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)

Primary tumors of the spine are relatively infrequent lesions compared with metastatic disease, multiple myeloma, and lymphoma. However, when a solitary lesion of the spine occurs, these neoplasms represent an important group of entities for diagnostic consideration. A wide variety of benign neoplasms can involve the spine, including osteoid osteoma, osteoblastoma, aneurysmal bone cyst, giant cell tumor, enostosis, and osteochondroma. Common primary nonlymphoproliferative malignant neoplasms of the spine include chordoma, chondrosarcoma, Ewing sarcoma or primitive neuroectodermal tumor, and osteosarcoma. The imaging features of these lesions of the spine are often characteristic. These changes include a small sclerotic focus with irregular thorny margins in the vertebral body (enostosis), a small radiolucent nidus with central calcification in the posterior elements of the vertebral body (osteoid osteoma), a large expansile lesion with multiple fluid-fluid levels (aneurysmal bone cyst), and an aggressive mineralized mass (chondroid or osteoid) with osseous and soft-tissue involvement (chondrosarcoma or osteosarcoma). Radiologists should be aware of the appearance of these unusual neoplasms in order to provide a complete differential diagnosis and to guide clinical colleagues in patient treatment.
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PMID:From the archives of the AFIP. Primary tumors of the spine: radiologic pathologic correlation. 888 95

The risk of second malignancy after retinoblastoma is reported to be as high as 20% at 10 years after initial diagnosis. This incidence may be an overestimate because of difficulties in distinguishing a second malignancy from recurrent tumor. We encountered a patient with bilateral retinoblastoma who developed a temporal mass 3.5 years after initial treatment for what had first been diagnosed as rhabdomyosarcoma; further study suggested that it was recurrent retinoblastoma manifesting as primitive neuroectodermal tumor (PNET) with multilineage differentiation. Chromosome 13 abnormalities were compatible with either rhabdomyosarcoma or recurrent retinoblastoma. To determine how often second malignancies in retinoblastoma patients may be confused with recurrent primary tumor, we reviewed our experience at Children's Hospital of Pittsburgh. Of 43 retinoblastoma patients seen between 1951 and 1992, presumed second malignancies were documented in four, including the current case. Of the three other second tumors, one had both neural and skeletal muscle differentiation; another was diagnosed as rhabdomyosarcoma unclassifiable as embryonal or alveolar; the last was an osteosarcoma. Only the osteosarcoma was clearly a second neoplasm; two and perhaps three of the other cases may be recurrent retinoblastoma. The distinction between second malignancy and recurrent retinoblastoma may be difficult but is worth determining, because treatment may differ, depending on the correct designation.
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PMID:Malignancy after retinoblastoma: secondary cancer or recurrence? 902 3

Trk receptors have been identified by immunohistochemical methods in primitive neuroectodermal tumor (PNET)/Ewing's sarcoma (ES). However, the presence of different members of the Trk family of receptors in PNET/ES has not been specified. We have examined whether Trk A, B, and C receptors are specifically expressed in ES both with and without features of neural differentiation. Ten ES tumors (five primary tumors of bone and five extraosseous tumors transplanted into nude mice) were investigated for expression of Trk receptors by immunohistochemistry and reverse transcription-polymerase chain reaction. One primary ES and the five grafted ES tumors exhibited signs of neural differentiation; the remaining four primaries were undifferentiated ES. Other tumor types were analyzed as controls; they included three neuroblastomas (NB), two lymphomas, and single cases of pheochromocytoma (PHEO), schwannoma (SCHW), osteosarcoma, and carcinoma of breast, colon, and kidney. Trk receptors were detected in paraffin-embedded tumor tissue sections by means of a pan-Trk polyclonal antibody raised against the 14 carboxy-terminal residues of gp140trk, and trk A, B, and C transcripts were specifically detected by polymerase chain reaction-based amplification on cDNAs derived from tumor RNA with MuLV reverse transcriptase. Reactivity to the pan-Trk antibody was exhibited by six ES tumors, the three NBs, and the single PHEO and SCHW cases; immunoreactivity was restricted to differentiated tumors, in the case of ES. Tumor types positive for immunostaining were also distinguished by containing transcripts of TRK genes. However, the trk A, B, and C expression pattern of ES differed from that of NBs, PHEO, and SCHW. Transcripts of trk A, B, and C were detected in seven, four, and one case of ES, respectively, and in five, two, and five cases of NB, PHEO, and SCHW, respectively. Interestingly, all differentiated ES tumors contained trk A transcripts. Tumors of neuroectodermal phenotype and/or derivation were thus characterized by a distinct consensus expression pattern: trk A+/B-/C+ for differentiated ES and trk A+/B-/C+ for NB-PHEO-SCHW. These results indicate that the TRK gene family is frequently activated in ES; they also suggest that Trk A receptor is a feature of ES with neural differentiation, whereas Trk B and C receptors seem to be present in undifferentiated ES.
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PMID:Activation of TRK genes in Ewing's sarcoma. Trk A receptor expression linked to neural differentiation. 902 32

