Adrenal Cortical Adenoma Research Articles

6629 Pure Androgen Secreting Adrenal Adenoma In A Premenopausal Woman

Abstract Disclosure: S. Syed: None. Background: Pure androgen secreting adrenal adenomas are extremely rare; most of the reported cases occur in post-menopausal women, with significant virilization of acute onset and are usually malignant. Here we present a case of an androgen secreting adrenal adenoma found in a young female with regular menstrual cycles and hirsutism. Clinical Case: A 27-year-old female with no significant past medical history was referred to Endocrinology clinic for evaluation of elevated testosterone level noted on a routine OBGYN exam. Patient had miscarriage six months prior to initial endocrine clinic visit. She had a routine follow up visit with her gynecologist after miscarriage and noted to have mild male pattern hair growth on her chin, worse after stopping birth control pills two years ago. Patient had menarche at 14 years of age and had regular menstrual cycle until started birth control pills. The skin examination revealed coarse male pattern hair on chin. Scattered acne was present on back. No signs of hypercortisolism were present. Labs done at time of initial consultation showed unremarkable comprehensive metabolic panel, thyroid and gonadotropic hormones. Testosterone was elevated at 122ng/dl (0-75ng/dl). Pelvic ultrasound done in the year prior showed normal uterus and adnexal structures. Further work up showed elevated DHEAS 781 µg/dL (18-391 µg/dL) and normal 17 OH progesterone and androstenedione levels. MRI abdomen with and without contrast done at outside facility showed a 4 cm x 3.3 cm x 4.1 cm well-circumscribed round shaped, mildly heterogeneously enhancing mass arising from right adrenal gland, with restricting diffusion, no signal dropout in out-of-phase imaging. Normal ovaries were noted. Given the large size of lesion, adrenalectomy was recommended. Patient underwent laparoscopic right adrenalectomy. The histologic features on pathology specimen were consistent with an adrenal cortical adenoma. No evidence of malignancy was seen (based on modified Weiss criteria). 3 weeks post op labs showed Testosterone level reduced to 19 ng/dl (0-75ng/dl) and DHEAS 138 ug/dl (18-391 µg/dL). Patient is recovering from recent childbirth. Conclusion: In adults, pure androgen secreting adrenal tumors are extremely rare. Diagnosis of malignancy is made with histopathological examination and Weiss score calculation. Our patient has unique presentation of having mild hirsutism in setting of regular menstrual cycles with no other symptoms of virilization and is noted to have unexpectedly large adrenal adenoma. Given the size of adenoma, there was concern for adrenal cortical carcinoma, however histological examination was consistent with a benign lesion. Presentation: 6/1/2024

Clinical and imaging presentations are associated with function in incidental adrenocortical adenomas: a retrospective cohort study.

The aim of this study is to assess whether clinical and imaging characteristics are associated with the hormonal subtype, growth, and adrenalectomy for incidental adrenal cortical adenomas (ACAs). This is a single-center cohort study. Consecutive adult patients with incidental ACA were diagnosed between 2000 and 2016. Of the 1516 patients with incidental ACA (median age 59 years, 62% women), 699 (46%) had nonfunctioning adenomas (NFAs), 482 (31%) had mild autonomous cortisol secretion (MACS), 62 (4%) had primary aldosteronism (PA), 39 (3%) had Cushing syndrome, 18 (1%) had PA and MACS, and 226 (15%) had incomplete work-up. Age, sex, tumor size, and tumor laterality, but not unenhanced computed tomography Hounsfield units (HU), were associated with hormonal subtypes. In a multivariable analysis, ≥1 cm growth was associated with younger age (odds ratio [OR] = 0.8 per 5-year increase, P = .0047) and longer imaging follow-up (OR = 1.2 per year, P < .0001). Adrenalectomy was performed in 355 (23%) patients, including 38% of MACS and 15% of NFA. Adrenalectomy for NFA and MACS was more common in younger patients (OR = 0.79 per 5-year increase, P = .002), larger initial tumor size (OR = 2.3 per 1 cm increase, P < .0001), ≥1 cm growth (OR = 15.3, P < .0001), and higher postdexamethasone cortisol (OR = 6.6 for >5 vs <1.8 μg/dL, P = .002). Age, sex, tumor size, and laterality were associated with ACA hormonal subtype and can guide diagnosis and management. Tumor growth was more common with younger age and longer follow-up. Unenhanced HU did not predict hormonal subtype or growth. Adrenalectomy for MACS and NFA was mainly performed in younger patients with larger tumor size, growth, and elevated postdexamethasone cortisol.

