Adrenocortical carcinoma: A population-based study on incidence and survival in the Netherlands since 1993

Kerkhofs, Thomas M.A.; Verhoeven, Rob H.A.; Zwan, Jan Maarten van der; Dieleman, Jeanne P.; Kerstens, Michiel N.; Links, Thera P.; Poll‐Franse, Lonneke V. van de; Haak, Harm R.

doi:10.1016/j.ejca.2013.02.034

Cited by 242 publications

(161 citation statements)

References 25 publications

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“…A recent population-based study estimated the incidence between 1.0 and 1.3 patients per million person-years (1). According to a study from the USA Surveillance, Epidemiology and End Results (SEER) database, the incidence is even lower among patients under 20 years of age: 0.2-0.3 patients per million (2).…”

Section: Introductionmentioning

confidence: 99%

Adrenocortical carcinoma in children: First population-based clinicopathological study with long-term follow-up

et al. 2014

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Abstract. Adrenocortical carcinoma (ACC) is rare in both adult and pediatric populations. Literature suggests significant differences between children and adults in presentation, histological properties and outcome. The aim of this first nationwide study on pediatric ACC was to describe the incidence, presentation, pathological characteristics, treatment and survival in The Netherlands. All ACC patients aged <20 years at diagnosis and registered in the population-based Netherlands Cancer Registry between 1993 and 2010 were included. Clinical data were extracted from medical records. Archival histological slides were collected via the Dutch Pathology Registry (PALGA). We compared our findings to all clinical studies on pediatric ACC that were found on PubMed. Based on the results, 12 patients were identified: 8 females and 4 males. The median age was 4

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Section: Introductionmentioning

confidence: 99%

Adrenocortical carcinoma in children: First population-based clinicopathological study with long-term follow-up

et al. 2014

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“…Adrenocortical carcinoma (ACC) is a rare malignancy, with an estimated incidence of 0.7-2.0 cases per million population each year (Kebebew et al 2006, Golden et al 2009, Fassnacht et al 2013, Kerkhofs et al 2013). …”

Section: Introductionmentioning

confidence: 99%

Methylation of IGF2 regulatory regions to diagnose adrenocortical carcinomas

Creemers

Koetsveld

Kemenade

et al. 2016

Endocrine-Related Cancer

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Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis. Discrimination of ACCs from adrenocortical adenomas (ACAs) is challenging on both imaging and histopathological grounds. High IGF2 expression is associated with malignancy, but shows large variability. In this study, we investigate whether specific methylation patterns of IGF2 regulatory regions could serve as a valuable biomarker in distinguishing ACCs from ACAs. Pyrosequencing was used to analyse methylation percentages in DMR0, DMR2, imprinting control region (ICR) (consisting of CTCF3 and CTCF6) and the H19 promoter. Expression of IGF2 and H19 mRNA was assessed by realtime quantitative PCR. Analyses were performed in 24 ACCs, 14 ACAs and 11 normal adrenals. Using receiver operating characteristic (ROC) analysis, we evaluated which regions showed the best predictive value for diagnosis of ACC and determined the diagnostic accuracy of these regions. In ACCs, the DMR0, CTCF3, CTCF6 and the H19 promoter were positively correlated with IGF2 mRNA expression (P < 0.05). Methylation in the most discriminating regions distinguished ACCs from ACAs with a sensitivity of 96%, specificity of 100% and an area under the curve (AUC) of 0.997 ± 0.005. Our findings were validated in an independent cohort of 9 ACCs and 13 ACAs, resulting in a sensitivity of 89% and a specificity of 92%. Thus, methylation patterns of IGF2 regulatory regions can discriminate ACCs from ACAs with high diagnostic accuracy. This proposed test may become the first objective diagnostic tool to assess malignancy in adrenal tumours and facilitate the choice of therapeutic strategies in this group of patients.

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“…Distant metastases at presentation occur in 50% of cases 1. Preoperative transcutaneous biopsy—though performed in this case—may not be necessary and has a complication rate of 11%, but 70% diagnostic sensitivity 2.…”

Section: Descriptionmentioning

confidence: 84%

“…ACC is a rare tumour which has poor prognosis and the 5-year survival rate is barely 20% 1. Distant metastases at presentation occur in 50% of cases 1.…”

Section: Descriptionmentioning

confidence: 99%

Young male with left adrenal mass

2016

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DESCRIPTIONA 20-year-old male patient presented with a 5-month history of left hypochondriac pain and mass. He denied any associated constitutional symptoms. Clinically, he was overweight with no cushingoid or virilising features. Abdominal examination revealed a huge non-tender mass arising from the left hypochondrium. There was no hepatomegaly and the testicles were normal.Routine blood investigations including tumour markers and plasma and urine hormonal assays were normal. CT scan ( figure 1A) and MRI (figure 1B) revealed a large left retroperitoneal mass (15×12 cm) with small bilateral lung deposits. After excluding phaeochromocytoma, US-guided transcutaneous biopsy raised the possibility of nonfunctioning adrenocortical carcinoma (ACC). Laparotomy revealed locally advanced adrenal tumour (figure 2A) with segments V and VII hepatic deposits. Hence, a palliative resection was carried out (figures 2B) and the histopathology confirmed ACC (weight: 2.11 kg; size: 24×10×9 cm) with negative lymph nodes (figure 3). Immunohistochemistry expressed focal positivity for vimentin, inhibin and cytokeratin. He was later lost to follow-up and therefore presumed dead after 3 months. ACC is a rare tumour which has poor prognosis and the 5-year survival rate is barely 20%.1 Distant metastases at presentation occur in 50% of cases. Preoperative transcutaneous biopsy-though performed in this case-may not be necessary and has a complication rate of 11%, but 70% diagnostic sensitivity. 2 Although the resectability rate is 50%, 3 treatment is by aggressive surgical resection with or without lymph node dissection even in presence of metastases.1 3 ACC exhibits poor response to chemotherapy, but mitotane remains the only drug that produces tumour and metastatic regression in incompletely-resected or non-resectable carcinomas. Learning points▸ Adrenocortical carcinoma is a rare tumour which is often large in size, locally advanced and metastatic at presentation. ▸ It is associated with poor prognosis; the 5-year survival rate barely exceeds 20%. ▸ Although the resectability rate is <50%, treatment is aggressive surgical resection with or without lymph node dissection even in presence of metastases.

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Adrenocortical carcinoma: A population-based study on incidence and survival in the Netherlands since 1993

Cited by 242 publications

References 25 publications

Adrenocortical carcinoma in children: First population-based clinicopathological study with long-term follow-up

Adrenocortical carcinoma in children: First population-based clinicopathological study with long-term follow-up

Methylation of IGF2 regulatory regions to diagnose adrenocortical carcinomas

Young male with left adrenal mass

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