Nonfunctioning Adrenal Tumors Research Articles

Are comorbidities of patients with adrenal incidentaloma tied to sex?

A recent cross-sectional study showed that both comorbidities and mortality in patients with adrenal incidentaloma (AI) are tied to sex. However, few longitudinal studies evaluated the development of arterial hypertension, hyperglycemia, dyslipidemia and bone impairment in patients with AI. The aim of this study is to analyze the impact of sex in the development of these comorbidities during long-term follow-up. We retrospectively evaluated 189 patients (120 females, 69 males) with AI, from four referral centers in Italy and Croatia. Clinical characteristics, comorbidities and cortisol after 1-mg dexamethasone suppression test (1-mg DST) were assessed at baseline and at last follow-up visit (LFUV). Median follow-up was 52 (Interquartile Range 25-86) months. The rates of arterial hypertension and hyperglycemia increased over time both in females (65.8% at baseline versus 77.8% at LFUV, p=0.002; 23.7% at baseline versus 39.6% at LFUV, p<0.001; respectively) and males (58.0% at baseline versus 69.1% at LFUV, p=0.035; 33.8% at baseline versus 54.0% at LFUV, p<0.001; respectively). Patients were stratified in two groups using 1.8 µg/dl as cut-off of cortisol following 1-mg DST: non-functional adrenal tumors (NFAT) and tumors with mild autonomous cortisol secretion (MACS). In the NFAT group (99 patients, females 62.6%), at baseline, we did not observe any difference in clinical characteristics and comorbidities between males and females. At LFUV, males showed a higher frequency of hyperglycemia than females (57.6% versus 33.9%, p=0.03). In the MACS group (89 patients, females 64.0%), at baseline, the prevalence of hypertension, hyperglycemia and dyslipidemia was similar between sexes, despite females were younger (60, IQR 55-69 versus 67.5, IQR 61-73, years; p=0.01). Moreover, females presented higher rates of bone impairment (89.3% versus 54.5%, p=0.02) than males. At LFUV, a similar sex-related pattern was observed. Patients with AI frequently develop arterial hypertension and hyperglycemia and should be periodically checked for these comorbidities, regardless of sex. In patients with MACS, the lack of difference between sexes in the frequency of cardiometabolic comorbidities despite that females are younger, and the higher frequency of bone impairment in females, suggest a sex-specific effect of cortisol.

The incidence of cancers in patients with non-functional adrenal tumors: a swedish population-based national cohort study

The incidence of cancers in patients with non-functional adrenal tumors: a swedish population-based national cohort study

Mild autonomous cortisol secretion: pathophysiology, comorbidities and management approaches.

The majority of incidentally discovered adrenal tumours are benign adrenocortical adenomas and the prevalence of adrenocortical adenomas is around 1-7% on cross-sectional abdominal imaging. These can be non-functioning adrenal tumours or they can be associated with autonomous cortisol secretion on a spectrum that ranges from rare clinically overt adrenal Cushing syndrome to the much more prevalent mild autonomous cortisol secretion (MACS) without signs of Cushing syndrome. MACS is diagnosed (based on an abnormal overnight dexamethasone suppression test) in 20-50% of patients with adrenal adenomas. MACS is associated with cardiovascular morbidity, frailty, fragility fractures, decreased quality of life and increased mortality. Management of MACS should be individualized based on patient characteristics and includes adrenalectomy or conservative follow-up with treatment of associated comorbidities. Identifying patients with MACS who are most likely to benefit from adrenalectomy is challenging, as adrenalectomy results in improvement of cardiovascular morbidity in some, but not all, patients with MACS. Of note, diagnosis and management of patients with bilateral MACS is especially challenging. Current gaps in MACS clinical practice include a lack of specific biomarkers diagnostic of MACS-related health outcomes and a paucity of clinical trials demonstrating the efficacy of adrenalectomy on comorbidities associated with MACS. In addition, little evidence exists to demonstrate the efficacy and safety of long-term medical therapy in patients with MACS.

