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)

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

The natural history of pancreatic neuroendocrine tumors (PNET) remains poorly defined. Our objectives were to examine the clinicopathologic features of PNETs, to assess treatment trends over time, and to identify factors associated with undergoing resection. From the National Cancer Data Base (1985-2004), 9,821 patients were identified with PNETs. Clinicopathologic features and treatment trends were examined. Multivariable logistic regression was used to assess factors associated with undergoing resection. Of 9,821 patients with PNETs, 85% were nonfunctional, 7.1% were functional, and 7.9% were carcinoid tumors. Of the 3,851 (39.0%) patients who underwent pancreatectomy, 449 (11.7%) received adjuvant chemotherapy, and 254 (6.6%) received adjuvant radiation. From 1985 to 2004, utilization of pancreatectomy increased from 39.4 to 44.3% (P < 0.0001). Patients were less likely to undergo resection if they were > 55 years old, had tumors in the head of the pancreas, tumors > or = 4 cm, or had distant metastases (P < 0.0001). Patients treated at NCCN/NCI, academic, or high-volume hospitals were more likely to undergo resection. There are disparities in the utilization of pancreatectomy for PNETs. As PNETs have a better prognosis than adenocarcinoma, concerns regarding the morbidity and mortality of pancreatic surgery and neoplasms should not preclude resection.
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PMID:Clinicopathologic features and treatment trends of pancreatic neuroendocrine tumors: analysis of 9,821 patients. 1784 54

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

A 28-year-old woman presented with right flank pain. A large, firm, fixed mass was palpable in the right side of the abdomen. Computed tomography revealed a solid mass of the right kidney with extension into the renal vein and inferior vena cava. The patient underwent right radical nephrectomy with en bloc resection of the inferior vena cava containing tumor thrombus and right adrenalectomy. Histologically the tumor consisted of small tumor cells with rosette formation. Immunohistochemical staining was positive for CD99 and NSE. Analysis with polymerase chain reaction (PCR) demonstrated the EWS/FLI1 fusion products resulting from a chromosomal translocation. These findings were consistent with primary renal primitive neuroectodermal tumor (PNET). Two months after surgery, multiple lung, liver and lymph node metastases were found. The patient received 2 cycles of chemotherapy with cisplatin, ifosfamide, etoposide, resulting in a partial remission. She subsequently received 1 cycle chemotherapy with paclitaxel and carboplatin, resulting in no response. The metastatic lung and liver diseases progressed and she died 5 months after diagnosis.
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PMID:[Case of primitive neuroectodermal tumor of the kidney]. 1840 86

Primitive neuroectodermal tumor of the sternum is rare. A 59-year-old woman referred to our department with anterior chest pain and a tumor in the sternum. The patient was diagnosed as primitive neuroectodermal tumor of the sternum by core biopsy of the lesion. She received 2 cycles of preoperative chemotherapy with vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide. She underwent a total sternectomy with resection of adjacent bilateral costal cartilages and sternal ends of the clavicles. The skeletal defect of chest wall was reconstructed by polypropylene mesh-resin sandwich. The myocutaneus defect was reconstructed by the pedicled latissimus dorsi myocutaneus flap and the bilateral breast flaps. The postoperative course was uneventful and adjuvant radiotherapy was started 6 weeks after the operation. She died of distant metastases 3 months after the operation, although this patient was free from local recurrence.
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PMID:[Sternal resection and chest wall reconstruction for primitive neuroectodermal tumor of the sternum]. 1878 70

Primary orbital primitive neuroectodermal tumor (PNET) is rare with no reported series. We report six cases of orbital PNET treated at a tertiary care oncology center in northern India from 2003 to 2008. None of them had distant metastases. All were treated with neoadjuvant chemotherapy followed by exenteration in two, radiotherapy and adjuvant chemotherapy in five cases. Three out of six achieved complete remission at end of therapy with globe salvage in three and vision in two cases. Chemoradiotherapy may help us to avoid mutilating surgery in large or locally advanced tumors, allowing preservation of vision or the globe.
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PMID:Therapy and outcome of orbital primitive neuroectodermal tumor. 1909 May 47

We present the very unusual case of a young woman suffering from a brain tumor 22 years after a stage IV spinal neuroblastoma as an infant, demonstrating the difficulties of differentiating late neuroblastoma relapse from secondary supratentorial primitive neuroectodermal tumor (sPNET). Lacking specific immunohistochemical features, the first cerebral tumor at the age of 21 was regarded as sPNET, and we pursued a therapeutic approach consisting of neurosurgical resection as well as irradiation and high-dose alkylator-based chemotherapy according to the HIT2000 protocol. Two years later the patient suffered from a diffusely infiltrating local recurrence, changing its imaging appearance as well as its immunohistochemical characteristics, now revealing disseminated positivity for neuron-specific enolase and neural cell adhesion molecule. Moreover, the lack of PNET-specific translocations (EWS/FLI1 gene fusion) in both brain tumors as well as the development of hepatic metastases was more compatible with the diagnosis of a very late relapse 22 years after initial stage IV spinal neuroblastoma.
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PMID:CNS tumor 22 years after spinal neuroblastoma IV: diagnostic dilemma between recurrence and secondary malignancy. 1925 32

Primitive neuroectodermal tumor is an invasive neoplasm with neuronal differentiation, which frequently results in metastasis in various organs. We report the case of a patient with primitive neuroectodermal tumor whose primary site was the axilla. The patient presented with metastases in the lung, pleura, bone, iliac muscle and bone marrow. We highlight the uncommon finding in the pleural fluid cytology.
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PMID:Uncommon pleural effusion: pleuropulmonary metastasis from primitive neuroectodermal tumor. 1961 40


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