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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pleuropulmonary blastoma (PPB) is a rare and aggressive malignant tumor of the lung. Approximately 80 cases of PPB have been published, and in only three cases high-dose chemotherapy with autologous hematopoietic stem cell transplantation (HSCT) was applied. A 5-year-old girl presenting with cough, fever, and shortness of breath was referred to the authors in March 1999. A computed tomography scan of the chest showed a tumor mass in the left hemithorax. The lesion was biopsied and the histopathologic report suggested the diagnosis of PPB. The patient received chemotherapy comprising vincristine, actinomycin D, and cyclophosphamide with only a minor response, and treatment was switched to ifosfamide, carboplatin, and etoposide, which produced a partial response. Tumor resection was performed, but margins were positive for PPB. Due to the high risk of recurrence, the authors elected to administrate high-dose chemotherapy using melphalan, etoposide, and carboplatin, followed by autologous HSCT. The patient achieved complete hematologic recovery, and reimaging after HSCT showed no evidence of disease. She relapsed 4 months later and died about 9 months after the completion of high-dose therapy. The role of high-dose chemotherapy and autologous HSCT is likely to be limited in PPB.
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PMID:High-dose chemotherapy and autologous peripheral blood stem cell rescue in a patient with pleuropulmonary blastoma. 1254 79

We report a case of a large, B-cell lymphoma of the atria in a 65-year-old man who presented with obstructive right-heart failure, shortness of breath, cirrhosis, and ascites. A computed tomographic scan revealed a large cardiac tumor occupying both atria. The patient underwent debulking of the tumor and postoperative chemotherapy. Six months postoperatively he was alive and his symptoms of obstructive right-heart failure had improved; however, he had developed brain metastasis.
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PMID:Large B-cell lymphoma of the atria. 1263 78

The majority of patients with non-small cell lung cancer (NSCLC) present with advanced disease, which is associated with a poor prognosis and symptoms such as pain, coughing, and shortness of breath. In patients who present at an earlier stage, the progressive nature of NSCLC and its resistance to treatment often result in recurrence, with the associated symptoms of advanced disease. These symptoms negatively affect patient quality of life and performance status rating, both of which are predictive of treatment response and survival. There is increasing interest in using assessments of improvements in symptoms and quality of life as outcomes in clinical trials for patients with advanced NSCLC. Patients with NSCLC have limited therapeutic options. Even those patients who are able to tolerate chemotherapy can expect median survival increases of only 2 to 4 months. The new targeted therapies for lung cancer, in contrast, are relatively nontoxic and may provide benefits for symptoms and quality of life in addition to tumor responses. The Functional Assessment of Cancer Therapy-Lung (FACT-L) scale is a validated, sensitive, and reliable patient questionnaire that evaluates and quantifies quality of life across several dimensions, including lung cancer-related symptoms (Lung Cancer Subscale). The Lung Cancer Subscale ranges from 0 (severe debilitation) to 28 (asymptomatic). A change of two points reflects a clinically significant change in NSCLC-related symptoms and quality of life. In phase I studies and also in the Iressa Dose Evaluation in Advanced Lung Cancer (IDEAL)-1 and IDEAL-2 phase II monotherapy trials, treatment of patients with advanced NSCLC with the epidermal growth factor receptor-tyrosine kinase inhibitor ZD1839 (Iressa; AstraZeneca Pharmaceuticals LP, Wilmington, DE) has shown tumor responses as well as rapid improvements in NSCLC-related symptoms and quality of life. In IDEAL-1 and IDEAL-2, improvements in NSCLC-related symptoms and quality of life, as measured by FACT-L, correlated with tumor response, and improvements in symptoms also correlated with progression-free and overall survival. Although symptom response is correlated with tumor response, it is also uniquely predictive of progression-free and overall survival. The FACT-L questionnaire has also been included in phase III trials of ZD1839 treatment in combination with chemotherapy regimens.
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PMID:Impact of ZD1839 on non-small cell lung cancer-related symptoms as measured by the functional assessment of cancer therapy-lung scale. 1264 83

