CT Of Abdomen Research Articles

6572 Acute Adrenal Crisis Secondary To Undiagnosed Addison's Disease

Abstract Disclosure: J.M. Deirmenjian: None. H. Nickowitz: None. T. Kavafyan: None. Addison’s disease, also known as primary adrenal insufficiency, is when the adrenal gland has a defect in making cortisol. Signs and symptoms of Addison’s disease include nausea, vomiting, diarrhea, fatigue, dizziness, hyperpigmentation, and mood changes. Acute Adrenal Crisis can occur as a result of Addison’s disease when patients experience acute physiologic stress such as acute illness. Patient is a 35- year-old woman with a PMHx of iron deficiency anemia presenting with intractable nausea and vomiting for 10 days and a single witnessed syncopal episode two days prior to presentation. She was also had intermittent dizziness. Patient initially presented to urgent care where she was found to be hyponatremic and then was sent to the ED. She had no seizure like activity or rashes prior to admission. On initial physical exam, her skin was warm and dry with skin discoloration and hyperpigmentation. She was awake, alert, and oriented to person, place, time, and situation. Patient had no neurological deficits and no lateral or vertical nystagmus on initial assessment. On admission, patient was hyponatremic to 114 mEq/L, potassium was 5.0 mEq/L, and glucose level was 79 mg/dL. Despite receiving a 500 cc NS bolus, 1L NS bolus, 25 grams of albumin, and D5-0.45 NS at 50 cc/hr, the hyponatremia was difficult to correct. Her sodium initially corrected to 120 mEq/L but the sodium later decreased to 117 mEq/L. The morning after admission, patient became hypotensive and hypoglycemic. Random cortisol was measured and found to be 2.2 mcg/dL, raising concern for acute adrenal crisis as a result of undiagnosed Addison’s Disease. ACTH level was 17 pg/mL. Endocrinology was consulted and she was treated with IV 100 mg Hydrocortisone in addition to IV hydrocortisone 50 mg IV q6h. Patient was able to be transitioned to PO hydrocortisone 15 mg qAM and 5 mg qPM and was started on PO fludrocortisone 0.1 mg daily. On day of discharge, sodium improved to 127 mEq/L. CT Abdomen with contrast was completed to evaluate the adrenal glands and it showed a normal appearance of the adrenal glands. The etiology of the patient’s acute adrenal crisis was secondary to undiagnosed Addison’s Disease. It is interesting to note that patient was not hypoglycemic on admission but later became hypoglycemic during her hospital course. Adrenal crisis is a possible fatal complication of undiagnosed Addison’s disease. Therefore, a possible Addison’s Disease diagnosis is important to consider when patients are presenting with symptoms such as nausea, vomiting, dizziness, and electrolyte derangements including hyponatremia. If patient’s present with hyponatremia, ruling out possible adrenal insufficiency by checking a cortisol level may change the course of patient’s care. After discharge, she was able to establish care with Endocrinology and remain stable on treatment with PO hydrocortisone and PO fludrocortisone. Presentation: 6/3/2024

7019 Persistently Elevated Estradiol Levels in a Transgender Male; An Unexpected Pregnancy

Abstract Disclosure: M. Kanin: None. S. Grock: None. N. Agrawal: None. Background: Testosterone is the backbone of gender-affirming hormone therapy (GAHT) for transmasculine and non-binary individuals assigned female at birth (AFAB). While testosterone can affect fertility, it should not be used as contraception and is contraindicated in pregnancy. It is important to counsel patients on fertility preservation, pregnancy, and contraceptive options prior to testosterone initiation. However, the Endocrine Society Guidelines and World Professional Association for Transgender Health Standards of Care provide minimal guidance on reproductive health and contraceptive options. Case Description: An 18-year-old individual AFAB on GAHT with testosterone was referred to endocrinology for elevated estradiol levels. On presentation, the patient denied vaginal bleeding or breast tenderness. The date of last menstrual period was reported as two years prior. There was no significant past medical or surgical history. Medications included subcutaneous testosterone cypionate 20 mg weekly. Physical exam was notable for elevated BMI 42.6kg/m², mild facial hair and central adiposity. Hormone analysis revealed testosterone of 172 ng/dL (adult male: 200-1000) and estradiol 27,322 pg/mL (mid-cycle Female: 80-400). Estradiol by mass spectrometry obtained due to concern for immunoassay interference resulted at > 10,000 pg/mL. FSH <0.1 mIU/mL, LH<0.1 mIU/mL, DHEA-S 2,460 ng/mL (adult female: 400-3600), androstenedione 1.85 ng/mL (0.260 - 2.140), prolactin 328 ng/mL (3 -23.1). CT abdomen and pelvis and pituitary MRI were ordered to assess for ovarian, adrenal and/or pituitary tumor. Later that day additional laboratory tests revealed alpha fetoprotein 126 ng/mL (0 -6.7), progesterone 100.2 ng/mL (luteal phase: 3.0-25.0), beta-hCG 32,571 mIU/ml, inhibin B<10, inhibin A 500.1pg/ml (luteal phase: 40-80) and CA-125 19 U/mL (0 - 35). Given the elevated beta-hcg the patient was asked if they were having sex in a way that could result in pregnancy to which they answered affirmatively. CT and MRI were cancelled, and abdominal ultrasound was obtained which revealed a single living intrauterine pregnancy with estimated gestational age 35 weeks 2 days. Testosterone was stopped and they subsequently delivered a healthy baby girl. Conclusion: Gender diverse patients on testosterone can experience unplanned pregnancy. As testosterone is contraindicated in pregnancy, counseling on reproductive health and contraception is needed. A pregnancy test should be considered prior to initiating hormones for appropriate individuals. It is important to be aware that Individuals AFAB may not desire estrogen containing birth control and placement of intrauterine devices can be dysphoric. Further investigation into how contraceptive options may affect outcomes for individuals on GAHT is needed to minimize barriers to care and to enable patients to make well informed decisions. Presentation: 6/2/2024