Heart rupture – a complication of adrenal cortical adenoma – case report

Heart rupture – a complication of adrenal cortical adenoma – case report

A novel method of tunneling retroperitoneoscopic adrenalectomy: a prospective study.

To introduce the surgical technique and our team's extensive experience with tunnel method in laparoscopic adrenalectomy. From July 2019 to June 2022, we independently designed and conducted 83 cases of " Tunnel Method Laparoscopic Adrenalectomy," a prospective study. There were 45 male and 38 female patients, ages ranged from 25 to 73 years(mean: 44.6 years).The cases included 59 adrenal cortical adenomas, 9 pheochromocytomas, 6 cysts, 4 myelolipomas, 1 ganglioneuroma, and 4 cases of adrenal cortical hyperplasia. In terms of anatomical location, there were 39 cases on the left side, 42 on the right side, and 2 bilateral cases. Tumor diameters ranged from 0.6 to 5.9cm(mean: 2.9cm). Utilizing ultrasound monitoring, percutaneous puncture was made either directly to the target organ or its vicinity, and the puncture path was manually marked. Then, under the direct view of a single-port single-channel laparoscope, the path to the target organ in the retroperitoneum or its vicinity was further delineated and separated. This approach allowed for the insertion of the laparoscope and surgical instruments through the affected adrenal gland, thereby separating the surface of the target organ to create sufficient operational space for the adrenalectomy. All 83 surgeries were successfully completed. A breakdown of the surgical approach reveals that 51 surgeries were done using one puncture hole, 25 with two puncture holes, and 7 with three puncture holes. The operation time ranged from 31 to 105min (mean: 47min), with a blood loss of 10 to 220mL (mean: 40 mL). Notably, there were no conversions to open surgery and no intraoperative complications. Postoperative follow-up ranged from 6 to 28 months, during which after re-examination using ultrasound, CT, and other imaging methods, there were no recurrences or other complications detected. The completion of the tunnel method laparoscopic adrenalectomy represents a breakthrough, transitioning from the traditional step-by-step separation of retroperitoneal tissues to reach the target organ in conventional retroperitoneoscopic surgery. This method directly accesses the target organ, substantially reducing the damage and complications associated with tissue separation in retroperitoneoscopic surgery, As a result, it provides a new option for minimally invasive surgery of retroperitoneal organs and introduces innovative concepts to retroperitoneoscopic surgery.

Neoplasia epitelial adrenocortical com intenso pleomorfismo celular em paciente jovem: Um relato de caso

Adrenocortical epithelial neoplasms represent a diverse spectrum of tumors originating in the adrenal cortex, such as adrenal cortical carcinomas (ACCs) and adrenal cortical adenomas (ACAs), which are rare but clinically significant tumors. The aim of this article is to report the clinical case of a 29-year-old patient who was affected by an adrenocortical epithelial neoplasm with intense cellular pleomorphism. Surgical treatment was performed urgently to resolve the initial condition, and the diagnosis was aided by imaging and anatomopathological methods.

Hand2 Immunohistochemistry in the Diagnosis of Paragangliomas and Other Neuroendocrine Neoplasms.

Hand2 is a core transcription factor responsible for chromaffin cell differentiation. However, its potential utility in surgical pathology has not been studied. Thus, we aimed to investigate its expression in paragangliomas, other neuroendocrine neoplasms (NENs), and additional non-neuroendocrine tumors. We calibrated Hand2 immunohistochemistry on adrenal medulla cells and analyzed H-scores in 46 paragangliomas (PGs), 9 metastatic PGs, 21 cauda equina neuroendocrine tumors (CENETs), 48 neuroendocrine carcinomas (NECs), 8 olfactory neuroblastomas (ONBs), 110 well-differentiated NETs (WDNETs), 10 adrenal cortical carcinomas, 29 adrenal cortical adenomas, 8 melanomas, 41 different carcinomas, and 10 gastrointestinal stromal tumors (GISTs). Both tissue microarrays (TMAs) and whole sections (WSs) were studied. In 171 NENs, previously published data on Phox2B and GATA3 were correlated with Hand2. Hand2 was positive in 98.1% (54/55) PGs, but only rarely in WDNETs (9.6%, 10/104), CENETs (9.5%, 2/21), NECs (4.2%, 2/48), or ONBs (12.5%, 1/8). Any Hand2 positivity was 98.1% sensitive and 91.7% specific for the diagnosis of PG. The Hand2 H-score was significantly higher in primary PGs compared to Hand2-positive WDNETs (median 166.3 vs. 7.5; p < 0.0001). Metastatic PGs were positive in 88.9% (8/9). No Hand2 positivity was observed in any adrenal cortical neoplasm or other non-neuroendocrine tumors, with exception of 8/10 GISTs. Parasympathetic PGs showed a higher Hand2 H-score compared to sympathetic PGs (median H-scores 280 vs. 104, p < 0.0001). Hand2 positivity in NENs serves as a reliable marker of primary and metastatic PG, since other NENs only rarely exhibit limited Hand2 positivity.

