Introduction: Pheochromocytoma is a catecholamine-secreting adrenal tumor associated with major perioperative hemodynamic instability. When concurrent major pelvic pathology requires surgery, operative planning becomes particularly challenging. We describe the successful single-stage management of pheochromocytoma and a giant uterine fibroid, highlighting the importance of multidisciplinary coordination and perioperative optimization. Case: A 49-year-old female with symptomatic uterine fibroid was found to have proliferative endometrium on a pipelle biopsy. Computed tomography (CT) abdomen incidentally detected a right adrenal mass alongside a large posterior uterine fibroid (11.9 × 17.4 × 14.6 cm). CT adrenal protocol demonstrated a heterogeneously enhancing right adrenal mass (6.9 × 6.9 × 9.6 cm) with high unenhanced attenuation. Biochemical evaluation revealed markedly elevated 24-hour urinary metanephrine (4.7× upper limit) and normetanephrine (2.4× upper limit). Following multidisciplinary discussions, a single-stage surgical approach was planned after careful assessment of feasibility and perioperative risk in view of the uncertain malignant potential of the pelvic mass and to minimize repeated exposure to anesthesia. Preoperative optimization included transitioning from terazosin to phenoxybenzamine, with subsequent addition of bisoprolol for hemodynamic control. The operative strategy prioritized pheochromocytoma resection first, given its potential for significant hemodynamic instability. Progression to hysterectomy was contingent upon achieving adequate intraoperative hemodynamic stability following adrenalectomy, with continuous reassessment by the anesthetic and surgical teams. Right adrenalectomy was performed first, followed by total abdominal hysterectomy with bilateral salpingooophorectomy. Significant hemodynamic lability occurred during tumor manipulation, with hypertensive surges managed using sodium nitroprusside and remifentanil infusions. Following adrenal vein ligation and tumor removal, hypotension was managed with noradrenaline and additional adrenaline support as required. Total operative time was approximately 6 hours. Postoperatively, transient noradrenaline support was required but was rapidly weaned as hemodynamic stability was achieved. Conclusion Single-stage adrenalectomy and major pelvic surgery can be safely performed in selected patients with pheochromocytoma when guided by meticulous preoperative optimization, clear intraoperative sequencing, and close multidisciplinary coordination.
Pheochromocytoma ; Adrenalectomy ; Leiomyoma ; Hysterectomy

Introduction: Hemorrhagic suprarenal masses presenting with hypertensive emergency pose a significant diagnostic challenge, particularly when biochemical and radiological findings are inconclusive. The clinical presentation may mimic catecholamine-secreting tumors, necessitating consideration of a broad differential diagnosis, including pheochromocytoma, adrenocortical carcinoma, retroperitoneal hemorrhage, and hematological-related extramedullary lesions. Accurate diagnosis is essential, as management strategies differ significantly. Case: We report a 48-year-old male smoker with no known prior medical illness who presented with sudden left-sided chest pain radiating to the epigastrium, associated with vomiting. On arrival, he was markedly hypertensive (207/131 mmHg). He reported a 1-year history of paroxysmal palpitations, headaches, migraines, and intermittent diaphoresis. Computed tomography angiography excluded aortic dissection but demonstrated a left retroperitoneal hemorrhage with non-visualization of the adrenal gland, suggestive of adrenal or tumor-related hemorrhage. He was initially managed empirically as a pheochromocytoma while undergoing biochemical evaluation; however, urinary metanephrines were only mildly elevated. Repeat imaging demonstrated interval enlargement of a non-enhancing suprarenal mass, raising concern for tumor-related hemorrhage. Subsequent ultrasonography, however, favored a liquefied hematoma, and percutaneous drainage yielded 750 mL of sanguineous fluid, resulting in marked clinical improvement. Notably, an elevated hematocrit prompted further evaluation for erythrocytosis. Subsequent testing confirmed JAK2 mutation-positive polycythemia rubra vera, providing a unifying explanation for both the erythrocytosis and spontaneous adrenal hemorrhage. The patient was commenced on hydroxyurea and referred for hematology follow-up. Conclusion Adrenal hemorrhage may closely mimic pheochromocytoma in hypertensive emergencies. A systematic, multidisciplinary approach integrating clinical, biochemical, and imaging findings is essential to avoid misdiagnosis and guide appropriate management, particularly in patients with underlying hematological disorders.
Hypertensive Crisis ; Pheochromocytoma ; Polycythemia Vera ; Hematoma

