Intrarenal hemodynamics and kidney function in pheochromocytoma and paraganglioma before and after surgical treatment.

PURPOSE: Current evidence regarding renal involvement in pheochromocytoma and paraganglioma (PPGL) is scant. More accurate diagnostic methods, such as renal Doppler ultrasound for intrarenal hemodynamic studies, may provide more detailed information on renal function. It might be postulated that renal function in PPGL patients might be altered by high blood pressure and excess secretion of catecholamines. The aim of this prospective study was to assess intrarenal blood flow parameters in PPGL patients included in the prospective monoamine-producing tumour (PMT) study and to evaluate the effects of normalisation of catecholamine production after surgical treatment on long-term renal function. MATERIALS AND METHODS: Seventy consecutive patients (aged 46.5 ± 14.0 years) with PPGL were included. Forty-eight patients from the PMT study cohort, matched for age, gender, blood pressure level and presence of hypertension, served as a control group. Renal artery doppler ultrasound spectral analysis included mean resistance index (RRI) and pulsatility index (PI). Forty-seven patients completed 12 months follow-up. RESULTS: There were no differences in renal parameters such as RRI, PI and kidney function between PPGL and non-PPGL patients as assessed by renal ultrasound, serum creatinine, eGFR and albumin excretion rate. No correlations between kidney function parameters, intrarenal doppler flow parameters and plasma catecholamines were observed in PPGL patients. At 12 months after surgery, no differences in creatinine level, eGFR, albumin excretion rate, RI and PI were found as compared to baseline results. CONCLUSIONS: In contrast to patients with other forms of secondary hypertension, our study did not show differences in intrarenal blood flow parameters and renal function between PPGL and non-PPGL subjects. Intrarenal hemodynamics and renal function did not change after normalisation of catecholamine levels by surgical treatment.

Primary pigmented nodular adrenocortical disease (PPNAD) as an underlying cause of symptoms in a patient presenting with hirsutism and secondary amenorrhea: case report and literature review.

Hypercortisolemia in females may lead to menstrual cycle disturbances, infertility, hirsutism and acne. Herewith, we present a 18-year-old patient, who was diagnosed due to weight gain, secondary amenorrhea, slowly progressing hirsutism, acne and hot flashes. Thorough diagnostics lead to a conclusion, that the symptoms was the first manifestation of primary pigmented nodular adrenocortical disease (PPNAD). All symptoms of Cushing syndrome including hirsutism and menstrual disturbances resolved after bilateral adrenalectomy. Our report indicates that oligo- or amenorrhea, hirsutism, acne in combination with weight gain, growth failure, hypertension and slightly expressed cushingoid features in a young woman requires diagnostics towards hypercortisolemia. Despite PPNAD is a very rare cause of ACTH-independent Cushing syndrome, it has to be taken into consideration, especially when adrenal glands appear to be normal on imaging and paradoxical rise in cortisol level in high-dose dexamethasone test is observed. Unlike in our patient, in vast majority of patients, PPNAD is associated with Carney complex (CC). Therefore, these patients and their first-degree relatives should be always carefully screened for symptoms of PPNAD, CC and genetic mutations of PRKAR1A, PDE11A, and PDE8B genes.

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Among patients undergoing radical cystectomy for muscle-invasive bladder cancer, local and distant recurrence is the main reason for cancer-related deaths. In case of adrenal location of metachronous metastases, the standard treatment consists on cisplatin-based systemic chemotherapy. While surgical removal of metastasis remains controversial approach, more and more data support such treatment as effective and safe. We report two cases of patients submitted to laparoscopic adrenalectomy as a sole treatment mode due to metachronous metastases of bladder cancer. No adjuvant systemic therapy was implemented. After a follow-up of 12 months patients remain disease free. This report is another voice in the on-going debate on the role of surgical treatment of patients with metastatic bladder cancer.

What parameters from 18F-FDG PET/CT are useful in evaluation of adrenal lesions?