Primary neoplasms of the cardiac valves are extremely rare. However, papillary fibroelastoma is the third most common primary tumor of the heart [Ann Thorac Surg 52 (1991) 1127]. These tumors can be found anywhere in the heart, but most commonly involve the cardiac valves [Ann Thorac Surg 52 (1991) 1127; McAllister HA, Fenoglio JJ. Tumors of the cardiovascular system. In: Atlas of tumor pathology, 2nd series, vols. 1-3. Washington (DC): Armed Forces Institute of Pathology; 1978. p. 20-5]. Most papillary fibroelastomas do not cause symptoms and are usually incidental findings by routine echocardiography or at autopsy. However, early diagnosis of this condition is important, since it represents a surgically correctable cause of systemic emboli, stroke, myocardial infarction, and sudden cardiac death [Ann Thorac Surg 52 (1991) 1127; Ann Thorac Surg 68 (1999) 1881; J Am Soc Echocardiogr 9 (1996) 353; Tex Heart Inst J 22 (1995) 327; Tex Heart Inst J 26 (1999) 298]. The echocardiographic findings should be confirmed by histology, since the clinical differential diagnosis includes myxoma, vegetation, thrombi, lipoma, and pseudopapillary fibroelastoma [Tex Heart Inst J 26 (1999) 298; J Am Soc Echocardiogr 11 (1998) 92; J Natl Med Assoc 87 (1995) 68]. Review of the literature reveals that multiple papillary fibroelastomas are extremely rare [Am Heart J 125 (1993) 1443; J Am Soc Echocardiogr 7 (1994) 315; Ann Thorac Surg 48 (1989) 119]. Li Manduri et al. [J Am Soc Echocardiogr 7 (1994) 315] reported multiple masses on the tricuspid valve, the larger of which was 1 cm in diameter. De Virgilio et al. [Ann Thorac Surg 48 (1989) 119] reported a case of multiple 1-cm papillary fibroelastomas located on mitral valve, left ventricular outflow tract, and along septum. We report an unusual case of multiple papillary fibroelastomas in a woman, who initially was admitted because of a shortness of breath and recent cerebrovascular accident.
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PMID:An unusual case of multiple papillary fibroelastoma, review of literature. 1276 58

This report describes a 40-year-old man with a remote history of testicular mixed nonseminomatous germ cell tumor (NSGCT) treated by surgery and chemotherapy. He presented 10 years later with shortness of breath and was found to have a mass occupying the right atrium, based high in the superior vena cava. He also had multiple pulmonary emboli. The sarcomatous and myxomatous histological appearance of the neoplasm closely resembled a primary cardiac sarcoma. However, immunohistochemical studies confirmed the diagnosis of metatstatic sarcomatoid germ cell tumor. Metastatic spindle cell tumors have been reported in patients with NSGCTs. These neoplasms are thought to arise from the spindle cell component of the yolk sac tumor that is resistant to chemotherapy.
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PMID:Sarcomatoid intracardiac metastasis of a testicular germ cell tumor closely resembling primary cardiac sarcoma. 1460 47

Cardiac tumors in infants and children are extremely rare. Their clinical manifestations vary widely from asymptomatic presentations to life-threatening cardiac events. Improvements in diagnostic techniques, such as those offered by echocardiography, have made early detection of cardiac masses possible, with or without the presence of clinical symptoms. Fifteen pediatric cases of cardiac tumor were diagnosed at our institution between July 1989 and July 2002 (male-female ratio, 10:5; age range, one day to nine years). Eleven of the cases involved primary cardiac tumors [rhabdomyoma (n = 10) and fibroma (n = 1)]. Ninety percent of the rhabdomyomas (9/10) were associated with tuberous sclerosis. Four of the fifteen cases were secondary metastatic tumors [hepatoblastoma (n = 2), hepatoma (n = 1) and rhabdomyosarcoma (n = 1)]. Clinical manifestations of the cardiac tumors included shortness of breath (n = 5), seizure (n = 4), cardiac murmur (n = 6), and cyanosis (n = 3). Surgery was performed for three of 11 patients with primary cardiac tumor (27%) due to severe obstruction of flow (n = 2) and other cardiac defects (n = 1). The primary cardiac tumor spontaneously regressed in five of the tuberous sclerosis patients. All four of the patients with secondary cardiac tumors continued to receive chemotherapy, and only one of them subsequently experienced regression. Based on our experiences, we conclude that: 1) rhabdomyoma is the most common primary cardiac tumor in children; 2) most pediatric tumors are associated with tuberous sclerosis; 3) clinical presentation is determined by the tumor size and number of tumors, and whether expansion of the malignancy has resulted in cardiac blood-flow obstruction; 4) there is a strong possibility of regression of the primary cardiac tumor, with surgery recommended only when cardiac symptoms are severe; and, 5) unless there is a significant obstruction of blood flow, chemotherapy is still the treatment of choice for secondary cardiac tumors.
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PMID:Cardiac tumors in infants and children. 1467 25