Prevalence and Incidence of Fractures in Patients With Nonfunctional Adrenal Tumors

It is unclear whether nonfunctional adrenal tumors (NFATs) are associated with fractures. To analyze fracture frequencies in individuals with NFATs. A national retrospective cohort study was conducted in patients with NFATs diagnosed in Sweden between January 1, 2005, and December 31, 2019, and control participants without adrenal tumors followed up until death or the end of 2019. Individuals with a diagnosis of adrenal hormonal excess or previous malignant tumors were excluded. Sensitivity analyses were performed in subgroups of individuals with a combination of gallbladder, biliary tract, and pancreas diseases (for whom it was assumed that controls would also have undergone computed tomography) and 3- and 12-month survival free of malignant tumors after the NFAT diagnosis. The data were analyzed from September to November 2023. Diagnosis of NFATs. Main study outcomes were prevalence and incidence of fractures after adjustment for sex, age, and comorbidities. Secondary outcomes were fragility fractures, fractures with fall on the same level, and fracture locations (distal arm and vertebral and hip fractures). Fracture incidence after adrenalectomy was also studied. Among 20 390 patients, 12 120 (59.4%) were women, and the median (IQR) age was 66 (57-73) years; among 125 392 controls, 69 994 (55.8%) were women, and the median (IQR) age was 66 (57-73) years. Previous fractures were more common in patients diagnosed with NFATs compared with controls (4310 of 20 390 [21.1%] vs 20 323 of 125 392 [16.2%]; odds ratio [OR], 1.39; 95% CI, 1.34-1.45; adjusted OR [AOR], 1.27; 95% CI, 1.23-1.33). During the follow-up period (median [IQR], 4.9 [2.2-8.2] years), incident fractures were more common in patients with NFATs (3127 of 20 390 [15.3%] vs 16 086 of 125 392 [12.8%]; hazard ratio [HR], 1.40; 95% CI, 1.34-1.45; adjusted HR [AHR], 1.27; 95% CI, 1.22-1.33). An association between NFATs and vertebral fractures was found (AOR, 1.51; 95% CI, 1.33-1.72; AHR, 1.83; 95% CI, 1.60-2.09). In men younger than 50 years, NFATs were associated with fractures (AOR, 1.45; 95% CI, 1.21-1.74; AHR, 1.48; 95% CI, 1.20-1.82). There was no association among individuals who had undergone adrenalectomy (AHR, 1.12; 95% CI, 0.90-1.38). The association between NFATs and fractures remained significant and of similar magnitude in all sensitivity analyses. In this cohort study, NFATs were associated with fractures, particularly among younger men; thus, patients with NFATs should have bone health evaluation with appropriate treatment and monitoring, especially in younger men.

Challenges in the diagnosis of the enigmatic primary adrenal leiomyosarcoma: two case reports and review of the literature

BackgroundPrimary adrenal leiomyosarcoma is a rare and aggressive mesenchymal tumor derived from the smooth muscle wall of a central adrenal vein or its tributaries; therefore, tumors tend to invade the inferior vena cava and cause thrombosis. The great majority of tumors grow rapidly, which makes the disease difficult to diagnose in its early clinical stages and needs differentiation from adrenocortical carcinomas for the selection of chemotherapy including mitotane which causes adrenal insufficiency.Case presentationWe presented two patients with adrenal leiomyosarcoma who were referred to our hospital with abdominal pain and harboring large adrenal tumors and inferior vena cava thrombosis. The endocrine findings, including serum catecholamine levels, were unremarkable. These two patients were considered clinically inoperable, and CT-guided core needle biopsy was performed to obtain the definitive histopathological diagnosis and determine the modes of therapy. The masses were subsequently diagnosed as primary adrenal leiomyosarcoma based on the histological features and positive immunoreactivity for SMA (smooth muscle actin), desmin, and vimentin.ConclusionsAdrenal leiomyosarcoma derived from the smooth muscle wall of a central adrenal vein or its tributaries is rare but should be considered a differential diagnosis in the case of nonfunctioning adrenal tumors extending directly to the inferior vena cava. CT-guided biopsy is considered useful for histopathological diagnosis and clinical management of patients with inoperable advanced adrenal tumors without any hormone excess.

