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. 2014;66(4):437-9.
doi: 10.5173/ceju.2013.04.art12. Epub 2014 Jan 27.

A spontaneous paraganglioma-pheochromocytoma syndrome

Sadegh Toutounchi  1 , Ryszard Pogorzelski  1 , Maciej Siński  2 , Izabela Loń  2 , Lukasz Zapała  3 , Patryk Fiszer  1 , Ewa Krajewska  1 , Maciej Skórski  1
Affiliations

A spontaneous paraganglioma-pheochromocytoma syndrome

Sadegh Toutounchi et al. Cent European J Urol. 2014.

Abstract

We present a case of a 40-year old woman diagnosed with a four-place spontaneous paraganglioma-pheochromocytoma syndrome, which was treated surgically. The presence of the succinate dehydrogenase complex subunit D (SDHD) mutation that causes the pheochromocytoma was confirmed but no mutations in the family members were found. After the excision of the paragangliomas located in the areas of the division of carotid arteries, and mediastinum, as well as a tumor on the left site of the celiac trunk, the patient remains asymptomatic and is regularly followed-up.

Keywords: SDHD mutation; paraganglioma; pheochromocytoma.

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Figures

Figure 1
Figure 1
The extraadrenal lesion and its relation to the tumor of the left adrenal gland in CT scan (marked with white arrows).
Figure 2
Figure 2
The postoperative specimen.
See this image and copyright information in PMC

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