6402 Unsuspected Bladder Mass Turns Out To Be A Paraganglioma

Abstract Disclosure: S. Lamichhane: None. A. Flowers: None. D.T. Price: None. A. Kaur: None. Background: Urinary bladder (UB) paragangliomas are extremely rare tumors comprising of less than 0.05% of all bladder neoplasms and 10% of all paragangliomas. The diagnosis is usually suspected based on clinical features of painless hematuria, episodic headaches, hypertension, palpitations associated with micturition or bladder and confirmed biochemically by elevated urinary and/or plasma fractionated metanephrines (MN). Here, we present a case of a UB mass that was initially diagnosed on biopsy and subsequent biochemical workup revealed elevated MN. Case Report: A 64-year-old man with type 2 diabetes and hypertension, admitted for revision surgery of subdural hematoma was incidentally found to have a 3.3 x 3.4 x 3.9 cm polypoid heterogenous hyper enhancing UB mass attached to the anterior wall on CT abdomen done for abdominal pain. Cystoscopy and TURBT were complicated by hypertensive urgency. Pathology turned out to be paraganglioma. Detailed history at this time revealed a long-standing history of frequency, urgency, nocturia 4 times nightly, recurrent urinary tract infections, an episode of gross hematuria 1-2 years ago, and difficulty controlling blood pressure on 4 anti-hypertensives. Subsequently, labs showed plasma free MN of 97 pg/ml (ref range <=57), free normetanephrine (NMN) of 2392 pg/ml (ref range <=148), and total MN of 2489 pg/ml (ref range <=205). Genetic panel was negative for SDHA, SHD, AF2, SDHB, SHDC, SDHD, VHL, RET, TMEM127. He received 6 doses of Lantreotide due to suspicion of metastasis based on abdominal, pelvic, and inguinal lymphadenopathy seen on DOTATATE scan. After preparation with alpha and beta blockers, he underwent uneventful robot-assisted cystectomy with lymph node resection that resulted in a well-differentiated paraganglioma negative for margins, lymph node involvement, and extra bladder extension. A week later, MN reduced, plasma free MN 37 pg/ml, free NMN 178 pg/ml, and total plasma MN 215 pg/ml. His BP improved, and micturition-induced palpitations resolved. Conclusion: Due to the rarity of UB paraganglioma, the diagnosis can be missed and delayed. This has a serious implication due to the risk of hypertensive crisis during the surgery and increased risk of cardiovascular complications. This case highlights the need for awareness among physicians of the clinical presentation associated with UB paraganglioma, its early recognition and management. Presentation: 6/2/2024

8194 Extramedullary Hematopoiesis Involving Bilateral Adrenal Glands In A Patient With Sickle Cell Anemia

Abstract Disclosure: V.T. Pham: None. L.N. Sendos: None. Title: Extramedullary hematopoiesis involving bilateral adrenal glands in a patient with sickle cell anemia Background: Extramedullary hematopoiesis refers to blood cell production occurring outside the bone marrow. It is a reactive process caused by inadequate production or excessive destruction of blood cells typically seen in patients with hereditary anemias or infiltrative bone marrow disorders. Extramedullary hematopoiesis usually occurs in the spleen, liver and lymph nodes, but it can also very rarely arise from the adrenal glands. Case Presentation:We present a case of a 42-year-old male with a history of sickle cell anemia who presented with chest pain and shortness of breath and had a chest CT angiography which incidentally showed a 5.6 x 3.8 cm left adrenal mass and a 2.9 x 2.1 cm right adrenal mass. Further evaluation with an MRI abdomen with and without contrast revealed a 5.5 cm lobulated left adrenal mass with patchy areas of microscopic fat and faint delayed heterogeneous enhancement as well as a 2.2 cm right adrenal nodule. The nodules were described as markedly T2 hypointense with heterogeneous T1 hyperintense/isointense signal on MRI with a broad differential diagnosis including extramedullary hematopoiesis in bilateral adrenal glands, lipid poor adenoma or neoplasm. Regarding his sickle cell disease history, the patient was diagnosed as an infant and had very rare episodes of sickle cell pain crisis as a child. However, once he entered adulthood, he began to have multiple episodes of sickle cell pain crisis requiring hospitalizations and transfusions. On further review of patient’s prior imaging scans during these previous hospitalizations, he was first noted to have bilateral sub centimeter adrenal nodules on a CT Abdomen without contrast done almost a decade prior to his current presentation. We ordered biochemical evaluation which did not show any evidence of adrenal hormonal hypersecretion. His case was discussed at our multidisciplinary conference with the radiology and surgery team and it was concluded that the imaging characteristics were most consistent with extramedullary hematopoiesis in the setting of bilateral adrenal gland involvement and patient’s history of sickle cell anemia. Therefore, no immediate surgical resection was recommended. Given the size of the left adrenal mass being greater than 4 cm in size, we advised follow-up imaging for surveillance. Discussion:There has been a rise in detection of adrenal incidentalomas due to the increased use of advanced diagnostic imaging in healthcare settings. When patients with hematologic diseases are found to have adrenal incidentalomas, it is important to consider extramedullary hematopoiesis in the differential diagnosis to avoid unnecessary invasive procedures and surgeries. Presentation: 6/2/2024

8358 A Diagnostic Challenge: Occult Paraganglioma Misdiagnosed as Pheochromocytoma Leading to Unnecessary Adrenalectomy

Abstract Disclosure: A.R. Kasireddy: None. S. Alshami: None. S. Gummalla: None. C. Balasubramaniapandian: None. J.A. Henske: None. A. Albashaireh: None. Background: Pheochromocytomas and paragangliomas are rare neuroendocrine tumors. Catecholamine-secreting paragangliomas, like pheochromocytomas, can present with hypertension, sweating and episodic headaches. Thoughtful consideration must be given to distinguish between the two and potentially avoid a critical misdiagnosis. Case: A 61-year-old woman with hypertension and diabetes presented for evaluation of glomus jugulare discovered on CT of the head during evaluation of worsening hearing loss and tinnitus. She reported a longstanding history of episodic headaches, palpitations, anxiety spells and excessive sweating with more recent onset of ear fullness. When symptoms first presented, she had a left adrenalectomy 3 years prior. CT abdomen at that time showed 1.8 x 2.2 cm left adrenal nodule with a density of 12.2 HU and early contrast washout. Plasma metanephrines were normal but normetanephrines were elevated at 5.1 nmol/L (nl: less than 0.9 nmol/L). 24-hour urine metanephrines were normal but normetanephrines were elevated to 892mcg/24h (nl: 122-676 mcg/24h). MIBG scan was done and showed increased activity in the left adrenal nodule. Decision was made to proceed with left adrenalectomy. Surgical pathology was benign. Present evaluation after discovery of the glomus tumour with worsening symptoms showed normal plasma metanephrines, elevated plasma normetanephrines at 1529.9 pg/ml (0- 285.2 pg/ml), elevated plasma norepinephrine at 3280 pg/ml (0-874 pg/ml), with normal dopamine and epinephrine levels. CT head showed a 3 x 2.7 x 2.1 cm lytic destructive mass within the petrous temporal bone at the left skull base containing multiple internal calcifications. Given the lab and imaging findings, paraganglioma was diagnosed. Genetic testing revealed SDHB mutation. Neurosurgery consultants determined that the tumour was not amenable to resection. She was treated with radiation therapy, alpha blockade, and close blood pressure monitoring. Discussion: This case highlights the importance of reviewing the degree of hormonal elevations as well as the pattern of elevation to obtain a correct diagnosis. Paragangliomas commonly secrete normetanephrines rather than metanephrines and are often not immediately localized. Understanding the most likely genetic mutations when a paraganglioma is suspected can direct appropriate choice of radionuclide scanning agents. In this case, an earlier diagnostic scan may have prevented unnecessary adrenalectomy and facilitated earlier discovery of the paraganglioma. Presentation: 6/2/2024

