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INTRODUCTION: It is estimated that 30-40% of patients with apparently sporadic pheochromocytomas (PHEOs) have an inherited predisposition syndrome. The aim of our study was to develop a predictive model of hereditary PHEO based on the clinical, hormonal, and radiological features present at the diagnosis of patients with PHEOs. METHODS: A retrospective multicenter cohort study of patients with PHEOs with available genetic study from 18 tertiary hospitals. Clinical, biochemical, and radiological features were used to build a multivariate logistic regression model. The estimation of all possible equations was used to select the model with the best diagnostic accuracy (lower Akaike index). RESULTS: A total of 245 patients were included: 169 (69.0%) patients with sporadic PHEOs and 76 (31%) with hereditary PHEOs. The parsimonious predictive model with the highest diagnostic accuracy for the prediction of hereditary PHEO combined the variables age, non-cardiovascular disease, urinary norepinephrine levels, and tumor size. The area under the ROC curve of this model was 0.800 (0.705-0.887). Based on the predictive model, the probability of hereditary PHEO in patients older than 60 years with cardiovascular disease, high levels of urinary norepinephrine and unilateral PHEOs >60 mm was <2%. And if the age was above 80 years, lower than 1%. The probability of sporadic PHEO linearly increased with age (MH Test for linear Trend: χ2 (1) = 30.05; p < 0.001). CONCLUSION: In certain populations such as old patients with cardiovascular disease, with high levels of urinary norepinephrine and large tumors in which the probability of hereditary PHEO is very low, genetic testing could be avoided in the absence of specific suspicion.
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Neoplasias das Glândulas Suprarrenais , Feocromocitoma , Humanos , Idoso de 80 Anos ou mais , Feocromocitoma/diagnóstico , Feocromocitoma/genética , Feocromocitoma/patologia , Estudos de Coortes , Testes Genéticos , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/genética , Neoplasias das Glândulas Suprarrenais/patologia , NorepinefrinaRESUMO
PURPOSE: To analyze surgical outcomes and predictive factors for long-term overall and disease-specific survival in patients undergoing surgical resection of adrenal metastasis. METHODS: A multicenter retrospective study included patients who underwent adrenalectomy for adrenal metastasis in two Spanish hospitals between 2005 and 2021. Clinical variables associated with surgical complications and survival during follow-up were analyzed. RESULTS: Thirty-three patients were included. Adrenalectomy was performed laparoscopically in 27 patients and by an open approach in 6. The most common primary tumor site was the lung (n=15), followed by the kidney (n=7). Most patients had metachronous lesions (n=28). Six patients (18.2%) had intra- and/or postoperative complications; synchronous metastasis was a risk factor (odds ratio 12.5 [1.45-107.6]) for their development. Progression-free survival and disease-specific survival were 7.5months (range 1-64) and 22.5months (6-120), respectively. Survival rates at 1, 2, 3 and 5years were 94%, 65%, 48% and 29%, respectively. Survival was significantly lower in patients with lung cancer than with other cancers (hazard ratio 4.23 [1.42-12.59]). CONCLUSIONS: Adrenalectomy for solitary adrenal metastases was associated with intra- or postoperative complications in 18% of cases. Synchronous metastasis was a risk factor for complications.
