Left Adrenal Gland Research Articles

FRI601 Unique Considerations For Workup And Management Of An 11 Year Old Male With Concern For A Neuroendocrine Tumor

Abstract Disclosure: N.C. Gunaratne: None. D. Deplewski: None. Background: Pheochromocytoma/paragangliomas are very rare in children. In patients who are undergoing work up for paragangliomas and pheochromocytomas, special considerations need to be made to distinguish neuroendocrine tumor findings from medication and other symptoms. Clinical Case: An 11 yo male with past medical history of migraines presented with new onset seizure, hypertension and tachycardia. Initial exam was remarkable for severe lower back pain, which eventually progressed to generalized pain. His tachycardia and hypertension were initially thought to be related to his pain, but were not improved with escalating pain and intermittent anti-hypertensive medications – leading to admission to the intensive care unit. He was started on lisinopril and metoprolol, but continued to have persistent tachycardia and hypertension.Initial work up included an echocardiogram revealing concentric left ventricular hypertrophy suggesting long-standing hypertension, MRI brain significant for Posterior Reversible Encephalopathy Syndrome, MRI abdomen and spine with no significant findings, and elevated urine metanephrines. Urine and serum catecholamines and serum metanephrines were also elevated, but not at levels typical of neuroendocrine tumors. Due to concern for possible pheochromocytoma/paraganglioma, patient was started on alpha-blocker phenoxybenzamine, and had marked improvement in hypertension. Patient was also started on scheduled gabapentin one day after initiation of phenoxybenzamine and noted to have some improvement in pain – making cause of acute improvement in blood pressure less clear. Patient underwent Ga-68 DOTATATE imaging that was significant for “mild to moderately increased activity within the left adrenal gland,” but no distinct lesion was appreciated on scan. After discussion with endocrine surgery, plan was made to monitor the patient in outpatient setting with serial plasma free metanephrines and chromogranin A while continuing phenoxybenzamine. Conclusion: Pheochromocytoma/paragangliomas are very rare in children. In patients who are undergoing work up for paragangliomas and pheochromocytomas, special considerations need to be made when patient has concomitant pain or is on beta-blockers. Ability to interpret urine/plasma metanephrines and catecholamine levels while on beta-blockers and other pharmacologic agents is difficult due to known iatrogenic alterations of these levels. Management for patients with elevated metanephrines without a localized neuroendocrine tumor can consist of pharmacologic intervention with alpha-blockers and close monitoring with serial labs. Presentation: Friday, June 16, 2023

SAT558 Papillary Thyroid Carcinoma With Anaplastic Transformation in a Filipino Male Treated With Radioactive Iodine and Radiotherapy: A Case Report

Abstract Disclosure: A.A. Apa-ap: None. P. Abarquez: None. M.B. Sedurante: None. A. Ongbit: None. P. Carlos Miguel: None. F. Acenas: None. J. Gacula: None. V. Lee: None. S. Estreller: None. INTRODUCTION. Papillary thyroid carcinoma (PTC) is an epithelial malignancy showing follicular differentiation and distinctive nuclear features, with a generally excellent prognosis. On the other hand, anaplastic thyroid cancer (ATC) is rare and highly lethal, with an incidence of only 0.7% in the Philippines. CASE DESCRIPTION. This paper describes a 43year old Filipino male with a 3-year history of anterior neck mass with an initial fine needle aspiration biopsy read as probable PTC. He underwent total thyroidectomy and bilateral radical lymph node dissection and histopathology revealed conventional PTC, solid variant with 5% focal anaplastic transformation. Metastatic workup revealed multiple sub-centimeter pulmonary nodules and a tiny hypodense nodule on left adrenal gland on CT scan. Bone scintigraphy showed increased tracer uptake in the posterior 10th rib and the sphenoid's greater wing. While the multidisciplinary team wanted to proceed with Radiation therapy (RT) first to address the more aggressive histology, this was not possible logistically and thus RAI was performed first. This slight deviation from guidelines was deemed acceptable by Nuclear Medicine due to the possible decrease in iodine absorption from post radiation fibrosis. After a dose of 150 mci, 4 weeks after surgery, patient then completed 33 fractions of RT. Surveillance revealed stable pulmonary nodules and adrenal mass but increasing TSH-stimulated thyroglobulin and antithyroglobulin. Patient underwent another cycle RAI and is currently stable on his 16th month from diagnosis, 15th month post initial RAI, and 13th month post-RT. DISCUSSION. In several case series, patients with minor anaplastic component had 1- and 2-year survival rates of 88% and 43%, respectively with a median survival of 17 months. The overall prognosis of patients with PTC with minor ATC component points to the value of active surveillance compared to more aggressive treatment modalities in the setting of conventional ATC. Presentation Date: Saturday, June 17, 2023

FRI267 Carney Complex From A Single Familycarney Complex From A Single Family Followed Up Over 20 Years With Different Clinical Pattern Evolution

Abstract Disclosure: A.W. Kuhn: None. D.S. de Melo: None. H.L. Charchar: None. B.M. Mariani: None. M.Y. Nishi: None. F.L. Ledesma: None. F.Y. Tanno: None. V. Srougi: None. J.L. Chambo: None. A. Latronico: None. B.B. Mendonca: None. M.Q. Almeida: None. M.C. Fragoso: None. Background: Carney complex (CNC) is a hereditary syndrome with autosomal dominant inheritance, composed of multiple neoplasms, such as benign and malignant, endocrine and nonendocrine tumors (cardiac, cutaneous and neural). The primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of Cushing syndrome is the most common endocrine neoplasia in CNC. The most frequent genetic cause is related with the presence of germline pathogenic allelic variants on PRKAR1A. Overall CNC penetrance among carriers of the PRKAR1A allelic variant is approximately 97.5%, however, families with long-term follow up is rarely described in literature. Clinical Case: A 17-year-old male, was referred to our Hospital in 2003 due to weight gain (≈10 Kg), BMI 27.27 Kg/m2, acne, occurrence of abdomen purplish striae (>1cm), facial plethora and arrest of pubertal development for last 2 years. He has had pigmented facial lesions since 9 years old. Laboratory revealed suppressed ACTH <18.0 pg/mL, failure to suppress cortisol levels after low dose dexamethasone test (DST-1mg): cortisol 29 µg/dL (normal range: <1.8 µg/dL), and total urinary cortisol (TUC), 2809 µg/24 h (normal range: 30-300 µg/24h). Abdominal CT scan showed normal right adrenal gland and a micronodule (0.8 cm) in the left adrenal gland. Bone densitometry showed an important loss of density. Owing to ACTH-independent Cushing's syndrome and adrenal with micronodule, the most plausible diagnostic hypothesis was PPNAD associated to CNC. He underwent bilateral laparoscopic adrenalectomy and the histological data confirmed PPNAD on both adrenal glands. He developed primary adrenal insufficiency and he maintains continuous replacement with gluco and mineralocorticoids. The genetic analysis identified a heterozygous germline nonsense pathogenic variant on PRKAR1A (p.Tyr21X) in exon 2. His relatives, such as father, two younger sisters, one paternal aunt and a female cousin carried the same nonsense variant. All of them also presented facial lentiginous but without Cushing features. In the first investigation, all his relatives showed possible autonomous cortisol secretion. In follow-up of almost 20 years, the index case and his family members did not develop other manifestations of CNC. Only his father developed metabolic syndrome, but all relatives maintained subclinical hypercortisolism due to possible autonomous cortisol secretion without any clinical repercussions. Clinical Lessons: Carney complex is a rare disease and the follow-up of familial cases over the years can show different clinical pattern despite the same germline pathogenic variant. Probably somatic molecular mechanisms as modulators could trigger specifically clinical manifestations. Presentation: Friday, June 16, 2023

