scholarly journals SAT-200 Clinical Dilemmas of Adrenal Incidentaloma, Is Change in HU Clinically Significant?

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Azra Niaz ◽  
John Jun ◽  
Sophia Y Ali

Abstract Introduction There has been a recent increase in the incidence of adrenal incidentaloma (AI), defined as an adrenal mass that’s unintentionally discovered on imaging obtained for an indication other than suspected adrenal pathology, mainly because of increased availability and improved quality of imaging modalities. Differentiation based on malignant vs benign and secretory vs non secretory becomes the most important question on finding such mass. Another important question is long term follow up of these AI. We present a case that highlights the importance of follow up due to the potential of AI to increase in size and density over 3 year. Case A 58 years old male with a history of ESRD secondary to hypertension, controlled hypertension, CAD s/p CABG was seen for a right 1.9 cm AI (found in 11/2016 on noncontrast CT abdomen) with low attenuation of approximately 10 Hounsfield units (HU). He was asymptomatic, normal vitals, and an unremarkable physical exam. Hormonal work up was negative for hyperaldosteronism and Cushing syndrome. About 1 year later, repeat CT abdomen reported an unchanged right, 2.0 x 1.7 cm adrenal mass, 22 HU. Hormonal work-up was not completed and patient was lost to follow up. Two years later, repeat CT abdomen reported increase size of AI to 2.7 cm and increase in attenuation of 24 HU precontract which increased to 55 HU on the immediate postcontrast exam and 55 HU on 13-minute delayed images. Hormonal work up was negative for Cushing syndrome and hyperaldosteronism. Plasma metanephrine was 0.50 with elevated plasma normetanephrine of 3.85. In light of being a renal transplant patient and due to increase in size, change in HU, and significantly elevated normetanephrine levels patient was referred for right adrenalectomy. Discussion Change in adrenal mass size is known to be a significant predictor of malignant potential. An unenhanced attenuation value <10 HU is characteristic of a lipid-rich adenoma, can predict benign adrenal adenoma with 98% specificity, and has a very low likelihood of a pheochromocytoma. European Society of Endocrinology recommends if the AI is homogenous, < 4 cm, with a density ≤ 10 UH, no imaging follow up is recommended and biochemical testing for ruling out pheochromocytoma is indicated in adrenal tumors with an unenhanced attenuation value >10HU. However, the AACE/AAES guidelines recommends AI ≥ 1cm and less than 4cm, repeat imaging with noncontrast CT should be performed at 3–6 months and annually for 1-2 years and if the mass grows or becomes hormonally active then adrenalectomy should be performed. Conclusion There are no prospective studies of the optimal frequency and duration of follow up for AI. Considering the increasing incidence of AI, further studies on the clinical significance of change in HU, such as risk of developing pheochromocytoma, along with standardized international guidelines would be helpful for clinicians in managing patients with AI.

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Andreas Ebbehoj ◽  
Ravinder J Kaur ◽  
Dingfeng Li ◽  
Sumitabh Singh ◽  
Catherine Zhang ◽  
...  

