Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a unique case of a combined pulmonary large-cell neuroendocrine carcinoma and spindle-cell carcinoma. The patient was a 54-year-old female smoker who presented with a 4-month history of increased left-sided chest pain and exertional dyspnea. The left upper lobectomy specimen revealed an 8.0-cm mass with central necrosis. Microscopically, the epithelial areas were composed of well-defined nests of large cells with peripheral palisading expressing low-molecular-weight keratin, synaptophysin, chromogranin, and neuron-specific enolase. The spindle-cell component consisted of pleomorphic cells arranged in fibrosarcoma and malignant fibrous histiocytoma-like patterns. These spindle cells were positive for low-molecular-weight keratin and vimentin with focal expression of CD68 and muscle-specific actin. Electron microscopy in the spindle-cell areas showed cell junctions and numerous tonofilaments, indicative of epithelial differentiation. The tumor behaved aggressively and the patient died with extensive metastases 4 months after surgery. The combination of neuroendocrine malignancies and spindle-cell carcinomas appears to be uncommon in the lung. Previous reports have described this association in single case reports of anaplastic small-cell carcinoma and atypical carcinoid, but not in large-cell neuroendocrine carcinoma.
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PMID:Combined large cell neuroendocrine carcinoma and spindle cell carcinoma of the lung. 1151 7

Primary plasmacytoma of the lung is a rare tumor, thus presenting a diagnostic challenge to the clinician. So far, approximately 20 cases have been verified by immunohistochemistry. We describe an elderly patient presenting with progressive dyspnea on exertion, dry cough, weight loss and malaise. The main finding on plain chest radiography was a diffuse infiltration of pulmonary parenchyma in the lower parts of both lungs and in the middle part of the right lung. The histology of the open lung biopsy of the right middle lobe revealed massive and diffuse infiltration by well differentiated plasma cells with extracellular deposits of amyloid. The plasma cells and amyloid expressed a monoclonal lambda light chain. No monoclonal spike was shown by serum and urine immunoelectrophoresis. A skeletal survey and bone marrow biopsy specimen excluded a disseminated disease and a diagnosis of extramedullary plasmacytoma was made. The patient was considered for VI courses of VMCP chemotherapy after which a complete regression on chest roentgenography was evident. Almost five years after the diagnosis the patient is still alive without any evidence of disease recurrence or dissemination.
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PMID:Diffuse primary plasmacytoma of the lung. 1158 28

Primary leiomyosarcomas of the heart, particularly those affecting the right ventricle, are uncommon. We report the case of a 70-year-old Belgian woman presenting with the symptoms of progressive exertional dyspnea and left-sided pleuritic pain. A leiomyosarcoma which originated from the right lateral ventricle wall, causing pulmonary outflow obstruction, was diagnosed. Pathology revealed a neoplasm with a myxoid stroma, high mitotic activity and nuclei expressing atypia. Immunohistochemical staining was positive for vimentine and desmin. Seven months after complete surgical resection the tumor relapsed. This case demonstrates the poor outcome, the high relapse rate and inefficiency of treatment associated with primary cardiac leiomyosarcomas. The current literature regarding the incidence, diagnostic techniques, treatment strategies and survival rates of this rare but terminal disease is reviewed.
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PMID:Ventricular outflow tract obstruction secondary to leiomyosarcoma of the right ventricle. 1160 76

A metastatic synovial sarcoma of the left ventricle is described. A 26-year-old Japanese woman underwent resection of a synovial sarcoma of the right thigh 8 years prior to admission, which was followed by multiple surgical procedures for pulmonary metastasectomy. Echocardiography demonstrated obstruction of the outflow tract of the left ventricle caused by a metastatic tumor, which was surgically resected on the day of admission. The histologic characteristics of the cardiac tumor were identical to those of the pulmonary metastases. Additional cardiac metastasectomy was performed 9 months later. She complained of dyspnea on exertion 8 months after the second cardiac metastasectomy due to recurrence in the heart. Radiotherapy with LINAC (a total dose: 50 Gy) diminished the size of the tumor temporarily. The patient died 20 months after the initial cardiac metastasectomy.
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PMID:Metastatic synovial sarcoma of the left ventricle. 1160 77

We report a case of a 66-year-old female who presented exertional dyspnea and palpitation. Echocardiography, transesophageal echocardiography and computed tomography showed a right atrial cystic mass attached to the interatrial septum. The patient underwent successful excision of the mass. The histopathological findings confirmed the lesion as a cystic tumor of the atrioventricular nodal region. This is the third known case of this condition diagnosed antemortem and treated successfully with surgical excision.
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PMID:Cystic tumor of the atrioventricular nodal region. 1185 98

