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)

Primary primitive neuroectodermal tumor (PNET) of the urinary tract is a rare disease with aggressive behavior and poor prognosis. We analyzed 851 cases of urinary tract malignancies in our hospital between 1984 and 2004. Only three (0.035%) cases with PNET of the urinary tract were identified. Presenting symptoms included flank pain and hematuria. The first case was a 44-year-old man with left renal PNET who underwent hand-assisted laparoscopic radical nephrectomy and adjuvant chemotherapy. There was no recurrent tumor at the 4-year follow-up. The second case was a 75-year-old woman with right renal PNET with inferior vena cava (IVC) thrombosis extending to the right atrium. The patient underwent right radical nephroureterectomy and IVC thrombectomy with cardiopulmonary bypass. She died of metastatic disease 7 months later. The third case was a 45-year-old man with left ureteral PNET. Left ureteral segmental resection and partial cystectomy were performed. Tumor recurrence was noted 7 years later. The patient died of disseminated disease 1 year after the discovery of recurrence. Urinary tract PNET appears to be an aggressive malignancy. Long-term survival is possible if complete resection is performed at an early stage.
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PMID:Primary primitive neuroectodermal tumor of the urinary tract. 1718 43

Primary spinal primitive neuroectodermal tumor (PSPNET) is extremely rare and only 25 cases have been reported in the world literature so far. Three patients of 8, 9 and 18 years of age, who presented with variable grades of neurological deficit were diagnosed as having a dorsal intramedullary lesion, a holocord lesion and cervical extradural tumor with extraspinal extension, respectively, and were operated at our institute. The histopathology of all 3 children revealed PNET. The clinical course, image characteristics and outcome of the 3 children are described, and the relevant literature is reviewed. The following conclusions were drawn from the present study and review of the literature. PNET may manifest itself as a primary lesion of the spine unlike the more common drop metastases from an intracranial lesion. PSPNET may be intramedullary, intradural and extradural with variable extraspinal extension. PSPNET may present as holocord intramedullary lesion, an entity which has not been described earlier. These lesions have a short history, significant neurological deficits and rapid course of illness. PSPNET, though an established entity, did not find a place in the WHO 2000 classification of CNS tumors. Hence its status has to be defined.
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PMID:Primary spinal primitive neuroectodermal tumor: case series and review of the literature. 1719 Sep 80

For the subgroup of patients with inoperable gastrointestinal stromal tumors, progress has been made by the rapid development and approval of the targeted therapy imatinib mesylate. Small round cell sarcoma, such as Ewing/PNET, desmoplastic small round cell sarcoma and rhabdomyosarcoma, are chemotherapy-sensitive and potentially curable malignancies, which are treated with multimodality, dose-intensitive and neoadjuvant protocols regardless of size or overt metastatic disease. A limited number of effective agents available for the treatment of patients with metastatic adult soft-tissue sarcoma exists, which have failed anthracyline and ifosfamide-based chemotherapy. Most other high-grade (grading >I) so-called adult-type soft-tissue sarcomas such as fibro, lipo, pleomorphic and synovial sarcoma are treated with a anthracycline-based regimen with or without ifosfamide as front-line therapy. In this review, the therapeutic activities of drugs currently available as second-line treatment in patients with metastatic soft tissue sarcoma are summarized, providing an overview of contentious or emerging treatment issues. In relapsed 'adult-type' soft-tissue sarcomas trofosfamide, gemcitabine and ecteinascidin (ET-743) appear to be drugs associated with moderate activity and an acceptable toxicity profile. An interesting finding to be noted is that the different drugs have particular effects in distinct subtypes of soft-tissue sarcoma; however, it has to be taken into account that the number of patients included in those phase II trials are limited. The role of the newer agents (e.g. patupilone derivates, brostallicin) is currently not definable. The so-called selective therapy targeting vascular endothelial growth factor (receptor), epidermal growth factor receptor, c-kit, Raf kinase or platelet-derived growth factor receptor and bcl-2 antisensing, proteasome, protein kinase C/B, and mammalian target of rabamycin inhibition will continue to be tested in gastrointestinal stromal tumors patients refractory to imatinib mesylate as well as in selected sarcoma subtypes.
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PMID:Systemic treatment options for patients with refractory adult-type sarcoma beyond anthracyclines. 1726 55