Steroid metabolites producing adenoma: a case report

Hyperandrogenism is the most prevalent cause of menstrual cycle abnormalities and infertility in women. Here, we present a case of a 32-year-old woman with a 7-year history of menstrual irregularity and infertility. Laboratory test results revealed elevated 17-hydroxyprogesterone, progesterone 21-deoxycortisol. Abdominal computed tomography found a 3,9х2,9х2,6 cm left adrenal tumor. Non-classic congenital adrenal hyperplasia was diagnosed initially, however, treatment with supraphysiological doses of dexamethasone proved ineffective and progesterone and 17-hydroxyprogesterone levels remained high. Genetic testing revealed no 21-hydroxylase deficiency. Laparoscopic adrenalectomy was performed with subsequent pathological report being compatible with an adrenal cortical adenoma. 17-hydroxyprogesterone, progesterone 21-deoxycortisol levels returned to the normal range postoperatively and the patient’s menstrual cycle normalized without additional medication. Steroid metabolites producing adenomas are also extremely rare with only a few cases found in patients without previous CAH diagnosis. When a patient with clinically and biochemically diagnosed NCCAH demonstrate no typical features and shows poor response to steroid therapy, the patient should receive multisteroid LC–MS/MS assay for glucocorticoids and androgens, adrenal and ovarian imaging and undergo CYP21A2 gene mutation analysis.

P-07 Unraveling the Mystery-Conn's Syndrome

Abstract Background Conn's syndrome, also known as primary aldosteronism, is a rare disease that can manifest at a young age. Resolving the underlying cause of hypertension is crucial, as seen in this case report. Clinical Case A 17-year-old female patient was admitted with a history of recurrent fever over the past 6 months, bifrontal headaches, palpitations, and myalgia for the past 4 months. Examination revealed resistant hypertension, and laboratory results showed persistent hypokalemia and an elevated aldosterone/renin ratio. CT imaging revealed a left adrenal adenoma. A laparoscopic intervention successfully addressed the uncontrolled hypertension. Histological examination confirmed a diagnosis of a benign adrenal cortical adenoma. Conclusion Post-surgery, the patient's overall condition significantly improved, and blood pressure was effectively controlled. This case report underscores the rare presentation of Conn's syndrome in young individuals and emphasizes the importance of investigating specific underlying causes of hypertension to prevent associated morbidity and mortality.Figure 1.Conns syndrome, CT scan, histology section gross.CECT scan of abdomen and pelvis - left adrenal adenoma gross section - cut section adrenal gland -showing 2 cysts one on medial side 1*1cm and inferiolateral side 1*0.5cm histology of the removed adrenal confirmed diagnosis of benign adrenal cortical adenoma Table 1.Conns syndrome investigations Table 2.Conns syndrome investigations

Adrenal cortical adenoma arising in the setting of renal–adrenal fusion: a case report and review of the literature

BackgroundRenal–adrenal fusion is a rare entity resulting from incomplete encapsulation of the adrenal gland and kidney. Only 18 cases have been reported in English literature to date.Case presentationOur patient is a 77-year-old African American female who presented with a systolic blood pressure of 200 mmHg. Computed tomography showed an enhancing 9 × 6 cm mass anterior and medial to the left kidney. The patient underwent a left adrenalectomy with partial nephrectomy. Gross and histologic examinations revealed an adrenal cortical adenoma and renal–adrenal fusion.ConclusionRenal–adrenal fusion may pose a diagnostic challenge, particularly when there is a concurrent adrenal adenoma. It is important to be aware of this uncommon anomaly to avoid misdiagnosis and overtreatment.

