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)

48 cases of osteosarcoma, including 18 cases of osteoblastic type, 16 of fibroblastic type and 14 of chondroblastic type, were studied in accordance with Dahlin's classification. All specimens were stained with 11 specific markers using immunohistochemical ABC methods. Positive immunostaining for BMP was found in all of these cases. The results showed 48 vimentin positive cases, 46 actin positive cases, 5 keratin positive cases and 4 desmin positive cases. Neither cytokeratin nor EMA was identified in any of the cases. Positive reactions to S-100 protein, collagen type IV, UEA-1 and factor XIII were observed in 26, 20, 36 and 13 of the cases respectively. The results of this study confirm that osteosarcoma possesses the unique multidirectional differentiation potential toward osteoblastic, chondroblastic, myofibroblastic, fibrohistiocytic and epithelial cells, and also indicate that immunophenotypic analysis is useful for the diagnosis, differential diagnosis and classification of the tumor cells of osteosarcoma.
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PMID:[Osteosarcoma cell immunophenotype and heterogeneity]. 816 96

Two cases of pleomorphic sarcoma with malignant fibrous histiocytoma phenotype that recurred following therapy with a distinctly different, more mature histologic appearance and immunohistochemical profile are reported. The first case recurred predominantly as extraosseous osteosarcoma at the same site 17 months after wide local excision. The second case recurred as widespread pulmonary, mediastinal, and hepatic metastatic disease 5 years after wide local excision and adjuvant local radiotherapy and chemotherapy with doxorubicin hydrochloride (Adriamycin) and cyclophosphamide (Cytoxan). Fine-needle aspiration of the pulmonary metastatic lesions showed predominantly spindle cells without any large, pleomorphic giant cells typical of malignant fibrous histiocytoma. The patient was treated by radiotherapy to the lung and mediastinum and by chemotherapy with ifosfamide and mesna. Biopsy of a metastatic scrotal skin nodule 9 months later showed a malignant spindle cell lesion with the histologic appearance and immunohistochemical phenotype of leiomyosarcoma. Retrospective immunohistochemical evaluation of the primary tumor showed focal desmin expression, suggesting focal leiomyosarcomatous differentiation. However, the large proportion of the primary tumor had the phenotype of malignant fibrous histiocytoma. These two cases illustrate an unusual finding of "differentiation" rather than "dedifferentiation" in a recurrent sarcoma. The transformation to osteosarcoma and differentiated leiomyosarcoma demonstrates the potential for phenotypic changes in soft-tissue sarcomas and suggests that the malignant fibrous histiocytoma phenotype and more-differentiated sarcomas such as extraosseous osteosarcoma or leiomyosarcoma are related in a common pathway in differentiation from a primitive mesenchymal stem cell.
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PMID:Malignant fibrous histiocytoma phenotype in pleomorphic sarcoma differentiation in recurrent disease. 784 56

We report a case of primary embryonal rhabdomyosarcoma of long bone, presenting as a lytic destructive bone tumor in the right femoral diaphysis of a 7-year-old girl. To our knowledge, this is only the third report of this entity. The neoplasm was a pure embryonal rhabdomyosarcoma with numerous rhabdomyoblasts. Immunohistochemistry confirmed the diagnosis: The cells were reactive with antibodies directed against desmin, muscle-specific actin, and myoglobin. No other neoplastic mesenchymal component was present within the tumor. Although rare, primary rhabdomyosarcoma, along with Ewing's tumor and osteosarcoma, should be considered in the differential diagnosis of malignant bone tumors in childhood.
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PMID:Primary embryonal rhabdomyosarcoma of long bone. Case report and review of the literature. 855 14

