Treatment Of Pheochromocytoma Research Articles

FRI194 Pheochromocytoma Presenting As Catecholamine-Induced Cardiomyopathy: A Case Report

Abstract Disclosure: M. Tsikala Vafea: None. D. Lee: None. Introduction: Pheochromocytoma (PCC) is a rare catecholamine-secreting neuroendocrine tumor presenting with the classic triad of episodic headache, sweating and tachycardia. Catecholamine-induced cardiomyopathy (CICMP) can occur as a complication of PCC in 7-11% of patients. It presents more commonly as dilated cardiomyopathy and inverted Takotsubo cardiomyopathy but also as hypertrophic or unspecified cardiomyopathy. Following appropriate pharmacologic treatment and resection of PCC, these cardiac complications usually resolve. Case report: 36 year old man with history of hypertension presented to the hospital with worsening orthopnea and cough. Following antibiotic treatment failure for suspected pneumonia, he was re-admitted with hemoptysis, tachycardia, hypoxia, and hypertensive emergency. Troponin and brain natriuretic peptide were found to be elevated. CT chest and abdomen showed pulmonary nodules and a large left adrenal mass measuring 9.6x9.3x9.4 cm, concerning for metastatic PCC. He was subsequently started on doxazosin. Transthoracic echo showed bi-atrial enlargement, diffuse severe left ventricle hypokinesis, reduced ejection fraction 25% and diastolic dysfunction. Stress cardiac MRI showed non-ischemic cardiomyopathy, corroborating the diagnosis of CICMP. After achieving adequate alpha-adrenergic blockade, he was started on goal-direct medical therapy consisting of valsartan, metoprolol and empagliflozin. His pulmonary nodular opacities improved on repeat imaging, congruent with pulmonary edema rather than metastases. Plasma and 24-hour urine metanephrines (2,964 pg/mL and 23,310 mcg/24 hr, respectively) and normetanephrines (9,266 pg/mL and 33,927 mcg/24 hr, respectively), as well as dopamine (493 pg/mL), epinephrine (3,205 pg/mL) and norepinephrine (above reportable range of 4,800 pg/mL) eventually came back significantly elevated, confirming his diagnosis of PCC. A month after presentation, he underwent left adrenalectomy, which revealed an 11 cm composite PCC ganglioneuroma. One-month postoperatively, urine metanephrines and normetanephrines were normal but his ejection fraction remained unchanged. Discussion: CICMP should be considered in patients presenting with acute heart failure and an incidental adrenal mass to avoid delays in treatment. Acute pulmonary edema may resemble pulmonary nodules on CT, so interpretation of these findings should correlate with the clinical presentation. Timely initiation of alpha-adrenergic blockade upon suspicion of PCC is crucial as it can significantly improve hemodynamics, and biochemical markers take time to result. 96% of CICMP cases improve after resection of PCC within a variable time frame ranging from one week to several months. Our patient’s CICMP had not improved one month after his adrenalectomy, but it remains to be seen whether he eventually recovers his cardiac function. Presentation: Friday, June 16, 2023

Image-Guided Precision Medicine in the Diagnosis and Treatment of Pheochromocytomas and Paragangliomas.

In this comprehensive review, we aimed to discuss the current state-of-the-art medical imaging for pheochromocytomas and paragangliomas (PPGLs) diagnosis and treatment. Despite major medical improvements, PPGLs, as with other neuroendocrine tumors (NETs), leave clinicians facing several challenges; their inherent particularities and their diagnosis and treatment pose several challenges for clinicians due to their inherent complexity, and they require management by multidisciplinary teams. The conventional concepts of medical imaging are currently undergoing a paradigm shift, thanks to developments in radiomic and metabolic imaging. However, despite active research, clinical relevance of these new parameters remains unclear, and further multicentric studies are needed in order to validate and increase widespread use and integration in clinical routine. Use of AI in PPGLs may detect changes in tumor phenotype that precede classical medical imaging biomarkers, such as shape, texture, and size. Since PPGLs are rare, slow-growing, and heterogeneous, multicentric collaboration will be necessary to have enough data in order to develop new PPGL biomarkers. In this nonsystematic review, our aim is to present an exhaustive pedagogical tool based on real-world cases, dedicated to physicians dealing with PPGLs, augmented by perspectives of artificial intelligence and big data.