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Neoplasias das Glândulas Suprarrenais , Neoplasias Pulmonares , Humanos , Prognóstico , Estudos Retrospectivos , Neoplasias Pulmonares/patologia , Neoplasias das Glândulas Suprarrenais/patologia , Adrenalectomia , Complicações Pós-Operatórias , Taxa de Sobrevida , Resultado do Tratamento , Intervalo Livre de DoençaRESUMO
Background: Malignant ureteral obstruction (MUO) is a common condition that complicates the course of advanced malignancies. The aims of this study are to analyze the causes, management, and survival of patients with obstructive nephropathy due to malignant ureteric obstruction and to determine prognostic factors. Furthermore, we studied the complications and outcomes in patients who underwent urinary diversion. Materials and methods: A retrospective study was conducted on patients with computed tomography-confirmed MUO between January 2016 and November 2020. Demographic, clinical, radiological, laboratory, and management data were collected. Survival curves were estimated using the Kaplan-Meier method, and univariate and multivariate Cox proportional hazards models were used to test the association between parameters and survival. Results: A total of 188 patients were included. The mean age was 69.01 years (SD, 14.95 years), and the majority (54.8%) were male. The most common mechanism leading to MUO was compression by a pelvic mass (36.9%), and the 3 most frequent tumors causing MUO were prostate (17.6%), bladder (16.5%), and rectal cancer (11.7%).Forty-seven patients (25%) underwent urinary diversion: 23 (48.9%) underwent double-J stenting and 21 (44.7%) underwent percutaneous nephrostomy. The most common reason for urinary diversion was acute kidney injury (53.3%). Recovery of renal function was observed in 55.8% of the patients after urinary diversion. The most frequently identified complications after urinary diversion were urinary tract infection (24.4%), hematuria (17.0%), and urinary sepsis (14.9%). The median survival after hydronephrosis diagnosis was 6.43 months (interquartile range, 1.91-14.81 months). In patients who underwent urinary decompression, the median survival after urinary diversion was 8.67 months (interquartile range, 2.99-17.28 months). In the multivariate analysis, a lower grade of hydronephrosis and cancer cachexia negatively impacted survival. Conclusions: Cancer patients with MUO have a poor prognosis; therefore, the risk-benefit ratio of urinary diversion should be carefully considered. Cachexia and hydronephrosis grade can be useful in selecting suitable candidates for urinary diversion.
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PURPOSE: To identify presurgical and surgical risk factors for intraoperative hypertensive crisis in patients with pheochromocytomas and sympathetic paragangliomas (PGLs) (PPGLs). METHODS: Retrospective multicenter cohort study of patients with PPGLs from 18 tertiary hospitals. Intraoperative hypertensive crisis was defined as systolic blood pressure (SBP) greater than 200âmmHg lasting more than 1âmin and postoperative hypertensive crisis as SBP greater than 180âmmHg or diastolic blood pressure (DBP) greater than 110âmmHg. RESULTS: A total of 296 surgeries were included. Alpha presurgical blockade was employed in 93.2% of the cases and beta-adrenergic in 53.4%. Hypertensive crisis occurred in 20.3% ( n â=â60) of the surgeries: intraoperative crisis in 56 and postoperative crisis in 6 cases (2 cases had both types of crises). We identified as risk factors of intraoperative hypertensive crisis, absence of presurgical glucocorticoid therapy (odds ratio [OR] 3.48; 95% confidence interval [CI] 1.19-10.12) higher presurgical SBP (OR 1.22 per each 10âmmHg, 95% CI 1.03-1.45), a larger tumor size (OR 1.09 per each 10âmm, 95% CI 1.00-1.19) and absence of oral sodium repletion (OR 2.59, 95% CI 1.25-5.35). Patients with hypertensive crisis had a higher rate of intraoperative bleeding ( P â<â0.001), of intraoperative hemodynamic instability ( P â<â0.001) and of intraoperative hypotensive episodes ( P â<â0.001) than those without hypertensive crisis. CONCLUSION: Intraoperative hypertensive crisis occurs in up to 20% of the PPGL resections. Patients not pretreated with glucocorticoid therapy before surgery, with larger tumors and higher presurgical SBP and who do not receive oral sodium repletion have a higher risk for developing hypertensive crisis during and after PPGL surgery.