Unmasking the Silent Menace: A Case Report of Aldosterone-Secreting Adrenal Cortical Adenoma

Background: Adrenal cortical adenoma with aldosterone hypersecretion, the most prevalent cause of secondary hypertension, represents a significant subset of adrenal tumors with distinct clinical implications. Clinical Description: A 30-year-old female presented with generalized body pain for two years and multiple episodes of sudden onset progressive weakness of the upper and lower limbs. Management: The diagnosis was confirmed by serum aldosterone and plasma renin activity and a laparoscopic right adrenalectomy was performed. The histopathology of the removed specimen helped to rule out adrenal cortical carcinoma Conclusion: A prompt diagnosis and surgical management improves the quality of life for patients with such rare diseases.

Evaluation of adrenal masses using 18F-FDOPA PET/CT in patients suspected with pheochromocytoma.

Pheochromocytomas (PHEOs) are chromaffin cell-derived adrenal tumors. 6-[18F]-L-fluoro-L-3, 4-dihydroxyphenylalanine (18F-FDOPA) is a radiotracer taken up in neuroendocrine chromaffin cells via the L-type amino-acid transporter. 18F-FDOPA is useful in patients with PHEO. However, more information about the use of 18F-FDOPA PET/CT scan is needed. Thus, the current study investigated various PET parameters on preoperative 18F-FDOPA PET/CT scan. The 18F-FDOPA PET/CT scan findings of 29 patients who underwent adrenalectomy after PET/CT scans were evaluated according to their pathologic diagnosis. Thereafter, the patients were classified under different risk groups which were compared based on the Grading System for Adrenal Pheochromocytoma and Paraganglioma (GAPP). In terms of histopathologic results after surgery, 24 patients presented with PHEO. The remaining 5 patients were diagnosed with adrenal cortical adenomas or adrenal medullary hyperplasia. The maximum standardized uptake value, mean standardized uptake value, tumor-to-liver ratio, and tumor-to-contralateral adrenal gland ratio of PHEOs on preoperative 18F-FDOPA PET/CT scan were higher than those of other tumors. The metabolic tumor volume (MTV) and total lesion uptake of PHEOs in the intermediate-risk group (n = 19) were higher than those in the low-risk group (n = 5). The MTV and total lesion uptake were significantly correlated with the GAPP score. Preoperative 18F-FDOPA PET/CT is helpful to identifying PHEOs. In addition, imaging interpretation using the standardized uptake value of the suspected tumor or the tumor-to-liver/contralateral adrenal gland ratio can be effective. The metabolic parameters of PHEOs are positively correlated with the GAPP score.

FRI197 Patient Suffering From Resistant HTN For 15 Years With Negative CT Diagnosed With Hyperaldosteronism Due To Adrenal Cortical Adenoma

Abstract Disclosure: J. Seidenberg: None. M.J. Ansari: None. Background: Resistant HTN, blood pressure remaining above goal despite concurrent use of three antihypertensives of different classes at maximum doses is a common presentation in both endocrinology and primary care settings. Patients can often be prescribed multiple max-dosed antihypertensives with varying results and adverse effects and still spend years with uncontrolled HTN leading to long term health concerns. Hyperaldosteronism is diagnosed for resistant HTN in 4% of primary care patients and 10% endocrinology-referred patients. Adrenal adenomas account for 40% of all cases characterized by high aldosterone/renin activity ratio, hypokalemia, mild hypernatremia and hypomagnesemia commonly diagnosed with a mass found in the adrenals on CT. Clinical Case: Patient is a 39 year old Female who presented to the endocrinology office due to elevated urine metanephrine levels, BP 159/100. Patient had resistant HTN since her early 20s treated currently with labetalol and nifedipine, and hypokalemia treated with potassium supplements. Lab results revealed potassium 3.4 mmol/L (3.6 -5.2 mmol/L), normetanephrines 787 mcg/g (0-145 mcg/g) , aldosterone 11 ng/dL (2-9 ng/dL) , renin 0.2 ng/mL/h (0.7- 3.3 ng/mg/hr), aldosterone/renin activity ratio 55 (&amp;lt;25) . Renal US showed no renal artery stenosis. The patient was suspected of having hyperaldosteronism using cutoff ratios aldosterone renin ratio &amp;gt;30, and 15 years of potassium levels between 3.0 and 3.5. CT of the adrenals revealed no masses. Patient underwent IR guided venous sampling which indicated R adrenal gland producing 45x more aldosterone than the L adrenal gland. Patient underwent R sided adrenalectomy for definitive management of primary hyperaldosteronism. Pathology showed cortical adenoma. Patient was continued on chlorthalidone, losartan and nifedipine, and within 3 months her BP averaged 120/70 mmHg, with potassium within normal limits, Aldosterone 13 ng/dL, renin 1.25 ng/mL. Conclusion: Concurrent hyperaldosteronism with essential HTN is an uncommon but potential cause of resistant HTN. Patients with resistant HTN, prescribed multiple max-dosage antihypertensive medications while also having negative renal artery US result should undergo aldosterone and renin lab work. CT scans can have low sensitivity for adrenal adenoma. Concerning lab work should be followed up by IR-guided venous sampling for evaluation of an adrenal cortical adenoma, which can be treated surgically. Underlying essential HTN will still require treatment, but at significantly decreased medication dosages. Patients with resistant HTN should be evaluated for hyperaldosteronism early in their disease course regardless of a negative CT to prevent sequelae of untreated hyperaldosteronism including cardiac hypertrophy, arrhythmias, nephrogenic diabetes insipidus and neuromuscular symptoms. Presentation: Friday, June 16, 2023

