Adrenal Biopsy Research Articles

6634 Dual-Mediated PTH And Osteolytic Hypercalcemia In Metastatic Endometrial Carcinoma

Abstract Disclosure: C.K. Persaud: None. D.J. Beckman: None. Background: Hypercalcemia of malignancy occurs in 20-30% of advanced stage cancer patients. Etiologies include parathyroid hormone-related peptides (PTHrP)-mediated, osteolytic metastases-mediated, overproduction of 1,25-dihydroxycholecalciferol, and rarely parathyroid hormone (PTH)-mediated. PTH-mediated hypercalcemia of malignancy is rare and occurs through ectopic PTH production or primary hyperparathyroidism. We report a rare case of both PTH-mediated and osteolytic-mediated hypercalcemia in the setting of malignancy. Clinical Case: A 57-year-old female with history of endometrial cancer and multiple solid organ metastasis, presented with two weeks of progressive poor oral intake, nausea, emesis, and right flank pain. On hospital admission, she had a serum calcium of >20.1 mg/dL (8.6-10.2), PTH of 203 pg/mL (15.0-65.0), and creatinine of 1.7 mg/dL (0.7-1.2, baseline 1.3). Non-contrasted CT chest/abdomen/pelvis noted increased size of known right adrenal metastasis, new left adrenal metastasis, periaortic lymphadenopathy, and scattered nodules in the subcutaneous tissues of the abdomen and chest, however bone metastasis were not noted. Patient’s hypercalcemia was treated with zoledronic acid, calcitonin, cinacalcet, and eventually denosumab due to difficulty controlling hypercalcemia. Additional labs obtained during evaluation included ionized calcium >2.5 nmol/L (1.12-1.32), PTHrP <2.0 pmol/L (<2.0), 1,25-dihydroxycholecalciferol <10 pg/mL (21-65). Patient’s right adrenal metastasis biopsy one month prior to admission was subsequently stained for PTH and was negative. Technetium-99m sestamibi scan was obtained for localization of parathyroid adenoma or carcinoma. The scan did not localize, however did show numerous new lytic lesions in the skull, spine, sternum, rib cage, and right humerus. Patient’s hospital course was complicated by progressively worsening renal function, anemia, and thrombocytopenia. Patient was discharged to a long-term acute rehab facility off hypercalcemia treatment with a corrected calcium of 10.0 mg/dL. Conclusion: PTH-mediated hypercalcemia is a rare cause of hypercalcemia of malignancy. PTH levels are typically low or low-normal in osteolytic mediated hypercalcemia. Generally, if PTH is elevated in hypercalcemia, an alternate etiology is not evaluated. This case highlights a rare dual-mediated hypercalcemia of malignancy. The patient’s PTH level elevation was discordant with her high degree of hypercalcemia and prolonged treatment course. She was subsequently found to have widespread bone metastases contributing to hypercalcemia. While multiple etiologies of hypercalcemia can be rare, it is important to evaluate for all contributing causes, especially with additional clinical conditions, such as malignancy. Presentation: 6/3/2024

6701 Bilateral Adrenal Hematomas As Initial Presenting Sign Of Lung Adenocarcinoma

Abstract Disclosure: S. Gumireddy: None. R. Panta: None. Y. Shaikh: None. Introduction: Lung cancer typically presents with dyspnea, chest pain, hemoptysis, weight loss, or recurrent pulmonary infections. This case illustrates primary lung adenocarcinoma presenting with abdominal pain and bilateral adrenal hematomas. Clinical Case: 51-year-old male presented to the emergency department with left sided abdominal pain persisting for one week. CT abdomen showed a left suprarenal mass measuring 4.5 x 5 x 4.3cm indicating an acute adrenal hematoma. An MRI showed a partially solid left adrenal lesion that had hemorrhaged and a 1.3cm hemorrhagic nodule in the right adrenal gland. The patient remained hemodynamically stable. Hormonal evaluation done while patient was in the hospital showed renin level 0.417ng/mL/hr, aldosterone level <1.0ng/dL, epinephrine level 312pg/mL, norepinephrine level 333pg/mL, cortisol 11.3, ACTH level 13, plasma metanephrine levels 93pg/mL, plasma normetanephrine levels 111.7pg/mL, DHEA-S 151mcg/dL, potassium 4.1mmol/L, raising concern for hypersecretion. Patient was discharged with plans for outpatient follow-up but was readmitted in two months due to worsening abdominal pain. Repeat imaging revealed an increase in size of adrenal lesions, measuring 3.3 x 3.6 cm within the right adrenal gland and a 6.7 x 6.3 cm heterogeneously enhancing lesion within the left adrenal gland concerning for metastatic disease to bilateral adrenals and to the ileum causing small bowel obstruction. Chest imaging showed a 2.2cm irregular right apical nodule. Subsequent hormonal evaluation showed normalization of plasma metanephrine levels at 15.8pg/mL, renin level 3.314ng/mL/hr, aldosterone level 4.3ng/dL, deoxycorticosterone level 4.8ng/dL, androstenedione level 40ng/dL, plasma normetanephrines 146.4pg/mL. The patient underwent left adrenal gland mass biopsy which showed metastatic poorly differentiated adenocarcinoma of pulmonary origin. Tumor cells were positive for keratin cocktail, TTF1 and synaptophysin supporting the morphologic interpretation. Patient is currently receiving treatment with carboplatin, pemetrexed, and pembrolizumab. Conclusion: Adrenal masses can be unilateral (85%) or bilateral (15%). While most unilateral adrenal masses are benign and nonfunctional, bilateral adrenal masses are likely to be metastatic disease, hemorrhage, infiltrative disease, congenital adrenal hyperplasia, macronodular Cushing’s syndrome, or bilateral cortical adenomas. Although uncommon, our case highlights the importance of considering metastatic disease to the adrenal glands as a potential initial manifestation of primary cancers. The most likely primary cancers to metastasize to the adrenal glands are breast, lung, colon, kidney, and esophagus. Presentation: 6/1/2024

