Pathology reported soft tissue alveolar sarcoma. The first manifestation of this rare soft tissue tumor can be a lung or brain tumor. Imaging evidenced the primary tumor in the left calf. Histology confirmed the diagnosis and the metastatic nature of the lung tumor.
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The authors describe a case of laryngeal paraganglioma (LP) occurring in a 57-year-old-woman. To date, 70 cases have been described in the literature. It is benign and recurrences are infrequent. The differential diagnosis with typical and atypical carcinoids, hemangiopericytomas, alveolar soft-part sarcomas, medullary thyroid carcinomas, malignant melanomas and metastatic renal cell carcinomas is supported by immunohistochemistry. Moreover, this tumor shows the immunohistochemical expression of galanin, a variably expressed marker of paragangliomas that it is not expressed in carcinoid tumors. Nevertheless, our observations militate against its role as a solitary marker but advocate its use in conjunction with other antibodies for the differential diagnosis of neuroendocrine neoplasms of larynx.
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Granular cell tumours (synonyms: myoblastmyomas, granular neuromas, granular cell myoblastomas) are localized predominantly in the head and neck area, especially in the region of tongue and larynx. Only 2 observations of an occurrence in the parotid gland exist till now in the world literature. This paper reports a further case of an occurrence in the parotid gland in a 6-years-old girl. The about cherrygreat tumour was distinctly limited and showed histologically a typic lobular arrangement of acidophilic granular cells. No recidive was observed in the prevailing postoperative phase of 3 years. The differential diagnosis of the granular cell tumorus is demonstrated to other tumour types with similar histological arrangement, expecially to the alveolar soft-part sarcoma, the acinic cell tumour and the non-chrom-affine paraganglioma (chemodectoma).
Peripheral blood lymphocytes obtained from patients by leukapheresis were cultured in RPMI 1640 containing human plasma and interleukin 2. The morphology, phenotypes and cytotoxicity of induced LAK cells were studied. Lymphoblastoid cells mainly proliferated were OKIa1+ cells and were thought to be LAK cells. Maximal cytotoxicity was obtained after two weeks of incubation. IL-2 enhanced the cytotoxicity of LAK cells. Autologous LAK cells induced by two weeks of incubation were injected into patients. One case of pulmonary metastases of breast cancer showed reduction and two lesions showed partial regression. Also, no new lesions appeared in the lungs of a patient with alveolar soft-part sarcoma.
A case is described of a child presenting with an extremely large orbital tumour, which had an alveolar pattern on histological examination. The differential diagnosis, which included alveolar rhabdomyosarcoma, alveolar soft-part sarcoma and non-chromaffin paraganglioma, could only be made on electron microscopic examination. Exact diagnosis was important because of prognostic and therapeutic implications. The presence of neurosecretory granules in tumour cells was demonstrated on electron microscopy, confirming the diagnosis on non-chromaffin paraganglioma, a benign tumour which is extremely rare in this site.
40 patients with various forms of malignant disease, who had already been subjected to conventional regimens of treatment, were treated between 1976 and 1981 at the Department of Chemotherapy, Vienna University, with high-dose methotrexate (MTX) as sole therapeutic agent. 18 patients received MTX in moderately high doses of 250 mg/m2 to 750 mg/m2 at 10-day intervals. 19 patients were treated with high doses of 5 to 15 g MTX at 10-day intervals. In 2 cases of severe malignant non-Hodgkin's lymphoma one patient received 2 X 1 g MTX with an interval of 19 days between doses and the other received a single dose of 5 g MTX by infusion. One patient with alveolar soft part cell sarcoma was given ultra-high therapy, with a cumulative dose of 205 g. None of the patients in the group given moderately high-dose MTX therapy, whereas three in the high-dose group had an objective remission. Objective remission was obtained neither in the two lymphoma patients nor in the ultra-high-dose treated case. Complications such as leucopenia and/or thrombopenia were found in 9%, reversible transaminase activity increases in 45%, as well as a decrease in creatinine clearance in 10% of the cases. Irreversible severe kidney insufficiency was found in none of the cases. One patient with lymphoma died as a result of severe toxic epidermiolysis with involvement of the gastrointestinal mucosa, whilst the other suffered from pulmonary complications in the form of the respiratory distress syndrome. On the basis of our experience the use of high-dose MTX therapy as an alternative method following trials of all conventional regimens is not recommended.
Alveolar soft tissue sarcoma is an unusual tumor, known to have a poor prognosis. Although a muscular origin has been supported by most authors, the histogenesis of such tumors remains unclear. We report a case of alveolar soft tissue sarcoma with histological, ultrastructural, immunohistochemical and flow cytometry study. Pour results support a myogenic origin of these tumors and demonstrate its aneuploid nature. Pulmonary metastasis occurred early in spite of intensive chemotherapy and surgical removal of the tumor.