Primitive neuroectodermal tumors of the kidney are rare neoplasms that may mimic renal cell carcinoma, especially in the presence of locally advanced or metastatic disease. Although several cases have been identified as primary renal tumors, this is the first report of metastatic spread of a primitive neuroectodermal tumor to the kidney.
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PMID:Metastatic primitive neuroectodermal tumor to the kidney. 1740 44

Ewing sarcoma/primitive neuroectodermal tumors (EWS/PNET) are characterized by specific chromosomal translocations most often generating a chimeric EWS/FLI-1 gene. Depending on the number of juxtaposed exons assembled, several fusion types have been described with different incidences and prognoses. To assess the impact of each fusion type on the specific phenotypic, tumorigenic, and metastatic features of EWS/PNET, we developed an amenable system using a murine mesenchymal multipotent C3H10T1/2 cell line. Upon transduction of EWS/FLI-1, cells acquired dramatic morphological changes in vitro, including a smaller size and "neurite-like" membrane elongations. Chimeric fusion proteins conferred oncogenic properties in vitro, including anchorage-independent growth and an increased rate of proliferation. Furthermore, EWS/FLI-1 expression blocked mineralization, with concomitant repression of osteoblastic genes, and induced a dramatic repression of the adipocytic differentiation program. Moreover, EWS/FLI-1 promoted an aberrant neural phenotype by the de novo expression of specific neural genes. The intramuscular injection of transduced cells led to tumor development and the induction of overt osteolytic lesions. Analogously, to what was observed in human tumors, type 2 EWS/FLI-1 cells formed primary tumors in immunodeficient mice with a higher incidence and a lower latency than cells bearing types 1 and 3 fusions. By contrast, cells expressing types 2 and 3 fusions showed specific metastatic activity with a higher number of macroscopic metastases in soft tissues and osteolytic lesions in the limbs as compared to type-1-expressing cells. Therefore, the structure of each oncoprotein strongly influenced its tumorigenicity and metastagenicity. Thus, this model provides a basis for understanding the genetic determinants involved in Ewing tumor development and metastatic activity and represents a cellular system to analyze other oncoproteins involved in human sarcomagenesis.
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PMID:EWS/FLI-1 oncoprotein subtypes impose different requirements for transformation and metastatic activity in a murine model. 1745 69

Synovial sarcoma accounts for between 6 and 10% of childhood sarcomas and histological diagnosis can be challenging, even for experienced pathologists. Several other tumors enter the differential diagnosis, including malignant peripheral nerve sheath tumor, Ewing sarcoma/primitive neuroectodermal tumor and undifferentiated sarcoma. Several recent reports utilizing expression array techniques have documented expression of the MYCN oncogene in synovial sarcoma. In order to more fully investigate this finding, a series of 12 synovial sarcomas and 29 other sarcomas (four malignant peripheral nerve sheath tumors, 15 Ewing sarcoma/primitive neuroectodermal tumors, 10 undifferentiated sarcomas) were examined for MYCN expression and gene amplification. By RT-PCR, nine of 12 synovial sarcomas (75%) expressed MYCN. Five synovial sarcomas (42%) expressed MYCN at high levels. Of the other sarcomas, one malignant peripheral nerve sheath tumor (25%) and five Ewing sarcoma/primitive neuroectodermal tumors (33%) expressed MYCN at low levels, and all other cases were negative for MYCN. None of the synovial sarcomas had genomic amplification, suggesting that high MYCN expression levels resulted from epigenetic phenomena. Examination of selected downstream targets of MYCN in synovial sarcoma revealed expression of MCM7 (minichromosome maintenance protein 7) in all synovial sarcomas, and expression of nestin (n=10; 83%), ID2 (inhibitor of DNA binding protein 2) (n=6; 50%) and MRP1 (multidrug resistance protein 1) (n=1; 8%) in a subset of synovial sarcomas. Expression of downstream targets did not correlate with expression of MYCN. Neither MYCN nor expression of downstream targets significantly correlated with metastases at presentation, progression-free survival or overall survival in this small series. In summary, high levels of MYCN expression was useful for distinguishing synovial sarcoma from other childhood-spindled cell sarcomas with specificity and sensitivity of 100 and 42%, respectively, in this series. The clinical and biological significance of this finding deserves further study.
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PMID:Expression of MYCN in pediatric synovial sarcoma. 1746 17

