High Suspicion Research Articles

Exploring TFE3-Rearranged Renal Cell Carcinoma: A Case Study showing minimal expression of PAX-8

Abstract Introduction/Objective TFE3-rearranged renal cell carcinoma accounts for 1.6-4% of adult renal cell carcinomas (RCC). The histological characteristics of this entity can mimic many other renal cell neoplasms and definite diagnosis is based on immunohistochemical (IHC) and fluorescent in situ hybridization (FISH) analysis. Nonspecific IHC findings can lead to diagnostic challenges delaying confirmatory studies and underdiagnosis. Methods/Case Report A 28-year-old male with sickle cell disease and a right kidney multilocular cystic mass incidentally found during evaluation of splenomegaly underwent partial nephrectomy. The tumor showed papillary, alveolar and sheet-like growth patterns composed of eosinophilic to clear cells with pleomorphic nuclei, including multinucleate forms. Initial ancillary studies showed minimal neoplastic expression (less than 5%) of PAX-8. Repeat PAX-8 yielded similar results and laboratory controls were evaluated successfully. A full work-up showed positivity of AMACR (diffuse), CD10 (diffuse), Cathepsin K (patchy), CK7 (focal), CAIX (focal), and no significant expression of pancytokeratin, HMB-45, Melan-A and SMA. Fumarate hydratase was retained, and the tumor showed a non-specific staining pattern of S (2-succinyl)-cysteine. Controls were appropriate. A high suspicion for a TFE-rearranged RCC was maintained, and a final diagnosis was confirmed with FISH analysis demonstrating a TFE3 (Xp11.23) rearrangement. Results (if a Case Study enter NA) NA Conclusion Reports in the literature detail diffuse PAX-8 expression in TFE3-rearranged RCC. A high index of suspicion based on morphology in our case prompted FISH analysis leading to correct diagnosis. This case highlights the inconsistencies in the reported IHC profile of this entity. More studies are required to elaborate the presentations of this RCC subtype with numerous histologic mimics including the spectrum of immunostaining patterns to better assist patient care for this uncommon variant.

Astroblastoma: A molecularly defined entity, its clinico-radiological & pathological analysis of eight cases and review of literature

Astroblastoma, a unique entity of glial tumor, predominantly occur in young women with distinctive MN1 rearrangement, Given its limited documentation in existing literature, we report eight cases of astroblastoma, detailing their clinical, radiological, and histopathological characteristics along with molecular analysis. We conducted a retrospective analysis of our neuropathology archive database spanning the past 8 years. We included all cases that underwent Magnetic Resonance Imaging (MRI), surgical resection, histopathological examination, molecular testing, and follow-up. Histopathological examination involving immunohistochemistry and Fluorescence In Situ Hybridization (FISH) was carried out for all cases. All tumors were found to be located in the supratentorial region (cerebral hemisphere). The median age of the group was 35.1 years, with a female-to-male ratio of 1.6:1. The most common clinical presentation was headache. Morphologically, all tumors exhibited astroblastic features with pseudorosettes and perivascular hyalinization. Immunohistochemistry consistently revealed positivity for EMA and variable immunoreactivity for GFAP, OLIG2, and D2–40. Fluorescence In Situ Hybridization (FISH) analysis conducted for all cases showed MN1 rearrangement in 7 cases. The mean follow-up period was 45 months (ranging from 12 to 105 months). Radiotherapy was administered for high-grade and recurrent astroblastomas. All patients are currently alive and in good health.Astroblastomas are uncommon central nervous system (CNS) tumors with characteristics morphology and molecular signatures. They typically carry a favorable prognosis. High level suspicion is required for their diagnosis and molecular analysis is must to distinguish them from other morphological mimics.

Pneumocystis jirovecii pneumonia in hemato-oncological patients; 10 years of experience at Hospital Infantil Teletón de Oncología

Abstract Background Pneumocystis jirovecii pneumonia is an opportunistic infection that most frequently affects immunocompromised people, especially patients with HIV; clinical presentation is nonspecific and mortality rate in patients without HIV ranges are between 30% and 75%. In the international literature, there are few case reports of pediatric oncology patients. A series of cases is presented with the objective of describing the epidemiological characteristics, clinical presentation, treatment, and outcomes of Pneumocystis jirovecii pneumonia in this population group. Methods An observational, descriptive, and retrospective study was carried out in cancer patients under 18 years, with clinically compatible symptoms, an imaging study suggestive of the pneumonia, and identification of P. jirovecii using a PCR test of bronchial aspirate at Hospital Infantil Teletón de Oncología in Querétaro Mexico, since November 2014 to June 2023. Results A total of 17 events were reported with a median age of 9.8 years, 14/17 (82%) with a diagnosis of acute leukemias, 1/17 (6%) Ewing sarcoma, 1/17 (6%) lymphoma large cell anaplastic and 1/17 (6%) neuroblastoma. Regarding their therapeutic phases, 7/17 (41%) were in consolidation, 4/17 (23%) induction, 3/17 (18%) allogeneic HSCT (Hematopoietic stem-cell transplantation), 2/17 (12%) in surveillance, and 1/17 (6%) in maintenance. The clinical presentation in our patients was characterized by the presence of fever in 15/17 (88%), dry cough in 10/17 (59%) and 17/17 (100%) developed acute respiratory distress syndrome, which was classified as mild in 7/17 (41%), moderate in 5/17 (29%) and severe in 5/17 (29%). 13/17 (75%) presented distributive shock. All our patients underwent chest x-rays, 12/17 (70.5%) presented bilateral interstitial infiltrate, 4/17 (23.5%) consolidation, and 1/17 (6%) pleural effusion. Lung CT (computed tomography) was performed in 4/17 (23.5%), and 4/4 (100%) presented ground glass opacities. All of our patients were admitted to the PICU (Pediatric intensive care unit) and 16/17 (94%) required invasive mechanical ventilation. All receive treatment with TMP-SMX (Trimethoprim-sulfamethoxazole). 4/17 patients died (23%) of whom none received prophylaxis. 3/17 patients (18%) received prophylaxis with trimethoprim-sulfamethoxazole (TMP-SMX). Conclusion The present study is the first study in Mexico that compiles cases of Pneumocystis Jirovecii pneumonia in pediatric oncology patients. P. jirovecii pneumonia remains a rare cause, but represents a potentially fatal cause in oncology patients, so it is important to have a high clinical suspicion during the evaluation of an immunocompromised patient with evidence of pneumonia during all treatment phases with chemotherapy, in order to start treatment promptly. Thus, we also emphasize the importance of adequate prophylaxis as the most important preventive measure.