Introduction: Pheochromocytoma is a catecholamine-secreting tumor with diverse cardiovascular manifestations, including myocardial infarction with non-obstructive coronary arteries (MINOCA). We report a case of biochemically confirmed pheochromocytoma initially presenting as non-ST-elevation myocardial infarction (NSTEMI), later reclassified as MINOCA. Case: A 62-year-old female with type 2 diabetes mellitus and hypertension was admitted with presumed NSTEMI and commenced on dual antiplatelet therapy. Further history revealed recurrent presyncope associated with paroxysmal headache, palpitations, and profuse diaphoresis. During admission, her blood pressure was markedly labile, ranging from 75/45 to 220/122 mmHg, raising suspicion of pheochromocytoma. Biochemical evaluation demonstrated markedly elevated 24-hour urinary normetanephrine of 36.19 µmol/day (reference 0–2.13) and methoxytyramine of 3.90 µmol/day (reference 0.10– 1.79), consistent with catecholamine excess. Dedicated adrenal computed tomography identified a 3.8-cm right adrenal lesion with high attenuation (44 Hounsfield Unit [HU]), arterial enhancement (119 HU), and low washout (absolute 40%, relative 24%), without calcification or necrosis. Electrocardiography showed sinus rhythm with T-wave inversion in leads I, aVL, and V5–V6. Transthoracic echocardiography demonstrated a preserved left ventricular ejection fraction of 67% without regional wallmotion abnormalities. Coronary angiography subsequently showed normal coronary arteries, supporting a diagnosis of MINOCA likely secondary to pheochromocytoma-related catecholamine excess and hypertensive crisis. Antiplatelets were discontinued. She was commenced on α-blockade, with additional felodipine and low-dose β-blocker for blood pressure optimization, and subsequently underwent successful open right adrenalectomy. Postoperatively, she required transient inotropic support but was weaned within 36 hours. Conclusion Pheochromocytoma-associated MINOCA is uncommon but important to recognize. Catecholamine surges may cause myocardial injury through coronary vasospasm, myocardial oxygen supply-demand mismatch, and direct catecholamine-mediated cardiotoxicity. Recognition is crucial, as management differs fundamentally from atherosclerotic acute coronary syndrome and requires α-blockade before β-blockade.
MINOCA ; Non-ST Elevated Myocardial Infarction ; Pheochromocytoma

Introduction: 3β-Hydroxysteroid dehydrogenase (3β-HSD) deficiency and lipoid congenital adrenal hyperplasia (CAH) are rare disorders of steroidogenesis that result in impaired synthesis of all adrenal and gonadal hormones. Affected males typically present with ambiguous genitalia and concurrent mineralocorticoid and glucocorticoid deficiency. Distinguishing between these two conditions is crucial, as their management strategies differ. Case: We report a 24-year-old male, born to non-consanguineous parents, who was clinically diagnosed with 3β-HSD deficiency during infancy. He presented at day 40 of life with ambiguous genitalia, bilateral undescended testes, and generalized hyperpigmentation. His family history was significant for an elder brother with salt-losing CAH. Initial biochemical evaluation confirmed primary adrenal insufficiency and primary hypogonadism. Notably, his steroid precursors, dehydroepiandrosterone sulfate (DHEAS) and 17-hydroxyprogesterone, were suppressed. A karyotype confirmed 46,XY. In the absence of genetic testing at that time, a clinical diagnosis of 3β-HSD deficiency was made, and he was commenced on mineralocorticoid and supraphysiological glucocorticoid replacement. He underwent bilateral orchidopexy and hypospadias repair in childhood. Pubertal induction was required, followed by maintenance testosterone therapy. Upon transitioning to adult care, a review of his biochemical profile, particularly suppressed DHEAS, which is inconsistent with 3β-HSD deficiency, raised the suspicion of a more proximal defect, such as lipoid CAH. Differentiation is imperative, as lipoid CAH requires only physiological glucocorticoid replacement, whereas 3β-HSD deficiency often needs higher doses to suppress adrenocorticotropic hormone and DHEAS. To resolve this diagnostic uncertainty and guide longterm therapy, the patient was referred for genetic studies. Conclusion Differentiating 3β-HSD deficiency from lipoid CAH is important, as both have similar clinical presentation. The absence of elevated steroid precursors, particularly DHEAS, should raise suspicion of a more proximal defect. Given the different aims of glucocorticoid therapy, establishing a precise diagnosis through genetic studies is essential to guide clinical management and minimize the morbidity associated with supraphysiological corticosteroid dosing.
Pheochromocytoma ; Mutation

