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)

Multilocular cystic renal tumor is a term that encompasses two histologically distinct but grossly indistinguishable lesions: cystic nephroma and cystic partially differentiated nephroblastoma (CPDN). Cystic nephroma is a segmental, purely cystic mass characterized by multiple septations composed entirely of differentiated tissues, without blastemal elements. CPDN is also a multiloculated lesion without nodular solid components, but its septa contain embryonal cells. Multilocular cystic tumors primarily affect boys during early childhood, with a substantial number of the lesions containing blastema (CPDN), and adult women, with lesions that more commonly lack septal blastema (cystic) nephroma). As a rule, nephrectomy is curative and the clinical course benign, but CPDN may recur locally. Although cystic nephroma and CPDN cannot be distinguished radiologically, failure to do so has no practical impact on management, since all of these tumors are surgically removed. However, the differential diagnosis includes other pediatric cystic renal masses that may require different treatment stratagems: Wilms tumor with cyst formation due to hemorrhage and necrosis, cystic clear cell sarcoma, cystic mesoblastic nephroma, cystic renal cell carcinoma, multicystic dysplastic kidney, and segmental multicystic dysplasia in a duplicated renal collecting system.
 ...
PMID:Multilocular cystic renal tumor in children: radiologic-pathologic correlation. 762 70

Adamantinoma is a rare primary tumour of long bones containing mesenchymatous and epithelial cells. There has been some controversy over the pathogenesis. We observed a localization in the right tibia. A 22-year-old patient from Algeria was seen for spontaneous progressively increasing pain in the upper part of the right tibia. The patient's general health had deteriorated somewhat, with fever. On examination there was ulceration of the skin, costal and pelvic pain and inflammatory right inguinal lymph nodes. Radiography revealed lateral metaphyseal lytic image with interruption of the cortex also seen on magnetic resonance imaging which revealed invasion of the soft tissue and multiple bilateral pulmonary nodules. Bone scintigraphy showed several zones of hyperfixation. The diagnosis of adamantinoma was confirmed by pathology examination of the biopsy specimen. On surgical exeresis, the capsule of the knee joint was found to be involved without invasion of the knee joint. Node dissection showed inguinal and popliteal invasion. Macroscopically, the surgical specimen was a red-whitish osteolytic tumour. Microscopically, the tumour was composed of hyperchromatic epithelial cells in an abondant fibrous stroma. Immunohistochemical studies were negative for vimentine, cytokeratin and factor VIII. Adjuvant chemotherapy was based on a sarcoma protocol. Unfortunately, after two cycles, white cell counts fell sharply and multiple skin nodules appeared together with progression of the bone metastases. The chemotherapy was modified without any therapeutic effect and the patient died in February 1993. An epithelial origin would appear most probable, but at least two groups of adamantinoma can be described: one with typical epithelial differenciation and one overlapping to the differential diagnosis of osteofibrous dysplasia. Although considered as a low grade malignant tumour, we emphasize the aggressive forms with local relapse or metastatic resistance to chemotherapy. Treatment relies on wide surgery and prognosis is generally good. Neither chemotherapy nor radiotherapy has stood the test of time in cases with metastasis.
 ...
PMID:[Adamantinoma of the proximal end of the tibia. A case]. 782 79

Benign bone tumors are frequently undiagnosed since they rarely cause pain. Based on the records of the Basle Bone Tumor Registry, which contains 2194 primary benign bone tumors, the aspects of benign bone tumors in childhood and adolescence are described. Benign bone-forming lesions like osteoid osteoma and osteoblastoma occur fairly frequently in this age group; osteofibrous dysplasia is almost exclusively seen in younger patients. Benign cartilage-forming tumors are much more cellular at this age and therefore sometimes misdiagnosed as chondrosarcomas. Chondromyxoid fibroma and especially chondroblastoma frequently occur in patients younger than 20 years. Because of their pleomorphic appearance (chondromyxoid fibroma) and their cellular composition and matrix deposition (chondroblastoma) they can be confused with chondrosarcomas, giant cell tumors or osteosarcomas. Benign fibrous histiocytoma is a very rare intraosseous fibrohistiocytic tumor that also can be found during adolescence. Since no one has much experience with this lesion, patients should be carefully monitored after complete excision. Giant cell tumors of bone reach their typical epimetaphyseal location after growth plate closure. If the physis is still open, giant cell tumors are almost exclusively found in a metaphyseal location abutting on epiphyseal cartilage. With increasing closure of the growth plate, the lesion is found most frequently in an epimetaphyseal position. The transition of a primary benign bone tumor to a sarcoma is very rare and often occurs in connection with irradiation treatment. The risk of a sarcomatous change independent of therapy is very infrequent.
 ...
PMID:[Pathologic-anatomic characteristics of benign bone tumors]. 789 3

