bluuebunny/crossref_metadata_2025_split huggingface dataset:
Incidental Giant Cystic Phaeochromocytoma: A Case Report
Authors: Dultra Bastos Gabriel Fernando , Falcão Camila Kruchewsky , Lins Rolim Maria Creusa de Albuquerque 
Published: May 2021

Abstract
Background: This case report describes a large sporadic benign phaeochromocytoma with classic symptoms and an unexpected SARS-CoV-2 virus contagious during the first pandemic wave. Clinical Case: A 61-years-old healthy man was admitted in the hospital emergency with hypertension, facial flushing, headache, nausea, vomiting and tremors. The patient presented hypertensive encephalopathy requiring orotracheal intubation. During the procedure, after rocuronium administration, the arterial pressure levels suddenly incresead more. An abdomen CT performed for vomiting investigation showed a mass in the left upper quadrant measuring 12.3 x 8.1 x 11.4 cm, central cystic area and sparse coarse calcifications, solid component density of 30 UH in the non-contrast phase and absolut washout of 48%, suggesting phaeochromocytoma (FEO) etiology. The patient renal function impaired and hemodialysis was required as replacement therapy. Even with small amount of urine available for exams, 24h urine tests were compatible with FEO diagnosis: (normetanephrines above 4260 mcg/L, n&lt; 732 mcg/L, 250 mL of urine). Considering the severity of symptoms, a chest CT scan was provided, not demonstrating any metastatic disease. Parenteral antihypertensive medication to manage blood pressure was required and this withdraw was reached after the introduction of prazosin 2 mg twice a day. After adequate preoperative preparation, the surgical removal was performed. Systolic BP intraoperative fluctuations occurred, with variations between 70 and 210mmHg. Splenectomy was necessary due the intimate contact with the adrenal mass. No obvious metastases were observed. The anatomopathological showed a 390 grams adrenal mass confirming FEO (PASS score 5). Genetic panel for phaeochromocytoma and paraganglioma with complete sequencing found no pathogenic variants in any of the 22 genes analyzed, suggesting sporadic etiology. The patient got infected with COVID-19 after a family visitor confirmed by molecular test. He developed mild respiratory symptoms, delaying his hospital discharge. He was released seven weeks after surgery with complete kidney recover function using low doses of prazosin. Plasma metanephrines and normetanephrines were normal. Conclusion: Phaeochromocytoma is a rare disease with no specific clinical signs and symptoms. It implies in potentially lethal cardiovascular complications, especially during the COVID-19 pandemic. On the other hand it is a curable illness through the surgical tumor removal. Attention should be paid to high blood pressure levels even after the tumor is removed which can be justified by the remaining vascular hypertrophy as long as the suspicion of malignant FEO is excluded.
Incidental Pheochromocytoma: Silent but Violent
Authors: Bantu Sravani , Vallepu Shirisha R , Gunda Mouna , Thudi Vaishali 
Published: May 2021

Abstract
Background: Pheochromocytoma is a rare catecholamine secreting neuroendocrine tumor. It arises from the chromaffin cells of adrenal medulla. It is diagnosed in 5–6.5% of adrenal incidentalomas which is not common. The usual clinical presentation includes the classic triad of sweating, headache and tachycardia. However, asymptomatic cases are seen in 8% of the patients with pheochromocytoma. We present a clinically asymptomatic patient diagnosed during work up of adrenal incidentaloma. The possible etiology for silent presentation includes one of the following:(i) Presence of a smaller functional tissue (ii)Accelerated turnover of the tumor causing release of the unmetabolized catecholamines in small amounts (iii) Pulsatile tumor secretion (iv)Tumors triggered by stress (v) Laboratory errors due to inappropriate handling of specimen at high-temperature (vi) False negative test results secondary to caffeine ingestion in the prior 24 hours.
