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Adrenocortical cancer

Hiroko Narumi, Shunji Hasegawa, Kazuyuki Waki, Ken Fukuda, Yuji Ohnishi, Takuya Ichimura, Yousuke Fujimoto, Shunsaku Katsura, Hiroo Kawano, Eiji Ikeda, Satoshi Okada, Shouichi Ohga
Adrenocortical carcinoma (ACC) is a rare malignancy in childhood. Affected children with ACC mostly present with virilization, but not the pure form of Cushing's syndrome. A 9-year-old Japanese girl was hospitalized, because of the unstable emotions and excessive weight gain. She was diagnosed as having Cushing's syndrome and a left adrenal tumor. The adrenalectomy led to the pathological diagnosis of ACC without metastasis. There was no mutation of PRKACA in the tumor-derived DNA, or p53 in peripheral blood-derived DNA...
October 24, 2016: Journal of Pediatric Endocrinology & Metabolism: JPEM
Christina W Lee, Ahmed I Salem Md, David F Schneider, Glen E Leverson, Thuy B Tran, George A Poultsides, Lauren M Postlewait, Shishir K Maithel, Tracy S Wang, Ioannis Hatzaras, Rivfka Shenoy, John E Phay, Lawrence Shirley, Ryan C Fields, Linda X Jin, Timothy M Pawlik, Jason D Prescott, Jason K Sicklick, Shady Gad, Adam C Yopp, John C Mansour, Quan-Yang Duh, Natalie Seiser, Carmen C Solorzano, Colleen M Kiernan, Konstantinos I Votanopoulos, Edward A Levine, Sharon M Weber
BACKGROUND AND OBJECTIVES: Minimally invasive surgery for adrenocortical carcinoma (ACC) is controversial. We sought to evaluate the perioperative and long-term outcomes following minimally invasive (MIS) and open resection (OA) of ACC in patients treated with curative intent surgery. METHODS: Retrospective data from patients who underwent adrenalectomy for primary ACC at 13 tertiary care cancer centers were analyzed, including demographics, clinicopathological, and operative outcomes...
October 21, 2016: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
Yasunori Minami, Tomohiro Minami, Hirokazu Chishina, Masashi Kono, Tadaaki Arizumi, Masahiro Takita, Norihisa Yada, Satoru Hagiwara, Hiroshi Ida, Kazuomi Ueshima, Naoshi Nishida, Masatoshi Kudo
OBJECTIVE: Radiofrequency ablation (RFA) induces gas bubbles in ablation zones, and the ablative margin cannot be evaluated accurately on ultrasound (US) during and immediately after RFA. This study assessed the usefulness of US-US fusion imaging to visualize the ablative margin of RFA for liver metastasis. METHODS: RFA guided by US-US fusion imaging was performed on 12 targeted tumors in 10 patients. Secondary hepatic malignancies included patients with colorectal cancer (n = 4), breast cancer (n = 2), lung cancer (n = 1), gastrointestinal stromal tumor (n = 1), pancreatic neuroendocrine tumor (n = 1), and adrenocortical carcinoma (n = 1)...
2016: Digestive Diseases
Eden C Payabyab, Sanjeeve Balasubramaniam, Maureen Edgerly, Margarita Velarde, Maria J Merino, Aradhana M Venkatesan, Harshraj Leuva, Thomas Litman, Susan E Bates, Tito Fojo
The development of new therapies has lagged behind for rare cancers without defined therapeutic targets. Adrenocortical cancer is no exception. Mitotane, an older agent considered "adrenolytic," is used both to control symptoms in advanced disease and as adjuvant therapy after surgical resection. Molecular characterization of adrenocortical cancer has deepened our understanding of this genetically complex disease while identifying subgroups whose importance remains to be determined. Unfortunately, such studies have yet to demonstrate a therapeutic target for drug development, and to date, no targeted therapy has achieved meaningful outcomes...