Adrenal tumors in patients with neuroendocrine neoplasms

PurposeTo study the prevalence of primary adrenal tumors and adrenal metastases in patients with neuroendocrine neoplasms (NENs) and describe these in detail. NENs can be further divided into neuroendocrine tumor (NET) and neuroendocrine carcinoma (NEC).MethodsA review of medical files was conducted for all patients who underwent a 68Gallium-DOTATOC-PET/CT during 2010−2023 or adrenalectomy during 1999-2023 at the Karolinska University Hospital.ResultsIn total, 68Gallium-DOTATOC-PET/CT was performed on 1750 individuals with NEN, among whom 12 (0.69%) had adrenal tumors. Of these, 9 (0.51%) were NEN metastases. Out of 1072 adrenalectomies, 4 (0.37%) showed evidence of NEN metastases. Thus, 16 patients with NEN exhibited adrenal tumors. The adrenal tumors were found on average 5 years after the NEN diagnosis and 19% of the adrenal tumors with simultaneous NEN were benign. Few had all adrenal hormones measured. None had an adrenal insufficiency nor an adrenal biopsy. Another synchronous metastasis was found in 69% at the time of the adrenal tumor discovery. During the median 2-year follow-up, 38% of the subjects had deceased (with the exclusion of individuals presenting supposedly benign adrenal tumors 31%) all due to tumor complications. A comparison between individuals identified through 68Gallium-DOTATOC-PET/CT and those who underwent adrenalectomy revealed a higher prevalence of NETs in the former group and NECs in the latter group.ConclusionAdrenal primary tumors and adrenal metastases are infrequent occurrences in patients with NEN. Most cases involved the presence of NEN metastasis upon the initial discovery of adrenal tumors. The overall prognosis was found to be favorable.

Clinical indications, safety, and effectiveness of percutaneous image-guided adrenal mass biopsy: an 8-year retrospective analysis in 160 patients.

To assess indications, safety, and effectiveness of percutaneous adrenal mass biopsy in contemporary practice. This institutional review board-approved, retrospective study included all patients undergoing percutaneous image-guided adrenal mass biopsies at an academic health system from January 6, 2015, to January 6, 2023. Patient demographics, biopsy indications, mass size, laboratory data, pathology results, and complications were recorded. Final diagnoses were based on pathology or ≥ 1 year of imaging follow-up when biopsy specimens did not yield malignant tissue. Test performance calculations excluded repeat biopsies. Continuous variables were compared with Student's t test, dichotomous variables with chi-squared test. A total of 160 patients underwent 186 biopsies. Biopsies were indicated to diagnose metastatic disease (139/186; 74.7%), for oncologic research only (27/186; 14.5%), diagnose metastatic disease and oncologic research (15/186; 8%), and diagnose an incidental adrenal mass (5/186; 2.7%). Biopsy specimens were diagnostic in 154 patients (96.3%) and non-diagnostic in 6 (3.8%). Diagnostic biopsies yielded malignant tissue (n = 136), benign adrenal tissue (n = 12), and benign adrenal neoplasms (n = 6) with sensitivity = 98.6% (136/138), specificity = 100% (16/16), positive predictive value = 100% (136/136), and negative predictive value = 88.9% (16/18). Adverse events followed 11/186 procedures (5.9%) and most minor (7/11, 63.6%). The adverse event rate was similar whether tissue was obtained for clinical or research purposes (10/144; 6.9% vs. 1/42; 2.4%, p = 0.27), despite more specimens obtained for research (5.8 vs. 3.7, p < 0.001). Percutaneous adrenal mass biopsy is safe, accurate, and utilized almost exclusively to diagnose metastatic disease or for oncologic research. The negative predictive value is high when diagnostic tissue samples are obtained. Obtaining specimens for research does not increase adverse event risk.