PURPOSE: Prior studies have suggested that (18)F-FDG PET/CT can help characterize adrenal lesions and differentiate adrenal metastases from benign lesions. The aim of this study was to assess the value of (18)F-FDG PET/CT for the differentiation of malignant from benign adrenal lesions. METHODS: This retrospective study included 85 patients (47 men and 38 women, age 63.8 ± 10.8 years) who had undergone (18)F-FDG PET/CT (60 min after injection 300 - 370 MBq (18)F-FDG; Biograph 64 scanner) for evaluation of 102 nonsecreting adrenal masses. For semiquantitative analysis, the maximum standardized uptake value (SUVmax), adrenal to liver (T/L) SUVmax ratio, mean CT attenuation value and tumour diameter were measured in all lesions and compared with the pathological findings. RESULTS: Malignant adrenal tumours (68% of evaluated tumours) had a significantly higher mean SUVmax (13.0 ± 7.1 vs. 3.7 ± 3.0), a higher T/L SUVmax ratio (4.2 ± 2.6 vs. 1.0 ± 0.9), a higher CT attenuation value (31.9 ± 16. 7 HU vs. 0.2 ± 25.8 HU) and a greater diameter (43.6 ± 23.7 mm vs. 25.6 ± 13.3 mm) than benign lesions. The false-positive findings were tuberculosis and benign phaeochromocytoma. Based on ROC analysis, a T/L SUVmax ratio >1.53, an adrenal SUVmax >5.2, an attenuation value >24 HU and a tumour diameter >30 mm were chosen as the optimal cut-off values for differentiating malignant from benign tumours. The areas under the ROC curves for the selected cut-off values were 0.96, 0.96, 0.88 and 0.77, respectively. A multivariate logistic regression model revealed that the T/L SUVmax ratio was an independent prognostic factor for malignancy (p < 0.001); a CT attenuation value of >25 HU and a tumour diameter >30 mm had no additional individual importance in the diagnosis of malignancy. CONCLUSION: Using a T/L SUVmax ratio >1.53 and an adrenal SUVmax >5.2 in (18)F-FDG PET/CT led to high diagnostic sensitivity, specificity and negative predictive value for characterizing adrenal tumours. The diagnostic accuracies of the two parameters were comparable, but T/L SUVmax ratio was an independent predictor of malignancy.

Huge Hematoma as First Manifestation of Adrenocortical Carcinoma: A Case Report.

BACKGROUND Adrenocortical carcinoma (ACC) is a rare malignancy associated with unfavorable prognosis. It is mainly diagnosed in the fifth or sixth decade of life. Symptoms of ACC are associated with hormonal activity, presence of metastases, and size of the tumor. The treatment and prognosis depend on the stage of the disease assessed with the ENSAT staging system. CASE REPORT A 38-year-old White man was admitted to our department from the city hospital due to a huge hematoma of the right adrenal gland (130×100 mm). On admission, the patient's condition was stable, and no active bleeding or other complications were present. Therefore, initially, conservative treatment was performed. The control CT scan showed reduction of the hematoma (90×80 mm). Due to the unknown character of the tumor and the sudden onset of bleeding, the patient was prepared for elective surgery according to the phaeochromocytoma surgery protocol. Following preparation, the patient underwent right-sided adrenalectomy. In the postoperative histopathological examination, adrenocortical carcinoma was diagnosed, which allowed the patient to receive appropriate oncological treatment. CONCLUSIONS There is currently no clear algorithm for the management of adrenal hemorrhage. A hemodynamically unstable patient requires urgent surgical treatment. Patients in good general condition should be prepared for early elective surgery.

Familial SDHB gene mutation in disseminated non-hypoxia-related malignant paraganglioma treated with [90Y]Y/[177Lu]Lu- DOTATATE.