October 15, 2016: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
Vince Kornél Grolmusz, Katalin Karászi, Tamás Micsik, Eszter Angéla Tóth, Katalin Mészáros, Gellért Karvaly, Gábor Barna, Péter Márton Szabó, Kornélia Baghy, János Matkó, Ilona Kovalszky, Miklós Tóth, Károly Rácz, Péter Igaz, Attila Patócs
Adrenocortical cancer (ACC) is a rare, but agressive malignancy with poor prognosis. Histopathological diagnosis is challenging and pharmacological options for treatment are limited. By the comparative reanalysis of the transcriptional malignancy signature with the cell cycle dependent transcriptional program of ACC, we aimed to identify novel biomarkers which may be used in the histopathological diagnosis and for the prediction of therapeutical response of ACC. Comparative reanalysis of publicly available microarray datasets included three earlier studies comparing transcriptional differences between ACC and benign adrenocortical adenoma (ACA) and one study presenting the cell cycle dependent gene expressional program of human ACC cell line NCI-H295R...
2016: American Journal of Cancer Research
Raissa Coelho Andrade, Anna Claudia Evangelista Dos Santos, Joaquim Caetano de Aguirre Neto, Julián Nevado, Pablo Lapunzina, Fernando Regla Vargas
Li-Fraumeni and Li-Fraumeni like syndromes (LFS/LFL) represent rare cancer-prone conditions associated mostly with sarcomas, breast cancer, brain tumors, and adrenocortical carcinomas. TP53 germline mutations are present in up to 80 % of families with classic Li-Fraumeni syndrome, and in 20-60 % of families with Li-Fraumeni like phenotypes. The frequency of LFS/LFL families with no TP53 mutations detected suggests the involvement of other genes in the syndrome. In this study, we searched for mutations in TP53 in 39 probands from families with criteria for LFS/LFL...
October 6, 2016: Familial Cancer
Rafael H Abduch, Ana Carolina Bueno, Leticia F Leal, Marcelo M Cavalcanti, Débora C Gomes, Silvia R Brandalise, Maria J Masterallo, José A Yunes, Carlos E Martinelli, Luiz G Tone, Silvio Tucci, Carlos A F Molina, Fernando S Ramalho, Ayrton C Moreira, Izilda A Cardinalli, Carlos A Scrideli, Leandra N Z Ramalho, Margaret de Castro, Sonir R Antonini
BACKGROUND: Overexpression of the oncogene yes-associated-protein-1 (YAP1) is associated with increased cell proliferation in human cancers. YAP1 is a potential target of the Wnt/beta-catenin pathway, which plays an important role in adrenocortical tumors (ACT). The role of YAP1 in adrenocortical tumorigenesis has not been assessed. AIMS: To evaluate YAP1 expression in normal adrenals and pediatric ACT and its association with disease outcome. To investigate the interaction between YAP1 and the Wnt/beta-catenin pathway in adrenocortical cells...
October 1, 2016: Oncotarget
Maria Isabel Achatz, Gerard P Zambetti
A common criticism of studying rare diseases is the often-limited relevance of the findings to human health. Here, we review ∼15 years of research into an unusual germline TP53 mutation (p.R337H) that began with its detection in children with adrenocortical carcinoma (ACC), a remarkably rare childhood cancer that is associated with poor prognosis. We have come to learn that the p.R337H mutation exists at a very high frequency in Southern and Southeastern Brazil, occurring in one of 375 individuals within a total population of ∼100 million...
September 23, 2016: Cold Spring Harbor Perspectives in Medicine
Chiara Fiorentini, Martina Fragni, Paola Perego, Sara Vezzoli, Sara A Bonini, Monica Tortoreto, Diego Galli, Melanie Claps, Guido A Tiberio, Massimo Terzolo, Cristina Missale, Maurizio Memo, Giuseppe Procopio, Nadia Zaffaroni, Alfredo Berruti, Sandra Sigala
CONTEXT: Patients with adrenocortical carcinoma (ACC) frequently suffer from cortisol excess, which portends a negative prognosis. Rapid control of cortisol hypersecretion and tumor growth are the main goals of ACC therapy. Abiraterone acetate (AA) is a potent inhibitor of 17alpha-hydroxylase/17,20-lyase, a key enzyme of adrenal steroidogenesis. OBJECTIVE: To investigate the therapeutic use of AA in preclinical models of ACC. DESIGN: AA antisecretive and antiproliferative effects were investigated in vitro using NCI-H295R and SW13 ACC cell lines and human primary ACC cell cultures, as well as in vivo using immunodeficient mice...
