Case Report: Ectopic Adrenocortical Carcinoma in the Ovary

Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence of 0.7–2.0 cases/million habitants/year. ACCs are rare and usually endocrinologically functional. We present the case of a 59-year-old woman who experienced abdominal fullness for 6 months and increased abdominal circumference. A large pelvic tumor was observed. She underwent cytoreductive surgery and the pathological test results revealed local tumor necrosis and prominent lympho-vascular invasion. Neuroendocrine carcinoma was the first impression, but positivity for synaptophysin, alpha-inhibin, transcription factor enhancer 3 (TFE-3), calretinin (focal), and CD56 (focal) and high Ki-67-labeling proliferating index (>80%) confirmed the diagnosis of ectopic ACC. Ectopic primary aldosteronism could not be excluded. However, we did not perform saline infusion test or captopril test due to poor performance status. When pathological test reports reveal neuroendocrine features not typically found in the organ being examined, IHC staining should be performed to rule out ectopic ACC. Whether the ectopic ACC is functional or not requires complete survey.

Primärer Hyperaldosteronismus – warum diagnostizieren wir immer noch so wenige Patienten?

Was ist neu? Definition, Klassifizierung und Prävalenz Der primäre Hyperaldosteronismus (PA) ist die häufigste chirurgisch therapierbare Form der sekundären Hypertonie. Hauptursachen des PA sind ein 1-seitiges Aldosteron-produzierendes Nebennierenadenom oder eine bilaterale idiopathische Nebennierenrindenhyperplasie (Bilateral Adrenal Hyperplasia, BAH). In den letzten Jahren ist das Phänomen der autonomen Kortisol-Co-Sekretion (ACS) im Rahmen des Conn-Syndroms in den Fokus gerückt. Während der Mineralokortikoidexzess für die Hypertonie verantwortlich ist, scheint die ACS vor allem zu metabolischen Komorbiditäten wie erhöhtem BMI, gestörter Glukosetoleranz und Diabetes mellitus Typ 2 zu führen. Das sogenannte „Connshing“-Syndrom (zusammengezogen von Conn- und Cushing-Syndrom) wird als neuer metabolischer Subtyp angesehen. Diagnose Folgende Hypertoniegruppen sollen unter anderem gescreent werden (etwa 50 % aller Hypertoniker): Patienten mit therapierefraktärer Hypertonie, mit arterieller Hypertonie und Hypokaliämien, mit arterieller Hypertonie und obstruktivem Schlafapnoe-Syndrom (OSAS), mit arterieller Hypertonie und adrenalem Nebennierenzufallstumor, mit Hypertonie und Blutdruck > 150/90 mmHg sowie jüngere Patienten mit Hypertonie. Das Screening erfolgt mittels des Aldosteron-Renin-Quotienten. Der nächste Schritt ist häufig die Bestätigung der Diagnose PA mittels Kochsalzbelastungs- und/oder Captopril-Test. Neu ist, dass bei sehr ausgeprägtem biochemischem Phänotyp, z. B. bei Patienten mit spontaner Hypokaliämie sowie maximal supprimierten Renin- und Aldosteron-Werten > 550 pmol/l, ohne weiteren Bestätigungstest direkt eine Subtypdifferenzierung erfolgen kann. Die Subtypdifferenzierung erfolgt mittels CT-Bildgebung der Nebennieren und (als Goldstandard) Nebennierenvenenkatheterisierung. Bei technisch nicht erfolgreichen Katheterisierungen werden nuklearmedizinische Verfahren (z. B. CXCR4-PET/CT) in Einzelfällen eingesetzt. Therapie Bei gesichertem unilateralem PA ist eine Adrenalektomie indiziert. Entsprechend der Primary-Aldosteronism-Surgical-Outcome-Study-Kriterien profitieren Frauen, jüngere Patienten (< 50 Jahre), Patienten mit geringerer Laufzeit der Hypertonie (< 5 Jahre) und Patienten mit geringerer Anzahl (< 2) von Antihypertensiva am meisten von einer Adrenalektomie. Im Fall einer BAH sollte eine Therapie mittels Spironolacton (initial 25–50 mg/d) eingeleitet werden, mit entsprechender Steigerungsoption zum Anheben des Renin als Ziel.

Plasma Aldosterone After Seated Saline Infusion Test Outperforms Captopril Test at Predicting Clinical Outcomes After Adrenalectomy for Primary Aldosteronism

OBJECTIVE The saline infusion test (SIT) and the captopril test (CT) are widely used as confirmatory tests for primary aldosteronism (PA). We hypothesized that post-SIT and post-CT plasma aldosterone concentrations (PAC) indicate the severity of aldosterone-producing adenoma (APA) and might predict clinical outcome. METHODS We recruited 216 patients with APA in the Taiwan Primary Aldosteronism Investigation (TAIPAI) registry who received both seated SIT and CT as confirmatory tests. The data of 143 patients who underwent adrenalectomy with complete follow-up after diagnosis were included in the final analysis. We determined the proportion of patients achieving clinical success in accordance with the Primary Aldosteronism Surgical Outcome consensus. Logistic regression analysis was conducted to identify preoperative factors associated with cure of hypertension. RESULTS Complete clinical success was achieved in 48 (33.6%) patients and partial clinical success in 59 (41.2%) patients; absent clinical success was seen in 36 (25.2%) of 143 patients. Post-SIT PAC but not post-CT PAC was independently associated with clinical outcome. Higher levels of post-SIT PAC had a higher likelihood of clinical benefit (complete plus partial clinical success; odds ratio = 1.04 per ng/dl increase, 95% confidence interval = 1.01, 1.06; P = 0.004). Patients with post-SIT PAC &gt; 25 ng/dl were more likely to have a favorable clinical outcome after adrenalectomy. This cutoff value translated into a positive predictive value of 86.0%. CONCLUSIONS We suggest that post-SIT PAC is a better predictor than post-CT PAC for clinical success in PA post adrenalectomy.

Prevalence of primary aldosteronism among bulgarian hypertensive patients

Primary aldosteronism (PA) has long been considered a rare disease, but a higher prevalence was suggested recently. The aim of this study was to evaluate the prevalence of PA in a group of Bulgarian hypertensive patients, including patients with adrenal incidentalomas (AI). The aldosterone to renin ratio (ARR)>750 was used as a positive screening test and the Captopril test was performed to confirm the diagnosis. Adrenal CT scan was used to differentiate between the main subtypes of PA- aldosterone-producing adenoma (APA) and idiopathic hyperaldosteronism (IHA). The diagnosis of APA was retrospectively confirmed after surgery. After excluding other forms of endocrine hypertension, except PA, we investigated a total of 472 consecutive hypertensive patients, among them 96 patients with AI. Final diagnosis of PA was reached in 38 patients (8.05%) in the entire hypertensive population and in 12 patients (12.5%) among hypertensive patients with AI. In the group of PA, 15 patients (39.5%) were diagnosed with APA and 23 patients (60.5%) had an IHA. Among all patients with PA 21 (55.3 %) presented with hypokalemia. Our findings of a relatively high prevalence of PA support an early diagnosis of this potentially curable disease, especially in hypertensive patients with AI.