Except for clear cell carcinomas that metastasize to bone, with renal cell carcinoma being the principal representative of that group, clear cell osseous neoplasms are rare. The only distinct nosologic entity in this category that is primary in the bone is the clear cell chondrosarcoma (CCCS). This lesion, which is most often seen in the proximal femur or humerus, affects males more often than females and has a peak incidence during the third and fourth decades of life. Radiologic images of CCCS show a well-circumscribed, often calcified lytic lesion that may expand the bone, but only uncommonly breaches the cortex. Clear cell elements in CCCS are accompanied by "conventional" foci of chondrosarcoma in less than 50% of cases; noncartilaginous "secondary features," including areas of osteogenesis, osteoclast-like giant cells, and zones resembling aneurysmal bone cyst or giant cell tumor of bone, may be apparent as well. CCCS is a relatively indolent malignancy; roughly 25% of patients experience local recurrences of their tumors or suffer metastasis, but tumor-related death is uncommon, particularly when the lesion has been completely resected en bloc. Sporadic examples of other tumors in bone also may be focally or entirely composed of clear cells. These include osteosarcoma, chondroblastoma, chordoma, adamantinoma, Ewing's sarcoma, and primitive neuroectodermal tumor. The last two of these lesions represent the most common primary clear cell bone tumors in children, whereas metastatic renal clear cell sarcoma is the most frequent metastatic pediatric tumor in this category.
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PMID:Clear cell tumors of bone. 938 27

From January 1990 to December 1995 we treated 35 patients (pts) with bone and soft tissue sarcoma with ifosfamide (IFM). Sixteen patients had measurable metastatic pulmonary lesion and 9 had primary lesion. Histology of the tumor included osteosarcoma in 13 pts, Ewing sarcoma in 5 pts, malignant fibrous histiocytoma of bone in 2 pts, synovial sarcoma in 7pts, primitive neuroectodermal tumor in 2 pts, and other in 7 pts. The IFM at the dose of 12-18g/m2 (mean 15. 4g/m2) for 5 to 8 days continuous infusion was administered to patients in each treatment course. The uroprotector, mesna, was also given concomitantly in 60-100% dose of IFM. Eighteen pts received one course of IFM treatment. Other pts received 2 to 8 courses of IFM treatment at three to four week intervals. The overall response rate was 40% (PR in 14 pts, NC in 18 pts, and PD in 3 pts). The response rate of 13 pts with osteosarcoma was 54% (PR in 7 pts) and 30% in 15 pts with soft tissue sarcoma (PR in 5 pts).
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PMID:[The effects of high-dose ifosfamide in the treatment of bone and soft tissue sarcomas]. 938 23

The purpose of this study was to provide an overview of the spectrum of pediatric chest masses, to present the results of cross-sectional imaging with CT and/or MRI, and to define diagnostic criteria to limit differential diagnosis. Seventy-eight children with thoracic mass lesions were retrospectively evaluated using CT (72 patients) and/or MR imaging (12 patients). All masses were evaluated for tissue characteristics (attenuation values or signal intensity, enhancement, and calcification) and were differentiated according to age, gender, location, and etiology. Twenty-eight of 38 (74 %) mediastinal masses were malignant (neuroblastoma, malignant lymphoma). Thirty of 38 (79 %) pulmonary masses were metastatic in origin, all with an already known primary tumor (osteosarcoma, Wilms tumor). With one exception, all remaining pulmonary lesions were benign. Seventeen of 21 (81 %) chest wall lesions were malignant (Ewing sarcoma, primitive neuroectodermal tumor). The majority of mediastinal and chest wall tumors in children is malignant. Lung lesions are usually benign, unless a known extrapulmonary tumor suggests pulmonary metastases. Cross-sectional imaging with CT and/or MRI allows narrowing of the differential diagnosis of pediatric chest masses substantially by defining the origin and tissue characteristics. Magnetic resonance imaging is preferred for posterior mediastinal lesions, whereas CT should be used for pulmonary lesions. For the residual locations both modalities are complementary.
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PMID:Cross-sectional imaging with CT and/or MRI of pediatric chest tumors. 968 16