THU418 Steroid Profiles And Its Implication In Bone Mass And Quality In Premenopausal Women With Adrenal Tumors With Autonomous Cortisol Secretion

Abstract Disclosure: H. Nakao: None. K. Nakatani: None. M. Yokomoto-Umakoshi: None. H. Umakoshi: None. H. Kaneko: None. N. Iwahashi: None. M. Ogata: None. T. Fukumoto: None. F. Masamichi: None. O. Tatsuki: None. Y. Matsuda: None. R. Sakamoto: None. Y. Izumi: None. T. Bamba: None. Y. Ogawa: None. Context: Osteoporosis is a major complication in adrenal tumors with autonomous cortisol secretion (ACS). We conducted steroid profiling to elucidate how steroid metabolites affect bone mass and quality in patient with ACS. Methods: Ninety patients with ACS and 91 with non-functioning adrenal tumors (NFAT) were included, of whom 73 ACS (18 pre-, 26 postmenopausal women, and 29 men) and 85 NFAT (12 pre-, 34 postmenopausal women, and 39 men) were evaluated for 21 plasma steroid metabolites using liquid chromatography-tandem mass spectrometry. Results: Premenopausal women with ACS had lower lumbar spine (LS)-bone mineral density (BMD) and trabecular bone score (TBS) than those with NFAT. The discriminant and principal component analyses clearly separated ACS from NFAT in premenopausal women, but not in men and postmenopausal women. In premenopausal women, 8 steroid metabolites differed between ACS and NFAT, with 11-deoxycorticosterone having the largest effect size (Cohen’s d=1.600, false discovery rate (FDR)=1e-04). In premenopausal women with ACS, 11-deoxycorticosterone showed a negative correlation with LS-BMD Z-score (r=-0.603, FDR=0.064), and DHEA-S and androsterone-glucuronide showed a positive correlation with TBS (DHEA-S, r=0.647, FDR=0.019; androsterone-glucuronide r=0.681, FDR=0.020). The correlation of 11-deoxycorticosterone with LS-BMD Z-score and that of androsterone-glucuronide with TBS remained significant after adjustment for confounders. In men and postmenopausal women with ACS, no steroid metabolites evaluated were significantly correlated with BMD and TBS. Conclusion: This study provides the evidence for distinct steroid profiles in premenopausal women with ACS, suggesting that elevated 11-deoxycorticosterone, a mineralocorticoid metabolite, induces bone loss, and decreased androsterone-glucuronide, an androgen metabolite, deteriorates bone quality. Presentation: Thursday, June 15, 2023

FRI182 Cardiometabolic Risk And Outcomes In Patient With Adrenal Adenomas

Abstract Disclosure: H. Lam: None. H. Han: None. S. Athimulam: None. Background: Approximately 5% of adults undergoing cross-sectional imaging are reported to have an adrenal adenoma. Although most adenomas are considered non-functioning (NFAT), up to 48% can present with mild autonomous cortisol secretion (MACS). Published studies have reported an increased prevalence of cardiometabolic risk factors, cardiac and cerebrovascular events in MACS patients. Objective: To determine the prevalence of cardiometabolic disease and outcomes in a population-based cohort of patients with adrenal adenomas. Design: Retrospective cross-sectional study of patients with adrenal adenomas over a 10-year period (Jan 2012 – Dec 2021). Setting: Academic primary and tertiary care setting. Main outcomes measured: Prevalence of cardiometabolic disease, cardiovascular events requiring procedure (percutaneous coronary intervention, PCI or coronary artery bypass graft, CABG) and cerebrovascular events. MACS was diagnosed based on cortisol level ≥ 1.8cmg/dL after overnight 1mg dexamethasone suppression testing (DST) and NFAT if cortisol &amp;lt; 1.8mcg/dL. Patients who did not undergo 1mg DST were categorized as unknown cortisol status (UCS). Results: Preliminary results include 198 patients with adrenal adenomas [(135 women (67%) with a median age of 67y, 106 Black (54%), 83 White (42%) and 9 other races (4%)]. 74 (37%) were diagnosed with NFAT, 42 (21%) with MACS, and 82 (41%) with UCS. Hypertension (n=163,83%; MACS vs NFAT vs UCS: 23% vs 34% vs 43%) is the most prevalent cardiometabolic risk factor, followed by hyperlipidemia (n=146, 75%; MACS vs NFAT vs UCS: 21% vs 38% vs 41%), prediabetes (n=94, 50%; MACS vs NFAT vs UCS: 18% vs 43% vs 39%) and type 2 diabetes mellitus (n=82,43%; MACS vs NFAT vs UCS: 22% vs 35% vs 43%), respectively. 14% of patients with adrenal adenomas underwent procedures (PCI or CABG) for cardiovascular events (MACS vs NFAT vs UCS: 33% vs 26% vs 41%) and 11% were diagnosed with cerebrovascular events (MACS vs NFAT vs UCS: 29% vs 19% vs 52%). Conclusions: Patients with adrenal adenomas and mild autonomous cortisol secretion (MACS) are at increased risk of cardiovascular events requiring intervention and cerebrovascular events, compared to non-functioning adrenal tumors. 41% (n=82, 61% Blacks, 30% White, 2% Others) of patient population was classified as unknown cortisol status indicating further need to educate health care professionals on evaluation of adrenal adenomas to improve health care outcomes and disparities in management. Presentation: Friday, June 16, 2023