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Neoplasias das Glândulas Suprarrenais , Hipertensão , Crise Hipertensiva , Paraganglioma , Feocromocitoma , Humanos , Feocromocitoma/complicações , Feocromocitoma/cirurgia , Feocromocitoma/patologia , Hipertensão/epidemiologia , Estudos de Coortes , Glucocorticoides , Pressão Sanguínea/fisiologia , Paraganglioma/complicações , Paraganglioma/cirurgia , Fatores de Risco , Neoplasias das Glândulas Suprarrenais/complicações , Neoplasias das Glândulas Suprarrenais/cirurgia , Neoplasias das Glândulas Suprarrenais/patologia , Sódio , Estudos RetrospectivosRESUMO
OBJECTIVE: To evaluate the impact of obesity and overweight on surgical outcomes in a large cohort of patients who underwent adrenalectomy due to benign or malignant primary adrenal disease. METHODS: A retrospective single-center study of patients without history of active extraadrenal malignancy, with adrenal tumors operated on consecutively in our center between January 2010 and December 2022. Obesity was defined as a body mass index (BMI)≥30kg/m2 and overweight as BMI between 25.0 and 30.0kg/m2. RESULTS: Of 146 patients with adrenal tumors who underwent adrenalectomy, 9.6% (n=14) were obese, 54.8% (n=80) overweight and 35.6% (n=52) normal weight. Obese patients had higher diastolic blood pressure (87.6±12.22 vs. 79.3±10.23mmHg, P=0.010) and a higher prevalence of dyslipidemia (57.1% vs. 25.8%, P=0.014) and bilateral tumors (14.3% vs. 3.1%, P=0.044) than non-obese patients. The rates of intraoperative and of postsurgical complications were similar between obese/overweight patients and patients with normal weight. However, a significantly higher rate of postsurgical complications (27.3% vs. 5.7%, P=0.009) and a longer hospital stay (5.4±1.39 vs. 3.5±1.78 days, P=0.007) were observed in patients with obesity than in non-obese patients. In the multivariant analysis, obesity, age, ASA>2 and tumor size were independent risk factors for postoperative complications, with obesity being the most important factor (OR 23.34 [2.23-244.24]). CONCLUSION: Obesity and overweight are common conditions in patients who undergo adrenalectomy. Adrenalectomy is considered a safe procedure in patients with overweight, but it is associated with a higher risk of postsurgical complications and longer hospital stay in obese patients.
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Neoplasias das Glândulas Suprarrenais , Adrenalectomia , Humanos , Adrenalectomia/efeitos adversos , Adrenalectomia/métodos , Estudos de Coortes , Sobrepeso/complicações , Sobrepeso/epidemiologia , Estudos Retrospectivos , Resultado do Tratamento , Obesidade/complicações , Obesidade/epidemiologia , Obesidade/cirurgia , Neoplasias das Glândulas Suprarrenais/complicações , Neoplasias das Glândulas Suprarrenais/cirurgia , Neoplasias das Glândulas Suprarrenais/epidemiologiaRESUMO
Seminal vesicles can be affected by tumours originating in other locations. However, primary tumours of the seminal vesicle are extremely rare, with less than 100 cases reported in literature. Seminal vesicle adenocarcinoma is the most common type, but there are also other malign lesions. Diagnosis is challenging due to the lack of early symptoms and well-defined criteria. These tumours are usually asymptomatic and discovered incidentally during imaging tests or pelvic surgery. Definitive diagnosis requires anatomopathological analysis. Case report of 58-years-old man with schwannoma of the seminal vesicle. We describe the main characteristics of these tumours as well as their therapeutic approach.
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PURPOSE: to perform an external validation of our predictive model to rule out pheochromocytoma (PHEO) based on unenhanced CT in a cohort of patients with PHEOs and adenomas who underwent adrenalectomy. METHODS: The predictive model was previously developed in a retrospective cohort of 1131 patients presenting with adrenal lesions. In the present study, we performed an external validation of the model in another cohort of 214 patients with available histopathological results. RESULTS: For the external validation, 115 patients with PHEOs and 99 with adenomas were included. Our previously described predictive model combining the variables of high lipid content and tumor size in unenhanced CT (AUC-ROC: 0.961) had a lower diagnostic accuracy in our current study population for the prediction of PHEO (AUC: 0.750). However, when we excluded atypical adenomas (with Hounsfield units (HU) > 10, n = 39), the diagnostic accuracy increased to 87.4%. In addition, in the whole cohort (including atypical adenomas), when MRI information was included in the model, the diagnostic accuracy increased to up to 85% when the variables tumor size, high lipid content in an unenhanced CT scan, and hyperintensity in the T2 sequence in MRI were included. The probability of PHEO was <0.3% for adrenal lesions <20 mm with >10 HU and without hyperintensity in T2. CONCLUSION: Our study confirms that our predictive model combining tumor size and lipid content has high reliability for the prediction of PHEO when atypical adrenal lesions are excluded. However, for atypical adrenal lesions with >10 HU in an unenhanced CT scan, MRI information is necessary for a proper exclusion of the PHEO diagnosis.