Myeloid sarcomas with CBFA2T3 : GLIS2 fusion: clinicopathologic characterization of 4 cases mimicking small round cell tumors.

Acute myeloid leukemia with CBFA2T3::GLIS2 fusion can initially present as extramedullary lesions (myeloid sarcoma), leading to a misdiagnosis of nonhematologic pediatric solid tumors. We characterized the clinicopathologic features of 4 cases of CBFA2T3::GLIS2 fusion-positive myeloid sarcoma in pediatric patients where the sarcoma presented either without leukemic involvement (isolated myeloid sarcoma; 3/4 [75%]) or had concurrent leukemic disease (1/4 [25%]). All cases mimicked nonhematopoietic tumors at morphologic and immunophenotypic levels, so the initial evaluation did not raise suspicion for acute myeloid leukemia/myeloid sarcoma. After extensive workup, however, including molecular studies, the diagnosis of myeloid sarcoma with CBFA2T3::GLIS2 fusion was rendered. This study highlights the need for a high suspicion index of GLIS2-rearranged myeloid sarcoma in the differential diagnosis of pediatric small round cell tumors in tissue biopsies and the application of adequate workup to avoid misdiagnosing this entity.

Severe hydronephrosis complicated with primary aldosteronism: a case report and review of the literature

BackgroundPrimary aldosteronism is characterized by high plasma aldosterone and low renin. The plasma aldosterone-to-renin ratio is recommended for screening. Severe hydronephrosis leads to renal parenchymal ischemia, resulting in increased renin secretion. Since nonsuppression of renin may cause a negative result in the aldosterone-to-renin ratio test, severe hydronephrosis and primary aldosteronism occurring simultaneously in a patient are challenging to diagnose.Case presentationA 54-year-old Chinese man of Han ethnicity was diagnosed with hypertension and severe hypokalemia (minimum 1.57 mmol/L) 13 years prior, and was also diagnosed with severe hydronephrosis due to congenital ureteral stenosis on the left side. His clinical features suggested primary aldosteronism, but the aldosterone-to-renin ratio result of the patient was negative every time he underwent the primary aldosteronism screening test. No further treatment for primary aldosteronism was performed, which led the patient to suffer from severe hypokalemia, such that he was taking 12–15 g/day potassium chloride orally to keep his blood potassium between 3.0 and 3.5 mmol/L (reference value, 3.5–5.5 mmol/L) for 13 years, and the patient needed to be hospitalized in the intensive care unit for rescue several times. At admission, although the aldosterone-to-renin ratio result of the patient was negative, we still did the saline stress test and captopril inhibition test, and the results showed that the plasma aldosterone level was not lower after the test than before the test. Adrenal enhanced computed tomography suggested an adenoma in the left adrenal gland, and the results of adrenal vein sampling suggested that the left side was the dominant side. Therefore, laparoscopic total resection of the left adrenal gland was performed, and 2 weeks later, the patient developed short-term renal function impairment and hyperkalemia, but his renal function and blood potassium returned to normal after treatment that included fluid rehydration. The patient’s biochemical test results and clinical symptoms were completely normal after 1 year.ConclusionWe suggest that for patients with a high suspicion of primary aldosteronism in the clinic, comprehensive analysis must be performed in combination with clinical characteristic assessments, such as severe hydronephrosis, if renin is within the normal range or if the aldosterone-to-renin ratio result is negative at screening and diagnostic tests, and adrenal vein sampling should be performed if necessary. It can help avoid misdiagnoses and contribute to the treatment of patients with severe hydronephrosis and primary aldosteronism.

12393 Myxedema Presenting With Impending Cardiac Tamponade : A Case Report

Abstract Disclosure: A. Hoskote: None. V. Gupta: None. J. Seidenberg: None. The widespread availability of thyroid stimulating hormone (TSH) assays, routine screening and affordable replacement therapy has resulted in decreased prevalence of complications of untreated hypothyroidism. “Myxedema heart” is a rare syndrome that results in bradycardia, cardiomyopathy and pericardial effusion. We present a rare case of cardiac tamponade due to myxedema in a patient with unidentified hypothyroidism. A 49-year-old male was brought to the ER by his family for several weeks of cognitive slowing, weakness and inappropriate behavior involving defecating on himself eating old food and barricading in his room. The patient denied past medical or psychiatric history, medication use or recent physician evaluation. On exam he had macroglossia, was disheveled, forgetful but alert with slow speech, ataxia and muscle wasting. He was afebrile and hemodynamically stable. EKG showed sinus bradycardia. Labs were notable for sodium 133, hemoglobin 8.8g/dL and TSH was incidentally >100U. Levothyroxine was begun. The next day, he was noted to have increased lethargy, bradycardia and hypoglycemia and was transferred to the intensive care unit with concern for myxedema coma. Free T4 was <0.3 ng/dL (0.6-1.6) and free T3 was 1.6pg/mL (2.5-3.9). An echocardiogram revealed a large circumferential pericardial effusion with right atrial and ventricular collapse with tamponade. Treatment with 5 mcg liothyronine, intravenous levothyroxine and stress dose hydrocortisone was begun. A pericardial drain was placed, draining 980mL of sanguineous fluid. After clamping trials, the drain was removed 48 hours later. Labs revealed thyroglobulin <0.1ng/mL (1.6-50), thyroid peroxidase (TPO) antibody 574 IU/mL (<9), thyroglobulin antibody >2500 (<4) and thyroid ultrasound showed a normal sized thyroid gland with heterogenous echogenicity confirming a diagnosis of Hashimoto’s thyroiditis. Serial echocardiograms during the admission and a follow-up two months later showed a stable posterior effusion with no recurrence of the anterior pericardial effusion. Gradually mentation, hemodynamics and electrolyte derangements improved, and he was discharged on oral levothyroxine. TSH values showed sequential improvement to 48U in 15 days and to 11U in 2 months. Myxedema is a manifestation of severe hypothyroidism. The pericardium, a collagen-rich structure, is susceptible to myxedematous infiltration, leading to pericardial effusion. While small pericardial effusions are common with chronic hypothyroidism, a large pericardial effusion with tamponade is a rare life-threatening complication that requires prompt recognition and management. Clinicians must promptly identify myxedema and replace thyroid hormone to prevent progression of pericardial effusion to tamponade, maintaining especially high suspicion in those at risk of poor access to healthcare or inadequate health literacy. Presentation: 6/3/2024