7109 "My Stomach hurts": A Rare Case of Adrenal Cortical Carcinoma

Abstract Disclosure: R.C. Attah: None. I. Sirisena: None. Pheochromocytoma/Paragangliomas are rare neurogenic tumors that arise from chromaffin cells present in the adrenal medulla or extra-adrenal paraganglia, these cells produce catecholamines. Typically, these tumors are discovered as incidentalomas. They can be hereditary as seen in genetic syndromes like multiple endocrine neoplasia type 2A or 2B, neurofibromatosis type 1 or Von Hippel Lindau syndrome but can occur sporadically. Patients typically present with hormonal hypersecretion signs or symptoms like abdominal pain, episodic headaches, sweating, hypertension and tachycardia, but some patients are asymptomatic. Patient is a 37-year-old female with past medical history of hypertension, type 2 diabetes mellitus and fatty liver who presented to the ED with abdominal pain for a few weeks. On arrival, her vitals were BP 205/124, HR 102, RR 14 and SpO2 100% on room air. CT abdomen was obtained which showed a 7.6 x 4.7 x 5.7 cm mass replacing left adrenal gland with small left retroperitoneal hemorrhage worrisome for adrenal cortical carcinoma, metastatic disease or pheochromocytoma. Hormonal workup revealed elevated 24-hr urine cortisol 124.2mcg (ref 4.0-50.0mcg/24h), normal aldosterone/renin ratio 10.63ng/dL (ref <30ng/dl), failed low dose dexamethasone suppression test with post-dex AM cortisol 7.20ug/dL (ref <1.8ug/dL), slightly elevated serum total catecholamine 1256pg/mL (242-1125 pg/mL), norepinephrine 1155pg/mL (ref 217-1109 pg/mL), total metanephrine and normetanephrine 341pg/mL (reg <205 pg/mL), normetanephrine 244.1 (ref <210.1pg/mL), elevated 24-hr urine normetanephrine 700ug/L (ref 131-612ug/L), metanephrine 136ug/L (ref 36-209ug/L) and DHEA-s level 285ug/dl (ref 23-266ug/dL). Due to elevation in cortisol, adrenal cortical carcinoma was suspected as these tumors are frequently associated with cortisol secretion. However, pheochromocytoma with co-secretion of ACTH could not be excluded. She was started on doxazosin for alpha blockade and eventually started on beta blockade with Metoprolol. She underwent a left adrenalectomy and was started on steroid taper post-op due to concern of cortisol co-secretion. Surgical pathology was positive for Left adrenal malignant pheochromocytoma and ganglioneuroma. Genetic panel was negative with no mutation identified in the 54 gene Hereditary paraganglioma-pheochromocytoma panel. This patient had a large adrenal tumor (>4cm mass) but mildly elevated catecholamines which could have led to misdiagnosis as her catecholamine levels were less than 2-3x ULN which is unusual for pheochromocytomas. Roughly 25% of people with pheochromocytoma are undiagnosed throughout their lifespan. This condition can be life-threatening, hence early diagnosis and intervention can be lifesaving. Prognosis of malignant pheochromocytoma is dependent on tumor size, presence of SDHB gene mutation or visceral metastases. Presentation: 6/1/2024

7019 Persistently Elevated Estradiol Levels in a Transgender Male; An Unexpected Pregnancy

Abstract Disclosure: M. Kanin: None. S. Grock: None. N. Agrawal: None. Background: Testosterone is the backbone of gender-affirming hormone therapy (GAHT) for transmasculine and non-binary individuals assigned female at birth (AFAB). While testosterone can affect fertility, it should not be used as contraception and is contraindicated in pregnancy. It is important to counsel patients on fertility preservation, pregnancy, and contraceptive options prior to testosterone initiation. However, the Endocrine Society Guidelines and World Professional Association for Transgender Health Standards of Care provide minimal guidance on reproductive health and contraceptive options. Case Description: An 18-year-old individual AFAB on GAHT with testosterone was referred to endocrinology for elevated estradiol levels. On presentation, the patient denied vaginal bleeding or breast tenderness. The date of last menstrual period was reported as two years prior. There was no significant past medical or surgical history. Medications included subcutaneous testosterone cypionate 20 mg weekly. Physical exam was notable for elevated BMI 42.6kg/m², mild facial hair and central adiposity. Hormone analysis revealed testosterone of 172 ng/dL (adult male: 200-1000) and estradiol 27,322 pg/mL (mid-cycle Female: 80-400). Estradiol by mass spectrometry obtained due to concern for immunoassay interference resulted at > 10,000 pg/mL. FSH <0.1 mIU/mL, LH<0.1 mIU/mL, DHEA-S 2,460 ng/mL (adult female: 400-3600), androstenedione 1.85 ng/mL (0.260 - 2.140), prolactin 328 ng/mL (3 -23.1). CT abdomen and pelvis and pituitary MRI were ordered to assess for ovarian, adrenal and/or pituitary tumor. Later that day additional laboratory tests revealed alpha fetoprotein 126 ng/mL (0 -6.7), progesterone 100.2 ng/mL (luteal phase: 3.0-25.0), beta-hCG 32,571 mIU/ml, inhibin B<10, inhibin A 500.1pg/ml (luteal phase: 40-80) and CA-125 19 U/mL (0 - 35). Given the elevated beta-hcg the patient was asked if they were having sex in a way that could result in pregnancy to which they answered affirmatively. CT and MRI were cancelled, and abdominal ultrasound was obtained which revealed a single living intrauterine pregnancy with estimated gestational age 35 weeks 2 days. Testosterone was stopped and they subsequently delivered a healthy baby girl. Conclusion: Gender diverse patients on testosterone can experience unplanned pregnancy. As testosterone is contraindicated in pregnancy, counseling on reproductive health and contraception is needed. A pregnancy test should be considered prior to initiating hormones for appropriate individuals. It is important to be aware that Individuals AFAB may not desire estrogen containing birth control and placement of intrauterine devices can be dysphoric. Further investigation into how contraceptive options may affect outcomes for individuals on GAHT is needed to minimize barriers to care and to enable patients to make well informed decisions. Presentation: 6/2/2024

5141 Metastatic Adrenocortical Carcinoma

Abstract Disclosure: N. Anwaar: None. Adrenocortical carcinomas are rare and frequently aggressive tumors that may be functional (hormone secreting) and cause Cushing's syndrome and/or virilization or nonfunctional and present as an abdominal mass or as an incidental finding. We report a case of a 72-year-old lady who was presented to the ER with chest pain and elevated blood pressure. CTA was performed to evaluate for aortic dissection, which incidentally found a 5.0 x 3.7 cm right-sided adrenal mass. 1 mg overnight dexamethasone suppression study showed non-suppression of cortisol. The ACTH was suppressed, consistent with the autonomous production of cortisol from the adrenal gland. CT abdomen with adrenal protocol showed an indeterminate suspicious 5.1 x 3.9 cm right-sided adrenal mass. She underwent an adrenalectomy and the final pathology showed adrenocortical carcinoma. Postoperatively she was treated with adjuvant radiotherapy and chemotherapy. A few years later, she developed metastatic lesions and overt Cushing syndrome. She was started on pembrolizumab, which was later discontinued due to poor response and then placed on mitotane. The metastatic disease progressed, and the patient was transitioned to hospice care. Due to tumor’s rarity, the understanding and experience of management modalities are limited. New treatment options may be available in the coming years. Presentation: 6/1/2024