FRI265 I123-MIBG Positive, CU64-Dotatate PET Negative Pheochromocytoma In A Patient With Bilateral Adrenal Incidentalomas

Abstract Disclosure: P. Pagadala: None. V. Patel: None. Bilateral incidental adrenal nodules represent 10-23% of all incidental adrenal nodules. Most are benign nonfunctioning adenomas however these can be catecholamine-secreting tumors that arise from chromaffin cells of the adrenal medulla called pheochromocytomas. Identifying pheochromocytoma using anatomic imaging alone in cases of bilateral adrenal nodules is challenging. Functional imaging with radioisotope tracers can be helpful such as MIBG (meta-iodobenzylguanidine) and DOTATATE scans. We describe a case of pheochromocytoma, which was negative on DOTATATE but positive on MIBG scan which localized the pheochromocytoma. A 65-year-old male with a history of hypertension, chronic kidney disease and obstructive sleep apnea was found to have bilateral adrenal nodules on routine imaging. MRI abdomen showed a right adrenal nodule 1.7cm x 1.8cm x 1.7cm and a left adrenal nodule 2.3 cm in maximum dimension. He had no apparent symptoms of catecholamine excess. Biochemical testing revealed elevated total plasma metanephrines on serial assessments along with elevated 24-hour urine dopamine levels. A CT scan with adrenal protocol further characterized the right adrenal nodule with precontrast density of 43 Hounsfield Units (HU) with a rapid washout of 75% within 5 minutes, consistent with benign adrenal adenoma. The left adrenal gland had 2 nodules. A smaller superior nodule 1.3 cm AP x 1.0 cm transverse x 0.9 cm craniocaudal in size, density of -3 HU on the precontrast images, with approximately 85% washout most consistent with a benign adrenal adenoma. A larger inferior nodule was 2.3 cm AP x 2.2 cm transverse x 2.3 cm craniocaudal in size and precontrast density of 32 HU, rising to 58 HU during the portal venous phase and remaining at about 58 HU on the delayed images, indicating no significant washout and is indeterminate. A Cu64 labeled DOTATATE scan showed the 1.6 cm right adrenal nodule demonstrated activity similar to that of contralateral background adrenal activity. There was no comment regarding the two left adrenal nodules. A I123 MIBG scan showed the larger of the two left adrenal nodules had a strong uptake of the MIBG highly likely to be a pheochromocytoma. The smaller left adrenal nodule and the right adrenal nodule had no significant MIBG uptake, making these adrenal adenomas. The patient underwent left robotic adrenalectomy and pathology confirmed a 2.3 cm pheochromocytoma in addition to adrenocortical nodular disease. Three months post-surgery, plasma and 24-hour urinary measurements for metanephrines and catecholamines had normalized. Further research is needed to assess the benefit of I123MIBG when DOTATATE is negative. A potential cause of false negatives on DOTATATE is variability in somatostatin receptor subtype expression. Better imaging modalities for pheochromocytomas and guidelines for the management of bilateral adrenal nodules would assist in treatment decisions. Presentation: Friday, June 16, 2023

FRI198 Acute Pancreatitis And Diabetic Ketoacidosis As A Presentation Of Subclinical Cushing Syndrome

Abstract Disclosure: W.H. Alkhidady: None. N. Almefgaie: None. A. Ekhzaimy: None. Case Report: A 43-year-old Saudi woman, presented to the emergency department with two days history of right upper quadrant pain, radiating to the back. Associated with nausea and vomiting with no history of diarrhea, constipation, or constitutional symptoms. She has a 4-year history of hypertension on 5 mg amlodipine with no history of endocrine or malignant tumors. On Examination she was alert, severely dehydrated with temperature of 37 °C, pulse of 124 per minute, blood pressure of 147/61 mmHg. The abdomen was tender in the right upper quadrant area. A laboratory examination showed lipemic sample with high triglycerides level 11mmol/L (0.4-1.48), high amylase 763nu/l (25-115), lipase 5773 u/l (73-393), low potassium level 2.6 mmol/l (3.50-5.10) and low calcium level 1.25 mmol/l (2.10-2.55). The urine analysis showed 4+ glucosuria and ketonuria. Her venous blood gas showed pH of 7.18 and bicarbonate of 10 mmol/L, random blood glucose was 16 mmol/L and anion gap could not be calculated at that time. A diagnosis of DKA secondary to acute pancreatitis secondary to high triglycerides level was made and management was started along with plasmapheresis. A CT pancreatitis protocol was done which showing a 4.2 x 3.5 cm well-circumscribed left adrenal lesion. An MRCP showed 4.3 cm well defined collision tumor in left adrenal gland, its peripheral part is suggestive of adenoma. further work up showed negative 24-hours urine metanephrins, normal Aldosterone Renin ratio, High 24-hour urine cortisol level 644 nmol/l, low ACTH 0.6 pmol/l and morning cortisol level post 1 mg DST was not suppressed on two different occasions. Since the patient had no phenotypical presentation of Cushing’s a diagnosis of SCS was made. She underwent left robotic assisted adrenalectomy, with pathological diagnosis of adrenocortical adenoma. 2 days later, AM cortisol level reduced to 230 nmol/L. while blood pressure and serum potassium remained normal with no signs of adrenal insufficiency. Her Diabetes mellitus and Hypertriglyceridemia resolved after left adrenalectomy. Conclusion: This case report demonstrates the uncommon association of subclinical Cushing syndrome and acute pancreatitis as a first presentation therefore it highlights the significant role of endocrine evaluation of any young patient with unexplained acute metabolic presentations. Presentation: Friday, June 16, 2023