FRI214 Adrenal Adenoma With Biochemical Findings Suggestive Of Cushing's Disease

Abstract Disclosure: A. Calimag: None. U. Tarabichi: None. M. Ahmad: None. M. Kazi: None. Introduction: We are presenting a case of a young female with prior history of multiple endocrinopathies presenting with a left adrenal adenoma with biochemical findings suggestive of Cushing's disease. Case Presentation: A 22-year-old female with a history of Type 1 Diabetes Mellitus was found to have a left adrenal adenoma 2.8 cm on CT. Absolute washout of 85 % and a relative washout of 63%, having 12 Hounsfield units (HU) on the non-contrast phase, findings suggestive of a benign adenoma. Further work up including 1 mg Dexamethasone suppression test (DST) showed unsuppressed plasma ACTH 17 pg/ml and total cortisol 9.4 mcg/dL. 8 mg DST showed ACTH 12 pg/ml, AM cortisol 8.8 mcg/dL, and Dexamethasone 991 ng/dl. Late night salivary cortisol 0.07 ug/dl, and 0.09 ug/dl, 24 hr urine cortisol 40.7. Other labs showed Aldosterone level was normal at 6.7, Renin level was also normal at 1.1. 24 hr urine catecholamines were Norepinephrine 35 mcg/24 hr, Epinephrine 3.9 mcg/24 hr, while urine Metanephrine level was &amp;lt;0.24, and normetanephrine level at 0.34. TSH 1.2mcU/ml. One day prior to surgery ACTH level was suppressed at 2.1 pg/ml drawn from a different lab. MRI of the Pituitary showed a slightly enlarged pituitary gland with uniform contrast enhancement and no definite lesion. The patient underwent a laparoscopic left adrenalectomy. Pathology showed an adrenal cortical neoplasm consistent with adrenal cortical adenoma, with no significant cytologic atypia, mitotic activity, or necrosis. Immunoperoxidase stain, the tumor is positive for inhibin and negative for PAX8. Post-op she developed adrenal insufficiency which was treated with hydrocortisone. The follow-up cortisol level was 11.3 mcg/dL when the patient was off hydrocortisone. Discussion: In this case, the biochemical exam showed elevated cortisol with ACTH not fully suppressed at both 1 mg and 8 mg DST which was done twice, and normal 24-hour urine cortisol. Determining whether this is adrenal vs pituitary mediated was one of the clinical dilemmas of this case. Initially, the patient’s ACTH was unsuppressed however the day before surgery ACTH was suppressed and the patient’s cortisol level normalized after the left adrenalectomy. Immunoassays for ACTH levels may occasionally be susceptible to analytical errors. (1-2) There are several case series and studies mentioning assay interference when using Immulite Assay versus Cobas ACTH assay. (1-2) Therefore, it is important to analyze clinical findings and biochemical testing and if lab tests are discordant, recheck using a different analytical platform in a different lab or an alternate ACTH assay to confirm ACTH levels. (1-2)