7703 Adrenal Cortical Carcinoma With Vascular Involvement - Complex Case Requiring Life-Saving Surgery With Bovine Pericardium- IVC Reconstruction

Abstract Disclosure: V. mehta: None. M. Renzu: None. K.N. Pereira: None. S. Sampath: None. S. Sruthi: None. E.B. Ruby: None. Adrenocortical carcinomas (ACCs) are rare endocrine tumors that exhibit unusual biological characteristics. Roughly 66% of cases are functional and have excessive hormone release causing classic symptoms of hypercortisolism, including plethora, diabetes, muscle weakness/atrophy, virilization, and osteoporosis. Few present with vague signs due to mass effects, including but not limited to abdominal/flank pain, fullness, or early satiety; the rest are incidental findings. We present a case of a 55-year-old female with no medical history presented following weeks of progressive abdominal pain with abdominal wall erythema, leg swelling, and dyspnea along with hirsutism, leukocytosis, elevated BNP, hematuria concerning for abdominal wall cellulitis and new-onset CHF. Initial CT chest revealed incompletely visualized incidental adrenal mass. Further investigations with CT abdomen and lab work revealed a left adrenal mass suspicious for ACC, extending into the left renal vein and IVC, elevated urine metanephrines and normetanephrine, and elevated cortisol levels with low ACTH. The dexamethasone suppression test confirmed inadequate cortisol suppression. Patient underwent aggressive surgery with exploratory laparotomy revealing tumor thrombus in left renal vein extending into Inferior vena cava (IVC). A left adrenalectomy, nephrectomy, and IVC reconstruction using a Bovine Pericardial Patch (BPP), which is majorly used for arterial reconstruction, was performed. Pathology results indicated ACC, measuring 13.5 cm, with extension into renal vein and vascular invasion, staged as pT4pN0 cM0 without evidence of deficient mismatch repair protein expression. ACCs are rare and profoundly aggressive malignancies with an incidence rate of 0.7–2.0 per million per year with bimodal age distribution. Disease peaks before the age of five and in the fourth to fifth decade of life, with increased incidence in females. Diagnosis cannot be conclusively established by a single histological technique, imaging, hormonal work-up, or immunohistochemical labeling, but surgical excision is the gold standard. Complete surgical resection is the only possible approach for curing or improving survival chances in patients with adrenal mass/metastasis. A retrospective case study of 36 venous reconstructions using BPP showed few complications like thrombosis in 14% (versus 11% in autologous venous repair), 11% reoperated, 3-4% developed partial thrombus and hematoma. Our case exemplifies the successful management of challenging ACC with vascular involvement using BPP, which is mostly used for arterial repairs and is now expanding to venous repairs. Outcomes stress the ongoing need for research and collaborative efforts to refine and standardize the ideal approach to ACC patients with IVC thrombosis and the use of BPP in venous repair. Presentation: 6/1/2024

8267 A Case of Pineal Gland Cyst and Adrenal Adenoma- Is this a New Syndrome?

Abstract Disclosure: M. Siddiqui: None. U. Rafat: None. S. Shaik: None. J.L. Gilden: None. Background: The Pineal Gland receives information through a complex multi-neuronal pathway and is responsible for the secretion of melatonin, a hormone regulated by circadian rhythm and suppressed by light stimuli. Melatonin is involved in biological rhythms and regulates immunomodulation, temperature homeostasis and maturation of hypothalamic-pituitary and gonadal axis, and can also modulate thyroid, prolactin, growth hormone, cortisol, and ACTH activity. Other effects include downregulation of thyroid activity. In addition, studies have shown that pineal body extracts can cause a minor increase in the zona glomerulosa and fasciculata of the adrenal gland. Case: A 54-year-old male with recurrent nephrolithiasis, obstructive sleep apnea and chronic rhinosinusitis, first presented to Endocrinology Clinic in 2018 for evaluation of an incidental adrenal adenoma (CT abdomen -2.8 cm, less than 10 Hounsfield units, left sided lipid rich adrenal adenoma). He had new onset erectile dysfunction, decreased libido, and fatigue. Initial lab work including LH, FSH, TSH, midnight salivary cortisol, ACTH, plasma cortisol AM, free and total testosterone, aldosterone, plasma and urine metanephrines/normetanephrines were all normal. MRI brain-1 cm thin-walled pineal cyst with internal characteristics compatible with proteinaceous benign pineal cyst. He was then lost to follow-up for 5 yrs due to the COVID pandemic. He had tried taking “testosterone protein builders”, but his symptoms remained. In addition, he now has twice weekly dull occipital headaches, relieved by ibuprofen. Lab tests-elevation in 1of 2 MN salivary cortisol samples at 0.58 mcg/dL (nl≤0.09), ACTH 12pg/mL(0-47), IGF1 261 ng/mL(50-317), and low total testosterone 222 ng/dl (250-1100) Free testosterone 36 pg/mL(35-155), borderline low TSH 0.386 µIU/ml(0.55-4.78), Free T3 and Free T4 were normal, thyroid antibodies-negative . Rest of the hormone evaluation was otherwise normal, LH 4.90 mIU/mL(1.5-9.3), FSH 11.10 mIU/mL (1.4-18.1), Prolactin 6.8 ng/mL(2.1-17.7). Thyroid Ultrasound-homogeneous echogenicity of the thyroid gland without discrete thyroid nodules. Repeat MRI brain- benign pineal cyst which was stable with no midline shift or mass effect. Repeat CT adrenal gland-lipid rich adrenal adenoma slightly reduced in size from 2.8 to 2.4 cm. Lab tests returned to normal one month later. Conclusion: Although it is unclear whether there is an exact association between this patient’s pineal cyst and adrenal adenoma, there have been intermittent hormonal abnormalities and symptoms consistent with hypogonadism and/or adrenal, also possibly related to the pineal abnormality. Although more research needs to be conducted explaining the relationship between the pineal and pituitary, and the adrenal gland, this clinical case suggests that there may be a relationship between these two abnormalities and symptoms. Presentation: 6/2/2024

7094 Post Unilateral Adrenalectomy Hyporeninemic Hypoaldosteronism: Requiring Long Term Fludrocortisone In Managing Hyperkalemia And Renal Dysfunction

