Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019079 (hemoptysis)
 6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Synovial sarcoma arises most commonly in the lower extremity, particularly in the region of the lower thigh and knee. Yet the occurrence of thus mesenchymal neoplasm in the head and neck area has been convincingly documented, albeit confined almost exclusively to cervical and parapharyngeal sites. Therefore, in view of its rarity in the head and neck, we analyzed a group of 11 synovial sarcomas arising in the orofacial region. The series comprised nine men (82%) and two women (18%). In common with synovial sarcoma at more conventional sites, this is a disease of young adults: ages ranged from 16-49 years (median, 34 years). Topographically, two subsets were delineated, a more common facial group with eight cases (four cheek, two parotid region, one infraorbital, one submental), all arising as gradually enlarging, usually nontender, solitary tumors; and three intraoral ones (two tonsillar, one lingual), two of which were polypoid and one was an exophytic tonsillar mass which presented with hemoptysis and stridor. Follow-up data, obtained for nine patients (range, 1.3-15.0 years), disclosed three (33%) tumor-related deaths, all belonging to the facial group. Treatment, difficult to significantly correlate with survival in this small series, varied from surgical excision alone to a multimodality approach including both irradiation and chemotherapy. Histologically, all the neoplasms revealed characteristic biphasic features, predominantly fibrosarcomatous in one, but, more typically, showing epithelial clefts and/or pseudoacinar spaces in the others. Differential diagnosis, depending on the proportion of the biphasic components, ranged from spindle cell mesenchymal neoplasms to various adenocarcinomas, including those arising in mixed tumors of salivary gland.
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PMID:Orofacial synovial sarcoma: a clinicopathologic study of 11 new cases and review of the literature. 628 38

We present 25 cases of a primary pulmonary sarcoma bearing histological, immunohistochemical, and ultrastructural features indistinguishable from those of monophasic synovial sarcoma of soft tissue. The patients were 11 men and 14 women between the ages of 16 and 77 years. Clinically, the most common symptoms were chest pain, cough, shortness of breath, and hemoptysis. The lesions involved all lung segments. Grossly, they varied in size from 0.6 to 20 cm and were described as soft to rubbery tumors with areas of necrosis and hemorrhage, some with cystic changes. Two lesions involved the bronchial wall and in one case the tumor was described as encircling the bronchial tree. Histologically, all of the lesions were characterized by an atypical spindle cell proliferation with a solid growth pattern. Areas of myxoid, neural, hemangiopericytic, and epithelial-like growth pattern were observed. Mitoses, necrosis, and hemorrhage were seen in all lesions in varying proportions. Immunohistochemical studies for epithelial membrane antigen (EMA) and keratin showed strong focal positivity in 25 of 25 and 23 of 25 lesions, respectively. Immunohistochemical study for vimentin showed diffuse strong positivity in all lesions. Other immunostains, including desmin, smooth muscle actin, and S-100 protein, were negative. Electron microscopy in three cases showed spindle cells with elongated nuclei containing abundant cytoplasmic rough endoplasmic reticulum and well developed desmosome type intercellular junctions. Follow-up information ranging from 2 to 20 years was obtained in 18 patients. Six patients died of their tumors, whereas four patients died of unrelated causes without evidence of recurrence or metastases. Eight patients were alive with disease (recurrence and/or metastases) from 1 to 7 years after diagnosis. Four patients were alive and well without evidence of recurrence or metastases from 2 to 20 years (mean follow-up, 12.5 years). The present group of lesions appears to constitute a distinctive and as yet previously undescribed primary sarcoma of the lung, which probably represents the visceral counterpart of monophasic synovial sarcoma of soft tissue in a pulmonary location. Because of their distinctive biology these lesions should be distinguished from a variety of primary and metastatic malignancies of the lung.
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PMID:Primary pulmonary sarcomas with features of monophasic synovial sarcoma: a clinicopathological, immunohistochemical, and ultrastructural study of 25 cases. 775 Sep 31

We present a 26 year old patient with a primary malignant synovial sarcoma of the lung that was observed for more than one year by a general practitioner and a pulmologist. Finally, because of recurrent hemoptysis a central tumor of mesenchymal origin of the left lower lobe was diagnosed by bronchoscopy. The invasion of the left atrium as far as to the mitral valve was diagnosed by MRT. The patient was operated on by extended pneumonectomy with extracorporeal circulation. The partial excision of the left atrium required plastic reconstruction. In the postoperative course the patient underwent chemo-therapy, 6 cycles adriablastine/ifosfamid. 8 months after the first operation an extensive tumor recurrency occurred with infiltration of the chest wall. The patient refused further radio- or chemotherapy and died 14 months after the operation. Because of the small number of cases therapeutic strategy conceptions do not exist. The resection of the tumor is generally recommended. Chemo- and radiotherapy are accepted as an option for advanced tumor stage.
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PMID:[Primary synovial sarcoma of the lung - a rare tumor]. 1220 Jul 37

Synovial sarcoma is an extremely rare primary pulmonary tumor whose description is based on a limited number of cases. We report two cases diagnosed by thoracotomy. One patient was initially treated surgically, and a later recurrence was controlled by combined chemotherapy and a second operation after three years of monitoring. In the second case, surgery was imperative to treat massive hemoptysis and was followed by combined chemotherapy after diagnosis of lesions consistent with extrapulmonary metastasis.
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PMID:[Primary pulmonary synovial sarcoma: a report and diagnosis of 2 cases]. 1262 74