Twenty-eight epithelial and 22 nonepithelial feline tumors were studied immunohistochemically. Epithelial tumors were 10 squamous cell carcinomas, two basal cell tumors, two sebaceous gland carcinomas, three apocrine gland carcinomas, three thyroid papillary carcinomas, one thyroid solid carcinoma, one renal clear cell carcinoma, one renal papillary carcinoma, one endometrial carcinoma, and four lung bronchioloalveolar carcinomas. Nonepithelial tumors were 10 fibrosarcomas, one liposarcoma, one leiomyosarcoma, one rhabdomyosarcoma, one hemangiosarcoma, two mast cell tumors, one osteosarcoma, three melanomas, and two lymphomas. Commercially available antibodies directed against high- and low-molecular-weight keratins (keratin, RCK-102, NCL-5D3), vimentin, desmin, glial fibrillary acidic protein (GFAP), and neurofilament intermediate filament (IF) proteins were used in the avidin-biotin-peroxidase complex technique on formalin-fixed, paraffin-embedded tumor tissue samples. All epithelial tumors except the endometrial carcinoma expressed some type of keratin protein. Squamous cell carcinomas expressed high-molecular-weight keratins exclusively. Coexpression of high- and low-molecular-weight keratins was observed in one basal cell tumor, sebaceous and apocrine adenocarcinomas, and thyroid, renal, and lung carcinomas. In addition to keratins, vimentin immunoreactivity was found in all basal cell tumors, all sebaceous gland, thyroid papillary, renal, and lung adenocarcinomas, and one of the apocrine gland adenocarcinomas. Immunoreactivity with GFAP antibody was found in one basal cell tumor and one sebaceous gland adenocarcinoma. The endometrial carcinoma did not react with any of the antibodies applied. Nonepithelial tumors analyzed expressed either vimentin (fibrosarcomas, liposarcoma, haemangiosarcoma, mast cell tumors, osteosarcomas, melanomas) or vimentin and desmin (leiomyosarcoma, rhabdomyosarcoma, one fibrosarcoma) IF proteins exclusively. Lymphomas did not react with any of the antibodies employed. These findings indicate that IF proteins antibodies can be included in diagnostic panels of antibodies for immunocharacterization of feline tumors. In addition, they can be used as a basis for the diagnoses of poorly differentiated or undifferentiated feline neoplasms.
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PMID:Immunohistochemical distribution pattern of intermediate filament proteins in 50 feline neoplasms. 859 5

A follow-up investigation of 25 cases of extraskeletal osteosarcomas diagnosed at the Center for Bone and Soft Tissue Tumors, Aarhus University Hospital, Denmark, in the period from 1970-1995 was undertaken. The immunohistochemical profile of these tumors was evaluated using a panel of 10 antibodies, and the value of alkaline phosphatase staining in differential diagnostic situations also was considered. The study revealed that this tumor is high-grade malignant and affects adults (median age, 67 years; range, 35-82 years) at diagnosis. The thigh (52%) was the most common tumor location. Seven tumors were superficial, whereas the remaining 18 were intramuscular. Two patients with superficial tumors previously received radiation to the area. Local recurrences developed in 9 (36%) patients and distant metastases developed in the lungs in 15 (60%) patients as the most common site. Median survival time was 24 months, and the cause-specific survival rate at 5 years was less than 25%. Thirteen (52%) intramuscularly located extraskeletal osteosarcomas were of the fibroblastic subtype, often with sparse amounts of osteoid. They could be separated from malignant fibrous histiocytoma on the basis of a strongly positive alkaline phosphatase reaction. Immunohistochemistry did not reveal characteristic features because positivity for vimentin, occasional positivity for desmin, actin, S-100, epithelial membrane antigen, cytokeratin, and p-53 may be observed in many other pleomorphic sarcomas. Various histopathologic factors, such as tumor size, tumor depth, histopathologic subtype, malignancy grade (IIIA versus IIIB), MIB-1, and p53 reactivity were analyzed in relation to clinical course. Only MIB proliferation was correlated to prognosis, with significantly longer survival in patients with tumors with MIB-1 values less than 24%. Our study has shown extraskeletal osteosarcoma to behave in a highly aggressive fashion. Alkaline phosphatase staining compared with immunohistochemistry proved to be superior in the differentiation from other pleomorphic sarcomas.
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PMID:Extraskeletal osteosarcomas: a clinicopathologic study of 25 cases. 959 29

A 14-year-old spayed female shepherd/collie crossbred dog had a 6 x 4-cm mass excised from below the right ear. The partially encapsulated, neoplastic mass had a necrotic core and was embedded in the mandibular salivary gland. Histologically, the mass was composed of numerous multinucleate giant cells and large, oval, pleomorphic cells that produced osteoid. Neoplastic cells were immunoreactive for vimentin and actin but not for keratin, desmin, or S-100 protein. At postmortem examination 1 month later, an 11-cm spherical mass had recurred at the surgical site, and there were metastatic nodules in the lungs, ipsilateral mandibular lymph nodes, and kidney. The tumor was diagnosed as an osteosarcoma of the mandibular salivary gland with pulmonary, lymphatic, and renal metastasis. In addition, a 17-year survey of canine salivary-gland neoplasms revealed that most were adenocarcinomas or carcinomas.
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PMID:Extraskeletal osteosarcoma of the mandibular salivary gland in a dog. 992 59

