Adrenocortical carcinoma: The management of metastatic disease

Fay, André P.; Elfiky, Aymen; Teló, Gabriela Heiden; McKay, Rana R.; Kaymakcalan, Marina D.; Nguyen, Paul L.; Vaidya, Anand; Ruan, Daniel T.; Bellmunt, Joaquim; Choueiri, Toni K.

doi:10.1016/j.critrevonc.2014.05.009

Cited by 54 publications

(66 citation statements)

References 90 publications

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“…23 Most patients with ACC experience rapid disease progression, and the median overall survival (OS) remains dismal at 9 months for patients with stage IV disease. 24 Mitotane, the only FDA-approved agent for ACC, is often used in combination with etoposide, doxorubicin, and cisplatin. 25 However, treatment with mitotane and chemotherapy is associated with considerable toxicity that frequently makes it difficult for patients to continue therapy.…”

Section: Discussionmentioning

confidence: 99%

Phase 2 study of pembrolizumab in patients with advanced rare cancers

Naing

Meric‐Bernstam

Stephen

et al. 2020

J Immunother Cancer

108

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BackgroundPatients with advanced rare cancers have poor prognosis and few treatment options. As immunotherapy is effective across multiple cancer types, we aimed to assess pembrolizumab (programmed cell death 1 (PD-1) inhibitor) in patients with advanced rare cancers.MethodsIn this open-label, phase 2 trial, patients with advanced rare cancers whose tumors had progressed on standard therapies, if available, within the previous 6 months were enrolled in nine tumor-specific cohorts and a 10th cohort for other rare histologies. Pembrolizumab 200 mg was administered intravenously every 21 days. The primary endpoint was non-progression rate (NPR) at 27 weeks; secondary endpoints were safety and tolerability, objective response rate (ORR), and clinical benefit rate (CBR).ResultsA total of 127 patients treated between August 15, 2016 and July 27, 2018 were included in this analysis. At the time of data cut-off, the NPR at 27 weeks was 28% (95% CI, 19% to 37%). A confirmed objective response (OR) was seen in 15 of 110 (14%) evaluable patients (complete response in one and partial response in 14). CBR, defined as the percentage of patients with an OR or stable disease ≥4 months, was 38% (n=42). Treatment was ongoing in 11 of 15 patients with OR at last follow-up. In the cohort with squamous cell carcinoma (SCC) of the skin, the NPR at 27 weeks was 36%, ORR 31%, and CBR 38%. In patients with adrenocortical carcinoma (ACC), NPR at 27 weeks was 31%, ORR 15%, and CBR 54%. In the patients with carcinoma of unknown primary (CUP), NPR at 27 weeks was 33%, ORR 23%, and CBR 54%. In the paraganglioma–pheochromocytoma cohort, NPR at 27 weeks was 43%, ORR 0%, and CBR 75%. Treatment-related adverse events (TRAEs) occurred in 66 of 127 (52%) patients, and 12 (9%) had grade ≥3 TRAEs. The most common TRAEs were fatigue (n=25) and rash (n=17). There were six deaths, all of which were unrelated to the study drug.ConclusionsThe favorable toxicity profile and antitumor activity seen in patients with SCC of skin, ACC, CUP, and paraganglioma–pheochromocytoma supports further evaluation of pembrolizumab in this patient population.Trial registration numberNCT02721732

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Section: Discussionmentioning

confidence: 99%

Phase 2 study of pembrolizumab in patients with advanced rare cancers

Naing

Meric‐Bernstam

Stephen

et al. 2020

J Immunother Cancer

108

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“…ACC is a rare and highly malignant endocrine neoplasm accounting for an estimated 0.02% of all cancer cases [5,6]. The majority of adrenal tumors are mostly sporadic and unilateral but some of them are associated with Li -Fraumeni syndrome, type1 mul tiple endocrine neoplasia, Beckwith -Wiedemann syndrome and Carney complex [10,13].…”

Section: Discussionmentioning

confidence: 99%

A Case Report of Late Local Relapse of Adrenocortical Carcinoma 18 Years After Adrenalectomy

Tkachenko¹,

Головко²,

Kuryk³

2018

Exp. Onc.

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Adrenocortical cancer is an extremely rare tumor presenting with extensive locoregional spread at the time of diagnosis. Due to the diagnostic difficulties preoperatively and a lack of effective treatment options, patients have poor prognosis. Patients succumb to metastases within a couple of months. Only 20 cases have been so far reported in the literature with a medium disease-free survival up to 2 years. We present a case of a locoregional recurrence of adrenocortical cancer 18 years after left adrenalectomy.

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“…ACC is a rare and highly aggressive malignancy accounting for an estimated 0.02% of all cancers [5]. Adrenal tumors are mostly sporadic and unilateral but some of them are bilateral and associated with Li-Fraumeni Syndrome, Type-1 Multiple Endocrine Neoplasia, Beckwith-Wiedemann Syndrome, and Carney complex [6].…”

Section: Discussionmentioning

confidence: 99%

A Case Report of Bilateral Adrenal Sarcomatoid Carcinoma

İyidir

Cerit

Özkan

et al. 2016

Case Reports in Surgery

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Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy. Sarcomatoid adrenal carcinoma is even more aggressive type of ACC. Bilateral malignant adrenal tumors are extremely rare except for those that represent metastasis from an extra-adrenal organ. Here we report a 53-year-old woman who presented with abdominal pain and weight loss. Abdominal computed tomography revealed bilateral adrenal masses and a mass in her liver. Surgical specimens showed pleomorphic tumor cells with epithelial and spindle cell morphology and immunohistochemical staining was compatible with sarcomatoid carcinoma. Sarcomatoid adrenal carcinoma should be kept in mind during the management of bilateral adrenal masses.

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Adrenocortical carcinoma: The management of metastatic disease

Cited by 54 publications

References 90 publications

Phase 2 study of pembrolizumab in patients with advanced rare cancers

Phase 2 study of pembrolizumab in patients with advanced rare cancers

A Case Report of Late Local Relapse of Adrenocortical Carcinoma 18 Years After Adrenalectomy

A Case Report of Bilateral Adrenal Sarcomatoid Carcinoma

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