A 23-year-old female presented with headache, palpitation, and hypertensive spells. There was no similar family history. Twenty-four (24) hour urine testing showed elevated normetanephrine level with normal metanephrines [metanephrines 123 mcg/24 hrs (74-297); normetanephrines 5321.16 mcg/24 hrs (73-808)]. A biochemical diagnosis of normetanephrine-secreting pheochromocytoma was made. Considering the age and urine reports, a functional scan was ordered. Imaging with 18-FDG PET CT was done which showed uptake indicative of a large left adrenal mass, as well as uptake in the mediastinal, abdominopelvic, lymph nodes and metabolically active mesenteric, peritoneal and omental thickness. This suggested a left adrenal pheochromocytoma with the possibility of an associated lymphoproliferative disorder or active lesions in brown fat. To describe these extra-adrenal lesions, a Ga-68 This work DOTANOC PET CT was obtained which showed a diffuse somatostatin receptor-expressing large soft tissue mass lesion in the left adrenal likely to be pheochromocytoma without any other lesion elsewhere in the whole body survey. This depicts the confusion created by the metabolically active brown adipose tissue (BAT) in the FDG PET scan. Brown fat is involved in non-shivering thermogenesis and is typically located in the cervical, supraclavicular, mediastinal, and abdominal regions. High uptake in the BAT can make interpretation of the FDG PET report difficult and misleading. Some precautions like avoidance of cold and beta blockers can minimize BAT uptake in FDGPET scans.
Adipose Tissue, Brown ; Pheochromocytoma

Adrenocortical carcinoma (ACC) is a rare malignant tumour originating from the adrenal cortex. Half of the cases are functional, with ACTH independent autonomous cortisol production being the most common. It is rare for ACC to present with markedly elevated metanephrine levels which is a typical characteristic of pheochromocytoma. We report a case of a large functioning adrenal tumour with overlapping biochemistry features of ACC and pheochromocytoma. Biopsy confirmed the histopathological diagnosis of metastatic ACC.
Adrenocortical Carcinoma ; Pheochromocytoma

OBJECTIVE

This study aims to describe the epidemiologic profile and determine the clinical outcomes of patients with pheochromocytoma at the University of the Philippines Philippine General Hospital (UP-PGH).

We reviewed the medical records of 30 patients with histopathology-proven, clinical, and biochemical diagnosis of pheochromocytoma. Demographic, clinical characteristics, and clinical outcomes were collected for each patient.

The median age at diagnosis of pheochromocytoma was 37.5 years (IQR 28-55) and the most common metabolic comorbidities were glucose intolerance (60%) and hypertriglyceridemia (23.3%). Majority of the patients were hypertensive (90%). Two third of the patients presented with classic features of pheochromocytoma while the remaining third presented as adrenal incidentaloma. Recurrence was found in 17% of subjects, who were significantly younger (25 years vs 46.5 years P = 0.0229), and had higher rates of bilateral pheochromocytoma (0 vs 75%), p = 0.002). Metastatic pheochromocytoma was found in 10% of the subjects.

Our study demonstrated that patients with pheochromocytoma in our setting exhibit great variability in terms of clinical behavior. Although majority of the patients presented with symptoms related to catecholamine excess, almost one-third of the patients were only incidentally discovered. Incidence of pheochromocytoma recurrence and metastasis in our setting are comparable with current available foreign studies.

