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)

158 cases of the Cooperative Ewing's Sarcoma Trials (CESS 81/86), which have been documented at the Pediatric Tumor Registry, Kiel, were studied by conventional light microscopy and immunohistochemistry. There were 77 cases of typical Ewing's sarcoma with 70 cases being located in the skeleton and 7 in soft tissues. Of the 14 cases of atypical Ewing's sarcoma 7 cases each were localized in bone and in soft tissue, respectively. In contrast to typical Ewing's sarcoma, cells of atypical Ewing's sarcoma were larger and displayed more heterochromatin. Both, typical and atypical Ewing's sarcoma reacted positively for vimentin. Other stains were negative, notably the neuron specific enolase (NSE). In 55 cases a diagnosis of malignant peripheral neuroectodermal tumor (MPNT) was made. Histologically most of these tumors resembled atypical Ewing's sarcoma. By immunohistochemistry positive reactions were found for NSE, vimentin, protein S-100, neurofilaments and glial fibrillary acidic protein. In 3 cases a diagnosis of small cell osteosarcoma was made. There were 2 cases of undifferentiated sarcoma of bone, 2 cases of soft tissue sarcoma of undetermined histogenesis and 2 cases of rhabdomyosarcoma. Of the 4 tumors which could be investigated for response to polychemotherapy, 1 each corresponded to grade II and III, respectively, and 2 to grade IV according to the classification of histologic grade of regression established by Salzer-Kuntschik et al. (1983).
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PMID:[Cooperative Ewing's Sarcoma Studies 81/86: pathologico-anatomic and immunohistochemical findings and differential diagnosis of Ewing sarcoma]. 306 61

Small round cell tumors (SRCTs) of the bone make up a family of primary bone sarcomas with morphologically, biologically, and clinically specific features. Among them, Ewing's sarcoma (ES) is the most common entity, but several varieties such as atypical ES, large cell ES, and ES with neuroectodermal differentiation (peripheral primitive neuroectodermal tumor of the bone or neuroepithelioma of the bone) have been identified recently. Histology and electron microscopy together with the variable expression of several epitopes (as shown by immunohistochemistry, mainly HBA/71 [Mic2 antigen]) provide the basis for characterizing the group within the context of neuroectodermal-derived neoplasms. A number of other ES-like tumors with small round cells, mimicking those previously described, have been characterized; Askin's tumor of the thoracopulmonary region will be considered as an ES similar to those already described, but within a particularly anatomic location. On the other hand, the presence of an endothelial appearance within a poorly differentiated neoplasm may be present in some ES-like SRCTs (atypical ES with endothelial features). The differential diagnosis with other sarcomas defined by small round to spindle cell contours might prove difficult. Particular attention must be paid to small cell osteosarcoma and mesenchymal chondrosarcoma. Likewise, "primitive sarcoma of bone" is considered in this study because it is a very rare neoplasm differing from the formerly discussed types; its pluripotentiality provides this tumor a blastemic character and a multiphenotypic expression. Malignant non-Hodgkin's lymphoma is an unusual presentation when primary to the bone, previous to any other anatomic location. Several subtypes have been considered within a histology that encompasses that seen in lymph nodes.
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PMID:Histology, immunohistochemistry, and electron microscopy of small round cell tumors of bone. 887 7

The antitumor activity of topotecan administered as a 72-h continuous i.v. infusion was evaluated in children with refractory neuroblastoma and sarcomas of soft tissue and bone. We also attempted to increase the dose intensity of topotecan by including an intrapatient dose escalation in the trial design. Ninety-three children (85 eligible and evaluable for response) with recurrent or refractory neuroblastoma, osteosarcoma, Ewing's sarcoma/peripheral neuroectodermal tumor, rhabdomyosarcoma, or other soft-tissue sarcomas received topotecan administered as a 72-h i.v. infusion every 21 days. The initial dose was 1.0 mg/m2/day, with subsequent intrapatient dose escalation to 1.3 mg/m2/day for those patients who did not experience dose-limiting toxicity after their first cycle of topotecan. There was one complete response in a patient with neuroblastoma (n = 26) and one partial response in a patient with Ewing's sarcoma/peripheral neuroectodermal tumor (n = 25). No complete or partial responses were observed in 17 patients with osteosarcoma, 15 patients with rhabdomyosarcoma, or 2 patients with other soft-tissue sarcomas; however, 8 patients had prolonged (15-48 weeks) stable disease while receiving topotecan. Topotecan was well tolerated. The most commonly observed toxicities were myelosuppression (dose-limiting) and nausea and vomiting. Intrapatient dose escalations were performed in 68% of the patients who received more than one cycle of topotecan, and 1.3 mg/m2/day was tolerated by 79% of the patients who received the higher dose and were evaluable for hematological toxicity. In conclusion, topotecan administered as a 72-h continuous infusion every 21 days is inactive (objective response rate, < 20%) in children with refractory or recurrent neuroblastoma and sarcomas of soft tissue or bone.
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PMID:Phase II trial of topotecan administered as 72-hour continuous infusion in children with refractory solid tumors: a collaborative Pediatric Branch, National Cancer Institute, and Children's Cancer Group Study. 951 23

