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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A virilizing adrenocortical carcinoma was treated by excision of the primary tumor and later lobectomy for metastases to the lung. Virilization was abolished, fertility restored, and the patient is without evidence of recurrent tumor 25 years later.
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PMID:Pulmonary lobectomy for metastatic adrenocortical carcinoma: a 25-year survival. 648 3

The data are summarized on the incidence and morphofunctional characteristics of gonadal tumors in different varieties of false male hermaphroditism, namely the testicular feminization syndrome (marked and unmarked forms), demonstrable masculinization and dysgenesis and compared with the disease clinical manifestations. The two syndromes--testicular feminization (marked form) and dysgenesis were found to be associated with a high risk of cancer development. In the first case there develop sertolioma-like tumors and in the second one, tumors similar to gonocytoma and dysgerminoma. These tumors became malignant in rare cases, they did not recur or metastasize. The treatment schedule for such patients has been developed. It includes the removal of the tumor-affected gonads and transfer of the tumor-free gonads into subcutaneous abdominal or scrotal areas and administration (during castration) of continuous substitution hormonotherapy according to the sex chosen.
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PMID:[Testicular tumors in male pseudohermaphroditism]. 651 82

Information about epidemiology, natural history and prognostic factors of adrenocortical carcinoma in Italy is extremely scarce. We report here 35 patients of adult age who were referred to our institution in the last two decades. Nine patients had non functioning, and 26 had functioning tumors. In non-functional tumors initial symptoms were abdominal pain in 90% of cases, fever, weakness, malaise, weight loss in 30%. Only one patient was asymptomatic. Of patients with functioning tumors, 18 presented with Cushing's syndrome, 6 with Cushing's syndrome and virilization, 1 with Cushing's syndrome and feminilization and 1 with hyperaldosteronism. Twenty-two of all cases (63%) had metastases at diagnosis; most frequent sites were lung, liver and distant lymph nodes. The results of tumor staging, according to MacFarlane system, were: stage I, 1 patient (3%); stage II, 10 patients (28%); stage III-IV, 24 patients (69%). Twenty-six out of 35 patients underwent removal of the mass with complete adrenalectomy. Twelve patients received mitotane alone; 8 mitotane and chemotherapy; 5 chemotherapy alone; 2 radiotherapy associated with mitotane or chemotherapy; 1 anthalgic radiotherapy. Survival time ranged from 1 to 108 months. One-year survival rate was 60%, and 5-year survival rate was 10%. Lower survival rate compared with that reported from other countries is probably related to the referring of patients at very advanced stages of disease. Early recognition and referral, in addition to optimization of therapeutic protocols by multicenter studies, may improve prognostic aspects.
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PMID:Adrenocortical carcinoma: epidemiology and natural history. 765 Dec 87

The endocrine side effects are mainly androgenic and concern particularly but not exclusively females and children. Depending on individual sensitivity, the dosage used and the androgenicity of the drug concerned, signs of virilization ranging from slight voice disturbances to severe derangement of reproduction can develop, the latter occurring in both sexes. Progestational effects are of little importance. Hepatic alterations are caused almost exclusively by 17 alpha-alkylated steroids and can range from abnormal liver function tests to life-threatening liver tumours. Atherogenic changes in the lipid-lipoprotein balance, again a domain of the 17 alpha-alkylated preparations, might increase the risk of coronary heart disease. The metabolic influences of anabolic compounds can--at excessive dosage levels--create a prediabetic condition and polycythaemia. The influence of anabolic agents on psyche and behavior in normal doses are mostly positive, rendering the drugs useful for adjuvant therapy in patients whose general condition is poor, irrespective of the origin. If given in excessive doses they can cause grave psychic and behavioral disturbances and possibly dependence. Anabolic-androgenic steroids should be used with caution in patients who are particularly sensitive to side effects, where fluid retention must be prevented, in subjects with liver diseases, skeletal metastases of mammary carcinoma, and when longitudinal growth is not completed. They are contraindicated in carcinoma of the prostate and mammary carcinoma in the male and their use should be discouraged in pregnancy and during lactation.
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PMID:Side effects of anabolic steroids and contraindications. 825 57

In the last 20 years, eleven children with adrenocortical functional tumors were treated in the National Children Hospital of Costa Rica. There were nine females and two males and their ages ranged from nine months to 14 years. Eleven patients had features of virilism, five had stigmas of Cushing's syndrome and three hyperaldosteronism. The clinical diagnosis was established given the symptoms, hormonal tests and radiological and imagenological studies. The histologic diagnosis was carcinoma in six by clinical picture in one, and were adenoma in four. Three patients had regional and distant metastases. Four patients with carcinoma were treated by surgery and five received chemotherapy, two of them in presurgical stage, and four received radiotherapy. Two patients with carcinoma are alive and had no evidence of tumor recurrence ten and six years after diagnosis. Five are dead, two of them after partial response to chemotherapy. The four patients with adenoma were cured by complete surgical tumor resection, furthermore one of them received chemotherapy because there was not sure of his histologic benign condition. It is necessary more studies in use of chemotherapy in treatment of this tumors but in our experience CFM, VCR, Epi and Actin is a regimen that appears to be an active combination for the treatment in presurgical stage of adrenal cortical carcinoma.
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PMID:[Functioning tumors of the adrenal cortex in children. Clinical and therapeutic considerations on 11 cases]. 837 47

