Familial paraganglioma syndrome (FPS) is a rare genetic disorder characterized by the development of paragangliomas (PGLs) and pheochromocytomas (PCCs). Here, we describe the case of a 42-year-old man with a family history of FPS, who presented with episodic chest pain and was diagnosed with acute coronary syndrome secondary to cardiac PGL-induced vasospasm. A thorough review of the family history confirmed several cases of PGLs and PCCs in the immediate family circle. A pathogenic variant in the succinate dehydrogenase (SDH) gene was revealed, elucidating the hereditary nature of the condition. Subsequent gallium (68Ga)-edotreotide positron emission tomography confirmed the presence of multiple lesions with increased uptake consistent with PGLs, including 2 primary cardiac PGLs that may have accounted for a coronary vasospasm due to the secretion of catecholamines.

Genetic testing and sequencing technologies offer a comprehensive understanding of cancer genetics, providing rapid and cost-effective solutions. In particular, these advanced technologies play an important role in assessing the complexities of the rare cancer types affecting several systems including the bone, endocrine, digestive, vascular, and soft tissue. This review will explore how genetic testing and sequencing technologies have contributed to the identification of biomarkers across several rare cancer types in diagnostic, therapeutic, and prognostic stages, thereby advancing PM.

A comprehensive literature search was conducted across PubMed (MEDLINE), EMBASE, and Web of Science using keywords related to sequencing technologies, genetic testing, and cancer. There were no restrictions on language, methodology, age, or publication date. Both primary and secondary research involving humans or animals were considered.

In practice, fluorescence in situ hybridization, karyotype, microarrays and other genetic tests are mainly applied to identify specific genetic alterations and mutations associated with cancer progression. Sequencing technologies, such as next generation sequencing, polymerase chain reaction, whole genome or exome sequencing, enable the rapid analysis of millions of DNA fragments. These techniques assess genome structure, genetic changes, gene expression profiles, and epigenetic variations. Consequently, they help detect main intrinsic markers that are crucial for personalizing diagnosis, treatment options, and prognostic assessments, leading to better patient prognosis. This highlights why these methods are now considered as primary tools in rare cancer research. However, these methods still face multiple limitations, including false positive results, limited precision, and high costs.

Genetic testing and sequencing technologies have significantly advanced the field of rare cancer research by enabling the identification of key biomarkers for precision diagnosis, treatment, and prognosis. Despite existing limitations, their integration into clinical and research fields continues to improve the development of personalized medicine strategies for rare and complex cancer types.

Paraganglioma (PGL) is a neuroendocrine tumor originating from paraganglia that can occur in various locations, such as the head, neck, chest, abdomen, and pelvis. Retroperitoneal PGLs are rare, and recurrent cases in this area are particularly uncommon, posing considerable surgical complexities. Owing to their neuroendocrine activity, PGLs are capable of secreting hormones like catecholamines, thereby presenting significant challenges in hemodynamic management during the perioperative period.

We report a 64-year-old man with a recurrent retroperitoneal PGL. The patient underwent retroperitoneal mass resection in 2013, with postoperative pathology revealing a PGL. Regular follow-up was not conducted until April 2024, when a computed tomography scan revealed a huge mass in the retroperitoneum, closely adjacent to the abdominal aorta. Laboratory examinations revealed elevated levels of catecholamines in the patient's blood serum. Upon admission, volume expansion and blood pressure (BP) monitoring were carried out for one week, with catecholamine levels reviewed and normalized. Adequate preoperative preparation was conducted, including central venous access, arterial BP monitoring, and the preparation of vasoactive agents. During tumor resection, the patient experienced acute, significant fluctuations in BP. The timely intervention of the anesthesiologist stabilized the BP, facilitating the successful resection of the tumor which was confirmed as a recurrent PGL. Postoperative follow-up revealed no evidence of tumor residual or recurrence.

PGL recurrence is rare but non-negligible. PGLs adjacent to major arteries complicate surgery, and perioperative hemodynamic stability demands meticulous attention.

Pheochromocytoma is a rare adrenal medulla tumor that overproduces catecholamines, causing major cardiovascular issues. It is often found incidentally during imaging, but large tumors pose unique perioperative challenges. Patients are usually symptom-free until the tumor grows, risking an adrenergic crisis during surgery. This case underscores the complexities of managing large pheochromocytomas, highlighting the risks of unstable hemodynamics and the need for thorough preoperative planning and a multidisciplinary approach for successful surgery.

We present a case of a 55-year-old Chinese man admitted for surgery after a routine exam revealed a 7.8 cm mass in his left adrenal gland. Initially asymptomatic, he was diagnosed with pheochromocytoma via blood tests and computed tomography scans. Pre-surgery, he received an α-receptor blocker. During the tumor removal, which was large and adhered to nearby vessels and tissues, he suffered an adrenergic crisis with unstable hemodynamics.We implemented emergency measures to stabilize the patient's vital signs in a rare case of large pheochromocytoma with adrenal crisis. With multidisciplinary team management, the patient recovered well post-surgery.

This article discusses the disease's unique clinical features, reviews literature on the link between massive pheochromocytoma and adrenal crisis, and outlines perioperative management strategies as a reference.

En-bloc resection of bladder tumors is performed for bladder cancer with the goal of achieving precise tumor resection and providing an informative pathology specimen. We report the first case of a safely resected bladder paraganglioma by en-bloc resection.

A 57-year-old woman presented with an incidental bladder tumor detected on computed tomography. Cystoscopy revealed a 10 mm submucosal tumor. Magnetic resonance imaging indicated a suspected bladder paraganglioma, whereas endocrinological tests showed no abnormalities. The tumor was completely resected in one piece without an intraoperative increase in blood pressure or bladder perforation. Pathological examination revealed a bladder paraganglioma with negative surgical margins. No evidence of recurrence was observed at 9 months follow-up.

En-bloc resection of bladder tumors may be a useful approach for selected cases of bladder paragangliomas, potentially decreasing the risk of intraoperative blood pressure fluctuations and ensuring complete tumor resection.