An Unusual Cause of Bilateral Adrenal Incidentaloma: A Case Report of Primary Adrenal Lymphoma

Primary adrenal lymphoma (PAL) is an extremely rare among the causes of adrenal incidentaloma. Most were diagnosed with adrenal insufficiency and B symptoms (unexplained weight loss, night sweats, fever). This article presented a 57-year-old woman who was investigated for bilateral adrenal masses found incidentally on computed tomography (CT). Physical examination and laboratory tests revealed no evidence of adrenal insufficiency or B symptoms. Only 24-hour urinary metanephrine and normetanephrine excretion were increased. Tumour F-18 fluorodeoxyglucose (FDG) positron emission tomography (PET/CT) scan showed the greatest dimension was 14 cm in the left adrenal mass and the maximum standardized uptake value (SUV max) was 26.1 (relative to mean SUV in the normal liver parenchyma, which was 2). An adrenal biopsy was performed after taking adequate precautions against the possibility of a catecholamine crisis. Histopathology revealed high-grade B-cell lymphoma. Bone marrow involvement and brain metastasis were not observed. She received the R-EPOCH (rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) regimen and intrathecal methotrexate therapy as central nervous system prophylaxis. The patient responded well to treatment, and close clinical follow-up continues. PAL should always be considered when a bilateral adrenal mass is detected.

FRI228 Primary Adrenal T-cell Lymphoma: A Case Report

Abstract Disclosure: H. Tabassum: None. S. Xu: None. A. Manavalan: None. Introduction: Primary T-cell adrenal lymphoma (PAL) is exceedingly rare. Adrenal insufficiency (AI) is common in these cases. Here we describe a patient with bilateral, large, T-cell PAL with rapid progression, without adrenal insufficiency. Clinical Case: A 62-year-old male, with coronary artery disease and recent coronary intervention, hyperlipidemia and hypertension presented to the hospital with abdominal pain, fever, night sweats and weight loss for a month. Work up revealed a 5.8 cm left adrenal mass with 14% absolute washout, measuring 34 Hounsfield Units (HU) pre-contrast, 68 HU postcontrast, and 63 HU on delayed images, and a 5.2 cm right adrenal mass with 42% absolute washout, measuring 35 HU pre-contrast, 81 HU postcontrast, and 62 HU on the delayed images with no other suspicious lesions or lymphadenopathy. The patient demonstrated no clinical symptoms or signs of adrenal hormone excess or deficiency. Levels of renin, aldosterone, serum cortisol, ACTH, 24-hour urine free cortisol, serum metanephrines, and thyroid function were normal. Additional tests to rule out adrenal cortical carcinoma (ACC) revealed normal levels of androstenedione, 17-hydroxyprogesterone, dehydroepiandrosterone sulfate, and estradiol. Persistent non-significant elevation of 11-deoxycortisol was observed at 51 ng/dL and 63ng/dL (Ref &amp;lt; 42 ng/dL). Adrenal biopsy was suggested but deferred due to the increased risk of hemorrhage given recent coronary revascularization and use of dual antiplatelet therapy. Follow up imaging in a few weeks revealed diffuse, retroperitoneal lymphadenopathy with unchanged adrenal masses. A lymph node biopsy confirmed a diagnosis of peripheral T-cell lymphoma. The left adrenal gland demonstrated an uptake SUV of 22.7 and the right, 19.17 on FDG PET. The patient was initiated on chemotherapy with etoposide, prednisone, vincristine sulfate, cyclophosphamide, and doxorubicin. Imaging three months later revealed a marked decrease in FDG activity SUV 6.98 g/mL on the left and SUV up to 3.32 g/mL on the right. Adrenal function remained intact on follow up. Conclusion: Peripheral T-cell lymphoma with adrenal involvement is an extremely rare and aggressive disease with rapid progression. Mostly seen in males over the age of 50, it may present with abdominal pain, B symptoms or both. While ACC is usually unilateral, lymphoma and metastatic disease may present bilaterally, however, other imaging features can be similar. AI is expected when 90% of the adrenal glands are destroyed by tumor infiltration, however, this does not always correlate with tumor size. In our patient, AI was not observed on presentation or follow up. As adrenal insufficiency can occur at any time in the disease course and may mimic symptoms of cancer or chemotherapy, the clinician should always be vigilant for its development in these cases. Presentation: Friday, June 16, 2023