Abstract Disclosure: R. Rani: None. K. Bidani: None. R. Roy: None. Adrenalectomy, seen as a potentially curative measure for unilateral aldosterone-producing adenoma (APA), targets both biochemical and clinical resolution. However, postoperative challenges may arise, including acute renal failure and hyperkalemia, due to inadequate aldosterone secretion, a consequence of prolonged suppression of the renin-angiotensin-aldosterone system (RAAS) on the contralateral side. Our case underscores the postoperative challenge of isolated aldosterone deficiency leading to renal function deterioration and hyperkalemia, necessitating fludrocortisone therapy. We are presenting case of 71-year-old male with a history of uncontrolled hypertension for over 30 years with hypokalemia (taking KCL 20mEq daily). Initial laboratory findings revealed elevated aldosterone levels 79ng/dL and an aldosterone-to-renin ratio (ARR)> 20, K- 3.2mmol/L, creatinine 1.32mg/dl, Na-145 mmol/L with normal cortisol and plasma metanephrines levels. CT abdomen disclosed a 21 x 18 mm right adrenal adenoma, with subsequent confirmation of lateralization through Adrenal vein sampling. The patient underwent right adrenalectomy. Two weeks post-surgery, he presented to the emergency department with symptoms of fatigue and weakness. Laboratory results indicated hyperkalemia (K- 6.5 mmol/L) and elevated creatinine (3.52mg/dl) with undetectable aldosterone and renin levels. Urinalysis and CT abdomen ruled out other causes of acute renal injury. Initial management included intravenous fluids and Potassium supplementation. However, due to persistent hypokalemia, the patient was initiated on fludrocortisone (0.1 mcg daily). Subsequent follow-up demonstrated improvement in renal function and potassium levels, however, his aldosterone and renin levels remain undetectable. Hyperkalemia incidence post-adrenalectomy in primary aldosteronism (PA) ranges from 6.3% to 29.1%. Notably, older age and preexisting impaired renal function, particularly in cases with prolonged hypertension are identified as potential risk factors for postoperative hyperkalemia and renal failure. Understanding the potential for a further decline in the glomerular filtration rate (GFR) post-surgery becomes crucial. This decline might manifest as a transient or permanent issue, necessitating prolonged fludrocortisone treatment. This case highlights the importance of tailored preoperative assessment and postoperative management to mitigate complications in high-risk populations. Presentation: 6/1/2024

6980 A Case Report Of Severe Hypercalcemia In Acute Decompensated Liver Cirrhosis

Abstract Disclosure: M.M. Eid: None. R.M. Sargis: None. Introduction: Hypercalcemia in advanced liver disease in the absence of malignancy is a rare condition. Case: A 26 year old female with history of liver cirrhosis secondary to alcohol use disorder, presented with hematemesis and ascites. She was diagnosed with acute decompensated liver cell failure and transferred to our facility for liver transplantation. On Day 10 of her hospital course, she had an acute rise in serum calcium (Ca) to 10.8 mg/dl (N 8.7-10.5),albumin (Alb) 2.6 g/dl, corrected calcium (CCa) 11.9mg/dl. On day 13 Ca peaked to 13.5 mg/dl, CCa 14 mg/dl, ionized Ca 6.5mg/dl (N 4.2-5.4 mg/dl). Serum Ca was normal on day 9, Ca 8.7mg/dl, Alb 2.5 g/dl and CCa 9.9 mg/dl. She was not taking lithium, vitamin A or antacid. No personal or family history of bone disorder, fracture or kidney stone. On exam, blood pressure 85/45, heart rate 110 bpm, jaundiced with abdominal distension and respiratory distress. Labs: hemoglobin 8.1 g/dl, WBC 32.5K/Ul, platelet 136 K/Ul, serum creatinine 1.82 mg/dl, Na 133 mmol/l, K 3.5 mmol/l, Total/Direct bilirubin 29.9/18.1 mg/dl, alkaline phosphatase 166 U/L, AST/ALT 60/42U/l, Mag 2.3 mg/dl, Phos 5 mg/dl, INR 3, PTT 50 sec, parathyroid hormone (PTH) 12 pg/ml (N12-88), TSH 1.02 mciu/ml (N 0.34-4), 25 hydroxy vitamin D 17 ng/ml (N 20-80), parathyroid related peptide (PTHrP) 3.9 pmol/l (N<3.4), 1,25 di-hydroxy vitamin D 9.8 pg/ml (N 19.9-79.3), alpha fetoprotein 9.1 ng/ml (N <9). Normal anti smooth muscle and anti mitochondrial antibodies. Negative viral hepatitis serology. CT abdomen did not reveal hepatobiliary mass or tumor nor kidney stone. She was intubated and mechanically ventilated. Vasopressor support was initiated. One dose of calcitonin 4 units/kg and zoledronic acid 4 mg were given. Serum Ca decreased to 12 mg/dl, CCa 12.6 mg/dL then to 10.2 mg/dl, CCa 11mg/dl within hrs. Calcium normalized to 8.4mg/dl, CCa 9.2 mg/dl, ionized Ca 4.3 mg/dl within 24 hrs. Her condition deteriorated and withdrawal of care was decided. Conclusion: Our case demonstrates non PTH and non vitamin D mediated severe hypercalcemia in setting of acute decompensated cirrhosis and absence of malignancy. Previous literature has noted poor prognosis of hypercalcemia with acute decompensated cirrhosis. While hypercalcemia in cirrhosis may arise from paraneoplastic processes, in absence of malignancy it is speculated that acute hypercalcemia may arise from the inflammatory mediators inducing osteoclastic bone resorption as osteoclast activating factor, prostaglandin and immobilization. Some reports found an association between hypercalcemia and elevations in bilirubin and creatinine(1). Further research is needed to understand the pathophysiology and the management of hypercalcemia in acute decompensated cirrhosis.

8257 Pancreatic Neuroendocrine Tumor Presenting as Carcinoid Syndrome

Abstract Disclosure: D. Phung: None. Introduction Pancreatic neuroendocrine tumors (NETs) are rare tumors that arise in neuroendocrine cells of the pancreas. These tumors secrete a wide array of hormones. Carcinoid syndrome is a result of well-differentiated neuroendocrine tumors that secrete biologically active amines and peptides that cause the clinical manifestation of flushing, diarrhea, cardiac involvement, and wheezing. Case Presentation A 57-year-old male presented to the clinic for symptoms of tachycardia, shortness of breath, dizziness, and sweating for the past year. He had past medical history of chronic kidney disease, history of previous cardiac arrest, history of previous myocardial infarction, congestive heart failure, hypertension and gastric reflux disease. No history of tobacco, alcohol, or drug consumption. No previous surgeries. Family history with heart disease and prostate cancer in his father. His symptoms began over the course of the past year. Initially began with shortness of breath, chest pain, weight gain which then developed into dizziness with standing, episodes of night time sweating. He had associated headaches that were occurring daily. Vomiting would occur intermittently three to four times weekly. He noted episodes of flushing with rash occurring in the upper chest and bilateral arms. Diarrhea and loose bowel movements would occur occasionally. On examination he had tachycardia with heart rate at 103 BPM. Lung fields clear throughout. Abdomen was soft, nontender, without masses palpated. On biochemical testing chromogranin A was highly elevated and remained elevated upon repeat testing. Initial imaging with CT abdomen and pelvis with contrast was negative. PET scanning confirmed and localized his NET, revealing increased tracer uptake and hypervascularity in the neck of pancreas as well as two regions in the right lower lobe of the liver. Following confirmation and localization of his tumor he was then referred for surgical evaluation and treatment. Discussion In our presented case, the patient presented with symptoms suggestive of carcinoid syndrome which was confirmed biochemically with elevated chromogranin A. Clinical suspicion remains critical in diagnosis of NETs. The patient presented with clinically suspicious carcinoid syndrome, but initial testing with CT scan was negative and required further PET-based tracing to uncover tumors. Our case highlights the importance of clinical examination and acumen in diagnosing neuroendocrine tumors. Presentation: 6/2/2024

