Could we assess the functional status, of hormone secreting, or non-secreting of the adrenal masses regarding their Magnetic Resonance Imaging (MRI) characteristics?

Background: Diagnostic imaging techniques including magnetic resonance imaging (MRI) should also perform on all patients with incidentalomas. However, there is a limited study whether the quantitative measurements (signal intensity index, adrenal to spleen ratio) in MRI could predict the functional status of adrenal adenomas. Material-Method: Between 2015-2020; 404 patients (265 females, 139 males) with adrenal mass who were referred to the university hospital for further investigation were included. After detailed diagnostic hormonal evaluation, all patients underwent MRI 1.5 T device (Signa, GE Medical Systems; Milwaukee, USA). The signal intensities of the adrenal lesions on T2W images were qualitatively evaluated and noted as homogenous or heterogeneous in comparison with the liver signal intensity (SI). A chemical-shift SI index and chemical shift adrenal-to-spleen SI ratio were also calculated. Results: While 331(81.9%) of the patients had nonfunctional adrenal mass, the rest of them (n=73, 18.1%) were patients with functional (autonomous cortisol secretion-ACS, cushing syndrome-CS, pheochromocytoma, primary hyperaldosteronism-PA) adrenal masses. In phase vs phase values of patients with NFAI, Pheo(n=17), ACS (n=30), CS (n=11), and PA (n=15) were 474.04±126.7 vs 226.6±132.4, 495.3±182.8 vs 282.17±189.1, 445.2±134.8 vs 203.3±76.2, 506.8±126.5 vs 212.2±73.6 and 496.2±147.5 vs 246.6±102.1, respectively. Mean signal intensity index (SII) and adrenal to spleen ratio (ASR) of all groups (NFAI, Pheo, ACS, CS, PA) were 52.0±24.8 and 0.51, 44.9±22.5 and 0.55, 49.5±24.5 and 0.53, 56.2±16.4 and 0.43, 47.6±25.1 and 0.54, respectively. Based the current accepted measurements in the case of ASR and SII, all lesions were similar and shown as fat rich adenomas (p*= 0.552, p** = 0.45). Conclusion: The quantitative assessment (SII, ASR) of intracellular lipids in an incidentally discovered adrenal tumour could only help distinguish adrenal masses in case of adenomas or non-adenomas As initial diagnostic evaluation, clinical and laboratory assessment ,to distinguish hormone secretion, should be taken in all patients with adrenal incidentalomas.

Bilateral adrenal masses due to tuberculosis: how to diagnose without extra-adrenal tuberculosis

Summary Primary adrenal insufficiency is a rare disease and can masquerade as other conditions; therefore, it is sometimes incorrectly diagnosed. Herein, we reported the case of a 39-year-old Vietnamese male with primary adrenal insufficiency due to bilateral adrenal tuberculosis. The patient presented to the emergency room with acute adrenal crisis and a 3-day history of nausea, vomiting, epigastric pain, and diarrhoea with a background of 6 months of fatigue, weight loss, and anorexia. Abdominal CT revealed bilateral adrenal masses. Biochemically, unequivocal low morning plasma cortisol (<83 nmol/L) and high plasma adrenocorticotropic hormone levels were consistent with primary adrenal insufficiency. There was no evidence of malignancy or lymphoma. As the patient was from a tuberculosis-endemic area, extra-adrenal tuberculosis was excluded during the work up. A retroperitoneal laparoscopic left adrenalectomy was performed, and tuberculous adrenalitis was confirmed by the histopathological results. The patient was started on antituberculous therapy, in addition to glucocorticoid replacement. In conclusion, even without evidence of extra-adrenal tuberculosis, a diagnosis of bilateral adrenal tuberculosis is required. A histopathological examination has a significant role along with clinical judgement and hormonal workup in establishing a definitive diagnosis of adrenal tuberculosis without evidence of active extra-adrenal involvement. Learning points Primary adrenal insufficiency can be misdiagnosed as other mimicking diseases, such as gastrointestinal illness, leading to diagnostic pitfalls. Adrenal insufficiency can be confirmed with significantly low morning plasma cortisol levels of <83 nmol/L without a dynamic short cosyntropin stimulation test. Tuberculous adrenalitis is an uncommon treatable condition; however, it remains an important cause of primary adrenal insufficiency, especially in developing countries. In the absence of extra-adrenal involvement, adrenal biopsy plays a key role in the diagnostic process. Alternatively, adrenalectomy for histopathological purposes should be considered if CT scan-guided fine needle aspiration is infeasible in cases of small adrenal masses.

Compliance Issues in Managing 21 Hydroxylase Deficiency and their Short/Long-Term Consequences

Objective: To report a case of untreated classic 21 hydroxylase (OH) deficiency congenital adrenal hyperplasia (CAH) in a transgender patient resulting in pulmonary embolisms (PEs) and bilateral adrenal masses. Methods: A 36-year-old male (birth sex: female) presenting with bilateral PEs in the setting of long-standing, untreated classic 21OH CAH was also found to have bilateral adrenal masses (unconfirmed myelolipomas). Results: Further history revealed a known diagnosis of CAH. The patient had been treated with glucocorticoid and mineralocorticoid replacement in childhood but stopped taking these medications against medical advice. During his hospital admission, he was noted to have elevated 17-hydroxyprogesterone, low cortisol with elevated ACTH levels, and male-level testosterone measurements. CT of the abdomen/ pelvis revealed a 23 cm mass in the left renal fossa and a 2.5 cm mass in the right renal fossa consistent with bilateral adrenal myelolipomas. The patient attended follow-up in clinic, but declined any further hormonal treatment as he identified as male and felt further treatment was unnecessary. Conclusion: This case demonstrated the unique long-term effects of untreated classic CAH due to 21OH deficiency, including bilateral adrenal myelolipoma, adrenal compensation to the point of producing male-level androgens, and possibly PEs. Treatment with hydrocortisone was recommended to suppress ACTH and it was planned that the patient would eventually start on testosterone (although this would have been complicated by his bilateral PEs). Potential aetiologies for the PEs included vascular compression of the renal artery (which could explain the elevated EPO/erythrocytosis contributing to hypercoagulability) or the renal vein by the adrenal mass.