FRI245 Non-classic CAH Presenting As Bilateral Adrenal Incidentalomas

Abstract Disclosure: Y.A. Chin: None. T. Puar: None. T. King: None. Introduction: Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is an autosomal recessive disorder. Non-classic CAH usually presents in late childhood or asymptomatic may remain undiagnosed. Adrenal incidentalomas have been reported as a presentation of CAH. Clinical Case: A 56-year-old man presented with a 4-day history of epigastric pain associated with chest discomfort and diaphoresis. There was no postural giddiness, vomiting, paroxysm or constitutional symptoms. He was a smoker, his parents were non-consanguineous and he had fathered 2 children. He was tachycardic at 114 bpm, but normotensive (129/89 mmHg) with no postural hypotension and no abnormal pigmentation. He had evidence of heart failure with bibasal crepitations and pedal oedema. Secondary sexual characteristics were normal and testicular volume was 15 mls bilaterally with no palpable mass. CT abdomen showed grossly enlarged bilateral adrenal glands with heterogenous nodular appearance with several focal hypodense cystic components, with absolute washout &amp;lt;60%. Biochemistry was consistent with primary adrenal insufficiency (PAI); 8am serum cortisol 188 nmol/L, with peak cortisol post 250mcg synacthen of 195 nmol/L. Serum ACTH was elevated at 254.6 ng/L, aldosterone and renin were normal and 24hr urinary normetanephrine was mildly elevated at 1982 nmol/d (NR 600-1900 nmol/d). Echocardiography revealed severe global hypokinesia (EF 20%) but myocardial perfusion imaging was negative for ischemia. In view of suspicion for tuberculosis, malignancy or infiltrative disease, CT guided adrenal biopsy was performed. Histology showed hyperplastic adrenal cortex with lipofuscin pigments, focal lymphohistiocytic infiltrates. No lymphoplasmacytic infiltrate with IgG 4 plasma cells, granuloma, malignant cells or amyloid deposits were seen. Serum 17-OHP level later came back elevated at 39.7 nmol/L (NR 1.8-10.4 nmol/L). DNA sequencing of CYP21 revealed compound heterozygous mutations in c.292+1G&amp;gt;A and c.518T&amp;gt;A, both of which have been reported to be causative of CAH. He was transitioned from stress dose to physiological glucocorticoid replacement and ACTH normalised on subsequent outpatient visits. Follow up imaging after 6 months and 2 1/2 years revealed no change in size or morphology of the adrenal glands. Follow up echocardiography showed no improvement in ejection fraction despite intensive heart failure treatment. Conclusion: Nodular adrenal hyperplasia is well described among CAH individuals, especially in those with inadequate glucocorticoid replacement and chronically elevated ACTH levels. There was no change in size of the adrenal lesions despite adequate glucocorticoid replacement. Improvement of cardiac function with glucocorticoid replacement has been reported in paediatric patients with CAH, but our case did not show any improvement on serial echocardiography. Presentation: Friday, June 16, 2023

Isolated Castrate-resistant Prostate Cancer Metastasis to Both Adrenal Glands Detected on 68Ga PSMA PET/CT.

A 61-year-old male patient, who had undergone radical prostatectomy, underwent 68Ga labeled prostate-specific membrane antigen (PSMA) positron emission tomography/computerized tomography (PET/CT) for evaluation of suspected biochemical recurrence of prostate cancer (PCa). PET/CT scan showed increased 68Ga PSMA expressions in hypodense mass lesions in both adrenal gland localizations. An adrenal gland tru-cut biopsy was performed for the right side, which showed poor-differentiated carcinoma metastases associated with the patient's high-grade PCa. As far as we could determine based on an extensive literature search, this is the second case in which isolated adrenal metastasis was detected by 68Ga PSMA PET/CT study in a patient with PCa.

Acute heart failure with incidentally found cystic adrenal mass.

Pheochromocytomas are rare adrenal tumors characterized by excessive catecholamine secretion. Symptoms and signs associated with pheochromocytomas are usually intermittent and chronic but can rarely develop into life-threatening crises. We describe a case of acute severe congestive heart failure in a previously healthy female, who recovered rapidly (4 days after admission) with acute medical therapy. The etiology on evaluation was a spontaneous bleed in a previously undiagnosed pheochromocytoma, resulting in a pheochromocytoma crisis and transient stress cardiomyopathy, followed by quick recovery of cardiac function. Our aim is to describe pheochromocytoma as a rare cause of stress cardiomyopathy. We discuss the evaluation of pheochromocytoma during critical illness and triggers/treatment strategies for pheochromocytoma crises. Hemorrhage in a pheochromocytoma can result in a pheochromocytoma crisis, with sudden release of excess catecholamines resulting in multisystem organ dysfunction and high mortality. Acute decompensated heart failure can be a rare presentation of pheochromocytoma, in a patient with no cardiac risk factors. Measurement of metanephrines in acutely stressful clinical situations can have considerable overlap with the biochemical picture of pheochromocytoma. Early imaging studies may help with the differential diagnosis. Pheochromocytoma should be ruled out before performing an adrenal biopsy. Emergent adrenalectomy in pheochromocytoma crisis results in high mortality. Medical management of the acute crisis followed by elective adrenalectomy after alpha-blockade results in better outcomes.

Deep learning approach for differentiating indeterminate adrenal masses using CT imaging.

Distinguishing stage 1-2 adrenocortical carcinoma (ACC) and large, lipid poor adrenal adenoma (LPAA) via imaging is challenging due to overlapping imaging characteristics. This study investigated the ability of deep learning to distinguish ACC and LPAA on single time-point CT images. Retrospective cohort study from 1994 to 2022. Imaging studies of patients with adrenal masses who had available adequate CT studies and histology as the reference standard by method of adrenal biopsy and/or adrenalectomy were included as well as four patients with LPAA determined by stability or regression on follow-up imaging. Forty-eight (48) subjects with pathology-proven, stage 1-2 ACC and 43 subjects with adrenal adenoma >3 cm in size demonstrating a mean non-contrast CT attenuation >20 Hounsfield Units centrally were included. We used annotated single time-point contrast-enhanced CT images of these adrenal masses as input to a 3D Densenet121 model for classifying as ACC or LPAA with five-fold cross-validation. For each fold, two checkpoints were reported, highest accuracy with highest sensitivity (accuracy focused) and highest sensitivity with the highest accuracy (sensitivity focused). We trained a deep learning model (3D Densenet121) to predict ACC versus LPAA. The sensitivity-focused model achieved mean accuracy: 87.2% and mean sensitivity: 100%. The accuracy-focused model achieved mean accuracy: 91% and mean sensitivity: 96%. Deep learning demonstrates promising results distinguishing between ACC and large LPAA using single time-point CT images. Before being widely adopted in clinical practice, multicentric and external validation are needed.