FRI236 Primary Adrenal Insufficiency Due To Coccidiomycosis

Abstract Disclosure: T. Duro: None. Introduction: Primary adrenal insufficiency due to infectious adrenalitis is not common in the United States. When it occurs, the most common culprits are tuberculosis, histoplasmosis, and HIV-related manifestations. We present a rare case of disseminated coccidiomycosis presenting with hypercalcemia, adrenal insufficiency, and a right adrenal mass. Case Report: A 73-year-old Hispanic man living in New Mexico with history of paraesophageal hernia and a thoracic aortic aneurysm was admitted in the hospital during a COVID-19 infection due to severe weakness, poor oral intake, and abdominal pain. His labs on admission showed: mild eosinophilia, serum calcium corrected for albumin 13.8 mg/dL (n 8.4 - 10.4 mg/dL), PTH <6 pg/mL(n 18 - 84 pg/mL), PTH-rp 4.4 pmol/L (n 0.0 - 2.3 pmol/L), Vitamin D25-OH 18 ng/mL (n >29 ng/mL), Calcitriol 8.1 ng/mL (n 19.9 - 73.9 ng/mL), serum kappa/lambda light chain ratio 2.09 (n 0.29 - 1.65), SPEP and UPEP no M-spike, normal pattern, 8AM serum cortisol 0.9 ug/dL (n5.27 - 22.45 ug/dL), ACTH >1250 pg/mL (n 0 - 46 pg/mL), DHEA-S 20ug/dL (n 80 - 560 ug/dL), plasma normetanephrine 3.29 nmol/L (n 0.00 -0.89 nmol/L), plasma metanephrine <0.10 nmol/L (n 0.00 - 0.49 nmol/L). Abdominal CT scan showed a right hypodense adrenal mass measuring4.8 x 2.4 x 3.7 cm, with small areas of calcification. His left adrenal gland showed thickening and nodularity. Primary adrenal insufficiency was diagnosed based on labs above. He was given high dose IV hydrocortisone which was tapered to PO Hydrocortisone 15mg QAM and 5mg QPM. Fludrocortisone 0.1mg PO daily was also added. His presenting symptoms resolved. Work up for HIV, tuberculosis, and syphilis was negative. His hypercalcemia was thought to be due to acute adrenal insufficiency and less likely related to malignancy or other causes. His mildly elevated PTH-rp was thought to be due to his renal dysfunction, and his mildly elevated kappa/lambda ratio was thought to be non-specific by the oncology consultants after the patient had a negative nuclear bone scan. He was treated with IV fluids and his hypercalcemia resolved. Due to elevated plasma normetanephrines a pheochromocytoma could not be excluded. CT adrenal protocol imaging showed a 4.2 cm right adrenal mass with unenhanced attenuation: 40.6 HU. Absolute washout: 4.0 %, suggestive of hemorrhage. The left adrenal gland was unremarkable on this scan.Alpha and beta blockade were instituted, and the patient underwent aright adrenalectomy. Pathology showed: Necrotizing granulomatous inflammation and coccidioides-like organisms on GMS stain. His coccidioides serum antibodies were positive. He was referred to infectious diseases and was started on fluconazole 400mg daily. He is doing well. Clinical Lessons: Disseminated coccidiomycosis is a rare cause of infectious adrenalitis, but it should remain on the differential diagnosis of primary adrenal insufficiency, especially in endemic areas. Presentation: Friday, June 16, 2023

SAT330 Recurrence of Acth Independent Cushing Syndrome In Patient With Previously Operated On For Primary Bilateral Macronodular Adrenal Hyperplasia And Co-existence Of Papillary Thyroid Carcinoma

Abstract Disclosure: N. Vashakmadze: None. D. Nikoleishvili: None. Background: Cushing syndrome is a rare and usually sporadic endocrine disorder. Herein we describe a case of ACTH-independent Cushing syndrome and ACTH-dependent Cushing disease in non-identical twins. Moreover, recurrence of Cushing syndrome after 25 years of non-complete resection of adrenal glands and co-existence of papillary thyroid cancer. Clinical Case: A 57-year woman presented with hypertension, depression, easy bruising, proximal muscle weakness, fatigue, and facial plethora. According to the patient, Cushing’s syndrome caused by bilateral macronodular adrenal hyperplasia was diagnosed in 1997. Surgical treatment - left adrenalectomy and right adrenal gland resection was performed. She was receiving HRT during the first 6 months, since then she was not receiving HRT and her cortisol was normal. It is extremely interesting that her nonidentical twin sister was diagnosed with ACTH-dependent Cushing disease in 2006 and was successfully treated with transsphenoidal surgery. In 2019 patient was diagnosed with papillary thyroid cancer (PTC), thyroidectomy was performed and multifocal PTC pT1b(m)N0M0 was diagnosed. Patient received adjuvant therapy with 80 mCi of RAI. In 2019 cortisol and ACTH levels were routinely evaluated, ACTH was undetectable <3 ng/dl (N: 7.2-63); 1 mg dexamethasone suppression test showed no suppression of cortisol 116 ng/ml (N: <1.8 ng/ml). However, patient did not consult an endocrinologist with the results of the mentioned investigations. She admitted to our clinic in December 2022 with above-mentioned complains to determine the tactics of further investigation/treatment. The data obtained during our consultation was consistent to recurrence of Cushing syndrome: midnight salivary cortisol– 0.46 mcg/dl (0.02-0.34), ACTH– 4.2 ng/l (7.2-63), 1 mg of dexamethasone suppression test – 217 nmol/l (<50). CT showed right adrenal noduls (2.5 cm and 1.2 cm). These data confirmed the diagnosis of recurrence of Cushing’s syndrome and right adrenalectomy with steroid coverage and postoperative hormone replacement therapy was recommended. Other data showed normal OGTT, moderate hypercholesterolemia, taking into account Cushing’s syndrome and smoking, statins were initiated. DXA showed osteoporosis (forearm T-score = -3.9 SD). 25(OH)D3 – 22.35 ng/ml, Ca – 1.09 mmol/l (1.1-1.3). Denosumab, calcium and vitamin D supplements were started to the patient. In addition, TSH was markedly suppressed on 125 mcg LT4 therapy, TSH – 0.005 mIU/l (0.4-3.8); Tg <0.17 g/l, anti-Tg - N and neck ultrasound was normal, that is consistent with excellent response and the target TSH was established 0.5-2.0 mIU/l. The dose of LT4 was reduced to 100 mcg. Conclusion: To our knowledge existence of Cushing syndrome and Cushing disease in non-identical twins has not been reported yet. Also, co-existence of Cushing syndrome and thyroid cancer is rare, and no connection is established yet. Presentation: Saturday, June 17, 2023