Biomarker identification of immune-related genes in pheochromocytoma and paraganglioma.

Although we have a good understanding of the diagnosis and treatment of pheochromocytoma and paraganglioma (PPGL), the underlying pathogenesis and molecular pathways of PPGL need to be further studied. This study aimed to use bioinformatics to analyze the role of immune-related genes (IRGs) in the pathogenesis of PPGL. GSE19422 and GSE60459 microarray data were obtained from the Gene Expression Omnibus (GEO) database. Differentially expressed genes (DEGs) were identified using the "limma" package in R, and genes overlapping with IRGs were screened using the "VennDiagram" package. A protein-protein interaction (PPI) network was constructed in the Search Tool for the Retrieval of Interacting Genes/Proteins (STRING) database, and the core genes were identified by Cytoscape, followed by enrichment analysis and receiver operating characteristic (ROC) curve analysis to evaluate the diagnostic efficacy of the core genes. In addition, the level of immune cell infiltration of PPGL was analyzed and the target drug of the core gene was predicted. A total of 1,105 DEGs were identified from the 2 datasets, of which 94 were IRGs, suggesting that the occurrence of PPGL involved immune-related pathways. Through PPI and Cytoscape, a total of 2 core genes: fibroblast growth factor 2 (FGF2), FYN proto-oncogene (FYN), and vascular cell adhesion molecule 1 (VCAM1) were identified, and the ROC curve showed that these 3 core genes had good efficacy in the diagnosis of PPGL, and more than 50 potential therapeutic drugs could be predicted based on these 3 core genes. Subsequent immunoinfiltration analysis showed that mast cells activated were significantly elevated in patients with PPGL, negatively correlated with macrophages M2, and positively correlated with the level of dendritic cells activated. This study found that immunity is closely related to the occurrence of PPGL, and that FGF2, FYN, and VCAM1 may be potential biomarkers and therapeutic targets of PPGL.

Successful diagnosis and treatment of pheochromocytoma during severe coronavirus disease 2019 (COVID-19): a case report.

Pheochromocytoma is a rare but life-threatening condition due to catecholamine release induced by drug treatments such as β-blockers or glucocorticoids. We present a case of hypertensive crisis due to pheochromocytoma, induced after the initiation of dexamethasone and landiolol during intensive care for severe coronavirus disease 2019 (COVID-19). Based on a detailed medical history review, the patient was previously diagnosed with primary aldosteronism by confirmatory tests, moreover, an abdominal computed tomography scan identified an adrenal tumor 2 years before current admission. We tentatively diagnosed the patient with pheochromocytoma and initiated α-blockers without conducting a catecholamine report, leading to stable hemodynamics. We present a successfully managed case of pheochromocytoma concomitant with COVID-19, which has become a global crisis.

ДИАГНОСТИЧЕСКИЕ И ТАКТИЧЕСКИЕ ОШИБКИ ПРИ ВЕДЕНИИ БОЛЬНЫХ С ФЕОХРОМОЦИТОМОЙ

Objective: To analyze the causes of diagnostic and tactical errors in the management of patients with pheochromocytoma (PCC) and to develop a respective treatment and diagnostic algorithm. Methods: The mistakes made during clinical and instrumental diagnostics, preoperative preparation, and treatment of 55 patients with PCC were analyzed. Among the patients, there were 38 women (69.1%), and 17 men (30.9%). The mean age of the patients was 35.9±6.2 years. In 23 (41.8%) patients, right-sided localization of the tumor was noted, in 29 (52.7%) – left-sided one, while bilateral adrenal lesion occurred in 3 (5.5%) patients. The duration of arterial hypertension (AH) ranged from 6 months to 10 years (mean 4.56±1.7 years). Results: In 52.7% of cases, patients visited a doctor after more than three years from the onset of the first symptoms of the disease. The reasons for late treatment were: an insufficient examination of patients for the symptomatic genesis of hypertension due to low awareness of primary care physicians, self-administration of antihypertensive drugs, and treatment of patients by traditional healers. Errors in the interpretation of the results of ultrasound examination and computed tomography (CT) were noted in 7.3% of cases. During preoperative preparation, tactical errors were made in 7 patients, when instead of α-blockers, antihypertensive drugs of another class were prescribed, as a result of which two of the patients developed the syndrome of "uncontrolled hemodynamics" and died. In 5.5% of cases with large size of the tumor, iatrogenic injury to the inferior vena cava (IVC) and vessels of the right kidney occurred during the operation. In a histological examination of removed pheochromocytomas, the misinterpretation rate was 3.6%, and the diagnosis was reconsidered during repeated assessment. Conclusion: In more than half of the cases, during the primary diagnosis and treatment of PCC, various errors were made due to non-targeted examination of patients, incorrect interpretation of the results of radiological methods of examination, inadequate preoperative preparation of patients, as well as the iatrogenic injuries. In order to timely identify the discussed pathology in patients with AH, it is necessary to examine them in a targeted way for PCC. The basic medications for preoperative preparation of patients should be selective α-adrenoblockers. In order to prevent iatrogenic injuries of large vessels and organs of the retroperitoneal space, a thorough preoperative study of their relations with the tumor is necessary, as well as an improvement of the practical skills of the operating doctors. Keywords: Pheochromocytoma, diagnosis, errors, adrenalectomy, iatrogeny.

