LETTERS TO THE EDITOR
@ 2018 EDIZIONI MINERVA MEDICA
Online version at http://www.minervamedica.it Minerva Endocrinologica 2019 June; 44(2):232 DOI: 10.23736/S0391-1977.18.02800-6
Adrenocortical carcinoma: current knowledge
Adrenocortical neoplasms are common diseases. They can be subdivided in adrenocortical adenomas (ACAs) and adrenocortical carcinomas (ACCs). ACCs are rare, with an incidence of 0.7-2 million people per year. ACCs affect the population in two groups of age, one in the child- hood (until nearly six years) and another during adulthood (between approximately 30 to 60 years) and are more fre- quent in women (59%). Survival rate is variable, but it is estimated less than 30-40% at 5 years. Differently, ACA are more usual diseases, but in some cases are roughly discernable from ACC. Indeed, only accurate tests and histological examination can definitively separate the 2 forms of neoplasms. It is important to distinguish ACC from ACA, because of the different treatment and progno- sis. While ACAs can have a benign clinical course, ACCs can frequently metastasize and has low drug response.1
ACC can be classified in sporadic (frequent) or ge- netic forms, being the last rare and found especially in children. Moreover, ACC can be subdivided in secret- ing or non-secreting neoplasms. Most ACC patients (up to 60%) show typical signs of increased circulating hormones (secreting forms); hypercortisolism that can degenerate in the onset of Cushing’s Syndrome, hyper- androgenism that can easily see in women and children by hirsutism and virilization, hyperaldosteronism, that is more rare and defined by hypertension and hypokalemia. Contrarily non-secreting ACC can be difficult to diag- nose as they have very common symptoms (abdominal pain, sense of satiety, nausea or discomfort) and are dis- covered in late disease-stage. Together with clinical and laboratory examination, abdominal or total body imaging such as CT or MRI techniques can help diagnose ACC.1
However, the ultimate diagnosis of ACC requires histopathological analysis with the assignment of Weiss score, a set of criteria based on cellular morphology, pro- liferation and invasiveness. Discriminatory score (>3) can identify ACC or ACA (score <2) and suggest tumor staging and possible prognosis.2
Treatment options for ACC are poor and surgical re- section is currently the main curative approach. While radiation therapy is still conflicting, the use of mitotane as adjuvant has become the standard of care in ACC. Yet this disease shows a strong capacity to metastasize, most
frequently in the lungs, bones and livers. Metastatic ACC is removed by debulking surgery when possible other- wise mitotane treatment alone or in combination (etopo- side, doxorubicin, and cisplatin (EDP) or streptozotocin, both in combination with mitotane) seems the only help- ful tool to fight this malignancy.3 Due to the scarce ef- ficacy of available treatments and to the high tendency of ACC to metastasize, preclinical studies and clinical trials are underway. Many genetic and bimolecular alterations have been identified in ACC models, now being investi- gated for their potential effects of ACC treatments.4 Also in our lab different studies are ongoing (and has been completed) aimed at characterizing the use of promising anti-tumor molecules, nonetheless further collaborative efforts are still necessary to discover and enhance thera- peutic options for ACC and in this ENS@T group (ac- ronym for European Network for the Study of Adrenal Tumors) seems a good answer to address ACC treatment.
Raffaele PEZZANI 1,2 *
1Unit of Endocrinology, Department of Medicine (DIMED), University of Padua, Padua, Italy; 2Associazione Italiana per la Ricerca Oncologica di Base (AIROB), Padua, Italy
*Corresponding author: Raffaele Pezzani, Unit of Endocrinol- ogy, Department of Medicine (DIMED), University of Padua, via Ospedale 105, 35128 Padua, Italy.
E-mail: raffaele.pezzani@unipd.it
References
1. Libé R. Adrenocortical carcinoma (ACC): diagnosis, prog- nosis, and treatment. Front Cell Dev Biol 2015;3:45.
2. Papotti M, Libè R, Duregon E, Volante M, Bertherat J, Tis- sier F. The Weiss score and beyond-histopathology for adre- nocortical carcinoma. Horm Cancer 2011;2:333-40.
3. Fay AP, Elfiky A, Teló GH, McKay RR, Kaymakcalan M, Nguyen PL, et al. Adrenocortical carcinoma: the management of metastatic disease. Crit Rev Oncol Hematol 2014;92:123-32.
4. Szyszka P, Grossman AB, Diaz-Cano S, Sworczak K, Dworakowska D. Molecular pathways of human adrenocorti- cal carcinoma - translating cell signalling knowledge into diag- nostic and treatment options. Endokrynol Pol 2016;67:427-50.
Conflicts of interest .- The authors certify that there is no conflict of interest with any financial organization regarding the material discussed in the manuscript.
Article first published online: February 13, 2018. - Manuscript accepted: January 29, 2018. - Manuscript received: January 9, 2018.
(Cite this article as: Pezzani R. Adrenocortical carcinoma: current knowledge. Minerva Endocrinol 2019;44:232. DOI: 10.23736/S0391-1977.18.02800-6)
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