Surgical Treatment Of Pheochromocytoma Research Articles

Genetic background and intraoperative haemodynamic instability in patients with pheochromocytoma and paraganglioma: a multicenter retrospective study.

Perioperative management to maintain intraoperative haemodynamic stability is crucial during surgical treatment of pheochromocytomas and paragangliomas (PPGLs). Although approximately 70% of PPGLs carry pathogenic variants (PVs) in susceptibility genes, whether intraoperative haemodynamic instability (IHI) is associated with genetic background remains unclear. This study aimed to analyse IHI in patients with PPGL due to PVs in different genes. This retrospective study recruited 756 patients with abdominal PPGL from two tertiary care centres. Clinical information including sex, age, catecholamine-associated signs and symptoms (CAS), tumour location and size, biochemistry, and perioperative characteristics were collected. Genetic mutations were investigated using next-generation sequencing. Among the 671 patients included in the analysis, 61.8% (415/671) had IHI. IHI was significantly associated with genetic background in patients with PPGL. Most (80.9%, 89/110) patients with PPGL due to PVs in HRAS suffered IHI. In contrast, only half (31/62) of patients with PPGL due to PVs in VHL had IHI. In the multivariate regression analysis, compared to those with negative genetic testing results, patients with PPGL due to PVs in HRAS (OR 3.82, 95% CI 2.187-6.679, P<0.001), the other cluster 2 genes (OR 1.95, 95% CI 1.287-2. 569, P< 0.05), and cluster 1 genes other than VHL (OR 2.35, 95% CI 1.338-4.111, P<0.05) were independent risk factors for IHI, while PVs in VHL was not independent risk factor (OR 1.09, 95% CI 0.605-1.953, P>=0.05). In addition, age at diagnosis of primary tumour, presenting of CAS, and tumour size were identified as independent factors for IHI. The nomogram illustrated that genetic background as sharing the largest contribution to IHI, followed by tumour size, age, and presenting of CAS. IHI is associated with the genetic background in patients with PPGL. The perioperative management of patients with PPGL can be personalized according to their genetic backgrounds, tumour size, age, and presenting of CAS.

Comparison of intraoperative hemodynamics in classical and single-port adrenalectomy

BACKGROUND: Pheochromocytoma (PCC) is a tumor from the chromaffin tissue of the adrenal medulla, capable of hyperproduction of catecholamines. An important feature of pheochromocytoma, due to its hormonal activity, is the threat of sudden changes in hemodynamic parameters during surgical treatment. Retroperitoneoscopic access is currently becoming increasingly popular in the surgical treatment of chromaffin tumors of the adrenal glands. In clinical practice, two variants of the mentioned access are used: classic (three-port) and single-port. In this study, the influence of these options of retroperitoneoscopic access on intraoperative hemodynamic control was evaluated.AIM: Comparison of treatment results and intraoperative hemodynamic parameters of patients with adrenal pheochromocytomas operated with single-port and three-port retroperitonescopic access options.MATERIALS AND METHODS: A retrospective single-center cohort study was conducted on a sample of patients initially operated for pheochromocytoma at the Pirogov St. Petersburg State University High Medical Technology Clinic from October 2015 to February 2020 with intraoperative invasive hemodynamic control using PiCCO+ systems.RESULTS: 47 patients were examined, 24 of whom underwent single–port adrenalectomy (included in the first group), 23 — three-port (second group). The average tumor size in the second group was larger (40.12±9.3 and 56.7±21.5, p&lt;0.05), presumably, in this regard, the average duration of surgery was statistically significantly higher (73±21.5 and 111.7±36.1, p&lt;0.05). The body mass index (BMI) of patients in the two groups did not differ statistically significantly. Intraoperatively measured values of maximum and minimum mean systolic and diastolic blood pressure, the number of episodes of increased diastolic blood pressure above 120 mmHg and their duration did not differ statistically in both groups (p&gt;0.05). Episodes of increased systolic blood pressure above 180 mm Hg were more frequent in the second group (p=0.03), but their total duration in the two groups was without significant differences (p&gt;0.05). Intraoperative consumption of antihypertensive drugs during the release of the adrenal gland and vasopressor drugs after compression of the central vein of the adrenal gland were similar. Blood pressure after compression of the central vein in the first and second groups did not differ significantly (p&gt;0.05). No deaths and postoperative complications were registered in both groups.CONCLUSION: Single-port modification of retroperitoneoscopic access with sufficient experience of the anesthesiological team and the operating surgeon does not lead to deterioration of hemodynamic control and operation results, being safe and effective in the surgical treatment of pheochromocytoma.