Наблюдение и лечение пациентки с гормонально-активной опухолью надпочечника

In spite of all existing achievements, modern endocrinology comes across a rather complex issue such as treatment of patients with catecholamine secreting tumors. True prevalence of pheochromocytoma is not known, as data obtained during the research depend on criteria of patients’ selection and can vary significantly. The tumors have numerous pathophysiological mechanisms of disease development due to a wide variability of symptoms and complex diagnostics. Meanwhile, timely diagnosis produces a direct effect on prognosis and quality of life. Catecholamine secreting tumors are commonly not detected. In such cases, there is a high risk of severe cardiovascular complications up to a lethal outcome. Complex diagnostics of this pathology also means that the tumors can have adrenal and extraadrenal localization and that the disease is hereditary. This makes diagnostics and treatment of pheochromocytoma even more complex. Timely detection of concomitant tumor and hormonal manifestations belongs to an important factor of management of patients with genetically determined pheochromocytomas. Thus, examination of pheochromocytoma is a pressing issue of modern endocrinology.

Insidious enemy - phaeochromocytoma. Can diabetes be cured?

We present the case of a 54-year-old patient with long-term type 2 diabetes, treated with intensive insulin therapy, who was considered to be ordinary diabetes. In a random imaging examination it turned out to be a phaeochromocytoma. The patient underwent surgical treatment. It turned out that after the surgery, the diabetes resolved. It is a casuistic curiosity that the patient was "cured" of the disease after diagnosis and treatment for pheochromocytoma, and on the second day after the surgery. It is also a valuable guide when looking for all possible causes of "secondary" diabetes.

Management of Patients with Treatment of Pheochromocytoma: A Critical Appraisal.

Simple SummaryPreoperative medical preparation with an alpha blockade has been implemented early 1960s to prevent intraoperative hemodynamic instability and consequently decrease morbi-mortality in patients at a high risk of complications. Indeed, surgery at that time consisted of open adrenalectomies with a high morbidity and mortality rate. Current clinical guidelines are based on these early experiences. However, emerging technologies have permitted a drastic reduction of the morbi-mortality rate in patients treated for pheochromocytoma. However, the clinical guidelines have not evolved at the same rate. The aim of this systematic review is to assess the evolution of the management of pheochromocytomas and to appraise the current clinical guidelines to the current clinical practices.The management of pheochromocytomas has significantly evolved these last 50 years, especially with the emergence of new technologies such as laparoscopic procedures in the 1990s. A preoperative blockade using antihypertensive medications to prevent intraoperative hemodynamic instability and cardiocirculatory events is recommended by current clinical guidelines. However, these guidelines are still based on former experiences and are subject to discussion in the scientific community. The aim of this systematic review was to assess the evolution of the management of pheochromocytomas. Laparoscopic procedure is established as the standard of care in current practices. Preoperative medical preparation should be questioned because it does not significantly improve intraoperative events or the risk of postoperative complications in current clinical practice. Current clinical recommendations should be revised and upgraded to current clinical practices.

Nuclear Imaging and Treatment of Pheochromocytoma and Paragangliomas

Nuclear Imaging and Treatment of Pheochromocytoma and Paragangliomas

Pheochromocytoma and paraganglioma with negative results for urinary metanephrines show higher risks for metastatic diseases.

