Carney’s triad in an adult male from a tertiary care center in India: a case report

Abstract Background Carney’s triad is a rare syndrome comprising gastrointestinal stromal tumor, extra-adrenal paraganglioma, and pulmonary chondroma along with newer additions of adrenal adenoma and esophageal leiomyoma. The triad is completely manifest in only 25–30% cases, with most patients pres...

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Autores principales:	Ghazal Tansir, Nihar Ranjan Dash, Saurabh Galodha, Prasenjit Das, Shamim Ahmed Shamim, Sameer Rastogi
Formato:	article
Lenguaje:	EN
Publicado:	BMC 2021
Materias:	Wild-type GIST Chondroma Adrenal tumor Gastrectomy Medicine R
Acceso en línea:	https://doaj.org/article/031d80f49720497589fd007fb7d3054f
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spelling	oai:doaj.org-article:031d80f49720497589fd007fb7d3054f2021-11-21T12:39:45ZCarney’s triad in an adult male from a tertiary care center in India: a case report10.1186/s13256-021-03149-x1752-1947https://doaj.org/article/031d80f49720497589fd007fb7d3054f2021-11-01T00:00:00Zhttps://doi.org/10.1186/s13256-021-03149-xhttps://doaj.org/toc/1752-1947Abstract Background Carney’s triad is a rare syndrome comprising gastrointestinal stromal tumor, extra-adrenal paraganglioma, and pulmonary chondroma along with newer additions of adrenal adenoma and esophageal leiomyoma. The triad is completely manifest in only 25–30% cases, with most patients presenting with two out of three parts of the syndrome. Wild-type succinate-dehydrogenase-deficient gastric gastrointestinal stromal tumor forms the most common component of Carney’s triad and is usually multicentric and multifocal. It usually demonstrates indolent behavior and resistance to imatinib; hence, the management remains predominantly surgical. Pulmonary chondromas are commonly unilateral and multiple with slow-growing nature, which allows for conservative management. Adrenocortical adenomas are found in 20% of patients and are usually detected as incidentalomas. Case presentation A 49-year-old Asian male presented with upper gastrointestinal bleed and was diagnosed with multiple gastric succinate-dehydrogenase-deficient gastrointestinal stromal tumors. On evaluation, he was found to have left pulmonary chondroma and non-secretory adrenal adenoma, thus completing the Carney’s triad. He underwent surgery with sleeve gastrectomy and excision of the antral tumor nodule, while the adrenal and pulmonary tumors have been under close follow-up. Conclusion Literature regarding Carney’s triad is scarce, especially from the Indian setting. Our report aims to highlight the various manifestations of this syndrome with emphasis on management of wild-type succinate-dehydrogenase-deficient gastrointestinal stromal tumor. Radical gastric surgeries do not offer a survival advantage in this condition; hence, more conservative modalities of resection can be adopted.Ghazal TansirNihar Ranjan DashSaurabh GalodhaPrasenjit DasShamim Ahmed ShamimSameer RastogiBMCarticleWild-type GISTChondromaAdrenal tumorGastrectomyMedicineRENJournal of Medical Case Reports, Vol 15, Iss 1, Pp 1-6 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Wild-type GIST Chondroma Adrenal tumor Gastrectomy Medicine R
spellingShingle	Wild-type GIST Chondroma Adrenal tumor Gastrectomy Medicine R Ghazal Tansir Nihar Ranjan Dash Saurabh Galodha Prasenjit Das Shamim Ahmed Shamim Sameer Rastogi Carney’s triad in an adult male from a tertiary care center in India: a case report
description	Abstract Background Carney’s triad is a rare syndrome comprising gastrointestinal stromal tumor, extra-adrenal paraganglioma, and pulmonary chondroma along with newer additions of adrenal adenoma and esophageal leiomyoma. The triad is completely manifest in only 25–30% cases, with most patients presenting with two out of three parts of the syndrome. Wild-type succinate-dehydrogenase-deficient gastric gastrointestinal stromal tumor forms the most common component of Carney’s triad and is usually multicentric and multifocal. It usually demonstrates indolent behavior and resistance to imatinib; hence, the management remains predominantly surgical. Pulmonary chondromas are commonly unilateral and multiple with slow-growing nature, which allows for conservative management. Adrenocortical adenomas are found in 20% of patients and are usually detected as incidentalomas. Case presentation A 49-year-old Asian male presented with upper gastrointestinal bleed and was diagnosed with multiple gastric succinate-dehydrogenase-deficient gastrointestinal stromal tumors. On evaluation, he was found to have left pulmonary chondroma and non-secretory adrenal adenoma, thus completing the Carney’s triad. He underwent surgery with sleeve gastrectomy and excision of the antral tumor nodule, while the adrenal and pulmonary tumors have been under close follow-up. Conclusion Literature regarding Carney’s triad is scarce, especially from the Indian setting. Our report aims to highlight the various manifestations of this syndrome with emphasis on management of wild-type succinate-dehydrogenase-deficient gastrointestinal stromal tumor. Radical gastric surgeries do not offer a survival advantage in this condition; hence, more conservative modalities of resection can be adopted.
format	article
author	Ghazal Tansir Nihar Ranjan Dash Saurabh Galodha Prasenjit Das Shamim Ahmed Shamim Sameer Rastogi
author_facet	Ghazal Tansir Nihar Ranjan Dash Saurabh Galodha Prasenjit Das Shamim Ahmed Shamim Sameer Rastogi
author_sort	Ghazal Tansir
title	Carney’s triad in an adult male from a tertiary care center in India: a case report
title_short	Carney’s triad in an adult male from a tertiary care center in India: a case report
title_full	Carney’s triad in an adult male from a tertiary care center in India: a case report
title_fullStr	Carney’s triad in an adult male from a tertiary care center in India: a case report
title_full_unstemmed	Carney’s triad in an adult male from a tertiary care center in India: a case report
title_sort	carney’s triad in an adult male from a tertiary care center in india: a case report
publisher	BMC
publishDate	2021
url	https://doaj.org/article/031d80f49720497589fd007fb7d3054f
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Carney’s triad in an adult male from a tertiary care center in India: a case report

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