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      Pure aldosterone-secreting adrenocortical carcinoma in a patient with refractory primary hyperaldosteronism

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          We describe a young male patient with longstanding hypertension, who was diagnosed with primary hyperaldosteronism and treated by an attempted retroperitoneoscopic total unilateral adrenalectomy for a left-sided presumed aldosterone-secreting adenoma. Imaging had shown an unremarkable focal adrenal lesion with normal contralateral adrenal morphology, and histology of the resected specimen showed no adverse features. Post-operatively, his blood pressure and serum aldosterone levels fell to the normal range, but 9 months later, his hypertension recurred, primary aldosteronism was again confirmed and he was referred to our centre. Repeat imaging demonstrated an irregular left-sided adrenal lesion with normal contralateral gland appearances. Adrenal venous sampling was performed, which supported unilateral (left-sided) aldosterone hypersecretion. Redo surgery via a laparoscopically assisted transperitoneal approach was performed and multiple nodules were noted extending into the retroperitoneum. It was thought unlikely that complete resection had been achieved. His blood pressure returned to normal post-operatively, although hypokalaemia persisted. Histological examination, from this second operation, showed features of an adrenocortical carcinoma (ACC; including increased mitoses and invasion of fat) that was assessed as malignant using the scoring systems of Weiss and Aubert. Biochemical hyperaldosteronism persisted post-operatively, and detailed urine steroid profiling showed no evidence of adrenal steroid precursors or other mineralocorticoid production. He received flank radiotherapy to the left adrenal bed and continues to receive adjunctive mitotane therapy for a diagnosis of a pure aldosterone-secreting ACC.

          Learning points

          • Pure aldosterone-secreting ACCs are exceptionally uncommon, but should be considered in the differential diagnosis of patients presenting with primary aldosteronism.

          • Aldosterone-producing ACCs may not necessarily show typical radiological features consistent with malignancy.

          • Patients who undergo surgical treatment for primary aldosteronism should have follow-up measurements of blood pressure to monitor for disease recurrence, even if post-operative normotension is thought to indicate a surgical ‘cure’.

          • Owing to the rarity of such conditions, a greater understanding of their natural history is likely to come from wider cooperation with, and contribution to, large multi-centre outcomes databases.

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          Most cited references 5

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          Comparative histologic study of 43 metastasizing and nonmetastasizing adrenocortical tumors.

           Mark L. Weiss (1984)
          A series of 43 adrenocortical tumors was analyzed using nine histologic features. Mitotic activity, especially with atypical forms, and venous invasion correlated best with metastasizing or recurring tumors; however, no single criterion was useful alone. The combination of the following nine criteria was most useful in distinguishing malignant from benign tumors: nuclear grade III or IV; mitotic rate greater than 5/50 high-power fields; atypical mitoses; clear cells comprising 25% or less of the tumor; a diffuse architecture; microscopic necrosis; and invasion of venous, sinusoidal, and capsular structures. None of the 24 tumors with two or less of these criteria metastasized or recurred, while all but one of the 19 tumors with four or more of these criteria either recurred or metastasized.
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            Approach to the patient with an adrenal incidentaloma.

             K. Nieman (2010)
            Unsuspected adrenal masses, or incidentalomas, are increasingly found with the widespread use of thoracic and abdominal imaging. These masses may be hormonally active or nonfunctional and malignant or benign. Clinicians must determine the nature of the mass to decide what treatment, if any, is needed. Measurement of precontrast Hounsfield units (HU) and contrast washout on computed tomography scan provide useful diagnostic information. All patients should undergo biochemical testing for pheochromocytoma, either with plasma or urinary catecholamine measurements. This is particularly important before surgical resection, which is routinely recommended for masses larger than 4 cm in diameter without a clear-cut diagnosis and for others with hormonal secretion or ominous imaging characteristics. Hypertensive patients should undergo biochemical testing for hyperaldosteronism. Patients with features consistent with Cushing's syndrome, such as glucose intolerance, weight gain, and unexplained osteopenia, should be evaluated for cortisol excess. Here, the dexamethasone suppression test and late-night salivary cortisol may be preferred over measurement of urine cortisol. The ability of surgical resection to reverse features of mild hypercortisolism is not well established. For masses that appear to be benign ( 50%), small (<3 cm), and completely nonfunctioning, imaging and biochemical reevaluation (pheochromocytoma and hypercortisolism only) at 1-2 yr (or more) is appropriate. For more indeterminate lesions, repeat evaluation for growth after 3-12 months is useful, with subsequent testing intervals based on the rate of growth.
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              Weiss system revisited: a clinicopathologic and immunohistochemical study of 49 adrenocortical tumors.

              The definitive diagnostic criteria for malignant adrenocortical tumors are distant metastasis and/or local invasion. The Weiss histopathologic system is the most commonly used method for assessing malignancy because of its simplicity and reliability. Unfortunately, its application remains subjective. This current retrospective study evaluated the Weiss system and assessed the value of MIB-1 labeling in the diagnosis of adrenocortical malignancy. Twenty-four malignant tumors with distant metastasis, gross local invasion, or recurrence were selected and matched on their functioning status to 25 benign tumors. Two independent observers delineated the Weiss criteria. An MIB-1 labeling index was determined. Presence of three or more Weiss microscopic criteria was related to malignancy (specificity 96%, sensitivity 100%), thus confirming the value of the Weiss system. Interobserver agreement for the Weiss system (total score) was excellent (r = 0.94). The lack of reliability for some Weiss criteria led us to propose a statistically modified system, based on the most reliable criteria (2.mitotic rate x 2.cytoplasm x abnormal mitoses x necrosis x capsular invasion) with a significant correlation with the Weiss system (r = 0.98). The MIB-1 labeling index was significantly higher in malignant tumors (p <0.0001). MIB1 could also help to differentiate malignant from benign adrenocortical tumors.

                Author and article information

                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                EDM Case Reports
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                21 July 2015
                : 2015
                [1 ]Department of Endocrinology, St Bartholomew's Hospital , London, UK
                [2 ]Department of Pathology, St Bartholomew's Hospital , London, UK
                [3 ]Department of Surgery, St Bartholomew's Hospital , London, UK
                [4 ]Department of Radiology, St Bartholomew's Hospital , London, UK
                [5 ]Department of Endocrinology, Galway University Hospital , Galway, UK
                Author notes
                Correspondence should be addressed to S Hussain Email: shaziahussain@
                © 2015 The authors
                Unique/Unexpected Symptoms or Presentations of a Disease


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