Familial paraganglioma may be related to mutations in succinate dehydrogenase (SDH) enzyme complex genes. Among patients with hereditary paraganglioma, SDH subunit B (SDHB) gene mutations are associated with the highest morbidity and mortality related to a higher malignancy rate. We report a family with the c.689G>A (p.Arg230His) mutation in the SDHB gene identified in two family members, a father and his daughter. While the 14-year-old daughter had no evidence of clinical disease, recurrent and later disseminated [131I]metaiodobenzylguanidine uptake-negative head and neck paraganglioma with multiple bone metastases developed in the father who underwent peptide receptor radionuclide therapy with [90Y]Y/[177Lu]Lu-dodecane tetraacetic acid octreotate (DOTATATE) at the time of the genetic diagnosis. This treatment was repeated 6 years later due to disease progression and the patient, who is currently 49 years old, remains alive and in good overall clinical condition at 8 years of follow-up after the original presentation at our unit. The growing armamentarium of imaging methods available for such patients may inform decision making regarding choice of the optimal treatment approach, potentially contributing to improved outcomes.

Undiagnosed Pheochromocytoma Presenting as a Pancreatic Tumor: A Case Report.

Pheochromocytoma is a rare catecholamine-producing tumor of the adrenal gland. Patients with known pheochromocytoma undergoing surgery require preoperative treatment with alpha-blockers to reduce the risk of intraoperative complications related to catecholamine release. If undiagnosed, pheochromocytoma can lead to life-threatening surgical complications. We report the case of a patient with a suspected solid pseudopapillary neoplasm in the pancreatic tail, for whom pancreatoduodenectomy was scheduled. However, shortly after abdominal incision, hypertensive crisis developed and was followed by severe hypotension requiring intravenous vasopressors, which prompted discontinuation of the operation. Further diagnostic evaluation revealed marked elevations in urinary excretion of methylated catecholamines and suggested that the tumor was in fact a pheochromocytoma extending from the left adrenal gland. After preoperative treatment with doxazosin, the patient underwent lateral transperitoneal laparoscopic adrenalectomy, with no major complications and an uneventful postoperative course. The pathological report confirmed a diagnosis of pheochromocytoma. Due to the potential for life-threatening surgical complications in patients with pheochromocytoma not treated preoperatively with alpha-blockers, this tumor type should be included in the differential diagnosis of abdominal tumors of unknown origin.

Adrenal-sparing surgery for a hormonally active tumour - a single-centre experience.

INTRODUCTION: Surgeries that spare the adrenal cortex during adrenalectomy have profound justification. Indications for this type of surgery are fairly strict, and more than 30 years of observations continuously verify the results of the procedure. MATERIAL AND METHODS: Of a total of 650 adrenalectomies, 22 (3.4%) were adrenal cortex-sparing surgeries. There were 16 women and six men in this group. In 10 cases, surgery was performed for pheochromocytoma, eight cases involved Conn's syndrome, and in four cases - paragangliomas located in the para-adrenal region. Secretory activity was identified in all cases. RESULTS: Laparoscopic partial adrenalectomy was performed in 20 patients. Conversion to open laparotomy was necessary in two cases. In patients after bilateral resection of pheochromocytoma surgery, glucocorticoids were supplemented for six weeks. No significant surgical complications were observed in this group. CONCLUSIONS: Partial adrenalectomy for minor lesions should be a much more commonly utilised treatment method (of choice). Where bilateral adrenalectomy is necessary, a sparing procedure on one side protects patients from the need for hormonal substitution. The remaining part of the adrenal gland undertakes satisfactory secretory function after six weeks at the latest.

Left Ventricular Structural and Functional Alterations in Patients With Pheochromocytoma/Paraganglioma Before and After Surgery.