Pheochromocytomas are neuroendocrine tumors that derive from sympathetic adrenomedullary chromaffin tissue and produce catecholamines. Due to the excess release of catecholamines, they can produce arterial hypertension, tachycardia, sweating, headache and a large number of other clinical manifestations secondary to the stimulation of α and β adrenoreceptors. Screening for pheochromocytoma is recommended in patients with paroxysmal, resistant or early-onset arterial hypertension, in cases with symptoms suggestive of catecholamine hypersecretion, patients with hereditary syndromes associated with pheochromocytomas, diabetes mellitus of atypical presentation and in adrenal incidentalomas with radiological characteristics not typical of adenoma (with > 10 Hounsfield Units on non-contrast CT). In this article we present an exhaustive review of the clinical data and complications that can be associated with pheochromocytomas, and we summarize the main indications for pheochromocytoma screening.

This study aimed to characterize the causes of death and compute the risk of mortality due to each cause among patients with malignant adrenal tumours.

Data from malignant adrenal tumour patients were collected from the Surveillance, Epidemiology, and End Results (SEER) database (2004-2020). With reference data from the general population, the standardized mortality ratio (SMR) was calculated to assess all causes of death for malignant adrenal tumour patients.

A total of 1651 patients who died from primary malignant adrenal neoplasms were included; 854 cases of adrenocortical carcinoma (ACC)-related death, 118 cases of pheochromocytoma (PCC)-related death and 333 cases of neuroblastoma (NB)-related death were identified for further analysis. Approximately 56.78%~87.69% of patients died from primary malignant adrenal tumours, 7.21%~13.56% died from secondary malignant neoplasms (SMNs), and 5.11%~29.66% died from noncancer diseases. The main causes of death associated with SMNs included lung and bronchial cancer and soft tissue cancers, including heart, kidney and renal pelvis cancers; the noncancer causes of death included mainly heart disease, septicemia, and cerebrovascular disease. Compared with chemotherapy-naïve patients, chemotherapy-treated patients had higher SMRs of SMNs, including cancers of the colon (excluding the rectum), lung, bronchus, bones and joints; soft tissues, including the heart, kidney and renal pelvis; the brain and peripheral nervous system; and leukaemia, as well as nontumor diseases, including heart disease, septicemia, and cerebrovascular disease. Patients with NB were more likely to die from SMNs, including soft-tissue malignancies of the heart, bones and joints; brain; peripheral nervous system; the female genital system, including the ovary; leukaemia, including lymphocytic leukaemia; myeloid and monocytic leukaemia; and lymphoma, including non-Hodgkin lymphoma.

In addition to primary cancer, SMNs and nontumor diseases were important causes of death in patients with malignant adrenal tumours. Neuroblastoma patients and chemotherapy- treated patients are more likely to die from SMNs and should monitored closely.

Paragangliomas of the head and neck area should be detected early and diagnosed in their complexity at specialized centers, treated and, in the presence of paraganglioma syndrome, cared for for life. The continuing education article focuses in an abridged form on the most important basics of a complex clinical picture and deals with both sporadic head and neck paragangliomas and hereditary paraganglioma syndromes. It contains concise recommendations regarding genetic testing, the diagnosis of multicentric tumors, the multidisciplinary treatment approach and the need for lifelong monitoring.

Mediastinal paragangliomas are rare neoplasms arising from extra-adrenal neural crest cells, presenting as either functional or nonfunctional tumors. Clinical manifestations range from catecholamine-related symptoms to physical compression effects. Accurate recognition of these tumors is crucial for diagnosis and management due to their rarity and association with vital mediastinal structures.

We report the case of a 53-year-old Syrian male presenting with progressive dyspnea, fatigue, and weakness. Imaging revealed a highly vascular anterior mediastinal mass compressing the superior vena cava, diagnosed as a nonfunctional paraganglioma. Preoperative evaluations included endocrine consultation and blood transfusion preparations. The tumor was surgically excised via lateral thoracotomy without complications. Postoperative imaging confirmed the absence of residual mass, and two years of regular follow-ups revealed no recurrence or metastasis.

Mediastinal paragangliomas pose diagnostic and management challenges due to their rarity and potential for extensive vascular involvement. Nonfunctional paragangliomas, in particular, may remain asymptomatic until they exert significant mass effects. Surgical resection is the definitive treatment, often requiring a multidisciplinary approach. Tumor size and patient age influence malignancy risk and recurrence, highlighting the need for thorough perioperative planning and long-term follow-up.

This case underscores the importance of precise diagnosis, meticulous perioperative management, and surgical intervention in managing mediastinal paragangliomas. Lifelong monitoring is essential, especially for larger tumors, to ensure early detection of recurrence. Comprehensive understanding and documentation of such cases contribute to improved patient outcomes and enhanced clinical management strategies.

Pheochromocytoma/paraganglioma (PGPG) is a rare neuroendocrine tumor. Amino acid metabolism is crucial for energy production, redox balance, and metabolic pathways in tumor cell proliferation. This study aimed to build a risk model using amino acid metabolism-related genes, enhancing PGPG diagnosis and treatment decisions. We analyzed RNA-sequencing data from the PCPG cohort in the GEO dataset as our training set and validated our findings using the TCGA dataset and an additional clinical cohort. WGCNA and LASSO were utilized to identify hub genes and develop risk prediction models. The single-sample gene set enrichment analysis, MCPCOUNTER, and ESTIMATE algorithm calculated the relationship between amino acid metabolism and immune cell infiltration in PCPG. The TIDE algorithm predicted the immunotherapy efficacy for PCPG patients. The analysis identified 292 genes with differential expression, which are involved in amino acid metabolism and immune pathways. Six genes (DDC, SYT11, GCLM, PSMB7, TYRO3, AGMAT) were identified as crucial for the risk prediction model. Patients with a high-risk profile demonstrated reduced immune infiltration but potentially higher benefits from immunotherapy. Notably, DDC and SYT11 showed strong diagnostic and prognostic potential. Validation through quantitative Real-Time Polymerase Chain Reaction and immunohistochemistry confirmed their differential expression, underscoring their significance in PCPG diagnosis and in predicting immunotherapy response. This study's integration of amino acid metabolism-related genes into a risk prediction model offers critical clinical insights for PCPG risk stratification, potential immunotherapy responses, drug development, and treatment planning, marking a significant step forward in the management of this complex condition.