5142 Pheochromocytoma In The ICU- Hiding In Plain Sight

Abstract Disclosure: N. Chandrashekar: None. B. Arafah: None. Introduction: Pheochromocytomas are catecholamine-secreting tumors that are rare but dangerous and occur in <0.2% of patients with hypertension. Case: A 28 year old female with a 10 year history of hypertension and invasive stage 2b cervical cancer s/p chemoradiation presented to the hospital with circulatory shock from Takutsubo cardiomyopathy requiring intubation, vasopressors, CVVH, and ECMO. The endocrinology service was consulted due to a highly vascular, 3.6 cm, ∼23 Hounsfield units right adrenal mass evidenced on CT abdomen done months prior to admission. There were elevations in plasma normetanephrine to 15 nmol/L (ref <0.90 nmol/L), plasma metanephrine to 1.5 nmol/L (ref <0.50 nmol/L), urinary metanephrine to 565 ug/24-hours (ref 36 - 209 ug/24-hr), and urinary normetanephrine to 2580 ug/24-hr (ref 95 - 449 ug/24-hr). With her acute illness and recent use of vasopressors and quetiapine, these labs could not be accurately interpreted, and outpatient follow up was recommended. She was discharged home on metoprolol and hydralazine for hypertension management by her primary team. She was re-admitted to the CICU 2 days later for hypertensive emergency with flash pulmonary edema. With further questioning, she endorsed longstanding hypertension with episodes of headaches, palpitations, and vomiting. Her cancer therapy related menopause caused frequent nighttime hot flashes while inpatient. These episodes precipitated witnessed symptoms of catecholamine release with severe hypertension and palpitations. It was noted during her hospitalization that her symptoms were controlled during the day with frequent escape during the night despite continuous alpha and subsequent beta blockade every 6 hrs. Estrogen therapy would have been beneficial, however given a recent DVT, this was avoided and instead megestrol acetate and venlafaxine were used. With these additions, her adrenergic spells completely resolved. She then underwent a right adrenalectomy and had no significant fluctuations in her BP intraoperatively with complete resolutions of symptoms postoperatively. The pathology revealed a right 2.7 cm pheochromocytoma with no local invasion or distant metastasis. Conclusion: This is a case of pheochromocytoma crisis with cardiogenic shock followed by subsequent hypertensive emergency in the setting of inappropriate beta-blocker use. Confounders like vasopressors (increases catecholamines), intrinsic stress of critical illness (increases catecholamines), and quetiapine use (can increase normetanephrine levels) make it difficult to analyze labs. In these cases, a thorough history and imaging are key to establishing a diagnosis and initiating life-saving treatment. Another unique feature of this case is the control of the patient’s triggering menopausal hot flashes with megace and venlafaxine to preempt adrenergic spells during her admission. Presentation: 6/2/2024

8081 Case report of sympathetic paraganglioma presenting as chronic catecholamine induced cardiomyopathy

Abstract Disclosure: Z. Tan: None. A. Taiwo: None. Background: Sympathetic paragangliomas are rare neuroendocrine tumors (<1/100,000) arising from extra-adrenal autonomic paraganglia, generally in the thorax, abdomen, and pelvis. They secrete catecholamines predominantly norepinephrine, which can cause a rare complication of catecholamine-induced cardiomyopathy. Clinical Case: A 33-year-old male, with history of obesity and borderline hypertension not on medication, presented with dyspnea on exertion, worsening paroxysmal nocturia dyspnea of 1 month duration and sudden onset of severe right flank pain. Work up for his chronic shortness of breath revealed: Troponin negative. EKG showed sinus tachycardia. Echocardiogram revealed enlarged left ventricle, severely decreased LV systolic function of 25-30%. Given the absence of angina symptoms, negative troponin, and EKG findings, his new-onset cardiomyopathy was presumed to be non-ischemic. CT chest, abdomen and pelvis was performed for the new onset right flank pain, which revealed a 4.1 cm x 4.0 cm centrally necrotic aortocaval soft tissue mass. This raise a concern about malignancy. A CT-guided Fine-Needle Aspiration of the retroperitoneal mass suggested paraganglioma. Further investigation showed plasma normetanephrine at 5.56 nmol/L (Reference range: 0-0.89 nmol/L), 24 hour urine normetanephrine at 2411 ug/d (Reference range: 114-865 ug/d), and 24 hour urine norepinephrine at 179 ug/d (Reference range: 14-20 ug/d). Plasma metanephrines and plasma dopamine were within the normal range. A gallium DOTATE PET/CT revealed a necrotic aortocaval mass with peripheral uptake, consistent with the known paraganglioma. He was diagnosed with sympathetic paraganglioma complicated with chronic catecholamine-induced cardiomyopathy and had exploratory laparotomy excision of his paraganglioma. Preoperatively, he was treated with phenoxybenzamine and carvedilol. However, his post-surgical course was complicated by acute on chronic heart failure and pneumonia, and he subsequently passed away from a pulseless electrical activity arrest. Conclusion: Although sympathetic paragangliomas rarely presents as catecholamine-induced cardiomyopathy, this case emphasizes the importance of considering hormonal abnormalities in patients who present with non-ischemic cardiomyopathy without high risk factors. Reference: 1. Alessandra Giavarini, Antoine Chedid et al. Acute catecholamine cardiomyopathy in patients with phaechromocytoma or functional paraganglioma. Heart 2013; 99: 1438-1444. Presentation: 6/2/2024