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Purpose: To evaluate the rate of recurrence among patients with pheochromocytomas and sympathetic paragangliomas (PGLs; together PPGLs) and to identify predictors of recurrence (local recurrence and/or metastatic disease). Methods: This retrospective multicenter study included information of 303 patients with PPGLs in follow-up in 19 Spanish tertiary hospitals. Recurrent disease was defined by the development of local recurrence and/or metastatic disease after initial complete surgical resection. Results: A total of 303 patients with PPGLs that underwent 311 resections were included (288 pheochromocytomas and 15 sympathetic PGLs). After a median follow-up of 4.8 years (range 1-19), 24 patients (7.9%) had recurrent disease (3 local recurrence, 17 metastatic disease and 4 local recurrence followed by metastatic disease). The median time from the diagnosis of the PPGL to the recurrence was of 11.2 months (range 0.5-174) and recurrent disease cases distributed uniformly during the follow-up period. The presence of a pathogenic variant in SDHB gene (hazard ratio [HR] 13.3, 95% CI 4.20-41.92), higher urinary normetanephrine levels (HR 1.02 per each increase in standard deviation, 95% CI 1.01-1.03) and a larger tumor size (HR 1.01 per each increase in mm, 95% CI 1.00-1.02) were independently associated with disease recurrence. Conclusion: The recurrence of PPGLs occurred more frequently in patients with SDHB mutations, with larger tumors and with higher urinary normetanephrine levels. Since PPGL recurrence may occur at any time after the initial PPGL diagnosis is performed, we recommend performing a strict follow-up in all patients with PPGLs, especially in those patients with a higher risk of recurrent disease.
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Neoplasias das Glândulas Suprarrenais , Neoplasias Encefálicas , Segunda Neoplasia Primária , Paraganglioma , Feocromocitoma , Humanos , Feocromocitoma/patologia , Normetanefrina , Recidiva Local de Neoplasia , Paraganglioma/patologia , Neoplasias das Glândulas Suprarrenais/diagnósticoRESUMO
PURPOSE: To compare the evolution of the cardiometabolic parameters in patients with nonfunctioning adrenal incidentalomas (NFAI) and autonomous cortisol secretion (ACS) who underwent adrenalectomy and those who were conservatively managed. METHODS: A retrospective study of all patients with NFAI and ACS submitted to surgery or in follow-up in our center between January 2011-October 2020. NFAI was defined as an adrenal incidentaloma with cortisol post-dexamethasone suppression test (DST) ≤ 50 nmol/L and ACS as values >50 nmol/L without specific clinical signs of overt Cushing´s syndrome. RESULTS: A total of 486 patients with NFAI (16 in the surgical group and 470 in the control group) and 259 with ACS (42 in the surgical group and 217 in the control group) were included. At baseline, patients with ACS were older than those with NFAI (P = 0.010). After adjusting by age, patients with ACS had a higher prevalence of hypertension (adjusted OR = 1.9 [1.36-2.60]) and higher levels of fasting plasma glucose and HbA1c (adjusted ß = 6.9 [2.05-11.83] and adjusted ß = 0.4 [0.12-0.63]) than NFAI. During follow-up, ACS patients who underwent adrenalectomy had a greater decrease in glucose levels (-16.6 ± 45.07 vs. -1.0 ± 26.92 mg/dL, P = 0.035) and in triglycerides (-20.21 ± 55.97 vs. 1.3 ± 59.23 mg/dL, P = 0.029) than ACS patients conservatively managed. NFAI patients who underwent surgery experienced an improvement in systolic blood pressure compared to NFAI of the conservative group (-11.1 ± 15.94 vs 1.0 ± 17.54 mmHg, P = 0.009). CONCLUSION: The benefits of adrenalectomy in the cardiometabolic profile in adrenal incidentalomas are not limited only to the group of patients with ACS, an improvement in blood pressure control is also observed in NFAI patients after surgery.
