Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe a case of an occipital lobe primitive neuroectodermal tumor (PNET) in a 60-year-old man with immunocytochemical evidence of neuronal, glial, smooth muscle and adipose tissue differentiation in the primary tumor and its metastases. This is the first case of PNET of cerebrum in which adipose tissue and smooth muscle has been demonstrated. The possible cells of origin for these mesenchymal elements is discussed.
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PMID:Adipose tissue and smooth muscle in a primitive neuroectodermal tumor of cerebrum. 817 73

The cytogenetic and morphologic characteristics of a case with a primitive neuroectodermal tumor (PNET) arising from the left kidney in a 22 year old man are presented. The patient was detected as having a left renal mass with a tumor embolus in the inferior vena cava and multiple pulmonary metastases. A radical nephrectomy with tumor embolectomy of the inferior vena cava, along with a resection of the pulmonary nodules were performed. Histologic examination revealed a dense proliferation of small round cells with many Homer-Wright type rosettes and perivascular pseudorosettes. Immunohistochemically, the tumor cells stained strongly positive for HBA71(p30/32MIC2), a surface glycoprotein specific to PNET and Ewing's sarcoma. In addition, the tumor cells expressed several neural markers (neuron specific enolase, neurofilament, synaptophysin, and Leu-7) and vimentin, while the epithelial, muscular, and lymphocytic markers were negative in the tumor cells. Cytogenetic analysis of cultured tumor cells showed a reciprocal translocation t(11;22)(q24;q12) that is considered to be specific to PNET and Ewing's sarcoma. In conclusion, this case suggested that a karyotyping analysis is a useful diagnostic tool for renal PNET, and it may therefore be utilized to help distinguish between difficult cases of small round cell tumors and Wilms' tumor of the kidney.
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PMID:Renal primitive neuroectodermal tumor: an immunohistochemical and cytogenetic analysis. 872 54

Between May 1990, and June 1994, 79 patients with malignant tumors were treated radiosurgically using a Leksell gamma unit at Asan Medical Center. Of these patients, 57 were metastatic brain tumor, 12 were glioblastoma multiforme (GM), 4 were primitive neuroectodermal tumor, 3 were malignant germ cell tumor, 2 were recurrent lymphoma, and 1 was adenoid cystic carcinoma of the orbit. Among 57 patients with metastatic tumors, 28 patients harboring 60 tumors were followed clinically and radiographically. The median marginal dose for these tumors was 30 Gy and the median survival rate was 15 months. Twenty-one tumors disappeared and 32 tumors decreased in size during 2 to 6 months after radiosurgery on computed tomographic or magnetic resonance imaging scans. All 12 patients with GM were treated with conventional radiation (6,240 approximately 6,500 cGy) after surgical resection or biopsy prior to radiosurgery (13 approximately 15 Gy to margin). The results were varied. Radiosurgical treatment of two recurrent lymphomas and three recurrent mixed germ cell tumors after radiation and chemotherapy provided rapid clinical improvement with disappearance of the tumor. However, new lesions appeared in two lymphomas and one mixed germ cell tumor within 3 to 4 months. One patient with adenoid cystic carcinoma of the orbit, who was treated radiosurgically prior to resection, is alive without recurrence 31 months after the treatment. Gamma knife radiosurgery appears to be the best alternative method to surgical excision plus radiation therapy for single and multiple cerebral metastases. It also provides rapid palliation of symptoms due to recurrent malignant tumors. And it may have an adjuvant role in the treatment of some tumors delaying local recurrence, if given prior to resection. However, the preliminary results for the malignant gliomas were inconclusive.
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PMID:Gamma knife radiosurgery for malignant tumors. 875 65

The clinical, radiographic, and pathologic features of 17 patients with documented peripheral primitive neuroectodermal tumor (PNET) were evaluated in a retrospective study. The age at diagnosis ranged from 9 months to 46 years (median, 15.8 years). Primary sites of involvement were the abdomen (n = 8), extremities (n = 5), chest (n = 1), temporal bone (n = 1), maxilla (n = 1), and diploe (n = 1). At the time of diagnosis, six patients had distant metastases; all of these patients died, with an average survival of 8.8 months. Radiologic workup included standard radiographs, ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and bone scintigraphy. The radiographic appearance of these tumors was not specific for differentiation of PNETs from other types of bone and soft tissue tumors. The typical appearance resembled large non-calcified, soft tissue masses with cystic or necrotic areas. Heterogeneous enhancement with intravenous contrast agents was evident on CT, as was an intermediate signal intensity on Tl-weighted images and hyperintense signal on T2-weighted and STIR sequences. After gadolinium administration, variable enhancement was seen. MRI and CT were useful in predicting resectability, in detecting distant metastases, and in the evaluation of response to treatment. Surgery was performed in all cases, either for definitive diagnosis or for therapy. All patients received combined chemotherapy and radiotherapy and five patients received autologous bone marrow transplantation. Clinical follow-up was obtained over a mean period of 3 years (range 1 day to 6 years). Prognosis was poor with a median survival of 3.4 years. Our experience in 17 patients with peripheral neuroectodermal tumors indicates that although their radiologic features are non-specific, they should be included in differential diagnosis of soft tissue tumors of aggressive behavior, especially in a young age group. CT and MRI are useful in delineating the extent and resectability of tumor and in monotoring treatment.
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PMID:Peripheral primitive neuroectodermal tumors. CT and MRI evaluation. 877 15

