Korean J Endocr Surg.  2011 Dec;11(4):269-275. 10.0000/kjes.2011.11.4.269.

Clinical Analysis of Pheochromocytoma and Abdominal Paragangliomas

Affiliations
  • 1Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. jhkim15@skku.edu
  • 2Department of Surgery, Konkuk University School of Medicine, Seoul, Korea.

Abstract

PURPOSE
We compared clinical characteristics between pheochromocytoma and abdominal paragangliomas and identified predictive factors of malignancy.
METHODS
Between November, 1995 and January, 2011, we retrospectively reviewed the medical records of 145 patients with pheochromocytoma and abdominal paraganglioma at Samsung Medical Center. We compared two tumors (pheochromocytoma vs abdominal paraganglioma) about a potential of hypersecretion of cathecholamines and identified predictive factors of malignancy by analysis of clinical characteristics, biochemical markers, tumor features. Their postoperative results were also evaluated.
RESULTS
This study included 103 (71%) pheochromocytomas and 42 (29%) abdominal paragangliomas. Eighty-six percent were benign and 14% were malignant. Patients with paraganglioma were more predominantly men and exhibited a higher malignancy rate (P<0.01) than pheochromocytoma patients. Most (95%) pheochromocytoma was hyperfunctional, but abdominal paraganglioma were hyperfunctional in 74%. There were no significant differences in biochemical markers between the pheochromocytoma and paraganglioma groupd. When compared with benign tumor, malignant tumors were significantly related with higher mean PASS (P<0.01) and higher 24-hour urinary VMA (P=0.02), but not with larger tumor size.
CONCLUSION
It is not easy to distinguish malignant from benign tumors by clinical characteristics and pathologic features in the management of pheochromocytoma and paraganglioma. We should keep in mind that abdominal paraganglioma can be also hyperfunctional in many pheochromocytoma patients and has a higher risk of malignancy.

Keyword

Adrenal gland; Malignant pheochromocytoma; Paraganglioma

MeSH Terms

Adrenal Glands
Biomarkers
Humans
Male
Medical Records
Paraganglioma*
Pheochromocytoma*
Retrospective Studies
Biomarkers