THU094 Multiple Endocrine Neoplasia Type 1 Tumor Recurrence After Loss To Follow Up

Abstract Disclosure: S.J. Sternlieb: None. K.G. Romo: None. R.S. Boothe: None. D. Englert: None. N. Manuel: None. Q.Z. Iqbal: None. Multiple Endocrine Neoplasia Type 1 Tumor Recurrence After Loss to Follow-up Sternlieb, S. J., Romo, K.G., Boothe, R., Manuel, N., Iqbal, Q.Z., Grissett, B., Englert, D. Background: MEN1 is a rare, autosomal dominant tumor disorder due to mutations in the MEN1 tumor suppressor gene. It is characterized by parathyroid, enteropancreatic and pituitary tumors. We present a young woman with MEN1 with prolactinoma, primary hyperparathyroidism and multiple pancreatic neuroendocrine tumors. Clinical Case: A 30-year-old woman with MEN1 presented with complaints of abdominal pain and GI bleeding (GIB). She was diagnosed with a prolactinoma at age 14 after presenting for primary amenorrhea and galactorrhea. She didn’t tolerate medical therapy with bromocriptine or cabergoline and required transphenoidal resection. At age 19, she presented with hypoglycemia and was found to have multiple pancreatic neuroendocrine tumors (NET) as well as a left (L) adrenal gland tumor. She underwent distal pancreatectomy and L adrenalectomy. Ten tumors in the pancreas stained variably for insulin, glucagon, gastrin and VIP. Multiple cortical adenomas were found in the adrenal gland. Post-operatively she developed a spinal cord hematoma related to placement of an epidural catheter for pain control, which led to permanent paraplegia. At age 21, she was diagnosed with primary hyperparathyroidism. She underwent left parathyroidectomy, then was lost to follow-up for 9 years. EGD showed multiple superficial duodenal ulcers. MRI brain identified a right (R) cavernous sinus mass inseparable from the R pituitary. CT abdomen showed a pancreatic head mass. Given the concern for neuroendocrine etiology, she underwent an EUS showing a pancreatic mass and a liver mass. Pancreatic mass pathology confirmed low-grade NET with Ki67 proliferation rate <1%, positive synaptophysin and chromogranin staining. After multidisciplinary discussion, the consensus was to continue PPI and sucralfate for GI ulcers, start lanreotide, and obtain somatostatin receptor scintigraphy. Surgery was not recommended unless significant GIB occurred on maximal therapy. She was referred to follow-up with GI, endocrinology, and NSGY. Since discharge, she has presented several times to other hospitals for abdominal pain, but has not adhered to follow-up. Conclusions: This case of recurrent neuroendocrine and pituitary tumors despite prior resections demonstrates the consequences of prolonged loss to follow-up with MEN1. Without routine surveillance, recurrence is likely. This patient lives in a rural area with lack of access to nearby subspecialty care, which is a barrier. More resources are needed to support patients with social barriers and progressive endocrinopathies. Key words: MEN1, prolactinoma, gastrinoma, NET, health care barriers Presentation: Thursday, June 15, 2023

FRI251 A Combination Of Klinefelter Syndrome And The Classic Form Of Congenital Dysfunction Of The Adrenal Cortex: Clinical Case

Abstract Disclosure: N.I. Volkova: None. Introduction: Congenital adrenal hyperplasia (CAH) is a defect in one of the enzymes or transport proteins involved in the synthesis of cortisol in the adrenal cortex. Virile form of CAH is characterized by cortisol deficiency and hyperandrogenism. Klinefelter syndrome is one of the most frequent chromosomal diseases leading to the development of primary hypogonadism. The manifestation of these two diseases in one patient causes difficulties in diagnosis and medical treatment that leads to adverse consequences. Clinical Case: A 43-years-old patient consulted a physician complaining about the lack of erections for 4 years, breast enlargement. From anamnesis, it is known: that the patient at the age of 2 years and 8 months based on experienced growth of pubic hair, decreased level of 17-ketosteroids in the urine and genetic analysis diagnosis of CAH, virile form was suspected. Prednisone 5 mg daily was prescribed. At the age of 5, based on phenotypic features and karyotyping Klinefelter Syndrome (XXY) was diagnosed. At the age of 13, stimulating hormonal chorionic gonadotropin drug with only one course of 10 injections was prescribed. At the age of 18, the patient independently canceled the use of prednisone. Further, he did not receive medication therapy for CAH and Klinefelter syndrome. At the age of 42, adrenal CT revealed formation of the left adrenal gland. High levels of aldosterone and renin were detected. A diagnosis of left adrenal aldosteroma was made and a left-sided adrenalectomy was performed. Histological examination diagnosis of aldosteroma did not confirmed. On physical examination, BMI 30 kg/m2, genoid type of obesity, right testicle is not palpated, left testicle is dense, reduced in size. Small penis size.Laboratory examination revesled decreased level of total testosterone, normal level of SHBG, LH and FSH. Ultrasound of the scrotum organs revealed as well decrease in the size of the testicles and appendages, a volumetric formation of the right testicle, suspicious of tumor. Thus, diagnosis of CAH, virile form and Klinefelter syndrome, primary hypogonadism, right-sided cryptorchidism was confirmed. Hydrocortisone 30 mg daily was prescribed. Hormone replacement therapy with testosterone preparations was not prescribed until surgical treatment of neoplasm of the right testicle will be performed. Conclusion: We have demonstrated a combination of two endocrine pathologies and serious mistakes were made in the management of this patient that requires a multidisciplinary approach, which improves the prognosis and quality of life of these patients. Presentation: Friday, June 16, 2023

