Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The conventional radiotherapy and the associated treatments improved the prognostic of nasopharyngeal cancer. A better selection of the patients who must have a more aggressive treatment also probably contributed to this improvement. Even if a relation could be found between the locoregional relapse rate and the distant relapse rate, these two events remain often independent. It results from it that the improvement of local control rate necessarily does not result in a better control of the disease. The patients with a locally advanced tumor, with or not an invasion of the base of the skull and/or neurological symptoms, must have an aggressive locally treatment. This probably includes the increase in dose delivered to the tumor via a more conformational radiotherapy, a brachytherapy, radiotherapy in stereotaxic conditions or other techniques. Dose within the tumor must be at least 70 Gy and the prophylactic nodal dose, at least 50 Gy. CT scan and MRI are essential for delineating the volumes of interest. The protocols of hyperfractionated radiotherapy did not give convincing results. Association with chemotherapy allowed, on the other hand, an improvement of the prognostic locally advanced cancers. Neoadjuvant or adjuvant chemotherapy was largely used to attempt to limit the risks of systemic dissemination, but an improvement of results was not clearly demonstrated. An improvement of the rates of survival and control of the disease, on the other hand, was observed in a certain number of studies with the chemoradiotherapy. In the event of locoregional relapse, an aggressive attitude can allow the control of the disease in the absence of systemic dissemination. Salvage treatments are, however, disappointing for when distant relapse occurs which suggests a difference in chemosensitivity between primary tumor and metastasis.
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PMID:[Treatment of nasopharyngeal cancer: literature review]. 1203 85

Our purpose is to describe the incidence and clinical features of leptomeningeal dissemination (LM) in children with progressive low-grade neuroepithelial tumor (LGN). We have continuously tracked all patients with primary CNS tumors since 1986. Satisfactorily followed data were obtained on 427 of the 588 patients with localized LGN at diagnosis between 1986 and 1998, 177 (42%) of whom developed progressive or recurrent disease. LM was identified in 13/177 (7%). The median age at initial diagnosis was 5 years and at LM diagnosis was 8.5 years. The primary tumor sites were diencephalon (6), brainstem (3), cerebellum (2), cerebrum (1), and spinal cord (1). The histologies were pilocytic astrocytoma (4), ganglioglioma (4), fibrillary astrocytoma (3), mixed glioma (1), and glioneurofibroma (1). Management included chemotherapy (2) or radiotherapy (3) or both (7); 1 patient received only radical resections of symptomatic lesions. The 5-year progression-free survival rates for patients with localized versus LM disease at recurrence were 22% (95% confidence interval [CI], 13%-25%) versus 15% (95% CI, 0.1%-36%), respectively ( P = 0.28). The 5- and 10-year overall survival rates for patients with localized disease versus LM were 87% (95% CI, 82%-92%) and 83% (95% CI, 77%-89%) versus 68% (95% CI, 39%-91%) and 68% (95% CI, 39%-91%), respectively ( P = 0.05). The 7% incidence of LM is a low estimate because patients were not routinely staged at recurrence. Tumors arising from the diencephalon appeared to predispose to LM; no other predisposing features were identified. We strongly urge that for optimum treatment planning all patients with recurrent LGN be staged with an enhanced spine and brain MRI before adjuvant therapy is initiated. The good survival of patients with LGN and LM reflects a more indolent disease than malignant CNS tumors with LM.
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PMID:Leptomeningeal dissemination in children with progressive low-grade neuroepithelial tumors. 1235 55

