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Query: UMLS:C0392680 (shortness of breath)
 5,217 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

The clinical and pathological features of nine cases of pleuro-pulmonary endometriosis and the first case of pulmonary ectopic deciduosis are presented. The patients were all women between the ages of 27 and 74 years (median, 36 years) who presented with symptoms of catamenial pleural pain, shortness of breath, hemoptysis, or radiographically detected lung masses. Clinically, six patients were multiparous, one patient had pelvic endometriosis, and four patients had undergone prior pelvic surgical procedures, including dilatation and curettage. Radiographically, eight patients had pulmonary infiltrates or nodules, and four patients had pneumothorax. Three cases involved the visceral pleura and one case the parietal pleura. The other six cases, including the single case of ectopic deciduosis, involved the lung parenchyma. Histologically, the single or multifocal lesions were well circumscribed or infiltrative, nodular, cystic, or nodulo-cystic, and showed the characteristic features of proliferative or secretory endometrium with numerous mullerian metaplastic changes. Mucin stains were negative in five cases of endometriosis and in the single case of ectopic deciduosis. Immunohistochemical studies were performed in these same six cases using antibodies to epithelial, mesenchymal, vascular, and neuroendocrine markers. The glandular epithelium was decorated with antibodies to pan-cytokeratin, CK7, BER-EP4, ER, and PR, whereas the stromal cells showed positive staining for vimentin, actin, smooth muscle actin, desmin, ER, and PR. Follow-up information obtained in seven patients showed all women without recurrences after 1 to 20 years. The current study highlights the importance of recognizing intrathoracic endometriosis and ectopic deciduosis and properly assessing small biopsy specimens to avoid a misdiagnosis of malignancy.
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PMID:Pleuro-pulmonary endometriosis and pulmonary ectopic deciduosis: a clinicopathologic and immunohistochemical study of 10 cases with emphasis on diagnostic pitfalls. 986 38

Intrathoracic desmoid tumors of the pleura are unusual tumors that are often clinically and histologically confused with localized fibrous tumor of the pleura or benign neurogenic tumors. We studied four cases of intrathoracic desmoid tumor of the pleura and reviewed the clinical, histopathologic, and immunohistochemical features of the four patients. Two men and two women, ranging in age from 16 to 66 years (mean, 44 yr) comprised the study group. Three patients presented with chest pain and one with shortness of breath. Two patients had a history of associated trauma in the area of the tumor. Three of the lesions were based in the parietal pleura and one in the visceral pleura. Treatment included complete resection (two cases), subtotal resection (one case), and subtotal resection followed by radiation therapy and complete resection (one case). The mean tumor size was 12.5 cm, and all of the tumors exhibited a bosselated, firm, white, cut surface. The histologic features of intrathoracic desmoid tumors were similar to those of desmoid tumors at more conventional sites. Infiltration of the adjacent fat and skeletal muscle was invariably present. The tumor cells were immunoreactive for vimentin, desmin, smooth muscle actin, and muscle-specific actin in three of the four cases and were negative for S-100 protein. Follow-up to date shows stable residual disease at 12 months (one case) and two patients with no evidence of disease at 12 and 96 months, respectively. Intrathoracic desmoid tumors often exhibit clinical and radiographic features similar to localized fibrous tumor of the pleura. They generally have histologic and behavioral characteristics identical to those of desmoid tumors at conventional sites. Like desmoid tumors elsewhere, complete resection with negative margins is vital to prevent local recurrence. Desmoid tumor should be considered in the differential of localized fibrous tumor of the pleura.
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PMID:Desmoid tumors of the pleura: a clinicopathologic mimic of localized fibrous tumor. 995 Jan 56

The clinicopathologic and immunohistochemical findings in 30 cases of idiopathic fibroinflammatory lesions of the mediastinum are presented. There were 17 male and 13 female patients between 10 and 64 years of age; 19 were African-American, and 10 were Caucasian. Clinically, respiratory and/or systemic symptoms including cough, shortness of breath, and fever were present in 28 patients. Five patients also presented with evidence of superior vena cava syndrome. All of the lesions involved the anterior mediastinum with radiographic evidence of hilar and paratracheal involvement in nine and five patients, respectively. Histologically, the lesions were characterized by an inflammatory fibrosing process that showed three distinctive histologic patterns. On the basis of the histologic pattern, they were subdivided into three distinct groups (stages). Stage I demonstrated edematous fibromyxoid tissue with numerous spindle cells, eosinophils, mast cells, lymphocytes, plasma cells, and thin-walled blood vessels; Stage II showed thick glassy bands of haphazardly arranged collagen with focal interstitial spindle cells, lymphocytes, and plasma cells; and Stage III was characterized by dense acellular collagen with scattered lymphoid follicles and occasional dystrophic calcification. Immunohistochemical studies in 17 cases highlighted large numbers of vimentin- and actin-positive spindle cells and capillary-like vessels in Stage I lesions, with fewer numbers of vimentin-positive, actin-negative spindle cells and vessels in Stage II lesions. Our findings suggest that "sclerosing mediastinitis" represents the final stage of an evolving, dynamic process with different morphologic appearances akin to abnormal wound healing. Thus, we propose the term fibroinflammatory lesion of the mediastinum to convey the true nature of the process.
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PMID:Idiopathic fibroinflammatory (fibrosing/sclerosing) lesions of the mediastinum: a study of 30 cases with emphasis on morphologic heterogeneity. 1010 10