Background: Multiple endocrine neoplasia type 2A (MEN 2A) is a rare hereditary cancer syndrome caused by pathogenic variants in the rearranged during transfection (RET) gene and is characterized by medullary thyroid carcinoma (MTC), pheochromocytoma (PHEO), primary hyperparathyroidism (PHPT), cutaneous lichen amyloidosis (CLA), and Hirschsprung's disease. Phenotypic data on the RET C611Y variant remain sparse. Consequently, we aimed to establish a clinical risk profile. Methods: We conducted a nationwide study of all cases (n = 128) born after 1 January 1930 and recognized as carrying the RET C611Y variant in Denmark before 1 April 2021. Results: The median follow-up after birth was 47 years (range, 3-92). Age-related penetrance at age 70 years for MTC was 98% (CI, 91-100), for PHEO 24% (CI, 16-37), and for PHPT 10% (CI, 5-20). None had CLA or Hirschsprung's disease. The age-related progression of MTC was significant (p < 0.001). The median age at T0N0M0 was 11 years (2-62), at T1-4N0M0 was 37 years (12-65), at TxN1M0 was 47 years (16-79), and at TxNxM1 was 50 years (28-70). At the last follow-up, 56% of thyroidectomized cases (n = 103) were biochemically cured. Overall survival at 70 years was 74% (CI, 59-84). Conclusions: RET C611Y is associated with a very high penetrance of MTC and a low penetrance of PHEO and PHPT. CLA and Hirschsprung's disease almost never occur. MTC seems moderately aggressive, but large variability can be seen. Overall survival may be comparable to that of the general population.

The aim of the study was to investigate sex differences in presentation of pheochromocytomas and paragangliomas (PPGLs).

This is a retrospective cohort study including 183 patients with confirmed PPGL (females n=100, pheochromocytoma n=156) between year 2005 and 2023, attending Department of Endocrinology, Karolinska University Hospital, Stockholm. The collected data included the mode of presentation, symptoms, biochemical, genetic and histopathological test results.

The mean age at surgery/diagnosis was 54.9 ± 17.0 years. Sweating was more common in females compared to males (44% vs 23%, p=0.003), and also takotsubo syndrome (10% vs 0% p=0.002). Males, on the other hand, were more likely to experience pallor (16% vs 4%, p=0.009), and were more often diagnosed due to investigations of a suspected PPGL (31% vs 18%, p=0.039) although no difference was found in the classic triad (sweating, palpitations and headache). Left-sided pheochromocytoma was more common among males than females (48% vs 29%, p=0.009). No differences between sexes were found in biochemical, genetical or histopathological results, or presence of metastasis at diagnosis.

The reported symptoms by patients with PPGL were generally similar between the sexes, except for pallor and sweating. Takotsubo syndrome was more common among females. More males with PPGL were found based on suspicion than females. Further research into sex differences in various aspects of PPGL should be pursued.

Adrenal diseases pose significant diagnostic challenges due to the wide range of neoplastic and non-neoplastic pathologies. Radiologists have a crucial role in diagnosing and managing these conditions by, leveraging advanced imaging techniques. This review discusses the vital role of computed tomography (CT), magnetic resonance imaging (MRI), and nuclear medicine in adrenal imaging, and focuses on morphological and functional evaluations. First, the anatomy and physiology of the adrenal glands are described, followed by a discussion on ectopic adrenocortical adenomas and how they develop. The concepts and imaging findings of congenital diseases, such as congenital adrenal hyperplasia (CAH), adrenal rest tumors, and adrenocortical nodular disease, considering recent updates to the WHO Classification of Tumours (5th ed.) terminology are highlighted. The diagnostic value of dynamic contrast-enhanced CT and chemical-shift MRI for identifying adrenocortical adenomas are emphasized, alongside the use of adrenocortical scintigraphy such as 131I-adosterol scintigraphy for diagnosing Cushing's disease, Cushing's syndrome (CS), subclinical CS, and ectopic adrenocorticotropic hormone-producing tumors. Systemic complications associated with CS, and the diagnosis and treatment of pheochromocytomas, paragangliomas (PPGLs), and neuroblastomas, will also be discussed focusing on 123I-metaiodobenzylguanidine (MIBG) imaging and 131I-MIBG therapy. Pitfalls in 123I-MIBG imaging and the increasing importance of diagnosing hereditary PPGLs due to increased genetic testing are also be discussed. Additionally, the broad differential diagnosis for adrenal masses-including malignancies like adrenal carcinoma, metastases, and malignant lymphoma, as well as benign conditions like myelolipoma and ganglioneuroma, and complications, such as adrenal hemorrhage, infarction, and infections-will be outlined. The goal of this review was to provide an overview of adrenal diseases that includes the most recent information for radiologists to stay updated on the latest imaging techniques and advancements that can ensure accurate diagnosis and effective management.

The purpose of this review is to outline the current knowledge on epidemiology, diagnosis and management of neuroendocrine neoplasms (NENs) that develop in the context of Von Hippel-Lindau (VHL) syndrome.

Pancreatic NENs develop in 8-17% of VHL patients (vPNENs) and are mostly multi-focal, cystic and non-functioning. Surgical resection is recommended for vPNENS > 3 cm that exhibit higher metastatic potential or in tumors with short doubling time while in the 20% of cases with metastatic disease the HIF-2 A inhibitor belzutifan is considered a promising option. Pheochromocytomas arising in VHL type 2 are often bilateral and have a noradrenergic phenotype while they are associated with increased risk of recurrence. High-specific activity [131I]-MIBG and sunitinib are the treatment options with the highest level of evidence whereas studies on belzutifan are evolving. Life-long surveillance and management in the context of a multidisciplinary team are suggested to achieve the best clinical outcome.

Pheochromocytoma is a rare neuroendocrine tumour that develops from chromaffin cells in the adrenal medulla and is characterised by the excessive production of catecholamines and their metabolites. Diagnostic confirmation is performed by detecting elevated levels of catecholamines and/or their metabolites in plasma or 24-h urine. In the case of moderate elevations of normetanephrine, the clonidine suppression test may be useful to differentiate between endogenous hypersecretion and false positive results. Once the biochemical diagnosis is performed, the tumour localisation is carried out using imaging techniques and sometimes with nuclear medicine imaging tests. Furthermore, in all patients with pheochromocytomas it is recommended to perform a genetic study to identify hereditary disorders that may be present in more than 30% of cases and to perform a cardiological evaluation to rule out the presence of cardiovascular involvement secondary to the catecholamine hypersecretion.