8248 Glucocorticoid Induced Adrenal Crisis Masked By Metastatic Lung Carcinoma

Abstract Disclosure: N. Abrahimi: None. A. Abrahimi: None. Background: Chronic glucocorticoid use of three weeks or longer can cause hypothalamic-pituitary-adrenal axis inhibition by negative feedback, causing adrenal suppression. (1) Adrenal crisis is diagnosed by an acute clinical decompensation, persistent hypotension that resolves with high dose steroid administration. (2) Adrenal crisis is precipitated by several causes such as illness, dehydration, trauma, emotional distress and sometimes due to no identifiable cause. (2) Clinical Case: 64-year-old male past medical history of lung carcinoma metastatic to mediastinum who presented to the hospital with tachycardia. On presentation, patient was tachycardic, tachypneic, and hypotensive. Labs significant for WBC 22, Na+ 129, K+ 3.2, Cr 3.4. Patient received 2.5 L bolus of NS and was given one dose of azithromycin due to concern for sepsis. After fluid resuscitation, hypotension and tachycardia resolved. Presenting symptoms included ongoing nausea, decreased appetite and malaise for several weeks. Repeat labs post fluid resuscitation showed resolution of leukocytosis, improvement of AKI, and repletion of electrolytes. CT chest abdomen pelvis showed questionable pneumonia, however, consolidations were difficult to distinguish from known malignant masses. Clinical picture was not consistent with pneumonia and so additional antibiotics were held. Several hours later, blood pressure decreased, and patient began requiring vasopressors. Cefepime was started given concern for septic shock. Nearly 48 hours after initiation of broad-spectrum antibiotics hypotension persisted and patient remained on vasopressors. Given clinical decline, etiology of shock was reconsidered. Patient reported daily prednisone use over the last several months for COPD management. Given high clinical suspicion of adrenal crisis in setting of chronic steroid use and glucocorticoid withdrawal, patient was started on hydrocortisone 100 mg every 8 hours. Shortly after initiation of stress dosed steroids, the patient was successfully weaned off vasopressors with complete resolution of hypotension. Conclusion: Presentation and symptoms of adrenal insufficiency and crisis including nausea, anorexia, fatigue, malaise, hypotension and electrolyte abnormalities are nonspecific, can go unrecognized and be masked by other disease processes, such as metastatic cancer. (1) Adrenal crisis is life threatening and must be diagnosed and treated early to prevent mortality. (2) Therefore, adrenal crisis should always be on the differential in patients who present with shock.

6983 Pembrolizimab Induced Hypophysitis: Uncommon Side Effect Leading To Late Diagnosis

Abstract Disclosure: T. Chaudhary: None. O. Syed: None. A. Shridar: None. R. Kaur: None. O. Chaudhary: None. M.S. Zubair: None. Background: Pembrolizumab, an immune checkpoint inhibitor, has shown efficacy inducing remission in various malignancies. Associated immune-related adverse effects (irAEs) have been recorded but cases of concomitant hypophysitis and thyroiditis are rare. Clinical case: The patient is a 41-year-old male with a past medical history significant for metastatic melanoma of the left calf, status post sentinel lymph node biopsy and left inguinal lymph node dissection, treated with pembrolizumab for one year. He initially presented with a severe headache that was preceded by two months of fatigue and impaired concentration. CT and MRI head were both unremarkable while lab work was significant for cortisol 0.2 ug/dL and adrenocorticotropic hormone (ACTH) 1.1 pmol/L. A full hormone panel was ordered which showed normal insulin-like growth factor-1 (IGF-1), luteinizing hormone (LH), and follicle-stimulating hormone (FSH). Free testosterone was at the lower end of normal. Prolactin was elevated at 27.3 ng/mL. The thyroid function tests showed primary hypothyroidism with elevated thyroid stimulating hormone (TSH) at 109 uIU/mL, low T4 at 1.5 ug/dL, and low free T4 at 0.4 ng/dL. There were no signs or symptoms of posterior pituitary dysfunction. The patient was diagnosed with hypophysitis and started on hydrocortisone 20 mg once daily along with levothyroxine 175 mcg once daily. DEXA scan showed normal bone mineral density. Subsequently, the patient presented to our care and reported symptom improvement after taking the prescribed medication however he continued to develop fatigue by the end of the day. Therefore, hydrocortisone prescription was switched to 15 mg in the morning and 10 mg at noon. A repeat thyroid function panel, sex hormone binding globulin, and LH were ordered, to be followed up at the patient's next visit. The patient was also advised to wear a medical alert bracelet at all times moving forward. Conclusion: Due to non-specific symptoms at onset, hypophysitis can be undiagnosed or misdiagnosed therefore it is important to have a high clinical suspicion with patients on PD-1 inhibitors presenting with such symptoms. It is also imperative to order a full set of hormones as this patient had concomitant primary hypothyroidism. Presentation: 6/1/2024

7617 Two Cases of Severe Beta-hCG-Mediated Hyperthyroidism with Gestational Trophoblastic Disease

Abstract Disclosure: A. Morvant: None. M. Mashayekhi: None. Introduction: Hyperthyroidism is most commonly caused by Graves’ disease and toxic nodules and affects 2.5% of the world’s population. Hyperthyroidism due to elevations in beta-hCG (b-hCG), which has thyroid-stimulating activity due to homology with TSH, is a rare process that requires high clinical suspicion. We present two cases of hyperthyroidism caused by severely elevated b-hCG due to gestational trophoblastic disease (GTD), with rapid resolution of hyperthyroidism after treatment of the underlying conditions. Clinical Cases Case 1: A 35-year-old at 28-weeks gestation presented with dyspnea, palpitations, and 6-pound weight loss over the prior month. Labs revealed b-hCG 5.2 million (0-5 mIU/mL), TSH <0.015 (0.35-3.6 mcU/mL), and free T4 2.42 (0.7-1.37 ng/dL). Imaging revealed extensive pulmonary lesions concerning for metastatic disease and a 2.4 cm ovarian mass. Due to worsening respiratory distress and the need to start chemotherapy urgently, she underwent Cesarean section to deliver the viable infant and take out the ovarian mass. Pathology confirmed choriocarcinoma. Methimazole was started post-operatively, and she started chemotherapy. Labs after cycle one of chemotherapy showed b-hCG 1.3 million, TSH <0.015, and free T4 1.46. The methimazole dose was decreased and ultimately stopped as free T4 levels decreased with subsequent chemotherapy cycles. After three cycles, labs showed b-hCG 4,652, TSH 1.55, and free T4 0.68. She was discharged without thyroid-targeting therapy. Case 2: A 33-year-old woman at 12-weeks gestation presented with nausea, vomiting, and 10-pound weight loss over 1 month. Labs showed TSH <0.015, free T4 2.39, and total T3 351 (58-160 ng/dL), which were thought to be due to hyperemesis gravidarum. Two months later she presented with vaginal bleeding and imaging showed an enlarged placenta with a moth-eaten appearance and theca lutein cysts concerning for partial molar pregnancy. Beta-hCG level was 3.3 million, TSH <0.015, free T4 1.76, and total T3 289. She underwent dilation and evacuation and pathology confirmed partial hydatidiform mole. Beta-hCG level decreased to 1.8 million and free T4 normalized after surgery, with b-hCG 3,300, normal TSH, and normal total T3 one month later. Conclusion: These cases highlight the importance of considering possible GTD when evaluating a patient with hyperthyroidism, particularly in women of childbearing age and cases of early pregnancy when the degree of hyperthyroidism seems out of proportion to that expected. Clinicians should consider checking a b-hCG level in consultation with an obstetrician when indicated. Treatment is aimed at the GTD and can yield rapid resolution of hyperthyroidism.