Brain metastases occur in 20-40% of patients with cancer and their frequency has increased over time. Lung, breast and skin (melanoma) are the commonest sources of brain metastases, and in up to 15% of patients the primary site remains unknown. After the introduction of MRI, multiple lesions have outnumbered single lesions. Contrast-enhanced MRI is the gold standard for the diagnosis. There are no pathognomonic features on CT or MRI that distinguish brain metastases from primary malignant brain tumors or nonneoplastic conditions: therefore a tissue diagnosis by biopsy should be always obtained in patients with unknown primary tumor before undergoing radiotherapy and/or chemotherapy. Some factors are prognostically important: a high Performance Status, a solitary brain metastasis, an absence of systemic metastases, a controlled primary tumor and a younger age. Based on these factors, subgroups of patients with different prognosis have been identified (RPA class I, II, III). Symptomatic therapy includes corticosteroids to reduce vasogenic cerebral edema and anticonvulsants to control seizures. In patients with newly diagnosed brain metastases prophylactic anticonvulsants should not be used routinely. The combination of surgery and whole-brain radiotherapy (WBRT) is superior to WBRT alone for the treatment of single brain metastasis in patients with limited or absent systemic disease and good neurological condition. Complete surgical resection allows a relief of intracranial hypertension, seizures and focal neurological deficits. Radiosurgery, alone or in conjunction with WBRT, yields results which are comparable to those reported after surgery followed by WBRT, provided that lesion's diameter does not exceed 3-3.5 cm. Radiosurgery offers the potential of treating patients with surgically inaccessible metastases. Still controversial is the need for WBRT after surgery or radiosurgery: local control seems better with the combined approach, but overall survival does not improve. Late neurotoxicity in long surviving patients after WBRT is not negligible; to avoid this complication patients with favorable prognostic factors must be treated with conventional schedules of RT, and monitoring of cognitive functions is important. WBRT alone is the treatment of choice in patients with single brain metastasis not amenable to surgery or radiosurgery, and with an active systemic disease, and in patients with multiple brain metastases. A small subgroup of these latter may benefit from surgery. The response rate of brain metastases to chemotherapy is similar to the response rate of the primary tumor and extracranial metastases, some tumor types being more chemosensitive (small cell lung carcinoma, breast carcinoma, germ cell tumors). New radiosensitizers and cytotoxic or cytostatic agents, and innovative technique of drug delivery are being investigated.
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PMID:Management of brain metastases. 1238 50

Neuroblastoma and Wilms' tumor are the most common noncentral nervous system solid tumors in children. Imaging plays a crucial role in the evaluation of the primary tumor and regional and metastatic disease. There is a growing body of literature supporting the use of MRI as the technique of choice for the evaluation of local and regional disease in children with suspected neuroblastoma; however, in children with suspected Wilms' tumor, MRI will likely continue to play a role as a problem-solver when the results of CT are equivocal or indeterminant.
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PMID:Imaging of neuroblastoma and Wilms' tumor. 1242 47

A wide variety of metabolic features of brain tumors can be imaged using PET, including glucose metabolism, blood flow, oxygen consumption, amino acid metabolism, and lipid synthesis. Currently, FDG is the most widely available PET tracer for body imaging and brain imaging. Malignant brain tumors, like many other soft tissue tumors, show increased glucose metabolism, which is reflected on FDG-PET imaging. FDG-PET imaging of brain tumors provides information on tumor grade and prognosis. Compared with other organ systems, FDG-PET imaging of the brain presents unique challenges because of the high background glucose metabolism of normal gray matter structures. Coregistration of the MRI (or CT) and FDG-PET images is essential for accurate evaluation of brain tumors and is performed routinely at the authors' institution. The heterogeneous nature of gliomas can result in significant sampling errors when patients are biopsied for primary tumor diagnosis or recurrent disease. FDG-PET can be used to define the most metabolically active targets for stereotactic biopsy. This in turn can improve diagnostic accuracy and reduce the number of biopsy samples required. FDG-PET is also useful for evaluating residual or recurrent tumor following therapy, and can be used to survey patients with low-grade brain tumors for evidence of degeneration into high-grade malignancy. In the case of suspected tumor recurrence or progression, PET can aid in defining appropriate targets for biopsy. One limitation of FDG-PET is the occasional inability to distinguish radiation necrosis from recurrent high-grade tumor. A second limitation is that FDG-PET is less sensitive than contrast-enhanced MRI for detecting intracranial metastases, and it is the authors' experience that brain studies should not be included as part of routine whole-body PET studies. Other tracers, such as 11C-methionine and FCH, also avidly accumulate in brain tumors and have the advantage of low background cortical activity. The relationship between degree of uptake of these agents and tumor grade is not established. These tracers may be useful in specific clinical situations, however, such as tumor localization for treatment planning or evaluation of low-grade tumors.
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PMID:Positron emission tomography imaging of brain tumors. 1268 15

