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)

This is a brief report of a patient with Wiedemann-Beckwith syndrome and nephroblastoma with metastases, apparently cured by surgery, chemotherapy, and radiation.
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PMID:A case of EMG (exomphalos, macroglossia, and gigantism) syndrome with associated renal tumor. 19 24

Between 1979 and 1989 21 renal tumors (8 girls and 13 boys) were diagnosed and treated in the Pediatric Hospital of the University of Erlangen. Additionally, there was evidence of nephroblastomatosis in 5 children with Beckwith-Wiedemann Syndrome and hemihypertrophy. One of these infants developed a Wilms' tumor at the age of 3 1/2 years. The most frequent tumor was the Wilms' tumor, which was diagnosed in 14 children. Wilms' tumor are sonographically well delineated, round or oval tumors which often enclose small cysts (72%) but rarely calcifications (5%) and show inhomogenous liver like echogenicity. Metastasis in liver, spleen or abdomen occurred in 2 infants. The most frequent renal tumor in neonates was the mesoblastic nephroma (3 infants). All mesoblastic nephromas were well delineated round tumors with inhomogenous echo-texture and equal or increased echogenicity in comparison to the liver. They often enclosed small cysts but no metastasis or calcifications. Angiomyolipomas of the kidneys could be diagnosed in two children with tuberous sclerosis. These tumors were echogenic nodules spread all over the kidney. We found multilocular nephroblastomas with multiple irregularly delineated cysts in one child. In an other child multiple renal lymphomas simulating solid tumors with liver-like echogenicity could be found.
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PMID:[Ultrasound differential diagnosis of kidney tumors in childhood]. 217 4

The authors present an unusual case of thyroid neoplasia firstly diagnosed as an anaplastic carcinoma with no rise in plasma thyroglobulin (Tg) and treated with total thyroidectomy and radioiodine administration. After 18 months regional lymph node metastases were present with a rise in plasma calcitonin (Ct) (8000-14000 pg/ml); lymphectomy and external radiation were performed and histology revealed a metastasis from thyroid medullary carcinoma. After 3 years, mediastinal and right supraclavicular masses were present with a concomitant rise in plasma calcitonin (from 700 to 3400 pg/ml); all neoplastic lesions showed radioiodine uptake and plasma Tg was 8.9 ng/ml. A biopsy of the supraclavicular region was taken and 131I therapy was attempted, but the patient died after 6 months. Immunocytochemistry of the biopsy revealed the presence of a medullary carcinoma-follicular variant: the neoplastic cells were variably reacting with anti-Ct and anti Tg, and, moreover, the two antigens were sometimes observed in the same cell bodies. The metabolic pattern and the clinical course of this tumour are discussed, and the authors propose that Ct and Tg plasma levels be evaluated and a total body scan (WBS) with radioiodine be performed in all cases of medullary or poorly differentiated thyroid carcinomas.
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PMID:Thyroid carcinoma with biphasic clinical course and evolution in medullary carcinoma-follicular variant. A case report and an immunocytochemical demonstration of calcitonin and thyroglobulin in the same neoplastic cells. 224 75

Metastases of differentiated thyroid cancer may show different uptake patterns for fluorine-18 fluorodeoxyglucose and [131I]NaI. FDG positron emission tomography (PET), iodine-131 whole-body scintigraphy (131I WBS) and magnetic resonance imaging were performed in 58 unselected patients, and spiral computed tomography (CT) of the lung in 25 patients. Thirty-eight patients presented with papillary carcinomas, 15 patients with follicular carcinomas and five patients with variants of follicular carcinoma. Primary tumour stage (pT) was pT1 in 3, pT2 in 19, pT3 in 11 and pT4 in 25 cases. For the detection of metastases, FDG PET was found to have a sensitivity of 50%, 131I WBS a sensitivity of 61%, and the two methods combined a sensitivity of 86%. When FDG PET was limited to patients with elevated thyroglobulin (Tg) levels and negative 131I WBS, the sensitivity of this algorithm was 82%. Of the 21 patients with lymph node metastases, seven presented with FDG uptake but no iodine uptake. In four of them, a second FDG hot spot appeared in a lymph node metastasis of normal size. Five of the seven patients underwent surgery. None of the eight patients with pulmonary metastases smaller than 1 cm exhibited FDG uptake, while five of them had iodine uptake. All had positive results on spiral CT. In conclusion, FDG PET cannot be substituted for 131I WBS. If the Tg level is elevated and 131I WBS is negative, FDG PET can be used to detect lymph node metastases and complements anatomical imaging. A spiral CT of the lung is useful to exclude pulmonary metastases before planning a dissection of iodine-negative lymph node metastases.
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PMID:Fluorine-18 fluorodeoxyglucose positron emission tomography and iodine-131 whole-body scintigraphy in the follow-up of differentiated thyroid cancer. 937 65

