Current and Emerging Therapies for Advanced Adrenocortical Carcinoma

Tacon, Lyndal; Prichard, Ruth; Soon, Patsy; Robinson, Bruce G.; Clifton‐Bligh, Roderick; Sidhu, Stan

doi:10.1634/theoncologist.2010-0270

Cited by 35 publications

(57 citation statements)

References 164 publications

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“…Advanced ACC has a poor prognosis [2,6,30]. In our series the mean overall survival for ENSAT stages III and IV was quite disappointing.…”

Section: Discussionmentioning

confidence: 52%

“…Although our experience is far from the ideal, 40% (2 of 5 patients) of our patients experienced mitotane side effects: severe skin rash and liver dysfunction. Abnormalities in liver function analysis are common, but significant hepatotoxicity is rare [30].…”

Section: Discussionmentioning

confidence: 99%

“…Treatment options for advanced ACC are limited [30]. Results of cytotoxic chemotherapy have been disappointing [30].…”

Section: Discussionmentioning

confidence: 99%

“…Results of cytotoxic chemotherapy have been disappointing [30]. The most encouraging results deliver from the combination of lowdose mitotane with etoposide, doxorubicin and cisplatin (EDP) [33,34].…”

Section: Discussionmentioning

confidence: 99%

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Adrenocortical Carcinoma - A 25 Years Tertiary Centre Experience and Short Review of the Literature

Nunes¹

2018

EMIJ

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We performed an informatics search of the medical registers of our centre (filtered by age -over 18-years-old) for the diagnosis codes 'adrenal neoplasia' and 'adrenocortical carcinoma', and Review ArticleEndocrinol Metab Int J 2018, 6(1): 00148 AbstractPurpose: Adrenocortical carcinoma (ACC) is an uncommon and aggressive malignancy. We aimed to investigate the clinical and pathological findings of ACC diagnosed/treated in our hospital from January/1988 to January/2013.Methods: Analysis of presentation, imagiology, histopathology review, staging and survival data. Results:We reviewed 31 patients. The average age at diagnosis was 54.6±12.6years and 51.6% were females. At diagnosis, 11.8% were asymptomatic, 23.5% presented back pain, 17.6% general malaise, 9.4% hypercortisolism and 17.6% hirsutism/virilization. Tumours had 11.7±6.6cm, 53.3% located on the right side, 72.7% with local invasion, 25% inferior vena cava invasion and 63.7% distant metastasis. Ten were submitted to adrenalectomy: 80% presented atypical mitosis, 20% diffuse architecture, 60% ≤25% clear cells, 90% necrosis, 70% venous invasion, 90% sinusoidal invasion, 60% capsule invasion, mitotic index 41.2/50HPF (±36.8). One patient was treated with adjuvant radiotherapy, three with cytotoxic therapy and five with adrenolytic treatment (the remaining didn't start mitotane due to death or difficulties in obtaining the drug). Two patients, on stage II ENSAT, maintain follow up with mitotane and no evidence of recurrence. The mean overall survival was 59.2±52.4 months for stages I+II and 6.2±6.8 months for stages III+IV (ENSAT) (p=0.004). Conclusion:Our series reinforces the importance of early diagnosis by demonstrating a worse prognosis in more advanced stages and the need for a multidisciplinary approach from a team of experts in the management of such a rare malignancy.

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“…Advanced ACC has a poor prognosis [2,6,30]. In our series the mean overall survival for ENSAT stages III and IV was quite disappointing.…”

Section: Discussionmentioning

confidence: 52%

Section: Discussionmentioning

confidence: 99%

“…Treatment options for advanced ACC are limited [30]. Results of cytotoxic chemotherapy have been disappointing [30].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Adrenocortical Carcinoma - A 25 Years Tertiary Centre Experience and Short Review of the Literature

Nunes¹

2018

EMIJ

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“…In this case, the presentation is classical and other siblings should be offered testing at around 18 years of age unless there are earlier symptoms. Advances in surgery and, more recently, in chemotherapy, with mammalian target of rapamycin signaling inhibitors including everolimus and with tyrosine kinase inhibitors such as sorafenib, have allowed better prognosis in this disorder [22]. Individual with sarcoma at Ͻ45 years of age A first-degree relative with any cancer at Ͻ45 years of age A third family member who is a first-or second-degree relative with either a sarcoma at any age or any cancer at age Ͻ45 years Tumors seen include soft tissue sarcoma, osteosarcoma, brain tumors including the choroid plexus, adrenocortical cancer, Wilms' tumor, leukemia…”

Section: Case D: Lfsmentioning

confidence: 99%

Familial Pediatric Endocrine Tumors

et al. 2011

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After completing this course, the reader will be able to:1. Explain the contribution of genetics to heritable aspects of pediatric cancer.2. Describe the applications of presymptomatic gene testing in family members with familial pediatric cancers to the early detection, prevention, and management of tumors in patients and their family members.This article is available for continuing medical education credit at CME.TheOncologist.com. CME CME ABSTRACT

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