A rare case of rapidly growing osteosarcoma that developed in the uterine corpus of a 62-year-old woman is presented. The tumor occupied almost the entire pelvic cavity and extended into the abdominal cavity, with marked involvement of the intestines. Histopathologically, the tumor was composed of an osteoblastic component, accompanied by conspicuous bone formation, and a fibroblastic component. The tumor cells were positive for vimentin and osteocalcin, as well as desmin, alpha-smooth muscle actin and muscle-specific actin, but negative for h-caldesmon. The results indicated myofibroblastic differentiation in a part of the tumor. A review of 14 reported cases and our case of uterine osteosarcoma revealed that this tumor has a biologically aggressive nature, although its histopathological and immunohistochemical features are similar to those of osteosarcomas in soft tissue and bone. As the prognosis of patients with this tumor is poor, it is of importance to differentiate this tumor from other types of tumors arising from the uterine corpus.
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PMID:Primary osteosarcoma of the uterine corpus: case report and review of the literature. 1194 Feb 22

Rhabdomyosarcomas (RMSs) are classified into embryonal (ERMS), alveolar (ARMS), and pleomorphic (PRMS) subtypes. ERMS, including botryoid variants, typically occurs in young children, ARMS typically occurs in older children and young adults, and PRMS occurs in older adults. Although ARMSs show thin fibrous bands separating nests of cells, abundant extracellular matrix production is rare in RMS. In the course of reviewing hyalinizing sarcomas we discovered a distinctive RMS in adults that closely mimicked osteosarcoma or chondrosarcoma because of the extensive matrix production. Four RMSs with hyalinized matrix were retrieved from our files. These cases were evaluated with respect to patient age and sex, tumor site and size, growth pattern, nuclear grade, cellularity, mitotic figures/20 high power fields, vascular invasion, necrosis, the presence of rhabdomyoblasts, multinucleated cells, and alveolar growth pattern. Immunohistochemistry for desmin, myogenin, MyoD1, actin, cytokeratin, S-100 protein, collagen II, and CD99 was performed. Reverse transcriptase polymerase chain reaction for the ARMS-associated PAX3/FKHR and PAX7/PKHF was also performed on three cases. The cases involved the forearm, hand, orbit, and nasopharynx of a 40-year-old woman, a 50-year-old man, an 18-year-old man, and a 21-year-old man, respectively. The tumors ranged from 3.7 to 8 cm and consisted of lobules and infiltrating cords of small round malignant cells embedded in a densely hyalinized matrix having both a chondroid and osteoid-like appearance. No definite lacunae or matrix calcification was present. An alveolar pattern was only present focally, and tumor giant cells were not present. One case had a single focus of rhabdomyoblastic differentiation with strap cells. Mitotic activity was >20 mitotic figures/20 high power fields in three of four cases. Immunohistochemically, one case strongly expressed desmin, whereas three cases expressed it focally, with a dot-like pattern. Myogenin was only focally positive, but MyoD1 was present in nearly every cell of each case. Two cases expressed actin and one expressed CD99. No case expressed cytokeratin, S-100 protein, or collagen II. Only one case contained adequate RNA for reverse transcriptase polymerase chain reaction, and this case was negative for the ARMS-associated gene fusions. Follow-up showed one patient to be dead of metastatic disease at 60 months despite intensive therapy, another patient to be disease free at 26 months, and the third patient to be disease free at 5 months. The fourth case is recent. These cases are a distinctive-appearing rhabdomyosarcoma easily mistaken for variants of chondrosarcoma, osteosarcoma, or even sclerosing epithelioid fibrosarcoma because of their hyalinizing appearance compounded by their typically focal and dot-like desmin expression. These four cases are essentially identical to the three unusual RMSs recently reported by Mentzel and Katenkamp as "sclerosing, pseudovascular rhabdomyosarcoma in adults." Although the focal alveolar architecture and the primitive cytologic appearance of these hyalinizing RMS suggest a relationship with ARMS, the presence of abundant strap cells in one case, the predominant expression of MyoD1 rather than myogenin, and the absence of ARMS-associated fusions genes point more strongly toward a variant of ERMS. However, the late adult age in two cases is unusual for both EMRS and ARMS, suggesting that sclerosing RMS may prove to be a distinct subtype of RMS. Study of additional cases will be necessary to more fully elucidate its place among RMS and its prognostic significance.
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PMID:Sclerosing rhabdomyosarcoma in adults: report of four cases of a hyalinizing, matrix-rich variant of rhabdomyosarcoma that may be confused with osteosarcoma, chondrosarcoma, or angiosarcoma. 1221 74