THU619 Atypical Clinical Presentation Of Adrenal Hemorrhage

Abstract Disclosure: T.L. Madsen-Barbosa: None. F.H. Antoni: None. A. Ghazi: None. Introduction: Adrenal hemorrhage (AH) is an uncommon condition that is challenging to diagnose due to its nonspecific findings. We illustrate a case of AH that presented with acute clinical deterioration and had catecholamine overproduction. Case: A 58-year-old woman with a history of migraine headaches and prediabetes presented to the emergency department for “the worst headache of her life” and palpitations of 4 hours duration. On admission, her blood pressure was 224/139 mmHg and heart rate was 102 bpm. Her physical examination was significant for diaphoresis and active vomiting. Initial laboratory tests showed hyperglycemia and leukocytosis. Her ECG, non-contrast CT scan of the head and CT angiography of the head and neck were unremarkable. Her hospital course was complicated by recurrent hypertensive episodes, arrhythmias including Torsades de pointes, and left flank pain. A CT chest, abdomen, and pelvis revealed a large left retroperitoneal hemorrhagic collection which seemed to arise from the inferior aspect of the left adrenal gland. She subsequently underwent successful IR embolization of the left inferior suprarenal artery. Further workup showed elevated urinary fractionated metanephrine and normetanephrine levels, as well as elevated levels of plasma free metanephrine and normetanephrine. Given concern for pheochromocytoma, phenoxybenzamine was started. A repeat CT abdomen after six days demonstrated shrinkage in hematoma and a 7 mm hypoattenuating nodule arising from the medial limb of the left adrenal gland. Outpatient follow-up CT abdomen showed left adrenal thickening with a 2.3 cm nodular area with 78.9 Hounsfield units and absolute washout of 60.9% suggestive of adenoma. After one month, repeat catecholamine levels normalized. Discussion: The clinical manifestation of AH varies widely. Acute adrenal insufficiency is seen if bilateral glands and 90% of the cortices are compromised. However, unilateral AH is most often asymptomatic - found incidentally on imaging. Having elevated catecholamines is described in 30% of patients in one case series. Differentiating spontaneous AH from hemorrhagic adrenal tumors is also a dilemma. Among the hemorrhagic adrenal masses, pheochromocytoma is the most common. Conclusion: This case highlights that clinicians should be aware of clinical manifestations of catecholamine excess when evaluating patients with AH. A catecholamine producing adrenal mass (pheochromocytoma) should also be ruled out in these situations. Reference:1- Elhassan YS, Ronchi C, Wijewickrama P, Balvedeweg SE. Approach to the patient with adrenal hemorrhage. J Clin Endocrinol Metab. 2023, 108 (4):995-1006.2- Karwacka IM, Obolonczyk L, Sworczak K. Adrenal hemorrhage: a single center experience and literature review. Adv Clin Exp Med. 2018; 27 (5): 681-687. Presentation: Thursday, June 15, 2023

THU615 Rhabdomyolysis: A Rare Presentation Of Extra-adrenal Neuroendocrine Tumor

Abstract Disclosure: M. Saleem: None. S.M. Harman: None. J. Targovnik: None. Introduction:Paraganglioma is a rare neuroendocrine tumor (NET) arising from neural crest progenitors located outside of the adrenal gland and predominantly produce norepinephrine if functional. Here, we present a case of epinephrine producing extra-adrenal sympathetic neuroendocrine tumor. Case: A 66-year-old male with history of resistant hypertension (on amlodipine, lisinopril & metoprolol), hyperlipidemia & obesity presented for evaluation of possible pheochromocytoma/paraganglioma. He reported paroxysmal episodes of muscle spasm & paresthesia accompanied by hypertension (up to 200 mm Hg), palpitation, sweating & tremor lasting about 30-60 minutes. His CPK was elevated up to 428 U/L (NR 30-170 U/L) during those episodes. He reported 7 "major episodes" beginning ∼15 years prior to diagnosis, of which 3 occurred after major surgery with "small episodes" occurring monthly, exacerbated by sun & heat. Family history was remarkable for sister with recurrent rhabdomyolysis. Labs showed elevated epinephrine 181 pg/ml (normal <57), Dopamin 67 pg/ml (normal <10) and normal norepinephrine, significantly elevated 24h urine metanephrine 855 mcg/24h (normal 90-315), normal normetanephrine & dopamine levels. CT showed a 2.2 x 2.2 x 2.1 cm retroperitoneal mass with 84% absolute concerning for possible paraganglioma. FDG-PET and DOTATATE showed hypermetabolic activity (SUV max 6.63) and DOTATATE avid left retroperitoneal mass respectively. After discontinuing beta blocker and optimizing alpha blockage with doxazosin, the patient was referred to endocrine surgery and had a successful resection of this mass and left adrenalectomy. Pathology was consistent with chromaffin cell neoplasm and normal left adrenal gland. Patient had clinical and biochemical resolution of his symptoms post-op. Genetic counseling was done with pending results. Discussion: Pheochromocytomas & paragangliomas (PPGLs) are rare NET arising from chromaffin cells of the adrenal medulla or neural crest progenitors located outside of the adrenal gland, and predominantly produce epinephrine and norepinephrine respectively. Adrenal medullary tissue secretes epinephrine due to the activity of PNMT, induced by high local levels of cortisol from the adrenal cortex, which attaches a methyl radical to the catechol amino group. Extramedullary sympathetic secretory cells normally lack this enzyme and secrete norepinephrine. Thus, it is likely that the neoplasm we report had ectopic expression of this enzyme. PPGLs have variable signs and symptoms which mainly reflect the hemodynamic and metabolic actions of the catecholamines produced and secreted by the tumors with hypertension being the most common sign. Rhabdomyolysis is an unusual manifestation. This case is unique in that it presented as an extra-adrenal NET predominantly produced epinephrine and presented with rhabdomyolysis. Presentation: Thursday, June 15, 2023

SAT323 Management Of Severe Cushing’s Syndrome Due To Cortisol Secreting Adrenal Tumor In A Critically Ill Patient

Abstract Disclosure: H. Obeid: None. E.D. Buras: None. T. Else: None. Introduction: Severe Cushing’s syndrome is associated with multiple life threatening complications. Clinical Case: A 69 year-old man with Cushing’s syndrome was admitted to the hospital with septic shock due to bacteremia with necrotic left lower extremity infection. He required above knee amputation. His hospital course was complicated with invasive pulmonary Aspergillosis and ventilator associated pneumonia. He developed acute kidney injury that required dialysis, altered mental status, deep venous thrombosis bilateral pulmonary embolisms, bleeding duodenal ulcer and retroperitoneal hematoma. Prior to admission: Random cortisol was 31.7 mcg/dL (Normal: 4.8-19.5 mcg/dL), AM Cortisol following 1 mg oral dexamethasone was 47 mcg/dL (Normal: <1.8 mcg/dL), DHEA-s was 210 mcg/dL (normal: 33.6-249 mcg/dL) and 24 hour urine free cortisol was 622 mcg/day (Normal: <60 mcg/day). ACTH was undetectable. An FDG-PET scan showed a 6.5 centimeters hypermetabolic left adrenal mass with internal coarse calcifications but no evidence of metastasis. Etomidate drip, Metyrapone and Osilodrostat were tried to lower excess cortisol production. During admission while on Osilodrostat: Cortisol level: 20 mcg/dL (normal: 8-19 mcg/dL). Deoxycorticosterone: 53 ng/dL (Normal: 2-19 ng/dL). 11-Desoxycortisol: 580 ng/dL (Normal: 12-158 ng/dL). 17-alpha-hydroxyprogesterone: 45 ng/dL (Normal: 27-199 ng/dL). Surgery was considered, but adrenalectomy was deemed high risk and not pursued. Therefore, the patient underwent computed tomography (CT) cryoablation of the left adrenal gland. Labs following cryoablation: Cortisol: 7.7 mcg/dL. Deoxycorticosterone: 10 ng/dL. 11-Desoxycortisol: 34 ng/dL. 17-alpha-hydroxyprogesterone: 25 ng/dL. The procedure was complicated by T11/T12 intercostal artery injury leading to hemothorax. He was continued on physiologic doses of hydrocortisone. On follow up 4 weeks following discharge, he had complete resolution of hypercortisolism symptoms. A repeat whole body PET scan showed persistent FDG-avid neoplasm in left adrenal area, as well as metastasis in the liver and the duodenum. Conclusion: It is critical to control hypercortisolism in patients with Cushing’s syndrome. Several medications can be used. Surgical adrenalectomy remains the gold standard for treatment. CT-guided cryoablation of the adrenal tumor is a reasonable alternative when patient is not a surgical candidate. However, it is not a completely benign procedure as it can be associated with complications. Our treatment approach improved short term outcome for our patient, but did not provide long term benefit since he developed metastasis. Presentation: Saturday, June 17, 2023