SAT339 Osteopenia In The Setting of Mild Autonomous Cortisol Secretion

Abstract Disclosure: J.J. Mao: None. K.C. McCowen: None. C. Choe: None. Jimmy J. Mao, MD (1); Karen C. McCowen (1); Charles H. Choe (1)(1) Division of Endocrinology, Diabetes, and Metabolism, University of California San Diego, La Jolla, CA. Background: Over 80% of adrenal incidentalomas are benign adrenal cortical adenomas, of which 30-50% are found to have mild autonomous cortisol secretion (MACS). Bone fragility is a known complication of MACS. Clinical Case: A 57-year-old postmenopausal female with no prior history of fracture, venous thromboembolism, and atherosclerotic cardiovascular disease, was referred for incidental bilateral adrenal nodules. Body mass index was normal at 21 kg/m2, and no clinical stigmata of Cushing syndrome were noted on physical exam. Unenhanced computed tomography scan revealed a 4.6 cm right adrenal nodule and a 3.3 cm left adrenal nodule; both nodules measured less than -18 Hounsfield Units. Biochemical work-up for pheochromocytoma, primary aldosteronism, and non-classical congenital adrenal hyperplasia was negative. Evaluation for hypercortisolism revealed a morning cortisol of 20.3 mcg/dL (ref. 4-22 mcg/dL) with concurrent adrenocorticotropic hormone of &amp;lt;5 pg/mL (ref. 6-50 pg/mL), 24-hour urine free cortisol of 16.9 mcg (ref. 4-50 mcg), and midnight salivary cortisol of 0.07 mcg/dL (ref. &amp;lt;0.09 mcg/dL). A 1 mg dexamethasone suppression test measured on two separate occasions was elevated at 6.3 mcg/dL and 7.9 mcg/dL (ref. &amp;lt;1.8 mcg/dL). A subsequent 8 mg dexamethasone suppression test was also elevated at 6.1 mcg/dL (ref. &amp;lt;1.8 mcg/dL). The diagnosis of MACS was made. A bone mineral density (BMD) scan revealed T-scores at the right femoral neck, left femoral neck, and lumbar spine at -1.6, -1.3, and -0.8, respectively. Ten-year probability of major osteoporotic fracture and hip fracture were 8.3% and 1.0%, respectively. To reduce the risk for future fractures, the decision was made to proceed with adrenal vein sampling (AVS) in preparation for potential adrenalectomy. Conclusion: MACS is the most frequent hormonal abnormality in adrenal incidentalomas, with an estimated prevalence of 0.8-2 cases per 1000 persons. A previous prospective study found that surgical cure of unilateral MACS may lead to a substantial risk reduction (up to 15-fold) of future incident asymptomatic vertebral fractures. Additionally, the highest risk for clinical fracture in those with adrenal adenomas (either nonfunctional or with cortisol hypersecretion) appears to occur at 7-10 years after initial diagnosis. BMD does not perfectly correlate with fracture risk, so unilateral adrenalectomy (if lateralization is observed on AVS) and/or pharmacotherapy should be strongly considered in patients with an expected longevity of &amp;gt;10 years even with mild osteopenia and low fracture risk assessment tool scores. Presentation: Saturday, June 17, 2023

FRI206 A Rare Case Of Coexisting Acromegaly And Adrenal Cushing’s Syndrome

Abstract Disclosure: T. Chamakkala: None. T. Gallagher: None. Introduction: Acromegaly has been associated with higher risk of cancer morbidity and mortality. Acromegaly coexisting with adrenal Cushing’s disease has been rarely described in literature. We present a patient who was found to have acromegaly during evaluation for Cushing’s syndrome. Clinical Case: A 50-year-old woman presented to the office for endocrinology evaluation at the referral of PCP for elevated 24h urine cortisol level 176ug/24h. She had noticed facial plethora over several years, fatigue, and easy bruising. She had recently been diagnosed with hypertension, poorly controlled on two medications at the time of evaluation. Repeat 24h urine cortisol and midnight salivary cortisol levels were elevated; dexamethasone suppression test was positive. ACTH was &amp;lt;1.5pg/mL, consistent with ACTH independent hypercortisolemia. Adrenal CT showed 2.6x3cm left adrenal adenoma. She underwent left adrenalectomy and pathology showed adrenal cortical adenoma. During evaluation for Cushing’s disease and new onset hypertension, IGF1 was evaluated and found to be elevated at 354ng/mL. She additionally endorsed symptoms of worsening carpal tunnel syndrome and increasing ring size. After adrenalectomy, IGF1 was repeated and remained elevated at 429ng/mL. Growth hormone suppression test showed nadir of 0.56ng/mL which is higher than &amp;lt;0.3ng/mL expected of high sensitivity GH assays. Pituitary MRI showed three suspected pituitary microadenomas, 4mm and 5mm in left gland, 7mm in right gland. Given difficulty with surgical approach and higher risk of hypopituitarism, she was started on lanreotide for treatment without surgery. She underwent genetic counseling and was offered genetic testing. Conclusion: This case highlights the rare coexistence of acromegaly and adrenal Cushing’s disease in a 50 year old woman. Though the relationship between acromegaly and cancer has been historically controversial, more recent studies have shown an increased risk of both benign and malignant tumors with acromegaly. Untreated Cushing’s Disease has been associated with mildly elevated IGF1 which then decreases after remission; it is unclear if the same pathological process is involved in other etiologies of hypercortisolemia like adrenal Cushing’s syndrome, but re-evaluation of IGF1 after treatment of Cushing’s should be considered. As Cushing’s and acromegaly share similar consequences such as worsening HTN, changing facial appearance, and worsening diabetes, it is prudent to consider each as differential diagnoses when signs do not remit after treatment. Acromegaly has additionally been associated with other endocrinopathies in syndromes including MEN1 syndrome, McCune Albright Syndrome, and Carney Complex. Evaluation for these syndromes and consideration for genetic testing should be considered in the evaluation of a patient with acromegaly and additional associated endocrinopathies. Presentation: Friday, June 16, 2023

