The online version of this article (doi:10.1186/1477-7819-10-218) contains supplementary material, which is available to authorized users.
All authors declare no competing interest.
SH and MK carried out the MR examinations and drafted the manuscript. FH performed genetic analysis and drafted the manuscript. JJ and EC performed physical examinations and patient follow-up and drafted the manuscript. All authors read and approved the final manuscript.
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.
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.
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.
Burnichon N, Vescovo L, Amar L, Libe R, deReynies A, Venisse A, Jouanno E, Laurendeau I, Parfait B, Bertherat J, Plouin PF, Jeunemaitre X, Favier J, Gimenez-Roqueplo AP: Integrative genomic analysis reveals somatic mutations in pheochromocytoma and paraganglioma. Hum Mol Genet. 2011, 20: 3974-3985. 10.1093/hmg/ddr324. CrossRefPubMed
Bikhazi PH, Roeder E, Attaie A, Lalwani AK: Familial paragangliomas: the emerging impact of molecular genetics on evaluation and management. Am J Otol. 1999, 20: 639-643. PubMed
Hensen EF, Siemers MD, Jansen JC, Corssmit EP, Romijn JA, Tops CM, van der Mey AG, Devilee P, Cornelisse CJ, Bayley JP, Vriends AH: Mutations in SDHD are the major determinants of the clinical characteristics of Dutch head and neck paraganglioma patients. Clin Endocrinol (Oxf). 2011, 75: 650-655. 10.1111/j.1365-2265.2011.04097.x. CrossRef
van den Berg R, Verbist BM, Mertens BJ, van der Mey AG, van Buchem MA: Head and neck paragangliomas: improved tumor detection using contrast-enhanced 3D time-of-flight MR angiography as compared with fat-suppressed MR imaging techniques. AJNR Am J Neuroradiol. 2004, 25: 863-870. PubMed
Zanin L, Rossi G, Poletti A, Piotto A, Chiesura-Corona M, Pessina AC: Necrosis of a phaeochromocytoma associated with spontaneous remission of diabetes and hypertension. Clin Endocrinol (Oxf). 1993, 39: 613-617. 10.1111/j.1365-2265.1993.tb02417.x. CrossRef
Yamamoto K, Hanada R, Kikuchi A, Ichikawa M, Aihara T, Oguma E, Moritani T, Shimanuki Y, Tanimura M, Hayashi Y: Spontaneous regression of localized neuroblastoma detected by mass screening. J Clin Oncol. 1998, 16: 1265-1269. PubMed
Nakagawara A, Nakamura Y, Ikeda H, Hiwasa T, Kuida K, Su MS, Zhao H, Cnaan A, Sakiyama S: High levels of expression and nuclear localization of interleukin-1 beta converting enzyme (ICE) and CPP32 in favorable human neuroblastomas. Cancer Res. 1997, 57: 4578-4584. PubMed
Eggert A, Grotzer MA, Ikegaki N, Liu XG, Evans AE, Brodeur GM: Expression of the neurotrophin receptor TrkA down-regulates expression and function of angiogenic stimulators in SH-SY5Y neuroblastoma cells. Cancer Res. 2002, 62: 1802-1808. PubMed
Lindvall P, Brannstrom T: Spontaneous regression of two putative supratentorial haemangioblastomas in one patient. Acta Neurochir (Wien ). 2008, 150: 73-76. 10.1007/s00701-007-1453-1. CrossRef
- Case of spontaneous regression of carotid body tumor in a SDHD mutant: a discussion on potential mechanisms based on a review of the literature
Jeroen C. Jansen
Eleonora P. M. van der Kleij-Corssmit
Frederik J. Hes
Mark C. Kruit
- BioMed Central
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