Clinical Case: 59 years old Caucasian female with past medical history of type 2 diabetes mellitus, obesity, essential hypertension, nonischemic cardiomyopathy, and asthma presented to the emergency room with complaints of worsening shortness of breath and pedal edema for 1 month. Physical exam: Blood pressure 146/78 mm of Hg and heart rate 82 beats/min, mild pedal edema, no pulmonary crackles. On imaging, CT angio chest showed irregularly enhancing right adrenal mass measuring 3.4 cm. This adrenal incidentaloma was not visualized on imaging done 5 years ago. Further, MRI abdomen revealed 4.1 cm right adrenal mass. Laboratory testing showed high total plasma
metanephrines: 890 pg/ml (&lt; or = 205), 24-hour urine metanephrines: 2337 (140–785), A1C: 10%. This confirmed the diagnosis of adrenal pheochromocytoma. Preoperatively, she was started on phenoxybenzamine 10 mg BID and encouraged on liberal salt intake. During the course, her blood pressure and heart rate were monitored daily. She underwent right adrenalectomy. Surgical pathology revealed 4.1 cm pheochromocytoma, negative margins with extension to the adipose tissues and vascular invasion, PASS score = 4. Post operatively, patient declined to get labs done. Due to high risk behavior of the tumor, patient needs to be monitored annually for lifelong.
Conclusion: Pheochromocytoma is an uncommon tumor with varied clinical presentation. It can manifest itself widely from being silent to aggressive disease. This warrants high suspicion, early detection and management, thereby reducing the morbidity and mortality. Lately, there has been increased incidence of adrenal incidentalomas owing to widespread use of radiological investigations. We report a case of incidental pheochromocytoma which is biochemically active but clinically asymptomatic. This emphasizes the importance of being more vigilant during the evaluation of adrenal incidentalomas.
Inflammatory Myofibroblastic Adrenal Tumor: An Extreme Rare Cause of Adrenal Incidentaloma
Authors: Elshimy Ghada , Gandhi Anand , Jyothinagaram Sathya G 
Published: May 2021

Abstract
Introduction: An inflammatory myofibroblastic tumor (IMT) is considered a complex disease with a suggested neoplastic nature. It has a variable histological and clinical presentation. Up to now, the exact etiology is still unknown. Various sites such as the lungs (first site described in 1939), the heart, the gastrointestinal tract, and the genitourinary tract have been reported in the literature. The adrenal glands are considered an extremely rare location with no recurrence reported after surgical management. Up to our knowledge, only 9 cases have been described in the literature. The age of presentation was variable (from newborn to 57 years old). Case Report: A 70 years old female with a past medical history of well-controlled diabetes type 2, and hypertension presented with a progressively increasing chronic right flank pain for the past couple of months, associated with 10 lb weight loss. Workup was done and an incidental right adrenal mass was found on CT scan. The mass was 3.2cm x 3.3 cm partially necrotic with adjacent stranding abutting the right adrenal gland and the liver. The opposite gland was normal and there was no evidence of any other mass lesion in the abdomen. MRI adrenal showed 3.2x2.5x3.6 cm solid heterogeneous mass in the right adrenal gland (Hypointense on T1 and T2 weighted images. She had no sign or symptoms suggestive of Cushing and/or pheochromocytoma. Her hypertension was well controlled on lisinopril 10 mg. Hormonal workup was within normal range except for a mild elevation of DHEAS. Given the size of the mass, the worrisome radiological characteristics, and a family history of pheochromocytoma in her daughter, surgical options were discussed with the patient. Subsequently, the patient underwent the right robotic adrenalectomy. The postoperative course was uneventful. Pathology revealed a 6.5x6x 5 cm IMT of the adrenal. The CT scan done 6 months postoperatively didn’t show any recurrence. Discussion: and conclusion: Adrenal IMTs are extremely rare but they should be considered in the differential diagnosis of adrenal masses. Follow-up is warranted since its behavior still remains uncertain. More cases are needed to unmask the true biological behavior and pathogenesis of adrenal IMTs.
Interpreting Normetanephrines-the Significance of Clinical Context
Authors: Kumar Nitya , D’Alessio David A 
Published: May 2021

Abstract
Introduction: Pheochromocytoma ranges in presentation, but the diagnostic hallmarks are increased catecholamine secretion in patients with an adrenal mass. However, interpretation requires consideration of the clinical state.