Takotsubo syndrome after surgical treatment of pheochromocytoma

Takotsubo syndrome after surgical treatment of pheochromocytoma

Pheochromocytoma as a rare cause of ventricular fibrillation

This case report describes a rare cause of refractory ventricular fibrillation which was identified as a clinical manifestation of pheochromocytoma. It illustrates the pitfalls of diagnostics and surgical treatment of pheochromocytoma and the dilemma whether to implant in such patient an implantable cardioverter-defibrillator (ICD). Although the cause of cardiac arrest accompanying pheochromocytoma could be considered reversible, implantation was performed in this case due to presumed ventricular substrate with depressed left ventricular ejection fraction and with the prospect of potential recurrence of pheochromocytoma after surgery. To our best knowledge, only a few case studies of pheochromocytoma presenting with ventricular tachycardia have been reported. However, none of the published cases required prolonged cardiopulmonary resuscitation due to refractory ventricular fibrillation.

Perioperative sequential monitoring of hemodynamic parameters in patients with pheochromocytoma using the Non-Invasive Cardiac System (NICaS)

Surgical treatment of pheochromocytoma is associated with a high risk of hemodynamic instability. To reduce the risk of perioperative complications, adequate medical treatment to normalize blood pressure and restore blood volume is required. Accurate evaluation of the circulating blood volume (CBV) in perioperative patients with pheochromocytoma is clinically important. In the present study, we adopted whole-body bioimpedance monitoring technique using the Non-Invasive Cardiac System (NICaS), which can non-invasively measure cardiac output (CO) values. NICaS-derived CO values were evaluated in eight preoperative patients with pheochromocytoma and were compared with simultaneous CBV values measured by a conventional indicator dilution method using (131)I-labeled human serum albumin. In these patients with pheochromocytoma, the NICaS-derived CO values were significantly correlated with the CBV values measured by (131)I-labeled human serum albumin (4.86 ± 1.05 L/min vs 4.79 ± 1.02 L; r = 0.906; P = 0.002). Sequential NICaS-derived CO values confirmed that CBV increased after preoperative treatment with an α-blocker, with or without volume loading. The results of this study indicate that NICaS can be used to accurately and non-invasively evaluate the hemodynamic status. By sequential monitoring of NICaS-derived CO values, we are able to confirm whether adequate CBV in a patient with pheochromocytoma is obtained by preoperative medical treatment with α-blockers or volume loading, to avoid perioperative complications.

Role of Preoperative Adrenergic Blockade with Doxazosin on Hemodynamic Control during the Surgical Treatment of Pheochromocytoma: A Retrospective Study of 48 Cases

Authors evaluated the effects of selective adrenergic blockade by means of doxazosin on blood pressure in 48 patients operated on for pheochromocytoma by a multicenter retrospective study. Age, tumor size, surgical approach, and operative time were analyzed as predictive factors of intraoperative hypertensive crises. Forty-eight patients underwent adrenalectomy--four open surgery and 44 laparoscopic surgery--for pheochromocytoma of adrenal glands from 1998 to 2008 after preoperative administration of doxazosin. Perioperative cardiovascular status modifications and surgical medium- and long-term outcomes were analyzed. There was no mortality, conversion rate was 4.5 per cent, and morbidity rate was 8.3 per cent. Intraoperative hypertensive crises (180/90 mmHg or higher) were observed in 14.5 per cent (seven of 48) of patients and were treated pharmacologically with no aftermath. None of the examined variables influenced the occurrence of intraoperative hypertensive episodes. Postoperative hypotension (lower than 90/60 mmHg) was observed in four of 48 patients (8.3%) and was treated by crystalloids and hydrocortisone. In the surgical treatment of pheochromocytoma, the preoperative adrenergic blockade by doxazosin does not prevent intraoperative hypertensive crises. Nevertheless, in our series, they were of short duration and were not associated with major cardiovascular complications. Perioperative hemodynamic instability was managed by preoperative pharmacological treatment, allowing low morbidity.