Coagulation disorders are common in cancer patients. In patients with solid tumors, a low-grade activated coagulation can result in systemic and cerebral arterial or venous thrombosis. Cancer treatments may also contribute to this coagulopathy, which usually, but not exclusively, occurs in the setting of advanced malignant disease. There may be TIAs or cerebral infarctions. Because of the widespread distribution of cerebral thromboses, there may be a superimposed encephalopathy; sometimes this is the only sign. Concurrent systemic thrombosis is present in many patients and is a useful clue to the diagnosis. In cerebral venous occlusion, the initial symptom is usually a headache. Except for cerebral intravascular coagulation that is unassociated with NBTE, neuriomaging studies usually demonstrate one or more parenchymal infarctions. MRI or MRV may demonstrate venous thrombosis. The laboratory evidence of coagulopathy is difficult to distinguish from the asymptomatic coagulopathy that often accompanies advanced cancer, and the test results must be interpreted cautiously. NBTE can be diagnosed by transesophageal echocardiography. There is no established treatment for the thrombotic coagulopathy associated with cancer, but anticoagulation should be considered. In leukemia and lymphoma, the coagulopathy is typically acute DIC that can lead to systemic and brain hemorrhages. It is especially common in acute myelogenous leukemias. The clinical signs of cerebral hemorrhage are fulminant and may be fatal. The bleeding usually occurs in the brain or subdural compartment, and rarely in the subarachnoid space. The diagnosis can be suspected by the clinical setting and by systemic thrombosis or hemorrhage. It can be established by examination of the peripheral smear, the platelet count, and tests of coagulation function. Therapy of acute DIC is controversial and should be individualized for the clinical setting. Cerebrovascular disorders can complicate metastatic or primary tumor in the brain, skull, dura, or leptomeninges. The clinical signs of infarction are indistinguishable from other causes of stroke, except that tumor-related venous occlusion will usually first produce signs of increased intracranial pressure. The diagnosis of tumor-related infarction can usually be established by neuroimaging studies that show infarction and may show extracerebral sites of tumor. CSF examination is useful in diagnosing leptomeningeal metastasis. A search for lung or cardiac tumor should be performed when embolic tumor infarction is suspected. Primary or metastatic tumors in the brain or dura may hemorrhage, producing the initial clinical signs of the brain tumor or a change in chronic signs induced by the tumor. There are helpful clues to a neoplastic hemorrhage on brain CT or MRI scans. The brain hemorrhage may require evacuation and the underlying tumor will usually require additional antineoplastic treatment. Hyperleukocytosis (extreme elevation of the cell count) in acute myelogenous leukemia is a less common cause of brain hemorrhage in recent years because of improved methods to lower the cell count. Cerebral arterial or venous thrombosis is sometimes the result of cancer therapy. The attribution of thrombosis to chemotherapy in many published cases is only speculative, because carefully conducted prospective studies that include investigation for other thrombotic causes are not available. The best-known associations with thrombosis are L-asparaginase, which is typically used in the induction therapy of acute lymphocytic leukemia, and combination hormonal therapy and chemotherapy for breast cancer. Radiation to the head and neck, typically administered for head and neck epithelial cancers or lymphoma, may result in delayed carotid atherosclerosis. The distribution of stenosis or occlusion is within the radiation portal and is typically more extensive than is atherosclerosis that develops in the absence of radiation. Small clinical series suggest that surgical treatment is equally effective as in nonirradiated carotid atherosclerosis. In children, the cerebral vessels can be affected by brain radiation resulting in stenosis or occlusion. Brain hemorrhages can result from chemotherapy effects on the hemostatic system or a microangiopathic anemia. Hemorrhages from radiation-induced vascular abnormalities are rare. Opportunistic infections, especially fungal infections, can complicate cancer or its treatment. Septic cerebral emboli may result in focal cerebral signs, seizures, or encephalopathy. Sometimes there is an associated hemorrhagic vasculitis or cerebritis. Rarely, mycotic aneurysms may bleed. A high index of suspicion is needed to diagnose fungal infection because of the difficulty in culturing the organism from the blood or CSF. A clinician can usually establish the cause of stroke in the cancer patient by performing a careful review of the clinical setting--including the type and extent of cancer and the type of antineoplastic therapy--in which the stroke occurred. Systemic thrombosis, embolism, or hemorrhage can be a clue to the cause, and appropriate neuroimaging and coagulation studies to aid in the diagnosis are available. Therapy may ameliorate symptoms or prevent further episodes. The identification of one of these unusual stroke syndromes that leads to the diagnosis of an occult and treatable cancer can be particularly rewarding.
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PMID:Cerebrovascular complications in cancer patients. 1269 Jun 49