A 30-year-old female presented with hemoptysis, chest pain, and a rapidly enlarging pleural-based mass, and was found to have primary synovial sarcoma of the lung. Primary pulmonary sarcomas comprise <1% of all primary lung malignancies. They present clinically in young adults with cough, chest pain, shortness of breath, or hemoptysis, with a mass on x-ray and computerized tomography scan. Diagnosis is made by histology and immunohistochemistry. Histologic diagnosis has recently been supplemented by cytogenetic analysis, which offers important prognostic information. The mainstay of treatment remains complete surgical excision. Prognosis is poor, with an overall 5-year survival rate of 50%.
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PMID:Primary pulmonary synovial sarcoma: a case report and review of current diagnostic and therapeutic standards. 1516 89

Endobronchial metastases (EBM) from extrapulmonary malignant tumors are rare. The most common extrathoracic malignancies associated with EBM are breast, renal and colorectal carcinomas. In this study, we aimed to evaluate the clinical, radiographic and bronchoscopic aspects of patients with EBM who were diagnosed between 1992 and 2002. Data about patients' clinical conditions, symptoms, radiographic and endoscopic findings, and histopathological examination results were investigated. EBM was defined as bronchoscopically visible lesions histopathologically identical to the primary tumor in patients with extrapulmonary malignancies. We found 15 cases with EBM. Primary tumors included breast (3), colorectal (3), and renal (2) carcinomas; Malignant Melanoma (2); synovial sarcoma (1), ampulla of Vater adenocarcinoma (1), pheochromocytoma (1), hypernephroma (1), and Hodgkin's Disease (1). The most common symptoms were dyspnea (80%), cough (66.6%) and hemoptysis (33.3%). Multiple (40%) or single (13.3%) pulmonary nodules, mediastinal or hilar lymphadenopathy (40%), and effusion (40%) were the most common radiographic findings. The mean interval from initial diagnosis to diagnosis of EBM was 32.8 months (range, 0-96 months) and median survival time was 18 months (range, 4-84). As a conclusion, various extrapulmonary tumors can metastasize to the bronchus. Symptoms and radiographic findings are similar with those in primary lung cancer. Therefore, EBM should be discriminated from primary lung cancer histopathologically. Although mean survival time is usually short, long-term survivors were reported. Consequently, treatment must be planned according to the histology of the primary tumor, evidence of metastasis to other sites and medical status of the patient.
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PMID:Endobronchial metastases from extrathoracic malignancies. 1647 29

This report describes the clinical, histological and immunohistochemical features of two patients with primary pulmonary synovial sarcoma in the context of the literature. Chest pain, cough, haemoptysis and an enlarging pleural-based mass are the main clinical manifestations. Diagnosis depends on identifying epithelioid or spindle cells microscopically and on immunohistochemistry showing positivity for cytokeratin and vimentin and epithelial membrane antigen stains. Surgical excision is the main treatment approach.
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PMID:Primary pulmonary synovial sarcoma, a rare primary lung neoplasm: two case reports and review of the current literature. 1871 96

A 48-year old man presented with chest pain and haemoptysis. Chest computed tomography showed a 60-mm mass in the left upper lobe of the lung, adjacent to the distal aortic arch. Bronchoscopic cytology revealed the presence of malignant cells and, in the absence of evidence of distant metastasis, a thoracotomy was performed. Although the tumour was firmly adherent to the distal aortic arch, under temporary bypass from the left subclavian artery to the descending aorta, it was successfully resected en bloc with the section of the aorta attached to it. The tumour was diagnosed as a primary synovial sarcoma of the lung on the basis of histopathological findings and fluorescent chromogenic in situ hybridization, showing SS18 gene rearrangement.
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PMID:Primary synovial sarcoma of the lung successfully resected under temporary bypass. 2376 Feb 20

Primary pulmonary synovial sarcoma (PPSS) is a rare malignancy. Its etiology, imaging features and optimal treatment are not well understood. Pulmonary pseudoaneurysms and lymphadenopathy are rare complications of synovial sarcomas. A 40-year-old woman with mild hemoptysis and thoracic back pain underwent a computed tomography scan that revealed multiple pulmonary lesions, paraesophageal lymphadenopathy and incidental bilateral pulmonary emboli. A diagnosis of PPSS was made through the identification of an SS18 translocation by fluorescence in situ hybridization. She was started on adriamycin, ifosfamide and mesna chemotherapy. Over the subsequent two months, she developed three pulmonary artery pseudoaneurysms, ultimately requiring endovascular coiling. Seven months after starting treatment, the patient was asymptomatic. The lesions and lymphadenopathy decreased in size. The present case highlights complications of a rare malignancy and demonstrates positive response to ifosfamide-based chemotherapy in the setting of PPSS.
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PMID:Primary pulmonary synovial sarcoma: a case report with unique and impressive computed tomography findings. 2566 59

Fewer than 40 cases of primary pleural synovial sarcoma have been reported so far with only 3 cases of cystic synovial sarcoma including cases originating from sites other than the pleura. Here, we present an exceedingly rare case of cystic synovial sarcoma originating from the mediastinal side of the visceral pleura in a 25-year man presenting with hemoptysis. On contrast-enhanced computed tomography (CT), cystic synovial sarcoma and cystic thymoma were difficult to be distinguished due to mediastinal location. Histopathological examination showed spindled morphology of tumor cells with hypercellularity and nuclear atypia. As these features are associated with both monophasic fibrous synovial sarcoma and type Athymoma, immunohistochemistry was performed. Adiagnosis of synovial sarcoma was confirmed by detection of CD99 and EMAand negativity of other markers. Fluorescence in situhybridization (FISH) was not done. Surgical excision was done and followed by oncology referral.
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PMID:Cystic Pleural Synovial Sarcoma. 2866 5


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