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

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.

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

OBJECTIVE: To explore the surgical strategy and experience of reoperation for pheochromocytoma and paraganglioma which is very challenging. METHODS: The clinical data of 7 patients with pheochromocytoma and paraganglioma who underwent reoperation in Department of Urology, Peking University Third Hospital from August 2016 to February 2021 were analyzed retrospectively. There were 4 males and 3 females, with an average age of (44.1±11.5) years (28-60 years), 6 cases on the right side and 1 case on the left side. The causes of the operations included: (1) 2 cases of tumor recurrence after resection; (2) The primary operations failed to completely remove the tumors in 3 cases, because the tumors were large and closely related to blood vessels. (3) Pheochromocytoma and paraganglioma wasn't diagnosed before primary operation, therefore, drug preparation wasn't prepared. Two cases were interrupted by severe blood pressure fluctuations during the primary operations. Imaging evaluation, catecholamine biochemical examination and adequate adrenergic α receptor blockers were administrated in all the cases. The surgical approaches included open transperitoneal surgery in 4 cases, robot-assisted laparoscopy in 1 case and retroperitoneal laparoscopy in 2 cases. The innovative techniques included mobilization of the liver, inferior vena cava transection and anastomosis, and transection of left renal vein. RESULTS: The average tumor size was (8.0±3.2) cm (3.6-13.9 cm). The median interval between the reoperation and the primary operation was 9 months (IQR: 6, 19 months). The median operation time was 407 min (IQR: 114, 430 min) and the median blood loss was 1 500 mL (IQR: 20, 3 800 mL). Operations of 5 cases were performed successfully, and 1 case failed only by exploration during the operation. One case died perioperatively. There were 5 cases of intraoperative blood transfusion, the median transfusion volume of red blood cells was 800 mL (IQR: 0, 2 000 mL). One case experienced postoperative lymphorrhagia, and recovered after conservative treatment. The renal function was normal in 2 cases after resection and anastomosis of inferior vena cava or transection of left renal vein. The average postoperative hospital stay was (7.2±3.3) d (4-13 d). The median follow-up time of 6 patients was 33.5 months (IQR: 4.8, 48.0 months). The case who failed in the reoperation still survived with tumor and there was no recurrence in the rest of the patients. CONCLUSION The reoperation of pheochromocytoma and paraganglioma, which can not be resected in the primary operation or recurred postoperatively, is difficult with high risk of hemorrhage, and there is a risk of failure and perioperative death. Different surgical approaches and strategies need to be adopted based on the different situation.
Adrenal Gland Neoplasms/surgery* ; Adult ; Female ; Humans ; Laparoscopy ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Paraganglioma/surgery* ; Pheochromocytoma/surgery* ; Reoperation ; Retrospective Studies

Adrenal Gland Neoplasms ; Heart Failure ; Humans ; Pheochromocytoma

OBJECTIVE: Plasma free metanephrines (MNs) have been widely used as an initial test for pheochromocytoma and paraganglioma (PPGL). PPGL without MNs elevation has been reported on rare occasions. The objective of this study was to analyze the clinical profile of sporadic PPGL patients with normal MNs. METHODS: In the study, 104 patients with sporadic PPGL diagnosed by histopathology in Peking University First Hospital from March 2015 to January 2020 were enrolled. All the patients had plasma MNs result, of whom, eight (7.69%) were with normal MNs. The reasons for their medical visits, clinical manifestations, the levels of plasma free MNs, 3-methoxytyramine (3-MT), catecholamines and chromogranin A (CgA), and the imaging findings were documented. Their preoperative diagnosis, perioperative medical management, and intraoperative blood pressure were analyzed. All the data mentioned above were compared with the MNs elevated group. The postoperative follow-up for MNs normal patients were applied. RESULTS: For the eight PPGL patients with normal plasma MNs, the most common clinical symptoms were sweating (3/8), abdominal and back pain (3/8), headache (2/8), palpitations (2/8), and fatigue (2/8). There were no significant differences in plasma free 3-MT and catecholamines' diagnostic positive rate between the MNs normal group and MNs elevated group, but the rate for plasma CgA was significantly decreased in the MNs normal group (2/5 vs. 41/43, P=0.005). No significant difference was found for the incidence of typical findings by enhanced CT between the two groups. In these eight MNs normal patients, six were diagnosed with PPGL by the previous history of PPGL, typical symptoms and CT findings, or elevation of 3-MT, CgA levels or positive results of PET-CT; two patients were misdiagnosed as nonfunctioning adenoma or primary aldosteronism. All these MNs normal patients underwent preoperative management with alpha adrenergic receptor blockers, of whom, one had an average intraoperative arterial pressure < 60 mmHg during surgery. The median follow-up time for the eight patients was 1.5 (0.5－4.5) years. No evidence of new tumors was found on the enhanced CT scans. Two MNs normal patients' plasma 3-MT and (or) CgA decreased to normal. CONCLUSION For patients with adrenal or retroperitoneal tumors, typical symptoms or a previous history of PPGL, normal plasma MNs is not a sufficient exclusion for PPGL. Plasma 3-MT, catecholamine, CgA results and the imaging findings are helpful for the diagnosis of PPGL. We recommend patients with suspected MNs normal PPGL take alpha adrenergic receptor blockers as preoperative blockade, but should avoid overdose. Postoperative follow-up for patients with normal MNs should focus on the positive biochemical markers before surgery.
Adrenal Gland Neoplasms ; Humans ; Metanephrine ; Paraganglioma ; Pheochromocytoma ; Positron Emission Tomography Computed Tomography

Objective To analyze the clinical features of Von-Hippel-Lindau(VHL)syndrome and explore the diagnostic value of abdominal ultrasound for this disease.Methods The clinical features including age at first diagnosis,symptoms,signs,affected organs,number of operations,and diagnostic examinations of 35 patients with VHL syndrome admitted to our center from January 1994 to December 2017 were retrospectively analyzed.The diagnostic value of abdominal ultrasound for VHL syndrome was analyzed.Results Pheochromocytoma(=14)and nervous system hemangioblastoma(=13)were the common firstly-identified tumors.Nervous system hemangioblastoma(=21),pheochromocytoma(=19),renal carcinoma(=17),and pancreatic mass(=15)were common tumors.The main surgical reasons were nervous system hemangioblastoma(=22),pheochromocytoma(=23)and renal carcinoma(=13).Abdominal organ involvements were found in 33 patients,which were first detected by abdominal ultrasound in 20 patients and were found accidently during routine health checkups in 6 patients.The ultrasound results were accurate in 27 of 33 adrenal gland scans,13 of 16 pancreas scans,and 8 of 19 kidney scans.Conclusions When multiple tumors are detected in the kidney,adrenal gland,and pancreas by ultrasound,the possibility of VHL syndrome should be considered.When the clinical findings suggest the possibility of VHL syndrome,ultrasound can discover and diagnose the abdominal tumors and can also be used for the long-term follow-up of the tumors.Therefore,ultrasound is an important method in the screening and follow-up of patients with VHL syndrome.
Abdomen ; diagnostic imaging ; Adrenal Gland Neoplasms ; diagnostic imaging ; Hemangioblastoma ; diagnostic imaging ; Humans ; Pheochromocytoma ; diagnostic imaging ; Retrospective Studies ; Ultrasonography ; von Hippel-Lindau Disease ; complications ; diagnostic imaging