OBJECTIVES: This study sought to evaluate left ventricular (LV) structure and function in pheochromocytoma and paraganglioma (PPGL) patients before and after curative surgery. BACKGROUND: Data on catecholamine-induced effects on LV structure and function in patients with PPGL are limited and conflicting. METHODS: The study evaluated 81 consecutive patients with a PPGL, among whom 66 were evaluated 12 months after tumor removal. Fifty patients matched for age, sex, hypertension presence, and blood pressure (BP) levels served as a control group (non-PPGL group). Echocardiography was employed to assess the LV mass index (LVMI), systolic function including speckle tracking echocardiography, and diastolic function. RESULTS: Patients with PPGL were characterized by higher LVMI (median 103 [interquartile range (IQR): 88 to 132] g/m2 vs. median 94 [IQR: 74 to 106] g/m2; p = 0.006) and frequency of LV hypertrophy (44.4% vs. 24.0%; p = 0.018) compared with the non-PPGL group. Patients with PPGLs were characterized by lower global longitudinal strain (GLS) and early diastolic mitral annular velocity compared with patients in the non-PPGL group (median -17.2% [IQR: 15.6% to 18.9%] vs. median -19.3% [IQR: 17.7% to 20.6%]; p < 0.001; and median 11.1 [IQR: 8.3 to 13.0] cm/s vs. median 12.3 [IQR: 10.6 to 14.6] cm/s; p = 0.018, respectively). Presence of LV hypertrophy and GLS were independently associated with plasma free metanephrine concentrations. In operated patients, there were lower frequencies of LV hypertrophy (39.4% vs. 22.7%; p = 0.003), LVMI (median 98 [IQR: 85 to 115] g/m2 vs. median 90 [IQR: 76 to 109] g/m2; p < 0.001), and the ratio of transmitral early diastolic velocity to early diastolic mitral annular velocity (median 6.8 [IQR: 5.5 to 8.6] vs. median 6.0 [IQR: 5.0 to 7.6]; p = 0.005) but higher values for GLS (median -17.4 [IQR: -15.8 to 19.1] vs. median -18.5 [IQR: -17.1 to 20.1] p < 0.001) after compared with before surgery. CONCLUSIONS: Catecholamine excess in patients with PPGLs can lead not only to LV hypertrophy, but also to impairment of systolic LV function and subclinical alterations of diastolic LV function, independently of BP levels. These structural and functional changes are reversible after surgical intervention.

Retinal arterial remodeling in patients with pheochromocytoma or paraganglioma and its reversibility following surgical treatment.

OBJECTIVE: Structural abnormalities in resistance arteries are a hallmark of patients with hypertension. In hypertensive patients with pheochromocytoma or paraganglioma (PPGL), it is still a matter of debate whether structural vascular changes are because of elevated blood pressure (BP) or to toxic effects of elevated circulating catecholamines. Hence, the aim of our study was to assess whether catecholamine excess and/or elevated BP affect the structure of small retinal arteries in patients with catecholamine-producing tumors. METHODS: The study included 27 patients with PPGL and 27 hypertensive patients. All patients underwent biochemical tests for catecholamine excess, echocardiography and analyses of scanning-laser-Doppler-flowmetry (SLDF) both at baseline and 12 months following surgical resection of PPGL. RESULTS: Baseline retinal arterial diameter, arterial wall thickness and wall cross sectional area (WCSA) were higher in patients with PPGL as compared with subjects without PPGL (arterial diameter: 110 ±â€Š16.5 vs. 99.5 ±â€Š10.8 µm, wall thickness: 16.3 ±â€Š6.0 vs. 13.5 ±â€Š4.0 µm, WCSA: 4953.9 ±â€Š2472.8 vs. 3784.1 ±â€Š1446.3 µm, P < 0.05). Significant correlations were noted between wall thickness and WCSA and echocardiographic parameters assessing diastolic and systolic function of left ventricle. No correlations between retinal parameters, BP level and plasma concentrations of metanephrines were observed. In patients with PPGL, there were postoperative decreases in wall thickness (16.4 ±â€Š15.8 vs. 14.8 ±â€Š4.7 µm; P = 0.011) and WLR (0.42 ±â€Š0.13 vs. 0.37 ±â€Š0.10; P = 0.003) at 12 months after surgical removal of tumors. CONCLUSION: This is the first study to demonstrate that catecholamine excess is related to thickening of retinal arteries independent of BP and reversible after surgical cure. These data support a role of catecholamines in vascular remodeling in PPGL patients.