Abstract Background: Adrenal tumors are reported in 5% of adults, with malignancy rates in 1–12%, and rates of overt hormonal excess in 1–15 %. However, most estimates originate from convenience samples. Our objective was to determine the incidence, prevalence and clinical presentation of adrenal tumors in a population-based setting. Methods: We used a centralized epidemiologic database to identify patients diagnosed with adrenal tumors in a local community 1995 to 2017. The database is a unique medical records linkage system that allows access to hospital and community medical records for local residents (population 137,000). We calculated incidence rates (IR) as the number of new patients diagnosed while living in the study area, and prevalence as the number of patients living in the study area on Dec 31 2017. IR and prevalence were sex- and age-adjusted to the 2010 US Census population. Results: Of 1,287 patients diagnosed with adrenal tumor, the median age of diagnosis was 62 years (IQR 52–72), 713 (55%) were women, and 13 (1%) were younger than 18 years at diagnosis. IR was highest in patients >65 YO, followed by patients 40–64 YO, 18–39 YO and <18 YO (142 vs 66 vs 9 vs 2 per 100,000 persons years). IR per 100,000 increased from 4.4 (CI95% 0.3–8.6) in 1995 to 47.8 (CI95% 36.9–58.7) in 2017. Overall prevalence in the population was 0.53% in 2017, ranging from 0.01% among 0–17 YO to 1.9% among +65 YO. Malignant adrenal mass was diagnosed in 8.7% patients (4 patients with adrenal cortical carcinoma (0.3%) and 108 (8.4%) patients with other malignant mass). Pheochromocytoma was diagnosed in 11 (1.1%) patients and benign adrenal mass was diagnosed in 1,175 (90.2%) patients (1,076 (83.6%) with adrenal adenoma and 85 (6.6%) with other benign mass). Median tumor size was 15 mm (range 5–255), and 184 (14%) of patients had bilateral tumors. Only 255 (20%) patients had dexamethasone suppression test, 93 (36%) with cortisol>1.8 mcg/dl. Of 1,076 adrenal adenomas, 53 (4.9%) had overt hormone excess, 140 (13%) had nonfunctioning adrenal adenomas, and 88 (8.2%) had mild autonomous cortisol secretion. Hormonal work up for was incomplete in 795 (73.9%) adenomas. Patients discovered incidentally (1,050, 81.6%) had a lower rate of malignancy and hormone excess (5.3% vs 52% of patients with non-incidental discovery, p <0.001). Rate of malignancy was highest in children (67% vs 8% >18YO, p<0.001), bilateral tumors (16% vs 8% unilateral, p<0.001), tumors ≥4 cm (33% vs 7% in < 4 cm, p<0.001). Conclusion: IR of adrenal tumors increased 10-fold since 1995, and was highest in patients >65 YO. 8.7% of tumors were malignant, with a majority represented by malignant adrenal tumors other than adrenal cortical carcinoma. The risk of malignancy was highest in non-incidental discovery, children, and tumors > 4 cm. Overt hormone excess was diagnosed in 4.5% of patients. The majority of patients with adrenal adenomas had a suboptimal work up for hormone excess.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Sonia Sharma ◽  
Steven N Levine ◽  
Xin Gu

Abstract Introduction: In an adult endocrine clinic, the majority of patients referred for evaluation of an adrenal incidentaloma are older than 30 years of age. It is important to be reminded that a patient may be diagnosed with an adrenal mass at any age but the etiology may vary depending on the age at presentation. Clinical case: An 18 year-old African American female with no significant past medical problems presented with a 2 month history of flank and abdominal pain associated with nausea and vomiting. An abdominal CT scan and a dedicated adrenal CT showed a right adrenal mass measuring 2.2 x 2.6 cm. The noncontrast Hounsfield units were 23, enhanced Hounsfield units 210, and delayed Hounsfield units 72. The calculated washout was 44%, not consistent with an adrenal adenoma. An MRI of the abdomen showed a 2.5 cm right adrenal nodule. The lesion did not demonstrate significant loss of signal between in and out of phase imaging, therefore the characteristics were not consistent with a lipid rich adenoma. Laboratory tests included an ACTH of 31 pg/mL (6-48 pg/mL), cortisol 8.7 ug/mL at 10:57 am (7-9 am 5.27-22.45 ug/mL), aldosterone 10.1 ng/dL (6-48 ng/dL), renin 2.2 ng/mL/hr (upright 0.5-4.0 ng/mL/hr), DHEA-sulfate 129 ug/dL (44-248 ug/dL), plasma free metanephrine 0.10 nmol/L (0.00-0.49 nmol/L), and plasma free normetaneprhine 0.41 nmol/L (0.00-0.89 nmol/L). The 24-hour urine norepinephrine, epinephrine, and metanephrine were all normal, however the 24-hour urine dopamine was elevated, 824 ug/24 hrs (52-480 ug/24 hrs). Subsequently, plasma dopamine, norepinephrine, and epinephrine were all within the reference range. The patient had a robotic-assisted right adrenalectomy removing a 5.7 x 3.5 x 1.7 cm gland, weighing 16.3 grams. The pathology demonstrated a ganglioneuroma within the right adrenal gland measuring 2.2 x 2.0 x 2.7 cm, negative for neuroblastoma or blastic components. Focal hemorrhage was noted, there was no tumor necrosis, and no mitotic figures were present. The tumor appeared to be encapsulated in the adrenal gland and the Ki-67 stain was negative in ganglioneuroma cells. Conclusion: Adrenal adenomas that appear as incidentalomas in young adulthood are extremely rare. Evaluating younger versus older adults found to harbor an adrenal “incidentaloma“ requires a unique approach for each age group, as the differential diagnosis varies widely. In our patient, the imaging was extremely concerning and diagnostic considerations included neuroblastoma, adrenocortical malignancy, pheochromocytoma, or ganglioneuroma. Adrenal ganglioneuromas are most frequently diagnosed in fourth and fifth decades of life. In younger adults ganglioneuromas are usually found in the retroperitoneum and posterior mediastinum. For our patient, surgical resection of the adrenal mass confirmed the pathologic diagnosis and provided definitive cure.