Chromosome 11p15 has attracted considerable attention because of the biological importance of this region to human disease. Apart from being an important tumor suppressor locus showing loss of heterozygosity (LOH) in several adult and childhood cancers, 11p15 has been shown by linkage analysis to harbor the gene(s) for the Beckwith-Wiedemann syndrome. Furthermore, the clustering of known imprinted genes in the 11p15.5 region suggests that the target gene may also be imprinted. However, positional cloning efforts to identify the target genes have been complicated by the large size (approximately 10 Mb) and complexity of LOH at 11p15. Here, we have analyzed 94 matched normal and breast tumor samples using 17 polymorphic markers that map to 11p15.5-15.4. We have defined precisely the location of a breast tumor suppressor gene between the markers D11S1318 and D11S4088 (approximately 500 kb) within 11p15.5. LOH at this region occurred in approximately 35-45% of breast tumors analyzed. In addition, we have fine-mapped a second, critical region of LOH, that spans the markers D11S1338-D11S1323 (approximately 336 kb) at 11p15.5-p15.4, that is lost in approximately 55-60% of breast tumors. There is a striking correlation between the loss of the two 11p loci and the clinical and histopathological features of breast tumors. LOH at region 1 correlated significantly (P = 0.016) with early events in malignancy and invasiveness. In contrast, the loss of the more proximal region 2, is highly predictive (P = 0.012) of aggressive metastatic disease. Thus, two distinct tumor suppressor loci on chromosome 11p15 may contribute to tumor progression and metastasis in breast cancer. The fine mapping of this intriguing chromosomal region should facilitate the cloning of the target genes and provide critical clues to understanding the mechanisms that contribute to the evolution of adult and childhood cancers.
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PMID:Two distinct tumor suppressor loci within chromosome 11p15 implicated in breast cancer progression and metastasis. 953 95

The insulin-like growth factor II is mitogenic for a number of cell types and can inhibit apoptosis. The frequent expression of this gene in human and experimental animal tumors indicates that insulin-like growth factor-2 may play an important role in tumor development. It has also been hypothesized that overexpression of this growth factor may be responsible for the increased incidence of childhood tumors in patients with Beckwith-Wiedemann syndrome. To assess the effects of Igf2 on tumor development we produced six transgenic mouse lines that express the gene under the control of the H19 enhancers. Transgenic expression was initiated in the embryonic period but remained high in several adult tissues, including the mammary gland, lung, and liver. Adult transgenic females from five of the transgenic lines developed often multiple mammary tumors that had the ability to metastasize. Increased incidence of other solid tumors was also noted in older mice. These findings indicate that Igf2 expression increases the probability of malignant transformation and that the mammary gland is at a particularly high risk of tumor development in response to chronic increase in Igf2 gene dosage.
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PMID:Metastasizing mammary carcinomas in H19 enhancers-Igf2 transgenic mice. 957 68

Cervicomediastinal magnetic resonance imaging (MRI) was evaluated in 13 consecutive persistent or recurrent papillary thyroid carcinoma (PTC) patients, previously treated by total thyroidectomy and radioiodine ablation. All had elevated thyroglobulin (Tg) levels and were therefore submitted to a new therapeutic radioiodine dose followed by a posttherapeutic whole-body scan (131I-WBS) and subsequent MRI. Patients with known distant metastases were excluded from the study. Group 1 included 7 patients with a negative 131I-WBS, whereas cervical and/or mediastinal 131I-uptake was evidenced in the other 6 patients (group 2). MRI was thus compared to 131I-WBS, and additionally in 8 reoperated cases, to histology. MRI was positive in 11 of 13 (85%) patients, corresponding to 23 of 55 (41.8%) histologically confirmed sites. In group 1, MRI was positive in 5 of 7 patients, with a sensitivity of 47% (15/32 histologically positive sites), allowing appropriate indication of surgery: 4 neck surgery, and 1 mediastinal dissection because of too distant lymph node foci. In group 2, MRI always showed more localization than 131I-WBS; histology was obtained in 3. Because all the foci located in the mediastinal area (0.8 to 1.8 cm) were histologically confirmed (7/7 sites), MRI avoided underestimation of surgery in the 8 reoperated patients. However, additional images were also observed corresponding to a normal thymus, a small neuroma or inflammatory lymph nodes, but pretracheal and very small nodes (less than 0.5 cm) were missed. In conclusion, although less specific than radioiodine scintigraphy, MRI can detect local persistent or recurrent PTC, and seems particularly effective for evaluation of mediastinal involvement.
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PMID:Cervicomediastinal magnetic resonance imaging in persistent or recurrent papillary thyroid carcinoma: clinical use and limits. 1041 Nov 22