A Large Pheochromocytoma With Invasion of Multiple Local Organs

A Large Pheochromocytoma With Invasion of Multiple Local Organs

MAGNETIC RESONANCE IMAGING IN DYNAMIC EXAMINATION OF PATIENTS WITH ADRENAL TUMORS AND ARTERIAL HYPERTENSION: PP.18.193

Objectives: To evaluate MRI diagnostic possibilities dynamic examination of patients (pts) with adrenal tumors and arterial hypertension. Methods: Study included 291 pts (106 pts with aldosteroma, 95 pts with pheochromocytoma, 71 pts with corticosteroma, 19 pts with incidentaloma). MRI was performed with Magnetom Avanto, Siemens, 1.5T. Standard MRI included T1-, T2-weighted, fat saturation sequence; 137 pts underwent MRI with contrast enhancement (Gd-DTPA-BMA 0.2 mgkg). Duration of arterial hypertension before treatment was 1–6 years. Period after surgical treatment of adrenal tumors was 0.5–4 years. Control group included 25 healthy volunteers. Results: In 52% pts after surgical treatment of aldosteroma some enlargement of contra- lateral adrenal was found as a result of vicar hypertrophy; in 28% pts we determined inhomogeneity of adrenal structure, possibly because the small nodes or areas of dysplasia present in adrenal gland. In all pts after surgical treatment of pheochromocytoma we marked enlargement of contra- lateral adrenal gland. In 19% pts after 1–2 year new tumors (size less than 3 cm) of the second adrenal gland was found. Majority of pts did not show any changes in renals in comparison with first examination, but in 20% pts we determined pyelectasis. MRI after surgical treatment of corticosteroma showed no changes in the contra- lateral adrenal gland in 30% of pts, in 15% - reduction in size and 40% - enlargement compared with the data obtained before treatment. In 12 pts on the basis of clinical status and laboratory data presence another adrenal tumor data was suspected, which was confirmed by MRI. In pts with incidentaloma were present the same date. In all cases MRI allowed to estimate structure, shape, and size of contra- lateral adrenal gland, find tumor of adrenal or extra adrenal localization. Conclusion: MRI is method of high effectiveness dynamic examination of pts after surgical treatment of adrenal tumors and arterial hypertension. MRI with contrast enhancement gives the important additional information.

Diagnosis and Treatment of Pheochromocytoma in an Academic Hospital from 1997 to 2007

Background The diagnosis and treatment of pheochromocytoma pose a challenge to physicians. Several trends in the presentation, diagnosis, and surgical treatment of pheochromocytoma have emerged in the last 10 years. The diagnostic accuracy and consequences of misdiagnosis of pheochromocytoma are not well known. We aimed to systemically study the diagnostic accuracy and treatment outcomes of pheochromocytoma and to reveal the causes and consequences of misdiagnosis (including both overdiagnosis and underdiagnosis). Methods We reviewed the electronic and paper charts of 49 patients who underwent adrenalectomy or adrenal biopsy with either preoperative or pathologic diagnosis of pheochromocytoma in a large academic hospital from 1997 to 2007. Three groups of patients (overdiagnosed, correctly diagnosed, and underdiagnosed) were compared on clinical courses, biochemical tests, imaging studies, and surgical outcomes. Results Pheochromocytoma was overdiagnosed in 9 patients, correctly diagnosed in 30 patients, and underdiagnosed in 10 patients. The overdiagnosis rate was 23% (9/39), and the underdiagnosis rate was 25% (10/40). The 3 distinct groups of patients exhibited significant differences in clinical presentation, biochemical tests, and imaging characteristics. The most common causes of overdiagnosis were misinterpretation of borderline biochemical test results and overzealous imaging. Overdiagnosis subjected patients to unnecessary adrenalectomy and its complications. The most common cause of underdiagnosis was failure to consider and test for pheochromocytoma. Underdiagnosis resulted in dangerous adrenal biopsy or adrenalectomy with hypertensive crisis and nearly doubled the length of stay in hospital. Surgical resection of correctly diagnosed pheochromocytoma was largely effective and safe, but intraoperative and postoperative complications occurred in some patients. Conclusion We conclude that misdiagnosis of pheochromocytoma is not uncommon and causes serious adverse effects. Correct interpretation of biochemical tests and imaging is crucial to a correct diagnosis, and pheochromocytoma should always be included in the differential diagnosis of any adrenal mass. Our data suggest that physician education is needed to improve the diagnosis and treatment of pheochromocytoma.