Few studies have assessed the clinical features of pheochromocytoma and paraganglioma (PPGL) not producing excessive catecholamine. We aimed to clarify the clinical characteristics of PPGL patients with negative results for urinary metanephrines. This is a retrospective cross-sectional study. We established a database by combining datasets from the Nationwide Cohort Study on the Development of Diagnosis and Treatment of Pheochromocytoma in Japan (PHEO-J) and the Advancing Care and Pathogenesis of Intractable Adrenal diseases in Japan (ACPA-J). We compared the clinical differences between PPGL patients with negative results for urinary metanephrines and those with catecholamine-producing PPGL. Five hundred PPGL patients in the combined database were analyzed. Among them, 31 were negative for metanephrines. PPGL with negative results for urinary metanephrines was significantly associated with extra-adrenal disease (Odds ratio (OR) 6.58, 95% CI (confidence interval) 3.03-14.3, p < 0.001), the presence of metastatic disease (OR 4.22, 95% CI 1.58-11.3, p = 0.004), and negativity on meta-iodobenzylguanidine (MIBG) scintigraphy (OR 0.15, 95% CI 0.03-0.77, p = 0.023). Our findings demonstrate that PPGL patients with negative results for urinary metanephrines are associated with extra-adrenal lesions, metastatic disease, and negative MIBG findings. This suggests that PPGL patients with negative results for urinary metanephrines have a greater need for systemic whole-body imaging other than MIBG scintigraphy and close follow-up to monitor for metastasis than do patients with PPGL overtly producing excessive catecholamine.

Multidisciplinary practice guidelines for the diagnosis, genetic counseling and treatment of pheochromocytomas and paragangliomas

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and the sympathetic/parasympathetic neural ganglia, respectively. The heterogeneity in its etiology makes PPGL diagnosis and treatment very complex. The aim of this article was to provide practical clinical guidelines for the diagnosis and treatment of PPGLs from a multidisciplinary perspective, with the involvement of the Spanish Societies of Endocrinology and Nutrition (SEEN), Medical Oncology (SEOM), Medical Radiology (SERAM), Nuclear Medicine and Molecular Imaging (SEMNIM), Otorhinolaryngology (SEORL), Pathology (SEAP), Radiation Oncology (SEOR), Surgery (AEC) and the Spanish National Cancer Research Center (CNIO). We will review the following topics: epidemiology; anatomy, pathology and molecular pathways; clinical presentation; hereditary predisposition syndromes and genetic counseling and testing; diagnostic procedures, including biochemical testing and imaging studies; treatment including catecholamine blockade, surgery, radiotherapy and radiometabolic therapy, systemic therapy, local ablative therapy and supportive care. Finally, we will provide follow-up recommendations.

Deliberate Compensated Vasoplegia - a Novel Pharmacological Regimen for Controlling Arterial Blood Pressure During Surgery for Pheochromocytoma

Abstract Background: Intraoperative hemodynamic fluctuations are the most dreaded phenomenon associated with the treatment of pheochromocytoma. Preoperative alpha-adrenergic blockade protocols aimed at abating these fluctuations have achieved controversial results. No study to date has evaluated the use of intraoperative treatment protocols during surgery for pheochromocytoma. Deliberate compensated vasoplegia (DCV) is a novel pharmacological regimen developed at our institution intended to decrease severe hypertensive events. The aim of this study is to compare outcomes of pheochromocytoma resection with and without the DCV protocol. Methods: A retrospective analysis of all pheochromocytoma resections between the years 2012 and 2020 was performed. Resections performed with and without the DCV protocol were compared. The primary outcome measured was the incidence of severe intraoperative hypertension (mean arterial pressure ≥ 150 mmHg). Secondary outcomes included other abnormal blood pressure measurements as well as perioperative data and complications. Results: A total of 41 pheochromocytoma resections were included: 21 performed using standard practice and 20 with the DCV protocol. Analysis demonstrated no significant difference in preoperative parameters including tumor size, catecholamine levels, or alpha-blockade protocol. The use of the DCV protocol resulted in a significant decrease in the incidence of severe hypertensive episodes from 1.95±3.6 to 0.03±0.13 events/hour (p=0.008). The DCV protocol was not associated with any adverse events. Conclusions: This study demonstrates that DCV anesthesia protocol significantly decreases the incidence of severe hypertensive episodes during pheochromocytoma resection. This is the first study describing a highly effective protocol for controlling intraoperative hypertension and hemodynamic instability in pheochromocytoma patients.