September 14, 2016: Journal of Clinical Endocrinology and Metabolism
Fady Hannah-Shmouni, Fabio R Faucz, Constantine A Stratakis
Alterations in the cyclic (c)AMP-dependent signaling pathway have been implicated in the majority of benign adrenocortical tumors (ACTs) causing Cushing syndrome (CS). Phosphodiesterases (PDEs) are enzymes that regulate cyclic nucleotide levels, including cyclic adenosine monophosphate (cAMP). Inactivating mutations and other functional variants in PDE11A and PDE8B, two cAMP-binding PDEs, predispose to ACTs. The involvement of these two genes in ACTs was initially revealed by a genome-wide association study in patients with micronodular bilateral adrenocortical hyperplasia...
2016: Frontiers in Endocrinology
Benjamin Miron, Benjamin T Ristau, Jeffrey J Tomaszewski, Josh Jones, Bart Milestone, Yu-Ning Wong, Robert G Uzzo, Donna Edmondson, Walter Scott, Alexander Kutikov
Adrenocortical carcinoma (ACC) is a rare malignancy that is generally associated with a poor prognosis whose existence dictates the management of incidental renal masses. We report a case of ACC diagnosed and treated at its apparent inception in a patient undergoing close surveillance imaging of a prior malignancy. Despite timely detection and resection of a localized ACC this patient rapidly progressed to systemic disease. This case highlights the rapid growth kinetics of ACC and puts into perspective the challenges associated with the established treatment paradigm for patients diagnosed with an adrenal mass...
November 2016: Urology Case Reports
S G Creemers, P M van Koetsveld, E S R van den Dungen, E Korpershoek, F J van Kemenade, G J H Franssen, W W de Herder, R A Feelders, L J Hofland
CONTEXT: Treatment of patients with adrenocortical carcinomas (ACC) with mitotane and/or chemotherapy is often associated with toxicity and poor tumor response. New therapeutic options are urgently needed. OBJECTIVE: To evaluate the therapeutic possibilities of temozolomide (TMZ) in ACC cells and to assess the potential predictive role of the DNA repair gene O6-Methylguanine-DNA methyltransferase (MGMT) in adrenocortical tumors. METHODS: 3 human ACC cell lines and 8 primary ACC cultures were used to assess effects of TMZ in vitro...
September 7, 2016: Journal of Clinical Endocrinology and Metabolism
Nada El Ghorayeb, Solange Grunenwald, Serge Nolet, Vanessa Primeau, Stéphanie Côté, Christine M Maugard, André Lacroix, Louis Gaboury, Isabelle Bourdeau
BACKGROUND: Adrenocortical carcinoma (ACC) may rarely be a component of inherited cancer syndromes such as Li-Fraumeni syndrome and Beckwith-Wiedemann syndrome. ACC caused by a BRCA2 mutation has never been reported. METHODS: Nucleotide sequencing of BRCA2 in lymphocyte and tumoral DNA of a 50-year-old male who presented with an androgen-secreting ACC and a strong family history of breast, ovarian, and pancreatic cancers. RESULTS: A germline BRCA2 2 bp heterozygous deletion at nucleotide 8765 (8765delAG) leading to a frameshift mutation (p...
September 2016: Medicine (Baltimore)
Paola Nolé, Britt Duijndam, Adam Stenman, C Christofer Juhlin, Mikael Kozyra, Catharina Larsson, Magnus Ingelman-Sundberg, Inger Johansson
Human cytochome P450 2W1 (CYP2W1) enzyme is expressed in fetal colon and in colon tumors. The level of expression is higher in colon metastases than in the parent tumors and the enzyme is a possible drug target for treatment of colorectal cancer, as demonstrated in mouse xenograft studies. A previous study published in this journal reported that CYP2W1 is highly expressed in normal and transformed adrenal tissue. However, adrenal expression of CYP2W1 protein was not seen in previous studies in our research group...