We report on a 43-year-old man with a primary sarcoma of the liver. The patient was admitted to the hospital for evaluation of dyspnea, abdominal pain in the right upper quadrant, diarrhea, and fever. Physical examination revealed hepatomegaly. Increased laboratory values were found for gamma-GT, LDH, CA 125, and NSE, but not for aspartate and alanine aminotransferase. Computed tomography presented a tumor in the right lobe of the liver. Venous cavography revealed a caval tumor thrombus reaching up to the right atrium. Major liver resection combined with replacement of the vena cava inferior was proposed, but before operation the patient complained about shortness of breath. Spontaneous fragmentation of the tumor thrombus with consecutive embolization of the lungs was suspected. Despite lysis therapy the patient died because of right ventricular failure. Autopsy revealed a tumor measuring 8 cm in diameter, which was located in the right lobe of the liver and invaded the inferior vena cava. Because of multiple tumor aggregates seen in the left and right main pulmonary arteries acute tumor embolization of the lungs was regarded as cause of death. Histologically the tumor was composed of bizarre giant cells surrounded by irregular spindle cells. The positive immunoreactivity pattern of the tumor cells for vimentin, lysozym, and CD68 justified the diagnosis of a malignant fibrous histocytoma (MFH) of the liver.
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PMID:[Malignant fibrous histiocytoma of the liver]. 1076 47

A series of 15 cases of primary mediastinal neoplasms displaying histopathologic, immunohistochemical, and ultrastructural features of synovial sarcoma is presented. The patients' ages ranged from 3 to 83 years, with a male-to-female ratio of 2:1. Nine cases presented as anterior mediastinal masses with chest pain, shortness of breath, and pleural effusion, and 6 cases were in paravertebral location in the posterior mediastinum and presented with neck or back pain and pleural effusion. The tumors measured from 5 to 20 cm in greatest diameter and showed a tan white, soft to rubbery cut surface with areas of hemorrhage and necrosis and foci of gelatinous material. Four cases showed areas of cystic degeneration. In 7 cases, the tumors were well circumscribed; in 6 cases, the tumors grossly invaded the pleura, pericardium, heart, great vessels, chest wall, rib, and vertebra. Histologically, 5 cases displayed a biphasic growth pattern, with well-formed glandular elements admixed with a monotonous spindle cell population. Ten cases were exclusively composed of a monotonous atypical spindle cell proliferation. Immunohistochemical studies showed focal positivity of the tumor cells for cytokeratin and/or epithelial membrane antigen, and strong positivity for vimentin and bcl-2 in the spindle cells in all cases studied (10 of 10). Eight cases also showed focal positivity for CD99. Electron microscopic examination in 5 cases showed oval to spindle tumor cells with closely apposed cell membranes, abundant cytoplasmic intermediate filaments and rough endoplasmic reticulum, and immature desmosome-type cell junctions. Ten patients were treated by complete surgical excision and two by partial excision followed by radiation therapy. In 4 patients, the tumors were inoperable and treated with radiation therapy only. Clinical follow-up was available in 5 patients and showed local recurrence with metastases to lung, lymph nodes, and epidural space from 1 to 3 years in 4 cases and liver metastases and death due to tumor after 6 month in 1 case. Synovial sarcoma should be considered in the differential diagnosis of biphasic and monophasic spindle cell neoplasms of the mediastinum.
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PMID:Primary synovial sarcomas of the mediastinum: a clinicopathologic, immunohistochemical, and ultrastructural study of 15 cases. 1583 79

A 69-year-old man presented with cough, shortness of breath, and fatigue. He was initially treated for allergies and then for pulmonary embolism. Radiologically, a tumor mass was found to occlude the right pulmonary artery and involve the pulmonary trunk. A right pneumonectomy was performed. Histologically, a cellular malignant spindle and epithelioid tumor with areas of necrosis and brisk mitotic activity was seen. In some areas, the tumor appeared to form vascular channels. Focal osteosarcomatous differentiation was present. Immunohistochemical studies were performed including vimentin, smooth muscle actin, desmin, CD31, CD34, S100, and pan-cytokeratin. The tumor cells were positive for CD31 and vimentin and negative for pan-cytokeratin, CD34, and S100. Two months after surgery, the patient was alive and well.
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PMID:Pulmonary artery angiosarcoma: a clinicopathologic and radiological correlation. 1608 54