Purpose To evaluate the diagnostic performance of gallium 68 (68Ga)-DOTA-NaI3-octreotide (68Ga-DOTANOC) and fluorine 18 (18F)-fluoro-l-3,4-dihydroxyphenylalanine (18F-FDOPA) PET/CT in detecting recurrent or metastatic paragangliomas. Materials and Methods This single-center retrospective study included patients with paragangliomas who underwent both 68Ga-DOTANOC PET/CT and 18F-FDOPA PET/CT between August 2021 and December 2023. The diagnostic performance of these two tracers in detecting recurrent or metastatic tumors was compared using several metrics, including sensitivity, negative predictive value, and accuracy. Results This study included 36 patients (median age, 52 years [range, 14-78 years]; 16 female, 20 male). Of these, nine underwent initial 68Ga-DOTANOC and 18F-FDOPA PET/CT examinations before treatment, and the remaining 27 underwent posttreatment examinations. Twenty-two of those 27 patients had recurrence or metastasis. According to lesion-level analysis, 68Ga-DOTANOC had higher sensitivity, negative predictive value, and accuracy for diagnosis of bone metastases than did 18F-FDOPA PET/CT (97% vs 78% [P < .001], 85% vs 42% [P = .02], and 97% vs 81% [P < .001], respectively). 18F-FDOPA PET/CT had higher sensitivity, negative predictive value, and accuracy for the diagnosis of liver metastases than did 68Ga-DOTANOC PET/CT (73% vs 15% [P < .001], 68% vs 41% [P = .04], and 83% vs 46% [P < .001], respectively). According to patient-level analysis, the sensitivity of 18F-FDOPA PET/CT for diagnosing liver metastases was higher than that of 68Ga-DOTANOC PET/CT (88% vs 25%; P = .04). Conclusion In patients with recurrent or metastatic paragangliomas, 68Ga-DOTANOC PET/CT showed better performance than 18F-FDOPA PET/CT in detecting bone metastases, and 18F-FDOPA PET/CT performed better in detecting liver metastases. Keywords: 68Ga-DOTANOC, 18F-FDOPA, Pheochromocytoma, Paraganglioma Published under a CC BY 4.0 license.

Phaeochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors with clinical heterogeneity and a high association with hereditary disease, affecting approximately 30 % of the cases. Differences in the presentation and genetic etiologies of PPGLs have been demonstrated between Chinese and European patients. The frequency of germline genetic diagnosis was remarkably higher in Brazilian patients (∼50 %) compared with other cohorts (Chinese 21 %, European 31 %, and The Cancer Genome Atlas Program cohort 27 %). Interestingly, germline SDHB genetic defects were also more prevalent in Brazilian patients (17 %) with PPGLs when compared with other cohorts (3-9 %). The SDHB exon 1 deletion was responsible for approximately 50 % of the SDHB pathogenic/likely pathogenic variants in Brazilian patients with PPGLs due to a founder effect. The germline SDHB exon 1 deletion represents ∼10 % of the germline drivers in Brazilian patients (and possibly in Latin America). Therefore, a single diagnostic PCR for the SDHB exon 1 deletion might be very useful in clinical practice for genetic testing and counseling of patients with PPGLs in Latin America.

Significant advances have been made in the past few decades in surgical management and outcomes of patients with pheochromocytoma and paraganglioma. Improvements in preoperative hypertensive control with the implementation of alpha- and beta-adrenergic blockade has resulted in better intra-operative blood pressure control and less incidence of hypertensive crises, which had been a large source of morbidity in the past. Emphasis on anesthesia and surgical team communication has also assisted in minimizing intraoperative hypertensive events at critical points of the operation. Shifting away from open resection, the now standard-of-care laparoscopic and minimally invasive adrenalectomy offers less pain, shorter hospitalizations, and quicker recoveries. Patient underlying germline mutations can guide the timing, approach, and extent of surgery. Postoperative outcomes have significantly improved with recent advancements in perioperative care in addition to regimented biochemical and radiographic surveillance. Here, we highlight the recent advancements in surgical approaches and outcomes for patients with pheochromocytoma and paraganglioma.

Catecholamine-producing tumours are rare entities that, even though their clinical diagnostic might be a challenge due to the non-specificity of the symptoms, have had a growing incidence due to the continuous improvement of medical imaging examinations and the evolution of molecular genetic testing. On the other hand, they can rarely manifest as serious complications, such as myocardial infarction, stroke, or alveolar haemorrhage. This paper describes the case of a 77-year-old Caucasian man who presented with acute onset of left upper quadrant abdominal pain. The first abdominal computed tomography (CT) scan showed an active haemorrhage originating from a retroperitoneal paraganglioma. The patient received intravenous fluids and prothrombin complex concentrate for reversal of anticoagulation therapy. A reassessment CT scan performed 12 hours later suggested increased bleeding, and laboratory findings showed a worsening of the anemia, so an angiography was performed which didn't show any evidence of haemorrhage. After a multidisciplinary discussion, it was decided to admit the patient for surveillance and imaging evaluation. Six days later, a second reassessment CT showed signs of haemorrhagic resolution, and the patient was discharged with subsequent follow-up in the outpatient clinic. The authors highlight this case for being a rare complication of an equally rare neuroendocrine tumour.

Control of hemodynamic features during adrenalectomy for pheochromocytoma is recommended to minimize perioperative cardiovascular complications. However, episodes of intraoperative hemodynamic instability have been observed during adrenalectomies with other indications than pheochromocytoma. The objective of this study was to compare the hemodynamic instability score assessed during unilateral adrenalectomy for pheochromocytoma without preoperative medical preparation to hemodynamic instability score in nonsecreting tumor.

This was an observational study with prospective intraoperative hemodynamic data collection (every 20 seconds) and retrospective analysis.

During the study period, 60 consecutive patients (30 pheochromocytomas vs 30 nonsecreting tumors) were included with a median number of data collections during total procedure time of 318 (interquartile range, 257-388). Mean cumulative intraoperative time outside the target blood pressure range expressed as a percentage of total procedure time was 13.3% vs 6.8% for systolic blood pressure >160 mm Hg (P = .01) and 2.4% vs 2.8% for mean arterial pressure <60 mm Hg (P = ns), respectively. The median hemodynamic instability score during total procedure time was 33 (interquartile range, 27-43) and 20 (interquartile range, 11-26) in the pheochromocytoma and nonsecreting tumor group, respectively (P < .01). Hemodynamic instability score were similar in patients with compared with without long-term antihypertensive treatment in each patient group (P = ns). The mean length of hospital stay was 2.0 ± 1.5 days, and 30-day morbidity rate was 6.6% (4/60) with no significant difference observed between both groups.

