Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0010200 (cough)
23,843 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 47-year-old woman was admitted to our hospital for cough and dyspnea. Roentgenologic studies and bronchoscopy revealed multiple lung tumors one of which obstructed the right main bronchus. Right pneumonectomy was performed for the pending obstruction of the trachea. The tumor in the right S1 was found to be protruding into the trachea through the right B1 and the main bronchus in a polypoid fashion. The pathological diagnosis of synovial sarcoma was made on the basis of the characteristic biphasic structure composed of spindle cells and epithelioid cells forming gland-like spaces. Three years and eight months after the pneumonectomy, a nodule in the tendon of the extensor hallucis longus muscle became palpable. It was also a synovial sarcoma pathologically. Synovial sarcoma is a soft tissue sarcoma which usually arises in the extremities. It is very rare for pulmonary metastasis of this tumor to be found while the primary tumor is undetectable.
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PMID:[A case report of multiple pulmonary tumors as a sole manifestation of synovial sarcoma]. 132 Jul 7

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

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

Pleuropulmonary synovial sarcoma (PPSS) is increasingly recognized as a subtype of sarcoma because of the recent identification of a distinctive chromosomal translocation specific to synovial sarcoma. Soft-tissue synovial sarcoma is far more common than PPSS and typically develops in para-articular locations of the extremities; affects young and middle-aged adults, with no difference in distribution between the sexes; and has well-documented radiologic manifestations. PPSS may arise in the chest wall, heart, mediastinum, pleura, or lung, and it shares patient demographics and several imaging features with its soft-tissue counterpart. Patients present with a cough, chest pain, or dyspnea. On chest radiographs, PPSS typically appears as a sharply marginated mass with uniform opacity, based either in the pleura or in the lung, and often accompanied by an ipsilateral pleural effusion. Computed tomographic images show a well-circumscribed heterogeneously enhanced lesion without associated involvement of bone and without calcifications (except in the case of a chest wall primary tumor). Magnetic resonance imaging provides superior demonstration of nodular soft tissue and multilocular fluid-filled internal components of PPSS, in addition to peripheral rim enhancement after the intravenous administration of a gadolinium-based contrast material such as gadopentetate dimeglumine. Current treatment consists of surgical resection followed by chemotherapy, radiation therapy, or both.
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PMID:From the archives of the AFIP: Pleuropulmonary synovial sarcoma. 1670 63

A 29-year-old female was admitted to our hospital complaining of severe dry cough and low-grade fever. A chest roentgenogram and a chest computed tomographic scan revealed a huge tumor and massive pleural effusion in the left pleural cavity. Pathological examination revealed spindle shaped malignant cells. SYT-SSX fusion gene transcripts were detected, so we diagnosed the tumor as a synovial sarcoma in the pleural cavity. After neoadjuvant chemotherapy we resected the tumor completely. A synovial sarcoma in the pleural cavity is extremely rare. To the best of our knowledge, this is the thirteenth case, and the first case to undergo neoadjuvant chemotherapy and complete resection. Because a synovial sarcoma in the pleural cavity has a poor prognosis and is characterized by a high incidence of recurrence, we must carefully follow up this patient.
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PMID:Resected case of synovial sarcoma in the pleural cavity. 1681 11

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

Pleuropulmonary synovial sarcoma (PPSS) is increasingly recognized as a subtype of sarcoma because of the recent identification of a distinctive chromosomal translocation specific to synovial sarcoma. Soft-tissue synovial sarcoma is far more common than PPSS and typically develops in para-articular locations of the extremities, affects young and middle-aged adults, with no difference in distribution between the sexes, and has well-documented radiologic manifestations. Pleuropulmonary synovial sarcoma can arise in the chest wall, heart, mediastinum, pleura, or lung, and it shares patient demographics and several imaging features with its soft-tissue counterpart. Patients present with a cough, chest pain, or dyspnea. On chest radiographs, PPSS typically appears as a sharply marginated mass with uniform opacity, based in the pleura or in the lung, and often accompanied by an ipsilateral pleural effusion. Computed tomographic images show a well-circumscribed, heterogeneously enhanced lesion without associated involvement of bone and without calcifications (except in the case of a chest wall primary tumor). Magnetic resonance imaging provides superior demonstration of nodular soft tissue and multilocular fluid-filled internal components of PPSS, in addition to peripheral rim enhancement after the intravenous administration of a gadoliniumbased contrast material such as gadopentetate dimeglumine. Current treatment consists of surgical resection followed by chemotherapy, radiation therapy, or both.
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PMID:Primary pleuropulmonary synovial sarcoma. 1882 48

Pleural synovial sarcomas are an extremely rare disease entity, and only 21 surgical cases, including this one, have been reported. We present a case of synovial sarcoma that underwent pneumonectomy for a recurrence of the disease. A 31-year-old female, who in June 2005 had undergone surgical resection of a monophasic synovial sarcoma in the left pleural cavity combined with the affected left diaphragm, was admitted to our hospital in January 2007 and underwent chemotherapy with ifosfamide, pirarubicin, and methotrexate for a recurrent synovial sarcoma in the left pleural cavity. The chest computed tomography revealed multiple tumors in the left pleura, and the tumors increased rapidly to occupy the left hemithorax. She began to complain of a severe dry cough. No other recurrent sites were found, and she underwent tumor resection combined with the entire left lung in March 2007. The tumor remained around the descending aorta. Pathological examination revealed dense proliferation of atypical spindle cells, the same findings as in the initial operation, and a diagnosis of synovial sarcoma was made. External radiation therapy up to 60 Gray was given to the tumor remaining around the descending aorta. She is now ambulatory without evidence of recurrent disease at 6 months after the second operation and 27 months after the initial surgery. Any aggressive repeat surgery as in the case here has not been previously reported. The present case suggests that aggressive tumor reduction followed by radiotherapy can achieve a good result for controlling local disease. Although optimal strategies for synovial sarcoma have yet to be established, aggressive repeat surgery could be considered in selected cases even when response to chemotherapy is insufficient and complete resection is difficult.
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PMID:Aggressive repeat surgery for a recurrent synovial sarcoma in the pleura. 1926 50

Primary sarcomas of lung are rare compared to metastatic sarcomas. Herein, we report a rare case of primary pulmonary synovial sarcoma with polypoid endobronchial growth in a 35-year-old lady who presented with cough and dyspnea. A malignant pulmonary tumor was suspected and left pneumonectomy was performed. Grossly, a non-encapsulated polypoidal endobronchial tumor measuring 6 cm in greatest diameter, with a solid, tan-white cut surface was identified. Microscopically, tumor was characterized by a proliferation of oval to spindle-shaped cells arranged in sheets and fascicles. Focal hemangiopericytomatous pattern was noted. Immunohistochemically, tumor cells were positive for vimentin, BCL-2, MIC-2 and calponin and focally positive for pancytokeratin and epithelial membrane antigen. A subsequent molecular analysis performed using reverse transcriptase-polymerase chain reaction with RNA extracted from paraffin-embedded tissue, revealed SYT/SSX1 fusion gene which confirmed the diagnosis of synovial sarcoma. The utility of immunohistochemistry and molecular techniques in diagnosis of such a rare case is stressed and the relevant literature is discussed.
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PMID:Primary endobronchial synovial sarcoma confirmed by SYT-SSX1 fusion gene transcript by reverse transcriptase polymerase chain reaction. 1980 61


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