Surgical treatment of abdominal paragangliomas.

INTRODUCTION: Extraperitoneal, abdominal paragangliomas most commonly originate from the sympathetic nervous system. Typical features include catecholamine overproduction and the potential for malignancy. Lesions are usually located paravertebrally, but when growing in a more expansive manner they may also appear between the inferior vena cava and aorta. In the authors' opinion this site excludes laparoscopic tumourectomy. MATERIAL AND METHODS: Twenty-eight patients were selected for surgical management of abdominal paragangliomas in the past eight years at our endocrine surgical centre. This group consisted of 21 (75%) women and seven (25%) men, aged 14 to 84 years (mean 47.9). In 13 (46.4%) cases paroxysmal hypertension was observed. Type 2 diabetes was noted in another 10 (35.7%) patients, and Takotsubo acute coronary syndrome in two (7.1%). Patients were preoperatively qualified for either open surgery or laparoscopic tumourectomy based on visualisation and location of the tumours in imaging studies. RESULTS: All patients were successfully operated. Eleven (39.3%) patients qualified for laparoscopy, while the remaining 17 (60.7%) were treated with an open surgical approach due to difficult access to the lesion. The mean operative time was 130 minutes for laparoscopy and 120 minutes for laparotomy (p = 0.2). There were no local or general complications after either type of procedure. CONCLUSIONS: The use of laparoscopic access is practically excluded in the treatment of paragangliomas located between the inferior vena cava and aorta, especially at the level of the renal vessels and extending superiorly to the diaphragm.

Lateral laparoscopic adrenalectomy in patients with previous abdominal surgery - single-center experience.

INTRODUCTION: Lateral transabdominal adrenalectomy (LTA) is the most common minimally invasive technique used to treat patients with adrenal tumors. AIM: To analyze intra-operative and post-operative complications and reasons for conversion to open surgery in patients who underwent LTA and had previous abdominal surgery. MATERIAL AND METHODS: Five hundred and nineteen patients underwent LTA in our center between 2005 and 2016. We identified a study group of 150 patients, with previous abdominal surgery. We analyzed the frequency of intra-operative and post-operative complications and the reasons for conversion from laparoscopic to open adrenalectomy. RESULTS: The patients' mean age was 58; they underwent LTA due to hormonally active tumors (n = 79, 53%) and non-functioning adrenal tumors (n = 71, 47%). The size of adrenal lesions ranged from 20 mm to 90 mm. Seventy-eight (52%) adrenal lesions were found in the right adrenal gland, and 72 (48%) lesions in the left adrenal gland. The mean operating time was 130 min. The mean stay in hospital was five days. The intra-operative complications included blood pressure fluctuations (n = 32), abnormal vascular supply of the adrenal glands causing difficulties with dissections (n = 3), and respiratory problems (n = 1). Two (1.3%) patients had post-operative bleeding at the site of removed adrenal glands; 1 patient had an exacerbation of asthma postoperatively. Of the 150 patients analyzed, 3 (2%) required conversion to open adrenalectomy. The conversions were not caused by abdominal adhesions. CONCLUSIONS: Lateral transabdominal adrenalectomy is feasible and safe in patients with previous abdominal surgery. In our study, conversion from laparoscopic to open adrenalectomy was not caused by abdominal adhesions.

Adrenal cysts - optimal laparoscopic treatment.