Excess hormone production from adrenal tumors caused by primary hyperaldosteronism or pheochromocytoma are common etiologies for secondary hypertension. Studies have shown that sustained long-term circulating hormones in excess affect the blood vessels and cardiac structures. Inflammation of cardiomyocytes leads to fibrosis and eventual cardiomyopathy and is clinically presented as arrhythmia, nonfatal myocardial infarction, heart failure, or even death. The tissue changes and/or impaired cardiac function are reversible if early diagnosis and removal of the adrenal tumor by unilateral adrenalectomy is done. However, the condition becomes challenging if the adrenal lesions are bilateral. This article introduces the concept of systemic hormonal unloading and will discuss the philosophy of quality of life in managing bilateral adrenal disease.
Hyperaldosteronism ; Pheochromocytoma ; Quality of Life

Background: Pheochromocytomas are rare catecholamine-secreting tumors that usually present with hypertension and palpitations. However, a subset of pheochromocytoma patients is asymptomatic, presenting as adrenal incidentaloma on imaging. Case: We present a case of a 32-year-old normotensive female who presented with a right suprarenal mass on abdominal ultrasound. Diagnosis of pheochromocytoma was made after biochemical testing revealed elevated 24- hour urine metanephrine of 1.96 mg/24hrs (NV:0-1 mg/24hrs) and epinephrine of 129 mcg/24hrs (NV: 2-24 mcg/24hrs). In addition, plasma chromogranin A was elevated at 225.38 ng/ml (NV:<100 ng/ml). CT scan of the abdomen showed a 3.0 x 4.0 x 3.0 cm heterogeneous well-circumscribed right adrenal mass, with 87Hu on contrast, an absolute washout of 21%, and a relative washout of 13% on a delayed scan. After adequate preoperative medical therapy with an alpha-adrenergic blocker, a right laparoscopic adrenalectomy was done, with histopathologic confirmation of pheochromocytoma. Repeat 24-hour urine metanephrine measurements done on multiple follow-ups after surgery were normal. Conclusion Asymptomatic pheochromocytoma should be included in the differential diagnoses of adrenal incidentalomas. As in our case, patients with normotension and adrenal incidentalomas should still undergo biochemical workup to rule out the presence of pheochromocytoma. Long-term complications from chronic exposure to high catecholamine levels lead to significant adverse cardiovascular effects. Early detection, adequate perioperative preparation, and timely surgical intervention can prevent a potential catastrophe.
Pheochromocytoma ; Blood pressure ; Filipino

OBJECTIVE: To explore the pathological characteristics and significance of RET proto-oncogene screening in multiple endocrine neoplasia type 2A (MEN2A) with cutaneous lichen amyloidosis (CLA). METHODS: Clinical data of 51 members from 7 unrelated pedigrees of MEN2A-CLA were collected. Systemic clinical investigations including biochemical testing, imaging examination, germline RET variant screening and histopathological examination were carried out. RESULTS: RET gene variants were detected in 28 patients with MEN2A (C634G/F/R/S/W and C611Y) including 12 males and 16 females, with the mean age of diagnosis being (41.1 ± 18.3) years old, which were consistent with their clinical manifestations. The incidence of medullary thyroid carcinoma (MTC), pheochromocytoma (PHEO), hyperparathyroidism (HPTH) and CLA among 28 MEN2A patients were 89.3%, 28.6%, 7.1% and 28.6%, respectively. Comparison of the incidence of MTC/PHEO/HPTH and CLA between C611Y and C634G/F/R/S/W, only PHEO and CLA in C611Y were lower than those in C634G/F/R/S/W (P < 0.05; P < 0.05). Among 8 patients with CLA, the male to female ratio was 2 : 6. The clinical features included pruritus in the interscapular region and presence of dry, thickened, scaly, brown pigment, clustered or desquamate-like plaques. The mean onset age of CLA [(18.4 ± 4.6) years] versus the mean age at diagnosis of CLA or MEN2A were significantly different (P < 0.001; P < 0.001). CONCLUSION MEN2A-CLA may be the early clinical manifestation of MEN2A and most frequently occurred along with RET-C634 variant. To facilitate the recognition of MEN2A-CLA, to combine family investigation and screening of RET variant are helpful for early diagnosis and standardized treatment, which can improve the long-term outcome of MEN2A-specific tumors.
Adolescent ; Adrenal Gland Neoplasms ; Adult ; Amyloidosis, Familial ; Carcinoma, Neuroendocrine ; China ; Female ; Humans ; Lichens ; Male ; Middle Aged ; Multiple Endocrine Neoplasia Type 2a/genetics* ; Pheochromocytoma ; Proto-Oncogene Proteins c-ret/genetics* ; Skin Diseases, Genetic ; Thyroid Neoplasms/genetics* ; Young Adult