The expression of alpha smooth muscle actin, muscle specific actin, desmin, h-caldesmon, and calponin was studied immunohistochemically in the following soft tissue and bone tumours and tumour-like lesions: muscle fibromatosis, inflammatory pseudotumours, chondroblastoma, enchondroma, chondrosarcoma, fibrous dysplasia, ossifying myositis, osteoblastoma, convential osteosarcoma, leiomyoma and leiomyosarcoma. Tumours and tumour-like lesions with myofibroblastic cells, osteoblasts and chondroblasts frequently exhibited intensive immunoreactivity for the muscle markers, and therefore, some of them may occasionally be confused with leiomyoma and leiomyosarcoma. Calponin does not help to differentiate various mesenchymal tumours expressing muscle markers, because it also stains intensively myofibroblasts, osteoblasts and chondroblasts. We confirmed that h-caldesmon was expressed intensely in leiomyomas and leiomyosarcomas, and never in the other tumours examined, with the exception of three chondroblastomas. The results have shown that h-caldesmon is a rather specific and sensitive marker for smooth muscle tumours, but it can also stain some actin positive myochondroblasts. It is possible that the positivity of h-caldesmon in some chondroblastomas is due to their complete myogenic transdifferentiation, and so we use the term myochondroblasts and myochondrocytes for designation of such S-100 protein, actin, and h-caldesmon positive cells.
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PMID:[Immunohistochemical differentiation of leiomyocellular tumors and tumors with myogenic differentiation]. 1287 3

Oncogenic osteomalacia (OO) is a rare paraneoplastic syndrome of osteomalacia due to phosphate wasting. The phosphaturic mesenchymal tumor (mixed connective tissue variant) (PMTMCT) is an extremely rare, distinctive tumor that is frequently associated with OO. Despite its association with OO, many PMTMCTs go unrecognized because they are erroneously diagnosed as other mesenchymal tumors. Expression of fibroblast growth factor-23 (FGF-23), a recently described protein putatively implicated in renal tubular phosphate loss, has been shown in a small number of mesenchymal tumors with known OO. The clinicopathological features of 32 mesenchymal tumors either with known OO (29) or with features suggestive of PMTMCT (3) were studied. Immunohistochemistry for cytokeratin, S-100, actin, desmin, CD34, and FGF-23 was performed. The patients (13 male, 19 female) ranged from 9 to 80 years in age (median 53 years). A long history of OO was common. The cases had been originally diagnosed as PMTMCT (15), hemangiopericytoma (HPC) (3), osteosarcoma (3), giant cell tumor (2), and other (9). The tumors occurred in a variety of soft tissue (21) and bone sites (11) and ranged from 1.7 to 14 cm. Twenty-four cases were classic PMTMCT with low cellularity, myxoid change, bland spindled cells, distinctive "grungy" calcified matrix, fat, HPC-like vessels, microcysts, hemorrhage, osteoclasts, and an incomplete rim of membranous ossification. Four of these benign-appearing PMTMCTs contained osteoid-like matrix. Three other PMTMCTs were hypercellular and cytologically atypical and were considered malignant. The 3 cases without known OO were histologically identical to the typical PMTMCT. Four cases did not resemble PMTMCT: 2 sinonasal HPC, 1 conventional HPC, and 1 sclerosing osteosarcoma. Three cases expressed actin; all other markers were negative. Expression of FGF-23 was seen in 17 of 21 cases by immunohistochemistry and in 2 of 2 cases by RT-PCR. Follow-up (25 cases, 6-348 months) indicated the following: 21 alive with no evidence of disease and with normal serum chemistry, 4 alive with disease (1 malignant PMTMCT with lung metastases). We conclude that most cases of mesenchymal tumor-associated OO, both in the present series and in the reported literature, are due to PMTMCT. Improved recognition of their histologic spectrum, including the presence of bone or osteoid-like matrix in otherwise typical cases and the existence of malignant forms, should allow distinction from other mesenchymal tumors. Recognition of PMTMCT is critical, as complete resection cures intractable OO. Immunohistochemistry and RT-PCR for FGF-23 confirm the role of this protein in PMTMCT-associated OO.
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PMID:Most osteomalacia-associated mesenchymal tumors are a single histopathologic entity: an analysis of 32 cases and a comprehensive review of the literature. 2021 75


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