Primary lymphomas of bone or skeletal muscle are rare entities. The most frequent among these diseases are primary non-Hodgkin's lymphomas of bone. They account for 3-5% of all bone tumors and 5% of all primary extranodal non-Hodgkin's lymphomas. Primary manifestations of Hodgkin's disease in bone or skeletal muscle are rarities. Primary non-Hodgkin's lymphomas of skeletal muscle are rarities as well. Primary non-Hodgkin's lymphomas of bone can be found in any patient age. A preference exists for the 3.-6. decade of life. The radiographic appearance of these entities resembles other aggressive bone tumors. Their differential diagnosis includes -- depending on the patient's age -- Ewing's sarcoma,malignant fibrous histiocytoma,metastases of small cell tumors and osteomyelitis.Further differential diagnoses are the peripheral primitive neuroectodermal tumor (PNET), osteosarcoma, eosinophilic granuloma and fibrosarcoma. Treatment of primary non-Hodgkin's lymphomas uses combinations of chemotherapy and radiation therapy. Operative treatment is reserved for the treatment of complications. The prognosis of primary non-Hodgkin's lymphomas is reflected by 10-year-survival-rates without recurrence of more than 80% in unifocal manifestations.
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PMID:[Musculoskeletal lymphomas]. 1248 52

The current study disusses a new approach to the group of small round cell tumors (SRCTs) independently of their primary anatomical location. We perform this analysis supported mainly by morphological means and particularly with the help of immunohistochemistry and electron microscopy; the last of which continues to play a decisive role in their differential diagnosis. The microscopical similarity of many of these tumors often makes the diagnosis in routine histology extremely difficult, due to the varying degree of heterogeneity present, and may have important therapeutic and prognostic implications. Thus a correct final diagnosis is mandatory for the clinic. Within the group of tumors that express a dominant or occasional small round cell pattern "SRCT" (neoplasms of the Central Nervous System excluded) are included: Ewing's sarcoma and peripheral neuroectodermal tumor (Es/pPNET) comprising its varieties, neuroblastoma, desmoplastic small round cell tumor, rhabdomyosarcoma, alveolar, solid and embryonal, small cell osteosarcoma, chondrosarcoma, myxoid and mesenchymal, round cell and myxoid liposarcoma, synovial sarcoma (monophasic undiffentiated), primitive malignant peripheral nerve sheath tumor (malignant small cell schwannoma), malignant non-Hogdkin lymphoma, Merkel cell tumor of the skin (small cell carcinoma including neuroendocrine carcinoma). This study discusses in each case not only the histology, supported by immunohistochemistry, but also the main ultrastructural characteristics. We are conscious that in some cases further cytogenetic or molecular biology support may be necessary, when considering the limits of morphology today. Thus, short references on molecular genetics, complementing the structural findings, are given.
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PMID:Electron microscopy and other ancillary techniques in the diagnosis of small round cell tumors. 1269 73