Angiomyolipoma is a common tumor of the kidney but has rarely been found in the mediastinum. We report a case of angiomyolipoma of the posterior mediastinum in a 62-year-old woman. She experienced exertional dyspnea and intermittent cough at admission. Computed tomography indicated a tumor located at the left paravertebral and upper posterior mediastinum and MRI imaging demonstrated a mass with low signal intensity in T1-weighted image at T4-5 level. Thoracotomy was done for surgical removal of the tumor and histologic examination revealed a mesenchymal tumor composed of mature fat, capillaries and smooth muscle fibers. The tumor was immunohistochemically positive for CD34 and factor-VIII (for vascular component) smooth muscle actin (for smooth muscle component) and S-100 protein (for fat component). There have been four case reports about mediastinal angiomyolipoma, namely three Japanese cases and one French case. It is suggested that angiomyolipoma could be considered for the differential diagnosis of mediastinal tumors.
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PMID:A case of mediastinal angiomyolipoma. 1185 61

This report describes the case of a 16-year-old boy who underwent surgical treatment of a cardiac malignant fibrous histiocytoma (MFH). He was admitted to our hospital for exertional dyspnea. An ultracardiogram (UCG) revealed a tumor about 10cm in diameter, in the left atrium. Extirpation of the left atrial tumor, including the part extending into the pulmonary veins, was performed under cardiopulmonary bypass. Histological examination confirmed a diagnosis of MFH. No adjuvant chemotherapy or radiotherapy was given. While there has been no evidence of local recurrence or metastasis in the 9 months since his operation, strict follow-up is being done by UCG and computed tomography. A few reviews of this entity have been reported; however, they have confused autopsy cases with surgical cases. Therefore, we reviewed only the surgical cases of this type of cardiac tumor documented in the literature. According to our review of the literature, this patient is the youngest among the 42 cases of surgically treated cardiac MFH reported to date.
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PMID:Surgical treatment of malignant fibrous histiocytoma in the left atrium and pulmonary veins: report of a case. 1199 16

Primary mediastinal liposarcomas are extremely rare malignancies that remain asymptomatic until large and, even then, initial symptoms are nonspecific. We report a 48-year-old man followed up for asymptomatic multiple bullae who suffered progressive weight loss and dyspnea on exertion. Radiography and computed tomography of the chest showed a large mass with calcified nodules in the left pleural cavity and giant bullae in the right pleural cavity. Previous computed tomography of the chest showed a small tumor of mediastinal adipose tissue with calcified nodules. Tumor growth was calculated at about 500 times the tumor volume per 3.6 years. We completely resected the mediastinal tumor and conducted a bullectomy through a median sternotomy. The microscopic pathological diagnosis was well-differentiated/sclerosing liposarcoma. The man underwent no postoperative adjunctive irradiation and remains well 8 months after surgery.
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PMID:Primary liposarcoma of the mediastinum. 1207 3

A 36-year-old female was admitted with dyspnea on exertion of one year's duration. Echocardiography revealed a tumor arising from interventricular septum with dynamic left ventricular outflow tract obstruction. On cardiopulmonary bypass with cardioplegic arrest, the tumor was approached through a transverse aortotomy, and excised from the interventricular septum through the aortic valve. Postoperative recovery was uneventful and the patient was asymptomatic with no recurrence at follow-up after one year.
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PMID:Lipoma of the left ventricle. 1207 76

We encountered a 75-year-old man who complained of exertional dyspnea. An echocardiographic examination showed aortic regurgitation and a tumor in the left ventricular outflow tract. Under complete extracorporeal circulation, we surgically made an incision of the ascending aorta with a slight thickening of the aortic valve and an enlarged annulus. After excising the aortic valve, an examination of the subvalvular region revealed mitral valve-like tissue extending from the annular region of the right coronary cusp to the ventricular septum, while the chordae tendinae was attached to the septum. This issue was excised, and the aortic valve was replaced with a 27-mm SJM valve. The postoperative course was uneventful, and the patient was discharged in good condition on postoperative day 30. An accessory mitral valve is extremely rare. Since this indication for surgical treatment is associated with congenital heart disease or a left ventricular outflow tract obstruction, most patients are young. Our patient had no associated cardiac anomalies and no pressure gradient attributable to a left ventricular outflow tract obstruction. This accessory mitral valve was discovered during aortic valve replacement surgery. To our knowledge, our patient is the oldest reported with an accessory mitral valve to have undergone a surgical resection.
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PMID:Accessory mitral valve associated with aortic regurgitation in an elderly patient: report of a case. 1210 77


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