An unusual cause of adrenal insufficiency with elevation of 17-hydroxyprogesterone: case report

BackgroundWe present an intriguing case of primary adrenal lymphoma, with associated primary adrenal insufficiency (PAI), in a patient presenting a transitory partial 21-hydroxylase deficiency during the active phase of the adrenal disease.Case presentationAn 85-years old woman was referred because of worsening asthenia, lumbar pain, generalized myalgia and arthralgia. During investigations a computed tomography (CT) scan evidenced two large bilateral adrenal masses, highly suspicious for primary adrenal tumor. The hormonal assessment revealed very low levels of morning plasma cortisol and 24-h urinary cortisol, elevated ACTH levels with low plasma concentration of aldosterone, pointing to the diagnosis of PAI. After diagnosis of PAI our patient started glucocorticoid and mineralcorticoid replacement therapy with clinical benefit. In order to further characterize the adrenal lesions, adrenal biopsy, was performed. The histology revealed a high grade non-Hodgkin lymphoma with an immunophenotype consistent with intermediate aspects between diffuse large B-cell and Burkitt lymphoma, with a high proliferation index (KI-67 > 90%). The patient received chemotherapy with epirubicin, vincristine, cyclophosphamide, and rituximab, associated with methylprednisolone that resulted in a complete clinical and radiological remission within one year. After 2 years from the diagnosis and a total of 6 cycles of rituximab, the patient was in good clinical condition and was taking only the replacement therapy for PAI. The patient initially presented also a slight increase of 17-hydroxyprogesterone (17-OHP) for age that normalize after resolution of lymphoproliferative disease.ConclusionsIn the presence of bilateral adrenal disease and/or in the presence of signs and symptoms of PAI clinicians must exclude the presence of PAL. The evidence of elevated ACTH-stimulated 17-OHP levels also in patients with other adrenal masses, together with the detection of elevated basal 17-OHP levels in our patient make it more plausible, in our view, an effect of the lesion on the “healthy” adrenal tissue residue than a direct secretory activity by the adrenal tumor.

Abstract #1406407: A Rare Case of Pituitary Metastasis from Esophageal Adenocarcinoma

Metastases to the pituitary gland are uncommon. Metastatic lesions represent < 1 % of sellar masses (1). Breast and lungs are the most common primary tumors associated with pituitary metastasis, representing nearly 60% of cases (1). Esophageal adenocarcinoma is a very rare cause of pituitary metastasis and only a few cases have been reported. We present a very rare case of a patient with pituitary metastasis from esophageal adenocarcinoma. 49-year-old male presented with a 2-month history of progressive fatigue, chills, lightheadedness, poor appetite, headaches, and decreased peripheral vision. One month before admission he was diagnosed with an invasive moderately differentiated esophageal adenocarcinoma. Head CT showed a heterogeneous hyperdense pituitary mass. Pituitary MRI confirmed a 2.13 x 0.9 cm heterogeneously enhancing, lobulated sellar mass with suprasellar extension and at least 2 sub-centimeters areas of enhancement within the right frontal lobe. CT chest, abdomen, and pelvis with splenomegaly, multiple lesions in the lungs, liver, and bilateral adrenal glands, concerning for metastases. Laboratories showed a cortisol level of 1.2 mcg/dL, TSH 1.074 (0.46-4.7 uIU/mL), free T4 < 0.42 (0.7-1.30 ng/dL). Prolactin 3.9 (2.5-17.0 ng/mL)), LH < 0.1 (0.8-7.6 mIU/mL), FSH 0.9 (0.7-11.1 mIU/mL), total testosterone < 4.33 (240-871 ng/dL), and IGF-1 76 (53.3-215 ng/mL). No evidence of diabetes insipidus. The patient was started on levothyroxine 150 mcg daily and hydrocortisone 15 mg in the morning and 5 mg in the afternoon. Ophthalmologic evaluation showed multiple elevated retinal lesions in both eyes, concerning for metastatic implants. No acute intervention per neurosurgery. After discharge, an adrenal biopsy confirmed metastatic esophageal adenocarcinoma. The patient underwent whole brain radiotherapy (WBRT) and was started on FOLFOX, trastuzumab, pembrolizumab, and zometa. MRI 3 months later showed decrease in size of enhancing lesion most suspicious for metastasis within the right frontal lobe and sella turcica. Pituitary metastases are usually diagnosed in the 6th and 7th decade of life with widespread metastatic disease. Esophageal adenocarcinoma is a rare cause of pituitary metastasis with few cases reported. Patients may be asymptomatic, but others may present with diabetes insipidus, visual disturbances, headaches, and panhypopituitarism (2). Our patient presented at a younger age with symptoms indicative of pituitary failure. Although these may be nonspecific, it is important to maintain awareness of them so that testing and treatment can be performed in a timely fashion.

