Sema Ciftci Dogansen

Pituitary stalk interruption syndrome is usually diagnosed during childhood, and data on outcomes in adulthood are limited. This retrospective, single-center study evaluated the diagnostic history, endocrine phenotype, and clinical outcomes of 14 adults with radiologically confirmed pituitary stalk interruption syndrome. The cohort comprised eight men and six women, with a mean age of 28.8±6.4 years and a mean documented follow-up at our center of 5.6±3.2 years. Hypopituitarism was diagnosed during childhood in 12 patients and during adulthood in 2 patients. Thirteen patients had deficiencies of all four anterior pituitary hormone axes, whereas one patient had growth hormone and gonadotropic deficiencies with preserved corticotropic and thyrotropic functions. In six patients diagnosed during childhood, pituitary stalk interruption syndrome was recognized only after later reassessment of pituitary imaging. Eleven patients received growth hormone treatment during childhood, and 13 of 14 patients reached their calculated target height. Metabolic dysfunction–associated steatotic liver disease and dyslipidemia were each present in 10 patients. Four patients had bone mineral density below the expected range for age. One female patient achieved a live birth following in vitro fertilization. In this real-world cohort, the skeletal and metabolic findings observed despite generally favorable growth outcomes support the need for lifelong endocrine follow-up.

Background/Objectives: Mild autonomous cortisol secretion (MACS) is associated with increased cardiometabolic morbidity, but its relationship with the retinal microcirculation is unknown. We compared retinal and peripapillary microvascular parameters between MACS and nonfunctioning adrenal adenoma (NFA) and, secondarily, examined their associations with continuously modeled post-dexamethasone cortisol. Methods: In this single-center, retrospective, cross-sectional study, 98 patients (45 NFA, 53 MACS) were included, and optical coherence tomography angiography (OCTA) and structural OCT data from one eye per patient were analyzed. The primary outcome was whole-image deep capillary plexus (DCP) vessel density. Multivariable models were adjusted for age, sex, body mass index, and cardiometabolic comorbidities; secondary analyses were corrected using the Benjamini–Hochberg false discovery rate (q values). Results: OCTA was evaluable in 89 patients. The primary outcome did not differ significantly between groups in the main analysis (NFA vs. MACS, 48.64 ± 5.71% vs. 47.31 ± 5.77%; adjusted difference −1.29 percentage points, 95% CI −3.97 to 1.38; p = 0.339) or in any sensitivity analysis. In secondary analyses, superior retinal nerve fiber layer (RNFL) thickness was greater in MACS (β = 17.38 µm; q < 0.001) and increased with ln(post-dexamethasone cortisol) (β = 13.74 µm; q = 0.010). Higher ln(post-dexamethasone cortisol) was also associated with lower parafoveal DCP (β = −2.72 percentage points; p = 0.013) and inside-disc radial peripapillary capillary vessel density (β = −3.13 percentage points; p = 0.026), which did not survive multiplicity correction; no superficial capillary plexus sector was associated with either exposure (all p ≥ 0.37). Conclusions: MACS was not significantly associated with whole-image DCP vessel density. The secondary associations with continuous cortisol are cross-sectional and hypothesis-generating and require prospective confirmation.

The potential association between mild autonomous cortisol secretion and psychopathological symptoms remains inadequately studied. This study aimed to investigate whether women with mild autonomous cortisol secretion exhibit greater psychological symptom severity and distress compared with patients with nonfunctional adrenal adenomas, as assessed by multidimensional self-report scales including the Beck Depression Inventory-II, Beck Anxiety Inventory, and Symptom Checklist‑90. A cross-sectional study was conducted at the endocrinology department of a tertiary-care hospital in Turkey between June 2022 and 2025. The study cohort included 91 women with adrenal adenomas, comprising 50 patients with mild autonomous cortisol secretion and 41 patients with nonfunctional adrenal adenoma. Questionnaire-derived scores from the 10 subscales and Global Severity Index of the Symptom Checklist-90, as well as the Beck Anxiety Inventory and Beck Depression Inventory-II, along with hormonal parameters, including morning cortisol, adrenocorticotropic hormone, dehydroepiandrosterone sulfate, 1-mg dexamethasone-suppressed cortisol, 24-hour urinary free cortisol, the dehydroepiandrosterone sulfate ratio, and the cortisol/dehydroepiandrosterone sulfate ratio, were compared between the groups. Women with mild autonomous cortisol secretion exhibited higher overall psychological distress, as measured by the Global Severity Index of the Symptom Checklist-90. Depressive (Beck Depression Inventory-II) and anxiety (Beck Anxiety Inventory) symptoms did not differ significantly between groups, although a non-significant pattern suggested that depressive symptom severity may increase with higher basal cortisol levels. No significant associations were found between hormonal parameters and psychological scores. These findings indicate that patients with mild autonomous cortisol secretion may experience increased general psychological distress, highlighting the importance of considering mental health in their clinical evaluation and warranting further research into the relationship between mild cortisol excess and specific psychiatric symptoms.

