Maciej Otto

Application of parametric ultrasound contrast agent perfusion studies for differentiation of hyperplastic adrenal nodules from adenomas—Initial study

OBJECTIVES To evaluate the possibilities of differentiation of non-malignant adrenal masses with the application of the new technique for the evaluation of enhancement after administration of an ultrasound contrast agent: parametric imaging. PATIENTS AND METHODS 34 non-malignant adrenal masses in 29 patients were evaluated in a dynamic examination after the administration of ultrasound contrast agent with parametric imaging. Patterns on parametric imaging of arrival time were evaluated. The final diagnosis was based on CT, MRI, biochemical studies, follow up and/or histopathology examination. RESULTS The study included: 12 adenomas, 10 hyperplastic nodules, 7 myelolipomas, 3 pheochromocytomas, hemangioma with hemorrhage and cyst. The pattern of peripheral laminar inflow of Sonovue on parametric images of arrival time of was 100% sensitive for hyperplastic nodules and 83% specific in regard to adenomas. CONCLUSIONS Parametric contrast enhanced ultrasound may accurately differentiate hyperplastic adrenal nodules from adenomas and could be complementary to CT or MRI. Incorporation of perfusion studies to CT or MRI could possibly enable one-shop complete characterization of adrenal masses. This could deliver additional information in diagnostics of patients with Conn Syndrome and warrants further studies in this cohort of patients.

Usefulness of Somatostatin Receptor Scintigraphy (99mTc-[HYNIC, Tyr3]-Octreotide) and 123I-Metaiodobenzylguanidine Scintigraphy in Patients with SDHx Gene-Related Pheochromocytomas and Paragangliomas Detected by Computed Tomography

Aims: The aim of this study was to assess the usefulness of somatostatin receptor scintigraphy (SRS) using 99mTc-[HYNIC, Tyr3]-octreotide (TOC) and 123I-metaiodobenzylguanidine (mIBG) in patients with SDHx-related syndromes in which paragangliomas were detected by computed tomography and to establish an optimal imaging diagnostic algorithm in SDHx mutation carriers. Methods: All carriers with clinical and radiological findings suggesting paragangliomas were screened by SRS and 123I-mIBG. Lesions were classified by body regions, i.e. head and neck, chest, abdomen with pelvis and adrenal gland as well as metastasis. Results: We evaluated 46 SDHx gene mutation carriers (32 index cases and 14 relatives; 28 SDHD, 16 SDHB and 2 SDHC). In this group, 102 benign tumors were found in 39 studied patients, and malignant disease was diagnosed in 7 patients. In benign tumors, the sensitivity of SRS was estimated at 77% and of 123I-mIBG at 22.0%. The SRS and mIBG sensitivity was found to be clearly region dependent (p < 0.001). The highest SRS sensitivity was found in head and neck paragangliomas (HNP; 91.4%) and the lowest was found in abdominal paragangliomas and pheochromocytomas (40 and 42.9%, respectively). The highest 123I-mIBG sensitivity was found in pheochromocytomas (sensitivity of 100%) and the lowest in HNP (sensitivity of 3.7%). In metastatic disease, SRS was superior to mIBG (sensitivity of 95.2 vs. 23.8%, respectively). Conclusion: SRS and 123I-mIBG single photon emission computed tomography (SPECT) sensitivity in SDHx patients is highly body region dependent. In malignant tumors, SRS is superior to 123I-mIBG SPECT.

Castleman's disease – an unusual indication for laparoscopic adrenalectomy

Castlemans disease is one of the rare entities which cause lymph node hyperplasia with no characteristic clinical symptoms. Pathomorphological examination usually enables diagnosis, especially in the case of a localized form. Its uncommon location in the retroperitoneal cavity is estimated at 12% in the literature. Asymptomatic nature of the disease and its untypical location in the adrenal field imitated incidentaloma of this gland in the imaging examinations. Surgical treatment, laparoscopic excision of the tumour (lymph node) and right adrenal gland from the lateral, transperitoneal approach allowed final diagnosis and offered effective therapy.

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.

Echogenicity of benign adrenal focal lesions on imaging with new ultrasound techniques - report with pictorial presentation.

Aim The aim of the research was to assess the echogenicity of benign adrenal focal lesions using new ultrasound techniques. Material and method 34 benign adrenal masses in 29 patients were analyzed retrospectively. The examinations were conducted using Aplio XG (Toshiba, Japan) ultrasound scanner with a convex probe 1–6 MHz in the B-mode presentation with the combined use of new ultrasound techniques: harmonic imaging and spatial compound sonography. The size of the adrenal tumors, their echogenicity and homogeneity were analyzed. Statistical analysis was conducted using the STATISTICA 10 software. Results The following adrenal masses were assessed: 12 adenomas, 10 nodular hyperplasias of adrenal cortex, 7 myelolipomas, 3 pheochromocytomas, a hemangioma with hemorrhage and a cyst. The mean diameter of nodular hyperplasia of adrenal cortex was not statistically different from that of adenomas (p = 0.075). The possibility of differentiating between nodular hyperplasia and adenoma using the parameter of hypoechogenicity or homogeneity of the lesion was demonstrated with the sensitivity and specificity of 100% and 41.7%, respectively. The larger the benign adrenal tumor was, the more frequently did it turn out to have a mixed and inhomogenous echogenicity (p < 0.05; ROC areas under the curve: 0.832 and 0.805, respectively). Conclusions A variety of echogenicity patterns of benign adrenal focal lesions was demonstrated. The image of an adrenal tumor correlates with its size. The ultrasound examination, apart from its indisputable usefulness in detecting and monitoring adrenal tumors, may also allow for the differentiation between benign lesions. However, for lesions found incidentally an algorithm for the assessment of adrenal incidentalomas is applicable, which includes computed tomography and magnetic resonance imaging.