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

Adrenal cortical carcinoma (ACC) is a rare neoplasm that affects all age groups, with a bimodal peak of incidence, in young individuals in the first decade or two of life and in older subjects in the fifth to seventh decades. It may be clinically "functional" with Cushing's syndrome, virilization, or feminization, or it may be "nonfunctional." We report on the case of a 42-yr-old woman who complained of abdominal pain and a large adrenal tumor measuring 20 cm in size. No endocrine symptoms were observed. Laboratory tests showed increased levels of adrenocorticotropic hormone (ACTH), serum cortisol, and urinary free cortisol. Cytohistologic features were typical of ACC. A striking presence of hyaline cytoplasmatic globules was seen in cytologic smears and histologically, being immunoreactive for vimentin, consistent with an intracellular store of intermediate filaments. The tumor showed high proliferative activity (40%) with Ki-67 and negativity for p53, cerbB2, and bcl-2. Although hyaline globules are more frequent in pheochromocytomas and other neoplasms, they may also be present in ACC. These globules may be observed in cytologic smears. Also, the identification and immunohistochemical characterization of these hyaline globules in metastases may be useful in determining the origin of primary occult tumors. Diagn. Cytopathol. 1999;21:394-397.
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PMID:Giant adrenal cortical carcinoma, clinically "nonfunctional": report of a case containing cytoplasmic hyaline globules of vimentin. 1057 70

A 60-year-old woman presented with diffuse scalp alopecia, hirsutism, and clitorimegaly, and the mean serum testosterone levels were greater than 200 ng/dL. Findings on computed tomography of both adrenal glands were normal. After bilateral oophorectomy, a unique histological picture consisting of diffuse stromal Leydig cell hyperplasia was found. Reinke crystals were present, but neither hilus cell hyperplasia nor stromal hyperthecosis was noted. Sequencing of the 11 exons of the gene for the luteinizing hormone receptor revealed no abnormality. Relevant data suggest that treatment of the postmenopausal woman with hyperandrogenism and virilization is bilateral laparoscopic oophorectomy if she has no pronounced ovarian enlargement or adrenal tumor on imaging. In this setting, an intensive endocrine evaluation or a search for metastatic disease seems to be unnecessary.
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PMID:Diffuse stromal Leydig cell hyperplasia: a unique cause of postmenopausal hyperandrogenism and virilization. 1072 57

Adrenocortical carcinoma (ACC) is a rare neoplasm with poor prognosis. Patients present with signs of steroid hormone excess (e.g. Cushing's syndrome, virilization) or an abdominal mass. Tumour size at presentation (mean diameter at diagnosis > 10 cm) is the most important indicator of malignancy. In addition, computed tomography (CT) typically demonstrates an inhomogeneous adrenal lesion with irregular margins and variable enhancement of solid components after intravenous contrast media. Magnetic resonance imaging (MRI) is equally effective as CT and is particularly helpful to visualize invasion into large vessels. Complete tumour removal (R0 resection) offers by far the best chance for long-term survival and therefore surgery is the treatment of choice in stage I-III ACC. Despite tumour resection for cure most patients will eventually develop local recurrence or distant metastases. Thus adjuvant treatment options need to be evaluated in high-risk patients (e.g. radiation therapy of the tumour bed and/or chemotherapy). In tumour recurrence re-operation should always be considered. In metastatic disease (stage IV ACC) not amenable to surgery mitotane (o,p'DDD) remains the first-line therapy. Drug monitoring is needed for effective treatment aiming at concentrations between 14 and 20 mg/l. Patients not responding to mitotane may benefit from cytotoxic chemotherapy (23% partial remissions, 4% complete remissions). Only large prospective multicentre trials comparing different treatment options will allow to make systematic progress in the management of ACC.
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PMID:Management of adrenocortical carcinoma. 1500 91

Childhood adrenocortical tumors (ACT) are very aggressive endocrine neoplasms whose incidence is quite low. Little is known about their pathogenesis, clinical presentation, and optimal treatment. In recent years, however, new information has been derived from the International Pediatric Adrenocortical Tumor Registry (IPACTR), and new clues to its pathogenesis have emerged. To provide an overview of the available data that may apply to pediatric ACT, we reviewed the epidemiology, pathogenesis, and treatment of ACT in adults and in children. Germline TP53 mutation is almost always the predisposing factor in childhood ACT. A unique germline mutation (TP53-R337H) has been described in Southern Brazil, where the incidence of ACT is 10-15 times the general incidence. Childhood ACT typically present during the first 5 years of life and has female predominance. Hormone hyperproduction is almost universal, and most patients present with virilization. Two-thirds of patients have resectable tumors. Surgery is the definitive treatment for ACT, and a curative complete resection should always be attempted. Cisplatin-based chemotherapy with mitotane is indicated for unresectable or metastatic disease, although its impact on overall outcome is slight. In childhood ACT, age, tumor size, and tumor resectability are the most important prognostic indicators. Outcome is stage-dependent; patients with small, resectable tumors have survival rates in excess of 80%, whereas the outcome for patients with unresectable disease is dismal. Patients with large, resectable tumors have an intermediate outcome. Childhood ACT are rare, but their unique epidemiology appear to implicate novel oncogenic pathways that are unique to the pediatric population. Multi-institutional and prospective studies are necessary to further our understanding of the pathogenesis and to improve outcomes.
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PMID:Biology, clinical characteristics, and management of adrenocortical tumors in children. 1574 38


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