Cytoreductive therapy is effective in the management of metastatic neuroendocrine tumors to the liver, independent of their functioning status. In functioning tumors, clinical endocrinopathies are relieved in most patients and this response usually lasts for several months. Major morbidity and mortality are not greater than the average complication rate for resection for nonneuroendocrine metastatic tumors at major centers; therefore, surgical outcomes appear to justify operative intervention. Patients whose primary tumor can be controlled, whose metastases outside the liver are limited, and who have a reasonable performance status are candidates for resection. The authors' data support the previous statements. The current mortality rate of 1.2% and major morbidity rate of 15% clearly represent the success of the operative approach in such complex cases (54% of patients received a resection of at least one lobe) [9]. A symptomatic response in the 95% range with a median response of 45 months adds many months of symptom-free survival to the lives of most patients [9]. In the literature reviewed for this article, more than half of the patients also underwent a major hepatic resection and 40% of them had concurrent resection of the primary tumor. These data confirm that resection in selected patients is not more complicated or risky than resection for other metastatic tumors. Endocrinopathies have not increased anesthetic or operative risk in this population; however, these results are the product of managing these patients over time, becoming familiar with their clinical syndromes, and being active in the prevention of life-threatening endocrine complications (i.e., carcinoid crisis). The authors have learned over time that patients with valvular disease are not good candidates for surgery. These patients develop right-sided heart failure with an increase in the central venous pressure. This condition can result in massive hemorrhage during the liver resection because of the difficulty in controlling backbleeding from the hepatic veins [26]. Correction of valvular disease is warranted for safe liver resection. The authors' current policy is to rule out valvular disease in every patient with carcinoid tumors and repair the valves prior to hepatic resection when indicated [27]. This policy clearly has decreased the complication rate. Even though liver transplantation seems to be very attractive as a means of eradicating the disease, this has not been common in clinical practice because of the shortage of allografts, and the overall costs and complications of the procedure override its benefits, especially when compared with partial hepatectomy. Current methods to detect the spread of disease that were not readily available in the past, such as MRI and indium-111 pentetreotide (Octreoscan), may expand the applications of transplantation and allow for better selection of candidates. The option of transplantation is still open for improvement and is dependent on organ availability and better staging of the disease. Metastases from neuroendocrine tumors are hypervascular, favoring the application of MRI as the single imaging method; MRI not only evaluates the location and characteristics of the lesions but also determines the relationship with major vessels and bile ducts. Spiral CT scan has been used extensively in the past with acceptable results. Indium-111 pentetreotide functions on the base of somatostatin receptors present in these tumors, but its use has not been established definitely in the work-up of these patients. Perhaps the best use of indium-111 pentetreotide is in the evaluation of disease beyond the primary and liver locations, including bone metastases; its use therefore will likely affect the preoperative work-up of candidates for transplantation [28]. Once the patient has been deemed to have resectable disease by the preoperative work-up, several steps need to be completed prior to surgery to decrease the effect of specific endocrinopathies. For patients with symptoms related to carcinoid tumors, preoperative preparation with 150 to 500 micrograms of somatostatin decreases the chances of carcinoid crisis, which is manifested by hemodynamic instability [29]. The use of this medication intraoperatively should be kept in mind because a carcinoid crisis can occur despite anesthetic premedication. For islet cell tumors, treatment of underlying endocrinopathy has been initiated before referral for surgical treatment in most patients. Surgery is appropriate for patients with metastatic neuroendocrine tumors for the following two reasons: (1) many of them still have the primary tumor in place and resection should be undertaken to avoid acute complications and (2) the addition of adjunctive ablative therapies to surgical resection accomplishes the control of greater than or equal to 90% of the bulk of the tumor. If preoperative evaluation indicates that less than 90% of the tumor is treatable, surgical therapy is contraindicated. Last, even when complete resections are performed, the recurrence rate for these tumors is extremely high. In practical terms, patients with metastatic neuroendocrine tumors are seldom cured. The best hope physicians can offer these patients is an extended survival period with minimal endocrine symptoms and decreased requirements of somatostatin analogs.
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PMID:Hepatic surgery for metastases from neuroendocrine tumors. 1273 41