8197 A Captivating Case Report of Teprotumumab Induced Hyperglycemic Hyperosmolar State

Abstract Disclosure: K.M. Tuna: None. J.P. Perdomo Rodriguez: None. Introduction: Teprotumumab, a monoclonal antibody targeting the IGF-1 receptor, is increasingly utilized for moderate to severe thyroid eye disease. Initial trials noted mild hyperglycemia in patients with diabetes, while recent trials revealed a significant elevation in Hemoglobin A1c (HbA1c) among those with diabetes or pre-diabetes post-infusion. Here, we discuss a case of a patient with diet-controlled diabetes who developed hyperglycemic hyperosmolar state (HHS) two months after Teprotumumab infusion. Case Presentation: A 43-year-old male, with class III obesity, obstructive sleep apnea, dyslipidemia, coronary artery disease, and diet-controlled diabetes (HbA1c 6.5%) presented with worsening proptosis, decreased color vision, severe visual field depression. Laboratory evaluation revealed elevated TSH (6.3 mIU/ml), normal free T4 (0.67 ng/dl, ref: 0.60-1.2 ng/dl), and undetectable Thyroid Stimulating Immunoglobulin (<0.1 IU/L). Blood cell count, renal and liver function tests were normal. Due to high clinical suspicion of thyroid eye disease, he was treated with timolol, dorzolamide, brimonidine, and methylprednisolone 1g daily for 3 days. Glycemic control was achieved with 30 units of daily insulin while on pulse steroids. On outpatient ophthalmology follow-up, Teprotumumab was initiated and he received 2 doses. Eight weeks later, the patient was hospitalized with altered mental status caused by HHS (glucose 617 mg/dl, serum osmolality 316 osm). HbA1c was found to be significantly increased to 12% and the patient required 150 units total daily insulin and metformin upon discharge. Of note, the patient did not have significant weight changes during this time. GAD, IA2, and Zinc Transporter-8 antibodies were obtained prior to discharge and results are pending. Discussion: The IGF-1 receptor, a partial homologue of the insulin receptor, plays a pivotal role in glucose homeostasis. Inhibiting this receptor can lead to hyperglycemia. Initial randomized controlled trials (RCTs) reported a 10% incidence of mild hyperglycemia. A recent RCT from a cohort of 42 patients demonstrated a 50% incidence of hyperglycemia, in which two-thirds of the patients remained in a dysglycemic state with one patient with pre-existing prediabetes developing diabetic ketoacidosis (DKA). Our patient mirrored this scenario with no identifiable confounding factors. Conclusion: Teprotumumab-induced hyperglycemia, though rare, can lead to life-threatening complications. Baseline glycemic assessment, identification of high-risk patients, and comprehensive discussions on risks and benefits are crucial before initiating therapy. Close monitoring, especially in those with prediabetes, is recommended. Patient education plays a pivotal role in early symptom recognition, averting delayed diagnoses, and subsequent hospitalization. Presentation: 6/3/2024

8437 Improved Quality Of Life&Blood Pressure Following Diagnosis And Medical Management Of Primary Aldosteronism (PA) / Georgian Experience

Abstract Disclosure: Q. Gvazava: None. N. Zavrashvili: None. N. Shonia: None. Improved Quality of Life and Blood Pressure Following Diagnosis and Medical Management of Primary Aldosteronism (PA) - Georgian Experience Introduction: PA is the most common cause of secondary hypertension which still remains as an underdiagnosed condition in many countries, including Georgia. The choice of pharmacological or surgical therapy depends on the results of computed tomography scans of the adrenal glands and adrenal venous sampling. In patients with bilateral aldosterone hypersecretion, the optimal is a low-sodium diet and lifelong treatment with a mineralocorticoid receptor antagonist administered at a dosage to reach a high-normal serum potassium concentration. Discussion: 60 y/o male with a 17-year history of hypertension. He was treated with 3 drug-program: valsartan, amlodipine, bisoprolol. BP was not adequately managed with systolic BP >140 mm/Hg. Patient had a history of hypokalaemia and required regular potassium supplements. Patient had never been evaluated for possible PA. We planned case detection test, which came up positive, with increased aldosterone, suppressed renin concentration and increased aldosterone-renin ratio. Abdominal CT performed 1 year ago for another reason revealed “normal” adrenals.After discussing treatment options patient preferred medical management and Eplerenone was started, dose titrated accordingly, K supplements were discontinued which over time resulted in decrease in requirement of his other antihypertensive medications, with BP adequately managed. Patient’s quality of life improved significantly, he has shared his test results and progress on his follow up visits; He is very grateful and feels content with the treatment. We present another case of 49 y/o male patient with a 19 year history of hypertension. Patient was treated with 4 drug-regimen: Valsartan, Hydrochlorothiazide, Amlodipine, nebivolol with average BP findings – 140/90 mm/Hg.Case detection test was positive with increased aldosterone, suppressed renin and increased aldosterone-renin ratio. Patient required confirmatory testing. Salt loading was done which confirmed the diagnosis of PA. On unenhanced CT of the abdomen, bilateral adrenal hyperplasia was found. Patient was started on Eplerenone, on follow up, his BP findings are significantly improved, number of BP meds are also decreased. Conclusion: Diagnosis of PA provides clinicians with unique opportunity to cure or effectively improve hypertension and hypokalaemia, as well as to prevent potential target organ damage and complications. That’s why the importance of increased awareness and having high clinical suspicion cannot be understated. Presentation: 6/3/2024