Endocrine ◽  
2020 ◽  
Author(s):  
Pierpaolo Falcetta ◽  
Francesca Orsolini ◽  
Elena Benelli ◽  
Patrizia Agretti ◽  
Paolo Vitti ◽  
...  

Abstract Purpose To evaluate the risk of mass enlargement and endocrine function modification in patients with adrenal incidentaloma (AI). Methods In this retrospective study, we examined clinical and hormonal characteristics of 310 patients with AI (200 females and 110 males; age: 58.3 ± 12.9 years), followed up for a median (interquartile range) of 31.4 months (13.0–78.6) and evaluated for possible modification in adrenal mass size and hormonal function. The hormonal evaluation included morning serum cortisol and plasma ACTH at 8 a.m., aldosterone, plasma renin activity/direct renin concentration, and 24-h urine metanephrines/normetanephrines. One microgram overnight dexamethasone suppression test (DST) was performed. Autonomous cortisol secretion (ACS) was diagnosed in the presence of cortisol after 1 mg DST > 5 μg/dl (138 nmol/l) or >1.8 and ≤5 μg/dl (50–138 nmol/l) and at least one of the following: (i) low ACTH; (ii) increased 24-h urinary-free cortisol; (iii) absence of cortisol rhythm; and (iv) post-LDDST cortisol level > 1.8 μg/dl (50 nmol/l). When there was no biochemical evidence of adrenal hormonal hyperactivity, AIs were classified as nonfunctioning (NFAIs). The mass was considered significantly enlarged when the size increase was more than 20% and at least 5 mm compared to baseline. Results At diagnosis, NFAIs were found in 209 patients, while ACS and overt adrenal hyperfunction were diagnosed in 81 and 20 patients, respectively. During follow-up, 3.3% and 1.5% of patients with NFAI developed subtle and overt endocrine hyperfunction, respectively, while a significant mass enlargement was observed in 17.7% of all AIs. The risk of developing ACS was significantly higher in patients with adenoma >28 mm (hazard ratio [HR] 12.4; 95% confidence interval [CI], 2.33–66.52, P = 0.003), in those with bilateral adrenal tumors (HR: 5.36; 95% CI, 1.17–24.48, P = 0.030), and with low/suppressed ACTH values (HR: 11.2, 95% CI 2.06–60.77; P = 0.005). The risk of mass enlargement was lower for patients in the fourth quartile of body mass index than those in the first quartile (HR 0.33; 95% CI, 0.14–0.78; P = 0.012). Conclusions In patients with AI, the risk of developing hormonal hyperfunction and mass enlargement is overall low, although some tumor characteristics and anthropometric features might increase this risk. Taking account of all these aspects is important for planning a tailored follow-up in AI patients.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Ramon B Larrazabal