The diagnosis of a bone tumor is often an arduous task, even for the most experienced orthopedic pathologist. As a starting point, the classification of bone tumors is based on a histogenetic perspective encompassing the type of matrix produced (or not produced) by the tumor. In general, the surgical pathology report should include data pertinent to the treatment and prognostication of an individual patient, and the report should be delivered to the clinician in a clear, concise fashion. Reporting of most bone tumors is similar and includes such information as the type of surgery done, anatomic site, histological type and grade of the tumor (if applicable), and the adequacy of surgical margins. Special emphasis is needed for those tumors with distinct and well-established prognostic and therapeutic features such as osteosarcoma and Ewing's sarcoma/peripheral neuroectodermal tumor. Our recommendation emphasizes a standardized protocol for these sarcomas, especially in light of evidence that postchemotherapeutic tumor necrosis is of prognostic significance. It is also important to note that radiographic imaging plays a very important, often critical, role in allowing the pathologist the opportunity to reach the best final diagnosis. This is especially true when a malignant interpretation is contemplated and in subtyping lesions. We recommend close collaboration between musculoskeletal radiologists, clinicians, and pathologists when dealing with complicated neoplasms of bone.
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PMID:Recommendations for the reporting of bone tumors. Association of Directors of Anatomic and Surgical Pathology. 1549 83

Sinonasal tract neoplasms composed of light microscopically seemingly "undifferentiated" small round cells often generate considerable diagnostic difficulty. Although the careful review of H&E-stained sections remains of critical and central importance in this evaluation, the recent improvements in the immunohistochemical diagnostic armamentarium and molecular diagnostic techniques applicable to paraffin-embedded tissue samples may add diagnostically valuable information. Accordingly, this review will discuss the differential diagnosis of undifferentiated small blue cell tumors of the sinonasal tract based on the light microscopic and clinical features and, as needed, the results of these ancillary studies. Tumors discussed include olfactory neuroblastoma, sinonasal undifferentiated carcinoma, small cell undifferentiated (neuroendocrine) carcinoma, undifferentiated (lymphoepithelioma-like) carcinoma, malignant melanoma, pituitary adenoma, Ewing sarcoma/peripheral neuroectodermal tumor, rhabdomyosarcoma, mesenchymal chondrosarcoma, small cell osteosarcoma, synovial sarcoma, extranodal natural killer/T-cell lymphoma, nasal type, and extramedullary plasmacytoma.
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PMID:"Undifferentiated" small round cell tumors of the sinonasal tract: differential diagnosis update. 1646 21

Although rare, sarcomas represent a source of significant morbidity and mortality with nearly one reported death for every two new diagnoses. The detection and surveillance of circulating tumor cells (or CTCs) has been found to have significant clinical utility in epithelial malignancies, such as carcinoma of the colon, breast and prostate. Here, we summarize what is known regarding CTCs in sarcomas. Although still in its relative infancy, the detection of CTCs in sarcoma patients may help to diagnose and predict recurrence or metastasis as well as improve the overall management of sarcoma patients. CTCs are most often detected via reverse transcriptase polymerase chain reaction or antibody-based detection of cell surface proteins, including flow cytometry. Samples may be obtained from either peripheral blood or bone marrow. CTC detection in translocation sarcomas is perhaps most promising, as a recurrent abnormal gene fusion product can be detected in involved individuals but not in the normal patient. Studies in Ewing's sarcoma/peripheral neuroectodermal tumor, synovial sarcoma and alveolar soft part sarcoma have confirmed the feasibility of this approach. Other investigators have turned toward detection of more universal markers of sarcomas, such as the pan-mesenchymal marker Vimentin. In the case of osteosarcoma, more specific markers of osteogenic differentiation (Type I Collagen) have been utilized. In summary, although in its relative nascency, the use of CTC detection for the management of sarcoma patients shows initial promise.
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PMID:Circulating tumor cells in sarcomas: a brief review. 2549 Nov 43

Ewing Sarcoma is a malignant small round blue cell tumor most commonly found in bones and soft tissues of the axial skeleton and extremities. The Ewing family of tumors, including peripheral neuroectodermal tumor, represent the second most common malignancy in the pediatric population and second most common primary bone tumor after osteosarcoma. In a majority of Ewing Sarcoma cases, there is a translocation between chromosomes 11 and 22. Extraskeletal Ewing Sarcoma of the stomach is exceptionally rare, with only a handful of case reports. Here we report a case of primary Ewing Sarcoma of the stomach found initially as a filling defect in the stomach on technetium-99m pertechnetate scintigraphy to evaluate for gastrointestinal bleeding.
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PMID:Gastric Ewing Sarcoma identified on a Meckel's scan. 3257 40