7D.07: RETINAL ARTERIOLAR STRUCTURE IN PATIENTS WITH PHEOCHROMOCYTOMA.

Objective: The aim of the ongoing study is to analyze retinal arteriolar structure in patients with pheochromocytoma (Pheo), a form of secondary hypertension characterized by excessive catecholamine secretion as compared with well-matched patients with essential hypertension (EHT). Figure. No caption available. Design and method: We examined 24 (15f/9m) patients with Pheo (mean age 46.3 ± 11.8 years) and 24 (15f/9m) age, gender, body mass index, glycemic status and blood pressure levels matched patients (46.7 ± 12.0 years) with (EHT). In all patients evaluation of plasma free normetanephrine (NMN) and metanephrine (MN) concentrations by liquid chromatography with tandem mass spectrometry was performed. Diagnosis of pheochromocytoma was made based on increased free NMN and/or MN concentration and confirmed on pathological examination. Retinal arterioles morphology were assessed by Heidelberg Retina Flowmetry using scanning laser Doppler flowmetry (SLDF). Outer diameter (AD) and lumen diameter (LD) were measured with automatic full-field perfusion imaging analysis (AFFPIA). Wall-to-lumen ratio (WLR), wall thickness (WT) and wall cross-sectional area (WCSA) of retinal arterioles were calculated. Retinal capillary blood flow (RCF) was also assessed by SLDF. Results: Pheo and EHT groups were well matched for age, gender, BMI, office and ambulatory blood pressure levels as well as for glycemic status and number of hypertensive medication (p > 0.05 for all comparisons). Patients with pheochromocytoma were characterized by higher AD, LD, WT and WCSA as compared with EHT (Table). A tendency towards higher WLR in patients with pheochromocytoma as compared to EHT was also noted (Table). There was no difference in RCF between patients with pheochromocytoma and EHT. Conclusions: Patients with pheochromocytoma as compared with matched patients with essential hypertension are characterized by higher outer wall diameter, lumen diameter, higher wall thickness and higher wall cross-sectional area of retinal arterioles. This may indicate potential deleterious effect of high catecholamine levels on small caliber arterioles evaluated non-invasively by SLDF.

Prognostic factors in adrenocortical carcinoma: data from a large Polish series

Introduction Adrenocortical carcinoma (ACC) is a rare malignancy, associated with poor outcome and few therapeutic options. Despite increasing attention, the knowledge about the clinical course and treatment of these tumors is limited. Objectives Survival rates in ACC are still low and the percentage of relapse is high. Thus, it is crucial to identify the prognostic factors of overall survival (OS) and recurrence‑free survival (RFS). Patients and methods This was a retrospective analysis of 66 patients diagnosed with ACC between 2002 and 2015. Results The median OS was 43.5 months, 78.19 months for stage I + II, 22.95 months for stage III, and 19.54 months for stage IV ACC. Older age, stage IV ACC, margin status R2, and no mitotane treatment were associated with poor OS. Low Ki67 and mitotic indices were related to improved OS in a univariate analysis. The median RFS was 101.1 months. Disease recurrence after potentially curative surgery was reported in 1 patient (25%) with stage I, 12 patients (46%) with stage II, and 9 patients (45%) with stage III ACC. Male sex and no mitotane treatment were associated with a reduced RFS in a multivariate analysis and higher Ki67 and mitotic indices in the univariate analysis. Conclusions Ki67 and mitotic indices should be considered as prognostic factors when planning the adjuvant treatment of ACC. Mitotane treatment may be independently associated with better outcomes regardless of the tumor stage.

Contents Vol. 101, 2015

I.J. Clarke, Clayton, Vic. C. Coen, London M.A. Cowley, Clayton, Vic. W.W. de Herder, Rotterdam S.L. Dickson, Gothenburg A. Enjalbert, Marseille A.B. Grossman, Headington D. Jezová, Bratislava A.S. Kauff man, La Jolla, Calif. M.J. Kelly, Portland, Oreg. P.A. Kelly, Paris X. Ni, Shanghai S.R. Ojeda, Beaverton, Oreg. (Reviews) G. Rindi, Rome H. Sasano, Sendai J.Y. Seong, Seoul M. Tena-Sempere, Cordoba H. Tsukamura, Nagoya E.J. Wagner, Pomona, Calif. B. Wiedenmann, Berlin Z. Zeng, Beijing Editorial Board

Adrenal-preserving surgery of adrenal tumours

Currently, laparoscopic adrenalectomy is seen as more than just the preferred method, in fact as a routine procedure, always bearing in mind, however, the developed restraints on its usage. The size of the tumour, recurrent disease, and the existing invasive process all remain factors which determine the indications for operation. The situation is similar in cases of qualifying for sparing (non-radical) operations on adrenal glands. The basic challenge in terms of qualification, choice of technique and the range of operation of adrenal lesions remains: obtaining a reliable preoperative diagnosis, the localisation of lesions including their ectopic location, the evaluation of the imaging phenotype, as well as a proper pre-surgical preparation. Maintaining one third of properly vascularised adrenal mass allows one to avoid a substitutive therapy, with the possible necessity of its application in stressful situations. The first partial adrenalectomy has been conducted on a patient with a bilateral pheochromocytoma, in order to retain the glucocorticoid adrenal functions. The recommendations for its administration remain: hereditary pheochromocytoma of at least 2cm diameter, unilateral adenoma of at least 5cm diameter in Cushings syndrome without any concomitant lesions in the oposite adrenal gland, and unilateral adenoma in Conns syndrome. It seems that non-radical resection should always be considered for patients at risk of bilateral adrenalectomy in cases of concomitant lesions in both adrenals.