Although intraoperative hemodynamic instability remains greater in the pheochromocytoma group without preoperative medical preparation, both groups have similar hypotensive episodes. These data highlight the need to better understand the role of preoperative medical preparation in pheochromocytoma patients.

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors derived from chromaffin cells, with 80-85% originating in the adrenal medulla and 15-20% from extra-adrenal chromaffin tissues (paragangliomas). Approximately 30-40% of PPGLs have a hereditary component, making them one of the most genetically predisposed tumor types. Recent advances in genetic research have classified PPGLs into three molecular clusters: pseudohypoxia-related, kinase-signaling, and WNT-signaling pathway variants. Specifically, the detection of SDHB-related tumors indicates an increased risk of metastatic disease, which may impact decisions regarding functional imaging in patients with high suspicion of metastasis and influence targeted treatment strategies. Diagnosis of PPGLs primarily relies on biochemical testing, measuring catecholamines or their metabolites in plasma or urine. However, molecular testing, functional imaging, and targeted therapies have greatly enhanced diagnostic precision and management. Personalized treatment approaches based on genetic profiling are becoming integral to the clinical management of these tumors. In South American countries like Colombia, functional imaging techniques such as positron emission tomography/computed tomography (PET/CT) with tracers like 18F-DOPA, 18F-fluorodeoxyglucose (18F-FDG), and 68Ga-DOTA-conjugated somatostatin receptor-targeting peptides (68Ga-DOTA-SST) are used to guide follow-up and treatment strategies. Radionuclide therapy with lutetium-177 DOTATATE is employed for patients showing uptake in 68Ga-DOTA-SST PET/CT scans, while access to 131-MIBG therapy remains limited due to high costs and availability. Recent clinical trials have shown promise for systemic therapies such as sunitinib and cabozantinib, offering potential new options for patients with slow or moderate progression of PPGLs. These advancements underscore the potential of personalized and targeted therapies to improve outcomes in this challenging patient population.

Pheochromocytomas and paragangliomas (PPGLs) represent the highest degree of heritability of any known tumor types in humans. Previous studies have characterized a dramatic difference between Chinese and European Caucasians with regards to both genetics and clinical features of PPGLs. The proportion of PGLs in Chinese patients was higher than in Caucasians, and the prevalence of metastasis was much lower in Chinese patients. Compared with Caucasians, there were more pathogenic variants (PVs) found in HRAS and FGFR1, but less in NF1 and SDHB. There were less germline PVs found in Chinese patients. Importantly, in Chinese patients, there was a large proportion of PGLs with PVs found in HRAS and FGFR1, mostly with epinephrine-producing capacity. This finding provided solid evidence that genetics (cluster 1 vs. 2), rather than location (PCC vs. PGL), determines the catecholamine-producing phenotype. Besides, the lower prevalence of SDHB partially explained lower occurrence of metastatic lesions in Chinese patients. These findings underscore the importance of considering ethnic differences when evaluating PPGLs and patient outcomes.

Circulating tumor DNAs (ctDNAs) are fragments of malignant tissue DNA that can simply signify the real time genetic change and epigenetic modification of a solid tumor tissue. Pheochromocytomas (PCCs) and Paragangliomas (PGLs) are malinancy of adrenal gland tissue that have the possible diagnosis by ctDNAs. In this study the methylation quanifcation of three target genes RDBP, SDHB, and SDHC in the ctDNA of PCCs/PGLs patients were measured as a diagnostic biomarker.

The biological samples include blood and fresh frozen tissue of twelve PCCs/PGLs patients and blood of 12 non tumoral patients as controls were recruited. Semi quantification methylation status of RDBP, SDHB, and SDHC (two CpG lslands of each gene named 1 and 2) was assesed between PCCs/PGLs patients and controls by Methylation specific-high resolution melting (MS-HRM) technique.

Between six candidate CpG island of RDBP, SDHB, and SDHC, promoter methylation quantification of SDHC1 and RDBP2 was expressively unsimilar in PCCs/PGLs compare to the controls. SDHC1 was hypermethylated in 49.93% of PCCs/PGLs cases vs. 8.33% of control samples, p-value: 0.026, area under curve AUC = 0.757, and RDBP2 in 74.9% of PCCs/PGLs cases vs. 25.0% of control samples, p-value: 0.032, AUC = 0.750.

Our result shows that the ctDNA hypermethylation of SDHC1 and RDBP2 have role in tumorgenesis of adrenal gland and can consider for diagnosis of PCCs/PGLs.

The online version contains supplementary material available at 10.1007/s40200-024-01466-8.

Pathogenic variants (PVs) in EPAS1, which encodes hypoxia-inducible factor-2α (HIF-2α), could be the underlying genetic cause of about 3%-6% of pheochromocytoma and paragangliomas (PPGLs). EPAS1-related PPGLs may occur as isolated tumors or as part of Pacak-Zhuang Syndrome (PZS) with two or more of a triad of PPGL, polycythemia, and somatostatinoma. HIF-2α plays a critical role in the regulation of the cellular hypoxia pathway. When a gain-of-function PV is acquired, HIF-2α evades steady-state hydroxylation by the prolyl hydroxylase type 2 (PHD2), which accelerates von Hippel-Lindau (VHL)-mediated proteasomal degradation. In this situation, HIF-2α is stabilized and can translocate to the nucleus, inducing the expression of several genes involved in tumorigenesis. This leads to the development of PPGL and other manifestations of PZS. EPAS1-related PPGLs usually occur in the second or third decade of life, more frequently in females, and are usually multiple, adrenal and extra-adrenal, and norepinephrine-secreting. In addition, these tumors carry an increased metastatic potential and have been reported with metastatic disease in up to 30% of cases. While polycythemia is fairly common in PZS, somatostatinomas are rare. It has been suggested that the character of the acquired PV in EPAS1, which affects its binding to PHD2, correlates with certain phenotypes in PZS. PVs in EPAS1 that have been found in related sporadic PPGLs have also been associated with hypoxic conditions including cyanotic congenital heart disease, hemoglobinopathies and high altitude. Understanding the hypoxia pathway and its role in the pathogenesis of PPGL may open a new avenue for developing effective therapies for these tumors. Indeed, one of these therapies is Belzutifan, a HIF-2α inhibitor that is being tested in the treatment of metastatic PPGLs.