Adrenal steroid metabolites and bone status in patients with adrenal incidentalomas and hypercortisolism

Autonomous cortisol secretion (ACS), resulting from cortisol-producing adenomas (CPA), causes endogenous steroid-induced osteoporosis (SIOP). However, the risk of endogenous SIOP cannot be explained by cortisol excess alone, and how other steroid metabolites affect bone status is unclear. ACS was diagnosed as serum cortisol ≥1.8μg/dL after the 1-mg dexamethasone suppression test (DST-cortisol). Using liquid chromatography tandem mass spectrometry, 21 plasma steroid metabolites were measured in 73 patients with ACS and 85 patients with non-functioning adrenal tumors (NFAT). Expression of steroidogenic enzymes and relevant steroid metabolites were analyzed in some of CPA tissues. Discriminant and principal component analyses distinguished steroid profiles between the ACS and NFAT groups in premenopausal women. Premenopausal women with ACS exhibited higher levels of a mineralocorticoid metabolite, 11-deoxycorticosterone (11-DOC), and lower levels of androgen metabolites, dehydroepiandrosterone-sulfate, and androsterone-glucuronide. In premenopausal women with ACS, DST-cortisol negatively correlated with trabecular bone score (TBS). Additionally, 11-DOC negatively correlated with lumbar spine-bone mineral density, whereas androsterone-glucuronide positively correlated with TBS. The CPA tissues showed increased 11-DOC levels with increased expression of CYP21A2, essential for 11-DOC synthesis. Adrenal non-tumor tissues were atrophied with reduced expression of CYB5A, required for androgen synthesis. This study demonstrates that unbalanced production of adrenal steroid metabolites, derived from both adrenal tumor and non-tumor tissues, contributes to the pathogenesis of endogenous SIOP in premenopausal women with ACS. JSPS KAKENHI, Secom Science and Technology Foundation, Takeda Science Foundation, Japan Foundation for Applied Enzymology, AMED-CREST, JSTA-STEP, JST-Moonshot, and Ono Medical Research Foundation.

Mortality in Patients With Nonfunctional Adrenal Tumors

It is unclear if nonfunctional adrenal adenomas (NFAAs) are associated with increased mortality. To analyze mortality and causes of death in patients with NFAA. A national retrospective register-based case-control study was conducted and included 17 726 patients with a diagnosis of adrenal adenoma in Sweden from 2005 to 2019 who were identified and followed up until death or 2020 as well as 124 366 controls without adrenal adenoma. Individuals with diagnoses indicating adrenal hormonal excess or cancer were excluded. Follow-up started after 3 months of cancer-free survival following the date of the NFAA diagnosis. Sensitivity analyses were performed in subgroups of individuals for whom it was assumed that controls would also have undergone computed tomography: those with acute appendicitis (for whom it was assumed that there was no concern of cancer) and in patients with a combination of gallbladder, biliary tract, and pancreas disorders and 6-month and 12-month cancer-free survival following the date of the NFAA diagnosis. The data were analyzed in 2022. Diagnosis of NFAA. The primary outcome was all-cause mortality among patients with NFAA after adjustment for comorbidities and socioeconomic factors. Secondary outcomes were mortality due to cardiovascular diseases and cancer. Among 17 726 cases, 10 777 (60.8%) were women, and the median (IQR) age was 65 (57-73) years; among 124 366 controls, 69 514 (55.9%) were women, and the median (IQR) age was 66 (58-73) years. Among cases, overall mortality during the follow-up period (median, 6.2 years [IQR, 3.3-9.6 years]) was higher compared with controls (hazard ratio [HR] 1.43; 95 CI, 1.38-1.48; adjusted HR [aHR], 1.21; 95% CI, 1.16-1.26). The relative association of NFAA with overall mortality was similar in women and men (aHR, 1.22 [95% CI, 1.15-1.28] vs 1.19 [95% CI, 1.11-1.26]; P < .001 in both groups). In contrast, NFAA was associated with a larger increase in mortality among individuals younger than 65 years (aHR, 1.44; 95% CI, 1.31-1.58) than in older individuals (aHR, 1.15; 95% CI, 1.10-1.20; P < .001 for interaction). Mortality due to cardiovascular diseases was increased (aHR, 1.21; 95% CI, 1.13-1.29), as was mortality due to cancer (aHR, 1.54; 95% CI, 1.42-1.67). The association between NFAA and mortality remained significant and of similar magnitude in all sensitivity analyses. The results of this case-control study suggest that NFAA was associated with an increased overall mortality and mortality of cardiovascular disease and cancer. The increase was more pronounced among younger individuals.

