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      CARCINOMA CORTICOADRENAL: REVISIÓN DE TEMA Translated title: ADRENOCORTICAL CARCINOMA: SUBJECT REVIEW Translated title: ADENOCARCINOMA CORTICOADRENAL: REVISÃO DE TEMA

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          Abstract

          Los adenomas son los tumores corticoadrenales más comunes, con una prevalencia del 4%, siendo los carcinomas corticoadrenales raros, con una incidencia de 1 caso por millón de habitantes. La mitad de estos carcinomas son funcionales con diagnósticos más tempranos por los síndromes de exceso de hormonas que producen, a diferencia del restante 50% que es silente, con hallazgo de metástasis al momento del diagnostico en más de la mitad de ellos. Pueden tener una presentación esporádica o hacer parte de síndromes de cáncer familiar con anormalidades en genes que resultan en un imbalance entre oncogenes y genes supresores de tumor; además de constituirse como potenciales marcadores diagnósticos, pronósticos y terapéuticos de esta enfermedad. Ante la sospecha de carcinoma de corteza suprarrenal, deben tenerse en cuenta criterios clínicos, bioquímicos y radiológicos para establecer adecuadamente el diagnóstico, el cual debe confirmarse por histología.

          Translated abstract

          Adenomas are the most common adrenocortical tumors with a prevalence of 4%, being the adrenocortical carcinomas rare with an incidence of 1 case por million people. Half these carcinomas are functional, with earlier diagnoses due to the excessive hormonal syndromes they produce unlike the remaining 50% which are silent, with metastases being found upon the diagnosis in more than one half of them. They can have a sporadic presentation or make part of familiar cancer syndromes with genetic disturbances resulting in an imbalance between the oncogenes and the tumor suppressor genes, aside from becoming potential diagnostic, prognostic and therapeutic markers of this disease. In the presence of adrenocortical carcinoma, several clinical, biochemical and radiological criteria must be taken into account to properly establish the diagnosis which should be confirmed through histology.

          Translated abstract

          Os adenomas são os tumores corticoadrenais mais comuns, com uma prevalência de 4%, sendo os adenocarcinomas corticoadrenais raros, com una incidência de 1 caso por milhão de habitantes. A metade destes carcinomas são funcionais com diagnósticos mais precoces pelas síndromes de excesso de hormônios que produzem, ao contrário do 50% restantes que é silente, com descobrimento de metástase no momento do diagnostico em mais da metade deles. Podem ter uma apresentação esporádica ou fazer parte de síndromes de câncer familiar com anormalidades em genes que resultam em um desequilíbrio entre oncogenes e genes supressores de tumor; além de constituir-se como potenciais marcadores diagnósticos, prognósticos e terapêuticos desta doença. Diante da suspeita de carcinoma do córtex suprarrenal, devem ser levados em consideração critérios clínicos, bioquímicos e radiológicos para estabelecer adequadamente o diagnóstico, que deve ser confirmado por histologia.

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          Most cited references82

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          Parental imprinting of the mouse insulin-like growth factor II gene.

          We are studying mice that carry a targeted disruption of the gene encoding insulin-like growth factor II (IGF-II). Transmission of this mutation through the male germline results in heterozygous progeny that are growth deficient. In contrast, when the disrupted gene is transmitted maternally, the heterozygous offspring are phenotypically normal. Therefore, the difference in growth phenotypes depends on the type of gamete contributing the mutated allele. Homozygous mutants are indistinguishable in appearance from growth-deficient heterozygous siblings. Nuclease protection and in situ hybridization analyses of the transcripts from the wild-type and mutated alleles indicate that only the paternal allele is expressed in embryos, while the maternal allele is silent. An exception is the choroid plexus and leptomeninges, where both alleles are transcriptionally active. These results demonstrate that IGF-II is indispensable for normal embryonic growth and that the IGF-II gene is subject to tissue-specific parental imprinting.
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            Adjuvant mitotane treatment for adrenocortical carcinoma.