Primary neoplasms of the adrenal cortex are rare in children and differ significantly in epidemiology, clinical characteristics, and biologic features from their counterparts in adults. In children, the inclusive term adrenocortical neoplasm is applied because adrenal adenoma and adrenal carcinoma may be difficult to distinguish histopathologically. Pediatric adrenocortical neoplasms typically occur before 5 years of age, affect young girls more commonly than boys, and are associated with hemihypertrophy and Beckwith-Wiedemann and Li-Fraumeni syndromes. Most children with an adrenocortical neoplasm present with signs and symptoms of endocrine abnormality, including virilization and Cushing syndrome. Cross-sectional imaging studies typically demonstrate a large, circumscribed, predominantly solid suprarenal mass with variable heterogeneity due to hemorrhage and necrosis. Calcification is not uncommon. Local invasion and metastases to the lungs, liver, and regional lymph nodes may be present at diagnosis. When friable tumor thrombus extends into the inferior vena cava, it poses a high risk of pulmonary embolization. The finding of increased retroperitoneal fat due to hypercortisolism on computed tomographic and magnetic resonance images of children with an adrenal mass favors the diagnosis of adrenocortical neoplasm. Surgical resection is the mainstay of therapy, with chemotherapy used for patients with metastases or persistent elevated hormone levels following surgery. Patients younger than 5 years with aggressive adrenocortical neoplasms fare better than older children.
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PMID:Adrenocortical neoplasms in children: radiologic-pathologic correlation. 1046 5

Iodine-131 is the most specific radionuclide to follow up patients with differentiated thyroid cancer (DTC). However there are some aspects that should be considered if 131I whole body scintigraphy (131I WBS) is performed. 1) Several prior conditions, including a bTSH above 30 mU/l and an urinary iodine excretion below 100-150 micrograms/g Crea, should be fulfilled. 2) Only about two thirds of metastases from DTC accumulate iodine. Therefore, in addition to 131I WBS, there is a need for other nonspecific tracers such as 99mTc Tetrofosmin WBS, 99mTc Sestamibi WBS or F-18 FDG PET to detect also iodine negative recurrences or metastases. There new tracers, especially F-18 FDG PET have demonstrated a very high detection rate of iodine negative metastases with mostly low differentiation. 3) The sensitivity of 131I WBS depends on the administered dose. Whereas the sensitivity of a diagnostic 131I WBS (up to 185 MBq) is below 60%, the value for a post-therapeutic 131I WBS (after 3700-7400 MBq) increases up to 75%. This means that in case of elevated serum thyroglobulin, iodine positive metastases cannot be excluded until WBS after 131I therapy is performed. 4) In patients with elevated serum thyroglobulin and/or known metastases, who are scheduled for 131I treatment, the question arises whether a diagnostic 131I WBS should be performed and if so, which dose should be administered to avoid thyroid stunning. There is evidence in the literature that the dose for a pre-therapeutic diagnostic 131I WBS should not exceed 74 MBq. 5) Despite the high specificity of 131I WBS, several pitfalls of iodine accumulation in non-malignant diseases and malignancies of other origin than thyroid cancer should be taken into account.
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PMID:131I whole body scintigraphy in thyroid cancer patients. 1056 34

Hypofunctioning nodules on scintiscan using Tc-99m Pertechnetate or I-123 have a higher probability of malignancy compared to eu- or hyperfunctioning nodules. However, in the preoperative assessment of thyroid nodules, ultrasonography and ultrasonography guided fine needle aspiration biopsy play the most important role, especially for papillary thyroid cancer. The problem of differentiating follicular adenoma from highly differentiated follicular carcinoma however remains. Also the additional use of a multi tracer imaging strategy (Tl-201/Tc-99m subtraction scan, Tc-99m Sestamibi, Tc-99m Tetrofosmin dual phase scintigraphy) has not solved this problem. Although it is unlikely, the question whether FDG PET is able to give a better differentiation between benign and malignant tumours in the preoperative assessment of thyroid nodules is not answered up to now. In contrast to preoperative diagnostics, FDG PET is of great value in the postoperative follow up of differentiated thyroid cancer. In case of elevated serum thyroglobulin but negative I-131 WBS FDG PET is the method of choice to detect I-131 negative recurrences and metastases. FDG uptake in metastases from differentiated thyroid cancer is correlated to low differentiation and maybe bad prognosis. There is also evidence that FDG PET may have a role in the follow up of anaplastic and especially in medullary thyroid cancer in the future.
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PMID:The role of F-18FDG PET in thyroid cancer. 1081 62


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