Pheochromocytomas and sympathetic paragangliomas (PPGL) are rare neuroendocrine tumors originating from chromaffin tissue of the adrenal medulla and extra-adrenal sympathetic paraganglia. Historically, many of these tumors were diagnosed postmortem, earning pheochromocytomas the moniker "great mimic" due to their diverse clinical manifestations that can resemble various other conditions. Over time, the clinical presentation of PPGL has evolved, with a shift from symptomatic or postmortem diagnoses to more frequent incidental discoveries or diagnoses through screening, with postmortem identification now being rare. The development of a clinical scoring system has improved the identification of patients at increased risk for PPGL. Notably, the proportion of PPGL patients with normal blood pressure ranges from 15 % to 40 %, varying based on the clinical context. Despite the tumor's reputation, PPGL is an exceedingly rare cause of resistant hypertension. Management of a pheochromocytoma crisis has advanced, with several classes of drugs available for treatment. However, PPGL during pregnancy remains a significant concern, associated with substantial maternal and fetal mortality rates. Additionally, PPGL can present as rare disorders, including catecholamine-induced cardiomyopathy, Cushing syndrome, and urinary bladder PGL. Given these varied presentations, heightened awareness and prompt recognition of PPGL are crucial for timely diagnosis and treatment, ultimately improving patient outcomes. In this article, we offer an in-depth analysis of the diverse clinical presentations of PPGL, highlighting their complexity and the associated diagnostic and treatment strategies.

Abdominal pheochromocytomas and paragangliomas (PPGLs) are characterized by the overproduction of catecholamines, which are associated with hemodynamic instability (HDI) during surgery. Therefore, perioperative management to prevent intraoperative HDI is imperative for the surgical treatment of PPGLs. Owing to the rarity and heterogeneous nature of these tumors, pre-surgical prediction of HDI is a clinical dilemma. The reported risk factors for HDI include perioperative preparation, genetic background, tumor conditions, body composition, catecholamine levels, and surgical approach. Additionally, several personalized algorithms or models including these factors have been developed. The first part of this review outlines the prediction models that include clinical features such as tumor size and location, body mass index (BMI), blood glucose level, catecholamine levels, and preoperative management with α-adrenoceptor blockade and crystal/colloid fluid. We then summarize recently reported models that consider additional factors such as genetic background, radiomics, robotic-assisted surgical approach, three-dimensional visualization, and machine-learning models. These findings suggest that a comprehensive model including risk factors is the most likely approach for achieving effective perioperative management.

A patient with a long history of bone predominant, metastatic paraganglioma who had multiple episodes of progressive disease despite prior treatments demonstrated a remarkable disease response to zoledronic acid. After 1 year of treatment, there was a complete resolution of lymphadenopathy and disappearance of all somatostatin receptor avid lesions by positron emission tomography-CT and radiopharmaceutical Gallium Ga 68 Dotatate. Stability of disease was further demonstrated by CT over several years. The patient continues on surveillance.

Background: Hemodynamic instability is common during adrenalectomy for pheochromocytoma and paraganglioma (PPGL). Most analyses focus on the risk factors for intraoperative hypertension, but hypotension is a frequent and undesirable phenomenon during PPGL surgery. This study aimed to analyze the risk factors for hypotensive episodes during the removal of PPGL, and whether these episodes are always associated with concomitant intraoperative hypertensive events. Methods: A consecutive series of 121 patients (91.7% receiving preoperative alpha-blockade) treated with transperitoneal endoscopic adrenalectomy at a university hospital were analyzed, and pre- and intraoperative risk factors for intraoperative hypotension with or without intraoperative hypertension were analyzed using univariable and multivariable logistic regression analyses. Results: In total, 58 (56.2%) patients presented with intraoperative hypotension. Of these, 25 (20.7%) patients showed only hypotensive episodes but no hypertensive episodes (group 1), and 43 (35.5%) patients had both intraoperative hypotension and hypertension (group 2). The remaining 53 patients did not present with hypotension at all (group 3). When comparing group 1 (hypotension only) to all other patients with incidental diagnosis, higher age and lower preoperative diastolic arterial blood pressure (ABP) were significant risk factors for intraoperative hypotension; only the latter two were still significant in multivariate analysis. The significant risk factors for hypotension independent of hypertension (group 1 + 2 vs. group 3) were age and incidental diagnosis, pre-existing diabetes mellitus, and intraoperative use of remifentanil. Incidental diagnosis and use of remifentanil reached the level of significance in multivariate analysis. Conclusions: Since older age, incidental diagnosis of PPGL, lower preoperative ABP, and diabetes mellitus are risk factors for intraoperative hypotension, preoperative alpha-blocker treatment should be individualized for those at risk for hypotension. In addition, remifentanil should be used cautiously in the risk group.

Heterogenous clinical manifestations, overlapping phenotypes, and complex genetic backgrounds are common in patients with endocrine tumors. There are no comprehensive recommendations for genetic testing and counseling of these patients compared to other hereditary cancer syndromes. The application of multigene panel testing is common in clinical genetic laboratories, but their performance for patients with endocrine tumors has not been assessed.

As a national reference center, we prospectively tested the diagnostic utility and cost-efficiency of a multigene panel covering 113 genes representing genetic susceptibility for solid tumors; 1279 patients (including 96 cases with endocrine tumors) were evaluated between October 2021 and December 2022 who were suspected to have hereditary tumor syndromes.

The analytical performance of the hereditary cancer panel was suitable for diagnostic testing. Clinical diagnosis was confirmed in 24% (23/96); incidental findings in genes not associated with the patient's phenotype were identified in 5% (5/96). A further 7% of pathogenic/likely pathogenic variants were detected in genes with potential genetic susceptibility roles but currently no clear clinical consequence. Cost-benefit analysis showed that the application of a more comprehensive gene panel in a diagnostic laboratory yielded a shorter turnaround time and provided additional genetic results with the same cost and workload.

Using comprehensive multigene panel results in faster turnaround time and cost-efficiently identifies genetic alterations in hereditary endocrine tumor syndromes. Incidentally identified variants in patients with poor prognoses may serve as a potential therapeutic target in tumors where therapeutic possibilities are limited.