Case: A 60-year-old man presented with 6 months of episodic flushing, night sweats, fatigue, back pain, and 20-pound weight loss. Vitals were notable for hypotension and mild tachycardia. He was thin with evidence of hyperpigmentation. Contrasted CT showed 15 cm heterogeneous masses of both adrenals and a rib lesion. Plasma normetanephrines were elevated on two separate measures (526 and 1,398 pg/mL, nl &lt;=148). Plasma metanephrines were normal. Urine normetanephrines were increased 4-fold (2,165mcg/g Cr, n 108–524). Labs noted low DHEA-S (2 mcg/dL, n 38–313), high ACTH (204 pg/mL, n 6–50), random cortisol (5.2 mcg/dL, n 3.5–18.3), undetectable aldosterone, and high plasma renin activity (83 ng/mL/h, n 2.9–10.8). Cortisol peaked at 6.7 mcg/dL 1 hour after 250mcg of cosyntropin. MRI revealed over 15cm infiltrating masses arising from adrenals with modest flare on T2 imaging. Dotatate PET/CT showed mild uptake of bilateral adrenals and metastases to liver and bone. He was started on steroid replacement and doxazosin for presumed pheochromocytoma.
He was transferred to our facility where he remained hypotensive despite adequate glucocorticoid and mineralocorticoid replacement. Once stabilized, repeat plasma normetanephrines were insignificant (218 pg/mL, n 0–145) and chromogranin was normal (68 ng/mL, n&lt;93). Biopsy of the rib lesion confirmed diffuse large B-cell lymphoma.
Discussion: Pheochromocytoma classically presents in the outpatient setting with palpitations, diaphoresis, pallor, and paroxysmal hypertension. Rarely, it results in hypertensive emergencies requiring hospitalization. In patients with catecholamine excess and an adrenal mass, pheochromocytoma is usually suspected. 3-fold or more increases of normetanephrines or metanephrines above upper cutoffs are rarely false-positives. However, this assumes clinical stability. Sympathoadrenal activation is a physiologic response to acute illness. Metanephrines in critically ill patients without pheochromocytoma approached those of pheochromocytoma in one study. Levels normalized upon recovery from illness, highlighting the importance of verifying biochemistries once the patient is stable. The degree of elevation in normetanephrines seen in this patient was a physiologic response to adrenal insufficiency and clinical instability. Pathology confirmed lymphoma rather than pheochromocytoma for which he was started on chemotherapy.
Conclusion: Elevated normetanephrines is an appropriate physiological response in the acutely ill. Careful consideration of the clinical picture in conjunction with biochemical data is critical.
Mixed Corticomedullary Tumors of the Adrenal Gland Harboring Both Medullary and Cortical Properties
Authors: Inoue Aiko , Inoue Sachika , Sawai Rui , Hamamatsu Keita , Okazaki Kyoko , Nishizawa Hitoshi , Yamazaki Yuto , Sasano Hironobu , Murabe Hiroyuki , Yokota Toshihiko 
Published: May 2021

Abstract
Adrenal cortex and medulla are derived from mesoderm and ectoderm, respectively. Mixed corticomedullary tumors (MCMTs), comprising an intimately admixed population of both adrenal cortical cells and pheochromocytes in a single adrenal tumor, are extremely rare and its pathogenesis has remained unknown. Here, we report a case of MCMT whose cells co-expressed cortical and medullary antigens in the same tumor cells.[Case description]A 40-year-old woman was referred to our hospital for investigating Takotsubo cardiomyopathy following resection of uterine fibroids. An abdominal CT scan depicted a 24 mm tumor on her left adrenal gland. Her basal serum ACTH, cortisol levels and urinary cortisol were 13.8 pg/mL, 9.5 μg/dL, and 26.5 μg/day respectively. The cortisol level was normally suppressed by an administration of 1 mg dexamethasone (1.4 μg/dL). Plasma renin activity, aldosterone levels and urinary aldosterone were 15.0 ng/mL/h, 122 pg/mL, and 5.0 μg/day, respectively (with administration history of azosemide). On the other hand, her plasma adrenaline and noradrenaline levels were elevated as high as 177 pg/mL and 536 pg/mL, and urinary metanephrine and normetanephrine were 2.12 mg/day and 1.10 mg/day. A 123I-metaiodobenzylguanidine scan revealed high uptake in the tumor. After adequate adrenergic α-receptor blockage, left adrenalectomy was performed. Her postoperative endocrine and clinical findings were normalized without any further complications.[Pathology] Immunohistochemistry (IHC) revealed the presence of MCMT. Cells morphologically consistent with pheochromocytoma and adrenocortical cells were confirmed by immunostaining of chromogranin A and SF-1, respectively. Chromogranin A-positive medullary-derived and SF-1-positive cortical-derived tumor cells were intermixed in the chimeric fashion. In addition, some tumor cells were positive for both proteins, indicating hybrid nature of the cells. Tumor cells of cortical origin expressed CYP11β1, 3β-HSD, p450c21, and p450c17, but not CYP11β2. Non neoplastic adrenal cortex were atrophic, whereas the glomerulosa was hyperplastic positive for CYP11β2, consistent with diffuse hyperplasia and adrenal medullar unremarkable. [Conclusions:]The adrenal tumor was clinically diagnosed as pheochromocytoma, but the pathological findings did reveal cortisol production in the tumor and aldosterone overproduction in the accompanying cortex. This is the first case of MCMT co-expressing adrenal medullary and cortical antigens in the same tumor cells as hybrid cells.