Reference

1.Kim JC., Yoon JH., Jegal YJ. A clinical analysis of pheochromocytoma. J Korean Surg Soc. 1997. 52:662–70.
Article
2.van der Harst E., Bruining HA., Jaap Bonjer H., van der Ham F., Dinjens WN., Lamberts SW, et al. Proliferative index in phaeochromocytomas: does it predict the occurrence of metastases? J Pathol. 2000. 191:175–80.
Article
3.Tischler AS. Pheochromocytoma and extra-adrenal paraganglioma: updates. Arch Pathol Lab Med. 2008. 132:1272–84.
Article
4.Grossrubatscher E., Dalino P., Vignati F., Gambacorta M., Pugliese R., Boniardi M, et al. The role of chromogranin A in the management of patients with phaeochromocytoma. Clin Endocrinol (Oxf). 2006. 65:287–93.
Article
5.Raue F., Frank-Raue K., Grauer A. Multiple endocrine neoplasia type 2. Clinical features and screening. Endocrinol Metab Clin North Am. 1994. 23:137–56.
Article
6.Thompson LD. Pheochromocytoma of the Adrenal gland Scaled Score (PASS) to separate benign from malignant neoplasms: a clinicopathologic and immunophenotypic study of 100 cases. Am J Surg Pathol. 2002. 26:551–66.
7.Eisenhofer G., Bornstein SR., Brouwers FM., Cheung NK., Dahia PL., de Krijger RR, et al. Malignant pheochromocytoma: current status and initiatives for future progress. Endocr Relat Cancer. 2004. 11:423–36.
Article
8.Harari A., Inabnet WB 3rd. Malignant pheochromocytoma: a review. Am J Surg. 2011. 201:700–8.
Article
9.Scholz T., Eisenhofer G., Pacak K., Dralle H., Lehnert H. Clinical review: Current treatment of malignant pheochromocytoma. J Clin Endocrinol Metab. 2007. 92:1217–25.
10.Wen J., Li HZ., Ji ZG., Mao QZ., Shi BB., Yan WG. A decade of clinical experience with extra-adrenal paragangliomas of retroperitoneum: Report of 67 cases and a literature review. Urol Ann. 2010. 2:12–6.
Article
11.Hayes WS., Davidson AJ., Grimley PM., Hartman DS. Extra-adrenal retroperitoneal paraganglioma: clinical, pathologic, and CT findings. AJR Am J Roentgenol. 1990. 155:1247–50.
Article
12.Mahoney EM., Harrison JH. Malignant pheochromocytoma: clinical course and treatment. J Urol. 1977. 118:225–9.
Article
13.Dahia PL. Evolving concepts in pheochromocytoma and paraganglioma. Curr Opin Oncol. 2006. 18:1–8.
Article
14.Szalat A., Fraenkel M., Doviner V., Salmon A., Gross DJ. Malignant pheochromocytoma: predictive factors of malignancy and clinical course in 16 patients at a single tertiary medical center. Endocrine. 2011. 39:160–6.
Article
15.Strong VE., Kennedy T., Al-Ahmadie H., Tang L., Coleman J., Fong Y, et al. Prognostic indicators of malignancy in adrenal pheochromocytomas: clinical, histopathologic, and cell cycle/apoptosis gene expression analysis. Surgery. 2008. 143:759–68.
Article
16.Agarwal A., Mehrotra PK., Jain M., Gupta SK., Mishra A., Chand G, et al. Size of the tumor and pheochromocytoma of the adrenal gland scaled score (PASS): can they predict malignancy? World J Surg. 2010. 34:3022–8.
Article
17.Bertherat J., Gimenez-Roqueplo AP. New insights in the genetics of adrenocortical tumors, pheochromocytomas and paragangliomas. Horm Metab Res. 2005. 37:384–90.
Article
18.Feng F., Zhu Y., Wang X., Wu Y., Zhou W., Jin X, et al. Predictive factors for malignant pheochromocytoma: analysis of 136 patients. J Urol. 2011. 185:1583–90.
Article
19.Rao F., Keiser HR., O'Connor DT. Malignant pheochromocytoma. Chromaffin granule transmitters and response to treatment. Hypertension. 2000. 36:1045–52.
20.Ayala-Ramirez M., Feng L., Johnson MM., Ejaz S., Habra MA., Rich T, et al. Clinical risk factors for malignancy and overall survival in patients with pheochromocytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators. J Clin Endocrinol Metab. 2011. 96:717–25.
Article
21.Khorram-Manesh A., Ahlman H., Nilsson O., Friberg P., Odén A., Stenström G, et al. Long-term outcome of a large series of patients surgically treated for pheochromocytoma. J Intern Med. 2005. 258:55–66.
Article
22.Safford SD., Coleman RE., Gockerman JP., Moore J., Feldman JM., Leight GS Jr, et al. Iodine-131 metaiodobenzylguanidine is an effective treatment for malignant pheochromocytoma and paraganglioma. Surgery. 2003. 134:956–62.
23.Gedik GK., Hoefnagel CA., Bais E., Olmos RA. 131I-MIBG therapy in metastatic phaeochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging. 2008. 35:725–33.
Article
24.Grogan RH., Mitmaker EJ., Duh QY. Changing paradigms in the treatment of malignant pheochromocytoma. Cancer Control. 2011. 18:104–12.
Article
25.Ahlman H. Malignant pheochromocytoma: state of the field with future projections. Ann N Y Acad Sci. 2006. 1073:449–64.
Article
26.Rose B., Matthay KK., Price D., Huberty J., Klencke B., Norton JA, et al. High-dose 131I-metaiodobenzylguanidine therapy for 12 patients with malignant pheochromocytoma. Cancer. 2003. 98:239–48.
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