Abstract BACKGROUND The National Institutes of Health defines adrenal incidentalomas (AIs) as clinically inapparent adrenal masses discovered inadvertently in diagnostic testing or treatment for conditions not related to the adrenal glands. Non-functional adenoma remains the most frequent (60–85%) cause, while functional adenomas at 5–16%. CLINICAL CASE A previously healthy 41 year-old female consulted for a 2-month history of left flank pain. Computed tomography (CT) scan of the kidneys revealed left calculi and incidental right adrenal mass. She was asymptomatic at this time. She then underwent shockwave lithotripsy for the renal calculi. However, post-operatively, she had elevated blood pressure and was started on anti-hypertensives. She remained hypertensive despite being on four different medications. Pertinent physical examination findings: plethora of the face and extremities, Moon facies, Buffalo Hump, and pendulous abdomen with grayish striae. Further work-up revealed the AI to be cortisol-secreting. Pertinent laboratories: 1 mg dexamethasone suppression test - 800 nmol/L (<50 nmol/L), 24-hr urine free cortisol - 1014.86 ug/24 hr (20–90 ug/24 hr), Adrenocorticotropic Hormone (ACTH) - 5.0 pg/ml (9–52 pg/ml). She then underwent a right laparoscopic adrenalectomy. Post-operatively, her blood pressure normalized without her anti-hypertensive medications. Tissue biopsy of the mass was consistent with an adrenocortical adenoma. On her follow-up after one month, signs of Cushing’s Syndrome were clinically improving. CONCLUSION This is the first case that demonstrated shockwave lithotripsy converting a non-functioning adrenal incidentaloma into a functioning one. It also shows how internists and surgeons (i.e. Urologists) can manage various aspects of patient care through the facilitation of medical treatments, surgical interventions, and ensuring a proper multidisciplinary approach based on the endocrinology clinical guidelines. So as not to delay the delivery of proper management to the patient. REFERENCES (1) Grumbach M, Biller B, Braunstein G. et al. “Management of the clinically inapparent adrenal mass (“incidentaloma”).” Annals of internal medicine 2003; 138:424–4292 Herrera M, Grant C, van Heerden J. et al. “Incidentally discovered adrenal tumors: an institutional perspective.” Surgery 1991; 110:1014.


Open Medicine ◽  
2018 ◽  
Vol 13 (1) ◽  
pp. 281-284 ◽  
Author(s):  
Ryszard Pogorzelski ◽  
Krzysztof Celejewski ◽  
Sadegh Toutounchi ◽  
Ewa Krajewska ◽  
Tomasz Wołoszko ◽  
...  

AbstractIntroductionIncidentaloma is defined as an tumor diagnosed accidentally using imaging studies performed due to other indications. The aim of this paper was to describe the diagnostic and treatment problems experienced by patients with adrenal incidentaloma in a clinical practice.Material and methodsIn years 2009-2012 there were 33(16,5%) adrenal tumors diagnosed incidentally out of 200 cases treated due to adrenal pathology. The group consisted of 54 patients aged 27-77. In 15(45,5%) patients the diagnosis was made based on ultrasound examination, while in 18(54,5%) the tumor was visualized in CT/MRI.ResultsOnly after the diagnostics was finalized, in which in all cases no signs of hormonal activity were detected, 26(78,8%) patients were qualified for the surgery. In 7(21,2%) cases no indications for such a management were found. The latter group was followed-up and in all patients the indications for the surgery arose due to enlargement of the tumor or/and the existence of hormonal activity. Our observations suggest that the incident finding of adrenal tumor is an indication of long-term observation. The analysis of our material shows that all patients observed needed surgical treatment.ConclusionsThe small adrenal tumors under follow-up have a tendency to enlarge and acquire hormonal activity. Long-term observation significantly increases the costs of treatment in that group, which eventually results in surgical management.