7510 Use Of Tumor Markers And Surgery In The Management Of Medullary Thyroid Cancer During Pregnancy

Abstract Disclosure: M. Garver: None. C. Vaz: None. Introduction: Thyroid cancer is the second most diagnosed cancer during pregnancy, predominantly differentiated thyroid malignancies. [1] MTC during pregnancy is rare and challenging to manage due to its aggressive nature. We describe the successful diagnosis and management of MTC during pregnancy. Clinical Case: A 28 yo woman presented with a rapidly growing neck lump for 2 months with an associated unintentional 10lb weight loss and without compressive symptoms. She had no history of childhood radiation exposure or family history of thyroid cancer. Physical exam revealed a hard L lobe thyroid nodule without palpable lymphadenopathy. The patient was 5 weeks pregnant at the time. A neck US revealed a 4.2x2.7x2.0 cm L solid isoechoic thyroid nodule with punctate echogenic foci and microcalcifications. The nodule was classified as TIRADS 4 and ATA high suspicion. FNA noted Bethesda IV with positive IHC stains for chromogranin and calcitonin. Afirma MTC classifier was positive, confirming diagnosis of MTC. At 7 weeks gestation, calcitonin was markedly elevated at 3508 pg/mL (n <5 pg/mL), CEA 29.5 ng/mL (n <2.5 ng/mL) and calcium 10.3mg/dL (8.6-10.2mg/dL). Plasma normetanephrines were elevated to176 pg/mL (n <148 pg/mL) with normal metanephrines 28 pg/mL (n <57 pg/mL) and normal 24-hour urine metanephrines. CT neck revealed 3.2x2.8cm L thyroid nodule without cervical lymphadenopathy. Due to pregnancy status, additional imaging to assess for metastatic disease was deferred until after delivery. Genetic testing did not identify mutations within the thyroid cancer panel including APC, CHEK2, DICER1, MEN1, PRKAR1A, PTEN, RET, SDHB, SDHD, and TP53. The patient underwent total thyroidectomy with neck dissection at 19 weeks gestation without complication. Two weeks postoperative Calcitonin was 2 pg/mL(n <5 pg/mL) and CEA was undetectable. Conclusions: The effect of pregnancy on MTC is not well studied because of low incidence. Guidelines on the timing of surgery for MTC diagnosed during pregnancy are lacking. While evidence confirms that surgery can be safely postponed until after delivery for low risk differentiated thyroid malignancies, there is no consensus on timing of surgery for MTC. Given the aggressive nature of MTC, along with marked elevation of calcitonin and CEA in this case, surgery was performed in the 2nd trimester with excellent outcome. Data is conflicting on accuracy of calcitonin and CEA during pregnancy, with some reports suggesting falsely high levels due to pregnancy. The sharp decline in calcitonin and CEA 2 weeks postoperatively suggested these were truly elevated due to MTC and unaffected by pregnant status. We suggest that calcitonin and CEA be followed similar to non-pregnant cases with MTC. Resources: 1.Khaled, H. M., Lahloubi, N. A., & Rashad, N. (2016). A review on thyroid cancer during pregnancy: Multitasking is required. Journal of Advanced Research, 7(4), 565–570. Presentation: 6/2/2024

8373 High suspicion for thyroid cancer in patient with thyroid Hemiagenesis

Abstract Disclosure: L. Aoun: None. S. Almardini: None. M. Anise: None. F. Haddadin: None. F. Saliba: None. J. Zaidan: None. Background: Thyroid Hemiagenesis (THA) is a rare condition characterized by congenital absence of one lobe of the thyroid gland. Notably, THA is more prevalent in females and the left lobe is more likely to be absent with or without the isthmus. Most patients are euthyroid, and diagnosis is usually incidental, so the true prevalence of THA is uncertain as there are certainly undiagnosed cases. Studies have shown a link between THA, thyroid cancer, and hyperparathyroidism. We present a case of THA and high suspicion for thyroid cancer. Case Presentation: 54-year-old female with history of Rheumatoid arthritis who presented for evaluation of incidental thyroid nodules, occasional palpitations, tremors, and mild change in her voice. She denied abnormal weight loss or diarrhea. No history of radiation to the neck and no family history of thyroid disease or thyroid cancer. The thyroid exam showed an enlarged left palpable nodule. Labs significant for TSH 1.6 mIU/L (normal: 0.4-4 mIU/L), FT4 0.8 ng/dl (normal 0.7-1.9 ng/dL) and T3 77ng/dL (normal: 60-180 ng/dL). Thyroid U/S showed left hemiagenesis and right sided nodules biggest is 1.1 cm solid, hypoechoic with coarse calcifications, FNA was recommended and showed follicular lesion of undetermined significance, molecular testing was done and Thyroseq came back intermediate to high risk RAS and EIF1AX positive reflecting a 70 % probability of cancer. Based on these results, surgery was recommended, and the patient underwent right thyroidectomy. Surgery was uncomplicated and despite reported higher risk of hyperparathyroidism patient’s calcium and PTH were normal, and no parathyroid adenomas were identified intraoperatively. The pathology from the thyroidectomy showed follicular adenoma of 1 cm and no malignancy. She was started on Synthroid 100mcg daily post op. Discussion: This case demonstrates a case of a benign thyroid nodule and preserved parathyroid levels in the setting of thyroid hemiagenesis. Usually in THA, the contralateral lobe is at an increased risk for pathology of compensatory hypertrophy or hyperplasia. According to available studies, cancer association with THA was more common in left hemiagenesis and parathyroid adenomas more common ipsilateral to hemiagenesis . Despite Our patient’s FNA revealed atypical findings, molecular testing revealed RAS and EIF1AX positive, marking intermediate to high cancer risk. However, the thyroidectomy pathology report unveiled no evidence of malignancy, contraindicating the anticipated high cancer risk in those with left THA. This case highlights the rarity of THA and different possible presentations and cancer risks. Presentation: 6/2/2024