7480 Isolated IgG4-Related Infundibulo-Hypophysitis

Abstract Disclosure: M. Allen: None. R.T. Beck: None. N.T. Zwagerman: None. R.J. Ryzka: None. D. Coss: None. A. Fisco: None. A.G. Ioachimescu: None. Background: 130 cases of IgG4-related hypophysitis (IgG4-RH) have been reported, usually in context of multi-organ IgG4-disease (e.g., siladenitis, pancreatitis, retroperitoneal fibrosis). Among patients with IgG4-disease, up to 5% develop hypophysitis. Presentation entails mass effects, AVP-D and anterior hypopituitarism.Case report: A 72-year-old man with history of hyperlipidemia presented with gradual decrease in strength, energy, appetite, libido, and depressed moods. Laboratory tests (8 AM) indicated undetectable AM cortisol, ACTH 6.8 pg/mL (7.2-63.3), TSH 0.20 mIU/mL (0.35-4.94), free thyroxine 0.64 ng/dL (0.7—1.48), prolactin 50.9 ng/mL (3.46-19.4), IGF-1 57 ng/mL (53-222), testosterone 7 ng/dL (240-950), LH 0.16 mIU/mL (0.57-12.07), and FSH 1.5 mIU/mL (1.0-12). MRI revealed a homogenously enhancing nodular suprasellar mass adjacent to the hypothalamus (0.8x0.7x0.8 cm) and contiguous with a 0.5 cm area of infundibular thickening. Neuro-ophthalmological evaluation was normal. In absence of mass effects, patient was monitored conservatively and treated with hydrocortisone, levothyroxine and testosterone replacement. Six months later, the mass enlarged to 1.2x0.8x1.1 cm. Patient underwent a transsphenoidal biopsy which revealed a dense fibrous mass with lymphoplasmacytic infiltration. Most cells stained for CD138 and IgG4. Postoperatively, serum IgG and complement levels, inflammatory markers, hemogram, serum protein electrophoresis, amylase and lipase were normal. CT chest and abdomen and thyroid ultrasound were unremarkable. After 4 weeks of prednisone 40 mg daily, the nodular lesion significantly decreased on MRI (0.6x0.5x0.4 cm). Prednisone was gradually tapered to 5 mg daily over 3 months. After 2 months of physiologic prednisone therapy, imaging remained stable. During follow-up of 1.5 years, no systemic IgG-4 disease was apparent and patient remained on glucocorticoid, thyroid and testosterone replacement.Discussion: Suprasellar masses can be inflammatory, infiltrative, infectious or neoplastic. The possibility of IgG4-RH should be considered even in absence of IgG4 systemic disease, as it happened in our case. Primary involvement of the infundibulo-hypophysis was suggested by a retrospective review study that found isolated IgG4-RH in 41% specimens initially diagnosed as primary autoimmune hypophysitis. Our case maintained a good radiological response after prednisone taper to physiologic doses. Response to prednisone is generally good in IgG4-RH, but relapse can occur upon taper, and long-term studies are lacking. Conclusion: We present a histologically-confirmed case of isolated IgG4-RH presenting with clinical manifestations of anterior hypopituitarism. Diagnosis and management of IgG4-RH requires multidisciplinary collaboration (endocrinology, rheumatology, neurosurgery, neuropathology and neuroradiology). Presentation: 6/3/2024

8790 A Case Of Ectopic Cushing’s Syndrome Secondary To Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH)

Abstract Disclosure: T. Goettemoeller: None. R. Lopez Fanas: None. K. Cedeno: None. A. Manavalan: None. Introduction: Ectopic Cushing’s syndrome is a rare disease accounting for <20% of cases of endogenous Cushing’s syndrome. DIPNECH is a poorly understood lung disease characterized by abnormal proliferation of neuroendocrine cells in the bronchial mucosa. It can be a precursor to pulmonary carcinoid tumors and has rarely been associated with ACTH production. Clinical Case: A 70-year-old female with intellectual disability and hypertension presented with diffuse edema and weakness. Examination was notable for hypertension, anasarca, and hirsutism. In addition to hypokalemia and metabolic alkalosis, hypercortisolism was observed: random serum cortisol - 50.1 ug/dL, late-night salivary cortisol - 1.72 mcg/dL (normal < 0.09 mcg/dL) and 24-hour urine free cortisol - 1,845.9 mcg (normal: 4.0 – 50.0 mcg/24h). Cortisol level remained > 25 ug/dL after high-dose dexamethasone suppression test. Ketoconazole was initiated and titrated to achieve normal 24-hour urine free cortisol levels and spironolactone was added to manage hypokalemia. An MRI of the sella and pituitary was unremarkable while an abdomen and chest CT identified a 2.5 cm well-circumscribed right middle lobe pulmonary nodule and mild thickening of the bilateral adrenal glands. A gallium-DOTATATE scan revealed a 2.1 cm right middle lobe pulmonary nodule with mildly increased uptake. A transbronchial biopsy of the right middle lobe nodule confirmed a carcinoid tumor. Immunohistochemistry (IHC) staining was positive for CHR, SYN, CD56, and AE1/3. Ki-67 stain showed a low proliferative index. A right middle lobectomy was performed with pathology revealing two typical carcinoid tumors measuring 2.8 and 0.7 cm each, with multiple carcinoid tumorlets and foci of neuroendocrine cell hyperplasia consistent with DIPNECH. IHC staining for ACTH was positive in the carcinoid tumors and tumorlets. Four days after surgery, AM cortisol was 8.1 ug/dL and potassium levels and blood pressure normalized off ketoconazole and spironolactone. The patient was discharged, but was readmitted a month later for anasarca, lethargy, hypokalemia, and metabolic alkalosis. A recurrence of Cushing’s syndrome was confirmed, and medical therapy was resumed. A chest CT revealed post-surgical changes. Her course was complicated by an aspiration event, septic shock, PEA arrest, resuscitation, and intubation. The patient’s care was ultimately transitioned to hospice. Conclusion: Florid Cushing’s syndrome due to ectopic ACTH production associated with DIPNECH is an exceedingly rare condition. Early recognition and treatment are crucial to prevent the numerous complications that may arise, and close follow-up after tumor removal is critical to ensure continued remission has been achieved. Presentation: 6/1/2024

