Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P04637 (p53)
 77,613 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fourteen cases (13 pleural and one intrapulmonary) of solitary fibrous tumors (SFTs) (the so-called fibrous mesothelioma) were studied. The lesions occurred more in females (nine cases) than males (five cases). The age of patients ranged from 44 to 73 years old (median 60 years). The tumors presented as cough with or without blood-tinged sputum, exertional dyspnea, chest pain, nausea, body weight loss, fever, or as asymptomatic masses detected by routine chest radiograph. Two patients with huge (tumor larger than 20 cm) malignant tumors had accompanying pleural effusion and one associated with hypoglycemia. Ten benign tumors measured 2-11 cm (median size 7 cm) while the remaining four histologically malignant ones measured 20-30 cm in size. All of them were well circumscribed and thinly encapsulated. Hemorrhage and necrosis were more frequently seen in the malignant tumors. Histologically, these lesions were characterized by 'patternless pattern' with occasional hemangiopericytic features (three cases). The tumor cells were all immunoreactive for vimentin, CD 34, and focally actin-positive in one case, but not for keratin, desmin, S-100 protein, carcinoembryonic antigen, alpha 1-ACT and F VIII-related antigen, supported a primitive mesenchymal origin. p53 protein was expressed in two of the malignant cases. Proliferating cell nuclear antigen stain was positive with 50 and 80% of the labeling index in the benign and malignant tumors, respectively, but retinoblastoma gene protein was negative in all tumors. This analysis confirmed the relationship between histological malignant SFTs and tumor size, cellularity, mitotic activity, necrosis and tumor suppressor gene expression. However, the clinical behavior was unpredictable. Complete respectability seemed to be the most important indicator of clinical outcome in the less aggressive tumors.
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PMID:Thoracic solitary fibrous tumor: clinical and pathological diversity. 1010 Jan 46

Thymic carcinoma (primary carcinoma of the thymic epithelium; type C thymoma) is a rare malignancy. It usually presents in middle-aged to elderly patients and can exhibit a wide variety of morphologic appearances. Thymic basaloid carcinoma (thymic BC) is a particularly rare subtype, with less than 20 cases published in the English literature, mostly in the form of individual case reports. In this study, we present the clinicopathologic and immunohistochemical features of 12 new cases of thymic BC. There were 10 (83%) men and 2 (17%) women. Ages at the time of initial diagnosis ranged from 34 to 77 years (mean 55 y). The 2 most common manners of presentation were dyspnea on exertion (3 patients) and as an incidental finding on radiographic imaging (2 patients). Tumors ranged in size from 4.4 to 17 cm (mean 10.1 cm). One of 12 cases (8.3%) was associated with a multilocular thymic cyst. Immunohistochemistry was performed in 8 cases. Pan-cytokeratin was positive in all cases. CD117 (c-kit) was positive in 6 of 8 cases (75%), p63 was positive in 7 of 8 cases (88%), p53 was positive in 7 of 8 cases (88%), ranging from <10% to 90%, CD5 was focally positive in 3 of 8 cases (38%), collagen type IV was positive in 4 of 8 cases (50%), and proliferative index, as estimated by Ki67, ranged from <1% to approximately 15%. In 1 of 2 cases with sarcomatoid differentiation, Ki67 was greater than 80% in the sarcomatoid area. Cases were negative for thyroid transcription factor-1 (0 of 8), S-100 (0 of 7), and synaptophysin (0 of 7). Long-term data was available in 8 patients with an average follow-up of 30 months. Five patients died of their disease at an average of 34 months from the time of diagnosis. Of the remaining 3 patients, 1 had a stable recurrence and died at 4 years from unrelated causes, and 2 were alive without the evidence of disease at 12 and 7 months, respectively. Thymic BC, although previously regarded as a low-grade neoplasm, has shown that it is capable of aggressive behavior and significant mortality. In this paper, we review the pertinent literature and discuss the possible relationship of thymic BC with thymic adenoid cystic carcinoma, as well as BCs and adenoid cystic carcinomas at other sites.
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PMID:Thymic basaloid carcinoma: a clinicopathologic study of 12 cases, with a general discussion of basaloid carcinoma and its relationship with adenoid cystic carcinoma. 1946 9

Cardiac myxoma is a benign neoplasm composed of stellate to plump, cytologically bland mesenchymal cells set in a myxoid stroma. Although benign, as they can lead to severe complications, they are often removed surgically. A 39-year-old female presented with a chief complaint of generalized fatigue. Patient had a history of a large 7cm x 2.5cm left atrial myxoma resected at the age of 32 years after she presented with symptoms of dyspnea on exertion. The dyspnea was due to prolapse of the mass through the mitral valve during diastole, leading to functional severe mitral stenosis. The mass was resected with clear margins confirmed on biopsy. On physical examination, heart rate was regular with no murmurs. No signs of congestive heart failure were noted. A 2D echo revealed a mobile structure in the left atrium along with mild mitral regurgitation. Cardiac MRI showed a 21mm x 9mm well defined, pedunculated, mobile mass in the left atrium arising from inter-atrial septum. The mass was hyperintense on T2 weighted images with patchy delayed hyper-enhancement consistent with recurrence of a myxoma. The patient underwent a repeat median sternotomy with the removal of left atrial mass and repair of atrial septum with hemashield patch. The mass was sent for pathological evaluation confirming the diagnosis of recurrent myxoma. On genetic testing, patient tested negative for mutations in PRKAR1A gene (mutated in up to 60%-80% cases with Carney complex), MEN1, RET and sarcoma (TP53) genes. Cardiac myxomas are rare primary benign tumors of the heart with a small recurrence rate. Follow-up studies have rarely reported recurrences after complete resection. However, in our case not only did the patient have the sporadic form of myxoma with recurrence, but it also occurred within three years of the previous resection despite complete removal with clear margins.
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PMID:Sporadic Form of Recurrent Atrial Myxoma: The Blob Strikes Back. 3294 60