A confluence of rarities: post sedation neuroexcitation following adrenal perineurioma resection

Adrenal masses are frequently discovered incidentally and warrant further workup to explore the etiology of the mass. We present the case of a patient who had an incidentally discovered nonfunctional adrenal mass, which was determined to be a perineurioma. This is the first case report, to our knowledge of a perineurioma occurring in this location. Additionally, we report an episode of post sedation excitation, or “propofol frenzy” in the same patient to add to the clinical spectrum of presentation of this phenomenon.

Adrenal wash-out CT: moderate diagnostic value in distinguishing benign from malignant adrenal masses

Objective: Reliable results of wash-out CT in the diagnostic workup of adrenal incidentalomas are scarce. Thus, we evaluated the diagnostic accuracy of delayed wash-out CT and determined thresholds to accurately differentiate adrenal masses. Design: Retrospective, single-center cohort study including 216 patients with 252 adrenal lesions who underwent delayed wash-out CT. Definitive diagnoses based on histopathology (n=92) or comprehensive follow-up. Methods: Size, average attenuation values of the adrenal lesions in all CT scan phases, absolute and relative percentage washout (APW/RPW) were determined by an expert radiologist blinded for clinical data. Adrenal lesions with unenhanced attenuation values >10HU built a subgroup (n=142). Diagnostic accuracy were calculated. Results: The study group consisted of 171 adenomas, 32 other benign tumors, 11 pheochromocytomas, 9 adrenocortical carcinomas and 29 other malignant tumors. All (potentially) malignant and 46% of benign lesions showed unenhanced attenuation values >10HU. In this most relevant subgroup, the established thresholds of 60% for APW and 40% for RPW misclassified 35.9% and 35.2% of masses, respectively. When we applied optimized cutoffs (APW>83%; RPW>58%) and excluded pheochromocytomas, we missed only 1 malignant tumor by APW and none by RPW. However, only 11% and 15% of benign tumors were correctly identified. Conclusions: Washout CT with the established thresholds for APW und RPW is insufficient to reliably diagnose adrenal masses. Using the proposed cutoff of 58% for RPW, malignant tumors will be correctly identified, but the added value is limited, namely 15% of patients with benign tumors can be prevented from additional imaging or even unnecessary surgery.

A Case Report of HIV-Related Bilateral Inflammatory Myofibroblastic Tumors of Adrenal Gland

Inflammatory myofibroblastic tumor (IMT) is a rare disease which mostly occurs at younger age and locates in lung in general population. We report a rare case of 44-year-old man diagnosed adrenal IMT with HIV infection, who refused regular highly active antiretroviral therapy (HAART) 13 years ago until in hospital because of findings of adrenal masses. The patient underwent CT-guided needle biopsy successfully, and the pathological analysis documented the diagnosis of IMT by the feature of proliferation of fibroblastic-myofibroblastic with inflammatory infiltration. We failed to perform tumor complete resection due to diffuse invasion of tumor under laparoscope. The patient was complicated with severely multiple pulmonary infection post to surgery because of immunodeficiency, that eventually caused the death of patient 2 months later. This case reminds us that IMTs may be too aggressive and progressed in HIV-positive patients with irregular HAART to perform surgical resections, and severe immunodeficiency can be more fatal. To our knowledge, this case is the second IMT patient with HIV infection worldwide, but the first case occurs at adrenal gland rather than lung in adult.

Characteristics of a Novel ATP2B3 K416_F418delinsN Mutation in a Classical Aldosterone-Producing Adenoma

In patients with primary aldosteronism (PA), the prevalence of ATP2B3 mutation is rare. The aim of this study is to report a novel ATP2B3 mutation in a PA patient. Based on our tissue bank of aldosterone-producing adenomas (APA), we identified a novel somatic ATP2B3 K416_F418delinsN mutation. The affected individual was a 53 year-old man with a 4 year history of hypertension. Computed tomography (CT) showed bilateral adrenal masses of 1.6 (left) and 0.5 cm (right) in size. An adrenal venous sampling (AVS) showed a lateralization index (LI) of 2.2 and a contralateral suppression index (CLS) of 0.12; indicating left functional predominance. After a left unilateral adrenalectomy, he achieved partial biochemical and hypertension–remission. This classical adenoma harbored a novel ATP2B3 K416_F418delinsN somatic mutation, which is a deletion from nucleotides 1248 to 1253. The translated amino acid sequence from 416 to 418, reading as lysine-phenylalanine-phenylalanine, was deleted; however, an asparagine was inserted due to merging of residual nucleotide sequences. The CYP11B2 immunohistochemistry staining demonstrated strong immunoreactivity in this classical adenoma. The ATP2B3 K416_F418delinsN mutation is a functional mutation in APA, since HAC15 cells, a human adrenal cell line, transfected with the mutant gene showed increased CYP11B2 expression and aldosterone production.