Case of spontaneous regression of carotid body tumor in a SDHD mutant: a discussion on potential mechanisms based on a review of the literature.
BACKGROUND
Head and neck paragangliomas are tumors associated with the parasympathetic nerve system and typically show an indolent growth pattern. Therefore a conservative management strategy is considered in selected cases.


RESULTS
We present a case of a female patient who presented in 2003 with bilateral carotid body tumors and a tympanic tumor, associated with a mutation in the succinate dehydrogenase -sub-unit-D (SDHD). She was operated on the right carotid body tumor and the tympanic tumor. Thereafter the follow-up was performed with MR examinations at 2-year intervals. After an initial stable phase, over the last 3 years a spontaneous near-total regression of the contralateral carotid body tumor was observed, with only subtle rest-abnormalities visible in 2011.


CONCLUSIONS
The present case underlines the indolent growth pattern of head and neck paragangliomas and for the first time describes a rare manifestation of spontaneous regression of a carotid body tumor. The literature was reviewed to discuss this phenomenon.
Department of Radiology, Leiden University Medical Center, Albinusdreef 2, Leiden, ZA 2333, The Netherlands. S.Hammer@lumc.nl
Sebastiaan
S
Hammer
Jeroen C
JC
Jansen
Eleonora P M
EP
van der Kleij-Corssmit
Frederik J
FJ
Hes
Mark C
MC
Kruit
D000328:Adult; D002345:Carotid Body Tumor / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D005260:Female; D006801:Humans; D009154:Mutation; D009365:Neoplasm Regression, Spontaneous; D013385:Succinate Dehydrogenase / Q000235:genetics*
C491545:SDHD protein, human; D013385:Succinate Dehydrogenase