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

Twenty-nine young men (mean age 29 years) had primitive neuroectodermal tumors (PNETs) arising in germ cell tumors (GCTs). Nine patients had PNETs confined to the testis, eight patients had PNETs in the testis and at metastatic sites, and 12 patients had PNETs identified only at extratesticular sites. Immunohistochemistry was of use in the further classification of these PNETs as neuroblastoma, medulloepithelioma, peripheral neuroepithelioma, or ependymoblastoma. The histologic pattern of PNETs in the testis (neuroblastoma or medulloepithelioma) did not predict which tumors metastasized. PNETs localized to the testis did not affect prognosis. Eight patients with no PNETs outside the testis were free of disease 1 month to 10 years after diagnosis. PNETs in extratesticular sites were an adverse prognostic factor. Nineteen patients with extratesticular PNETs had adequate clinical follow-up. Thirteen are dead of disease from 4 months to 5 1/2 years (mean 26 months) after diagnosis, four are alive with disease 6 months to 2 years after diagnosis, and two have no evidence of disease with short follow-up (6 and 17 months). Mean survival was longer (34 months) for patients whose extratesticular PNET was neuroblastoma than for those with other types of PNETs (13 months). Chemotherapy directed against GCTs was not effective in patients who developed metastatic PNETs of GCT origin. We conclude that extratesticular PNETs in patients with testicular GCTs are usually fatal, but patients with neuroblastomatous metastases may have a more prolonged course.
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PMID:Primitive neuroectodermal tumors arising in testicular germ cell neoplasms. 1007 29

There has been an explosion of new knowledge regarding the Ewing family of tumors over the past 5 to 10 years. Classical Ewing's sarcoma and PNET are now known to be the same tumor with variable differentiation, defined by a translocation between the EWS gene on chromosome 22 with one of three ETS-like genes, especially the FLI-1 gene on chromosome 11. Molecular techniques used to identify this translocation along with the knowledge that the protein product of the MIC2 gene is highly expressed on the cell surface have greatly improved our diagnostic abilities in this family of tumors. Controversy still exists as to whether surgery improves event-free survival when compared with radiotherapy in Ewing's sarcoma. The high second tumor rate, if nothing else, has started moving many physicians to preferentially use surgery when the functional results are predicted to be reasonable. The addition of ifosfamide and etoposide to standard therapy in Ewing's sarcoma has improved survival for patients without metastases at presentation. However, outcome for patients with metastases or who develop metastases while on therapy or shortly thereafter remains poor. Preliminary reports of better outcome with megatherapy are interesting but not yet definitive. The decades ahead will probably see marked changes in therapy for Ewing's sarcoma. The unique translocation seen in virtually all of these tumors is a potential target for a "magic bullet" therapy, because the protein product of this translocation is present only in the malignant cells. Hopefully either immune modulation against this unique protein or further knowledge of how to use antisense genes will move us toward exquisitely targeted therapy in the Ewing family of tumors.
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PMID:The Ewing family of tumors. Ewing's sarcoma and primitive neuroectodermal tumors. 928 96

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

The records of 116 patients from a single center (1970-1993) with newly diagnosed Ewing's sarcoma or primitive neuroectodermal tumor were reviewed retrospectively. The aim of this study was to ascertain the impact of pretreatment variables on disease-free survival. Median age was 14 years (range 1-34). Twenty patients presented with metastatic disease. Treatment consisted of systemic multiagent chemotherapy plus local irradiation (39%), wide resection (22%), or both (35%). Median potential follow-up was 10.7 years (range 2-26). Three patients developed second malignancies (1 breast carcinoma, 2 acute myeloid leukemias). Median time to relapse was 24 months (range 3-143). The actuarial disease-free survival was 37.4% at 5 years, 33.3% at 10 years and 27.8% at 15 years. Neoadjuvant chemotherapy and a therapy-induced tumor necrosis > or = 90% were associated with a better outcome. Patients undergoing surgical resection had a superior disease-free survival than those treated without surgery (45 vs. 18% at 10 years, p = 0.0009). Multiple regression analysis showed that raised serum lactate dehydrogenase levels (p < 0.001), hypoalbuminemia (p = 0.001) and distant metastases at diagnosis (p = 0.03) were independent adverse prognostic factors. In conclusion, one third of patients with Ewing's sarcoma become long-term survivors with combined modality treatment. Late relapses and second neoplasms are of concern. Prognostic factors should be considered in the selection of therapy, and the value of serum albumin warrants confirmatory studies.
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PMID:Long-term follow-up and prognostic factors in Ewing's sarcoma. A multivariate analysis of 116 patients from a single institution. 942 71

Pediatric central nervous system neoplasms include a spectrum of both glial and nonglial tumors that differ significantly in location and biological behavior from those of adults. Brain tumors in infants and children most often arise from central neuroepithelial tissue, whereas a significant number of adult tumors arise from central nervous system coverings (e.g., meningioma), adjacent tissue (e.g., pituitary adenoma), or metastases. Most adult brain tumors are supratentorial malignant gliomas, whereas the most common malignant pediatric brain tumor is the cerebellar primitive neuroectodermal tumor (medulloblastoma). This article reviews neuropathological characteristics of the more common pediatric brain tumors. Entities, such as the brainstem glioma, and less common neoplasms like the desmoplastic infantile ganglioglioma and the central nervous system atypical teratoid/rhabdoid tumor are reviewed because they occur almost exclusively in children. Known cytogenetic and molecular characteristics of childhood brain tumors are also reviewed.
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PMID:Neuropathology of pediatric brain tumors. 944 21


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