2016 ◽  
Vol 175 (2) ◽  
pp. G1-G34 ◽  
Author(s):  
Martin Fassnacht ◽  
Wiebke Arlt ◽  
Irina Bancos ◽  
Henning Dralle ◽  
John Newell-Price ◽  
...  

By definition, an adrenal incidentaloma is an asymptomatic adrenal mass detected on imaging not performed for suspected adrenal disease. In most cases, adrenal incidentalomas are nonfunctioning adrenocortical adenomas, but may also represent conditions requiring therapeutic intervention (e.g. adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma or metastasis). The purpose of this guideline is to provide clinicians with best possible evidence-based recommendations for clinical management of patients with adrenal incidentalomas based on the GRADE (Grading of Recommendations Assessment, Development and Evaluation) system. We predefined four main clinical questions crucial for the management of adrenal incidentaloma patients, addressing these four with systematic literature searches: (A) How to assess risk of malignancy?; (B) How to define and manage low-level autonomous cortisol secretion, formerly called ‘subclinical’ Cushing’s syndrome?; (C) Who should have surgical treatment and how should it be performed?; (D) What follow-up is indicated if the adrenal incidentaloma is not surgically removed?Selected recommendations:(i) At the time of initial detection of an adrenal mass establishing whether the mass is benign or malignant is an important aim to avoid cumbersome and expensive follow-up imaging in those with benign disease. (ii) To exclude cortisol excess, a 1mg overnight dexamethasone suppression test should be performed (applying a cut-off value of serum cortisol ≤50nmol/L (1.8µg/dL)). (iii) For patients without clinical signs of overt Cushing’s syndrome but serum cortisol levels post 1mg dexamethasone >138nmol/L (>5µg/dL), we propose the term ‘autonomous cortisol secretion’. (iv) All patients with ‘(possible) autonomous cortisol’ secretion should be screened for hypertension and type 2 diabetes mellitus, to ensure these are appropriately treated. (v) Surgical treatment should be considered in an individualized approach in patients with ‘autonomous cortisol secretion’ who also have comorbidities that are potentially related to cortisol excess. (vi) In principle, the appropriateness of surgical intervention should be guided by the likelihood of malignancy, the presence and degree of hormone excess, age, general health and patient preference. (vii) Surgery is not usually indicated in patients with an asymptomatic, nonfunctioning unilateral adrenal mass and obvious benign features on imaging studies. We provide guidance on which surgical approach should be considered for adrenal masses with radiological findings suspicious of malignancy. Furthermore, we offer recommendations for the follow-up of patients with adrenal incidentaloma who do not undergo adrenal surgery, for those with bilateral incidentalomas, for patients with extra-adrenal malignancy and adrenal masses and for young and elderly patients with adrenal incidentalomas


2018 ◽  
Vol 1 (1) ◽  
Author(s):  
Muzzammil Abdullahi ◽  
Mamuda Atiku ◽  
Imam Mohammed Ibrahim

Cushing’s syndrome is a clinical disorder caused by overproduction of cortisol. Adrenal adenoma is the cause in 5% of cases of Cushing syndrome. ACTH-independent Cushing’s syndrome in 90% is caused by unilateral adrenal tumors. Of these, adenomas are the cause in 80% of the cases, while the others are adrenocortical carcinoma. Rare causes of Cushing’s syndrome include adrenal hyperplasia. Overproduction of cortisol results in weakened protein structures leading to protuberant abdomen and poor wound healing, glucose is converted to fat and deposited in the abdomen, supraclavicular fossa and cheeks. Other presentations are diabetes, hypertension, osteoporosis, fractures, impaired immune function, glucose intolerance, and psychosis. We report a 21-year-old female who presented with clinical symptoms of newly diagnosed hypertension and diabetes mellitus which after poor response to treatment she was evaluated and diagnosed to have Cushing syndrome. She subsequently had left adrenalectomy and did very well with resolution of patients’ symptoms. The histology came out to be diffuse adrenal hyperplasia.


