Adrenal Lesions in a Large Kindred With Multiple Endocrine Neoplasia Type 1

Burgess, John; Harle, Robin; Tucker, Paul; Parameswaran, V.; Davies, Peter F.; Greenaway, TM; Shepherd, J. J.

doi:10.1001/archsurg.1996.01430190021006

Cited by 98 publications

(78 citation statements)

References 10 publications

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“…We have documented the presence, number and size of any adrenal nodules and compared these findings with those described in the normal population (7). The majority of adrenal lesions in MEN1 have previously been reported to be non-functioning (3,8,9). We reviewed the patients' serum cortisol secretory dynamics and sought potential correlations with adrenal morphology.…”

Section: Introductionmentioning

confidence: 99%

The appearance of the adrenal glands on computed tomography in multiple endocrine neoplasia type 1

Whitley¹,

Moyes²,

Park³

et al. 2008

European Journal of Endocrinology

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Aims: To review the morphology of the adrenal glands in multiple endocrine neoplasia type 1 (MEN1) on computed tomography (CT) to compare the results with established normal values for adrenal size and nodularity and to correlate adrenal size with serum cortisol secretory dynamics. Materials and methods: Two observers independently reviewed the adrenal CT in 28 patients with MEN1, measuring the maximum width of the body of the gland and the medial and lateral limbs. Incidence and location of nodules O5 mm within the gland were recorded. Following exclusion of known cases of Cushing's syndrome, adrenal gland size was compared with previously documented normative data. Adrenal gland size was compared between patients with normal and abnormal cortisol dynamics. Results: Comparison of mean adrenal size in MEN1 patients with normative data showed that the adrenal limbs were significantly larger in MEN1 than normal (P!0.0001 in all four limbs). Adrenal body was also significantly larger (P!0.05). Nodules were demonstrated in 17 (60%) of patients (versus 0.4-2% in the normal population). No statistically significant correlation was demonstrated between adrenal limb hyperplasia and abnormal cortisol dynamics. Conclusions: In patients with MEN1, adrenal limb hyperplasia and adrenal nodules are significantly more common than in the normal population, a phenomenon not previously documented in a quantitative manner. There was no significant correlation between adrenal limb hyperplasia and abnormal cortisol dynamics.European Journal of Endocrinology 159 819-824

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Section: Introductionmentioning

confidence: 99%

The appearance of the adrenal glands on computed tomography in multiple endocrine neoplasia type 1

Whitley¹,

Moyes²,

Park³

et al. 2008

European Journal of Endocrinology

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“…Nevertheless, the genetic cause for these cases hasn't yet been identified. In addition to those familiar reports, AIMAH has been described in MEN-1 with a frequency between 6% (Burgess et al, 1996) and 21% (Skogseid et al, 1992), and in rare cases of Gs alpha subunit mutations (Weinstein et al, 1991;Fragoso et al, 2003) or activating mutations of the ACTH receptor (MC2R) (Swords et al, 2002):  Gs alpha-subunit mutations -an activating mutation in the gene of the Gsa subunit of G-protein coupled receptors (stimulatory guanine nucleotide-binding protein, Gs) leads to constitutive activation of cAMP. These mutations may be responsible not only for increased production of cortisol but also for increased proliferation and consequently the formation of adrenal nodules (Weinstein et al, 1991;Fragoso et al, 2003).…”

Section: Aimah Pathogenesismentioning

confidence: 79%

“…These mutations may be responsible not only for increased production of cortisol but also for increased proliferation and consequently the formation of adrenal nodules (Weinstein et al, 1991;Fragoso et al, 2003).  MEN1 -In patients with multiple endocrine neoplasia syndrome type 1 (MEN1) caused by mutations in the the tumor suppressor gene menin, together with the more frequent endocrine tumors that are characteristic of the syndrome, adrenocortical adenomas or macronodular bilateral hyperplasias may also occur (Burgess et al, 1996;Skogseid et al, 1992).…”

Section: Aimah Pathogenesismentioning

confidence: 99%

Adrenal Cortex Tumors and Hyperplasias

Pignatelli¹

2011

Contemporary Aspects of Endocrinology

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“…Whereas the association of the MEN1 syndrome with adrenal tumors was reported to be a relatively uncommon feature with an incidence of adrenal tumors of 9.6% (26), other groups found adrenal lesions in 36-40% of MEN1 patients by computed tomography scan examination (1,27,28). Bilateral adrenal enlargement occurred in 21% of all patients.…”

Section: Discussionmentioning

confidence: 99%

Multiple endocrine neoplasia type 1 gene expression is normal in sporadic adrenocortical tumors

Zwermann

Beuschlein²,

Mora³

et al. 2000

European Journal of Endocrinology

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Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder with neoplasia of the anterior pituitary, the parathyroid, the endocrine pancreas and other endocrine tissues including the adrenal cortex. The tumor-suppressor gene causing this disease was identified at the gene locus 11q13. We recently reported that adrenocortical carcinomas frequently show loss of heterozygosity (LOH) of 11q13, but do not contain point mutations within the MEN1-coding region. To investigate whether reduced gene expression (for example by mutations within the MEN1 promoter) may contribute to the tumorigenesis of sporadic adrenocortical tumors, 24 adrenocortical specimen were studied by Northern blot analysis. This series included six adrenocortical carcinomas, four cortisol-producing adenomas, six aldosterone-producing adenomas, three endocrine-inactive adenomas and six normal adrenal glands. The presence of LOH of 11q13 was investigated using five polymorphic microsatellite markers (D11S956, PYGM, D11S4939, D11S4946 and D11S987) close to the MEN1 gene. Poly-A mRNA was hybridized with a PCR-generated cDNA probe of the MEN1 gene, a cDNA of the former MEN1 candidate gene phospholipase (PLC) b3 and a mouse b-actin cDNA for normalization. LOH of 11q13 was detected in five out of six carcinomas and two inactive adenomas, but in none of the hormone-producing adenomas. Compared with normal adrenals (100 Ϯ 6.5%, mean Ϯ S.E.M.) MEN1 mRNA in adrenocortical tumors was expressed in similar amounts (carcinomas 109 Ϯ 11%, cortisolproducing adenomas 131 Ϯ 10%, aldosterone-producing adenomas 113 Ϯ 13%, endocrine-inactive adenomas 111 Ϯ 2%) with the exception of one adrenocortical carcinoma with low MEN1 mRNA expression (66%). PLCb3 mRNA expression showed a variable pattern without reaching significant differences between the groups. We conclude that since mRNA levels were unaltered in the majority of tumors, mutations of the MEN1 gene causing altered gene transcription is unlikely to be a major pathogenic factor of sporadic adrenocortical tumors.

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Adrenal Lesions in a Large Kindred With Multiple Endocrine Neoplasia Type 1

Cited by 98 publications

References 10 publications

The appearance of the adrenal glands on computed tomography in multiple endocrine neoplasia type 1

The appearance of the adrenal glands on computed tomography in multiple endocrine neoplasia type 1

Adrenal Cortex Tumors and Hyperplasias

Multiple endocrine neoplasia type 1 gene expression is normal in sporadic adrenocortical tumors

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