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)

Tumors derived from a Li-Fraumeni syndrome cancer-susceptible family were examined for expression of the retinoblastoma susceptibility gene (RB). Whereas RB expression was normal in a primary breast carcinoma and its metastases from one member of this family, overexpression of RB was found in an adrenocortical carcinoma from another family member. This was in contrast to normal RB expression in normal tissue of this patient, the adrenocortical adenocarcinoma cell line SW-13, and the fibroblast cell line MRC-5, and low level RB expression in normal adrenal tissue. The overexpression in the adrenocortical carcinoma resulted in increased synthesis of the RB-encoded protein and did not appear to be associated with RB amplification or rearrangement. This result is novel as it is usually the loss of expression or production of an altered RB transcript exhibiting deletions that is associated with carcinogenesis. In light of the recent discovery of p53 point mutations in the affected Li-Fraumeni syndrome family members tested, RB overexpression may constitute a secondary event in Li-Fraumeni syndrome tumorigenesis.
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PMID:Overexpression of the retinoblastoma gene in a familial adrenocortical carcinoma. 175 10

Ten patients, seven women and three men, ages 47 to 76 years (mean 58.6 years), treated for adrenal cortical carcinoma between 1971 and 1989, were reviewed. Three (30%) of the tumors were nonfunctioning. The remaining seven (70%) were functioning, six of them occurring in women. Common presenting features were hormonal excess, distant metastases, weight loss, and abdominal pain. The primary tumor was resected in all patients, only two of whom had disease confined to the adrenal gland. Tumor diameter ranged from 9 to 21 cm (mean 15.7 cm). Inferior vena caval or right atrial extension of tumor thrombus was present in two patients. Excluding two deaths from postoperative complications, seven patients died of their disease after a mean survival of 25 months (range 2 to 84 months). Of seven patients who received o,p'-DDD treatment for metastatic or recurrent tumor, three (43%) had an objective response. In two patients, tumor regression was complete and was associated with prolonged survival. The first patient underwent resection of recurrent tumor on two occasions in addition to receiving o,p'-DDD and survived 84 months. The second patient had complete regression of pulmonary and liver metastases confirmed at laparotomy and thoracotomy and remains free of disease at 78 months. None of the five patients treated with various combinations of cytotoxic chemotherapy had an observable response, and no measurable effect was seen in a single patient following abdominal radiotherapy. It is concluded that resection for local recurrence may prolong survival and that significant and lasting tumor regression is possible with o,p'-DDD administration. Beneficial results from cytotoxic agents, however, could not be demonstrated.
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PMID:Adrenocortical carcinoma. 192 92

We report our experience with the management of 7 primary nonrenal parenchymal malignancies with vena caval tumor thrombus. Included are 3 cases of adrenal cortical carcinoma and 1 each of transitional cell carcinoma, embryonal cell testicular carcinoma, pheochromocytoma and primary small cell carcinoma of the lung with metastases to the kidney. Surgical treatment and followup are presented, as well as a review of the literature. An aggressive surgical approach is warranted because prolonged survivals free of disease are possible.
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PMID:Management of primary nonrenal parenchymal malignancies with vena caval thrombus. 198 10

In a case of metastasizing hormonally non-functioning carcinoma of the cortex of the left adrenal, leading to the death of a man aged 42, the activity of uroporphyrinogen decarboxylase and the concentrations of tissue porphyrins were determined. In the carcinoma a quantitative rise was observed of uroporphyrin, coproporphyrin and, particularly, protoporphyrin, while the total concentration of these three porphyrins was increased in the bone marrow fourfold, in the kidney eightfold in relation to the primary tumour. The correlation between the reduced activity of uroporphyrinogen decarboxylase and the increased concentration of uroporphyrin was noted in the adrenocortical carcinoma and its metastases to the liver and lymph node, and also in the bone marrow and renal medulla.
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PMID:Organ porphyrins and uroporphyrinogen decarboxylase activity in a case of non-functioning adrenocortical carcinoma. 210 82

