Indian Journal of Urology 2007-Oct
Para aortic ganglioneuroma presenting as Cushing's syndrome.
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We present a case of an eight-year-old female presenting with four months history of progressive weight gain, short stature, obesity, mild acanthosis, moon facies and buffalo hump. Biochemically, low-dose and high-dose Dexamethasone tests were not suppressible, ACTH was raised and 24h urinary metanephrines were normal. The CECT scan showed a 3cm paraganglioma. Tumor was excised via 11(th) rib transcostal approach and the mass was found arising from the sympathetic chain. Histopathology was suggestive of ganglioneuroma positive for ACTH immunostaining.