Approach to pheochromocytoma and paraganglioma in children and adolescents: A retrospective clinical study from a tertiary care center

Pheochromocytoma (PCC) and paraganglioma (PGL) are rare tumors in childhood. They are catecholamine secreting tumors and present with signs or symptoms related to their excess. Most common signs and symptoms are hypertension, headache and diaphoresis. The management of children usually depend on experience of adulthood. This study is conducted to present the clinical characteristics, surgical management and outcome of childhood PCC and PGL in a tertiary care center. We reviewed clinical records of all patients operated for PCC and PGL between 2000 and 2020 retrospectively. There were 18 children operated for PCC and PGL in the study period. The female to male ratio was 1:1. The median age at diagnosis was 13 (IQR, 9-15) years. The most common presenting symptoms were headache and diaphoresis. Hypertension was the most common sign. Three patients had von Hippel-Lindau (VHL). Tumors of two patients with VHL were detected during routine follow-up. Three patients had multifocal disease. Medical preparation for surgery was carried out in all patients. Antihypertensive treatments were administered preoperatively. Since the patients are at risk for postoperative hypotension due to chronic vasoconstriction and blood volume contraction, high salt diet was recommended. Intravenous normal saline at a rate of 3000ml/m2 body surface area per day was started for intravascular volume expansion preoperatively. The mean duration for preoperative medication to achieve normal blood pressure was 22 days (range, 16-30). Twenty-five tumors were excised in eighteen patients. One patient who had bone metastases on diagnosis and is on I131MIBG therapy. The median follow-up time was 5.6 years (range, 1 months - 21 years). Five patients reached adulthood during the study period. Four of these had recurrent metastases (n=2) and new tumors (pancreatic neuroendocrine tumor, n=1 and pancreatic neuroendocrine tumor and renal cell carcinoma, n=1) after the age of 18. Multidisciplinary approach is necessary to achieve safe surgical treatment and surveillance of PCC and PGL. Detection of associated familial cancer susceptibility syndromes and long-term follow-up is essential to detect late recurrences and new tumors.

PDK1 Inhibitor GSK-470 Exhibits Potent Anticancer Activity in a Pheochromocytoma PC12 Cell Tumor Model via Akt/mTOR Pathway.

Phosphoinositide-Dependent Kinase 1 (PDK1) is now widely studied in malignant solid tumors. Researchers have previously revealed that targeting PDK1 is thought of as a promising anticancer treatment strategy. The aim of this study was designed to evaluate the anticancer activity of GSK-470, a novel and highly specific inhibitor of PDK1, in Pheochromocytoma (PCC) tumor model. PC12 cells were xenografted into nude mice to build PCC tumor model. Animals were treated with GSK-470 vs vehicle. Mean tumor volume was calculated and compared across groups. TUNEL was used to detect apoptosis. The effects of PDK1 inhibitor GSK-470 on activation of the Akt signaling and its downstream Akt/mTOR pathway in xenotransplant tumor tissues were examined by western bolt. The mean tumor volume in GSK-470 group was significantly less than that in control group. TUNEL results found that cell apoptosis was markedly increased in GSK-470 group compared with the control group. The western bolt analysis showed that the phosphorylation of Akt at threonine 308 was significantly reduced in GSK-470 group. Also, GSK-470 strongly inhibited phosphorylation of mTOR on Ser2448, a marker for mTORC1 activity, as well as phosphorylation of p70S6K, best characterized targets of mTOR. Our results showed that GSK-470 exhibited potent anticancer activity in PC12 tumor-bearing mice. Also, we found that this effect appeared to be mediated by the inhibition of the Akt/mTOR pathway. The present study once again provides new insights into the therapeutic effects of inhibiting PDK1 in the treatment of malignant PCC. Therefore, we propose that GSK-470 might be an effective therapeutic agent for the treatment of malignant PCC.