Adrenalectomy and conservative management are therapeutic approaches for mild autonomous cortisol secretion; however, their comparative clinical impact in routine practice remains uncertain. We aimed to evaluate real-world hormonal, clinical, and metabolic outcomes according to the treatment strategy in patients with mild autonomous cortisol secretion. This single-center retrospective observational study included consecutive patients with adrenal incidentaloma fulfilling guideline-based diagnostic criteria for mild autonomous cortisol secretion between January 2015 and December 2024. Sixty-five patients with complete hormonal evaluation and follow-up data were analyzed and classified into surgery (n=23) and conservative (n=42) groups. Demographic characteristics, adenoma features, comorbidities, hormonal parameters, and metabolic outcomes were assessed at baseline and at the final follow-up. The median follow-up duration was approximately 3 years and similar between groups (p>0.05). At baseline, the body mass index, adenoma size, and cortisol levels after the 1-mg dexamethasone suppression test were significantly higher, while adrenocorticotropic hormone levels were lower in the surgery group (p=0.02, p=0.02, p=0.036, and p<0.01, respectively). During the follow-up, adrenocorticotropic hormone levels increased and post-dexamethasone suppression test cortisol levels significantly decreased after adrenalectomy (p=0.001 and p=0.036, respectively), whereas metabolic parameters and comorbidity profiles remained largely unchanged. In the conservative group, total cholesterol increased modestly over time (p=0.048), with no significant changes in other clinical outcomes. No significant difference in comorbidity progression was observed between treatment strategies. In this real-world cohort, adrenalectomy resulted in clear hormonal improvement without parallel short-term metabolic or clinical benefits compared with conservative management. These findings highlight the heterogeneous clinical expression of mild autonomous cortisol secretion and underscore the importance of individualized patient selection for surgery.

Objective: The utility of the 1-mg overnight dexamethasone suppression test (DST) for assessing dehydroepiandrosterone sulfate (DHEA-S) suppressibility is not well defined. Hence, this study aimed to assess DHEA-S response and identify predictors of non-suppression in hirsute women with elevated DHEA-S levels. Subjects and methods: This retrospective study included 362 hirsute women with elevated DHEA-S. Suppression was defined as a post-DST DHEA-S level normalized to the age-specific upper limit of normal (ULN) or a decrease of 50% from baseline. Patients were classified as suppressed (Group 1) or non-suppressed (Group 2). Logistic regression and receiver operating characteristic curve analysis were used to identify predictors of suppression, including the “percent excess” (% excess) above the ULN. Results: DHEA-S was suppressed in 65.5% of patients (Group 1), while 34.5% was not (Group 2). The non-suppressed group had significantly higher levels of total testosterone, a greater frequency of total testosterone >ULN, and higher 17-hydroxyprogesterone (17-OHP) levels. No differences were observed in luteinizing hormone, follicle-stimulating hormone, or homeostatic model assessment of insulin resistance. Logistic regression confirmed that both % excess (odds ratio [OR] = 0.947) and DHEA-S levels >700 μg/dL (OR = 0.069) were inversely associated with suppression. Receiver operating characteristic analysis for % excess showed good predictive performance (area under the curve = 0.827), with an optimal cut-off of 32.22%. Conclusion: Hirsute women with elevated DHEA-S, 34.5% exhibited a lack of DHEA-S suppression on the 1-mg DST. The % excess (cut-off >32.22%) and an absolute DHEA-S threshold of >700 μg/dL may help identify this subgroup, which was associated with higher total testosterone and 17-OHP levels despite similar luteinizing hormone levels. Overall, the lack of DHEA-S suppression on the 1-mg DST, together with higher total testosterone and 17-OHP despite similar luteinizing hormone, suggests a possible functional adrenal contribution to the hyperandrogenic phenotype.

Purpose Bilateral inferior petrosal sinus sampling (BIPSS) is the gold standard for localizing ACTH-dependent Cushing’s syndrome (CS). While corticotropin-releasing hormone (CRH) was initially used for stimulation, desmopressin has become a common alternative. This research evaluates desmopressin’s effectiveness in lateralizing Cushing’s disease (CD) during BIPSS compared to CRH stimulation. Methods The study included 33 individuals with ACTH-dependent CS who underwent BIPSS and had diagnoses confirmed by endoscopic endonasal transsphenoidal pituitary surgery (EETPS). Fourteen participants underwent BIPSS with CRH and 19 with desmopressin. A comparative analysis was conducted. Results BIPSS accurately lateralized 76% of cases, specifically, 71% with CRH and 79% with desmopressin (p = 0.2). For tumors < 6 mm on MRI, overall accuracy was 82%, namely, 75% with CRH and 90% with desmopressin (p = 0.4). IPSS achieved 100% accuracy in the four cases with no lesion on preoperative MRI. Conclusion This study demonstrates no significant difference in lateralization accuracy between desmopressin and CRH for IPSS. In challenging cases, especially those with microadenomas or non-lesional CD, desmopressin with IPSS aids in preoperative lateralization.