INTRODUCTION: Adrenal cysts develop in up to about 0.2% of the overall population. They may account for up to 11% of all pathologies of adrenal glands. AIM: Is laparoscopic resection of adrenal cysts a method for the treatment of these pathologies? MATERIAL AND METHODS: In the years 2010-2017, a total of 27 patients underwent surgery due to adrenal cysts; those included 18 (66.7%) women and 9 (33.3%) men aged 29 to 84 years (mean age: 42.7). Cyst diameter ranged from 55 to 130 mm. After exclusion of hormonal hyperactivity, parasitic cysts, or, to the best possible extent, cancer lesions, patients were qualified for adrenal-sparing laparoscopic surgery. RESULTS: All patients were subjected to laparoscopic surgery. Cystic wall resection was performed in 15 (55.6%) patients while adrenalectomy was performed in the remaining 12 (44.4%) patients. The decision regarding the extent of the surgery was made intraoperatively. Histopathological assessment revealed pathological adrenal lesions in as few as 3 (11.1%) patients, with the rest of the study population, i.e. 24 (88.9%), presenting with normal adrenal tissue. CONCLUSIONS: Laparoscopic resection of adrenal cysts appears to be recommendable as a method for the treatment of these pathologies. It is simpler than adrenalectomy and associated with low risk of any pathological lesion remaining within the adrenal gland following careful intraoperative assessment by an experienced surgeon.

Adrenal Incidentaloma - Diagnostic and Treating Problem - Own Experience.

INTRODUCTION: Incidentaloma is defined as an tumor diagnosed accidentally using imaging studies performed due to other indications. The aim of this paper was to describe the diagnostic and treatment problems experienced by patients with adrenal incidentaloma in a clinical practice. MATERIAL AND METHODS: In years 2009-2012 there were 33(16,5%) adrenal tumors diagnosed incidentally out of 200 cases treated due to adrenal pathology. The group consisted of 54 patients aged 27-77. In 15(45,5%) patients the diagnosis was made based on ultrasound examination, while in 18(54,5%) the tumor was visualized in CT/MRI. RESULTS: Only after the diagnostics was finalized, in which in all cases no signs of hormonal activity were detected, 26(78,8%) patients were qualified for the surgery. In 7(21,2%) cases no indications for such a management were found. The latter group was followed-up and in all patients the indications for the surgery arose due to enlargement of the tumor or/and the existence of hormonal activity. Our observations suggest that the incident finding of adrenal tumor is an indication of long-term observation. The analysis of our material shows that all patients observed needed surgical treatment. CONCLUSIONS: The small adrenal tumors under follow-up have a tendency to enlarge and acquire hormonal activity. Long-term observation significantly increases the costs of treatment in that group, which eventually results in surgical management.

Effectiveness of unilateral laparoscopic adrenalectomy in ACTH-independent hypercortisolaemia and subclinical Cushing's syndrome - a retrospective study on a large cohort.

INTRODUCTION: To assess the effectiveness of early unilateral laparoscopic adrenalectomy in ACTH-independent and subclinical hypercor-tisolaemia. MATERIAL AND METHODS: We conducted a unicentric, retrospective study. Between 2010 and 2015, 356 laparoscopic adrenalectomies were performed in the Department of General and Endocrine Surgery of the MUW. Hypercortisolaemia was found in 50 (14%) patients, while overt hypercortisolaemia was found in 31 patients. In the hypercortisolaemia group, ACTH-dependent hypercortisolaemia was diagnosed in five (10%) and ACTH-independent hypercortisolaemia in 25 patients (50%). One patient with overt hypercortisolaemia had cancer of the adrenal cortex. The remaining 19 (38%) patients had subclinical Cushing's syndrome. For our study, we compared patients with ACTH-independent hypercortisolaemia (n = 25) with those with Cushing's syndrome (n = 19). Patients with ACTH-dependent hyper-cortisolaemia (n = 5) and the patient with cancer of the adrenal cortex (n = 1) were excluded. RESULTS: Patients from both groups (n = 44) underwent a unilateral transperitoneal adrenalectomy. Good early outcomes were observed in 42 patients (93.3%). In one patient, an additional laparoscopic surgery was necessary on postoperative day 0 due to bleeding. In another patient, on day 22 post-surgery, we found an abscess in the site of the excised adrenal gland, which was drained under laparoscopic guid-ance. In three patients (6.8%) with substantial obesity, temporary respiratory insufficiency of varying degrees occurred. We did not observe any thromboembolic complications. All patients with overt hypercortisolaemia and nine patients with subclinical hypercortisolaemia had secondary adrenal insufficiency postoperatively. CONCLUSIONS: Transperitoneal unilateral laparoscopic adrenalectomy is an efficient and safe treatment option in patients with ACTH- -independent hypercortisolaemia, both overt and subclinical.