SAT-212 Management of VHL-Associated Pheochromocytoma in Pregnancy

Background: The appropriate treatment of pheochromocytoma during pregnancy is crucial because, while its incidence is rare (0.007%), it is associated with increased maternal and fetal mortality1. Clinical Case: A 32 year old Caucasian primigravida female presented with intrauterine pregnancy at 25 weeks for evaluation and management of a 9 mm left adrenal mass. The patient was previously diagnosed at age 10 with Von-Hippel Lindau (VHL) syndrome and genetic testing was positive for a VHL gene mutation (Type 2C VHL). At age 14, she underwent laparoscopic adrenalectomy to excise a right sided pheochromocytoma. At age 20, imaging revealed an enhancing left adrenal mass with imaging characteristics consistent with pheochromocytoma and with catecholaminergic labs that were normal or only mildy increased to 1.5 times upper normal range. Pre-partum the patient had been experiencing periodic episodes of headaches and palpitations. She reported experiencing baseline levels of palpitations and sweating with new onset of pre-syncope and hypotension during her pregnancy. Labs revealed normal serum levels of fractionated free metanephrines. Alpha adrenergic blockade, beta adrenergic blockade, and surgery were considered but deemed unnecessary as the patient was asymptomatic, imaging indicated the left adrenal mass was stable in size, and the patient wished to avoid lifelong adrenal replacement therapy. The patient’s blood pressure and heart rate were monitored closely throughout pregnancy and the use of alpha blockade or beta blockade was not needed. The patient had an uncomplicated induced delivery at 39 weeks. MRI with contrast of abdomen performed 10 months postpartum confirmed the presence of an enhancing 8 mm lesion on the inferior pole of the left adrenal gland with imaging characteristics again consistent with pheochromocytoma. Conclusion: This case demonstrates that a conservative approach of monitoring blood pressure and heart rate can be sufficient and appropriate for management of some patients with pheochromocytoma and VHL during pregnancy.Citation:1. ENDOCRINOLOGY IN PREGNANCY: Pheochromocytoma in pregnancy: case series and review of literature in: European Journal of Endocrinology Volume 177 Issue 2 (2017).

PD31-09 PARTIAL ADRENALECTOMY VERSUS TOTAL ADRENALECTOMY IN THE TREATMENT OF PHEOCHROMOCYTOMA IN PATIENTS WITH VON HIPPEL LINDAU

INTRODUCTION AND OBJECTIVE: Pheochromocytomas are functional tumors of the adrenal gland, that have an incidence rate of approximately 20% in patients with Von Hippel Lindau (VHL). Traditional management of these tumors has been total adrenalectomy. However, adrenal sparing surgical techniques have been recommended as a standard of care for these tumors. This study queried the National Inpatient Sample (NIS) database to examine the utilization of partial adrenalectomy in the treatment of pheochromocytomas in patients with VHL. METHODS: All patients admitted to the hospital between 2005 and 2014 with a diagnosis of pheochromocytoma were identified in the NIS database. VHL patients were selected and admissions involving total and partial adrenalectomies were identified. Demographic and hospital characteristics were extracted, and differences were compared using descriptive statistics. RESULTS: There were 33,535 admissions of patients with pheochromocytomas who underwent an adrenalectomy, with 0.7% of these patients also having VHL. Of the patients with VHL, 33% underwent a partial adrenalectomy and 67% underwent total adrenalectomy. The average age of patients who had a partial adrenalectomy was 20 years and 31 years for patients who underwent a total adrenalectomy. The mean length of stay of patients who had a partial adrenalectomy was 4 days compared to 8 days for total adrenalectomy, however no differences were observed in the type of hospital where the patients were treated. In regard to comorbidities of patients who received a partial vs total adrenalectomy, no differences in the rates of hypertension [29% vs. 47%, p=0.34], coronary artery disease [0% vs. 0.9%, p=0.45], or diabetes mellitus [0% vs. 14%, p=0.22] were observed. No differences in rates of transfusion or post-operative adrenal insufficiency were observed [p=0.21 and 0.76 respectively]. CONCLUSIONS: Partial adrenalectomy offers the benefit of sparing adrenal tissue, which can reduce the probability of adrenal insufficiency and steroid dependence in patients with VHL who are at risk for recurrent tumors as well as bilateral disease involvement. This analysis demonstrated that the majority of VHL patients with pheochromocytomas underwent total adrenalectomy. Patients who underwent partial adrenalectomies were on average younger and had shorter lengths of stay. Despite recommendations, partial adrenalectomy continues to be under-utilized as a surgical management strategy for VHL pheochromocytomas. Source of Funding: N/A

Canadian Urological Association Best Practice Report: Long-term surveillance following resection of pheochromocytoma.