The importance of CT-guided adrenal biopsy in an oncological patient: a case report

The importance of CT-guided adrenal biopsy in an oncological patient: a case report

Etiology, clinical characteristics and mortality among Indian patients with Addison's disease.

The etiology, presentation and mortality of patients with primary adrenal insufficiency (PAI) in developing countries may differ from economically developed nations. However, information in this regard is scanty. The aim of this study was to determine the etiology and compare the clinical characteristics and mortality in infectious and autoimmune causes of PAI in Indian patients. All eligible (n = 89) patients (ages 15-83 years) diagnosed with PAI between 2006 and 2019 were studied. Patients were followed for a median duration of 5.9 (range 0.1-15.7) years. Eighty-six subjects underwent an abdominal computerized tomography scan or ultrasonography, and adrenal biopsy was performed in 60 patients. The most frequent etiologies of PAI were adrenal histoplasmosis (AH, 45%), adrenal tuberculosis (AT, 15%), autoimmunity (AI, 25%) and primary lymphoma (6%). Forty-two percent of patients presented with an acute adrenal crisis. AH and AT could not be differentiated on the basis of clinical features, except for a greater frequency of hepatomegaly-splenomegaly and type 2 diabetes mellitus (63% vs 15%, P < 0.01) in the former. Patients with an autoimmune etiology had a higher frequency of 21-hydroxylase antibodies (41% vs 3%) and autoimmune thyroid disease (46% vs 5%) vs those with infectious etiologies. Mortality was significantly higher in AH (45%) compared with AT (8%) or AI (5%) (P = 0.001). Causes of death included adrenal crises, progressive AH and unexplained acute events occurring at home. In conclusion, infections, especially AH, were the most frequent cause of PAI in north India. Despite appropriate therapy, AH had very high mortality as compared with AT and AI.

Neuroblastoma Presentation With Multiple Cranial Nerve Involvement

Objective NA. Background We report a case of neuroblastoma, a pediatric neuroendocrine tumor of the sympathetic nervous system, in a 3-year-old female with multiple cranial nerve involvement. Design/Methods A 3-year-old afebrile, lethargic female presented with bilateral eyelid droop, right head tilt, slurred speech, gagging, abnormal walking and no bowel movement. Neurological examination noted bilateral ptosis, dysarthria, left tongue deviation, proximal weakness in upper and lower extremities, areflexia in biceps and patellar tendons, dysmetria, and wide-based gait. MRI of the brain showed heterogeneous appearance of the clivus and MRI of the spine showed right adrenal mass and heterogeneous enhancement of multiple vertebrae, suggesting possible metastatic disease. Serum and cerebrospinal studies were unremarkable. Patient was treated with intravenous methylprednisolone and plasmapheresis for suspected paraneoplastic syndrome; however, she continued to clinically progress. Adrenal mass biopsy results and elevated urine VMA and HVA levels were consistent with the diagnosis of neuroblastoma. Nuclear imaging and meta-iodobenzylguanidine scan were negative. Paraneoplastic panels and Lambert Eaton panel were negative for autoantibodies. Total resection of the abdominal mass and right adrenal gland with continued steroid taper, resulted in reported near total symptom resolution. Results Our patient presented with antibody negative paraneoplastic polyneuropathy due to neuroblastoma. Cranial nerve involvement in neuroblastoma can result from tumor involvement of the sympathetic chain, paraneoplastic syndromes, or metastasis to the skull. Our patient's initial imaging suggested potential metastatic or inflammatory involvement of the clivus, suspected of causing her cranial nerve symptoms. However, nuclear studies were negative for metastatic disease and patient's symptoms resolved after resection of the main mass suggesting a paraneoplastic etiology. Although her paraneoplastic panel and Lambert-Eaton panel were negative for common associated antibodies, it is likely an unmeasured autoantibody, cytokine, hormones, or peptide was at play. Conclusions Neuroblastoma should be considered as a differential for a neurological presentation involving multiple cranial nerves in a child.

