Adrenocortical Carcinoma: Clinical Update

Endocrinology and Diabetes Unit, Department of Medicine I, University Hospital Wuerzburg, Josef-Schneider-Str. 2, 97080 Wuerzburg, Germany.
Journal of Clinical Endocrinology &amp Metabolism (Impact Factor: 6.31). 06/2006; 91(6):2027-37. DOI: 10.1210/jc.2005-2639
Source: PubMed

ABSTRACT Adrenocortical carcinoma (ACC) is a rare and heterogeneous malignancy with incompletely understood pathogenesis and poor prognosis. Patients present with hormone excess (e.g. virilization, Cushing's syndrome) or a local mass effect (median tumor size at diagnosis > 10 cm). This paper reviews current diagnostic and therapeutic strategies in ACC.
Original articles and reviews were identified using a PubMed search strategy ( covering the time period up until November 2005. The following search terms were used in varying combinations: adrenal, adrenocortical, cancer, carcinoma, tumor, diagnosis, imaging, treatment, radiotherapy, mitotane, cytotoxic, surgery.
Tumors typically appear inhomogeneous in both computerized tomography and magnetic resonance imaging with necroses and irregular borders and differ from benign adenomas by their low fat content. Hormonal analysis reveals evidence of steroid hormone secretion by the tumor in the majority of cases, even in seemingly hormonally inactive lesions. Histopathology is crucial for the diagnosis of malignancy and may also provide important prognostic information. In stages I-III open surgery by an expert surgeon aiming at an R0 resection is the treatment of choice. Local recurrence is frequent, particularly after violation of the tumor capsule. Surgery also plays a role in local tumor recurrence and metastatic disease. In patients not amenable to surgery, mitotane (alone or in combination with cytotoxic drugs) remains the treatment of choice. Monitoring of drug levels (therapeutic range 14-20 mg/liter) is mandatory for optimum results. In advanced disease, the most promising therapeutic options (etoposide, doxorubicin, cisplatin plus mitotane, and streptozotocin plus mitotane) are currently being compared in an international phase III trial ( Adjuvant treatment options after complete tumor removal (e.g. mitotane, radiotherapy) are urgently needed because postoperative disease-free survival at 5 yr is only around 30%, but options have still not been convincingly established. National registries, international cooperations, and trials provide important new structures for patients but also for researchers aiming at systematic and continuous progress in ACC. However, future advances in the management of ACC will mainly depend on a better understanding of the molecular pathogenesis facilitating the use of modern cancer treatments (e.g. tyrosine kinase inhibitors).

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    • "Likewise, in our case, the tumor diameter size was 12 cm. Adrenocortical carsinomas (ACCs) are thought to be very rare with incidence of approximately one or two per million population per year [16]. Although most cases of ACCs appear to be sporadic, some have been related as a component of several hereditary syndromes (Li- Fraumeni Syndrome, Beckwith-Wiedemann Syndrome etc.). "
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    • "Functioning tumors occur in an estimated 40–60% of all adult patients, and these tumors may secrete excessive glucocorticoids (e.g., Cushing's syndrome), mineralocorticoids, and their downstream products, including androgens (virilization), estrogens (feminization), and aldosterone, which can cause hypertension [3] [7]. In contrast, non-functioning tumors may present with pain or distention in the abdomen due to mass effect, weight loss, weakness, fever, and myalgia, or may be discovered incidentally during un-related radiographic imaging (e.g., abdominal CT or magnetic resonance imaging) [8] [9]. In this case, the patient was referred to our hospital based on a suspicion of hyperaldosteronism, due to pedal edema, hypokalemia, and hypertension, and Cushing's syndrome was confirmed by the loss of the diurnal cortisol patterns and elevated urine levels of free cortisol. "
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    ABSTRACT: Adrenal cortical carcinoma (ACC) is a very rare type of tumor that generally has a poor prognosis. Little has been reported on repeated liver resections with recurrent metastasis still confined to the liver. In this report, we describe a case of functioning ACC in a 65-year-old woman with 2 liver metastases of the ACC (at 1.5 and 4 years) after the right adrenalectomy. A 65-year-old woman was referred to our hospital based on a suspicion of hyperaldosteronism. Abdominal computed tomography revealed a lesion at the right adrenal gland; therefore, we performed right adrenalectomy and subsequently diagnosed the lesion as ACC. However, follow-up computed tomography at 1.5 and 4 years after the right adrenalectomy revealed liver metastasis of ACC; liver resection was performed for both metastases. Complete surgical resection is the established approach for the treatment of ACC. The prognosis of ACC is usually dismal, and recurrence rates of up to 85% have been reported. However, the appropriate treatment for recurrent ACC is not well established, and the effectiveness of other modalities, such as chemotherapy and radiotherapy, is not proven. Therefore, surgical resection may currently be the most appropriate treatment modality, as the patient achieved a disease-free interval of 2.5 years after the first liver resection. In selected patients with recurrent or metastatic ACC, resection is likely to be associated with prolonged survival. However, a full cure is generally not achievable, and a multidisciplinary approach is likely needed to achieve long-term disease-free status and survival. Copyright © 2015 Z. Published by Elsevier Ltd.. All rights reserved.
    02/2015; 7. DOI:10.1016/j.ijscr.2015.02.041
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    • "The incidence of ACC is estimated to be one to two cases per million people [33]. These tumors most often present with clinical symptoms due to adrenal hormone hypersecretion, most notably cortisol [34]. In this series of 49 patients, four adrenal malignancies were identified, two of which were ACC. "
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    ABSTRACT: BACKGROUND: Adrenal masses are common incidental findings on radiologic imaging. The association between malignancy and hormonal hyperactivity found in incidentally discovered adrenal tumors, however, remains unclear. METHODS: A retrospective analysis of prospectively collected data from patients who underwent adrenalectomy for incidentally discovered adrenal tumors at a single institution. Outcomes and operative data were compared by univariate analysis. Area under the curve was used to analyze the effect of tumor size in predicting malignancy. RESULTS: There were 49 patients who initially presented with adrenal incidentalomas that underwent adrenalectomy. Most patients were Caucasian women with an average age of 51 ± 14 years. Of this group, 24 patients underwent resection for hyperfunctioning adrenal glands. There were no significant differences in malignancy rates between hyperfunctional and nonfunctional tumors (4.1% vs. 12.0%, P = 0.32). On final histopathology, there were four patients with adrenal malignancies: two adrenocortical carcinomas and two metastatic from renal carcinoma. Only one patient with a hyperfunctioning adrenal tumor had underlying malignancy. Overall, invasion of adjacent structures (P < 0.001), presence of lymphadenopathy (P = 0.02), metastasis (P = 0.03), irregular tumor margins (P = 0.01), heterogeneity (P = 0.05), and tumor size >6 cm (P = 0.04) on radiologic imaging were strongly associated with malignancy in adrenal incidentalomas. CONCLUSIONS: The risk of concomitant malignancy and hormonal hyperactivity in adrenal incidentalomas is very low. Tumor size (>6 cm) and radiographic features remain the most important predictors of adrenal malignancy, regardless of tumor function.
    Journal of Surgical Research 03/2013; DOI:10.1016/j.jss.2013.03.032 · 2.12 Impact Factor
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