SUMMARY: Specific gene fusions observed in solid tumors are extremely useful diagnostic markers. We report the development of a method based on real-time PCR which enables the detection upon identical PCR conditions of the different fusions specifically observed in Ewing tumors (ET), alveolar rhabdomyosarcoma (ARMS), synovial sarcoma (SS), small round cell desmoplastic tumors (SRCDT), extraskeletal myxoid chondrosarcoma, malignant melanoma of soft parts, congenital fibrosarcoma, and anaplastic large cell lymphoma. A simple assay, based on multiplexing of primers and probes, is described for the routine genetic diagnosis of small round cell tumors of children. It enables the detection of the five EWS-ETS, the two PAX-FKHR, the three SYT-SSX, and the EWS-WT1 fusions of ET, ARMS, SS, and SRCDT, respectively. The sensitivity of this test is high enough to detect all fusions, including the large EWS-FLI-1 transcripts, with the equivalent of 100 tumor cells as a starting material. This multiplex fluorescent analysis of chromosome translocations (MFACT) was validated in comparison with conventional RT-PCR on a series of 79 tumors. A major advantage of this method is that it completely abolishes the manipulation of PCR-products. It, therefore, considerably lowers the risk of cross-contamination linked to carry-over of RT-PCR products. It also constitutes an important step toward the complete automation of the detection of cancer-specific gene fusions.
Clinical and morphological manifestations of alveolar soft tissue sarcoma (ASTS) are presented on the basis of 19 examinations performed by the authors and data available in the literature. ASTS has been found to be more common in young women and to be usually located in the thick layers of the proximal parts of the extremities. The tumor generally shows slow growth and late occurrence of metastases. It has been demonstrated that ASTS has an organoid structure, namely alveolar grouping of cells, abundance of capillaries, and its cells contain PAS-positive inclusions. In atypical ASTS, the prognosis has been found to be less favourable. The crystalloid inclusions that are absent in other soft tissue neoplasms are a characteristic ultrastructural sign of ASTS. The genesis of ASTS remains controversial but it cannot be ruled out that it is related to the elements of the APUD-system.
UNLABELLED: Epithelioid sarcoma (ES) is a rare tumor, but extremely versatile, simulating easily both clinically and morphologically multiple benign lesions as granulomas and malignant tumors as achromic melanomas or carcinomas. Especially the histological aspects are suggesting for an epithelial tumor. The atypical biological behavior of tumor is conferred by: a propensity for lymph nodes metastases, large dimensions of tumor, unusual localizations. AIM: To report some peculiar histological and immunophenotypic aspects in soft parts epithelioid sarcoma. MATERIALS AND METHODS: We analyzed retrospectively three cases of epithelioid sarcomas with unusual clinico-morphological (large dimensions), histopathological (absence of necrosis) and immunohistochemical (IHC) patterns selected from 200 consecutive soft parts malignant tumors of "Victor Babeş" National Institute Bucharest files. For the immunohistochemical (IHC) staining, the following antibodies were used: vimentin, EMA, cytokeratins (KL1, CK19), S-100 protein, CD68, p53 protein, vascular markers (CD31, CD34, FVIII-related antigen), HMB45, MELAN A. RESULTS: The tumors had unusually large dimensions, the absence of necrosis, a vaguely alveolar pattern; the IHC staining showed a strong positivity of S-100 protein, a variable expression of cytokeratins KL1 and CK19, in association to a scarce positivity for EMA, zonal positivity for CD34 and diffuse reaction for vimentin. CONCLUSION: The immunophenotype of ES may have a large variability and the correlation to clinico-morphological aspects is necessary for a good diagnostic assessment.
OBJECTIVE: To investigate the expression of the chimeric genes resulting from the specific chromosomal translocations in soft tissue sarcomas (STS) and its diagnostic significance for STS. METHODS: The variety of fusion transcripts were detected in 103 cases of STS, including 30 cases of synovial sarcoma (SS), 15 cases of rhabdomyosarcoma (RMS), 25 cases of Ewing's sarcoma/peripheral primitive neuroectodermal tumors (ES/pPNET), 12 cases of dermatofibrosarcoma protuberans (DFSP), 14 cases of aveolar soft part sarcoma (ASPS), 3 cases of leiomyosarcoma (LMS), 2 cases of malignant fibrous histocytoma (MFH), and 2 cases of fibrosarcoma (FS); and 20 cases of control tumors by reverse transcription-polymerase chain reaction (RT-PCR) using formalin fixed, paraffin embedded specimens. RESULTS: Of the 34 cases of SS 28 (93.3%) expressed SSX-SYT chimeric transcripts (14 were positive for SYT-SSX1, 9 for SYT-SSX2). Four of the six cases of alveolar RMS had a PAX3/PAX7-FKHR fusion transcript. None of the 9 cases of embryonic and polymorphic RMS expressed PAX3/PAX7-FKHR. Of the 25 cases of ES/pPNET, 19 were positive for EWS-FLI1 fusion transcript and 1 for EWS-ERG fusion transcript. COL1A1-PDGFB fusion transcript was expressed in 8 of the 12 cases (66.7%) of DFSP. Of the fourteen cases of ASPS, ten expressed ASPL-TFE3 fusion transcript. None of the 3 cases of LMS, 2 cases of MFH, 2 cases of FS, and 20 control cases contained any of the fusion transcript. CONCLUSION: Chimeric gene transcript resulting from specific chromosomal translocations is a reliable index for the molecular diagnosis of STS and RT-PCR assay for detection of specific fusion gene provides a useful tool for confirmation of the diagnosis of STS in diagnostically difficult cases and in retrospective studies.