OBJECTIVE: Currently available imaging modalities, including CT, ultrasound, and MRI are unsatisfactory in the detection of metastatic gastric cancer, especially lymph node metastases and peritoneal spread. The aim of this study is to evaluate FDG-PET in the diagnosis of primary as well as metastatic gastric cancer.METHOD: All patients (18M/5F; mean age 62, range 33-81) with gastric cancer referred for FDG-PET scan from 1/1/97 to 3/20/98 were studied. PET images of the neck, chest, and abdomen were acquired using a dedicated whole body scanner. A final diagnosis was reached in 21 patients by histology, surgical findings, or clinical follow-up.RESULTS: All but 1 primary tumor (12 out of 13) and both cases with local recurrence showed a high degree of FDG uptake, with mean SUV of 8.9 (range 4.8-17.6). The one false negative (FN) occurred in a poorly controlled diabetic with blood sugar of >400 at the time of study. Six cases with prior gastrectomy were true negative (TN) in the region of the stomach. This translates to a sensitivity of 93%, specificity of 100%, and accuracy of 95% for the primary site. In contrast, for intra-abdominal lymph node (LN) stations PET was true positive (TP) in 2, FN in 7, false positive (FP) in 1, and TN in 32, yielding a sensitivity of 22% and accuracy of 81%. PET detected only 2 out of 9 perigastric lymph node metastases. For the 8 patients in this series who had documented M1 disease, PET detected two with liver metastasis, one with colon metastasis, and one with submandibular LN. However, four cases of peritoneal spread were missed.CONCLUSION: FDG-PET is highly sensitive in detecting the primary lesion in gastric cancer and shows promise in the detection of liver and extra-abdominal metastasis. However, PET appears limited in the detection of perigastric lymphadenopathy as distinct from the primary tumor and in assessing peritoneal spread. Our preliminary assessment suggests that staging of gastric cancer with FDG-PET scanning is relatively effective at detecting distant metastatic disease and will complement standard staging methods such as laparoscopy, which are more effective at staging local nodal spread and peritoneal disease.
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PMID:Accuracy of FDG-PET in Gastric Cancer. Preliminary Experience. 1451 55

To investigate somatostatin receptor scintigraphy (SRS) usefulness compared to conventional imaging procedures (CIP) in non-functioning gastroenteropatic (GEP) neuroendocrine tumors, we studied 40 patients, 30 in follow-up (FU) after previous operation for primary tumor (27 cases) or non-operable for disseminated metastases (3 cases), and 10 in the phase of initial diagnosis and staging (IDS). All patients were asymptomatic for hormone overexpression, with slightly high serum chromogranine A in 6 FU. The definitive diagnosis was obtained by surgery, laparotomy or percutaneous CT/US guided biopsy. Within 1 month before scintigraphy, all patients had undergone at least 2 of 3 CIP (CT, MRI and US); 4 and 24 h after 250 MBq 111In-pentetreotide i.v. injection, whole body scanning, planar and SPECT over the abdomen or other suspect regions were performed. Globally, 135 neoplastic lesions (84 hepatic, 34 abdominal extra-hepatic and 17 extra-abdominal) were ascertained in 32/40 patients; SPECT was positive in 29 cases, CIP in 28 and planar in 20, while all 3 procedures were true negative in the remaining 8 cases. Per patient sensitivity and accuracy were significantly higher at SPECT and CIP than planar. SPECT showed a significantly higher per lesion sensitivity (89.6%) than CIP (72.6%) and planar (53.3%) while CIP sensitivity was significantly higher than planar. SPECT correctly modified CIP patient classification and thus management in 18.7% of patients, while it downstaged the disease in 9.4% in respect of CIP; planar was incorrect in 51.3% of cases. SPECT and CIP combined use achieved 100% accuracy and correctly classified all patients. 111In-pentetreotide SRS, particularly SPECT, is a useful diagnostic tool in the detection of non-functioning GEP tumors, contributing to correct patient classification and appropriate therapeutic strategy. SPECT proved more sensitive than CIP, but their combined use achieved the highest accuracy values and gave the most accurate disease staging.
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PMID:Somatostatin receptor scintigraphy with 111In-pentetreotide in non-functioning gastroenteropancreatic neuroendocrine tumors. 1461 42

Cytokeratin (CK)7 and CK20, the low molecular weight cytokeratins, have been found to have a benefit in the differential diagnosis of some epithelial neoplasms. In the present study, the actual role of these markers in the search of primary tumors in 32 patients with craniospinal metastasis of an unknown primary site at presentation, is evaluated. A series of 36 patients with a known primary tumor were presented for comparison. In the first group, two CK7 and CK20 expression profiles were observed; 87% of metastatic tumors were CK7+/CK20- and 13% CK7-/CK20-. The lung was the major source (82%) of CK7+/CK20- metastatic tumors, whereas it represented only 38% of primary tumor in the second group of a known primary site (P=0.006). Given the fact that metastatic tumors to the craniospinal axis of an unknown primary site are frequently CK7+/CK20-, and they have commonly metastasized from the lung, it is doubtful that immunohistochemistry is really helpful. However, CT scan and MRI of the chest still play an important role. Many patients in the present study had to undertake these imaging studies, regardless of the CK7/CK20 result. The immunostains may be useful in cases with other expression profiles, but such examples constituted only a minority in the present study.
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PMID:Cytokeratin 7 and 20 as immunohistochemical markers in identification of primary tumors in craniospinal metastases: do they have a significant role? 1471 41


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