A female in late adolescence with severe chest pain, dyspnoea, diaphoresis and dizziness presented to the emergency department where she was found to have exceptionally high blood pressure (250/150 mm Hg) and a diastolic murmur. Initial examinations showed left ventricular hypertrophy, and urgent CT angiography confirmed a Stanford type A aortic dissection. Following successful surgical repair, further evaluations were prompted by her persistent, drug-resistant hypertension, revealing elevated normetanephrine and 3-methoxytyramine. Subsequent imaging identified bilateral pelvic paragangliomas, which were surgically removed, significantly reducing her hypertension. Her postoperative period showed biochemical remission, and genetic testing was positive for germline SDHB mutation. Monitoring and follow-up imaging are ongoing. This case highlights the rare association of pelvic paragangliomas with acute aortic dissection in young adults, emphasising the importance of possible endocrine hypertension in young people with hypertensive emergencies.

Pheochromocytoma and paraganglioma (PPGL) represent a group of rare neuroendocrine tumors known for their potential to metastasize. This study provides a comprehensive retrospective evaluation of 15 patients diagnosed with metastatic or recurrent PPGL at our institution over a two-decade span (2000-2020). Our primary objectives were to delineate the long-term clinical outcomes and pinpoint key prognostic determinants. Median duration from initial PPGL diagnosis to the onset of metastasis or recurrence stood at 5.8 years. Predominant sites for metastasis included the bone, lung, lymph nodes, and peritoneum. A salient finding was that surgical interventions targeting metastatic lesions significantly improved prognosis. Further analysis revealed that a Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) exceeding 7 closely associated with unfavorable outcomes. These insights not only underscore the clinical variability of PPGL's progression but also highlight the pivotal role of surgical management for metastatic or recurrent cases. The value of the PASS score as an informative prognostic tool was evident, suggesting its utility in shaping future therapeutic approaches. Given the intricacies of PPGL, collaborative studies involving larger patient cohorts will be crucial to optimize management strategies and prognostication.

Pheochromocytoma and paragangliomas (PPGLs) crises can be triggered by various factors, including norepinephrine reuptake inhibitors used to treat attention deficit hyperactivity disorder (ADHD), which worsen symptoms in patients with PPGLs. Therefore, attention should be paid to the potential for serious adverse reactions in patients with PPGLs taking ADHD medications. A 21-year-old man presented to the emergency department with acute onset of severe respiratory and circulatory failure after initiating atomoxetine treatment. During preparation for hospital admission, his respiratory and circulatory status deteriorated, requiring emergency intubation and mechanical ventilation and transfer to our institution for further evaluation and treatment. Profuse sweating and sinus tachycardia were observed, and echocardiography revealed a significantly reduced ejection fraction. Contrast-enhanced computed tomography of the trunk revealed a 50 mm tumour anterior to the inferior vena cava and a 20 mm enhancing tumour in the left adrenal gland. Treatment with the α-blocker, phentolamine, was initiated on the grounds of cardiogenic shock induced by an endocrine disorder such as PPGLs. However, the patient developed bradycardia and hypotension, progressing to pulseless electrical activity (PEA), for which cardiopulmonary resuscitation (CPR) was initiated. After the administration of adrenaline (1 mg), a return of spontaneous circulation was achieved. Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) was initiated to prevent further cardiac arrest. An intra-aortic balloon pump (IABP) was inserted to reduce the cardiac workload. Circulatory dynamics gradually stabilised, and treatment with VA-ECMO was discontinued on day 4 after admission as the ejection fraction improved to approximately 50%. On day 6, the patient was successfully extubated, respiratory support was discontinued, and he was discharged on day 25, confirming the diagnosis of PPGLs with no evidence of higher brain dysfunction. Outpatient management included dose adjustment of the α1-blocker, and he was readmitted for surgical removal of the tumour. The postoperative course was uneventful, with a notable improvement in ADHD symptoms. This case report highlights the importance of a multidisciplinary approach for the diagnosis and management of patients with symptoms suggesting psychiatric or endocrine disorders.

Paragangliomas are rare neuroendocrine tumors, often associated with catecholamine secretion. These tumors can arise in various locations, with the majority found in the abdomen and pelvis, while a smaller percentage occurs in the thorax and head and neck regions. Diaphragmatic paragangliomas are exceedingly rare, with only two documented cases in the literature. This report details a case of a primary diaphragmatic paraganglioma in a 59-year-old patient presenting with unexplained weight loss, tremors, and diaphoresis. Imaging studies revealed a mass in the right lobe of the liver, later identified as a diaphragmatic paraganglioma during surgery. The case underscores the importance of preoperative catecholamine assessment and careful surgical planning due to the risks associated with tumor manipulation. Complete surgical resection, although challenging, remains the definitive treatment, especially in hypervascular tumors located near major vascular structures.

Paragangliomas (PGLs) are neuroendocrine tumors originating from the neural crest. They usually arise from the adrenal medulla and sympathetic or parasympathetic ganglions. Approximately 10% of PGLs are located in the extra-adrenal gland. Renal PGL is a rare condition. In this case report, we present the 18F-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) and 68Ga-DOTATATE PET/CT findings of polycystic kidney-derived PGL.

Pheochromocytomas are rare neuroendocrine tumors characterised by the secretion of catecholamines and their metabolites. While some patients may be asymptomatic, they can also present with various symptoms including hypertensive crisis, headaches, palpitations, diaphoresis or other signs of catecholamine toxicity. Adrenal haemorrhage, though rare, is a potentially fatal complication that is often diagnosed during autopsy. In all patients with suspected pheochromocytoma, regardless of whether haemorrhagic conversion has occurred, prompt diagnosis is imperative. Early identification allows for the timely initiation of treatment, preventing potentially life-threatening complications. This case report details the haemorrhagic conversion of an undiagnosed pheochromocytoma in a female patient in her 30s.

This white paper provides practical guidance for clinicians encountering bilateral adrenal masses.

A case-based approach to the evaluation and management of bilateral adrenal masses. Specific clinical scenarios presented here include cases of bilateral adrenal adenomas, hemorrhage, pheochromocytomas, metastatic disease, myelolipomas, as well as primary bilateral macronodular adrenal hyperplasia.

