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)

Deposition of basement membrane extracellular matrix is influenced by adjacent tumor cells, and in some cases, the pattern of type IV collagen deposit is characteristic in malignant tumors. In this report, we analyzed the difference in type IV collagen deposition patterns between benign and malignant phyllodes tumors (PTs) of the breast. Of the 15 cases of PTs, 8 cases were benign PTs and 7 cases were malignant PTs. Three cases of other primary sarcomas of the breast (stromal sarcoma, angiosarcoma and osteosarcoma) and 2 cases of fibroadenomas were studied for comparison. The malignant PTs were distinguished from benign ones by increased mitotic figures, cellular atypism, and a higher proliferation index of stromal cells. Immunohistochemical staining against type IV collagen in malignant PTs revealed extensive to moderate deposition of type IV collagen around the small blood vessels in duplicate or multilayering pattern, while benign PTs showed minimal deposition in a single linear pattern. All of the three cases of other sarcomas revealed multilayering or meshwork pattern of type IV collagen around the blood vessels. The deposition of type IV collagen around the blood vessels may reflect the malignant behavior of the stromal tumors of the breast.
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PMID:Differential pattern of perivascular type IV collagen deposits in phyllodes tumors of the breast. 129 40

Twenty-one cases in which sarcomas metastasized to the ovaries are reported. The patients ranged from 18 to 79 (average 42) years of age; only five of them were over 50 years old. Eleven tumors were primary in the uterus and 10 outside the genital tract. Three uterine tumors were leiomyosarcomas, and eight, endometrial stromal sarcomas. The extragenital primary tumors were leiomyosarcoma of the stomach (1) and small intestine (2), retrovesical leiomyosarcoma (1), fibrosarcoma of the anterior abdominal wall (1), sarcoma of the mesentery of smooth muscle or neural type (1), hemangiosarcoma probably primary in the heart (1), osteosarcoma of the maxilla (1), chondrosarcoma of the rib (1), and Ewing's sarcoma of the pubic bone (1). The ovarian tumors, most of which were large, were discovered at the same time as the primary tumors in 11 cases; in seven cases, the ovarian tumor was discovered 7 months to 9 years after diagnosis of the primary tumor. In three cases, the ovarian tumors were discovered 4, 7, and 10 months before detection of the primary neoplasm. Two of these tumors were endometrial stromal sarcomas, and one, an epithelioid leiomyosarcoma of the stomach. Eleven ovarian metastases were bilateral. On microscopic examination, the greatest difficulty in pathologic interpretation was posed by the metastatic endometrial stromal sarcomas because of their simulation of sex cord-stromal tumors. Features helpful in their distinction from these tumors included the frequent presence of extra-ovarian disease, bilaterality, and a characteristic content of small arteries resembling the spiral arteries of the late secretory endometrium. The other tumor that caused major diagnostic difficulty was the metastatic epithelioid leiomyosarcoma from the stomach, which had a pattern that initially suggested the solid-tubular pattern of a Sertoli cell tumor.
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PMID:Sarcomas metastatic to the ovary: a report of 21 cases. 237 88

Low-affinity nerve growth factor receptor (p75) is a member of the tumor necrosis factor receptor family. It may modulate the binding of nerve growth factor (NGF) to the functional high-affinity receptor tyrosine kinase (trk) A. NGF is thought to be responsible for growth, apoptosis, and function of the nervous system. The presence of this receptor (p75) was determined in a large group of neural and nonneural tumors and fetal and adult tissues. One thousand one hundred fifty tumors were analyzed with monoclonal antibody for p75, along with selected normal fetal and adult tissues. Immunoreactivity for p75 was present in adult pericytes, perivascular fibroblasts, basal cells of several types of epithelia, perineurial cells, and dendritic reticulum cells. Additionally, a wide zone of subepithelial mesenchyme and skeletal muscle were positive in the first-trimester fetus, but were diminished or negative in the adult. Consistently positive nonneural mesenchymal tumors included dermatofibrosarcoma protuberans (DFSP), embryonal and alveolar rhabdomyosarcoma, synovial sarcoma, and spindle cell hemangio(endotheli)oma. Schwann cell tumors, ganglioneuroma, granular cell tumor, and malignant peripheral nerve sheath tumor (MPNST) were also p75 positive. Mesenchymal nonneural tumors that were variably positive (32% to 69%) for p75 included fibrosarcoma variants, solitary fibrous tumor, hemangiopericytoma, spindle cell lipoma, Ewing's sarcoma, mesenchymal chondrosarcoma, and malignant melanoma. Nervous system tumors such as paragangliomas, neuroblastoma, meningioma, and perineurioma and nonneural mesenchymal tumors, including extraskeletal osteosarcoma, benign fibrous histiocytomas, fibromas, alveolar soft part sarcoma, epithelioid sarcoma, smooth muscle and gastrointestinal stromal tumors, and angiosarcomas, were almost always negative for p75. Epithelial tumors that were consistently positive included mixed tumor and adenoid cystic carcinoma, whereas mesothelioma, adenocarcinomas, and most squamous cell carcinomas were negative. p75 is not a specific marker for nerve sheath tumors. It is present in a variety of other mesenchymal tumors including synovial sarcoma and in CD34-positive tumors such as DFSP, spindle cell lipoma, and hemangiopericytoma. The presence of p75 in nonneural tumors such as DFSP and rhabdomyosarcoma mimic its presence in early fetal mesenchyme and skeletal muscle, suggesting oncofetal expression in these tumors. p75 may be useful to distinguish DFSP from benign fibrous histiocytoma.
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PMID:Low-affinity nerve growth factor receptor (p75) in dermatofibrosarcoma protuberans and other nonneural tumors: a study of 1,150 tumors and fetal and adult normal tissues. 1156 28

