bluuebunny/crossref_metadata_2025_split huggingface dataset:
6516 A Curious Case of Cushing Syndrome
Authors: Bellavia Lauren , Pignanelli Marcelle , Aqbal Daliha 
Published: October 2024

Abstract
Disclosure: L. Bellavia: None. M. Pignanelli: None. D. Aqbal: None.
Background: Cushing Syndrome (CS) from iatrogenic steroid administration is a well- established consequence of steroid use. It is important to recognize the potential of this condition even after a 1-time intraarticular steroid injection received months earlier. Clinical Case: A 40-year-old woman presented for evaluation of rapid weight gain, abdominal stretch marks and excess hair growth. Work-up was consistent with an ACTH-independent etiology of CS, suggestive of exogenous steroid use: ACTH undetectable (&lt; 5 pg/mL, n6-50 pg/mL), 24 hr urinary free cortisol undetectable (&lt; 2 ug/24 hrs, n6-42 ug/24hr), morning cortisol undetectable (&lt; 0.5 mcg/dL, n4-22 mcg/dL). Historical review with patient included thorough review of medications and products without noted steroids. CT Abdomen pelvis completed 12 months prior for unrelated symptoms showed unremarkable adrenal glands without any masses. MRI Brain pituitary was normal. The source of her steroid exposure was still unclear, so a serum synthetic glucocorticoid screen was sent, which revealed elevated levels of Triamcinolone Acetonide (TCA) at 0.22 mcg/dL (n&lt;0.10 mcg/dL). Her pharmacy and her providers were contacted to assess for any TCA exposures, which revealed that she received 40mg of TCA intraarticular injection via ultrasound guided technique into her right hip bursa for hip bursitis 6 months prior to presentation. No other exposures to TCA were found on review. Two months after presentation (8 months after TCA exposure), she began to develop nausea, vomiting, fatigue, and weakness and was thus started on Hydrocortisone 10mg daily for developing adrenal insufficiency. Repeat testing at that time revealed a suppressed adrenal axis, with repeat morning cortisol &lt;0.5 mcg/dL, ACTH &lt; 5 pg/mL and TCA levels of 0.15 mcg/dL. The pharmacokinetics of TCA following intraarticular injections is not well established with variable reports of synovial half-life ranging from 1 day to greater than 3 weeks, with the maximum effect of TCA on cortisol suppression observed with TCA serum levels of &gt;0.19 mcg/dL. No other documented cases of TCA induced CS have demonstrated lasting clinical effects 8 months after TCA exposure. Conclusion: It is important to recognize the potential of rare adverse events that are attributable to the systemic absorption of intraarticular corticosteroids as their pharmacokinetics are not well known. This case raises the need for early general medical education for clinicians and patients about the potential risks of corticosteroids, administered from any route, even after isolated exposures. Reference: Dickson RR, Reid JM, Nicholson WT, Lamer TJ, Hooten WM. Corticosteroid and Cortisol Serum Levels Following Intra-articular Triamcinolone Acetonide Lumbar Facet Joint Injections. Pain Pract. 2018 Sep;18(7):864-870. doi: 10.1111/papr.12686. Epub 2018 Mar 23. PMID: 29436106.
Presentation: 6/3/2024
6853 A Curious Case of MACS
Authors: Dillon Martha , Syeda Asma , Kantorovich Vitaly 
Published: October 2024