Teratomas comprise the most common extragonadal germ cell tumors in childhood. Most teratomas involving the thyroid are benign and occur in children. However, the adult cases reported are mostly malignant and commonly arise in the thyroid. We report a case of a 31-yr-old female with a huge neck mass. Pathologic examination revealed it to be malignant teratoma composed of primitive neuroepithelial tissue with primitive neural tubes and loose myxoid to fibrous immature mesenchymal stroma. The patient underwent extensive evaluation of the thyroid gland with computed tomography (CT) scan and positron emission tomography (PET) scan, which revealed no evidence of metastatic disease. She underwent total thyroidectomy with bilateral modified radical neck dissection, intensive chemotherapy and radiotherapy. At 22-months of follow-up, the patient has remained euthyroid and showed no evidence of recurrence. This is the first case, to our knowledge, of malignant thyroid teratoma with a exuberant primitive neuroectodermal tumor component in Korea.
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PMID:Primary malignant teratoma with a primitive neuroectodermal tumor component in thyroid gland: a case report. 1759 74

About 7 years ago, we undertook the resection on a patient with a tumour of the wall of the left ventricle near the atrioventricular junction. The pathologic diagnosis after the operation was peripheral primitive neuroectodermal tumour (pPNET) of the left ventricular wall. Microscopically the cell had a small, round, deeply basophilic nucleus, rich in chromatin, and little or no surrounding perikaryon. To our knowledge, no article has ever reported a case of primary myocardial PNET which grew towards the pericardial cavity and there is no adequate data on optimal treatment of PNET in the heart at present. In our case since the tumour recurred in situ and had no evidence of distant metastases, we considered orthotopic cardiac transplantation.
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PMID:Peripheral primitive neuroectodermal tumour of left ventricular wall origin: a rare case. 1798 77

A 16-year-old student presented with a 4-week history of progressive shortness of breath, loss of appetite, and occasional blood-tinged sputum. The chest X-ray revealed massive right-sided pleural effusion with cardiomegaly. An echocardiogram revealed a large pericardial mass with massive pericardial effusion. Subsequent computed tomography of the thorax revealed a large heterogeneous mass in the right lung with extension into the pericardium. Lung biopsy revealed primitive neuroectodermal tumor (PNET) with small round blue cells, Homer-Wright rosettes, and CD99 positivity. We discuss pericardial metastases of PNET and its implication in this patient.
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PMID:Primitive neuroectodermal tumor of the lung with pericardial extension: a case report. 1800 74

We performed retrospective review of 29 adult patients with cerebellar medulloblastoma/primitive neuroectodermal tumor (PNET) who received craniospinal radiotherapy in Ankara Oncology Hospital between years 2000 and 2005. All patients were operated followed by craniospinal irradiation; 11 of 29 patients also received chemotherapy. All patients had no distant or spinal metastases at the time of diagnosis. Median follow up time was 26 months. Progression-free survival was 86% at 2 years, 55% at 5 years. Mean progression-free survival was 25 months in patients with PNET; 61.4 months in patients with medulloblastoma (P = 0.0016). Mean survival was 61.33% months in patients <25 age, 38 months in patients >25 age. (P = 0.04). Overall mean survival was 59.80 months in patients who received chemotherapy and 41.4 months in patients who did not have chemotherapy (P = 0.15). Cranial relapses were observed in 3 of 29 patients, and 3 of 29 patients had distant metastases. The mean time to cranial recurrence was 19 months; to distant metastases was 18 months. In conclusion, adult patients with PNET have worse survival rates than patients with medulloblastoma, like in childhood patients. Patients younger than 25 years of age also had statistically significant better survival.
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PMID:Medulloblastoma/primitive neuroectodermal tumor in adults: prognostic factors and treatment results: a single-center experience from Turkey. 1818 18


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