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Study shows possible 'key' to improved therapy for adrenocortical carcinoma

Multi-institutional, international effort identified three subtypes of aggressive rare cancer

Date:
May 9, 2016
Source:
University of Texas M. D. Anderson Cancer Center
Summary:
Significant new findings about adrenocortical carcinoma, a rare cancer with an often poor prognosis, have been revealed by a new study comprised of 39 international institutions.
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A study comprised of 39 international institutions revealed significant new findings about adrenocortical carcinoma (ACC), a rare cancer with an often poor prognosis.

The study, which conducted a comprehensive "pan-genomic" assessment of ACC, was led by The University of Texas MD Anderson Cancer Center and the University of Michigan, Ann Arbor. Research results are published in the May 9 issue of Cancer Cell.

ACC is an aggressive cancer originating in the adrenal gland. The disease affects less than two people per million annually, and is seen more commonly in children under age 5 and adults ages 30-40. The overall five-year survival rate is 20 to 35 percent.

Scientists examined 91 ACC tumor specimens from four continents, and observed "massive" DNA loss followed by whole genome doubling (WGD). WGD occurs when tumor cells acquire an extra copy of their entire genome. The researchers found that WGD was associated with aggressive clinical course, suggesting that it could be a hallmark of disease progression. They speculate that tumor growth could be slowed if they could prohibit WGD in future pre-clinical studies.

"Our results represent the most complete characterization of ACC tissues and may indicate a key to successful targeted therapy for this disease," said Roeland Verhaak, Ph.D., associate professor of Bioinformatics and Computational Biology. "The study findings illustrate how molecular data, combined with traditional clinical assessment, might inform therapeutic decisions and lead to significant advances in patient outcomes."

In addition, the study identified three ACC subtypes with distinct clinical outcomes and molecular alterations, said Verhaak, paving the way for a more precise clinical stratification of patients based on molecular biomarkers.

The team also identified novel ACC "driver" genes, expanding their understanding about genes already thought to lead to tumor formation, as well as defining new molecular pathways.

"Our understanding of ACC pathogenesis is incomplete and new therapies are needed," said Verhaak. "While standard clinical assessments are informative for patient management, molecular information may be able to more precisely predict patient outcome and direct optimal care."

The study relied on cancer molecular data provided through The Cancer Genome Atlas (TCGA).


Story Source:

Materials provided by University of Texas M. D. Anderson Cancer Center. Note: Content may be edited for style and length.


Journal Reference:

  1. Siyuan Zheng, Andrew D. Cherniack, Ninad Dewal, Richard A. Moffitt, Ludmila Danilova, Bradley A. Murray, Antonio M. Lerario, Tobias Else, Theo A. Knijnenburg, Giovanni Ciriello, Seungchan Kim, Guillaume Assie, Olena Morozova, Rehan Akbani, Juliann Shih, Katherine A. Hoadley, Toni K. Choueiri, Jens Waldmann, Ozgur Mete, A. Gordon Robertson, Hsin-Ta Wu, Benjamin J. Raphael, Lina Shao, Matthew Meyerson, Michael J. Demeure, Felix Beuschlein, Anthony J. Gill, Stan B. Sidhu, Madson Q. Almeida, Maria C.B.V. Fragoso, Leslie M. Cope, Electron Kebebew, Mouhammed A. Habra, Timothy G. Whitsett, Kimberly J. Bussey, William E. Rainey, Sylvia L. Asa, Jérôme Bertherat, Martin Fassnacht, David A. Wheeler, Gary D. Hammer, Thomas J. Giordano, Roel G.W. Verhaak. Comprehensive Pan-Genomic Characterization of Adrenocortical Carcinoma. Cancer Cell, 2016; 29 (5): 723 DOI: 10.1016/j.ccell.2016.04.002

Cite This Page:

University of Texas M. D. Anderson Cancer Center. "Study shows possible 'key' to improved therapy for adrenocortical carcinoma: Multi-institutional, international effort identified three subtypes of aggressive rare cancer." ScienceDaily. ScienceDaily, 9 May 2016. <www.sciencedaily.com/releases/2016/05/160509132455.htm>.
University of Texas M. D. Anderson Cancer Center. (2016, May 9). Study shows possible 'key' to improved therapy for adrenocortical carcinoma: Multi-institutional, international effort identified three subtypes of aggressive rare cancer. ScienceDaily. Retrieved May 23, 2017 from www.sciencedaily.com/releases/2016/05/160509132455.htm
University of Texas M. D. Anderson Cancer Center. "Study shows possible 'key' to improved therapy for adrenocortical carcinoma: Multi-institutional, international effort identified three subtypes of aggressive rare cancer." ScienceDaily. www.sciencedaily.com/releases/2016/05/160509132455.htm (accessed May 23, 2017).

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