Abstract
Metastases appear in approximately 10% of patients with pheochromocytoma. There is no predictive marker of malignancy. The aim is to describe clinical course of patients with malignant pheochromocytoma and to identify predictive features of malignancy. The method involves retrospective analysis of patients files diagnosed with malignant pheochromocytoma at our institution between January 1, 1980 and December 31, 2008. We identified 16 patients with malignant pheochromocytoma. There were more men than women (10/6). Mean age of patients at time of diagnosis was 37.75-year-old. Time of occurence of metastases ranged from 0 to 22 years after first diagnosis of pheochromocytoma. The mean size of the primary tumor was 12.1 cm. High levels of chromogranin A at the time of diagnosis were associated with the presence of metastases. The pheochromocytoma of the adrenal gland scoring scale (PASS) histological evaluation in adrenal primary tumors was above four in all cases but one. All patients had initial surgery, followed in most cases by palliative therapy: chemotherapy (streptozocin, cyclophosphamide-vincristine-dacarbazine, thalidomide, imatinib, everolimus) or 131I-MIBG; only the latter had replicable encouraging response evaluation criteria in solid tumor response rates. We observed a 10-year survival rate of 50% after initial diagnosis of pheochromocytoma, and 25% after diagnosis of metastasis. Metastasis can occur very late after the initial diagnosis of pheochromocytoma. High chromogranin A levels may be associated with the presence of metastases and poor prognosis. Histological adrenal PASS higher than 4 appears to be suggestive of malignancy. The best therapeutic approach remains to be established.
Similar content being viewed by others
References
H. Ahlman, Ann. N. Y. Acad. Sci. 1073, 449–464 (2006)
R. Adjalle, P.F. Plouin, K. Pacak, H. Lehnert, Horm. Metab. Res. 41, 687–696 (2009)
G. Eisenhofer, M.M. Walther, T.T. Huynh, L. Sheng-Ting et al., J. Clin. Endocrinol. Metab. 86, 1999–2008 (2001)
P.L.M. Dahia, Curr. Opin. Oncol. 18, 1–8 (2006)
A. Cascon, E. Lopez-Jimenez, I. Landa, S. Leskela et al., Horm. Metab. Res. 41, 672–675 (2009)
L. Amar, E. Baudin, N. Burnichon, S. Peyrard et al., J. Clin. Endocrinol. Metab. 92, 3822–3828 (2007)
B. Rose, K.K. Matthay, D. Price, J. Huberty et al., Cancer 98, 239–248 (2003)
L.D. Thompson, Am. J. Surg. Pathol. 26, 551–566 (2002)
H. John, W.H. Ziegler, D. Hauri, P. Jaeger, Urology 53, 679–683 (1999)
E. Van der Harst, H.A. Bruining, H. Jaap Bonjer, F. van der Ham et al., J. Pathol. 191, 175–180 (2000)
G.D. Hill, V. Pace, E. Persohn, C. bresser et al., Endocr. Pathol. 14, 81–91 (2003)
E. van der Harst, W.W. de Herder, R.R. de Krijger, H.A. Bruining et al., Eur. J. Endocrinol. 147, 85–94 (2002)
F. Rao, H.R. Keiser, D.T. O’Connor, Hypertension 36, 1045–1052 (2000)
E. Grossrubatscher, P. Dalino, F. Vignati, M. Gambagorta et al., Clin. Endocrinol. 65, 287–293 (2006)
L. Giovanella, N. Squin, A. Ghelfo, L. Ceriani, Q. J. Nucl. Med. Mol. Imaging 50, 344–347 (2006)
C. Proye, M. Vix, A. Goropoulos, P. Kerlo, M. Lecomte-Houcke, J. Endocrinol. Invest. 15, 651–663 (1992)
E. Carlsen, Z. Abdullah, S.M. Kazmi, G. Kousparos, Horm. Metab. Res. 41, 715–719 (2009)
D. Wu, A.S. Tischler, R.V. Lloyd, R.A. DeLellis et al., Am. J. Surg. Pathol. 33, 599–608 (2009)
A. Khorram-Manesh, H. Ahlman, O. Nilsson, P. Friberg et al., J. Intern. Med. 258, 55–66 (2005)
C.A. Koch, Nat. Clin. Pract. Endocrinol. Metab. 5, 76–77 (2009)
H.J. Timmers, G. Eisenhofer, J.A. Carrasquillo, C.C. Chen et al., Clin. Endocrinol. 71, 11–17 (2008)
G. Eisenhofer, S.R. Bornstein, F.M. Brouwers, N.K. Cheung et al., Endocr. Relat. Cancer 11, 423–436 (2004)
J.T. Adler, G.Y. Meyer-Rochow, H. Chen, D.E. Benn et al., Oncologist 13, 779–793 (2008)
T. Scholz, G. Eisenhofer, K. Pacak, H. Dralle, H. Lehnert, J. Clin. Endocrinol. Metab. 92, 1217–1225 (2007)
D.J. Gross, E. Schlank, E. Ipp, Arch. Intern. Med. 145, 367–368 (1985)
S.E. Bates, C.Y. Shieh, L.A. Mickley, H.L. Dichek et al., J. Clin. Endocrinol. Metab. 73, 18–29 (1991)
M.R. Druce, G.A. Kaltsas, M. Fraenkel, D.J. Gross, A.B. Grossman, Horm. Metab. Res. 41, 697–702 (2009)
D.J. Gross, G. Munter, M. Bitan, T. Sigal et al., Endocr. Relat. Cancer 13, 535–540 (2006)
P. Therasse, S.G. Arbuck, E.A. Eisenhauer, J. Wanders et al., J. Natl. Cancer Inst. 92, 205–216 (2000)
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Szalat, A., Fraenkel, M., Doviner, V. et al. Malignant pheochromocytoma: predictive factors of malignancy and clinical course in 16 patients at a single tertiary medical center. Endocr 39, 160–166 (2011). https://doi.org/10.1007/s12020-010-9422-5
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12020-010-9422-5