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Neoplasias das Glândulas Suprarrenais , Hipertensão , Adrenalectomia , Glicemia , Pressão Sanguínea , Humanos , Hidrocortisona , Lipídeos , Estudos RetrospectivosRESUMO
Background: The coronavirus disease 2019 (COVID-19) pandemic has affected care for diseases like cancer. The aim was to evaluate the impact of COVID-19 on waiting times for diagnosis and treatment of prostate cancer (PC), as well as the possible effect on the treatment results in PC patients undergoing radical prostatectomy. Methods: We compared the results of 497 patients who underwent biopsy prior to the COVID-19 pandemic (1 January-31 December 2019) with those of 290 patients biopsied during the COVID-19 pandemic (1 January-31 December 2020). Demographic data, tumour characteristics, type of treatment and diagnosis times were comparable. Prostate specific antigen (PSA) levels were recorded at consultation prior to biopsy and after treatment. Mann-Whitney and chi-square tests were used to compare continuous variables and percentages, respectively. Results: In 2020, there were fewer urology consultations (35,160 vs. 40,225 in 2019). The median PSA in 2020 was significantly higher (14.3 vs. 9.9 ng/dL in 2019). In 2019, 53.1% (N=264) of the biopsies were positive for cancer vs. 47.2% (N=137) in 2020 (P=0.104). In 2020, more patients presented with metastatic disease (7.3% vs. 1.9%, P=0.009). Also, in 2020 there was a longer waiting time for prostate biopsy (42.1 vs. 35.3 days in 2019, P=0.019). A total of 132 patients underwent laparoscopic radical prostatectomy (LARP). The median time until surgery was similar in both years (71.9 vs. 58.29 days). During 2020, a higher percentage of patients had ISUP grade 4 in the surgical specimen (34.3% vs. 17.5%, P=0.07). Furthermore, a higher percentage of aggressive (pT3) tumours were diagnosed (37.2% vs. 27.2%, P=0.08), and the percentage of patients with involvement of surgical margins was also higher (48.6% vs. 29.3%, P=0.027). There were no differences between the groups in terms of biochemical recurrence or persistent PSA at one year (P=0.711). Conclusions: Delayed biopsy during the COVID-19 period did not appear to adversely impact biopsy results. Patients biopsied in 2020 had higher PSA, possibly due to proper triaging. A higher rate of adverse pathology outcomes was observed in patients undergoing radical prostatectomy during the pandemic, probably due to understaging of the biopsy. This study serves to raise awareness of the risk of deterioration of care of PC patients due to possible underdiagnosis.
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The objective of our study was to determine the prevalence of glycemic disorders (diabetes mellitus and prediabetes) in patients with pheochromocytomas and sympathetic paragangliomas (PPGLs) and identify risk factors for their development and the likelihood of their resolution after surgery. A multicentric retrospective study of patients with PPGLs submitted to surgery between 2000 and 2021 in 17 Spanish hospitals was performed. Diabetes-specific data were collected at diagnosis, in the immediate- and long-term postsurgical follow-up. A total of 229 patients with PPGLs were included (218 with pheochromocytomas and 11 with sympathetic paragangliomas). Before surgery, glycemic disorders were diagnosed in 35.4% of the patients (n = 81): 54 with diabetes and 27 with prediabetes. The variables independently associated with a higher risk of glycemic disorders were sporadic PPGL (odds ratio (OR) = 3.26 (1.14-9.36)) and hypertension (OR = 3.14 (1.09-9.01)). A significant decrease in fasting plasma glucose and HbA1c levels was observed after surgery, in the short-term and long-term follow-up (P < 0.001). After a median follow-up of 48.5 months (range 3.3-168.9), after surgery, 52% of diabetic and 68% of prediabetic patients experienced a complete resolution. Lower body mass index (BMI) (P = 0.001), lower glucose levels (P = 0.047) and shorter duration of diabetes prior to surgery (P = 0.021) were associated with a higher probability of diabetes resolution. In conclusion, glycemic disorders in patients with PPGLs are present in more than a third of them at diagnosis. Sporadic PPGLs and hypertension are risk factors for their development. More than 50% of cases experience a complete resolution of the glycemic disorder after resection of the PPGLs.