Predicting morphological and functional variations of benign adrenal incidentalomas in relation to initial characteristics.

The follow-up strategy for unresected non-functional adrenal tumors (NFAT) is a major controversial issue in endocrinological clinical practice, as the natural history of adrenal incidentalomas (AI) is partially unknown and a consensus on their adequate management is lacking. In a recent longitudinal study by Ceccato etal., a large cohort of patients with conservatively treated AI were evaluated for possible radiological variations over time and their relationship with autonomous cortisol secretion (ACS). Starting from this paper, we performed a literature review of available longitudinal studies focus on the same issue. Notwithstanding the high variability in the duration of follow-up and in the criteria used to define ACS in the included studies, our findings support the idea that there is a not negligible risk of morphological and functional changes, which may have metabolic implications, especially after 5-10 years of follow-up. Unfortunately, these variations seem to be scarcely predictable. Therefore, it may be risky to interrupt the follow-up in patients with NFAT, in particular in the presence of larger diameter of the adenoma and higher cortisol levels at diagnosis. These results should be considered in defining the optimal management of these patients.

The degree of cortisol secretion is associated with diabetes mellitus and hypertension in patients with nonfunctioning adrenal tumors

BackgroundSimilarly to cortisol-secreting adrenal tumors, also non-functioning adrenal tumors (NFAT) may be associated with an increased cardiovascular risk. We assessed in NFAT patients: (i) the association between hypertension (HT), diabetes mellitus (DM), obesity (OB), dyslipidemia (DL) and cardiovascular events (CVE) and cortisol secretion; (ii) the cut-off of the cortisol secretion parameters for identifying NFAT patients with a worse cardiometabolic profile.Patients and methodsIn 615 NFAT patients (with cortisol levels after 1 mg overnight dexamethasone suppression test, F-1mgDST < 1.8 µg/dL [50 nmol/L]) F-1mgDST and adrenocorticotroph hormone (ACTH) levels and data on HT, DM, OB, DL and CVEs prevalence were retrospectively collected.ResultsHT, DM and HT plus DM were associated with F-1mgDST levels (area under the ROC curve: 0.588 ± 0.023, 0.610 ± 0.028, 0.611 ± 0.033, respectively, p < 0.001 for all comparisons) but not with ACTH. The cut-off for identifying patients with either HT or DM or HT plus DM was set at ≥ 1.2 µg/dL (33 nmol/L). As compared with patients with F-1mgDST < 1.2 µg/dL (n = 289), patients with F-1mgDST 1.2–1.79 µg/dL (33–49.4 nmol/L) (n = 326) had lower ACTH levels (17.7 ± 11.9 vs 15.3 ± 10.1 pg/mL, respectively, p = 0.008), older age (57.5 ± 12.3 vs 62.5 ± 10.9 years, respectively, p < 0.001), and higher prevalence of HT (38.1% vs 52.5% respectively p < 0.001), DM (13.1% vs 23.3%, respectively, p = 0.001), HT plus DM (8.3% vs 16.9%, respectively, p < 0.002) and CVE (3.2% vs 7.3%, respectively, p = 0.028). F-1mgDST 1.2–1.79 µg/dL was associated with either HT (odd ratio, OR, 1.55, 95% confidence interval, 95% CI 1.08–2.23, p = 0.018) or DM (OR 1.60, 95% CI 1.01–2.57, p = 0.045) after adjusting for age, gender, OB, DL, and DM (for HT) or HT (for DM), and with the presence of HT plus DM (OR 1.96, 95% CI 1.12–3.41, p = 0.018) after adjusting for age, gender, OB and DL.ConclusionsIn NFAT patients, F-1mgDST 1.2–1.79 µg/dL seems to be associated with a higher prevalence of HT and DM and a worse cardiometabolic profile, even if the poor accuracy of these associations suggests caution in interpreting these results.