Abstract
Disclosure: M. Dillon: None. A. Syeda: None. V. Kantorovich: None.
Introduction: Mild autonomous cortisol secretion (MACS) is characterized by biochemical evidence of mild hypercortisolemia inducing metabolic comorbidities without any obvious phenotypic stigmata of Cushing’s syndrome (CS). This case highlights the challenges faced in the diagnosis and management of MACS. Case: A 76-year-old female with past medical history of class II obesity (BMI 31.83 kg/m2), type 2 diabetes mellitus, hypertension, hyperlipidemia, osteoporosis and chronic kidney disease stage IIIb was diagnosed with MACS after an overnight 1 mg dexamethasone suppression test (DST) showed unsuppressed 8 AM cortisol of 2.4 mcg/dL (&lt;1.8 mcg/dL). Dexamethasone level was appropriate at 355 ng/dL (180-550 ng/dL), ACTH was &lt;5 pg/mL (6-50 pg/mL) and DHEA-S was normal. Late night salivary cortisol (LNSC) levels were elevated to 0.12 mcg/dL (&lt; 0.09 mcg/dL). The 24-hour urine cortisol level was normal. Baseline 8 AM cortisol level was 17 mcg/dL and ACTH was 19 pg/mL. Abdominal CT scan showed a 1 cm left adrenal nodule of 25 HU density and a normal right adrenal with no atrophy. Phaeochromocytoma and primary hyperaldosteronism work up was negative. She started on Mifepristone 300 mg daily and titrated up to 600 mg daily. She presented to our institution one month later with 1 week of nausea, vomiting and muscle weakness. Laboratory studies revealed acute kidney injury with creatinine of 6.69 mg/dL (baseline Cr 1.5 mg/dL) and rhabdomyolysis with creatinine kinase level of 12,819 U/L (24-173 U/L) and urine myoglobin level of 888 ng/mL (0-13 ng/mL). It was evident that her catastrophic presentation was from development of adrenal insufficiency in the setting of Mifepristone glucocorticoid receptor blockade. The drug was discontinued immediately. She was treated with intravenous hydration and intermittent diuretics. Over 10 days, her clinical status gradually improved and creatinine decreased to baseline levels. Discussion: MACS is a challenging diagnosis as cortisol levels are affected by several variables including obesity, drugs, and poorly controlled diabetes. Hence, a meticulous evaluation is mandated before confirming the diagnosis. Our patient’s baseline cortisol and ACTH levels were normal, and her DST and LNSC levels were mildly elevated. Unilateral cortisol producing adenoma causes contralateral gland atrophy due to suppressed ACTH. This was not evident in our patient, thus making the diagnosis questionable. Adrenalectomy is the treatment of choice in MACS with adrenal adenoma. Off label medical management with Mifepristone, a glucocorticoid receptor antagonist, is being pursued in select cases and was trialed due to our patient's age and surgical risk. Mifepristone is approved for the treatment of hyperglycemia due to CS. The data supporting its use in MACS is sparse. Therefore, it is important to be aware of associated risks of Mifepristone when advising patients.
Presentation: 6/3/2024
8358 A Diagnostic Challenge: Occult Paraganglioma Misdiagnosed as Pheochromocytoma Leading to Unnecessary Adrenalectomy
Authors: Kasireddy A R , Alshami S , Gummalla S , Balasubramaniapandian C , Henske J A , Albashaireh A 
Published: October 2024

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
8097 A Rare Case of a Renal Cell Carcinoma and Pheochromocytoma Collision Tumor
Authors: Daas Rana , Logan Sarah , Sexton Wade , Chumbalkar Vaibhav , McGettigan Melissa , Veloski Colleen 
Published: October 2024

Abstract
Disclosure: R. Daas: None. S. Logan: None. W. Sexton: None. V. Chumbalkar: None. M. McGettigan: None. C. Veloski: None.
Background: Collision tumors are neoplasms comprised of two or more distinct tumors. A collision tumor consisting of a renal cell carcinoma and pheochromocytoma is extremely rare with less than five cases reported in the literature. Clinical Case: A 64-year-old male with a history of hypertension and type 2 diabetes presented with hematuria. The patient was on 4 anti-hypertensive medications at the time. He noted increased road rage, anxiety, tremors, and insomnia. A CT abdomen/pelvis revealed an exophytic 8.6 x 7.9 cm left upper pole renal mass and a 7.5 x 6.2 cm left adrenal mass. Contrast-enhanced MRI abdomen showed a left upper/interpolar renal mass with heterogenous peripheral enhancement, restricted diffusion, and areas of central necrosis along with a heterogenous, enhancing left adrenal mass with associated restricted diffusion and areas of cystic necrosis. Both masses had associated areas of hyperintense signal on T1 fat-suppressed MRI suggestive of hemorrhage. Lab work revealed ACTH 26.6 pg/mL (7.2-63.3), cortisol 13 ug/dL (3.7-19.4), aldosterone 23.1 ng/dL (4-31), renin 4 ng/mL/hr (0.5-4), dopamine 136 pmol/L (&lt;240), epinephrine 121 pmol/L (&lt;330), norepinephrine 23,666 pmol/L (1050-4800), plasma normetanephrines 12.3 nmol/L (0-0.89), plasma metanephrines &lt;0.10 nmol/L (0-0.49), 24-hour urine normetanephrines 4,178 mcg (122-676) and 24-hour urine metanephrines 4,233mcg (224-832). The patient was treated with alpha blockade prior to left radical nephrectomy, left adrenalectomy and infrahilar paraaortic lymph node dissection. Despite aggressive preoperative alpha blockade with maximal doses of doxazosin, addition of metyrosine and other anti-hypertensives, he developed acute hypertension with adrenal mass manipulation during the procedure and then hypotension following removal. He was admitted to the ICU for management of hypotension with pressors and fluid resuscitation and did well. Post-operative labs showed plasma normetanephrines 0.98 nmol/L (0.00-0.89) and plasma metanephrines 0.11 nmol/L (0.00-0.49). Pathology revealed clear cell renal cell carcinoma (RCC) of the kidney. The adrenal gland showed 2 tumors; one consistent with pheochromocytoma and another intimately associated with the former consistent with metastatic RCC, morphology diagnostic of a collision tumor. Following surgical resection, the patient was referred for genetic testing, surveillance imaging and lab testing, and treated with pembrolizumab adjuvant therapy for stage IV RCC. Conclusion: Although collision tumors are rare, biochemical testing to rule out a pheochromocytoma must be performed for all indeterminate adrenal masses and presumed adrenal metastases of active malignancies.
Presentation: 6/2/2024
6857 Adrenal Incidentaloma In A Patient With Surgically Treated Lung Adenocarcinoma A Decade After Complete Remission
Authors: Kaur L , Kaur R , Haider F , Iqbal A , Patel N 
Published: October 2024

