Anna Kasperlik-Zaluska

Incidentally discovered adrenal mass (incidentaloma): investigation and management of 208 patients

OBJECTIVE Incidentally discovered adrenal masses are fairly common, although there are some controversies concerning the results of hormonal investigation (especially DHEAS values) and the methods of management. We summarize our experience in diagnosis, pathological findings and treatment of a large group of patients with incidentally found adrenal tumours.

Secondary adrenal insufficiency associated with autoimmune disorders: a report of twenty-five cases

Addisons disease is frequently a component of autoimmune polyendocrinopathies while secondary adrenal insufficiency associated with autoimmune disorders is believed to be a rare event. We present a series of patients with secondary adrenal insufficiency coexisting with autoimmune diseases and/or antithyroid autoantibodies.

The ratios of aldosterone / plasma renin activity (ARR) versus aldosterone / direct renin concentration (ADRR).

Primary aldosteronism (PA) is estimated to occur in 5–12% of patients with hypertension. Assessment of aldosterone / plasma renin activity (PRA) ratio (ARR) has been used as a screening test in patients suspected of PA. Direct determination of renin (DRC) and calculation of aldosterone / direct renin concentration ratio (ADRR) could be similarly useful for screening patients suspected of PA. The study included 62 patients with indication for evaluation of the renin-angiotensin-aldosterone system and 35 healthy volunteers. In all participants we measured concentrations of serum aldosterone, plasma direct renin, and PRA after a night’s rest and again after walking for two hours. The concentrations of aldosterone, direct renin, and PRA were measured by isotopic methods (radioimmunoassay (RIA) / immunoradiometric assay (IRMA)). Correlations of ARR with ADRR in the supine position were r = 0.9162, r2 = 0.8165 (p < 0.01); and in the up-right position were r = 0.7765, r2 = 0.9153 (p < 0.01). The cut-off values of ARR and ADRR ≥ 100 presented highest specificity (99%) for the diagnosis of PA; however, quite acceptable specificity and sensitivity (> 80% and 100%, respectively) appeared for the ratios ≥ 30. We suggest that for practical and economic reasons ARR can be replaced by ADRR.

Ectopic ACTH syndrome due to bilateral ovarian androblastoma with double, gynandroblastic differentiation in one ovary

A case of fulminant Cushings syndrome due to an ectopic ACTH secretion in a patient with bilateral ovarian sex-cord stromal tumour is reported. Surgical resection of the ovaries as well as the inhibitors of steroid synthesis and cytostatics caused only transient improvement because the widespread neoplastic dissemination progressed very quickly.

Successful pregnancy following surgery for a masculinizing adrenocortical carcinoma.

A 16-year-old girl with a masculinizing adrenal carcinoma treated surgically is presented. Following surgery a full clinical and steroid remission has been achieved, without any adjuvant chemotherapy. In the 7th year after the surgical treatment the patient became pregnant. Pregnancy and labour were uncomplicated. The patient continues in complete remission, and her son has been noted to have normal development at 21 mth of age.

Shear wave elastography of adrenal masses is feasible and may help to differentiate between solid and cystic lesions - an initial report.

INTRODUCTION The aim of this study was to evaluate the feasibility and usefulness of supersonic shear wave elastography (SSWE) in the diagnosis of nonmalignant adrenal masses. MATERIAL AND METHODS 13 patients with a total number of 16 adrenal masses were enrolled in the study. In each case, both conventional ultrasound imaging and SSWE for stiffness assessment were performed. The final diagnosis was based on CT, MRI, biochemical studies, surgery or more than one year of follow up. RESULTS The final diagnosis: nodular hyperplasia in six masses, six adenomas, three cysts, and one myelolipoma. All solid adrenal masses presented the elastography signal in contrast to cystic lesions that were devoid of it, as shear waves do not propagate through fluids. CONCLUSIONS SSWE is a feasible technique that can be applied during ultrasound of the abdomen and retroperitoneum. SSWE presents potential for the differentiation of solid and cystic adrenal lesions. Further large scale studies evaluating the possibility of differentiation of adrenal and other retroperitoneal masses with SSWE are warranted.

Adrenal 131I-6β-iodomethylnorcholesterol scintigraphy in choosing the side for adrenalectomy in bilateral adrenal tumors with subclinical hypercortisolemia

PurposeAdrenal scintigraphy with 131I-6β-iodomethylnorcholesterol is considered by several authors the gold standard for assessing tumors with subclinical hypercortisolemia. However, most of the described series consist mainly of cases with unilateral lesions. The aim of our study was to assess whether scintigraphy is useful in choosing the adrenalectomy side in the case of bilateral adrenal tumors with subclinical hypercortisolemia.MethodsThe study focused on 15 consecutive patients with benign bilateral adrenal tumors and subclinical hypercortisolemia. The scintigraphy with 131I-6β-iodomethylnorcholesterol was performed. Fourteen patients underwent unilateral adrenalectomy; the gland with predominant uptake on scintigraphy was removed. Cortisol and ACTH concentrations were measured one and six months after surgery. Post-dexamethasone cortisolemia was assessed six months after surgery. To date, the patients have been under postoperative observation for 1–4 years.ResultsFour patients showed unilateral uptake of radiotracer, and nine patients showed predominant accumulation of radiotracer in one of the adrenal glands. The smaller tumor was predominant in 2 cases. Percentage of activity on the predominant side correlates positively with the difference between tumors’ diameters. Unilateral uptake of radiotracer predicts long-lasting postoperative insufficiency of the second adrenal gland. Excision of predominating tumor led to cessation of hypercortisolemia in all patients.ConclusionsThe corticoadrenal scintigraphy is useful in choosing the side for operation in the case of bilateral adrenal tumors with subclinical hypercortisolemia.

