Adrenocortical carcinoma

Roman, Sanziana A.

doi:10.1097/01.cco.0000198976.43992.14

Cited by 77 publications

(88 citation statements)

References 36 publications

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“…Adrenocortical carcinoma (ACC) is a rare and aggressive endocrine tumor showing an annual incidence of 0.5-2 cases/million population (1,2). Surgery is the mainstay of therapy and complete surgical removal of ACC is the most important prognostic factor for longterm survival together with stage.…”

Section: Introductionmentioning

confidence: 99%

Mitotane levels predict the outcome of patients with adrenocortical carcinoma treated adjuvantly following radical resection

Terzolo

Baudin

Ardito

et al. 2013

European Journal of Endocrinology

121

109

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Context: Mitotane plasma concentrations R14 mg/l have been shown to predict tumor response and better survival in patients with advanced adrenocortical carcinoma (ACC). A correlation between mitotane concentrations and patient outcome has not been demonstrated in an adjuvant setting. Objective: To compare recurrence-free survival (RFS) in patients who reached and maintained mitotane concentrations R14 mg/l vs patients who did not. Design and setting: Retrospective analysis at six referral European centers. Patients: Patients with ACC who were radically resected between 1995 and 2009 and were treated adjuvantly with mitotane targeting concentrations of 14-20 mg/l. Main outcome measures: RFS (primary) and overall survival (secondary). Results: Of the 122 patients included, 63 patients (52%) reached and maintained during a median follow-up of 36 months the target mitotane concentrations (group 1) and 59 patients (48%) did not (group 2). ACC recurrence was observed in 22 patients of group 1 (35%) and 36 patients in group 2 (61%). In multivariable analysis, the maintenance of target mitotane concentrations was associated with a significantly prolonged RFS (hazard ratio (HR) of recurrence: 0.418, 0.22-0.79; PZ0.007), while the risk of death was not significantly altered (HR: 0.59, PZ0.20). Grades 3-4 toxicity was observed in 11 patients (9%) and was managed with temporary mitotane discontinuation. None of the patients discontinued mitotane definitively for toxicity. Conclusions: Mitotane concentrations R14 mg/l predict response to adjuvant treatment being associated with a prolonged RFS. A monitored adjuvant mitotane treatment may benefit patients after radical removal of ACC.

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Section: Introductionmentioning

confidence: 99%

Mitotane levels predict the outcome of patients with adrenocortical carcinoma treated adjuvantly following radical resection

Terzolo

Baudin

Ardito

et al. 2013

European Journal of Endocrinology

121

109

View full text Add to dashboard Cite

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“…The female:male ratio in this small sample size was 1:1. The majority of studies have demonstrated that women are more susceptible to the disease (1,28). Based on the collected data, there appear to be no significant differences in morbidity rates between male and female patients (Table 1).…”

Section: Discussionmentioning

confidence: 95%

“…Adrenal sarcomatoid carcinomas are more likely to occur in the kidney or bladder, rather than the adrenal glands (17). These carcinomas may be classified as either functional or nonfunctional tumors (1). Patients with functional tumors may present with symptoms caused by excessive hormones, including the following: i) Cushing's syndrome; ii) severe hypertension; iii) male feminization or iv) female masculinization.…”

Section: Discussionmentioning

confidence: 99%

“…Patients with functional tumors may present with symptoms caused by excessive hormones, including the following: i) Cushing's syndrome; ii) severe hypertension; iii) male feminization or iv) female masculinization. Patients with nonfunctional tumors may present with symptoms including the following: i) Weight loss; ii) abdominal masses; iii) abdominal pain; iv) low fever and v) asthenia (1).…”

Section: Discussionmentioning

confidence: 99%

“…Adrenocortical carcinoma is a rare malignant tumor, accounting for ~0.02% of global malignancies (1). Adrenocortical carcinoma has a bimodal distribution by age, with one peak age before 5 years, and the other between 40 and 50 years old, as well as a female predominance (2).…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Primary adrenal sarcomatoid carcinoma metastatic to the lung: Case report and review of the literature

Zhu¹,

Zheng²,

Mao³

et al. 2016

Oncology Letters

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Abstract. Adrenal sarcomatoid carcinoma is a rare adrenal carcinoma. To the best of our knowledge, only 11 cases have been reported since 1987. Adrenal sarcomatoid carcinoma presents a diagnostic challenge due to its atypical symptoms and histological patterns. At the time of diagnosis, a large percentage of patients are already at the metastatic stage and succumb within a few months. The present study reports a case of a 59-year-old man presenting with asthenia and weight loss with adrenal sarcomatoid carcinoma metastatic to the lung. A computed tomography (CT) scan and ultrasonography of the patient's abdomen suggested a large homogeneous mass in the right adrenal gland, and a CT scan of his chest suggested lung metastasis. Right adrenalectomy was performed. Histological examination revealed that the tumor was composed of sarcomatous and carcinomatous differentiation elements. Immunohistochemical examination revealed tumor cell positivity for vimentin and cytokeratin. At the 6-month follow-up the patient exhibited no disease progression and refused further proposed treatment. The patient was alive at the time of writing the current report. The present case report additionally reviews the literature, for the purpose of raising awareness of these rare lesions and assisting in achieving accurate diagnoses and effective treatment.

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Advanced adrenocortical carcinoma successfully treated with gemcitabine plus capecitabine as second‐line chemotherapy

et al. 2020

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Introduction: Adrenocortical carcinoma is a rare malignant tumor with an unfavorable prognosis in the advanced stage for which second-/third-line chemotherapy is not well established. Case presentation: A 34-year-old woman was referred to our institution for left adrenal tumor with multiple liver metastases and tumor thrombus extending to the inferior vena cava. According to her clinical diagnosis of adrenocortical carcinoma (T4N0M1, European Network for the Study of Adrenal Tumors stage IV), we resected the left adrenal tumor and tumor thrombus. Pathological examination confirmed the adrenocortical carcinoma diagnosis. After four courses of etoposide, doxorubicin, cisplatin, and mitotane therapy, the liver metastases progressed, and we started gemcitabine, capecitabine, and mitotane therapy as second-line chemotherapy. After 7 months, significant shrinkage of the liver metastases was observed, and they remained stable over 16 months. Conclusion: We reported a case of advanced adrenocortical carcinoma with significant shrinkage of liver metastases following gemcitabine, capecitabine, and mitotane therapy, with the effect maintained over 16 months.

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Adrenocortical carcinoma

Cited by 77 publications

References 36 publications

Mitotane levels predict the outcome of patients with adrenocortical carcinoma treated adjuvantly following radical resection

Mitotane levels predict the outcome of patients with adrenocortical carcinoma treated adjuvantly following radical resection

Primary adrenal sarcomatoid carcinoma metastatic to the lung: Case report and review of the literature

Advanced adrenocortical carcinoma successfully treated with gemcitabine plus capecitabine as second‐line chemotherapy

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