ODP016 A case of adrenal incidentaloma in a patient with latent tuberculosis

Abstract An adrenal incidentaloma can be either a nonfunctioning adrenocortical adenoma or may cause disease, such as adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma or metastasis. However, less commonly, it can be a manifestation of infection such as tuberculosis. The case is a 40-year-old male who emigrated from China two years ago, referred to the endocrine clinic after he was found to have a 3 cm right adrenal macroadenoma on MRCP after hospitalization for acute cholecystitis. The patient was also found to have positive Quantiferon, but CXR was clear. He was started on isoniazid therapy for latent tuberculosis and was later switched to rifampin postoperatively. One month after hospital discharge, he was evaluated by Endocrinology. Vital signs, including BP were normal with no symptoms of dizziness or orthostatic hypotension. He had no symptoms of adrenal insufficiency or excess. Electrolytes, renal, liver function tests, and CBC were within normal. Albumin was 4.3g/dL(RR 3.4-5). Fasting 8 AM labs: ACTH 9 pg/mL (RR 0-47), cortisol 10.10 ug/dL (RR 5.3-22.5), DHEA S 130 mcg/dL (RR 80-560), plasma metanephrines and normetanephrines and midnight salivary cortisol were normal, aldosterone 10 ng/dL, renin 1.49 ng/mL/h with ARR of 6.7. Adrenal CT w/wo contrast showed right adrenal mass with absolute washout 78% and relative washout 53% consistent with adenoma, as well as bilateral subcentimeter lipid rich adenomas and marked mesenteric lymphadenopathy. Mycobacterium tuberculosis complex spreads to the adrenal glands hematogenously. Clinical manifestations of adrenal insufficiency in tuberculosis may take years to take effect, after 90% of gland destruction occurs. The majority of patients with active or recently acquired disease (&amp;lt;2 years) have bilateral adrenal enlargement, while calcification and atrophy is found with remote infection or inactive disease. The adrenals can be enlarged in patients with pulmonary tuberculosis even without active involvement of the glands solely due to stress and inflammation. The hypothalamic-pituitary-adrenal (HPA) axis can be activated or even under-activated in active pulmonary tuberculosis. Cortisol levels return to baseline after anti-tuberculous treatment. Rarely tuberculosis can cause adrenal incidentaloma. Adrenal biopsy is not necessary for primary adrenal insufficiency with bilateral adrenal enlargement in a patient with proven extra-adrenal tuberculosis. However, about 12% of patients with adrenal tuberculosis have no evidence of active extra-adrenal tuberculosis. Adrenal biopsy was refused but is generally necessary in these patients to prove adrenal involvement by tuberculosis. Our case highlights the fact that tuberculosis should be considered in the differential of adrenal masses. Reference: Fassnacht M, Arlt W, Bancos I, et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2016 Aug;175(2): G1-G34. doi: 10.1530/EJE-16-0467. PMID: 27390021. Presentation: No date and time listed

ODP631 Infectious Disease Causing Damage Of The Adrenal Glands, A Life Threating Event

Abstract Introduction Primary Adrenal Insufficiency (PAI) is not a common cause of adrenal insufficiency. The most common cause is Addison disease that is an autoimmune condition. Other causes include hemorrhage, infiltration, infections, necrosis and metastasis. We present a rare case of primary adrenal insufficiency caused by disseminated histoplasmosis. Case presentation 87 yo male from the Midwest with a history of HTN, HLD, CAD s/p CABG was admitted to our hospital for fatigue and 15 lbs weight loss. In the ED, he was found to have persistent hypoglycemia (BG in the 50s mg/dl after several vials of D50%). He was admitted to ICU for continuous D10% infusion. His laboratory showed Na 137mmol/L, K 3.7mmol/L. Further work up for hypoglycemia showed BG of 53 mg/dl with C-peptide of 0.92ng/ml and Insulin &amp;lt;1uIU/ml. Due to the concern of non-insulin dependent hypoglycemia, AM cortisol was done which was low7ug/dl. Immediately, he was started on high dose hydrocortisone. Head CT was negative for pituitary lesions. CT showed lung nodules and bilateral peripherally calcified adrenal lesions 3.6 &amp; 2.6 cm homogenous, well defined borders (HU 20-30). Adrenal hormones work up showed normal metanephrines, Aldosterone &amp;lt;1ng/dl and PRA 1.35ng/ml/h (0.25-5.82). ACTH stim test revealed cortisol level of 9. 0 ug/dl, 9.4 ug/dl, and 9.2 ug/dl at baseline, 30min and 1hr respectively with a baseline ACTH of 539. Lung biopsy showed necrotizing granuloma and fungal spores compatible with histoplasmosis. He was started on amphotericin then transition to itraconazole for 1 year. Additionally, the dose of HC was decrease and Fludrocortisone 0.1mg daily was added. He refused adrenal biopsy at that point and wanted to continue current treatment with a plan to repeat imaging in 3 months and possible adrenal biopsy depending on CT results. On follow-up, patient regained weight and hypoglycemia resolved. Repeated adrenal CT showed shrinkage of the adrenal nodules bilateral. He was continued on PAI replacement. Discussion Histoplasmosis is a fungal infection caused by (H. capsulatum), found in soil contaminated with bird or bat droppings. Infection usually limited to respiratory disease in immunocompetent host but may cause disseminated infection in immunocompromised patient which can involve any organ system. Less commonly, disseminated type may occur in an immunocompetent host with the adrenal gland being the most common infected organ. Treatment involves systemic antifungal therapy; ketoconazole or itraconazole for mild-moderate forms and amphotericin B for 12-18 months for severe ones. Relapse rate is high; ∼ 7.5% and most patients require long-term glucocorticoid replacement. Because unrecognized disseminated histoplasmosis accompanying PAI usually results in a high mortality rate, considerable suspicion and appropriate investigations are necessary for correct diagnosis of this rare but treatable disease. Presentation: No date and time listed

ODP048 Large Bilateral Adrenal Lesions Abutting Right Hemidiaphragm in a Man from Texas