Two-dimensional echocardiography was used to study malignant metastatic neoplasms of the heart and great vessels in 20 patients, 13 males and seven females, whose ages ranged from 15 to 72 years. Five patients had lung cancer; two each had breast cancer, malignant melanoma, hepatoma and one each had gastric cancer, urinary bladder cancer, adrenocortical carcinoma, malignant lymphoma, angiosarcoma, fibrosarcoma, leiomyosarcoma; and two had cancers with unknown primaries. Tumor invasion was demonstrated echocardiographically in the left atrium in one each with breast cancer, fibrosarcoma and gastric cancer; in the right atrium in two with hepatomas; in the right atrium and right ventricle in one patient with adrenocortical carcinoma; in the left ventricle in one with lung cancer; and in the pulmonary artery in one with malignant melanoma. Massive pericardial effusion was observed in 11 of 20 patients; two with pericardial tumors including malignant lymphoma and lung cancer. We conjectured that metastatic tumors in the right cardiac cavities came through the inferior vena cava, and other tumors in the left atrium, left ventricle and pericardium developed from direct extension of the primary lesions. There was an 80% mortality of the patients during the observation period, and the average survival period after the diagnosis of cardiac metastases was 5.5 months. However, one patient was still living after two years of radiation therapy and chemotherapy. Echocardiography proved a useful, non-invasive means for the detection and follow-up observation of metastatic cardiac tumors.
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PMID:[Echocardiography in patients with malignant metastatic neoplasms of the heart and great vessels]. 210 13

Epidermal growth factor (EGF) receptors were examined immunohistochemically in 64 adrenocortical carcinomas obtained at autopsy, and in 23 adrenocortical adenomas and seven pheochromocytomas obtained during surgery. In the nonneoplastic adrenal gland, EGF receptors were scattered to the zona glomerulosa, zona fasciculata, and zona reticularis. Adrenocortical carcinomas (63 of 64), more than adrenocortical adenomas (10 of 23) or pheochromocytomas (four of seven), stained positively for EGF receptors (P less than .01). The immunoreactivity was limited to the cytoplasm, cell membrane, and chromatin. When the antibody was immunoabsorbed with an excess of immunogen peptide, there was no evidence of immunostaining. The adrenocortical carcinomas could be classified into 16 cases of the well-differentiated type, 33 cases of the moderately differentiated type, and 15 cases of the poorly differentiated type. There was no relationship between histologic grading and staining intensity of the EGF receptors. On the other hand, more than 80% of the cases of adrenocortical carcinomas revealed a moderate to high intensity for EGF receptors. In 62 of the 64 patients, there was already metastases to other organs. We conclude that the expression of EGF receptors is associated with tumor growth and/or metastatic potential in adrenocortical carcinoma.
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PMID:Immunohistochemical expression of epidermal growth factor receptors in human adrenocortical carcinoma. 231 5

Adrenocortical carcinoma is a rare tumor, and only limited information is available about its natural history and the effects of therapy. We studied 105 patients (75 female and 30 male; mean age, 46 years) with adrenocortical carcinoma who were referred to us between 1963 and 1987. The average duration of symptoms before diagnosis was 8.7 months. At the time of diagnosis, 68 percent of the patients had endocrine symptoms, and 30 percent had distant metastases. Hormonal studies showed that 79 percent of the tumors were functional. Eighty patients underwent surgery, and 59 also received the adrenal cytotoxic agent mitotane. The median disease-free interval after surgery was 12.1 months (range, 1 to 175). Tumor dissemination occurred in 82 percent of the patients, most commonly to the lung, liver, and adjacent organs. The median survival time was 14.5 months (range, less than 1 to 175), and the five-year survival was 22 percent. Age over 40 years and the presence of metastases at the time of diagnosis were the only factors recognized as indicating a poor prognosis. Mitotane controlled hormonal secretion in 75 percent of the patients. Eight mitotane-treated patients had partial tumor regression, but the drug did not have a significant effect on survival. We conclude that adrenocortical carcinoma carries a poor prognosis. Mitotane therapy may offer transient benefits, particularly in controlling endocrine symptoms.
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PMID:Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy. 238 76