            Adrenocortical carcinoma is a rare neoplasm characterized by a high risk of recurrence after radical resection. Whether the use of mitotane is beneficial as an adjuvant treatment has been controversial. Our aim was to evaluate the efficacy of adjuvant mitotane in prolonging recurrence-free survival. We performed a retrospective analysis involving 177 patients with adrenocortical cancer who had undergone radical surgery at 8 centers in Italy and 47 centers in Germany between 1985 and 2005. Adjuvant mitotane was administered to 47 Italian patients after radical surgery (mitotane group), whereas 55 Italian patients and 75 German patients (control groups 1 and 2, respectively) did not receive adjuvant treatment after surgery. Baseline features in the mitotane group and the control group from Italy were similar; the German patients were significantly older (P=0.03) and had more stage I or II adrenocortical carcinomas (P=0.02) than did patients in the mitotane group. Recurrence-free survival was significantly prolonged in the mitotane group, as compared with the two control groups (median recurrence-free survival, 42 months, as compared with 10 months in control group 1 and 25 months in control group 2). Hazard ratios for recurrence were 2.91 (95% confidence interval [CI], 1.77 to 4.78; P<0.001) and 1.97 (95% CI, 1.21 to 3.20; P=0.005), respectively. Multivariate analysis indicated that mitotane treatment had a significant advantage for recurrence-free survival. Adverse events associated with mitotane were mainly of grade 1 or 2, but temporary dose reduction was needed in 13% of patients. Adjuvant mitotane may prolong recurrence-free survival in patients with radically resected adrenocortical carcinoma. Copyright 2007 Massachusetts Medical Society.
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              Adrenocortical carcinoma in the United States: treatment utilization and prognostic factors.

              Adrenocortical carcinoma (ACC) is a rare tumor with a relatively poor prognosis. The authors' objectives were to examine treatment utilization and factors associated with long-term survival after resection of ACC in a large, national, patient population. Patients diagnosed with ACC from 1985 to 2005 were identified from the National Cancer Data Base (NCDB). Patient, tumor, treatment, and hospital factors associated with survival after resection were examined. For the current study, 3982 patients with ACC were identified. Median age at diagnosis was 55 years. Median tumor size was 13 cm. Of the patients with nodes examined, 26.5% had nodal metastases. Distant metastases were found on presentation in 21.6% of patients. A total of 57.4% of patients underwent surgical resection alone, whereas 16.0% underwent resection with adjuvant chemotherapy or radiation. A total of 19.4% had margin-positive resections. Treatment utilization remained unchanged from 1985 to 2005 (P = .28). Median follow-up was 24 months. Overall 5-year survival for all patients who underwent resection was 38.6% (median survival, 31.9 months). Multivariable analysis demonstrated a higher risk of death with increasing age, poorly differentiated tumors, involved margins, and nodal or distant metastases. Overall survival remained unchanged from 1985 to 2000 (P = .08). ACC carries a poor prognosis for patients commonly presenting with large, locally invasive tumors, involved margins, and metastatic disease. Survival is not affected by size but is diminished with increasing age, poorly differentiated tumors, involved margins, and the presence of regional and distant disease. Identification of novel therapies may help to increase survival, which has remained unchanged over the last 20 years. (c) 2008 American Cancer Society

                Author and article information

                Journal
                med
                Revista Med
                rev.fac.med
                Universidad Militar Nueva Granada. Facultad de Medicina (Bogotá, Distrito Capital, Colombia )
                0121-5256
                1909-7700
                December 2011
                : 19
                : 2
                : 207-216
                Affiliations
                [01] orgnameHOMIC
                Article
                S0121-52562011000200007 S0121-5256(11)01900207
                de8b402b-762c-45d2-b068-f903d8477728

                This work is licensed under a Creative Commons Attribution 4.0 International License.

                History
                : 28 December 2011
                : 02 November 2011
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 55, Pages: 10
                Product

                SciELO Colombia

                Categories
                Artículos de revisión

                carcinoma,fisiopatología,alteraciones genéticas,estadiaje,terapia,seguimiento,pathophysiology,genetic disturbances,staging,therapy,follow-up,fisiopatologia,alterações genéticas,estadia,acompanhamento

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