1999 ◽  
Vol 84 (2) ◽  
pp. 520-526
Author(s):  
Luisa Barzon ◽  
Carla Scaroni ◽  
Nicoletta Sonino ◽  
Francesco Fallo ◽  
Agostino Paoletta ◽  
...  

The natural course of adrenal incidentalomas and the risk that such lesions evolve toward hormonal hypersecretion or malignancy are still under evaluation. Of 246 consecutive patients with adrenal incidentaloma studied at our institution in the last 15 yr, 91 underwent surgery. Of the remaining patients, a group of 75 (52 females and 23 males; median age, 56 yr; range, 19–77 yr) with incidentally discovered asymptomatic adrenal masses (60 unilateral and 15 bilateral; median diameter, 2.5 cm; range, 1.0–5.6) was enrolled in an endocrine and morphological follow-up of at least 2 yr after diagnosis (median, 4 yr; range, 2–10). During follow-up, no patients developed malignancy; 9 showed mass enlargement, with appearance of a new mass in the contralateral gland in 2; 3 developed adrenal hyperfunction (overt Cushing’s syndrome in 2, nonclinical hypercortisolism in 1); and 3 showed adrenal mass enlargement associated with adrenal hyperfunction (nonclinical hypercortisolism in 2, pheochromocytoma in 1). The estimated cumulative risks to develop mass enlargement and hyperfunction were 8% and 4%, respectively, after 1 yr, 18% and 9.5% after 5 yr, and 22.8% and 9.5% after 10 yr. Nine risk factors for adrenal mass enlargement or hyperfunction were arbitrarily selected and evaluated: sex, age, presence of obesity, hypertension, diabetes, abnormal endocrine tests, mass size, mass location, and scintigraphic uptake pattern. Three of them attained statistical significance: mass size of 3 cm or more at diagnosis and exclusive radiocholesterol uptake by the mass at scintigraphy had relevance for the occurrence of adrenal hyperfunction, whereas the presence of endocrine test abnormalities at diagnosis had predictive value for mass enlargement. It is concluded that subtle hormonal abnormalities are risk factors for mass size increase, which is not a sign of malignant transformation. Both mass size of 3 cm or more at diagnosis and exclusive radiocholesterol uptake, indicating higher risks of hyperfunction, should be considered to plan a more thorough endocrine follow-up.


2019 ◽  
Vol 8 (7) ◽  
pp. 838-845 ◽  
Author(s):  
Gamze Akkuş ◽  
Isa Burak Güney ◽  
Fesih Ok ◽  
Mehtap Evran ◽  
Volkan Izol ◽  
...  