The degree of cortisol secretion is associated with cardiometabolic complications in patients with nonfunctioning adrenal tumors

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Tuberculosis of Adrenal Glands—A Population-based Case-control Study

Adrenal tuberculosis (ATB) can cause primary adrenal insufficiency (PAI) or may be misdiagnosed as nonfunctional adrenal tumors (NFATs) in patients with tuberculosis. Very little is known about its epidemiology in a modern, high-income setting. The aim was to investigate adrenal involvement and associated mortality in patients with tuberculosis. By using national registers, patients with tuberculosis and adrenal lesions were compared with controls without adrenal tumors. To analyze mortality in individuals with ATB or possible adrenal affection (ie, tuberculosis and NFAT), a subgroup of controls with tuberculosis was selected. The study population was included from 2005 to 2019 and followed until death or 2020. In mortality adjustments were made for age and sex. Eight patients with ATB, 23 232 patients with NFAT, and 144 124 controls were included. Among those with NFAT, we found 34 with tuberculosis and NFAT. Among controls, 129 individuals diagnosed with tuberculosis were identified. The risk of having an adrenal tumor was increased in tuberculosis (odds ratio, 1.64; 95% CI, 1.12-2.39). Of those with ATB, 7 (88%) had PAI. One patient (3%) with tuberculosis and NFAT and 1 (0.8%) control with tuberculosis had PAI. Compared with controls with tuberculosis, mortality was increased in patients with ATB (hazard ratio, 5.4; 95% CI, 2.2-13.2; adjusted hazard ratio, 6.2; 95% CI, 2.5-15.6), and in patients with tuberculosis and NFAT (1.3; 0.6-2.7; 2.3; 1.1-5.1). PAI was a contributing factor in 4/6 (67%) deaths in patients with ATB. Tuberculosis with adrenal lesions was extremely rare. Most patients with ATB had PAI and mortality was increased.

GIANT NON-FUNCTIONING ADRENOCORTICAL CARCINOMA

Objective: This study aims to report a case of a 51 year old male who was referred to our Department of Urology and was diagnosed with a nonfunctioning giant ACC. Case(s) Presentation: The case report describes a 51-year-old Indonesian man who presented to our department with complaints of persistent upper left abdomen discomfort, previously misdiagnosed as dyspepsia. He was diagnosed as having non-functioning adrenocortical carcinoma and underwent open adrenalectomy. Discussion: Although the patient is 51 years old and the tumor has grown to a signify cant size, no lymph node involvement or metastases was found and resection margins were found to be negative. In our patient, functional adrenal work-up results were normal. This has led us to suspect a non-functioning adrenal tumor. Metastatic work-up with CT cranial, chest, and bone survey were performed, with negative result. The biopsy result after resection confirmed the diagnosis of ACC. Conclusion: ACC is a rare aggressive tumor, malignant with poor prognosis. In our case, the non-secretory mass was diagnosed late by manifestation of mass effect symptoms in the upper left quadrant of the abdomen and was previously misdiagnosed as symptoms of dyspepsia. The functional work-up of adrenal masses helped in the determination of its non-functional status. Early diagnosis of ACC and early surgical excision helps in improving the overall survival rate of the patient.&#x0D; Keywords: Non-functioning, adrenocortical carcinoma (ACC), adrenalectomy, case report.

Iliopsoas muscle to visceral fat ratio on CT predicts Cushing's syndrome in elderly females with adrenal tumors.