Unusual cardiogenic shock due to pheochromocytoma: Recovery after bridge-to-bridge (extracorporeal life support and DeBakey ventricular assist device) and right surrenalectomy

Clinical Summary A 49-year-old man with no previously known pathology or risk factors complained of brutal abdominal pain, vomiting, headache, and malaise. On arrival to his referring hospital, he presented with a massive pulmonary edema, followed by cardiogenic shock and cardiac arrest requiring cardiopulmonary resuscitation. Initial laboratory findings showed troponin I and pro-brain natriuretic peptid levels at 0.23 g/L and 5667 pg/mL, respectively (normal, 0.06 g/L and 84 pg/mL, respectively). Arterial blood gases revealed a partial oxygen pressure of 218 mm Hg at a fraction of inspired oxygen of 1. Electrocardiography did not identify signs of ischemia or infarction, and chest radiography showed bilateral infiltration consistent with pulmonary edema. An echocardiogram revealed a severe global hypokinesia (left ventricular ejection fraction, 12%) without ventricular dilatation, no valvulopathy, no pulmonary artery hypertension, and a nonincreased septal thickness. Hemodynamic and respiratory status worsened despite mechanical ventilation, volume expansion, and an intake of dobutamine (8 g) and norepinephrine (2 g). The results of coronorography were normal, and during the procedure, an intraaortic balloon pump was introduced. A thoracoabdominal computed tomographic (CT) scan revealed a 3 3-cm mass in the right adrenal gland. The diagnosis was orientated toward pheochromocytoma, and initial dosages of catecholamines were provided. We were approached as the patient’s condition got worse with signs of multiorgan failure. We then decided to implant an ECLS device (Medos Delta stream) by means of peripheral cannulation (common femoral artery and vein) in the referring hospital’s emergency department (located 200 km from our department). Medical transport, although risky, then ensued safely by road to our institution. ECLS provided an adequate flow (4 L/min), allowing norepinephrine and dobutamine disruption on day 2. The dosage of catecholamines was consistent with the presumed diagnosis of pheochromocytoma: both epinephrine and norepinephrine levels were high, at 12.5 nmol/L and 3.5 nmol/L (normal, 4 nmol/L), respectively, and the urinary vanyl mandelic acid value was 81.23 mg/24 hours (normal, 7 mg/24 hours). At day 4 on ECLS, the patient presented with an intractable hypertension, despite triple therapy (calcium-channel blocker, –blocker, and vasodilator), which was soon followed by a massive pulmonary edema. We decided to carry on with the surgical treatment of pheochromocytoma and the placement of a left ventricular assist device (DeBakey axial pump) as a bridge to recovery. ECLS was used as extracorporeal circulation to achieve the surgical procedure, including sternotomy, right subcostal laparotomy, right surrenalectomy, and DeBakey axial pump ventricular assist device (Micromed) implantation. Histologic examination of the adrenal gland confirmed the diagnosis of pheochromocytoma, and cardiac biopsy specimens showed slight nonspecific changes (hypertrophy and fibrosis). Blood and urinary samples for the dosage of catecholamines were obtained on postoperative days 10 and 30. Norepinephrine was at 10.9 and 4.8 nmol/L, and epinephrine was at 0.54 nmol/L and nondetectable, respectively. Urinary metanephrine was 3.8 mol/24 hours (normal, 3.7 mol/24 hours) and 1.3 mol/24 hours, respectively. Progressive cardiac function recovery was confirmed by means of echocardiography and an isotopic scintigraphy, allowing DeBakey ventricular assist device explantation on day 70. The postoperative course was uneventful, and the patient was discharged from the hospital on day 90. At 12 months’ follow-up, he has recovered a normal cardiac function (left ventricular ejection fraction, 60%). The thoracoabdominal control CT scan shows no abnormality.