Chromogranin A (CgA) in adrenal tumours.

INTRODUCTION Adrenal tumours can produce specific hormones and cause characteristic symptoms. The majority of adrenal incidentalomas are clinically silent, but some may be malignant or pose other potential threats to life. Chromogranin A (CgA) is the main, nonspecific marker of neuroendocrine tumours (NET). In the adrenals, CgA is produced by chromaffin cells localised in the adrenal medulla. Therefore its measurement in blood might be used as a screening test for pheochromocytoma after the exclusion of other various causes of an increased CgA level. The aim of our study was to investigate plasma CgA concentration in various adrenal tumours. MATERIAL AND METHODS EDTA2K plasma samples were obtained from 195 patients with adrenal tumours and from 50 blood donors. CgA was measured in plasma-EDTA2K by immunoradiometric (IRMA) method. RESULTS In the majority of patients with adrenal tumours not derived from neuroendocrine cells (chromaffin cells), except those with significant hypercortisolaemia and some patients with adrenal carcinoma, the plasma CgA concentrations were below the cut-off value. In adrenal tumours derived from chromaffin cells (pheochromocytoma), CgA levels were markedly elevated. CONCLUSION In differential diagnosis of adrenal tumours, non-increased CgA level might be useful initial screening evidence for the exclusion of pheochromocytoma.

Three-Dimensional Sonography in Diagnosis of Retroperitoneal Hemorrhage From Adrenocortical Carcinoma

onography has limited value in visualization and differentiation of adrenal masses, although sonography of the abdomen can usually show adrenal tumors with a maximal diameter of greater than 1 cm on the right side and 1.5 to 2 cm on the left side.1 Small tumors often have a homogeneous echo texture that becomes heterogeneous as the tumor grows.2 Central necrosis or hemorrhage within the tumor may result in a hypoechoic area.3 Sonography is useful for follow-up of nonhyperfunctioning adrenal masses diagnosed on the basis of computed tomography (CT) or magnetic resonance imaging as adenomas.4 Sonography is the method of choice for the visualization of adrenal masses in small children.5,6 Diagnosis of adrenal masses in pregnant women can be established only with noninvasive methods such as sonography or magnetic resonance imaging.7 Three-dimensional sonography has been recognized and studied for 20 years.8 However, only recently, because of developments in computer techniques and in the construction of scanners, has the acquisition of volumes with automatic 3-dimensional (3D) probes become more simple and the quality of the images acquired in 3D sonography improved to compare with conventional sonographic images. With the matrix or automatic 3D probes and fast computers now available, even real-time 3D sonography (4-dimensional sonography) has become possible. In 3D sonography, the volume of tissues can be analyzed interactively in 3 perpendicular planes in the multiplanar reformation (MPR) mode or as a rendered image of the 3D object on a plane through the application of various algorithms. From 2001 through 2003, we investigated the application of 3D sonography for the evaluation of adrenal masses. The purpose of the procedure was explained to the patients, and their informed consent was obtained. Here we report the diagnosis of retroperitoneal hemorrhage from adrenal carcinoma on the basis of 3D sonography. Received May 10, 2004, from the Department of Diagnostic Imaging (R.Z.S., M.S.-K., W.J.) and Second Department of Surgery (J.A.P.), Medical Faculty II, Medical University of Warsaw, Warsaw, Poland; Department of Endocrinology, Center for Postgraduate Medical Education, Warsaw, Poland (A.A.K.-Z.); and Department of Pathomorphology, Czerniakowski Hospital, Warsaw, Poland (K.B.). Revision requested May 18, 2004. Revised manuscript accepted for publication June 14, 2004. This study was supported by State Committee for Scientific Research (Komitet Badan Naukowych) grant 6 P05B 072 20. Address correspondence and reprint requests to Rafal Z. Slapa, MD, Department of Diagnostic Imaging, Medical Faculty II, Medical University of Warsaw, ulica Kondratowicza 8, 03-242 Warsaw, Poland. E-mail: r.slapa@acn.waw.pl. Abbreviations CT, computed tomography; MPR, multiplanar reformation; 3D, 3-dimensional; 2D, 2-dimensional

Isolated secondary adrenal insufficiency – an underestimated consequence of asymptomatic autoimmune hypophysitis

We read with great interest the paper by Trevor A. Howlett et al. on autoimmune hypophysitis and we found it very important for management of this disorder. Autoimmune hypophysitis with clinical features of a pituitary mass is a rare event. We believe that a subclinical form of this disease is much more frequent in clinical practice. We have in our observation a group of 305 patients with idiopathic isolated secondary adrenal insufficiency. Interestingly, 203 (67%) of them had associated various autoimmune disorders. Apart from the well-known hypothalamic-pituitary-adrenal suppression because of exogenous corticosteroids, only an autoimmune mechanism is able to influence selectively the secretory function of the corticotrophs in adult patients. The most frequent finding in MRI of our patients was partially empty sella turcica, probably as a consequence of asymptomatic autoimmune hypophysitis. Thyroid autoimmune diseases, both hypothyroidism and thyrotoxicosis, as well as Hashimoto’s disease without apparent clinical manifestations, were the most frequent autoimmune disorders in this group of patients. Vitiligo, premature ovarian failure, type 1 diabetes mellitus and pernicious anaemia were also rather frequently observed. Coexistence of these autoimmune abnormalities and antipituitary antibodies detected in some of the tested cases 5 suggested adrenocorticotrophic hormone deficiency of autoimmune origin. By all means, in our patients, there was no indication to perform pituitary biopsy. In our experience, it is very important to diagnose correctly patients with idiopathic isolated secondary adrenal insufficiency because some of them are falsely treated as gastroenteritis in situation of an imminent adrenal crisis.