FRI219 Pheochromocytoma Presenting As A Case Of Worsening Diabetes Mellitus

Abstract Disclosure: J. Eapen: None. E. Sedlis Singer: None. T.X. Lu: None. J.N. Shapiro: None. Introduction: Pheochromocytoma is often diagnosed incidentally during unrelated abdominal imaging, in patients with a family history of known disease, or in patients presenting with the classic triad of symptoms: episodic headache, sweating, and tachycardia. We describe an atypical case of pheochromocytoma presenting as acute decompensation of DM. Case presentation: A 79 year old female with DM previously well-controlled on glimepiride for 4 years presented with an acute worsening of symptoms notable for abdominal fullness, weakness and weight loss. Laboratory results revealed an increase in HbA1c from 6% to 14% (nl <5.7%) since 3 months prior. Metformin and basal insulin were added to her regimen with a subsequent improvement in HbA1c. An abdominal CT scan was done given the acuity of progression in her DM, which revealed a 5.8cm adrenal mass. The patient denied easy bruising, striae, mood changes, dizziness, or hypertension. Biochemical evaluation demonstrated normal potassium level 3.7 (3.5-5.3 mmol/dL) and aldosterone 8ng/mL (<28), non-suppressed cortisol 7.8mcg/dL after dexamethasone 1mg given the night prior, but Cushing’s Disease was ruled out by normal salivary cortisol 0.08 mcg/dL (nl 0.04-0.56 mcg/dL) and urine free cortisol 22.8 mcg/ 24h (nl 4-50 mcg/24h), and further testing revealed elevated metanephrines and catecholamines during 24 hour urine collection, Epinephrine 129 mcg/24h (nl 2-24 mcg/24h), Metanephrine 7121 mcg/24h (nl 90-315 mcg/24h) and Normetanephrine 2078 mcg/ 24h (nl 122-676 mcg/24h).Repeat CT scan again showed an indeterminate 5.4 x 3.8 x 5.5 cm right adrenal mass, a 6.9mm lipid-rich left adrenal adenoma, and left adrenal gland hyperplasia. Consequently, the patient underwent laparoscopic right adrenalectomy with pathology revealing a diagnosis of pheochromocytoma. Post-op plasma fractionated metanephrines normalized and have remained normal. Immediately post-op, all diabetes medications (including insulin, glimepiride, and metformin) were stopped and she has had resolution of diabetes with HbA1c 6 on no medications when 1-year post-op. Discussion: Excess catecholamines are known to cause disruption of glucose and insulin homeostasis from desensitization of the beta-adrenergic receptor and increased insulin resistance1. Despite this, less than a handful of pheochromocytoma cases have been reported to be presenting as an acute glycemic derangement as in our patient.

FRI152 Outcome Data From FABULAS: A Feasibility Study Of Radiofrequency Endoscopic Ablation, With Ultrasound Guidance, As A Non-surgical, Adrenal Sparing Treatment For Aldosterone Producing Adenomas

Abstract Disclosure: G. Argentesi: None. X. Wu: None. E. Goodchild: None. Y. Lee: None. K. Laycock: None. N. Alexander: None. R. Senanayake: None. J. MacFarlane: None. E.D. Benu: None. G. Goodchild: None. P. Wilson: None. E.M. Godfrey: None. M. Gurnell: None. H. Cheow: None. S.P. Pereira: None. W. Drake: None. M.J. Brown: None. Primary aldosteronism (PA) is the potentially curable cause of high-risk hypertension in 5-10% of unselected patients. Outcomes post total adrenalectomy, the standard treatment for unilateral aldosterone producing adenomas (APAs), are variable. Between 30-60% are cured (1), but prediction of outcome is unreliable, and some patients are reluctant to have an adrenalectomy. Endoscopic ultrasound (EUS)-guided radiofrequency ablation (RFA) is an alternative to surgery for epigastric malignancies but has rarely been attempted for adrenal lesions. Given the proximity of the left adrenal gland to the stomach, we conducted a multicentre pilot study of EUS-RFA for left-sided APAs, to determine the safety and efficacy of this adrenal sparing treatment. 29 patients were recruited with confirmed unilateral (or dominantly asymmetrical) left sided APAs confirmed by either standard AVS or molecular imaging (11C-Metomidate or 18F CETO PET scan) with SUV max lateralisation ratios >1.25(2). Patients had an average age of 56 years and either comorbidities dictating surgical caution or had declined surgery (personal preference). 35 ablations were performed across 3 centres in 28 patients (22 Male and 6 Female) with 7 patients having a repeat procedure following careful consideration from a multi-disciplinary panel. All patients underwent alpha and beta blockade 2 weeks prior to procedure, an anaesthetist was present at all procedures, with 31 undergoing general anaesthesia and 4 deep sedation only. A 5/10 mm 19G StarMedTM probe was introduced transgastrically by EUS into functioning adrenal nodules that ranged in size from 9-36 mm. An electrode/grounding pad was placed on all patients and energy generated at 15-30 watts to perform ablation. FNA samples were taken prior to ablation for immunocytochemistry to confirm (retrospectively) that CYP11B2 positive cells were the target and for further genotyping. Follow-up molecular imaging was used to assess completeness of ablation. The primary outcome was safety of procedure: to establish that rare events such as perforation, haemorrhage and infarction of major organs did not occur within the first 48 hours (assessed by examination, blood counts and CT abdomen). All adverse events, 5 in total, were reported and scored in severity by the team and reviewed by an independent safety committee. The conclusion of the committee was that EUS-RFA is a safe alternative to adrenalectomy for left-sided APAs. The secondary outcomes included the analyses of complete, partial or absent clinical and biochemical outcomes as determined by PASO criteria recorded at 1,3 and 6 months post-procedure. Data lock is at the end of January 2023; several patients have achieved complete PASO clinical success. Full primary and secondary outcomes, together with pre-and post-molecular imaging data, will be reported.1.Funder.The Management of PA.JCEM2016.2.Nature Medicine, in press. Presentation: Friday, June 16, 2023