Synchronous ipsilateral adrenal cortical adenoma and ganglioneuroma: A case report

ABSTRACT Synchronous tumors comprising of cortical as well as medullary differentiation are rare in the adrenal gland. Collision tumors comprise of two independent neoplasms arising from the cortex and the medulla without any histological admixture. Adrenal cortico-medullary mixed tumors are rare tumors comprising of adrenal cortical and chromaffin cells intermixed with each other. Ganglioneuroma is a rare benign tumor originating from the paravertebral sympathetic ganglia and is rarely seen in the adrenal medulla. The presence of a synchronous ganglioneuroma with an adrenal cortical adenoma in the ipsilateral adrenal gland is exceedingly rare. We report a collision tumor comprising of an adrenal cortical adenoma and a ganglioneuroma detected as an incidental finding in a pediatric patient who presented with hypertension and palpitations.

Asymmetric Adrenals: Sexual Dimorphism of Adrenal Tumors.

Sexual dimorphism has direct consequences on the incidence and survival of cancer. Early and accurate diagnosis is crucial to improve prognosis. This work aimed to characterized the influence of sex and adrenal asymmetry on the emergence of adrenal tumors. We conducted a multicenter, observational study involving 8037 patients with adrenal tumors, including adrenocortical carcinoma (ACC), aldosterone-producing adenoma (APA), cortisol-secreting adrenocortical adenomas (CSAs), non-aldosterone-producing adrenal cortical adenoma (NAPACA), pheochromocytoma (PCC), and neuroblastoma (NB), and investigated tumor lateralization according to sex. Human adrenal tissues (n = 20) were analyzed with a multiomics approach that allows determination of gene expression, catecholamine, and steroid contents in a single sample. In addition, we performed a literature review of computed tomography and magnetic resonance imaging-based studies examining adrenal gland size. ACC (n = 1858); CSA (n = 68), NAPACA (n = 2174), and PCC (n = 1824) were more common in females than in males (female-to-male ratio: 1.1:1-3.8:1), whereas NBs (n = 2320) and APAs (n = 228) were less prevalent in females (0.8:1). ACC, APA, CSA, NAPACA, and NB occurred more frequently in the left than in the right adrenal (left-to-right ratio: 1.1:1-1.8:1), whereas PCC arose more often in the right than in the left adrenal (0.8:1). In both sexes, the left adrenal was larger than the right adrenal; females have smaller adrenals than males. Adrenal asymmetry in both sexes may be related to the pathogenesis of adrenal tumors and should be considered during the diagnosis of these tumors.

Expression of EMP 1, 2, and 3 in Adrenal Cortical Neoplasm and Pheochromocytoma.