Solitary fibrous tumor (SFT) of the pleura typically forms a localized pleura-based mass, and most are benign. A rare case of disseminated malignant SFT of the pleura is reported. The patient was a 71-year-old man who presented with complaints of shortness of breath to his primary care physician. A diagnosis of malignant mesothelioma was suspected, based on clinical, radiological and needle biopsy findings. He was referred to our institution for surgery. An extrapleural pneumonectomy, encompassing all pleural masses, was performed. Gross examination of the resected specimen was remarkable for numerous masses, ranging in size from 0.2 to 13.5 cm, covering the majority of the visceral pleura. Histologically, the tumor was composed of short spindle cells admixed with variable proportions of collagenous stroma. There were great intra- and intertumoral heterogeneity in tumor growth pattern, cellularity, pleomorphism and mitoses. Histologically malignant areas were present in all of the masses examined. The neoplastic cells were diffusely and intensely positive for bcl-2. Most tumor cells were also strongly stained for CD34 and CD99. Staining for cytokeratin was negative. The tumor also revealed p53 over-expression. Thus, the histological and immunohistochemical features of the tumor were consistent with a disseminated malignant SFT. This report shows that SFT rarely presents with disseminated pleural involvement, and a panel with CD34, bcl-2 and cytokeratin are valuable for differentiating SFT from malignant mesothelioma and other malignant spindle cell neoplasms of the pleura.
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PMID:Disseminated malignant solitary fibrous tumor of the pleura. 1472 Jan 42

The case of a 28-year-old male patient with a locally aggressive lesion of the distal tibia is presented. Following the diagnosis of giant cell tumor of bone (GCT) on biopsy and curettage, a rapid malignant course was observed with recurrence 2.5 months later. Multiple metastases appeared 6 months after initial presentation. Following initial chemotherapy according to the COSS protocol and later with carboplatin and VP-16, therapy was changed to Adriamycin and later gemcitabine due to progressive disease. Good palliation was achieved, and the patient felt well with less shortness of breath on exertion and was ambulatory with walking aids. The malignant nature of the tumor was not detected in the initial pathologic examinations. Review of the pathologic material provided histologic clues permitting the diagnosis of a primary malignant GCT with a fibrohistiocytic/fibrosarcomatous component. Malignancy in a giant cell tumor is a much debated diagnostic dilemma when a frank sarcomatous component is lacking. Cytologic atypias and flame-like tufts of infiltration of soft tissue are important clues. Surgical treatment should be commensurate. Monotherapy with Adriamycin or gemcitabine can be considered in order to inhibit the disease progression.
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PMID:[Giant cell tumor of bone with rapid malignant course]. 1500 59

Malignancy of the pericardium is a very rare entity. We describe here a case of a 38-year-old male admitted because of chest pain and shortness of breath. Following magnetic resonance imaging and CT-guided biopsy of the mediastinal mass, spindle cell sarcoma of the pericardium was diagnosed. The tumor was deemed unresectable due to invasion of vital mediastinal structures. The patient then underwent neoadjuvant chemotherapy with three cycles of ifosfamide, doxorubicin, and mesran. This resulted in a noticeable reduction in tumor size confirmed by follow-up magnetic resonance imaging and repeat transesophogeal echo. Both studies showed marked reduction in tumor bulk with no obvious invasion of the left atrium or pulmonary veins. The pericardial mass was resected utilizing cardiopulmonary bypass through a left thoracotomy in order to remove the tumor from the superior pulmonary veins and pulmonary hilum. The patient was discharged on postoperative day 6. Spindle cell sarcoma of the pericardium is a very rare tumor and other pericardial sarcomas may be best treated by combined neoadjuvant therapy followed by aggressive surgical resection when necessary, possibly utilizing cardiopulmonary bypass.
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PMID:Spindle cell sarcoma of the pericardium: a case report. 1501 51

We present 10 cases of "ancient" (sclerosing) thymomas in 4 women and 6 men (age range, 34-73 years; mean, 53.5 years). Clinically, 4 patients had a history of shortness of breath and chest pain of several weeks' duration, 1 had a history of myasthenia gravis, and 5 were asymptomatic (anterior mediastinal masses discovered on routine chest radiographs). All underwent complete surgical resection of the anterior mediastinal mass. The tumors were light tan and solid, without areas of hemorrhage or necrosis, and 5 to 10 cm in greatest diameter. Extensive areas of hyalinized fibroconnective tissue constituting about 85% to 90% of the tumor mass was the main tumor feature. Focal areas of conventional thymoma also were present in all tumors. Follow-up information was obtained for 8 patients. Of these 8 patients, 6 died, all of unrelated causes (congestive heart failure, renal insufficiency, and pulmonary edema), and 2 were alive 1 and 6 years after surgical resection. The present cases highlight an important histopathologic feature of thymomas, which can pose problems in diagnosis, namely when dealing with small mediastinoscopic biopsy specimens.
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PMID:"Ancient" (sclerosing) thymomas: a clinicopathologic study of 10 cases. 1519 59


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