Fibrous dysplasia is usually a slowly progressive, benign disease that develops over several years and presents with deformity or mild symptomatology. Five of 34 patients (ages 4-21 years), who were subsequently diagnosed histologically as having fibrous dysplasia of the maxillary sinus, rapidly developed soft tissue masses of the malar region over a period of less than 4 months with accompanying pain (2 patients) and nasal obstruction and exophthalmos (2 patients). Each was clinically suspected of having a sarcoma; two had been thought to have an "osteofibrosarcoma" on initial biopsy at outside hospitals. After resection, all lesions developed regrowth. At histopathologic examination, both initial and recurrent masses proved to be typical fibrous dysplasia with spicules of woven bone in cellular, sometimes vascular, fibrous tissue. No malignant degeneration was found. On conventional radiography, aggressive fibrous dysplasia produced opacification and expansion of the maxillary sinus and apparent disruption of its wall with an associated soft tissue mass. Computed tomography (CT) demonstrated voluminous heterogeneous masses with "ground glass appearance", calcifications, areas of enhancement, low attenuation, cystic areas, and a thinned, sometimes interrupted, maxillary wall. Despite the aggressive clinical course for both initial and recurrent lesions, the CT findings of a "ground glass" mass with calcifications surrounded by a maxillary sinus wall, even if incomplete, can suggest the diagnosis of aggressive fibrous dysplasia.
 ...
PMID:Aggressive fibrous dysplasia of the maxillary sinus. 829 Oct 7

Jeune's syndrome is a rare autosomal disorder characterized by osseous dysplasia, fetal respiratory distress, and renal failure in later life. We describe a 27-year-old man with Jeune's syndrome who underwent renal transplantation and 6 years later developed a sarcoma (primitive neuroectodermal tumor [PNET]) in the soft tissue of the chest wall, a principal site of dysplasia in this disorder.
 ...
PMID:Primitive neuroectodermal tumor of the chest wall in a patient with Jeune's syndrome and renal transplant. 838 18

Mice carrying a Moloney murine sarcoma virus-(MSV) simian virus 40 large T transgene develop heritable tumors including endocrine pancreatic tumors. We have established several independent transgenic mouse lines expressing this transgene. One of these lines, designated MSV125, is characterized by the development of congenital cataracts and either pancreatic or brain tumors. The development and histopathology of the pancreatic tumors were studied by light microscopy and immunocytochemistry for large T antigen, neuron-specific enolase, insulin, proinsulin, glucagon, somatostatin, pancreatic polypeptide, gastrin, and serotonin. The 23 tumors examined were similar to human endocrine pancreatic tumors with respect to their macroscopic and histological features. We classified 91% of the tumors as insulinomas based on the predominance of insulin immunoreactivity. In newborn and young transgenic animals, nesidioblastosis and islet cell proliferation, consisting mostly of insulin containing beta cells, was obvious and persisted into adulthood. In transgenic animals more than 2 months old, islet hyperplasia and dysplasia predominated from which single tumors developed. Hyperplastic and dysplastic islets were composed mostly of beta cells. Large T antigen was detectable not only in tumor cells, but also in cells of normal and hyperplastic islets and in islet anlagen of newborn transgenic mice, indicating expression of the transgene in the endocrine part of the pancreas. Large T antigen-immunoreactivity was restricted to the beta cells. Insulinomas of the MSV-simian virus 40 T antigen-derived MSV125 transgenic mouse line may represent a valuable model for the study of the development and biology of insulinoma.
 ...
PMID:Endocrine pancreatic tumors in MSV-SV40 large T transgenic mice. 838 73

A recently described splice variant of CD44 expressed in metastasizing cell lines of rat tumors has been shown to confer metastatic potential to a non-metastasizing rat pancreatic carcinoma cell line and to non-metastasizing sarcoma cells. Homologues of this variant as well as several other CD44 splice variants are also expressed at the RNA level in human carcinoma cell lines from lung, breast, and colon, and in immortalized keratinocytes. Using antibodies raised against a bacterial fusion protein encoded by variant CD44 sequences, we studied the expression of variant CD44 glycoproteins in normal human tissues and in colorectal neoplasia. Expression of CD44 variant proteins in normal human tissues was readily found on several epithelial tissues including the squamous epithelia of the epidermis, tonsils, and pharynx, and the glandular epithelium of the pancreatic ducts, but was largely absent from other epithelia and from most non-epithelial cells and tissues. In human colorectal neoplasia CD44 variant proteins, including homologues of those which confer metastatic ability to rat tumors, were found on all invasive carcinomas and carcinoma metastases. Interestingly, focal expression was also observed in adenomatous polyps, expression being related to areas of dysplasia. The distribution of the CD44 variants in human tissues suggests that they play a role in a few restricted differentiation pathways and that in colorectal tumors one of these pathways has been reactivated. The finding that metastasis-related variants are already expressed at a relatively early stage in colorectal carcinogenesis and tumor progression, i.e., in adenomatous polyps, suggests the existence of a yet unknown selective advantage linked to CD44 variant expression. The continued expression in metastases would be compatible with a role in the metastatic process.
 ...
PMID:A human homologue of the rat metastasis-associated variant of CD44 is expressed in colorectal carcinomas and adenomatous polyps. 841 89