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 < 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

FRI205 Splenic Infarct: A Rare Presentation Of Cushing Syndrome

Abstract Disclosure: G. Virella: None. Splenic Infarct: A Rare Presentation of Cushing Syndrome Geover, Virella M.D. 1,2, Carlos Santiago Botero Suarez, M.D.1,2, Alfredo Palomino Abella, M.D. 1,2, Mustafa Kinaan, M.D. 1,2 1) University of Central Florida HCA Healthcare GME, Greater Orlando, Florida2) Department of Internal Medicine, University of Central Florida College of Medicine, Orlando, Florida Disclaimer: This research was supported (in whole or in part) by HCA Healthcare and/or an HCA Healthcare affiliated entity. The views expressed in this publication represent those of the author(s) and do not necessarily represent the official views of HCA Healthcare or any of its affiliated entities.Introduction Cushing’s syndrome (CS) is associated with a hypercoagulable state leading to increased incidence of thromboembolic events. The mechanism behind this phenomenon is due to an imbalance between activity of pro- and anticoagulant pathways. We report a case of newly diagnosed CS in a patient presenting with acute splenic and inferior vena cava (IVC) infarcts. Case Description A 59-year-old woman with no significant medical history presents with acute left upper quadrant pain. She denied other symptoms. Physical examination was remarkable for tachycardia, left upper quadrant tenderness, and centripetal adiposity with BMI of 19. Labs showed marked leukocytosis (WBC 26 x 109/L) and thrombocytopenia (platelet 96,000 cells/uL). She was also found to be diabetic (HgA1C 12.5%). Abdominal CT imaging showed a wedge-shaped 4.3 x 3.1 x 2.3 cm splenic infarction, peri-splenic abscess, and thrombosis of the left renal and gonadal veins extending into the IVC. Additionally, there was a left adrenal incidentaloma measuring 4.5 x 3.8 x 2.9 cm. MRI of the left adrenal gland was consistent with an adenoma. The right adrenal gland was unremarkable. Screening for pheochromocytoma and hyperaldosteronism was normal. A morning serum cortisol was 33.4 mcg/dL, and ACTH level of < 1.5 pg/mL. An overnight 1-mg dexamethasone suppression test revealed post-dexamethasone cortisol of 4.58 mcg/dL. These findings were consistent with Cushing syndrome secondary to adrenal cause. The patient was initially treated with heparin infusion and underwent a successful thrombectomy of the IVC and gonadal vein. She later underwent a successful laparoscopic left adrenalectomy and splenectomy. Pathology reports confirmed an adrenocortical adenoma. A random post-surgical cortisol was 22.8. The patient had an uneventful postoperative course and was discharged home with outpatient endocrine follow-up planned to evaluate for hypercortisolism resolution and management of diabetes. Conclusion Cushing Syndrome patients have been shown to have an almost 18-fold higher rate of venous thromboembolism (VTE). This is an often-overlooked complication of hypercortisolism. The pathogenesis is not fully understood but is believed to be related to shortening of activated partial thromboplastin time (aPTT), increased clot lysis time, and glucocorticoid-induced increase in procoagulant factors such as fibrinogen, von Willebrand factor, and factor VIII. Our case will closely demonstrate the link between hypercortisolism and hypercoagulability. Presentation: Friday, June 16, 2023

SAT326 Massive Adrenomyelolipomas Due To Longstanding CAH

Abstract Disclosure: Q. Aziz: None. A. Champion: None. Introduction: Congenital Adrenal Hyperplasia (CAH) is an autosomal recessive disorder caused by deficiency in adrenal corticosteroid biosynthesis. 21 Hydroxylase Deficiency is common in 90-95% of CAH cases and have a defect in converting 17 OH Progesterone to 11 deoxycortisol. Therefore, they have an increase in ACTH secretion due to the decrease negative feedback drive along with excess adrenal androgens. Adrenal myelolipoma are common in patients with CAH and the tumors are benign and lipomatous. Clinical Case: Patient is a 47 year old female with past medical history of CAH presents to the clinic for evaluation of generalized fatigue and abdominal pain. She was diagnosed with CAH- 21 hydroxylase deficiency at birth and required vaginal reconstruction surgery. She was on hydrocortisone and fludrocortisone until age 18, but thereafter stopped taking medications or seeing physicians for nearly 30 years. CT abdomen/pelvis showed large bilateral adrenal myelolipomas, right adrenal gland measuring 23.2 cm and left adrenal gland measuring 20 cm. MRI confirmed enlarged adrenal glands. Initial labs included ACTH 299, 17-OH Progesterone >2000, 11 deoxycorticosterone 23.6, total testosterone 160, free testosterone 30.3, DHEA-S 192, Cortisol 2.8. She was started on fludrocortisone 50 mcg and Hydrocortisone 10 mg in the morning and 5 mg in the evening and was referred to an endocrine surgeon out of state, but surgery was not offered due to concerns of medication nonadherence. She returned to clinic 6 months after presentation, she admitted to missing multiple doses of her hydrocortisone. Repeat CT Abdomen and Pelvis showed severe compression of the IVC and right lobe of the liver along with mild compression of the kidneys and pancreas. To improve adherence, she was switched to prednisone, and eventually dexamethasone 0.75mg and fludrocortisone 100mcg. She complains of fatigue but improved abdominal pain. After a period of good adherence with medication administration, she was referred to a local surgical oncologist who agreed to do bilateral adrenalectomy due to mass effect. Conclusion: CAH is a challenging chronic disease with variable penetrance in phenotype, ranging from mild hirsutism to frank adrenal insufficiency. Massive adrenomyelolipomas are thought to be ACTH driven, especially seen in undertreated and longstanding CAH. Without the 21 hydroxylase enzyme to convert 17 OH Progesterone to 11 deoxycortisol, levels of ACTH rise. While these myelolipomas are benign and can generally be monitored, surgery should be considered if the size impairs function of surrounding organs. Bilateral adrenalectomy can also be considered in patients with CAH for refractory hypertension, hyperandrogenism and virilization, Cushing syndrome, infertility, or mass effect. Presentation: Saturday, June 17, 2023