Background Pheochromocytoma is a tumor of the catecholamine-producing cells of the adrenal medulla. The incidence is estimated to be 1–2 cases per 100 000 individuals and they make up approximately 5% of incidental adrenal masses.1 Classical symptoms of pheochromocytomas include headache, episodic perspiration, tachycardia, flushing, nausea, and hypertension, although many tumors can be asymptomatic.1 These tumors can be sporadic or hereditary. 2 Familial cases account for up to 30% of tumors.3 Pheochromocytomas can also occur outside the adrenal gland, known as paragangliomas, in up to 25% of cases.4 About 10% of pheochromocytomas are malignant.5 Although there are several clinical and genetic factors associated with an increased risk of malignancy, at this time, there are no molecular, cellular, or histological criteria that can reliably differentiate benign from malignant disease.5 Therefore, malignancy is defined by the presence of clinical metastases. The most common sites for metastases are lymph nodes, bones, liver, and lungs.6 Evaluation of suspected pheochromocytoma begins with confirming a biochemical disturbance by measuring plasma-free metanephrines or urinary fractionated metanephrines, followed by computed tomography (CT) imaging. Once a pheochromocytoma is confirmed, complete surgical resection of the tumor is advised, preferably via laparoscopic or robot-assisted adrenalectomy. The full details regarding workup and treatment of pheochromocytomas is beyond the scope of this review, but there are recent published guidelines on this topic.7 Following surgery, patients are at risk for tumor persistence and recurrence. Despite an overall good prognosis, the disease can recur in up to 16% of patients within 10 years following surgery.8,9 Recurrences may be local or metastatic and have been reported up to 53 years post-initial resection, making long-term followup essential.10 Extra-adrenal disease, hereditary pheochromocytomas, right-sided tumors, bilateral tumors, and larger tumors are thought to be risk factors for recurrence. Currently, there is no consensus on the proper methodology for followup. There have been no randomized studies addressing optimal followup nor prospective registries to provide higher-quality evidence for this issue. Important clinical questions remain regarding duration of followup and which tests should be used to detect and monitor recurrences.

Radiotherapy of malignant pheochromocytoma—a case report

Pheochromocytomas (PCC) are rare tumors with an estimated incidence of 0.4 to 9.5 cases per 1 million per year. About 5–26% of PCC are malignant and presents with metastasis, for which there is currently no effective therapy. The treatment of choice is for PCC is radical surgery to reduce tumor burden, to provide symptomatic relief of catecholamine excess although complete eradication of the lesions is often not feasible. A number of case reports have been published on the role of radiotherapy for the treatment of PCC. Here we present a 53-year-old male stage III malignant PCC patient who received postoperative adjuvant radiotherapy. A review of current literature is also presented.

Pheochromocytomas and Paragangliomas: New Developments with Regard to Classification, Genetics, and Cell of Origin.

Pheochromocytomas (PCC) and paragangliomas (PGL) are rare neuroendocrine tumors that arise in the adrenal medulla and in extra-adrenal locations, such as the head, neck, thorax, abdomen, and pelvis. Classification of these tumors into those with or without metastatic potential on the basis of gross or microscopic features is challenging. Recent insights and scoring systems have attempted to develop solutions for this, as described in the latest World Health Organization (WHO) edition on endocrine tumor pathology. PCC and PGL are amongst the tumors most frequently accompanied by germline mutations. More than 20 genes are responsible for a hereditary background in up to 40% of these tumors; somatic mutations in the same and several additional genes form the basis for another 30%. However, this does not allow for a complete understanding of the pathogenesis or targeted treatment of PCC and PGL, for which surgery is the primary treatment and for which metastasis is associated with poor outcome. This review describes recent insights into the cell of origin of these tumors, the latest developments with regard to the genetic background, and the current status of tumor classification including proposed scoring systems.

CON: Surgical treatment of phaeochromocytoma - query pre-treatment?

CON: Surgical treatment of phaeochromocytoma - query pre-treatment?