Abstract
Disclosure: L. Kaur: None. R. Kaur: None. F. Haider: None. A. Iqbal: None. N. Patel: None.
Adrenal incidentaloma is an adrenal mass &gt;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
12325 Adrenocortical Carcinoma Masquerading As Pheochromocytoma In A Patient With Congenital Adrenal Hyperplasia
Authors: Marta Niedzialkowska Ewelina , Ahamad Banka Ajaz 
Published: October 2024

Abstract
Disclosure: E.M. Niedzialkowska: None. A.A. Banka: None.
Introduction: The development of adrenocortical carcinoma (ACC) in patients with congenital adrenal hyperplasia (CAH) is rare. The patients with CAH are at increased risk of adrenal tumors which is attributed to ACTH stimulation of adrenal gland if the disease is not appropriately treated. ACC cases mistaken for pheochromocytomas have been reported in the literature and are attributed to the variety of immunohistochemical markers expressed by the tumors. Case description:59 y.o female with salt-wasting CAH diagnosed at the age of 2 (21-hydroxylase deficiency) on hydrocortisone supplementation reported to the hospital due to episodes of sweating, tachycardia and hypertension. Work up revealed mildly elevated plasma normetanephrine 1 nmol/l (N &lt;0.9), urine normetanephrine 1762 mcg (N&lt;899), VMA 11.2 mcg/24hr (N 3.3-7.2). MRI of the abdomen revealed 2 left adrenal masses of 3.7 and 2.4 cm, right adrenal gland hyperplasia and low T1 and T2 enhancement. I-123 MIBG revealed increased activity and thickness of the right adrenal gland and no significant uptake on the left with nodular masses. The patient underwent left adrenalectomy with pathological report consistent with pheochromocytoma due to positive chromogranin and synaptophysin staining and moderate Ki activity. A year later follow up imaging showed an enhancing mass up to 7 cm at the suprarenal fossa invading the left kidney that was positive for nodular adrenocortical hyperplasia per pathology report. The patient continued to have elevated 17-OH- progesterone (2740 ng/l, N &lt;51) despite compliance with her steroid therapy and suppressed ACTH. She underwent mass resection along with nephrectomy, splenectomy, partial colectomy and partial pancreatectomy. Pathology report was positive for adrenocortical carcinoma (positive chromogranin, synaptophysin, CAM 5.2, polycystic cytokeratin, Ki positive in greater than 10% of the tumor cells) with renal and regional lymph node invasion and colonic metastases. The patient was started on mitotane therapy but a year later PET scan was positive for liver and omental metastases and was subsequently started on carboplatin/etoposide followed by streptozocin/adriamycin with initial good response, however repeat PET a year afterwards showed progression of the liver lesions, metastases to omentum and metastatic lesion to calvarium and the patient expired soon afterwards. Discussion: CAH predisposes patients to adrenal tumors which is attributed to increased ACTH stimulation of the adrenal gland but ACC in this population is extremely rare. In very rare cases ACC can present clinically as pheochromocytoma with elevated catecholamine levels and positive synaptophysin and chromogranin staining which poses a clinical challenge. Both CAH and ACC can lead to increased androgen levels and ACTH levels can help to differentiate the origin of the hyperandrogenemia.
Presentation: 6/2/2024
7320 A Case Of Concurrent Lithium-Associated Hyperthyroidism, Nephrogenic Diabetes Insipidus, Primary Hyperparathyroidism, And Acute Pancreatitis
Authors: Buchanan C , Ellenburg C , Beasey M 
Published: October 2024