A case of pleomorphic malignant fibrous histiocytoma of the right atrium and right ventricle is presented. Primary cardiac malignant fibrous histiocytoma (MFH) is extremely rare and its etiology and clinical course are not precisely known. We treated a 47-year-old female patient with persistent fever and cough, shortness of breath and anasarca. The patient died after eight months with symptoms of cardiac shock. Autopsy revealed a big soft yellow tumor in the right heart. Microscopically, atypical fibroblast-like cells, pleomorphic and multinucleated giant tumor cells with eosinophilic cytoplasm were observed within the myxoid and fibrous stroma. Immunohistochemically, tumor cells were positive for alfa-1-antitrypsin and partially positive for CD68 and vimentin. According to histopathologic and immunohistochemical features we diagnosed pleomorphic MFH.
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PMID:[Primary cardiac malignant fibrous histiocytoma in 47-year-old woman]. 1623 31

Primary extrarenal rhabdoid tumors (RT) are now recognized as a specific entity in pediatric oncological pathology practice. We present an unusual case of a small cell myxoid variant of a thoracic RT in an infant and highlight the importance of recent molecular developments in the diagnosis of these tumors. An 8-month-old child presented with a short history of cough and shortness of breath. Imaging demonstrated a large mass occupying the majority of the thoracic cavity on the right side. A percutaneous needle biopsy of the mass showed fragments of tissue composed of malignant tumor with a predominant "small ovoid cell" phenotype and extensive myxoid change, with small nests and islands of tumor cells; occasional cells demonstrated open vesicular nuclei, prominent nucleoli, and eosinophilic cytoplasmic inclusions. Immunohistochemical staining revealed focal strong cytoplasmic positivity for cytokeratin, focal strong paranuclear cytoplasmic vimentin positivity, and INI1 staining showed normal nuclear positivity in control tissues but was negative in tumor cell nuclei. Electron microscopy demonstrated characteristic paranuclear whorls of intermediate filaments confirming the diagnosis of extrarenal malignant RT. The diagnosis of malignant rhabdoid tumor may be difficult, particularly in cases, such as the present, with a predominant small-cell myxoid phenotype. The characteristic expression patterns of cytokeratin and vimentin provide strong clues to the diagnosis, and the use of INI1 antibody now makes definitive diagnosis possible even on needle core biopsies.
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PMID:Primary thoracic myxoid variant of extrarenal rhabdoid tumor in childhood. 1706 Jan 92

Minute pulmonary meningothelial nodules are rare lesions histologically composed of small nests of epithelioid cells located within the interstitium of the lung. These nodules are generally asymptomatic and are usually found incidentally at autopsy or in surgical specimens resected for unrelated causes. The lesions are most often single, although multiple lesions with unilateral involvement of one or even all lobes of the same lung have been described. To our knowledge, cases of meningothelial nodules with disseminated bilateral pulmonary involvement associated with clinical symptoms of restrictive pulmonary disease and radiologic evidence of diffuse reticulonodular pulmonary infiltrates have not been previously documented. We have studied 5 patients presenting with diffuse bilateral pulmonary involvement by numerous minute pulmonary meningothelial nodules. The patients were 4 women and a man aged 54 to 75 years who presented clinically with dyspnea and shortness of breath and the lesions were discovered on open lung biopsies performed for the evaluation of diffuse bilateral interstitial lung infiltrates found on chest x-rays and computed tomography scans. In 3 patients, there was a previous history of malignancy and the radiologic findings were suspected of representing diffuse metastatic disease. Histologically, the lesions were composed of small clusters of epithelioid cells with round to oval nuclei devoid of atypia and surrounded by abundant eosinophilic cytoplasm. Immunohistochemical studies showed positivity of the tumor cells for epithelial membrane antigen and vimentin, and negative staining for cytokeratin, actin, S-100 protein, CD34, chromogranin, and synaptophysin. Electron microscopic examination in 1 case confirmed the ultrastructural features of meningothelial cells, including complex cytoplasmic interdigitations joined by well-developed desmosomes and abundant intracytoplasmic intermediate filaments. The diffuse bilateral involvement of lung parenchyma in the present cases can lead to confusion on clinical and radiologic grounds with a variety of interstitial pulmonary processes, including idiopathic interstitial pneumonia and lymphangitis carcinomatosa. Diffuse pulmonary meningotheliomatosis should be considered in the clinical differential diagnosis of diffuse interstitial pulmonary infiltrates.
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PMID:Diffuse pulmonary meningotheliomatosis. 1741 11


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