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Neoplasias das Glândulas Suprarrenais , Hipertensão , Paraganglioma , Feocromocitoma , Estado Pré-Diabético , Neoplasias das Glândulas Suprarrenais/epidemiologia , Neoplasias das Glândulas Suprarrenais/cirurgia , Glicemia , Hemoglobinas Glicadas , Humanos , Hipertensão/epidemiologia , Paraganglioma/diagnóstico , Paraganglioma/epidemiologia , Paraganglioma/cirurgia , Feocromocitoma/epidemiologia , Feocromocitoma/cirurgia , Estudos RetrospectivosRESUMO
The purpose of our study was to develop a predictive model to rule out pheochromocytoma among adrenal tumours, based on unenhanced computed tomography (CT) and/or magnetic resonance imaging (MRI) features. We performed a retrospective multicentre study of 1131 patients presenting with adrenal lesions including 163 subjects with histological confirmation of pheochromocytoma (PHEO), and 968 patients showing no clinical suspicion of pheochromocytoma in whom plasma and/or urinary metanephrines and/or catecholamines were within reference ranges (non-PHEO). We found that tumour size was significantly larger in PHEO than non-PHEO lesions (44.3 ± 33.2 versus 20.6 ± 9.2 mm respectively; P < 0.001). Mean unenhanced CT attenuation was higher in PHEO (52.4 ± 43.1 versus 4.7 ± 17.9HU; P < 0.001). High lipid content in CT was more frequent among non-PHEO (83.6% versus 3.8% respectively; P < 0.001); and this feature alone had 83.6% sensitivity and 96.2% specificity to rule out pheochromocytoma with an area under the receiver operating characteristics curve (AUC-ROC) of 0.899. The combination of high lipid content and tumour size improved the diagnostic accuracy (AUC-ROC 0.961, sensitivity 88.1% and specificity 92.3%). The probability of having a pheochromocytoma was 0.1% for adrenal lesions smaller than 20 mm showing high lipid content in CT. Ninety percent of non-PHEO presented loss of signal in the "out of phase" MRI sequence compared to 39.0% of PHEO (P < 0.001), but the specificity of this feature for the diagnosis of non-PHEO lesions low. In conclusion, our study suggests that sparing biochemical screening for pheochromocytoma might be reasonable in patients with adrenal lesions smaller than 20 mm showing high lipid content in the CT scan, if there are no typical signs and symptoms of pheochromocytoma.
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Neoplasias das Glândulas Suprarrenais , Lipídeos/sangue , Modelos Biológicos , Feocromocitoma , Neoplasias das Glândulas Suprarrenais/sangue , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Feocromocitoma/sangue , Feocromocitoma/diagnóstico por imagem , Estudos Retrospectivos , Tomografia Computadorizada por Raios XRESUMO
We aimed to identify presurgical and surgical risk factors for intraoperative complications in patients with pheochromocytomas. A retrospective study of patients with pheochromocytomas who underwent surgery in ten Spanish hospitals between 2011 and 2021 was performed. One hundred and sixty-two surgeries performed in 159 patients were included. The mean age was 51.6 ± 16.4 years old and 52.8% were women. Median tumour size was 40 mm (range 10-110). Laparoscopic adrenalectomy was performed in 148 patients and open adrenalectomy in 14 patients. Presurgical alpha- and beta-blockade was performed in 95.1% and 51.9% of the surgeries, respectively. 33.3% of the patients (n = 54) had one or more intraoperative complications. The most common complication was the hypertensive crisis in 21.0%, followed by prolonged hypotension in 20.0%, and hemodynamic instability in 10.5%. Patients pre-treated with doxazosin required intraoperative hypotensive treatment more commonly than patients pre-treated with other antihypertensive drugs (51.1% vs 26.5%, P = 0.002). Intraoperative complications were more common in patients with higher levels of urine metanephrine (OR = 1.01 for each 100 µg/24 h, P = 0.026) and normetanephrine (OR = 1.00 for each 100 µg/24 h, P = 0.025), larger tumours (OR = 1.4 for each 10 mm, P < 0.001), presurgical blood pressure > 130/80 mmHg (OR = 2.25, P = 0.027), pre-treated with doxazosin (OR = 2.20, P = 0.023) and who had not received perioperative hydrocortisone (OR = 3.95, P = 0.008). In conclusion, intraoperative complications in pheochromocytoma surgery are common and can be potentially life-threatening. Higher metanephrine and normetanephrine levels, larger tumour size, insufficient blood pressure control before surgery, pre-treatment with doxazosin, and the lack of treatment with perioperative hydrocortisone are associated with higher risk of intraoperative complications.