12586 A Case Of Ventricular Fibrillation In Thyroid Storm

Abstract Disclosure: A.A. Luzuriaga Chavez: None. N. Aamir: None. J.P. Hidalgo: None. A. Rao: None. N. Patel: None. Thyrotoxicosis is a life-threatening condition caused by an excessive level of circulating thyroid hormones. It can lead to high mortality and morbidity, even if diagnosed and treated in its early stages. Symptoms can include fever, irritability, tachycardia, diarrhea, and seizures. Levothyroxine is a medication commonly used for hypothyroidism, but cases of acute overdose have been reported worldwide. This is a case of a 55-year-old woman who was admitted after a cardiac arrest caused by a possible overdose of 8 mg of levothyroxine. Case presentation:A 55-year-old woman with a history of major depressive disorder was found unconscious by her boyfriend next to a bottle of levothyroxine (which contained a 3-month supply) and was rushed to the emergency department (ED) by EMS. According to the patient's medical chart and family, she did not have a history of hypothyroidism. In the field, she suffered a V-fib cardiac arrest but was successfully resuscitated and brought to the ED. However, she had another cardiac arrest upon arrival but was revived again after 20 minutes. During the physical examination, the patient did not exhibit any signs of thyroid enlargement, exophthalmos, or jaundice. The initial electrocardiogram showed concerning signs of ST-elevation myocardial infarction so the patient was started on heparin drip and continuing amiodarone. The patient was then transferred to the ICU for post-arrest care. Bloodwork revealed TSH <0.005, T3 15.74, and T4 1.57. The Burch-Wartofsky point scale indicated a score of 55, which is consistent with a high suspicion of thyroid storm. The patient's condition continued to worsen, with increasing vasopressor requirements and hyperthermia. Despite the amiodarone drip, the patient's heart rate continued to be less than 100 beats per minute, propranolol could not be started. Furthermore, the patient started bleeding from the nasogastric tube, and the medical team was unable to start any oral propylthiouracil or methimazole. Consequently, they administered hydrocortisone 300 mg, followed by 100mg every 8 hours. The patient was transitioned to comfort care measures. Discussion:Massive ingestion of levothyroxine can lead to excessive thyroid hormone levels and cause sympathetic excitation.Reports of adults ingesting levothyroxine are rare, and those who do generally experience more severe symptoms such as cardiac arrhythmias, malignant hyperthermia, and renal failure. Excessive thyroid hormone levels lead to increased beta adrenergic receptors within the myocardium, rendering it more susceptible to both vagal and sympathetic stimulation. A few isolated case reports show thyrotoxicosis was associated with ventricular fibrillation. Thyroid storm can occur a few days after an overdose of levothyroxine. However, in this case, the patient developed thyrotoxicosis within 6 to 8 hours of ingestion, making it an interesting case to study. Presentation: 6/1/2024

6857 Adrenal Incidentaloma In A Patient With Surgically Treated Lung Adenocarcinoma A Decade After Complete Remission

Abstract Disclosure: L. Kaur: None. R. Kaur: None. F. Haider: None. A. Iqbal: None. N. Patel: None. Adrenal incidentaloma is an adrenal mass >1 cm in size commonly discovered incidentally in up to 5-7% of patients on imaging performed for reasons other than adrenal causes. Metastasis to the adrenal glands is common due to their rich blood supply, with lung cancer being the most common primary site, followed by the stomach, esophagus and the liver. Bilateral adrenal metastasis is common, however isolated metachronous or synchronous adrenal metastasis is rare, with an incidence of 1-6%. We present a case of incidentally discovered isolated right adrenal mass ten years after curative surgical resection of right lung adenocarcinoma. A 74 year old male with history significant for adenocarcinoma of the right upper lung lobe stage 1A , negative for metastasis status post lobectomy and no adjuvant chemotherapy or radiation 10 years ago presented to the hospital for his routine follow up evaluation. His vitals were within normal limits and physical examination was unremarkable. The follow up diagnostic CT imaging was done which showed an incidental 2.1 x 1.6 x 1.2 cm right adrenal mass without any pathological lesion or lymphadenopathy. A review of CT scan done one year ago revealed no prior adrenal mass or other suspicious findings. The patient was referred to endocrinology for evaluation of the adrenal mass. He denied abdominal or right flank pain, headaches, palpitations, elevated blood pressure, sweating, muscle weakness, weight gain/loss and was healthy appearing. Lab work up including biochemical studies for hormonal over production, including aldosterone-renin ratio, adrenocorticotropic hormone, plasma catecholamines with urine metanephrines and urine cortisol were within physiological ranges. CT scan was repeated after 2 months which revealed right adrenal mass of 3.2 x 1.8 x 1.7 cm in size with heterogeneity and high pre contrast density. Given the patient's history of lung cancer, imaging characteristics and rapid interval increase in size, there was high suspicion for metastatic disease. Other differentials considered due to fast growth rate included adrenocortical carcinoma and less likely adrenal adenoma. A decision was made to proceed with right adrenalectomy for definitive resection and histological diagnosis secondary to quickly growing adrenal mass with no lymphadenopathy. The patient underwent laparoscopic adrenalectomy and the final pathological report was consistent with metastatic lung adenocarcinoma. Postoperatively the patient recovered well. At 2 week and 3 month follow up he reported no concerns. Isolated adrenal metastasis is unusual and rare but may occur in lung cancer patients years after curative resection of the primary tumor and hence long term annual follow up imaging and monitoring is recommended for prompt diagnosis and timely treatment. Presentation: 6/2/2024

6668 Myxedema Coma and Takotsubo Cardiomyopathy

Abstract Disclosure: A.M. Skariah: None. S. Jain: None. Background: Takotsubo cardiomyopathy (TCM) also know as broken heart syndrome is a transient decrease in systolic cardiac function in the absence of obstructive CAD. TCM is likely related to hyperadrenergic conditions leading to increased cardiac sensitivity to catecholamine surge leading to cardiomyopathy. TCM is more associated with hyperthyroidism. Case presentation: Patient is an 84-year-old female with PMH of celiac disease,hypertension,prediabetes, and atrial fibrillation s/p cardioversion, on amiodarone. She was brought to the ER after having an unwitnessed fall. She was hypotensive 76/56 mm of Hg, hypothermic 35.5 degree C, HR 90 beats/min; labs significant for hyponatremia, macrocytic anemia, TSH 65.47uIU/ml, FT4 <0.4 ng/dl and mild QTC prolongation of 469ms. Family mentioned history of constipation, progressive weakness, poor oral intake, memory problems and cold sensitivity for a couple of months. Started on 1 pressor, 3 units of PRBC and 2 liters of IVF; BP improved; labs and history suggestive of myxedema coma with myxedema coma Popoveniuc score 85 suggestive of high suspicion for myxedema coma.She had TSH 50.39 uIU/ml and FT4 of 0.4 ng/dl for her memory issues workup 2 months prior to presentation but was not started on medication. Upon admission, started stress dose steroids followed by IV levothyroxine 100 mcg. Not started on LT3 given her old age and atrial fibrillation history. Received additional dose of LT4 100 mcg within few hours. TFT improved with TSH 35 uIU/ml and FT4 1 ng/dl after 2 doses. Later same day, shock worsened, and patient was on 3 pressors along with stress dose of steroids. Cardiology was consulted; recommended an Echocardiogram.Echo was done on day 2; EF 55-60 % with severe hypokinesis of apical myocardium consistent with TCM. Normal echocardiogram studies 4 months ago.Started on weight-based regimen of LT4 75 mcg IV on day 3 and switched to 100 mcg oral when patient tolerated pills.Patient was down to 2 pressors on day 3 and gradually off pressors by day 6. Repeat TFT -showed improvement TSH 12 uIU/ml and FT4 0.7 ng/dl—day 5. Eventually her mentation improved and was discharged on oral LT4. Conclusion: TCM is rare complication of treatment of myxedema coma. Our patient developed shock requiring 1 pressor which unexpectedly worsened to 3 pressors requirement after initial LT4 treatment. High doses of LT4 and LT3 which is part of myxedema coma treatment sensitizes myocardial tissue to catecholamines by increasing expression of beta adrenoreceptors in cardiac myocytes with subsequent ionotropic and chronotropic effects. This may contribute to cardiac stunning and TCM. It’s beneficial to evaluate patients with myxedema coma who develop worsening cardiac status after initiation of high dose of thyroid hormone replacement for TCM. Presentation: 6/2/2024

