Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C1261473 (sarcoma)
 25,952 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A prospective study of 32 patients with primary upper small intestinal lymphoma in our region revealed 10 cases of alpha heavy-chain disease. Patients were mostly in the second and third decades of life and males predominated. Weight loss, diarrhea, and abdominal pain were the most common complaints and clubbing the most frequent physical findings. Laboratory tests revealed a malabsorption pattern on intestinal x-rays, and malabsorption of xylose, fat, and vitamin B12 was frequently noted. Dense plasmacytic infiltrate of the lamina propria of small bowel was the most frequent pathologic finding while true neoplasm of the lymphoid system (ie, immunoblastic sarcoma) was encountered in 20% of the cases.
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PMID:Alpha heavy-chain disease in southern Iran. 41 71

The case of a primary esophageal carcinosarcoma, shown to express p53 protein, is presented. The patient, a 57-year-old male, presented with fever, weight loss, and clubbing, but without swallowing difficulties. A large intraesophageal tumor was found on radiologic imaging, and sarcoma was diagnosed on esophagoscopic biopsy. Despite total esophagectomy and adjuvant chemo-radiotherapy, pleural metastases developed 3 months postoperatively. Histologically, the tumor was composed of epithelial and sarcomatous elements. Using monoclonal and polyclonal anti-p53 antibodies, p53 protein was distributed heterogenously throughout the sarcomatous elements of the primary tumor. Immunoreactivity was also found in regional lymph node metastases. These observations further implicate the p53 tumor suppressor gene in the pathogenesis of human esophageal cancers.
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PMID:p53 immunoreactivity in carcinosarcoma of the esophagus. 800 79

A 43 year old woman presented with acute myeloid leukaemia, marked finger and toe clubbing, and a hilar mass. Biopsy of the hilar mass was not technically possible, but it almost certainly represented a granulocytic sarcoma since chemotherapy induced rapid resolution of the mass along with reversal of the clubbing and remission of the leukaemia. Relapse of the leukaemia 21 months later was associated with return of the clubbing. It is hypothesized that an abnormal circulation within the granulocytic sarcoma may have accounted for the development of clubbing.
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PMID:Reversible digital clubbing in acute myeloid leukaemia. 802 71

Hypertrophic osteoarthropathy (HOA), well known in adults, is rarely encountered in children. The clinical features include clubbing of the fingers and toes, arthritis, and a sometimes painful ossifying periostitis of the tubular bones. Apart from a hereditary form (primary HOA), most of the cases encountered in children are secondary and associated with conditions such as chronic suppurative lung processes (e.g., cystic fibrosis), congenital heart disease, biliary atresia, and polyposis coli. The association with malignant disorders, which is relatively common in adults, is very rare in children. In 1986 the authors published a case report of a patient with carcinoma of the nasopharynx who developed HOA. Another similar patient has been encountered. In both, the appearance of HOA was associated with a very poor prognosis. A meticulous research of the literature from 1890 to 1990 revealed only 24 children (19 boys, 5 girls) under the age of 18, with malignancy and associated HOA. Among them were 10 patients with a carcinoma of the nasopharynx, 8 with osteosarcoma, 3 with Hodgkin's lymphoma, 1 with a periosteal sarcoma, 1 with mesothelioma of the pleura, and 1 with carcinoma of the thymus. In five patients with HOA, there were no abnormalities of the lungs, mediastinum, or pleura, and none developed during the course of the disease. Many authors mention the predictive value of HOA, especially in association with malignant tumors. In contrast to suppurative processes in the lungs, in those with neoplastic disease involving the chest, HOA may precede pulmonary symptoms by 1-18 months. A striking feature of HOA in these instances is the reversibility of the complaints after successful treatment of the disorder of the chest, both in benign and malignant conditions. The present case is the second reported by the authors and the first description of a girl with carcinoma of the nasopharynx developing HOA.
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PMID:Hypertrophic osteoarthropathy in childhood malignancy. 841 3

A case of digital clubbing in a patient with pulmonary artery sarcoma and severe pulmonary hypertension is presented. The differential diagnosis of clubbing and clinical features of pulmonary artery sarcoma are reviewed with emphasis on the possible association of clubbing with pulmonary artery sarcoma.
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PMID:Digital clubbing associated with pulmonary artery sarcoma. 876 27

A 50-year-old woman presented with subacute cognitive decline, impaired eye movements, and simultanagnosia, components of the Balint syndrome. She had relatively low blood pressure in the left arm and left finger clubbing. Brain imaging identified multiple acute infarcts. Transesophageal echocardiography showed no cardiac abnormalities but demonstrated a thickened aortic wall and an intraluminal aortic arch mass. The surgical specimen revealed angiosarcoma. Of the few reported angiosarcomas involving the aorta, most have been located in the abdominal segment. This is only the second reported case of aortic arch sarcoma presenting with stroke.
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PMID:Multiple brain infarcts and Balint syndrome in aortic arch angiosarcoma. 1684 10