FRI234 A Pregnancy With Two Special Surprises A Baby And A Nonhemorrhagic Adrenal Infarction During Pregnancy

Abstract Disclosure: S. Idriss: None. N. Barua: None. I. Jamal: None. A. Jabiev: None. Introduction: Unilateral Acute adrenal infarction (AAI) is a rare cause of acute abdomen in pregnancy, with a reported incidence of 1.3%. It usually presents with sudden, severe, and unremitting flank pain. The most common cause of AAI is adrenal vein thrombosis. We present a 26-year-old pregnant female who presents with right flank and back pain, found to have AAI without hemorrhage. Case: 26-year-old female Primigravida without significant past medical history presented to the emergency room at 33 weeks of gestation with sudden onset of RUQ flank and back pain. She was afebrile and normotensive. Physical exam was positive for right flank point tenderness. She underwent multiple investigations including an abdominal US which did not reveal an acute pathology. She was admitted and started on opioids which failed to control her pain. An abdominal MRI was obtained subsequently revealing 4.6cm right adrenal gland lesion suspicious for AAI without hemorrhage and normal left adrenal gland. The pain was poorly controlled warranting Dilaudid drip and repeat imaging/CT showed adrenal gland mass with density of 41-52 HU suspicious for lipid poor adenoma versus adrenal hematoma on CT scan. Initial AM Cortisol level was 46 ug/dl, then it trended down to 31.7. DHEAS was low at 94.2 ug/dl. Plasma metanephrines and normetanephrines were normal. Course was complicated with hypotension and worsening pain on day 10 of admission, repeat MRI showed increase in right adrenal lesion size and cystic transformation, containing some T2 hypointense septations and debris without notable intrinsic T1 hyperintensity to suggest blood products, and restricted diffusion which is a finding compatible with AAI. Repeat AM Cortisol and DHEAS were low at 12 and 61.3 respectively. Despite normal left adrenal gland, clinical picture was consistent with adrenal insufficiency and she was started on stress steroids. Thrombophilia workup was negative. She ultimately underwent c-section on day 13 of admission. She was discharged on physiologic dose of steroids with a pending repeat MRI and outpatient follow up. Discussion: Adrenal glands, especially with preexisting nodules are predisposed to micro-vascular thrombosis and infarction during procoagulable states. Pregnancy is a hypercoagulable state that predisposes patients to thrombotic events. The relative risk of thrombosis is 5-6 times higher in pregnant than non-pregnant females of the same age. Our patient had evidence of adrenal insufficiency and required steroid replacement. Anticoagulation was not started due to possible subtle hematoma on CT scan on initial imaging. Follow up imaging is planned. Conclusion: Adrenal infarction is a rare cause of abdominal pain during pregnancy, and if missed, it can result in devastating clinical consequences for the mother and the child. Adrenal insufficiency if found should be addressed to prevent circulatory collapse. Presentation: Friday, June 16, 2023

FRI125 Recurrence Of Primary Aldosteronism Long-Term After Adrenalectomy For Aldosterone-Producing Adenoma With KCNJ5 Gene Mutation: A Two-Cases Report

Abstract Disclosure: K. Aiga: None. M. Kometani: None. D. Aono: None. S. Karashima: None. T. Yoneda: None. Primary aldosteronism (PA) is a major cause of secondary hypertension. PA is known to have higher prevalence of cerebral or cardiovascular complications, indicating the importance of early detection and treatment for PA. PA is caused by autonomous secretion of aldosterone in the adrenal glands. PA is characterized by high plasma aldosterone concentration, low plasma renin activity and hypokalemia. PA is classified into unilateral PA (aldosterone-producing adenoma [APA] or unilateral hyperplasia) and bilateral PA (bilateral adrenal hyperplasia). PA with aldosterone excess in bilateral adrenal glands is defined as bilateral PA. On the other hand, PA with aldosterone excess in unilateral adrenal gland is defined as unilateral PA. Strategies for PA treatment depends on the subtype of PA. Medication by mineralocorticoid receptor antagonists is a common treatment for bilateral PA. Adrenalectomy is the most efficient treatment for unilateral PA. In general, patients tend to maintain normal serum potassium level and blood pressure after adrenalectomy, and recurrence of PA is extremely rare. Recently, mutation in the gene named KCNJ5 was found to derive APA. Herein, we report two cases of PA recurrence more than 10 years after surgical treatment for APA. Somatic mutation in KCNJ5 was detected in the first occurrence of PA in both cases. First case, a 52-year-old woman was examined for hypertension 22 years after total adrenalectomy of the right adrenal gland. Recurrent PA was diagnosed based on high aldosterone-renin-ratio (ARR), identification of left adrenal gland tumor by computed tomography (CT), and a confirmatory test. Second case, a 65-year-old man was examined for hypertension 17 years after total adrenalectomy of the left adrenal gland. He had maintained his blood pressure using medication since the onset of hypertension 4 years after the surgery. A year later, a high ARR was observed. PA recurrence was determined by a right adrenal gland tumor noted on CT and a confirmatory test. Tissues of the adrenal gland were obtained from adrenalectomy in both cases. Histopathological analysis revealed presence of one adenoma in the first case, while two adenomas were confirmed in the second case. Somatic mutation in KCNJ5 gene was detected in both cases. To date, there are no specific guidelines established for the management of recurrent PA. Early detection is crucial for the prevention of severe cardiovascular diseases. Long-term follow-up is recommended after the treatment of PA. Presentation: Friday, June 16, 2023

High-grade B-cell Lymphoma Initially Presenting with Dysphagia, Upper Gastrointestinal Bleeding, and Adrenal Invasion

Abstract Gastric high-grade B-cell lymphoma (HGBL) with adrenal gland invasion is a rare clinical entity with few cases reported in the literature. Patients usually present with nonspecific symptoms that lead to delays in the diagnosis, initiation of treatment, and prognosis. In this report, we present a case of a middle-aged woman in her 60s who presented with shortness of breath, dysphagia, early satiety, and melena. Further investigations confirmed the presence of an ulcerated gastric mass with invasion to the left adrenal gland, and histopathological and molecular studies of the gastric mass confirmed the diagnosis of B-Cell Lymphoma with MYC and BCL-6 rearrangements, a rare aggressive lymphoma subtype known as HGBL. Consequently, she was started on R-CHOP-21 chemotherapy protocol with good outcomes and resolution of the mass on repeat imaging; the current report aimed to emphasize the importance of recognizing unusual presentations of HGBL, especially gastric HGBL, which will help in early recognition, treatment, and prognosis.