Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029463 (osteosarcoma)
 16,637 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report on a 26-year-old Caucasian woman who was referred to the Department of Surgery complaining of general malaise, feeling of fullness with occasional vomiting and intermittent jaundice. The patient had previously suffered from tibial osteosarcoma of the left leg which was resected 13 years ago and subsequently treated with radiation and chemotherapy. During clinical investigations a 12 x 12 x 6.5 cm large mass was found in the left lobe of the liver. This was resected, and subsequently shown to be a sporadic hepatic angiomyolipoma. In order to investigate a possible link between the two tumours, we investigated mutations in the p53-gene, loss of heterozygosity (LOH) at p53, Rb and p16, c-Myc expression, and the telomerase activity of the angiomyolipoma and the osteosarcoma. Whilst the tibial osteosarcoma showed LOH at p16, no genetic alterations or increased telomerase activity were found in the angiomyolipoma. The occurrence of both these tumours in this patient is therefore probably a coincidence.
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PMID:Hepatic angiomyolipoma in a 26-year-old Caucasian woman with a history of tibial osteosarcoma. 1060 97

Imaging and histology are two complementary morphological techniques which play a fundamental role in the diagnosis and management of soft tissue sarcomas. Imaging allows to identify some pseudosarcomatous benign lesions such as myositis ossificans, intramuscular hemangioma, angiomyolipoma, intramuscular lipoma, giant cell tumour of tendon sheath, desmoid tumour and elastofibroma. There is no formal criterion for diagnosing a sarcoma on magnetic resonance imaging (MRI) but malignancy is strongly suspected with the presence of necrosis and vascular, bone or joint invasion. Imaging may also suggest some histological types of sarcoma such as well-differentiated liposarcoma, dedifferentiated liposarcoma, synovial sarcoma or extraskeletal osteosarcoma. Imaging is also extremely helpful in determining the appropriate kind of sampling to carry out and in guiding the performance of a microbiopsy. The appearance observed on imaging should always be taken into consideration for the interpretation of the microbiopsy by the pathologist.
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PMID:Histology and imaging of soft tissue sarcomas. 1983 58

Mesenchymal neoplasms of the kidney in adults cover a wide spectrum with characteristic ontogeny and histologic findings and variable biologic profiles and imaging findings. Benign mesenchymal renal tumors include angiomyolipoma, leiomyoma, hemangioma, lymphangioma, juxtaglomerular cell tumor, renomedullary interstitial cell tumor (medullary fibroma), lipoma, solitary fibrous tumor, and schwannoma. Malignant renal tumors of mesenchymal origin include leiomyosarcoma, rhabdomyosarcoma, angiosarcoma, osteosarcoma, synovial sarcoma, fibrosarcoma, malignant fibrous histiocytoma, and solitary fibrous tumor. Cross-sectional imaging findings for mesenchymal renal tumors in adults are varied. Although angiomyolipomas and lipomas show macroscopic fat, lymphangiomas are cystic in appearance. Renal hemangioma may show phleboliths and a characteristic enhancement pattern. Leiomyoma typically arises from the capsule and causes buckling of the renal cortex. Although osteosarcoma may demonstrate characteristic dense ossification, most renal sarcomas demonstrate imaging features that are indistinguishable from the more common renal cell carcinoma. Although some renal mesenchymal tumors have typical imaging findings, biopsy is warranted to establish a definitive diagnosis. Awareness of the various mesenchymal renal tumors and familiarity with their imaging findings permit optimal patient management.
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PMID:Mesenchymal neoplasms of the kidney in adults: imaging spectrum with radiologic-pathologic correlation. 2107 73

Sirtuin, silent mating-type information regulation 2 homolog Saccharomyces cerevisiae 1 (SIRT1), is a protein that has been implicated in multiple mammalian functions including cell aging, stress resistance, and differentiation. SIRT1 has also been shown to be involved in multiple tumors. In addition, new pharmacotherapies have recently been approved that target SIRT1. The purpose of this study was to use immunohistochemistry to characterize SIRT1 protein expression in human soft tissue neoplasms with the hopes of finding new diagnostic and therapeutic modalities. SIRT1 immunoreactivity was reviewed in a series of 164 soft tissue tumors including alveolar soft part sarcoma, angiomyolipoma, clear cell sarcoma, desmoid/fibromatosis, desmoplastic small round cell tumor, Ewing sarcoma, gastrointestinal stromal tumor, glomus tumor, leiomyoma, leiomyosarcoma, lipoma, liposarcoma, malignant peripheral nerve sheath tumor, nodular fasciitis, osteosarcoma, rhabdomyosarcoma, schwannoma, solitary fibrous tumor, synovial sarcoma, undifferentiated pleomorphic sarcoma, and Wilms tumor. In addition, numerous benign tissues were tested for SIRT1 reactivity. In nonneoplastic tissue, strong cytoplasmic SIRT1 reactivity was observed in all prostate stroma, smooth muscle, and striated muscle. A similar pattern of cytoplasmic SIRT1 expression was observed in soft tissue neoplasms with myoid differentiation, namely, angiomyolipoma (100%), glomus tumor (100%), leiomyoma (90%), leiomyosarcoma (76.5%), and rhabdomyosarcoma (87%). The other lesions examined were negative. Although the physiologic role of SIRT1 remains to be clarified in myoid tissues and neoplasms differentiating along these lines, this observation points to a potential role for this marker in diagnostic immunohistochemistry. Furthermore, the recent emergence of drugs capable of selectively inhibiting SIRT1 raises the possibility of a potential application for targeted therapy. Additional studies are necessary to further characterise the role of SIRT1 in myoid tissues and neoplasms.
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PMID:Sirtuin 1 (SIRT1): a potential immunohistochemical marker and therapeutic target in soft tissue neoplasms with myoid differentiation. 2333 67