There is no computed tomography (CT)-based numerical index for predicting Cushing's syndrome (CS) in patients with adrenal incidentalomas. We tested the hypothesis that the iliopsoas muscle (Ip-M) to visceral fat (V-fat) ratio (IVR) on CT may predict CS in elderly female patients with adrenal tumors. We examined the V-fat area, subcutaneous fat (S-fat) area, Ip-M area, V-fat/S-fat ratio, and IVR at the third lumbar vertebra (L3) level using abdominal CT in female patients aged ≥50 years with cortisol-producing adrenal tumor diagnosed with CS or non-functioning adrenal tumor (NFT) in the derivation cohort. We performed receiver operating characteristic (ROC) analysis to evaluate the diagnostic value of the V-fat/S-fat ratio and IVR for predicting CS. We assessed the usefulness of the IVR in a separate validation cohort. In the derivation cohort, the IVR was significantly lower in the 9 patients with CS than in the 15 patients with NFT (p < 0.001). In ROC analysis with a cut-off value of 0.067, the IVR showed a sensitivity of 100%, specificity of 80.0%, positive likelihood ratio (PLR) of 5.000, and negative likelihood ratio (NLR) of 0.000. The area under the curve was significantly higher for the IVR than for the V-fat/S-fat ratio (0.933 vs. 0.704, respectively, p = 0.036). In 23 patients in the validation cohort, the IVR demonstrated a PLR of 5.714 and an NLR of 0.327. The novel IVR index, based on single-slice CT at the L3 level, predicted CS in elderly female patients with adrenal tumors.

Retroperitoneal laparoscopic partial adrenalectomy (RLPA) for 20-40mm nonfunctional adrenal tumors in the day surgery mode.

To investigate the outcome and safety of retroperitoneal laparoscopic partial adrenalectomy in the treatment of nonfunctional unilateral adrenal tumors in the day surgery mode. Nineteen patients bearing nonfunctional unilateral 20-40mm adrenal tumors were prospectively enrolled and underwent retroperitoneal laparoscopic partial adrenalectomy in the day surgery unit of our hospital between June 2021 and March 2022. All patients were diagnosed with non-functional adrenal tumors as outpatients before being admitted to the day surgery unit with their consent. Patient demographics and perioperative data were prospectively documented. The patients were followed up by telephone on day 1, 3 and 7 after discharge and followed up for 6 months. The patient's age was 50.5 ± 11.9 yr (range from 19.0 - 69.0). Seven patients were female. Twelve patients underwent surgery on the left side. The maximal diameter of tumor was 28.3 ± 5.7mm (20.0 - 40.0mm). Operation time was 72.1 ± 14.9min (58.0 - 120.0min). Mean blood loss was 64.7 ± 50.4ml (30.0 - 200.0ml). The gastrointestinal function recovery time was 9.7 ± 2.6h (6.0 - 16.0h). Retroperitoneal drainage was removed 24.8 ± 13.3h (range 18.0 - 72.0) after surgery. Four patients were transferred to the general ward for postoperative management, while others were discharged within 24 hours after surgery. Length of hospital stay was 48.8 ± 13.1h (38.0 - 85.0h). Hospitalization expense was 24168.4 ± 2910.3 RMB¥ (20844.3 - 34369.8 RMB¥). Postoperative pathology revealed 17 cortical adenoma, 1 pheochromocytoma and 1 lymphatic duct tumor. Retroperitoneal laparoscopic partial adrenalectomy for nonfunctional unilateral adrenal tumors in the day surgery mode is safe when strict selection criteria and perioperative management protocol are followed, which has the potential to shorten length of hospital stay and reduce lower hospitalization costs.

RF19 | PSUN33 Adrenocortical Carcinoma as a Rare Manifestation of Birt-Hogg-Dubé Syndrome

Abstract Adrenocortical carcinoma (ACC) can occur as part of hereditary cancer syndromes, particularly Lynch syndrome and Li-Fraumeni syndrome. Here we describe 5 patients (3 women) with adrenal tumors and Birt-Hogg-Dubé syndrome (BHDS). BHDS is a hereditary tumor syndrome characterized by fibrofolliculomas/trichodiscomas, renal tumors, lung cysts, and spontaneous pneumothoraces. All five patients had a confirmed germline FLCN pathogenic variant. One case was previously reported by our institution. Age of diagnosis (AD) ranged from 36 to 62 years with a mean AD of 46 years. All patients identified as Caucasian. Three patients had non-functioning adrenal tumors, while the remaining two patients did not undergo biochemical assessment preoperatively but did not have symptoms or history of adrenal hormone excess. Histologically, four patients had confirmed ACC while one had an adrenocortical tumor of uncertain malignant potential, most consistent with an oncocytoma. All patients were initially treated with mitotane. Three patients developed metastatic disease requiring additional systemic chemotherapy, immunotherapy, and/or radiation. A total of 8 confirmed ACCs in patients with BHDS have been reported to date, raising suspicions that these tumors might be a part of the BHDS tumor spectrum. When BHDS patients are screened for renal malignancies, special attention should be paid to the adrenal glands. Adrenal nodules should undergo prompt assessment as &amp;gt;50% of reported cases developed metastatic disease. Given possible association with BHDS, testing for FLCN pathogenic variants should be considered during the genetic evaluation of patients with ACC. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m., Sunday, June 12, 2022 12:54 p.m. - 12:59 p.m.