Abstract
Disclosure: C. Buchanan: None. C. Ellenburg: None. M. Beasey: None.
Background: Hyperthyroidism, nephrogenic diabetes insipidus, and hyperparathyroidism are all well-documented adverse effects of long-term lithium therapy. This report highlights a rare case of these three endocrinologic adverse effects occurring concurrently. Clinical Case: A 47-year-old man on long-term lithium therapy for bipolar disorder presented with slurred speech, fatigue, new tremor, and generalized muscle weakness. Laboratory testing was consistent with lithium toxicity with serum lithium level of 2.22 mmol/L (0.60-1.50). Thyrotoxicosis was present with a free T4 of 3.2 ng/dL (0.6-1.1) and TSH of 0.04 µlU/mL (0.24-5.60). CT of the neck revealed mild enlargement and heterogeneity of the left lobe of the thyroid gland. Methimazole was started. Calcium corrected for albumin was elevated at 12.4 mg/dL (8.6-10.0) and PTH was also elevated at 146.0 pg/mL (12.0-88.0). Serum sodium was elevated at 160 mmol/L (136-145) with urine osmolality of 327 mOsm/kg. DDAVP was trialed in addition to IV hypotonic fluids, although it had little effect. Acetazolamide was started a few days later with subsequent normalization of the serum sodium level. Acetazolamide was later discontinued in favor of hydrochlorothiazide because of the development of non-anion-gap metabolic acidosis. A dose of zoledronic acid was given and cinacalcet was started. Serum calcium normalized over the following week. The patient acutely developed right upper quadrant abdominal pain, nausea, and emesis on hospital day 13. Lipase was elevated at 575 u/L (11-82). CT of the abdomen demonstrated moderate inflammatory changes along the pancreas and duodenum. Acute pancreatitis was diagnosed. There were no gallstones or acute biliary tract abnormalities seen on ultrasound. The patient’s symptoms gradually improved and he was discharged from the hospital after a total of 18 days. Lithium was discontinued indefinitely. TSH and free T4 normalized within 3 months. He had continued to require hydrochlorothiazide, methimazole, and cinacalcet at the time of his 3-month primary care follow-up appointment. Conclusion: Despite its efficacy and widespread use in mood stabilization, lithium has a narrow therapeutic window and is strongly associated with several serious, sometimes irreversible, adverse effects. Routine monitoring of lithium levels, serum calcium, and thyroid hormone levels is required. Lapses in routine monitoring may, as in this case, result in significant morbidity and prolonged hospitalization.
Presentation: 6/1/2024
5133 A Rare Case Report Of Hypoketotic Hypoglycemia Induced Seizures In A 44-Year-Oldfemale
Authors: Alrestom Roua , Bangolo Ayrton , Elias Sameh , Sekhon Imranjot 
Published: October 2024

Abstract
Disclosure: R. Alrestom: None. A. Bangolo: None. S. Elias: None. I. Sekhon: None.
Carnitine deficiency is a rare metabolic condition that can result in fasting hypoglycemia. Carnitine deficiency could be primary or secondary to other conditions. Among secondary causes, antiepileptics such as valproic acid have been incriminated. Herein we report the case of a 44-year-old female with epilepsy that presented with breakthrough seizures associated with hypoglycemia despite being on appropriate antiepileptic therapy. The patient was later found to have carnitine deficiency. Discontinuation of valproic acid and supplementation with L-carnitine resolved the patient’s hypoglycemia and breakthrough seizures. With this case report, we hope to encourage clinicians to include carnitine deficiency in the differential diagnosis of unexplained hypoglycemia.
Presentation: 6/1/2024