6588 Insulin Autoimmune Syndrome Presenting with Severe Insulin Resistance and Recurrent Diabetic Ketoacidosis

Abstract Disclosure: E. Looi: None. R. Rosenberg: None. S. Sugar: None. H. Stone: None. A.W. Michels: None. D. Saxon: None. Background: Insulin autoimmune syndrome (IAS) is a rare cause of severe insulin resistance and is typically associated with intermittent hypoglycemia due to insulin antibodies (IA) with high binding capacity and low insulin affinity. However, consideration should be given for atypical presentation of IAS or co-presentation of IAS with type B insulin resistance syndrome (TBIRS) in the absence of hypoglycemia, as described in this case. Case: A 78-year-old African American female (BMI 21 kg/m2) with poorly controlled type 2 diabetes (A1c 11%) experienced recurrent episodes of diabetic ketoacidosis (DKA) despite adherence to escalating insulin doses (310 units daily, 5 u/kg/day). She was referred to endocrinology and continuous glucose monitoring revealed consistently elevated glucose levels (300-400 mg/dL) without hypoglycemia. She had normal renal function, hepatic function, and lipid panel. During a subsequent hospitalization, she required insulin drip rates of 50-70 u/hr. Insulin resistance workup revealed significantly elevated IA level (8.346, normal ≤ 0.010) measured using a fluid-phase radiobinding assay with high insulin binding capacity (1040 U insulin-binding/L blood) and low insulin binding affinity (1.9x107 M), which supported the diagnosis of IAS. Rheumatologic workup for other autoimmune diseases was negative. Due to recurrent DKA episodes, severe insulin resistance without obvious alternative cause, and lack of hypoglycemic events despite low IA binding affinity, co-morbid TBIRS was considered. As there are no commercially available insulin receptor antibody (IRAb) assays, diagnosis could not be confirmed before her condition worsened, and she was admitted for another episode of DKA within a week of being discharged. Due to clinical deterioration and high suspicion for IAS ± TBIRS, treatment with a combination of immunosuppressive agents was initiated in accordance with an NIH protocol (Klubo-Gwiezdzinska J, et al. 2018). Intravenous immune globulin (0.5g/kg/day) was administered over 2 days which resulted in a brief reprieve and followed by significant relapse. Two doses of rituximab (750 mg/m2) and 2 steroid pulses (dexamethasone 40 mg PO daily for 4 days) were then administered 2 weeks apart. Cyclophosphamide 100 mg daily was also started. Her hyperglycemia and insulin requirements subsequently improved, and she was eventually discharged with good glycemic control on a total daily dose of 40 units of insulin (0.5 u/kg/day), an 87.1% reduction in daily insulin requirement. Conclusion: This case demonstrates that IAS - although classically associated with hypoglycemic episodes - may also present with severe insulin resistance and recurrent DKA without hypoglycemia. Whether characterized by high IA binding capacity and affinity or coexisting with IRAb, a combination of immunosuppressants proved effective in treating both TBIRS and IAS in this instance. Presentation: 6/3/2024

6486 Gastroparesis Exacerbation by a GLP-1 Agonist

Abstract Disclosure: J.M. Deirmenjian: None. C. Dagher: None. Gastroparesis is characterized by a delay in gastric emptying that may present with nausea, vomiting, satiety, abdominal pain, and anorexia. The most common cause of gastroparesis is diabetes mellitus due to vagal autonomic neuropathy which prevents coordinated gastric contractions. Medications may also induce gastroparesis. Glucagon-like peptide (GLP)- 1 agonists such as semaglutide are being prescribed for diabetes control but also due to benefits of cardiovascular disease and weight loss. Concerns of GLP-1 agonists causing gastroparesis are valid as the mechanism of action includes delaying gastric emptying, especially with newly developed analogs that are more potent with longer half-lives. Patient is a 43-year-old female with a history of hypertension, uncontrolled diabetes mellitus type 2, diabetic gastroparesis, diabetic neuropathy, hyperlipidemia, and obesity who presented with a one-month history of postprandial abdominal pain, nausea, vomiting, and a 40-pound unintentional weight loss with decreased oral intake. Her abdominal pain was sharp and was exacerbated with eating. While she had several gastroparesis flares in the past, she was in remission for almost two years until she was transitioned from dulaglutide to semaglutide after which she had a recurrent flare after two doses. On physical examination, her abdomen was diffusely tender to palpation.On admission, her hemoglobin A1c was 9.7%. She was made NPO, started on peripheral fluids, and initiated on scheduled metoclopramide given high suspicion of a gastroparesis flare. She underwent a gastric emptying study with the gastric amount retained as follows: 97% (at 1 hour), 88% (at 2 hours), and 79% (at 4 hours). Her hospital course was complicated by persistent symptoms of gastroparesis. Gastroenterology recommended scheduled metoclopramide and erythromycin for motility and dietary adjustments including small volume liquid calories. Her oral intake was insufficient and she opted for total peripheral nutrition until symptoms improved. She was advised to never take a GLP-1 agonist again and should her symptoms not improve the next step would be to pursue a gastric pacemaker. The etiology of the patient’s gastroparesis was uncontrolled diabetes. The proximity between starting semaglutide and symptom onset is compelling as an inciting factor in this flare. This admission’s gastric emptying study results showed higher gastric retention for a longer period of time, which may shed light to the potency of these new GLP-1 analogs. The patient tolerated maximum dose dulaglutide for months but not even two doses of semaglutide. With the race to invent more potent GLP-1 agonists for diabetes control and weight loss on the horizon, knowing their side effects and mechanism of action is crucial. While not listed as a contraindication, there should be hesitancy to offer these agents to anyone with significant gastroparesis. Presentation: 6/2/2024