The usefulness of laparoscopic adrenalectomy in the treatment of adrenal neoplasms - a single-centre experience.

INTRODUCTION: Adrenal neoplasms comprise about 10% of all tumours affecting this organ and constitute a significant, at first diagnostic and subsequently therapeutic, problem, especially since a relatively high proportion of neoplastic lesions are asymptomatic. The number of diagnosed metastases to adrenal glands is increasing. Surgical treatment involves both open surgery as well as laparoscopy. MATERIAL AND METHODS: There were 245 adrenalectomies performed at our centre due to various indications over the past four years. In 27 (11.5%) cases neoplasms were diagnosed in the final histopahtological examination. In 11 (40.7%) cases primary adrenal cortex tumours were diagnosed, metastases from other solid organ tumours were identified in another 12 (44.4%) patients, and rarer neoplasms were diagnosed in the remaning 4 (14.8%) subjects. Cases of malignant pheochromocytoma were not included in this report. RESULTS: Laparoscopic adrenalectomy was performed in 23 (85.2%) subjects, while the ramaining 4 (12.9%) patients were subject to open adrenalectomy (conversion to open procedure in one case). There were no deaths or significant complications in the perioperative period. Comparing mean duration of open (140 minutes) and laparoscopic (190 minutes) procedures yielded a statistically significant difference (p = 0.02). There was no statistically significant difference found in the duration of operation with regard to laparoscopic adrenalectomies of tumours less than 50 mm and over 55 mm in diameter (p = 0.16). CONCLUSIONS: Laparoscopic adrenalectomy is a safe and effective method of treatment of adrenal tumours. Its oncological completeness is comparable (to open surgery) when performed by experienced surgical teams. Laparoscopy is the reason for the smooth postoperative course observed in most patients.

Comparison of phenoxybenzamine and doxazosin in perioperative management of patients with pheochromocytoma.

BACKGROUND: Adrenalectomy with preoperative pharmacological preparation is strongly recommended in patients diagnosed with pheochromocytoma, in order to prevent perioperative complications. AIM: To compare phenoxybenzamine (PhB) and doxazosin (DOX) in terms of perioperative haemodynamic status in patients with pheochromocytoma, who have been prepared for adrenalectomy. METHODS: Retrospective analysis of 44 patients with pheochromocytoma (aged 16-80 years, 29 females) who underwent adrenalectomy. Patients were divided into two groups: 35 patients on DOX and nine patients on PhB. RESULTS: Mean time of preparation for surgery was 38.8 days in the DOX group and 18.3 days in the PhB group (p = 0.04). No statistically significant differences between the DOX and PhB groups in intraoperative blood pressure (BP) fluctuations were found: < 170/100 mm Hg (34% vs. 44%, respectively, p = 0.42), ≥ 200/110 mm Hg (40% vs. 22%, respectively, p = 0.28). Mean greatest intraoperative systolic BP (195 ± 53 vs. 166 ± 42 mm Hg, p = 0.21) and diastolic BP (98 ± 20 vs. 89 ± 46 mm Hg, p = 0.21), and mean lowest intraoperative systolic BP (87 ± 13 vs. 79 ± 17 mm Hg, p = 0.25) and diastolic BP (49 ± 8 vs. 46 ± 12 mm Hg, p = 0.60) were not different between the DOX and PhB groups, respectively. Sodium nitroprusside was administrated in 30% DOX vs. 11% PhB patients (p = 0.25). Laparoscopic surgery was conducted in 97% DOX vs. 89% PhB patients (p = 0.64). Postoperative BP drop below 90/60 mm Hg was noted in 48% of the DOX vs. 43% of the PhB group (p = 0.56). Negative correlation was found between the length of DOX administration with maximal intraoperative systolic BP (r = -0.45, p = 0.006) and diastolic BP (r = -0.39, p = 0.019). CONCLUSIONS: There are no clinically relevant differences between patients with pheochromocytoma, who have been prepared for adrenalectomy with DOX or PhB.