2016: PloS One
Mabrouka Doghman-Bouguerra, Enzo Lalli
Steroidogenesis is a multistep process where interorganelle communications between the endoplasmic reticulum and mitochondria are critical. These intimate interactions physically occur through the Mitochondria-Associated ER membranes called MAMs. MAMs play important roles in mitochondrial morphology and in many cellular functions ranging from lipid metabolism, to calcium signaling and apoptosis together with a critical effect on steroidogenesis. Moreover, our recent characterization of new MAM resident proteins in adrenocortical cells extends the function of MAM in the mechanism of resistance of cancer cells to apoptotic stimuli and offers new perspectives in targeted therapeutic approaches for adrenocortical tumorigenesis...
September 1, 2016: Molecular and Cellular Endocrinology
Stefan Janssen, Tobias Bartscht, Dirk Rades
BACKGROUND/AIM: Adrenocortical cancer is a rare aggressive type of cancer. The prognosis is poor, particularly for metastatic disease. This study focused on metastatic spinal cord compression (MSCC) from adrenocortical carcinoma. PATIENTS AND METHODS: Data of three patients who received palliative irradiation of MSCC from adrenocortical carcinoma were retrospectively analyzed for motor function, ambulatory status and survival. RESULTS: One patient died before completion of radiotherapy...
September 2016: In Vivo
D Maccora, G V Walls, G P Sadler, R Mihai
INTRODUCTION The 2012 British Association of Endocrine and Thyroid Surgeons audit report showed that only 86 of 1359 patients who underwent adrenalectomy had a bilateral operation; thus the experience with this procedure remains limited. METHODS Retrospective review of patients undergoing bilateral adrenalectomy in a tertiary referral centre. RESULTS Between November 2005 and January 2016, bilateral adrenalectomy was performed in 23 patients (6 male, 17 female, age 43 ± 4 years) diagnosed with Cushing's disease (n = 13), hereditary phaeochromocytomas (n = 6), adrenocortical cancer (n = 2), colorectal metastatic disease (n = 1) and adrenocortical adenomas (n = 1)...
August 23, 2016: Annals of the Royal College of Surgeons of England
Maria Candida Barisson Villares Fragoso, Ingrid Quevedo Wanichi, Isadora Pontes Cavalcante, Beatriz Marinho de Paula Mariani
Somatic GNAS point mutations, commonly known as gsp mutations, are involved in the pathogenesis of McCune-Albright syndrome (MAS) and have also been described in autonomous hormone-producing tumors, such as somatotropinoma, corticotrophoma, thyroid cancer, ovarian and testicular Leydig cell tumors, and primary macronodular adrenocortical hyperplasia (PMAH) (1-3). The involvement of gsp mutations in adrenal tumors was first described by Lyons et al. Since then, several studies have detected the presence of gsp mutations in adrenal tumors, but none of them could explain its presence along or the mechanism that leads to tumor formation and hormone hypersecretion...
2016: Frontiers in Endocrinology
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
No abstract text is available yet for this article.
August 8, 2016: Cancer Cell
Phuong L Mai, Ana F Best, June A Peters, Rosamma M DeCastro, Payal P Khincha, Jennifer T Loud, Renée C Bremer, Philip S Rosenberg, Sharon A Savage
BACKGROUND: Li-Fraumeni syndrome (LFS) is an autosomal dominant cancer predisposition syndrome characterized by a very high lifetime cancer risk and an early age at diagnosis of a wide cancer spectrum. Precise estimates for the risk of first and subsequent cancers are lacking. METHODS: The National Cancer Institute's Li-Fraumeni Syndrome Study includes families meeting the diagnostic criteria for LFS or Li-Fraumeni-like syndrome, and individuals with a germline TP53 mutation, choroid plexus carcinoma, adrenocortical carcinoma, or ≥3 cancers...
August 6, 2016: Cancer
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