Abstract
An extra-adrenal pheochromocytoma is known as a paraganglioma. This report describes a patient with a rare primary functioning hepatic paraganglioma that resulted in hypertension. Computed tomography showed a highly vascular lesion located in segment 6 of the liver; it measured 6 x 5.5 cm. A right hemihepatectomy was subsequently performed; this was followed by an uneventful recovery and the disappearance of hypertension. The imaging characteristics and therapeutic principles of this rare tumor were gleaned from a review of the literature. Identification of this malignant tumor or possible recurrence is difficult, so longterm follow-up is recommended.
Similar content being viewed by others
References
Whalen RK, Althausen AF, Daniels G. Extra-adrenal pheochromocytoma.J Urol. 1992; 147: 1–10.
Jaeck D, Paris F, Welsch M, et al. Primary hepatic pheochromocytoma: a second case.Surgery. 1995; 117: 586–590.
Reif MC, Hanto DW, Moulton JS, Alspaugh JP, Bejarano P. Primary hepatic pheochromocytoma?Am J Hypertens. 1996; 9: 1040–1043.
D’Errico A, Pierangeli F, Fiorentino M, Altimari A, Grigioni WF. Primary paraganglioma strictly confined to the liver and mimicking hepatocellular carcinoma.Am J Surg Pathol. 2002; 26: 945–949.
Tanaka S, Ito T, Tomoda J, Higashi T, Yamada G, Tsuji T. Malignant pheochromocytoma with hepatic metastasis diagnosed 20 years after resection of the primary adrenal lesion.Intern Med. 1993, 32: 789–794.
Ichikawa T, Federle MP, Grazioli L, Madariag J, Nalesnik M, Marsh W. Fibrolamellar hepatocellular carcinoma: imaging and pathologic finding in 31 recent cases.Radiology. 1999; 213: 352–361.
Mozley PD, Kim CK, Mohsin J, Jatlow A, Gosfield E III, Alavi A. The efficacy of iodine-123-MIBG as a screening test for pheochromocytoma.J Nucl Med. 1994, 35: 1138–1144.
Eisenhofer G, Bornstein SR, Brouwers FM, et al. Malignant pheochromocytoma: current status and initiatives for future progress.Endocr Relat Cancer. 2004; 11: 423–436.
Ilias I, Pacak K. Anatomical and functional imaging of metastatic pheochromocytoma.Ann N Y Acad Sci. 2004; 1018: 495–504.
Pacak K, Eisenhofer G, Carrasquillo JA, Chen CC, Li ST, Coldstein DS. 6-[18F] fluorodopamine positron emission tomographic (PET) scanning for diagnostic localization of pheochromocytoma.Hypertension. 2001; 38: 6–8.
Quiros RM, Kim AW, Maxhimer J, Gattuso P, Xu X, Prinz RA. Differential heparanase-1 expression in malignant and benign pheochromocytomas.J Surg Res. 2002; 108: 44–50.
Gimenez-Roqueplo AP, Favier J, Rustin P, et al. Mutations in the SDHB gene are associated with extra-adrenal and/or malignant phaeochromocytomas.Cancer Res. 2003; 63: 5615–5621.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Chang, H., Xu, L. & Mu, Q. Primary functioning hepatic paraganglioma: A case report. Adv Therapy 23, 817–820 (2006). https://doi.org/10.1007/BF02850323
Issue Date:
DOI: https://doi.org/10.1007/BF02850323