| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH |
| |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Submitted on December 7, 2005
Accepted on March 9, 2006
Endocrinology and Diabetes Unit, Dept. of Medicine I, University Hospital Wuerzburg, Germany
* To whom correspondence should be addressed. E-mail: allolio_b{at}medizin.uni-wuerzburg.de.
Context: Adrenocortical carcinoma (ACC) is a rare and heterogeneous malignancy with incompletely understood pathogenesis and poor prognosis. Patients present with hormone excess (e.g. virilization, Cushing's syndrome) or a local mass effect (median tumor size at diagnosis > 10 cm). This paper reviews current diagnostic and therapeutic strategies in ACC.
Evidence Acquisition: Original articles and reviews were identified using a Pubmed search strategy (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi) covering the time period up until November 2005. The following search terms were used in varying combinations: adrenal, adrenocortical, cancer, carcinoma, tumor, diagnosis, imaging, treatment, radiotherapy, mitotane, cytotoxic, surgery.
Evidence synthesis: Tumors typically appear inhomogeneous in both computerized tomography and magnetic resonance imaging with necroses, irregular borders, and differ from benign adenomas by their low fat content. Hormonal analysis reveals evidence of steroid hormone secretion by the tumor in the majority of cases, even in seemingly hormonally inactive lesions. Histopathology is crucial for the diagnosis of malignancy and may also provide important prognostic information.
In stages I -III open surgery by an expert surgeon aiming at a R0 resection is the treatment of choice. Local recurrence is frequent, particularly after violation of the tumor capsule. Surgery plays also a role in local tumor recurrence and metastatic disease. In patients not amenable to surgery, mitotane (alone or in combination with cytotoxic drugs) remains the treatment of choice. Monitoring of drug levels (therapeutic range 14 - 20 mg/l) is mandatory for optimum results. In advanced disease, the most promising therapeutic options (etoposide, doxorubicin, cisplatin plus mitotane and streptozotocin plus mitotane) are currently compared in an international phase III trial (www.firm-act.org). Adjuvant treatment options after complete tumor removal (e.g. mitotane, radiotherapy) are urgently needed, as postoperative disease free survival at five years is only around 30%, but have still not been convincingly established. National registries, international cooperations and trials provide important new structures for patients, but also for researchers aiming at systematic and continuous progress in ACC. However, future advances in the management of ACC will mainly depend on a better understanding of the molecular pathogenesis facilitating the use of modern cancer treatments (e.g. tyrosine kinase inhibitiors).
This article has been cited by other articles:
 |
|
 |
|
  L Cerquetti, B Bucci, R Marchese, S Misiti, U De Paula, R Miceli, A Muleti, D Amendola, P Piergrossi, E Brunetti, et al. Mitotane increases the radiotherapy inhibitory effect and induces G2-arrest in combined treatment on both H295R and SW13 adrenocortical cell lines Endocr. Relat. Cancer, June 1, 2008; 15(2): 623 - 634. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 I G C Hermsen, H Gelderblom, J Kievit, J A Romijn, and H R Haak Extremely long survival in six patients despite recurrent and metastatic adrenal carcinoma. Eur. J. Endocrinol., June 1, 2008; 158(6): 911 - 919. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Quinkler, S. Hahner, S. Wortmann, S. Johanssen, P. Adam, C. Ritte, C. Strasburger, B. Allolio, and M. Fassnacht Treatment of Advanced Adrenocortical Carcinoma with Erlotinib plus Gemcitabine J. Clin. Endocrinol. Metab., June 1, 2008; 93(6): 2057 - 2062. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Hahner, A. Stuermer, M. Kreissl, C. Reiners, M. Fassnacht, H. Haenscheid, F. Beuschlein, M. Zink, K. Lang, B. Allolio, et al. [123I]Iodometomidate for Molecular Imaging of Adrenocortical Cytochrome P450 Family 11B Enzymes J. Clin. Endocrinol. Metab., June 1, 2008; 93(6): 2358 - 2365. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Hancock, R. A Still, A. M Fielding, J. F Doran, and J. W Stephens A rare cause of Cushing's syndrome demonstrates analytical discrepancies between the Roche E170 and Bayer Centaur testosterone assays Ann Clin Biochem, May 1, 2008; 45(3): 328 - 330. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. S. H. Soon, K. L. McDonald, B. G. Robinson, and S. B. Sidhu Molecular Markers and the Pathogenesis of Adrenocortical Cancer Oncologist, May 1, 2008; 13(5): 548 - 561. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T. Else, T. J. Giordano, and G. D. Hammer Evaluation of Telomere Length Maintenance Mechanisms in Adrenocortical Carcinoma J. Clin. Endocrinol. Metab., April 1, 2008; 93(4): 1442 - 1449. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 U. D. Lichtenauer, I. Shapiro, K. Geiger, M. Quinkler, M. Fassnacht, R. Nitschke, K.-D. Ruckauer, and F. Beuschlein Side Population Does Not Define Stem Cell-Like Cancer Cells in the Adrenocortical Carcinoma Cell Line NCI h295R Endocrinology, March 1, 2008; 149(3): 1314 - 1322. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. Bertherat, J. Coste, X. Bertagna, G. Dickstein, C. Shechner, O. Nativ, J. E. Lee, A. Machens, H. Dralle, M. Terzolo, et al. Adjuvant Mitotane in Adrenocortical Carcinoma N. Engl. J. Med., September 20, 2007; 357(12): 1256 - 1259. [Full Text] [PDF]
|
|
|
|
 |
|
 A Carmona-Bayonas, I. Soler, F. Gomez, E. Billalabeitia, H. Saura, M. Tafalla, and M. Diaz Tailored hormonal therapy in secretory adrenocortical cancer Ann. Onc., July 1, 2007; 18(7): 1281 - 1281. [Full Text] [PDF]
|
|
|
|
|
|
M. Terzolo, A. Angeli, M. Fassnacht, F. Daffara, L. Tauchmanova, P. A. Conton, R. Rossetto, L. Buci, P. Sperone, E. Grossrubatscher, et al. Adjuvant Mitotane Treatment for Adrenocortical Carcinoma N. Engl. J. Med., June 7, 2007; 356(23): 2372 - 2380. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
W. F. Young Jr. The Incidentally Discovered Adrenal Mass N. Engl. J. Med., February 8, 2007; 356(6): 601 - 610. [Full Text] [PDF]
|
|
|
|
 |
|
 R. Libe, L. Groussin, F. Tissier, C. Elie, F. Rene-Corail, A. Fratticci, E. Jullian, P. Beck-Peccoz, X. Bertagna, C. Gicquel, et al. Somatic TP53 Mutations Are Relatively Rare among Adrenocortical Cancers with the Frequent 17p13 Loss of Heterozygosity Clin. Cancer Res., February 1, 2007; 13(3): 844 - 850. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. Assie, G. Antoni, F. Tissier, B. Caillou, G. Abiven, C. Gicquel, S. Leboulleux, J.-P. Travagli, C. Dromain, X. Bertagna, et al. Prognostic Parameters of Metastatic Adrenocortical Carcinoma J. Clin. Endocrinol. Metab., January 1, 2007; 92(1): 148 - 154. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. S. Kirschner Paradigms for adrenal cancer: think globally, act locally. J. Clin. Endocrinol. Metab., November 1, 2006; 91(11): 4250 - 4252. [Full Text] [PDF]
|
|
|
|
|
|
M. Fassnacht, S. Hahner, B. Polat, A.-C. Koschker, W. Kenn, M. Flentje, and B. Allolio Efficacy of Adjuvant Radiotherapy of the Tumor Bed on Local Recurrence of Adrenocortical Carcinoma J. Clin. Endocrinol. Metab., November 1, 2006; 91(11): 4501 - 4504. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH |
| Endocrinology | Endocrine Reviews | J. Clin. End. & Metab. |
| Molecular Endocrinology | Recent Prog. Horm. Res. | All Endocrine Journals |
