Acromegaly in Carney complex

Cuny, Thomas; Mac, Thi Thom; Romanet, Pauline; Dufour, Henry; Morange, Isabelle; Albarel, F.; Lagarde, Arnaud; Castinetti, Frédéric; Graillon, Thomas; North, M.O.; Barlier, Anne; Brue, Thierry

doi:10.1007/s11102-019-00974-8

Cited by 22 publications

(19 citation statements)

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“…The incidence of GH-secreting pituitary tumors has been estimated to be less than 15%, although 80% of cases have asymptomatic increases in GH and IGF1 levels [7]. This is likely due to a certain degree of somatotroph hyperplasia which suggests that PRKAR1A mutations may lead to GH hyperplasia, which in turn may sometimes result, albeit not systematically, in the development of a GH-secreting micro or macro adenoma [8].…”

Section: Discussionmentioning

confidence: 99%

Acromegaly as an Expression of a Rare Disease: Description of an Unusual Clinical Case of Carney Complex

Costa¹,

Mercuri²,

D’Amico³

et al. 2020

CRCM

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Introduction: The Carney complex (CC) is an autosomal dominant syndrome, characterized by multiple endocrine neoplasms, pigmented skin lesions, endocrine hyper-activity and myxomas. Case study: C. E., 71 years old, was addressed to our center in August 1994, following the finding of a sellar enlargement in a skull radiography performed after an accident. A pituitary tomography showed the presence of a lesion extended up to the optic chiasm, a macroadenoma that was treated with transsphenoidal endoscopic surgery. A histological diagnosis of a "GH secreting macroadenoma" was performed. An echocardiographic study highlighted an atrial isolated myxoma that was subsequently removed. A contrast-enhanced abdominal magnetic resonance imaging (MRI) excluded adrenal lesions. In 2001, in relation to relapse of Acromegaly, the patient started therapy with somatostatin analogues which was replaced, in 2008, by Pegvisomant to obtain biochemical control of the disease. Conclusions: At the time of initial screening, it is important not to underestimate the possibility that certain clinical features fall within the context of more complex syndromes. The knowledge of how Acromegaly presents itself in relation to CC and its associated characteristics such as cardiac myxoma or typical skin lesions, will help clinicians to timely diagnose this rare disease and treat it appropriately.

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Section: Discussionmentioning

confidence: 99%

Acromegaly as an Expression of a Rare Disease: Description of an Unusual Clinical Case of Carney Complex

Costa¹,

Mercuri²,

D’Amico³

et al. 2020

CRCM

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“…У найбільшому огляді описано 57 випадків акромегалії в пацієнтів із КК (24 чоловіки і 33 жінки), середній вік на момент установлення діагнозу становив 28 ± 12 років (для спорадичної акромегалії -40-48 років). Приблизно половину випадків становили мікроаденоми, тоді як в інших випадках акромегалії переважають макроаденоми [5].…”

Section: ураження гіпофізаunclassified

Carney complex manifested itself in pituitary somatotropinoma and Cushing syndrome: a clinical case and current understanding of the problem

Черенько

Черенько²,

Martsinik

2021

Mìžnarodnij endokrinologìčnij žurnal

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Наявність у пацієнта декількох ендокринних захворювань, особливо коли йдеться про надлишок гормонів, завжди має викликати в лікаря підозру щодо синдромів множинної ендокринної неоплазії та інших генетичних синдромів, які викликають поліорганні пухлини. Клінічний випадок Жінка 39 років звернулася за консультацією з клінічними ознаками гіперкортицизму (місяцеподібне обличчя, абдомінальне ожиріння, артеріальна гіпертензія і вторинна аменорея). Лабораторно був підтверджений синдром Кушинга АКТГ (адренокортикотроп

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“…L'ipersecrezione di GH in questi pazienti, tuttavia, non è sempre indicativa di un AI rilevabile alla risonanza magnetica (RMN). L'acromegalia clinicamente evidente si osserva, infatti, in ∼ 10% dei casi CNC con imaging positivo per una lesione ipofisaria e rappresenta la prima manifestazione di malattia nel 7% dei casi [2]. Il par-G. Occhi gianluca.occhi@unipd.it ticolare profilo ormonale dei pazienti CNC può dipendere da iperplasia ipofisaria, tipicamente delle cellule mammosomatotrope che, in alcuni casi, precede la formazione dell'AI.…”

Section: Acromegaliaunclassified