Microarrays began to be used to study gene expression profiles in the mid-1990s, but it was only after 2000 that serious attempts have been made to apply this technology to investigate sarcomas. Microarray technologies provide a comprehensive survey of active molecular pathways and potential molecular targets for diagnosis and treatment, but are challenging to use because of issues of specimen collection, cost, and complexities in experimental design and data analysis. As a discovery-based technique, microarray analyses are most valuable when framed around specific gaps in our knowledge of tumor etiology and progression, challenges in differential diagnosis, and pressing therapeutic needs. To date, microarray analyses of sarcomas support their division into molecularly defined and molecularly heterogeneous categories, and have provided useful diagnostic markers for entities such as gastrointestinal stromal tumors, synovial sarcoma, and dermatofibrosarcoma protuberans. Signatures predicting outcome and response to therapy have been published for Ewing sarcoma and osteosarcoma, and receptor tyrosine kinase expression patterns have suggested novel therapeutic approaches which may be applied to several types of sarcoma. Nevertheless, results need to be interpreted in the context of histopathology and validated by complementary technologies and/or other research groups.
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PMID:Microarray analysis of sarcomas. 1685 50

A promising targeted therapy against NY-ESO-1 (CTAG 1B) using genetically modified T-cells in synovial sarcomas was recently demonstrated in a clinical trial at the NCI. To investigate the role of NY-ESO-1 immunohistochemistry in patient selection and gain better insight into the incidence of NY-ESO-1 expression in synovial sarcomas and other mesenchymal tumors, we evaluated NY-ESO-1 expression by immunohistochemistry in 417 tumors. This collection of samples included: 50 SS18/SSX1/2 fusion positive synovial sarcomas, 155 gastrointestinal stromal tumors (GIST), 135 other spindle cell sarcomas as well as 77 other sarcomas (chondrosarcoma, osteosarcoma, dedifferentiated liposarcoma, alveolar soft part sarcoma, rhabdomyosarcoma, angiosarcoma, malignant mesothelioma, and Ewing's sarcoma). We report that 76% of synovial sarcomas expressed NY-ESO-1 in a strong and diffuse pattern (2-3+, >50-70% of tumor cells). In contrast, only rare cases of other spindle cell mesenchymal tumor expressed NY-ESO-1 (GIST (2/155), malignant peripheral nerve sheath tumors (1/34), and dermatofibrosarcoma protuberans (2/20)). Individual cases of other sarcomas (angiosarcoma, malignant mesothelioma, chondrosarcoma, osteosarcoma, dedifferentiated liposarcoma, alveolar soft part sarcoma, and Ewing's sarcoma) were positive for NY-ESO-1. However, no positive cases were identified amongst our cohort of leiomyosarcomas (0/24), hemangiopericytoma/solitary fibrous tumors (0/40), and cellular schwannomas (0/17). In summary, we find that NY-ESO-1 is strongly and diffusely expressed in a majority of synovial sarcomas, but only rarely in other mesenchymal lesions. Beyond its role in patient selection for targeted therapy, immunohistochemistry for NY-ESO-1 may be diagnostically useful for the distinction of synovial sarcoma from other spindle cell neoplasms.
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PMID:NY-ESO-1 expression in synovial sarcoma and other mesenchymal tumors: significance for NY-ESO-1-based targeted therapy and differential diagnosis. 2238 61

The management of hepatic metastatic disease from solid tumors in adults has been extensively described and resection of metastatic liver lesions from colorectal adenocarcinoma, renal adenocarcinoma, breast cancer, testicular cancer, and neuroendocrine tumors (NET) have demonstrated therapeutic benefits in select patients. However, there are few reports in the literature on the management of hepatic metastatic disease in the pediatric and adolescent populations and the effectiveness of hepatic metastasectomy. This may be due to the much lower incidence of pediatric malignancies and the higher chemosensitivity of childhood tumors which make hepatic metastasectomy less likely to be required. We review liver involvement with metastatic disease from the main pediatric solid tumors, including neuroblastoma and Wilms tumor focusing on the management and treatment options. We also review other solid malignant tumors which may have liver metastases including germ cell tumors, gastrointestinal stromal tumors, osteosarcoma, desmoplastic small round cell tumors and NET. However, these histological subtypes are so rare in the pediatric and adolescent populations that the exact incidence and best management of hepatic metastatic disease are unknown and can only be extrapolated from adult series.
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PMID:Hepatic metastatic disease in pediatric and adolescent solid tumors. 2620 62