The use of fine-needle aspiration biopsy (FNAB) in the initial evaluation of pediatric bone and soft tissue tumors is controversial, especially for those patients being considered for histiogenetic-specific therapeutic protocols, e.g., the Intergroup Rhabdomyosarcoma Study Group, the Pediatric Oncology Group. We retrospectively reviewed 33 consecutive FNAB specimens (28 primary tumors, 5 metastases) from 32 pediatric patients (< or = 19 yr of age), none of whom had a previously established tumor diagnosis. In one patient, FNAB of the primary tumor and a presumed axillary metastasis were obtained concomitantly. The cytomorphologic analysis included osteosarcoma, eight patients; rhabdomyosarcoma, five; neuroblastoma, five; Ewing's sarcoma/primitive neuroectodermal tumor, four; Langerhans' cell histiocytosis, three; and one each synovial sarcoma, undifferentiated sarcoma, infantile myofibromatosis, fibroma, chondroblastoma, chondromyxoid fibroma, and desmoplastic small round-cell tumor. Ancillary studies, e.g., immunocytochemical analysis, were used in 13 cases. Cytogenetic analysis helped to confirm one Ewing's sarcoma [t (11;22) (q24;q12)] and one synovial sarcoma [t(X;18) (p11;q11)]. With adequate FNAB specimens, a histogenetic-specific diagnosis was rendered in 27 (93%) of 29 cases, and all were correctly recognized as either benign or malignant. One case each of Langerhans' cell histiocytosis, chondroblastoma, and infantile myofibromatosis yielded unsatisfactory specimens. Fibroma and desmoplastic small round-cell tumor were initially misclassified as nodular fasciitis and rhabdomyosarcoma, respectively. Of 18 patients clinically eligible for histogenetic-specific therapy protocols, an accurate diagnosis was obtained in 17 patients. With a multidisciplinary approach and judicious use of ancillary studies, FNAB represents a highly accurate and cost-effective technique for the diagnosis of pediatric bone and soft tissue tumors, especially sarcomas, and should be considered as a viable diagnostic technique for pediatric therapeutic protocols.
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PMID:The role of fine-needle aspiration biopsy in the initial diagnosis of pediatric bone and soft tissue tumors: an institutional experience. 979 16

High-dose chemotherapy with autologous peripheral blood stem cell transplantation was administered to 10 patients with refractory bone and soft tissue sarcoma (2 patients with primitive neuroectodermal tumor, 4 patients with Ewing's sarcoma, 3 patients with synovial sarcoma and one patient with osteosarcoma). Busulfan 4 mg/kg x 4, melphalan 140 mg/m2 and thiotepa 200 mg/m2 x 3 were used in the high-dose chemotherapy. Complications related to the treatment were limited to one patient who developed hepatic veno-occlusive disease, no serious complications were seen in the other patients. Four patients died of their disease, one patient was alive with the disease and 5 patients were alive with no evidence of disease. The prognosis for non-resectable primitive neuroectodermal tumor and Ewing's sarcoma is said to be very poor. However, there are some patients in whom the disease is kept in remission by high-dose chemotherapy with autologous peripheral blood stem cell transplantation, so this therapy may be a possible substitute for radical operation. With spindle cell sarcomas, the efficacy of this treatment was temporary, so it will be necessary to investigate frequent high-dose chemotherapy and to change the high-dose chemotherapy regimen.
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PMID:[High-dose chemotherapy with autologous peripheral blood stem cell transplantation (PBSCT) for refractory bone and soft tissue sarcomas]. 1050 May 30

Primary tumors of the spine are relatively infrequent lesions compared with metastatic disease, multiple myeloma, and lymphoma which are the more frequent neoplasms of the spine and usually manifest with multifocal lesions and thus pose little diagnostic dilemma. However, in the presence of a solitary spinal lesion, the more uncommon primary tumors of the spine represent an important group of entities for diagnostic consideration. The most common benign and malignant primary tumors of the spine are enostosis, osteoid osteoma, osteoblastoma, giant cell tumor, aneurysmal bone cyst, osteochondroma, chordoma, chondrosarcoma, Ewing sarcoma, primitive neuroectodermal tumor, and osteosarcoma. The imaging features of these lesions are often characteristic. Radiologists should be aware of the appearance of these unusual tumors in order to provide a complete differential diagnosis.
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PMID:Primary bone tumors and pseudotumors of the lumbosacral spine. 1096 37

The term "small round-cell tumor" describes a group of highly aggressive malignant tumors composed of relatively small and monotonous undifferentiated cells with high nuclear to cytoplasmic ratios. This group includes Ewing's sarcoma (ES), peripheral neuroepithelioma (aka, primitive neuroectodermal tumor or extraskeletal ES), peripheral neuroblastoma ("classic-type"), rhabdomyosarcoma, desmoplastic small round-cell tumor, lymphoma, leukemia, small-cell osteosarcoma, small-cell carcinoma (either undifferentiated or neuroendocrine), olfactory neuroblastoma, cutaneous neuroendocrine carcinoma (aka, Merkel-cell carcinoma), small-cell melanoma, and mesenchymal chondrosarcoma. Their clinical presentations often overlap, thus making a definitive diagnosis problematic in some cases. Yet, a clear understanding of their clinicopathologic features usually allows for a confident diagnosis, especially if immunohistochemistry is used. The following is a review of the immunohistochemistry of this small round-cell tumor group.
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PMID:Immunohistochemistry of small round-cell tumors. 1096 7


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