Background The management of adrenal incidentaloma is still a challenge with respect to determining its functionality (hormone secretion) and malignancy. In this light, we performed 18F-FDG PET/CT scan to assess the SUVmax values in different adrenal masses including Cushing syndrome, pheochromocytoma, primary hyperaldosteronism and non-functional adrenal adenomas. Methods Total 109 (73 F, 36 M) patients with adrenal mass (incidentaloma), mean age of 53.3 ± 10.2 years (range, 24–70) were screened by 18F-FDG PET/CT. Data of 18F-FDG PET/CT imaging of the patients were assessed by the same specialist. Adrenal masses were identified according to the calculated standardized uptake values (SUVs). Clinical examination, 24-h urine cortisol, catecholamine metabolites, 1-mg dexamethasone suppression test, aldosterone/renin ratio and serum electrolytes were analyzed. Results Based on the clinical and hormonal evaluations, there were 100 patients with non-functional adrenal mass, four with cortisol-secreting, four with pheochromocytomas and one with aldosterone-secreting adenoma. Mean adrenal mass diameter of 109 patients was 2.1 ± 4.3 (range, 1–6.5 cm). The 18F-FDG PET/CT imaging of the patients revealed that lower SUVmax values were found in non-functional adrenal masses (SUVmax 3.2) when compared to the functional adrenal masses including four with cortisol-secreting adenoma (SUVmax 10.1); four with pheochromcytoma (SUVmax 8.7) and one with aldosterone-secreting adenomas (SUVmax 3.30). Cortisol-secreting (Cushing syndrome) adrenal masses showed the highest SUVmax value (10.1), and a cut-off SUVmax of 4.135 was found with an 84.6% sensitivity and 75.6% specificity cortisol-secreting adrenal adenoma. Conclusions Consistent with the similar studies, non-functional adrenal adenomas typically do not show increased FDG uptake and a certain form of functional adenoma could present various FDG uptake in FDG PET/CT. Especially functional adrenal adenomas (cortisol secreting was the highest) showed increased FDG uptake in comparison to the non-functional adrenal masses. Therefore, setting a specific SUVmax value in the differentiation of malignant adrenal lesion from the benign one is risky and further studies, including a high number of functional adrenal mass are needed.


2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A117-A118
Author(s):  
Ahmad Al Maradni ◽  
Karyne Lima Vinales ◽  
Ricardo Rafael Correa

Abstract Introduction: Adrenal Incidentalomas (AI) are increasingly found with recent proliferation in imaging techniques. The precise prevalence of AI is not well established but estimated to be about 5%. Such findings require thorough work and follow up plans to assess for hormonal functionality and differentiate between benign or a malignant entity. Adrenocortical carcinoma (AC), schwannoma (SCH), ganglioneuroma (GN), myelolipoma (ML), neuroblastoma (NB), and ganglioneuroblastoma (GNB) are a rare presentation of AIs. In this case series, we present 2 cases of uncommon AIs. Cases Series: Case 1: A 55-year-old man presented to the hospital with episodic dizziness, nausea, vomiting, and diaphoresis. His physical examination and blood workup were unremarkable. He takes multiple antihypertensive medications at home. CT abdomen revealed an incidental 5 cm mass adjacent to the left adrenal gland which was confirmed to be of adrenal origin on MRI. The hormonal workup was negative. and patient. Laparoscopic left adrenalectomy was performed. Histopathological diagnosis pointed to a benign nerve sheath tumor. Tumor cells stained positive for S-100 and negative for CD34 and epithelial membrane antigen. The morphologic features and immunohistochemical profile consistent with adrenal SCH diagnoses, confirmed by two histopathologists. Postoperatively, Blood pressure improved, and the patient has no evidence of recurrence on subsequent follow-ups. Case 2: A 38-year-old female presented to the hospital with severe right groin pain. During the workup, a CT abdomen revealed a homogeneous, lobulated, minimally enhanced, 5.8 cm right adrenal mass with no calcifications. The hormonal workup was negative. FDG-PET scan showed low uptake in the right adrenal mass. Adrenalectomy was performed and histological examination revealed Schwan cells and neurites with occasional ganglion cells compatible with benign GN. Discussion: SCHs originate from Schwann cells in peripheral nerve sheaths. Adrenal SCHs are rare, they represent about 0.5% of all SCHs. Surgical resection is the initial management as imagings are non-diagnostic and appearance does not distinguish benign from malignant lesions. Adrenal GNs originate from neural crest cells in the adrenal medulla and represents between 20–30% of all GNs. They may be primary or evolve from differentiating NB. The majority of them are sporadic. GNs are typically benign and asymptomatic. However, larger GNs are associated with an increased risk of malignancy, a 33% risk for 5 cm compared to 92% for 6 cm. Surgical resection is necessary, especially for larger masses. Conclusion: Besides the common adrenal incidentalomas and their proper work and follow up plans, a clinician should consider atypical adrenal tumors with unusual presentation. Surgical treatment and histological examination are essential for an accurate diagnosis.


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