7099 Severe Hypothyroidism from Potassium Iodide Supplements: A Case of Failure to Escape from Wolf- Chaikoff Effect

Abstract Disclosure: M.G. Hamilton: None. A. Kulkarni: None. P. Lekprasert: None. Introduction: The Wolf-Chaikoff effect (WCE) is a physiological response of the thyroid gland to supraphysiologic iodide levels. Failure to escape the WCE can lead to thyroid dysfunction, and we present such a case in a patient with Hashimoto’s thyroiditis taking high-dose potassium iodide (KI). Case Description: A 44-year-old female with hypothyroidism secondary to Hashimoto’s thyroiditis presented with 10-pound weight gain, extremity swelling, fatigue, and abnormal menstruation. She was prescribed Levothyroxine several years ago but stopped taking it shortly after as she felt no improvement. On physical exam, she was bradycardic with heart rate of 58 bpm. There is no goiter but dry skin and edematous lower extremities. Labs revealed TSH of 127 pg/mL (0.35-4.94), free T4 0.1ng/dL (0.8-1.8), TPO Ab 645 (<9) IU/mL in comparison to her labs 2 months ago of TSH 11 pg/mL and free T4 0.9 ng/dL. Suspicion for worsening hypothyroidism from Hashimoto’s was low given this dramatic change on labwork and clinically. On further questioning, she had been taking KI 130 mg/day (Iodine 100 mg, daily requirement 150 mcg/day) as advised by her homeopathic doctor. The supplements were stopped, and she was re-started on Levothyroxine. After 8 weeks, she reported improvement of symptoms and weight loss. TSH is normal at 3.54pg/ml and free T4 1.2 ng/dL. Discussion:The WCE serves as a protective mechanism to decrease thyroid hormone production in the setting of high iodide levels to prevent development of hyperthyroidism. WCE is explained by the formation of Iodopeptides inhibits thyroid peroxidase (TPO) mRNA and protein synthesis resulting in an inhibition of thyroglobulin iodination. WCE is temporary and expected to wane over a few days to weeks and is known as the Wolf-Chaikoff escape due to down regulation of the sodium iodine symporter (NIS) thereby decreasing the intrathyroidal inorganic iodine concentration and permitting normal TPO activity. Rarely, failure to escape WCE can lead to hypothyroidism due to continued suppression of thyroid hormone synthesis. Risk factors include underlying thyroid diseases i.e. Hashimoto’s or other causes of thyroiditis, use of Amiodarone, Lithium or Iodine-containing radiocontrast media and KI supplements. In our case, Hashimotos’ thyroiditis predisposed her to thyroid dysfunction and failure to escape from WCE after taking high-dose KI supplements causing severe hypothyroidism. Thus, this report highlights that in clinical practice, especially in patients with pre-existing thyroid dysfunction, a significant TSH increase, a high suspicion index is needed to investigate for other causes like concurrent use of iodine-containing food/drug leading to the failed escape from WCE apart from verifying compliance to thyroid replacement therapy. Presentation: 6/3/2024

12262 Pheochromocytoma Masquerading As An Acute Myocardial Infarction And Takotsubo Cardiomyopathy In A Hispanic Woman: A Case Report

Abstract Disclosure: A. Hoskote: None. J. Seidenberg: None. V. Gupta: None. Pheochromocytoma is a rare neuroendocrine tumor (<1 per 100,000 person-years) that occurs sporadically or due to mutations. While most patients do not have the classic headache, sweating and tachycardia, about 50% have paroxysmal hypertension and 90% have headaches. We present a rare case of pheochromocytoma masquerading as myocardial infarction (MI) and cardiomyopathy. A 67-year-old Hispanic female with history of hypertension and uncontrolled Type 2 DM, presented to the ER for chest pain and diaphoresis. Home medications were metformin, sitagliptin, lisinopril, simvastatin and aspirin. EKG showed non-specific ST elevations in chest leads without STEMI, and labs revealed a dynamic troponin and hemoglobin A1C of 13.6%. CT angiography ruled out pulmonary embolism. A left heart catheterization and echocardiogram revealed non obstructive coronary artery disease (CAD), a left ventricular ejection fraction of 25%, apical ballooning and a laminar thrombus. She was diagnosed with acute myocardial infarction and Takotsubo cardiomyopathy and discharged on metoprolol and apixaban. During our evaluation 7 months later, we noted several ER and hospital admissions for symptomatic hypertensive emergency with a pattern of labile blood pressures and on review of initial CT scan, a sequentially growing left adrenal mass (3.9cm 29HU) was present. A careful history using Spanish interpretation revealed episodic headaches, diaphoresis and palpitations. Subsequently, on two separate occasions, plasma normetanephrine was 2006 pg/mL (ULN<148 pg/mL) and urine normetanephrine was 12516 mcg/g cr (ULN <524 mcg/g cr). Prazosin was prescribed with stabilization of hypertension. An adrenalectomy is now scheduled. Symptoms of pheochromocytoma and paraganglioma (PPGs) are caused by hypersecretion of one or combinations of catecholamines: norepinephrine, epinephrine and dopamine. In one study 27% were discovered due to symptoms while 61% were found incidentally. PPGs occur in up to 1:500 patients with arterial hypertension and can also be a secondary cause of diabetes. MI and acute or chronic cardiomyopathy are rare presenting features of PPGs. Takotsubo cardiomyopathy is the most common phenotype. In one acute MI study, Hispanic women were significantly more likely to receive delayed care as compared to non-Hispanic women, due to misinterpretation of symptoms by patients and providers. A meticulous history with formal interpretation in patients with limited English proficiency can lead to prompt diagnosis of this surgically curable cause of hypertension. Pheochromocytomas rarely present as acute myocardial infarction and stress cardiomyopathy. A high suspicion for PPGs must be maintained in patients with labile hypertension, cardiomyopathy and an incidental adrenal mass. History should be carefully revisited especially in patients with limited English proficiency. Presentation: 6/3/2024