8790 A Case of Ectopic Cushing’s Syndrome Secondary to Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH)

Abstract Disclosure: T. Goettemoeller: None. R. Lopez Fanas: None. K. Cedeno: None. A. Manavalan: None. Introduction: Ectopic Cushing’s syndrome is a rare disease accounting for <20% of cases of endogenous Cushing’s syndrome. DIPNECH is a poorly understood lung disease characterized by abnormal proliferation of neuroendocrine cells in the bronchial mucosa. It can be a precursor to pulmonary carcinoid tumors and has rarely been associated with ACTH production. Clinical Case: A 70-year-old female with intellectual disability and hypertension presented with diffuse edema and weakness. Examination was notable for hypertension, anasarca, and hirsutism. In addition to hypokalemia and metabolic alkalosis, hypercortisolism was observed: random serum cortisol - 50.1 ug/dL, late-night salivary cortisol - 1.72 mcg/dL (normal < 0.09 mcg/dL) and 24-hour urine free cortisol - 1,845.9 mcg (normal: 4.0 – 50.0 mcg/24h). Cortisol level remained > 25 ug/dL after high-dose dexamethasone suppression test. Ketoconazole was initiated and titrated to achieve normal 24-hour urine free cortisol levels and spironolactone was added to manage hypokalemia. An MRI of the sella and pituitary was unremarkable while an abdomen and chest CT identified a 2.5 cm well-circumscribed right middle lobe pulmonary nodule and mild thickening of the bilateral adrenal glands. A gallium-DOTATATE scan revealed a 2.1 cm right middle lobe pulmonary nodule with mildly increased uptake. A transbronchial biopsy of the right middle lobe nodule confirmed a carcinoid tumor. Immunohistochemistry (IHC) staining was positive for CHR, SYN, CD56, and AE1/3. Ki-67 stain showed a low proliferative index. A right middle lobectomy was performed with pathology revealing two typical carcinoid tumors measuring 2.8 and 0.7 cm each, with multiple carcinoid tumorlets and foci of neuroendocrine cell hyperplasia consistent with DIPNECH. IHC staining for ACTH was positive in the carcinoid tumors and tumorlets. Four days after surgery, AM cortisol was 8.1 ug/dL and potassium levels and blood pressure normalized off ketoconazole and spironolactone. The patient was discharged, but was readmitted a month later for anasarca, lethargy, hypokalemia, and metabolic alkalosis. A recurrence of Cushing’s syndrome was confirmed, and medical therapy was resumed. A chest CT revealed post-surgical changes. Her course was complicated by an aspiration event, septic shock, PEA arrest, resuscitation, and intubation. The patient’s care was ultimately transitioned to hospice. Conclusion: Florid Cushing’s syndrome due to ectopic ACTH production associated with DIPNECH is an exceedingly rare condition. Early recognition and treatment are crucial to prevent the numerous complications that may arise, and close follow-up after tumor removal is critical to ensure continued remission has been achieved. Presentation: 6/1/2024

6453 Cushing Syndrome And Bilateral Adrenal Incidentalomas In A Patient With Mucopolysaccharidosis

Abstract Disclosure: D. John: None. M. Kinaan: None. TITLE Cushing Syndrome & Bilateral Adrenal Incidentalomas in a patient with Mucopolysaccharidosis Affiliation of Authors Dieula John M.D., M.B.A.,a,b Mustafa Kinaan M.D.,a,baEndocrinology, Diabetes, and Metabolism Fellowship, UCF HCA Healthcare GME, Orlando, FL. bDepartment of Internal Medicine, UCF College of Medicine, Orlando, FL. 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: Mucopolysaccharidoses (MPS) is a group of hereditary and incurable storage diseases characterized by a deficiency of enzymes involved in the metabolism of glycosaminoglycans (GAGs) at the lysosomal level. MPS are usually inherited in autosomal recessive or x-linked pattern. MPS affects functioning of the cells, tissues and organs where the GAGs products typically accumulate. This can result in neurologic, developmental, and neoplastic issues. While endocrine disturbances in MPS have been reported, the literature on hypercortisolism and adrenal masses in MPS is limited. We present a case of a patient with MPS diagnosed with Cushing Syndrome (CS) as part of evaluation for adrenal incidentalomas. Case Description: A 50 year old male with a medical history of MPS, DVT, and developmental delay presented to our clinic accompanied by his mother for evaluation of adrenal incidentalomas. CT abdomen incidentally showed a left adrenal non-calcified, non-hemorrhagic mass measuring 3.9 x 3.6 cm with a pre-contrast attenuation of 45 Hounsfield units. Delayed washout was not obtained, and washout calculation couldn’t be performed. These radiologic features were suspicious for malignancy. Repeat CT with adrenal protocol showed left adrenal mass measuring 4.5 x 3.9 cm with central necrosis, unenhanced attenuation of 42 HU, and no drop-in attenuation on delayed phase indicating poor absolute and relative washout. There was also a right adrenal mass measuring up to 2.2 cm 54.5% absolute washout and 39.0% relative washout. Biochemical workup showed no pheochromocytoma, hyperaldosteronism, or adrenal hyperandrogenism. However, his dex suppression test was consistent with ACTH-independent CS. The patient was referred to an endocrine surgeon. We anticipate more information to be available based on the pathology of the excised tumor following his urgent surgery. Conclusion: MPS diseases are extremely rare and their effects on the endocrine system in general, and the adrenal gland specifically, is limited. Our case would serve as the first reported case of CS in MPS patient based on our literature search. In addition, we’ll discuss the pathology findings once the tumor is excised and highlight the connection between the adrenal mass and this lysosomal storage disease. Presentation: 6/3/2024

7536 Missed Silent Retroperitoneal Paraganglioma

Abstract Disclosure: S. Lamichhane: None. D.T. Price: None. J.J. Faria-Briceno: None. A. Kaur: None. Introduction: Paragangliomas are rare neuroendocrine tumors comprising about 15-20% of all chromaffin cell tumors and can arise anywhere along the sympathetic and parasympathetic ganglionic chain from skull base to the pelvic floor. About 75% of sympathetic paragangliomas arise in the abdomen out of which majority are functional and secrete catecholamines responsible for the typical clinical features. While the diagnosis is usually made by clinical features and lab elevation of catecholamines, it is estimated that 10-15% of paragangliomas are non-functional and another 10% do not manifest clinically according to the hormone activity, thus posing a significant diagnostic challenge. We report a case of a retroperitoneal paraganglioma diagnosed by biopsy as it was initially missed owing to its silent nature clinically and biochemically. Case report:A 48-year-old smoker with panic disorder, remote history of spontaneous pneumothorax, and exploratory laparotomy for motor vehicle accident visited ER due to left-sided abdominal fullness and weight loss of 10-20 pounds for several months. Review of systems was negative except for mild constipation. He denied taking any medications and BP had been normal. CT abdomen showed a large 13.3 cm mixed cystic and solid mesenteric mass. Core biopsy revealed pheochromocytoma and patient was referred to Endocrinology. Labs showed elevated plasma free metanephrines (MN) 80 pg/ml, normetanephrines (NMN) 190 pg/ml, total MN 270 pg/ml, all less than twice the upper limit of normal and inconclusive, non-elevated 24-hour urine MN and NMN but with inadequate collection, and normal plasma catecholamines. MRI revealed a 13.5 cm mass in the left retroperitoneal sympathetic chain separate from the left adrenal gland and inferior to the left kidney. After pre-op optimization with alpha and beta blocker, patient had an uncomplicated open resection of the mass along with the retroperitoneal lymph nodes (LN). Pathology resulted in 3 benign LNs, a 16.5 cm paraganglioma negative for margin, lymph-vascular invasion, and necrosis. Genetic panel was negative. Follow-up post-surgery at 1 and 3 months with mildly elevated catecholamines and MRI abdomen at 3 months without evidence of recurrent or residual mass and metastasis. PET scan is pending prior authorization. Conclusion: Silent retroperitoneal paragangliomas are exceedingly rare. The diagnosis is often missed when the tumors are asymptomatic, and biomarkers are not elevated. The lack of specific imaging characteristics also adds to the diagnostic challenge. Early recognition can avoid the potential risk of life-threatening hypertensive crisis during surgical intervention. Our case highlights the importance of considering this rare entity in the differential diagnosis of retroperitoneal mass. Presentation: 6/1/2024