The diagnosis of adrenocortical carcinoma (ACC) is often difficult, because this tumor may present with direct extension into adjacent renal parenchyma or with metastatic disease. Renal cell carcinoma and other histologically similar tumors are potentially confused with ACC by conventional light microscopy, and their separation from the latter is often impossible without the aid of additional studies. Furthermore, the distinction between adrenal cortical adenoma and ACC may also be problematic. Because of these factors, the authors studied 10 cases each of ACC, adrenocortical adenoma, and renal cell carcinoma (RCC) immunohistochemically, in an attempt to develop objective parameters which may aid in this differential diagnostic dilemma. Nontrypsinized, formalin-fixed, paraffin-embedded specimens were used in all cases, and tissue from the adrenocortical tumors was also studied for intermediate filament content after protease digestion. All 20 nontrypsinized adrenocortical neoplasms were positive for vimentin, but not for cytokeratin, epithelial membrane antigen, or blood group isoantigens. Conversely, each of 10 cases of RCC expressed epithelial membrane antigen, cytokeratin, and blood group isoantigens, but none was immunoreactive for vimentin. Two adrenocortical carcinomas and three adenomas manifested cytokeratin positivity after trypsin digestion. There were no significant differences between the immunostaining profiles of ACC and adrenocortical adenoma, which suggest that this distinction must still rely upon clinical and morphologic criteria.
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PMID:Adrenocortical carcinoma. An immunohistochemical comparison with renal cell carcinoma. 241 89

Recent studies have suggested that loss of heterozygosity at loci on the short arm of human chromosome 11 (11p) may be important in the pathogenesis of benign and malignant adrenal cortical tumors. To test this concept, adrenocortical carcinomas from nine patients and benign adrenal cortical lesions from eight patients were tested for loss of alleles at loci on human chromosomes 11, 13, and 17. All patients with adrenocortical carcinoma whose normal somatic tissues were heterozygous for a locus on chromosome 17p had lost alleles in the tumor. Four of six patients with adrenocortical carcinoma who were heterozygous for one or more alleles on chromosome 11p in normal tissues had lost 11p alleles in the tumor. Three of six patients with adrenocortical carcinoma showed loss of 13q alleles in the tumor. Loss of alleles on chromosomes 11p, 13q, and 17p was observed in primary tumors and metastases but not in adrenocortical adenomas or hyperplastic lesions of the adrenal cortex. One patient with adrenocortical carcinoma had a somatic mutation in the HRAS1 gene in the normal adrenal gland. The consistency of the genetic changes on chromosomes 11p, 13q, and 17p suggests that they are important in the pathogenesis of adrenocortical carcinoma.
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PMID:Genetic changes in human adrenocortical carcinomas. 256 50

A 49-year-old woman was admitted to our hospital with complaints of edema and abnormal shadow in right pulmonary area. CT scan and abdominal aortography showed left adrenal mass. Moreover, the lung metastasis measured 60 x 65 mm and bony metastases were suspected in three areas on bone scanning. Under diagnosis of left non-functioning adrenal tumor with pulmonary and bony metastases, left adrenalectomy was performed. Histological diagnosis was left adrenal cortical carcinoma without lymph node metastasis. Treatment consisted of 3 g o,p-DDD and 400 mg carmofur per day after surgery. At present, pulmonary metastasis has decreased in size and bony metastases decreased in uptake on bone scan 12 months postoperatively.
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PMID:[A case of non-functioning adrenal cortical carcinoma with pulmonary and bony metastases]. 261 88


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