The purpose of this study is to investigate the expression of the epithelial membrane proteins (EMP) 1, 2, and 3 in adrenal gland neoplasm and to explore the broader implications of this. Tissue microarrays were constructed for 132 cases of adrenal cortical neoplasms (ACN) (adrenal cortical adenoma (115 cases), and carcinoma (17 cases)) and 189 cases of pheochromocytoma. Immunohistochemical staining was performed to identify EMP 1, 2, and 3, and was compared with clinicopathological parameters. The H-score of EMP 3 (p < 0.001) was higher in pheochromocytoma when compared to that of ACN, and the H-score of EMP 1 (p < 0.001) and EMP 3 (p < 0.001) was higher in adrenal cortical carcinomas when compared to that of adrenal cortical adenomas. A higher EMP 1 H-score was observed in pheochromocytomas with a GAPP score ≥3 (p = 0.018). In univariate analysis, high levels of EMP 1 and EMP 3 expression in ACN were associated with shorter overall survival (p = 0.001). Differences were observed in the expression of EMPs between ACN and pheochromocytoma. EMPs are associated with malignant tumor biology in adrenal cortical neoplasm and pheochromocytoma, suggesting the role of a prognostic and/or predictive factor for EMPs in adrenal tumor.

Expression of Amine Oxidase Proteins in Adrenal Cortical Neoplasm and Pheochromocytoma.

We delved into the expression of amine oxidase family proteins and their potential significance in adrenal gland neoplasm. Tissue microarrays were prepared for 132 cases of adrenal cortical neoplasm (ACN) consisting of 115 cases of adrenal cortical adenoma (ACA), 17 cases of adrenal cortical carcinoma (ACC), and 163 cases of pheochromocytoma (PCC). Immunohistochemical stainings for MAOA, MAOB, LOX, and AOC3 were performed to evaluate the H-scores and compare them with clinicopathological parameters. The H-scores of MAOA (T; p = 0.005) and MAOB (T; p = 0.006) in tumor cells (T) were high in ACN, whereas LOX (T, S; p < 0.001) in tumor and stromal cells (S) and AOC3 (T; p < 0.001) were higher in PCC. In stromal cells, MAOA (S; p < 0.001) and AOC3 (S; p = 0.010) were more expressed in ACA than in ACC. MAOB (S) in PCC showed higher H-scores when the grading of adrenal pheochromocytoma and paraganglioma (GAPP) score was 3 or higher (p = 0.027). In the univariate analysis, low MAOA expression in stromal cells of ACN was associated with shorter overall survival (p = 0.008). In conclusion, monoamine oxidase proteins revealed differences in expression between ACN and PCC and also between benign and malignant cells.

Management of Adrenal Cortical Adenomas: Assessment of Bone Status in Patients with (Non-Functioning) Adrenal Incidentalomas

Our aim is to analyse the bone profile in adults with (non-functioning) adrenal incidentalomas (AIs), specifically addressing the impact of autonomous cortisol secretion (ACS). This narrative review, based on a PubMed search from inception to February 2023 (case reports, non-ACS, and other secondary causes of osteoporosis were excluded), included 40 original studies, a total of 3046 patients with female prevalence (female:male ratio of 1921:1125), aged between 20.5 and 95.5 years old. This three decade-based analysis showed that 37 studies provided dual-energy X-ray absorptiometry (DXA) information; another five studies reports results on bone micro-architecture, including trabecular bone score (TBS), spinal deformity index, and high-resolution peripheral quantitative computed tomography; 20 cohorts included data on bone turnover markers (BTMs), while four longitudinal studies followed subjects between 1 and 10.5 years old (surgical versus non-adrenalectomy arms). Post-dexamethasone suppression test (DST) cortisol was inversely associated with bone mineral density (BMD). TBS predicted incidental vertebral fractures (VFx) regardless of BMD, being associated with post-DST cortisol independently of age and BMD. Low BTMs were identified in ACS, but not all studies agreed. An increased prevalence of ACS-related osteoporosis was confirmed in most studies (highest prevalence of 87.5%), as well as of VFx, including in pre-menopause (42.5%), post-menopause (78.6%), and male patients (72.7%) depending on the study, with a 10-fold increased incidental VFx risk up to a 12-fold increased risk after a 2-year follow-up. No specific medication against osteoporosis is indicated in ACS, but adrenalectomy (according to four studies) should be part of the long-term strategy. This bone profile case sample-based study (to our knowledge, one of the largest of its kind) showed that AIs, including the subgroup designated as having ACS, embraces a large panel of osseous complications. The level of evidence remains far from generous; there are still no homogenous results defining ACS and identifying skeletal involvement, which might be a consequence of different investigation clusters underling adrenal and bone assessments over time. However, bone status evaluations and associated therapy decisions remain an essential element of the management of adults with AIs-ACS.