Paragangliomas in Carney–Stratakis Syndrome
2019 ◽
Vol 51
(07)
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pp. 437-442
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Keyword(s):
AbstractCarney-Stratakis Syndrome (CSS) comprises of paragangliomas (PGLs) and gastrointestinal stromal tumors (GISTs). Several of its features overlap with Carney Triad (CT) - PGLs, GISTs, and pulmonary chondromas. CSS has autosomal dominant inheritance, incomplete penetrance, and greater relative frequency of PGL over GISTs. The PGLs in CSS are multicentric and GISTs are multifocal in all the patients, suggesting an inherited susceptibility and associating the two manifestations. In this review, we highlight the clinical, pathological, and molecular characteristics of CSS, along with its diagnostic and therapeutic implications.
2005 ◽
Vol 11
(10)
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pp. 3668-3677
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2018 ◽
Vol 31
(05)
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pp. 295-300
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2004 ◽
Vol 22
(14_suppl)
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pp. 8537-8537
2010 ◽
Vol 34
(1)
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pp. 53-64
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