The weekly protocol of iv methylprednisolone therapy is more efficient and safer than the daily protocol for patients with active moderate-to-severe GO.
Objective Up to 40% of multiple endocrine neoplasia type 1 (MEN1) patients may have adrenal cortical tumors. However, adrenocortical carcinoma (ACC) is rare. The clinical manifestations, prevalence, inheritance and prognosis of ACC associated with MEN1 remain unclear. Here we report the clinical manifestations and prevalence of ACC in patients with MEN1.
Design and methods A retrospective analysis of ACC associated with MEN1 patients at a single tertiary care center from December 2001 to June 2017. Genetic analysis of MEN1 and other ACC associated genes, loss of heterozygosity (LOH) of MEN1 locus, immunohistochemistry staining of menin, P53 and β-catenin in ACC tissue were performed.
Results Two related patients had ACC associated with MEN1. The father had ENSAT stage IV tumor with excessive production of cortisol; the daughter had nonfunctional ENSAT stage I tumor. Both patients carried novel germline heterozygous mutation (c.400_401insC) of MEN1. The wild-type MEN1 allele was lost in the resected ACC tissue from the daughter with no menin staining. The ACC tissue had nuclear β-catenin staining, with heterozygous CTNNB1 mutation of 357del24 and P53 staining in only 20% cells.
Conclusions ACC associated with MEN1 is rare and may occur in familial aggregates.
Purpose Widespread use of sensitive ultrasound examination led to an increasing detection of medullary thyroid microcarcinoma (micro-MTC). This prospective study evaluated the diagnostic accuracy of Fine-needle Aspiration Biopsy Cytology (FNAB-C) and calcitonin assay in Fine-needle Aspiration Biopsy wash-out uid (FNAB-CT) in thyroid nodules less than 1cm with elevated serum calcitonin.Methods thyroid nodules from 60 patients with elevated serum calcitonin( 10pg/ml) were included and 51 were thyroid nodules less than 1cm. FNAB-CT and FNAB-C was performed to distinguish MTC lesions before surgery, histopathologic diagnoses served as main reference standards.Results FNAB-CT had a greater performance over FNAB-C for preoperative diagnosis of MTC (diagnostic accuracy: 98.85 vs 61.90 , sensitivity: 98.55 vs 55.07 , speci city: 100 vs 97.44 ), especially for micro-MTC: FNAB-C established a sensitivity and diagnostic accuracy of 48.78 and 58 respectively, while FNAB-CT reached 97.56% sensitivity and 98.04% diagnostic accuracy.Conclusions FNAB-CT demonstrated high diagnostic accuracy in diagnosing micro-MTC. Patients with micro thyroid nodules and elevated sCT level should perform FNAB-CT to exclude the diagnosis of MTC lesions.
Summary:An association of bilateral large adrenocortical androgen-producing adenomas with polycystic ovaries in a young female is presented. She developed mild hirsutism and secondary amenorrhoea at the age of.17, and was treated for 3 years with an anti-androgen (cyproterone acetate) and ethinyloestradiol. Routine follow-up at the age of21 showed bilateral large adrenal tumours and polycystic ovaries, together with high serum testosterone and dehydroepiandrosterone sulphate values. Bilateral adrenalectomy was performed, which resulted in lowering of the elevated androgens, and large bilateral adrenocortical adenomas were confirmed histologically. Contrary to expectations, the polycystic appearance of the ovaries persisted after adrenalectomy.This case supports the possible role of adrenal androgens in the pathogenesis of polycystic ovaries as well as the possibility of the persistence of polycystic ovaries without adrenal androgens once they have developed.
Purpose Widespread use of sensitive ultrasound examination led to an increasing detection of medullary thyroid microcarcinoma (micro-MTC). This prospective study evaluated the diagnostic accuracy of Fine-needle Aspiration Biopsy Cytology (FNAB-C) and calcitonin assay in Fine-needle Aspiration Biopsy wash-out fluid (FNAB-CT) in thyroid nodules less than 1cm with elevated serum calcitonin. Methods 87 thyroid nodules from 60 patients with elevated serum calcitonin(>10pg/ml) were included and 51 were thyroid nodules less than 1cm. FNAB-CT and FNAB-C was performed to distinguish MTC lesions before surgery, histopathologic diagnoses served as main reference standards.Results FNAB-CT had a greater performance over FNAB-C for preoperative diagnosis of MTC (diagnostic accuracy: 98.85%vs 61.90%, sensitivity: 98.55% vs 55.07%, specificity: 100% vs 97.44%), especially for micro-MTC: FNAB-C established a sensitivity and diagnostic accuracy of 48.78% and 58% respectively, while FNAB-CT reached 97.56% sensitivity and 98.04% diagnostic accuracy. Conclusions FNAB-CT demonstrated high diagnostic accuracy in diagnosing micro-MTC. Patients with micro thyroid nodules and elevated sCT level should perform FNAB-CT to exclude the diagnosis of MTC lesions.
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