Abstract Sporadic cases of disseminated histoplasmosis (DHI) have been reported in several regions of the US, mainly inimmunocompromised patients where involvement of the adrenal gland, either unilaterally or bilaterally, is often being the only demonstrable site of active fungal disease in the setting of nonspecific clinical complaints. While DHI is not immediately life-threatening, adrenal involvement might be the most concerning complications due to potential risk of the gland destruction by the expanding infection or its treatment; it is considered that adrenal insufficiency is one of the most common causes of death in patients with DHI. We present a case of 78-year-old immunocompetent gentleman with history of hypertension, ischemic heart disease, atrial fibrillation and 30 pack-years of cigarette smoking who presented with low-grade fever, anorexia and 30 lbs weight loss. As a part of diagnostic evaluation, CT abdomen was performed and bilateral adrenal lesions were reported: right adrenal mass measured 4.6×3.4 cm, and the left adrenal mass 4.1×3.2 cm in maximal axial dimensions. Of note, the right adrenal mass abutted the crus of the right hemidiaphragm and undersurface of the liver. Hormonal evaluation was performed and did not show any excess secretions. GI consult was called for evaluation of adrenal gland access and biopsy: endoscopic ultrasound guided FNA biopsy access was performed and revealed findings compatible with adrenal histoplasmosis and no malignant cells. ID consult was sought: evaluation and prompt start of antifungal therapy were undertaken: IV Amphotericin B, followed by oral Itraconazole for total of 12 months. Patient has remained stable during initial evaluation: due to his age and comorbid conditions, he was admitted to the hospital during which Cosyntropin stimulation test was performed: results have revealed 12.9 at baseline and 17.6 after stimulation. Interdisciplinary discussion was held and resulted in decision to start steroid replacement therapy due to rather mild response to stimulation and significant risk of collateral damage to the remaining adrenal tissue during the antifungal therapy. Patient has responded to therapy well, though had some worsening of his cardiac function which required optimization of diuretic therapy. Patient's vital signs have remained stable, he has reported improved appetite and started to regain some of the lost weight; his electrolytes remained stable. Six-month interval imaging has shown reduction of the size of the adrenal gland sizes and no evidence of gland hemorrhage; notably, adrenal gland improvement has generally paralleled imaging improvement in other involved organs. Steroid therapy is being continued for total of 12 months. DHI still remains a serious problem in certain regions of the US and the world and has to remain an essential part of the differential diagnosis of the adrenal lesions allowing earlier treatment, gland preservation and avoidance of life-threatening complications. Presentation: No date and time listed

LBMON138 Leg Tumeo: Is It Neuroendocrine Tumor?

Abstract Introduction Neuroendocrine tumors (NETs) are heterogenous group of epithelial neoplasms often known to produce and secrete peptides and bioamines. The presentation varies from symptomatic functional to non-functional tumors. The functional tumors cause symptoms based on the substance secreted: corticotropin, gastrin, glucagon, insulin, norepinephrine, serotonin, somatostatin, etc. The prognosis ranges from well-differentiated tumors being indolent while the poorly differentiated forms are aggressive. We present a case with an atypical presentation of NET which was noted to secrete an atypical hormone, cortisol. Case Presentation A 55-year-old Caucasian man presented with bilateral lower extremity grade 3 pitting edema for past 2 weeks. His past medical history was significant for hypertension. Lower extremity doppler showed no evidence of deep vein thrombosis. Serum potassium was critically low at 1.9mmol/L. Computed tomography of abdomen and pelvis showed a large mass involving the right hepatic lobe, upper pole of right kidney and hypoattenuated lesions in left hepatic lobe. The intrahepatic inferior vena cava appeared to be severely compressed by the mass. MRI abdomen was suggestive of hepatocellular cancer but alfaprotein levels was normal. Biochemical testing for evaluation of hormonal hypersecretion showed elevated cortisol levels (57.2mcg/dl) and markedly elevated dehydroepiandrosterone sulfate (2641mcg/dl). Serum adrenocorticotrophic hormone and aldosterone levels were within normal limits. Low dose dexamethasone suppression test showed no decline in serum cortisol level reflecting adrenal origin. Urinary evaluation showed elevated cortisol but normal metanephrine and catecholamine levels. With concerns for adrenal carcinoma, right adrenal mass biopsy was performed. The biopsy showed poorly differentiated malignant neoplasm with neuroendocrine features. Tumor cells were positive for synaptophysin (a marker for neuroendocrine differentiation) and CD 138. Soon after, he progressed to multisystem organ failure, tumor lysis syndrome and resorted to comfort care. The patient passed away within a week of diagnosis. Conclusion NETs usually originate in gastrointestinal tract, pancreas, adrenal, lung and rarely genitourinary tract. The incidence of NETs is on the rise with increased detection by imaging. Adrenal NETs, well-known as pheochromocytoma are mostly benign but small cell adrenal neuroendocrine carcinomas are rare and have a grave prognosis. Flushing, diarrhea are the commonest presentations (75-85%) for gastrointestinal NETs and symptoms are based on the substance secreted. This case highlights a case of adrenal neuroendocrine cancer presenting with lower extremity swelling and secreting an uncommon hormone, cortisol. Increasing awareness among the medical fraternity will help with early diagnosis and better outcomes. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

DISSEMINATED BLASTOMYCOSIS PRESENTED WITH ADRENAL MASS

DISSEMINATED BLASTOMYCOSIS PRESENTED WITH ADRENAL MASS