Growth hormone and risk for cardiac tumors in Carney complex

Bandettini, W. Patricia; Karageorgiadis, Alexander S.; Sinaii, Ninet; Rosing, Douglas R.; Sachdev, Vandana; Schernthaner-Reiter, Marie Helene; Gourgari, Evgenia; Papadakis, Georgios Z.; Keil, Meg; Lyssikatos, Charalampos; Carney, J. Aidan; Arai, Andrew E.; Lodish, Maya; Stratakis, Constantine A.

doi:10.1530/erc-16-0246

Cited by 9 publications

(9 citation statements)

References 45 publications

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“…Majority of the 140 analyzed patients were diagnosed with sporadic pituitary gland adenoma. In five patients, the symptoms of acromegaly resulted from rare syndromes, such as familial isolated pituitary adenoma (FIPA)—two cases [ 11 , 12 ]; Carney complex, comprising, among others, heart and cutaneous myxomas and pituitary gland adenoma—one case [ 13 ]; ectopic acromegaly due to bronchial neuroendocrine tumour secreting growth hormone-releasing hormone (GH-RH)—one case [ 7 ]; MEN1, characterized by multiple endocrine tumours—one case [ 1 , 10 , 14 ]. …”

Section: Methodsmentioning

confidence: 99%

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Heart in Acromegaly: The Echocardiographic Characteristics of Patients Diagnosed with Acromegaly in Various Stages of the Disease

Popielarz-Grygalewicz

Gąsior

Konwicka

et al. 2018

International Journal of Endocrinology

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To determine whether the echocardiographic presentation allows for diagnosis of acromegalic cardiomyopathy. 140 patients with acromegaly underwent echocardiography as part of routine diagnostics. The results were compared with the control group comprising of 52 age- and sex-matched healthy volunteers. Patients with acromegaly presented with higher BMI, prevalence of arterial hypertension, and glucose metabolism disorders (i.e., diabetes and/or prediabetes). In patients with acromegaly, the following findings were detected: increased left atrial volume index, increased interventricular septum thickness, increased posterior wall thickness, and increased left ventricular mass index, accompanied by reduced diastolic function measured by the following parameters: E'med., E/E', and E/A. Additionally, they presented with abnormal right ventricular systolic pressure. All patients had normal systolic function measured by ejection fraction. However, the values of global longitudinal strain were slightly lower in patients than in the control group; the difference was statistically significant. There were no statistically significant differences in the size of the right and left ventricle, thickness of the right ventricular free wall, and indexed diameter of the ascending aorta between patients with acromegaly and healthy volunteers. None of 140 patients presented systolic dysfunction, which is the last phase of the so-called acromegalic cardiomyopathy. Some abnormal echocardiographic parameters found in acromegalic patients may be caused by concomitant diseases and not elevated levels of GH or IGF-1 alone. The potential role of demographic parameters like age, sex, and/or BMI requires further research.

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Section: Methodsmentioning

confidence: 99%

“…Carney complex, comprising, among others, heart and cutaneous myxomas and pituitary gland adenoma—one case [ 13 ];…”

Section: Methodsmentioning

confidence: 99%

Heart in Acromegaly: The Echocardiographic Characteristics of Patients Diagnosed with Acromegaly in Various Stages of the Disease

Popielarz-Grygalewicz

Gąsior

Konwicka

et al. 2018

International Journal of Endocrinology

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“…CNC-associated cardiac myxomas constitute the predominant cause of death among these patients; they tend to be multiple and may occur as early as at birth 4 . Among patients with CNC, recurrence of myxomas has been associated with excess of growth hormone secretion 12 .…”

Section: Introductionmentioning

confidence: 99%

Predicting the risk of cardiac myxoma in Carney complex

Pitsava

Zhu

Sundaram

et al. 2021

Genetics in Medicine

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Purpose Carney complex (CNC), is an autosomal dominant multiple neoplasia and lentiginosis syndrome. We aimed to identify risk factors associated with the occurrence and recurrence of cardiac myxomas, the predominant cause of death in CNC patients. Methods Patients with CNC were monitored prospectively between 1995 and 2020 for the development of cardiac myxomas. Results Of the 319 patients studied, 136 (42.6%) developed myxomas. The mean age at diagnosis was 28.7 ± 16.6 years in females and 25.0 ± 16.4 years in males. By age 30, 35% of females and 45% of males had at least one myxoma. The CNC-related lesions: lentigines, cutaneous, mucosal, or breast myxomas, thyroid nodules, pituitary adenoma, and schwannoma were significantly more frequent (all p<0.05) among patients with myxomas. Forty-four percent of patients had recurrences; nearly all within the first 8 and 16 years for males and females, respectively. Recurrences were more common in females. Conclusion This is the largest study to date and provides the first-time risk estimates by age and gender for cardiac myxomas in CNC patients. Cardiac myxomas are common by age 30 and often recur, especially in women, but the risk drops in 10 to 20 years. These findings may guide the patient counseling, screening intervals and surgical approaches.

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“…Cardiac myxomas are the leading cause of mortality in Carneys complex patients who, in addition, often develop growth hormone (GH) excess. 15 There was a single recurrence after LAM tumor excision after 2 years which was excised by redo surgery in our series. But probabilty of recurrent tumor due to local tissue tumor seeding or incomplete removal can not be ruled out.…”

Section: International Journal Of Contemporary Medical Researchmentioning

confidence: 53%

Surgical Treatment of Cardiac Tumors: A Single Institutional Experience Over 12 Years

Dasbaksi¹,

Hossain²,

Haldar³

et al. 2020

IJCMR

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Introduction: Tumors of the heart represent an exceedingly rare entity in cardiac surgery and literature regarding management and outcome is less in comparison to other fields of cardiac surgery. 12 years of our experience in both diagnosis and optimal surgical treatment of this small but rare collection of patients was formed into a detailed analysis of patient prognosis, mean survival and risk of tumor relapse matched to the corresponding pathology. The overall objective of the present study was a thorough characterization of both primary cardiac tumor or tumor like mass and secondary malignant tumor mass in cardiac chambers, their nature as well as age and gender distribution and management. Material and methods: 17 patients with cardiac tumors, who underwent open-heart surgery at Medical College and Hospitals, Kolkata, for tumor excision between 2007 and 2019 were analyzed retrospectively. Mean follow-up was from 11 to 1 years. Results: There were 2 males and 15 female patients ranging in age from 7 years to 60 years, median age being 47 years. 12 of these tumors were primary left atrial myxoma, 2 were right atrial myxoma, 1 was right ventricular fibroma, 1 was intravenous extension into right atrium of renal cell carcinoma and 1 was multiple inflammatory pseudo tumors in left ventricle. Overall operative survival was 88.3%. Operative mortality was 11.7%. Conclusion: Cardiac tumors remain challenging in the clinical setting. Early operation is recommended after echocardiographic diagnosis as such patients can have sudden death or severe cardiac failure during preoperative waiting period. Follow up should be maintained based upon the histopathological diagnosis.

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Growth hormone and risk for cardiac tumors in Carney complex

Cited by 9 publications

References 45 publications

Heart in Acromegaly: The Echocardiographic Characteristics of Patients Diagnosed with Acromegaly in Various Stages of the Disease

Heart in Acromegaly: The Echocardiographic Characteristics of Patients Diagnosed with Acromegaly in Various Stages of the Disease

Predicting the risk of cardiac myxoma in Carney complex

Surgical Treatment of Cardiac Tumors: A Single Institutional Experience Over 12 Years

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