Persistent Hyperkalemia Post-Adrenalectomy for Aldosterone-Producing Adenoma
Authors: Borhan Marisa Khatijah , Sieng Tan Florence Hui 
Published: May 2021

Abstract
Background:Post-operative hypoaldosteronism due to chronic suppression of the renin-aldosterone axis of the contralateral gland can be complicated with hyperkalemia. We describe a case of persistent hyperkalemia post adrenalectomy for aldosterone-producing adenoma. Clinical Case: A 47-year-old male was first diagnosed with hypertension in 2011. He was investigated for secondary hypertension after hospital admission in 2019 for hypertensive urgency and symptomatic hypokalemia (potassium, K 1.9–2.3 mmol/L, n = 3.5–5.0). Subsequent laboratory investigation revealed elevated serum aldosterone (3565 pmol/L, n &lt; 103) with an aldosterone renin ratio of 115 (n &lt; 35). A confirmatory test with saline loading showed an unsuppressed serum aldosterone level of 1840 pmol/L. Adrenal CT reported a 4.1-cm, heterogeneous left adrenal lesion. A diagnosis of primary aldosteronism was made, and he underwent laparoscopic left adrenalectomy in July 2020. Histopathology examination was consistent with adrenal cortical adenoma. Both potassium supplementation and spironolactone were stopped immediately postoperatively. Two weeks later, he developed symptomatic hyperkalemia (K 6.0 mmol/L), requiring hospital admission, and started on potassium binder. Throughout clinic follow-ups, potassium remained high (K 5.4–6.1 mmol/L), despite low potassium diet and potassium binder. His case was co-managed with the nephrology team and given a trial of frusemide and sodium bicarbonate to normalize his potassium. However, after 4 months, he remained hyperkalemic. Repeated serum aldosterone was not elevated (&lt;103 pmol/L). He was then started on fludrocortisone and finally managed to achieve serum potassium normalisation (K 4.1–4.5 mmol/L). Conclusion: This case highlights the importance of monitoring potassium levels in all patients after adrenalectomy, particularly those with clinical risk factors. Retrospective studies by Park et al and Fischer et al reported that a long duration of hypertension, impaired preoperative renal function, older age, and large adenoma size represent risks for developing hyperkalemia postoperatively, whereas the use of mineralocorticoid receptor antagonists preoperatively does not prevent hyperkalemia. Treatment includes a low potassium diet, a high sodium diet, adequate hydration, potassium binder, frusemide, and fludrocortisone. In some cases, hyperkalemia may be prolonged, necessitating long-term fludrocortisone therapy, up to 11–46 months². References: 1. Park KS, Kim JH, Ku EJ, et al. Clinical risk factors of postoperative hyperkalemia after adrenalectomy in patients with aldosterone-producing adenoma. Eur J Endocrinol. 2015 Jun;172(6):725–31. 2. Fischer E, Hanslik G, Pallauf A, et al. Prolonged zona glomerulosa insufficiency causing hyperkalemia in primary aldosteronism after adrenalectomy. J Clin Endocrinol Metab. 2012 Nov;97(11):3965–73.
Pheochromocytoma Presenting in a Elderly Female With a History of Uterine Leiomyoma and Renal Cell Carcinoma
Authors: Rico Jeffrey Gara , Innerfied Ronald 
Published: May 2021

Abstract
Background: Adrenal incidentaloma are masses that are found coincidentally on radiologic imaging usually performed for indications other than evaluation for adrenal disease. Pheochromocytomas are rare, usually sporadic catecholamine-producing adrenal tumors and represent 5% of adrenal incidentalomas. While pheochromocytomas can present on their own, they can also present as part of a spectrum of disease found in certain familial syndromes. We present a case of pheochromocytoma found in a patient with a history of renal cell carcinoma and fallopian tube leiomyoma.