Primary Mature Cystic Teratoma of the Retroperitoneum Presenting as a Suprarenal Mass in an Adult Patient: A Case Report

Introduction: Primary retroperitoneal mature cystic teratomas (PRPMCT) are uncommon in adults. They typically occur in infants under six months old and young females. So far, some limited case reports have been documented in the literature. This case report presents a rare case of an adult patient with PRPMCT aged over 30 years. Case Presentation: The patient was a 34-year-old female with PRPMCT in the site of the left adrenal gland and abdominal discomfort. Computed tomography (CT) scan revealed a 66 mm hypodense lesion with fat attenuation and calcification in the left retroperitoneum. Laparotomy with resection of the retroperitoneal (RP) lesion was done. Gross examination showed a solid multicystic mass measuring 8x6.5x6 cm. Histopathologic examination revealed a neoplastic tissue composed of mature osseous and cartilaginous tissue associated with stratified squamous epithelium and lumens lined by ciliated columnar epithelium, and the diagnosis was mature cystic teratoma. The patient had an uncomplicated postoperative course without recurrence after seven months of follow-up. Conclusions: PRPMCT in adults is extremely rare. RP metastasis of primary gonadal teratoma and non-functioning adrenal tumors should be excluded first. Due to the diagnostic difficulty of posterior peritoneal teratoma with radiological imaging and its risk of malignancy, surgical resection would be the most effective diagnostic and therapeutic option. Complete resection of the mass is important to judge the existence of immature and solid components that require a long-time follow-up because of the increased risk of malignancy.

Hypotension in Posterior Retroperitoneoscopic Versus Transperitoneal Laparoscopic Adrenalectomy.

Despite preoperative optimization, hemodynamic instability can be a major challenge during adrenalectomy. Even brief episodes of intraoperative hypotension can be associated with ischemia-reperfusion injury. This study aimed to compare intraoperative hemodynamic parameters between posterior retroperitoneoscopic adrenalectomy (PRA) and transperitoneal laparoscopic adrenalectomy (TPA). This is a retrospective study of patients undergoing PRA and TPA without conversion or concomitant intraabdominal pathology from 2008 to 2019. The primary outcome was intraoperative hypotension defined by mean arterial pressure <60mm Hg or the need for ≥1 intravenous vasopressors at least 30min after anesthetic induction. Overall, 108 patients met the inclusion criteria; 33 (30.6%) had pheochromocytoma, 26 (24.1%) had aldosterone excess, 8 (7.4%) had corticosteroid excess, and 41 (38.0%) had nonfunctioning adrenal tumors. Of these, 68 (63.0%) underwent PRA and 40 (37.0%) underwent TPA. Age, sex, body mass index, preinduction blood pressure, number of preoperative antihypertensives, and histopathological diagnosis were similar in the two groups. Tumor size was greater in the TPA group. The presence of pheochromocytoma was an independent risk factor for hypotension. Multivariate analysis revealed that PRA was associated with a higher risk of experiencing a mean arterial pressure <60mm Hg (odds ratio 4.44, 95% confidence interval 1.27-15.54, P=0.02) and the need for ≥1 intravenous vasopressors (odds ratio 9.97, 95% confidence interval 3.34-29.78, P<0.001) compared with TPA. Although PRA offers several advantages over TPA, it carries a greater risk of intraoperative hypotension. A prospective trial is required to validate these findings. Nevertheless, institution of risk reduction strategies is encouraged to be considered for individuals undergoing PRA.

Mortality in patients with non-functional adrenal tumors: a swedish population-based national cohort study

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)