Outcomes of adrenal cortical carcinoma in the United States

“…[1][2][3][4][5] It is responsible for only approximately 0.2% of all cancer deaths in the United States annually, and it is the second most aggressive endocrine malignancy behind anaplastic thyroid cancer. 1 Women are more frequently affected than men (1.5:1), and patients present with evidence of adrenocortical hormonal excess in 40% to 60% of cases.…”

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confidence: 99%

“…considered the most important component of therapy with curative intent for both primary and recurrent ACC. [3][4][5]8 Recurrence occurs in approximately 70% to 80% of patients after resection, and a survival advantage has been demonstrated in some series for patients who undergo resection of recurrent disease. 2,5,6,9,10 Furthermore, resection of recurrent disease can provide palliation of hormonal symptoms of functional ACCs, 11 although surgical therapy is controversial for patients presenting with metastatic disease because overall prognosis is poor regardless of treatment modality.…”

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confidence: 99%

“…The most consistently cited factors associated with worse outcomes are advanced stage and incomplete surgical resection. [3][4][5]12 High grade, older age, hormonal hypersecretion, sex, and large tumor size have been found to be associated with poorer prognosis in some studies. 2,3,5,12 The era of treatment has also been considered a factor related to prognosis.…”

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confidence: 99%

“…Some studies have suggested an improved disease-specific mortality when comparing patients with ACC treated in the past 20 years to those treated in the 20 years prior, 3,5,12 but others have shown no change in stage at presentation or survival over the past 2 decades. 4,12 Thus far, no studies, to our knowledge, have been able to evaluate national treatment practice patterns in the United States for ACC. Prognostic factors are not well defined, largely because of the small sample sizes of studies to date.…”

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confidence: 99%

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Adrenocortical carcinoma in the United States

Bilimoria

Shen

Elaraj

et al. 2008

Cancer

432

210

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BACKGROUND.Adrenocortical carcinoma (ACC) is a rare tumor with a relatively poor prognosis. The authors' objectives were to examine treatment utilization and factors associated with long‐term survival after resection of ACC in a large, national, patient population.METHODS.Patients diagnosed with ACC from 1985 to 2005 were identified from the National Cancer Data Base (NCDB). Patient, tumor, treatment, and hospital factors associated with survival after resection were examined.RESULTS.For the current study, 3982 patients with ACC were identified. Median age at diagnosis was 55 years. Median tumor size was 13 cm. Of the patients with nodes examined, 26.5% had nodal metastases. Distant metastases were found on presentation in 21.6% of patients. A total of 57.4% of patients underwent surgical resection alone, whereas 16.0% underwent resection with adjuvant chemotherapy or radiation. A total of 19.4% had margin‐positive resections. Treatment utilization remained unchanged from 1985 to 2005 (P = .28). Median follow‐up was 24 months. Overall 5‐year survival for all patients who underwent resection was 38.6% (median survival, 31.9 months). Multivariable analysis demonstrated a higher risk of death with increasing age, poorly differentiated tumors, involved margins, and nodal or distant metastases. Overall survival remained unchanged from 1985 to 2000 (P = .08).CONCLUSIONS.ACC carries a poor prognosis for patients commonly presenting with large, locally invasive tumors, involved margins, and metastatic disease. Survival is not affected by size but is diminished with increasing age, poorly differentiated tumors, involved margins, and the presence of regional and distant disease. Identification of novel therapies may help to increase survival, which has remained unchanged over the last 20 years. Cancer 2008. © 2008 American Cancer Society.

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Antineoplastic Effects of Decitabine, an Inhibitor of DNA Promoter Methylation, in Adrenocortical Carcinoma Cells

Suh

Weng²,

Fernández-Ranvier³

et al. 2010

Arch Surg

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Hypotheses Decitabine recovers expression of silenced genes on chromosome 11q13 and has antineoplastic effects in adrenocortical carcinoma (ACC) cells. Design NCI-H295R cells were treated with decitabine (0.1–1.0μM) over 5 days. Cells were evaluated at 24-hour intervals for the effects of decitabine on ACC cell proliferation, cortisol secretion, and cell invasion. Expression was quantified for 6 genes on 11q13 (DDB1, MRPL48, NDUFS8, PRDX5, SERPING1, and TM7SF2) that were previously shown to be underexpressed in ACC. Setting Academic research. Study Specimen Human ACC cell line. Main Outcome Measures Adrenocortical carcinoma cell proliferation, cortisol secretion, and cell invasion were measured using immunometric assays. Quantitative reverse transcription–polymerase chain reaction was used to measure gene expression relative to GAPDH. Results Decitabine inhibited ACC cell proliferation by 39% to 47% at 5 days after treatment compared with control specimens (P < .001). The inhibitory effect was cytostatic, time dependent, and dose dependent. Decitabine decreased cortisol secretion by 56% to 58% at 5 days after treatment (P=.02) and inhibited cell invasion by 64% at 24 hours after treatment (P=.03). Of 6 downregulated genes on 11q13, decitabine recovered expression of NDUFS8 (OMIM 602141)(P < .001) and PRDX5 (OMIM 606583) (P=.006). Conclusions Decitabine exhibits antitumoral properties in ACC cells at clinically achievable doses and may be an effective adjuvant therapy in patients with advanced disease. Decitabine recovers expression of silenced genes on 11q13, which suggests a possible role of epigenetic gene silencing in adrenocortical carcinogenesis.

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Outcomes of adrenal cortical carcinoma in the United States

Cited by 92 publications

References 21 publications

Laparoscopic adrenalectomies: A nationwide single-surgeon experience

Laparoscopic adrenalectomies: A nationwide single-surgeon experience

Adrenocortical carcinoma in the United States

Antineoplastic Effects of Decitabine, an Inhibitor of DNA Promoter Methylation, in Adrenocortical Carcinoma Cells

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