Objective To compare clinical and hormonal data, neuroendocrine neoplasia (NEN) localization, treatment, and survival outcomes in ectopic Cushing’s syndrome (ECS) by sex. Methods Eleven experienced centers from our country participated in this retrospective study. The clinical and hormonal features, tumor imaging, pathological results, treatment modalities, and disease courses of the patients were evaluated. Results 28 female and 26 male patients with ECS were compared. The mean age at diagnosis, clinical characteristics, and hormonal evaluation results were similar. However, insulin-requiring diabetes mellitus (p = 0.04) and osteoporosis with fractures were more common in males (p = 0.03). While more patients with increased DHEA-S levels than the upper limit of normal were found to be higher in females, central hypothyroidism were higher in males (p = 0.02). At the diagnosis, 36 NENs (68% of females and 69% of males) were localized. Small cell lung carcinoma was higher in males (p = 0.02), and the frequency of other NENs was not different. Curative surgery was performed on 61% of females and 46% of males. Tumor size, Ki-67 labeling index, positive ACTH immunostaining, local lymph node and distant metastasis rates were similar in both sexes. In the follow-up, the tumor became visible in 7 of 10 females and 4 of 8 males after medical treatment and/or bilateral adrenalectomy. The remission rates (65% of females, 62% of males) and NEN-related death rates (14% of females, 30% of males) were similar. Conclusion While ECS has a similar disease course in many aspects in males and females, hyperglycemia and osteoporosis are more severe in males.

Objective: Primary hypophysitis might be challenging to diagnose, and there is a lack of evidence regarding optimal treatment strategies due to rarity of the disease. We aim to investigate the clinical features and compare the outcomes of different management strategies of primary hypophysitis in a large group of patients recruited on a nationwide basis. Design: A retrospective observational study. Methods: The demographic, clinical, and radiologic features and follow-up data were collected in study protocol templates and analyzed. Results: One hundred and thirteen patients (78.8% female, median age: 36 years) were included. Lymphocytic (46.7%) and granulomatous hypophysitis (35.6%) were the prevailing subtypes out of 45 patients diagnosed after pathologic investigations. Headache (75.8%) was the most common symptom, and central hypogonadism (49.5%) was the most common hormone insufficiency. Of the patients, 52.2% were clinically observed without interventions, 18.6% were started on glucocorticoid therapy, and 29.2% underwent surgery at presentation. Headache, suprasellar extension, and chiasmal compression were more common among glucocorticoid-treated patients than who were observed. Cox regression analysis revealed higher hormonal and radiologic improvement rates in the glucocorticoid-treated group than observation group (hazard ratio, 4.60; 95% CI, 1.62-12.84 and HR, 3.1; 95% CI, 1.40-6.68, respectively). The main indication for surgery was the inability to exclude a pituitary adenoma in the presence of compression symptoms, with a recurrence rate of 9%. Conclusion: The rate of spontaneous improvement might justify observation in mild cases. Glucocorticoids proved superior to observation in terms of hormonal and radiologic improvements. Surgery may not be curative and might be considered in indeterminate, treatment-resistant, or severe cases.

Objective : Postoperative data on Cushing’s disease (CD) are equivocal in the literature. These discrepancies may be attributed to different series with different criteria for remission and variable follow-up durations. Additional data from experienced centers may address these discrepancies. In this study, we present the results obtained from 96 endoscopic transsphenoidal surgeries (ETSSs) for CD conducted in a well-experienced center.Methods : Pre- and postoperative data of 96 ETSS in 87 patients with CD were included. All cases were handled by the same neurosurgical team between 2014 and 2022. We obtained data on remission status 3−6 months postoperatively (medium-term) and during the latest follow-up (long-term). Additionally, magnetic resonance imaging (MRI) and pathology results were obtained for each case.Results : The mean follow-up duration was 39.5±3.2 months. Medium and long-term remission rates were 77% and 82%, respectively. When only first-time operations were considered, the medium- and long-term remission rates were 78% and 82%, respectively. The recurrence rate in this series was 2.5%. Patients who showed remission between 3−6 months had higher longterm remission rates than did those without initial remission. Tumors >2 cm and extended tumor invasion of the cavernous sinus (Knosp 4) were associated with lower postoperative remission rates.Conclusion : Adenoma size and the presence/absence of cavernous sinus invasion on preopera-tive MRI may predict long-term postoperative remission. A tumor size of 2 cm may be a supporting criterion for predicting remission in Knosp 4 tumors. Further studies with larger patient populations are necessary to support this finding.