There are divergent opinions on the effect of ethanol in the carcinogenesis of gastroduodenal tumors. The effect of the synchronous application of 11% ethanol or wine (11% ethanol) and N-methyl-N'-nitro-N-nitrosoguanidine (100 micrograms/ml, MNNG) in a drinking solution on the incidence of gastroduodenal tumors was evaluated. Sixty outbred male Wistar rats were distributed among three groups. The animals drank MNNG and ethanol or wine for six months and consumed the same quantity of MNNG. Then they consumed a normal diet until the 13th month, when the experiment was terminated. The stomach and duodenum were examined histologically. In the stomach, 15 tumors (2 squamous paillomas, 4 squamous carcinomas, 1 sarcoma, and 8 adenocarcinomas) and 4 cases of dysplasia were found; in the duodenum, there were four cases of adenocarcinoma. There were 6 cases of multiple tumors. Incidence of forestomach tumors did not differ among the groups, whereas the incidence of glandular stomach carcinoma and duodenal carcinoma was significantly lower in the groups treated with 11% ethanol or wine than in the control group. MNNG was not inactivated by ethanol in the drinking solutions. We concluded that the inhibitory effect on gastroduodenal carcinogenesis is the result of 11% ethanol ingestion and its protective action on the mucosa and not of the wine's nonethanol components.
 ...
PMID:Inhibition of MNNG-induced gastroduodenal carcinoma in rats by synchronous application of wine or 11% ethanol. 891 Sep 16

Between 1991 and 1994, 582 operations were performed in our service; 19 (3.26%) were on primitive tumors of the chest wall. We analyze the data for these patients, including age, sex, clinical findings, chest images, diagnoses, therapy and course. Ten tumors were benign and 9 were malignant. The most frequent clinical findings were pain and/or tumor. Diagnosis was achieved before surgery in only 2 cases. Except when there are clear macroscopic and X-ray signs that the tumor is benign, we performed broad exeresis of the chest wall, sometimes also resecting adjacent structures. The defect was repaired directly in 12 cases. The defects were covered by prostheses and/or muscle plasty in the remaining patients. The most frequent tumor was chondrosarcoma (3 cases), followed by 2 cases of osteoblastoma and osteochondroma. Only 1 each of the following tumors were found: plasmocytoma, chondroma, fibrous dysplasia, eosinophilic granuloma, osteosarcoma, Ewing's tumor, epithelioid sarcoma, fibrosarcoma, hemangioma, benign neurilemmoma, desmoid tumor and liposarcoma. Two patients with chondrosarcoma were operated on for recurrences and there was also recurrence in the patient with Ewing's tumor. We conclude that: 1) chest wall tumors are infrequent, 2) radical exeresis is the treatment of choice and prosthesis is often necessary, and 3) chondrosarcoma, with poor outcome in our patients, is the most frequent tumor.
 ...
PMID:[Primary tumors of the thoracic wall (1991-1994)]. 1256 9

The Finkel-Biskis-Jinkins murine sarcoma virus, which carries v-fos, induces osteosarcomas, whereas high-level expression of exogenous c-fos in transgenic and chimeric mice leads to postnatal development of osteogenic and chondrogenic tumors, respectively. To test whether such target cell specificity of an oncogene can be detected even in early development, we induced ectopic expression of fos in chicken limb buds by microinjecting replication-competent retrovirus into the presumptive leg field of stage 10 embryos. This caused cartilage truncation of all the long bones of the injected leg, which was mainly attributable to chondrodysplasia due to severe retardation of differentiation of the proliferating chondrocytes into mature or hypertrophic chondrocytes, as well as a slight delay in precartilagenous condensation. Expression of genes for all the other known members of chicken AP-1, which include such transforming genes as c-jun and fra-2, however, caused no macroscopic abnormalities in limb formation, indicating a specific function of Fos proteins in embryonic endochondral bone differentiation. The extent of truncation was stronger with v-Fos than with c-Fos, and comparative analysis of these proteins, as well as v-Fos mutants, revealed that strong transforming activity of Fos protein is necessary to cause dysplasia, suggesting that common molecular mechanisms are involved in both embryonic chondrodysplasia and bone tumor formation in postnatal mice.
 ...
PMID:Chondrocytes as a specific target of ectopic Fos expression in early development. 910 93


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>