Bilateral adrenal masses represent approximately 10% to 20% of incidentally discovered adrenal masses. The general approach to the evaluation and management of bilateral adrenal masses follows the same protocol as the evaluation of unilateral adrenal masses, determined based on the patient's clinical history and examination as well as the imaging characteristics of each lesion, whether the lesions could represent a malignancy, demonstrate hormone excess, or possibly represent a familial syndrome. Furthermore, there are features unique to bilateral adrenal masses that must be considered, including the differential diagnosis, the evaluation, and the management depending on the etiology. Therefore, considerations for the optimal imaging modality, treatment (medical vs surgical therapy), and surveillance are included. These recommendations were developed through careful examination of existing published studies as well as expert clinical opinion consensus.

The evaluation and management of bilateral adrenal masses require a comprehensive systematic approach which includes the assessment and interpretation of the patient's clinical history, physical examination, dynamic hormone evaluation, and imaging modalities to determine the key radiographic features of each adrenal nodule. In addition, familial syndromes should be considered. Any final treatment options and approaches should always be considered individually.

Catecholamines (epinephrine; norepinephrine; and dopamine) and their O-methylated metabolites (metanephrine; normetanephrine; and 3-methoxytyramine) are biomarkers for pheochromocytoma and paraganglioma. Liquid chromatography coupled with tandem mass spectrometry (LC-MS/MS) was recommended by Endocrine Society for detecting these compounds. The influence of blood collection tubes on the analysis of the six analytes by LC-MS/MS was not thoroughly investigated, which we want to clarify in our study.

Blood samples of healthy individuals were collected into serum, lithium heparin, and K2EDTA plasma tubes separately. Samples were subjected to solid phase extraction and then analyzed by LC-MS/MS. The retention behavior and assay performance of the six analytes were assessed for samples from different collection containers. The impacts of potassium and sodium as the counter ions of EDTA on the retention time and matrix effect were also studied.

Compared with O-methylated metabolites, the results for catecholamines were more affected by the collection tubes, especially for norepinephrine, which displayed severely suppressed signal and very low extraction efficiency in K2EDTA plasma. Changing the counter ion of EDTA from potassium to sodium dramatically changed the retention behavior and matrix effect of norepinephrine.

It is necessary to evaluate blood collection tubes for catecholamines and their O-methylated metabolites analyzed by LC-MS/MS. In addition, attention should also be paid when the anticoagulant counter ion was changed.

Pheochromocytoma is a rare neuroendocrine tumor. Despite the low incidence, these tumors are of indisputable importance. This study aimed to analyze the management of pheochromocytoma in a referral center, with an emphasis on the minimally invasive adrenalectomy, which is the preferred therapeutic approach.

A retrospective analysis was performed on a cohort of patients diagnosed with pheochromocytoma who underwent adrenalectomy between January 2013 and December 2022. Clinical data including demographics, timelines, symptomatology, comorbidities, biochemical markers, genetic testing, surgical details, and follow-up outcomes, were collected and analyzed.

The cohort included 44 patients, predominantly women (52.27%), with a median age of 53.39 years (range 13-83). Most of patients exhibited paroxysmal symptoms suggesting catecholamine excess. Documented hypertension was the most frequent (86.36%), along with glucose anomalies (40.01%) and anxiety disorder (31.82%). Genetic testing was performed in 36 (81.81%) patients and 14 (38.88%) revealed a positive result, predominantly RET pathogenic variant. Laparoscopic surgery was performed in 34 (79.07%) patients, showing significantly shorter operative time (2.5 h vs. 4.25 h, t-test p < 0,001) and fewer complications (23.53% vs 77.78%, p = 0.008). Postoperative complications occurred in 36.36% of the patients, mostly mild (grade I, 56.25%), with no mortality. SDHB pathogenic variant correlated with both recurrent and metastatic disease (p = 0.006). One-year follow-up reported 9.09% recurrence and 6.82% metastasis.

Adrenalectomy demonstrated a high safety and effectiveness. This study exhibited a higher rate of genetic testing referral than other studies. Despite past advances, there is still a need for further studies to establish protocols and evaluate new techniques.

Most cases of high blood pressure have no identifiable cause, termed essential hypertension; however, in approximately 15% of cases, hypertension occurs due to secondary causes. Primary aldosteronism (PA) and pheochromocytoma and paraganglioma (PPGL) are representative endocrine hypertensive diseases. The differentiation of endocrine hypertension provides an opportunity to cure and prevent target organ damage. PA is the most common cause of secondary hypertension, which significantly increases the risk of cardiovascular disease compared to essential hypertension; thus, patients with clinical manifestations suggestive of secondary hypertension should be screened for PA. PPGL are rare but can be fatal when misdiagnosed. PPGL are the most common hereditary endocrine tumors; therefore, genetic testing using next-generation sequencing panels is recommended. Herein, we aimed to summarize the characteristic clinical symptoms of PA and PPGL and when and how diagnostic tests and treatment strategies should be performed.

Approximately 40% of pheochromocytoma and paraganglioma (PPGL) cases are familial, typically presenting earlier with more complex symptoms. This paper synthesizes literature and guidelines to inform on clinical characteristics and perioperative care for PPGL. Pheochromocytoma in von Hippel-Lindau (VHL) disease exhibits heightened secretion activity without significant perioperative hemodynamic changes. Tumors in multiple endocrine neoplasia type 2 (MEN2) have a stronger endocrine function, which may induce hemodynamic fluctuations during surgery. Therefore, pheochromocytoma screening is essential at all stages of MEN2. Neurofibromatosis type 1 (NF1) often presents multisystem lesions and can result in difficult airway. Pheochromocytoma should be evaluated when NF1 patients present hypertension. Pheochromocytoma and paraganglioma type 5 may present multiple lesions of pheochromocytoma or paraganglioma. In summary, hereditary PPGLs may present with severe lesions in other systems, beyond tumor function. A multi-disciplinary team (MDT) approach is often invaluable in perioperative management.

Pheochromocytomas are rare neuroendocrine tumors that derive from the chromaffin cells of the adrenal medulla and secrete catecholamines. The measurement of plasma or fractionated urine metanephrines is the hormonal determination of choice for the biochemical diagnosis. Once the biochemical diagnosis is confirmed, the next step is the localization study. It is recommended to request a genetic study in all patients with pheochromocytomas since 40% of cases are hereditary. Once the diagnostic study is completed, preoperative treatment with alpha blockers should be instituted at least 7-14 days before adrenalectomy. However, in low-risk patients, the omission of presurgical treatment could be considered if the surgery is performed in centers with experience and a strict monitoring of the patient is carried out during the perioperative period. This document offers a practical guide on the diagnosis and perioperative approach in patients with pheochromocytomas.