Intravascular Treatment of Left Subclavian Artery Aneurysm Coexisting with Aortic Coarctation in an Adult Patient.

Coexistence of aortic coarctation with aneurysm of subclavian artery is a uncommon situation and may require unusual treatment in patients. A 40-year-old patient diagnosed incidentally with left subclavian artery aneurysm coexisting with aortic coarctation. Patient was initially referred for hybrid treatment. Initially ostium of the left subclavian artery was covered with a stent-graft. Over a 30-month follow-up period aneurysm became thrombosed all the way up to the ostium of internal mammary artery. The patient did not present with neurological symptoms or signs of upper limb ischemia. Taking into consideration good blood supply to the axillary artery via reversed blood flow in the thyreocervical trunk, hence we decided not to proceed with cervicoaxillary bypass grafting. Implantation stent-graft into aorta coarctation with covering axillary artery is proper way of treatment and may need no other surgical procedures.

A single-centre experience of the implementation of adrenal vein sampling procedure: the impact on the diagnostic work-up in primary aldosteronism.

BACKGROUND: Primary aldosteronism is one of the most common causes of secondary hypertension. Adrenal vein sampling (AVS) remains a "gold standard" procedure in differentiation between unilateral (adenoma) and bilateral (hyperplasia) disease. AIM: The aim of this study was to present our single-centre experience in establishing and implementating the AVS procedure. METHODS: All patients had primary aldosteronism confirmed in a salt-infusion test. AVS was performed sequentially during a continuous intravenous infusion of cosyntropin and was preceded by multislice contrast-enhanced computed tomography (CT) examination of adrenal glands performed a few weeks before AVS in the majority of patients. AVS was regarded as successful if the ratio of each adrenal vein cortisol to inferior vena cava cortisol levels (selectivity index [SI]) was higher than 3.0. In the case of failure, a second attempt was considered in a few weeks. Patients were divided into four groups according to the order of the procedure by quartiles. RESULTS: Between 31 May, 2012 and 5 May, 2016, AVS was performed in 124 patients (69% males, aged 55.3 ± 10.3 years) and was successful in 120 (96.8%) patients. All failed cases were due to the failure of cannulation of the right adrenal vein. The first-attempt success rate was 94.3% (117 of 124 patients) and increased from 83.9% in the first 31 patients to 100% in the last 31 patients. Similarly, the overall success rate increased from 93.5% to 100%. The right SI was significantly higher than the left one (26.4 vs. 11.0, p < 0.0001). Both indices did not differ across quartiles of patients. No complications occurred during the procedure. CONCLUSIONS: The AVS procedure, preceded by adrenal CT, may be implemented into daily diagnostic practice safely with an excellent success rate.

The local spread of pheochromocytoma after adrenalectomy with a rupture of the tumor capsule at the time of the surgery.

INTRODUCTION: We present a case of a 29-year-old patient treated due to fully symptomatic pheochromocytoma of the right adrenal gland. CASE PRESENTATION: Patient was operated on and an open right-sided adrenalectomy was performed. At the time of the surgery, a rupture of the tumor capsule occurred. Five years post-operatively, a recurrence of the symptoms of chromaffin-cell tumor was noted. After the exact localization of the multiple recurrences, the patient was reoperated on. CONCLUSION: The case of pheochromocytoma is presented due to the possibility of chromaffin-cell seeding into the peritoneum, with no signs of distal metastases so far.