Case Presentation: A 67 year old female with a past medical history of diabetes mellitus type II, end stage renal disease on hemodialysis, hypertension, skin cancer, right fallopian tube leiomyoma, and right renal mass suspected for renal cell carcinoma presented to the clinic for evaluation of a left adrenal mass. Left adrenal mass was incidentally found 3 years ago on CT abdomen for evaluation for renal transplant and noted to be 2.2 cm in diameter. There were also incidental findings of a 1.3cm right renal mass and a 5.6x4.8cm right adnexal mass. Patient underwent robot assisted total laparoscopic hysterectomy with bilateral salpingo-oophrectomy and surgical pathology results showed leiomyoma of the myometrium. Surveillance CT imaging done 3 years after showed the left adrenal mass and right renal mass increased in size with the adrenal mass increasing to 4.3 cm in diameter with central necrosis and right renal mass increasing to 3.2 x 1.3 cm. Screening biochemical test were done, and initial test and confirmatory test confirmed pheochromocytoma. Dexamethasone suppression test and aldosterone/renin ratio results were normal. Surgical pathology confirmed pheochromocytoma and biopsy of the right renal mass 8 months later confirmed renal cell carcinoma.
Conclusion: Majority of pheochromocytomas are sporadic but up to 40% have been identified as inherited in origin due germline mutations. They have been known to present as a part of a spectrum of disease pathologies specific for certain familial syndromes such as Neurofibromatosis Type 1, Von Hippel Lindau Syndrome, Multiple Endocrine Neoplasia Type 2, and Familial Paragangliomas. One relatively uncommon syndrome, Hereditary Leiomyomatosis and Renal Cell Cancer Syndrome due to a mutation in the fumarate hydratase gene, has been known to present with pheochromocytoma in rare cases. Our patient presented with pheochromocytoma, renal cell carcinoma, and uterine leiomyoma which is suspicious for Hereditary Leiomyomatosis and Renal Cell Cancer Syndrome. The diagnosis was not confirmed as genetic testing was not done. Due to a high rate of inherited patterns of pheochromocytoma, routine genetic testing should be done as identification of gene mutations may lead to diagnosis, treatment, and surveillance for patients and their relatives.
Pheochromocytoma Rupture Associated With Systemic Anticoagulation: A Case Report
Authors: Wan WingYee , Graybill Sky Denniston 
Published: May 2021

Abstract
Background: Pheochromocytoma rupture is a rare endocrine emergency. There have been approximately 85 documented cases, and the mortality rate for patients who require emergent surgery is as high as 34% (1).
Clinical Case: A 30-year-old man with no past medical history presented to the Emergency Department with abdominal pain and vomiting. He was hypertensive, tachycardic, and hypoxic. CT Chest Angiogram revealed bilateral pulmonary emboli and a 6.5 x 5.5 x 6.4 cm left heterogenous adrenal mass. His plasma free metanephrines were elevated to 31,173 pg/mL (n&lt;62 pg/mL) with normetanephrines &gt;50,000 pg/mL (n&lt;145 pg/mL). His pulmonary emboli was treated with continuous intravenous heparin, and he was also treated with intravenous fluids and phentolamine due to concern for pheochromocytoma. Within hours, he became hypotensive, and his hemoglobin downtrended from 16.4 g/L to 7.3 g/L (n 14–18 g/L). Repeat CT showed that the adrenal mass had ruptured, enlarging to 15.4 x 9.7 x 10.0 cm. He was taken for an emergent laparotomy with piecemeal resection of the mass; surgical pathology confirmed a pheochromocytoma. Fortunately, the patient recovered well after surgery. After 6 months, his plasma free metanephrines normalized to 15 pg/mL with normetanephrines of 80 pg/mL. His DOTATATE scan was negative for malignancy and his genetic testing was negative.
Conclusion: The patient’s systemic anticoagulation and critical illness likely increased his risk for pheochromocytoma rupture. Standard preoperative pheochromocytoma management, to include aggressive fluid resuscitation and alpha blockade, is important in reducing risk of morbidity and mortality. Biochemical follow up is required, as there is a 16% risk of recurrence.
Reference: (1) Kobayashi T, Iwai A, Takahashi R, Ide Y, Nishizawa K, Mitsumori K. Spontaneous rupture of adrenal pheochromocytoma: Review and analysis of prognostic factors. Journal of Surgical Oncology. 2005;90(1).