Pheochromocytoma: A Brief Review

Pheochromocytoma, a rare tumor, can cause one of the most dramatic, life-threatening crises in the medical field of endocrinology. This catecholamine-producing tumor can occur from infancy to old age and may be associated with pregnancy. The tumor is difficult to diagnose and treat because of its location in the body and its association with a variety of symptoms, which often mislead and confuse physicians. Pheochromocytoma must be suspected in patients, especially young individuals, who experience resistant or episodic hypertension associated with sweating and headaches. Surgical treatment of pheochromocytoma is the treatment of choice. However, it is important to medically treat the patient prior to surgery to reverse the adverse effects (eg, hypertension, cardiovascular disease) associated with the excess circulating catecholamines. Pharmacists must be aware that pheochromocytoma is potentially curable if successfully treated. Thus, the urgency of diagnosis and treatment cannot be overemphasized.

Traditional versus laparoscopic surgery in the treatment of pheochromocytoma: a preliminary study.

Laparoscopic removal of adrenal masses has been successfully accomplished by many authors, but some doubts still remain about the surgical treatment of pheochromocytomas by the laparoscopic approach. The outcome of 6 patients who had laparoscopic removal of pheochromocytoma, 1 of which bilateral, was compared with that of 20 patients with pheochromocytoma who underwent open surgery. The following parameters were evaluated: frequency of hypertensive crises, time required for surgery, total volume of infused fluids, severity of postoperative pain, hospital stay, wound suppuration, or occurrence of incisional hernia, persistence or recurrence of hypertension. Laparoscopic patients had shorter hospitalizations, less postoperative fever, less fluid infusion, and absence of scar complications. There was no evident difference in the frequency of intraoperative hypertensive crises between patients who were treated by laparoscopic versus those treated by open procedure. The laparoscopic approach seems to be as safe as the open approach. However, it causes less postoperative distress, better cosmetic results, and permits a faster recovery.

Surgical Treatment of Pheochromocytomas

Pheochromocytomas are tumors that develop from chromaffin tissue of the embryonic sympathoadrenal system. These tumors may occur anywhere chromaffin tissue exists but most often develop in the adrenal medulla. Less than 50% of patients are diagnosed with pheochromocytomas while alive, and most of these tumors are found on autopsy. The classic signs and symptoms of pheochromocytomas are headache, perspiration, palpitations, pallor, and paroxysmal hypertension. Elevated levels of vanillylmandelic acid and metanephrines in patients' 24-hour urine collections are the most reliable diagnostic indicators of pheochromocytomas. Most patients with pheochromocytomas can be cured if diagnoses and surgical resections of tumors occur before irreversible cardiovascular disease and end-organ damage from hypertension develop.

Examination: Surgical Treatment of Pheochromocytomas

Examination: Surgical Treatment of Pheochromocytomas

Surgical treatment of pheochromocytoma: technique, complications, and results in six dogs.

Six dogs were diagnosed with pheochromocytoma and staged according to the World Health Organization's system for tumor classification. Two dogs had benign tumors (T1, N0, M0) and four dogs had malignant tumors (T2, N0, M1 or T3, N0, M0). All dogs had adrenalectomy, two dogs had concurrent nephrectomy, and three dogs had concurrent resection of a tumor thrombus from the vena cava. Anesthetic complications occurred in five dogs, including wide variations in heart rate (four dogs), blood pressure (five dogs), and cardiac arrhythmias (one dog). One dog died 12 hours after surgery from partial dehiscence of the suture line and hemorrhage from the vena cava, and one dog died 6 days after surgery during general anesthesia for treatment of laryngeal paralysis. Four dogs survived from 3 to 23 months (median, 15 months). One dog remained hypertensive after surgery. Benign and malignant pheochromocytomas seem to be amenable to surgical resection.

In memory of K. N. Kazeyev

On October 5, 1992, at the age of 62, a major surgeon-endocrinologist, doctor of medical Sciences, Professor Konstantin Nikolayevich Kazeev died. The whole working life of Konstantin Nikolaevich was connected with the Institute of endocrinology. He joined it in 1957 as a graduate student and worked until the very last day, going from Junior researcher to head of the Department, which he led for 17 years. Konstantin Nikolayevich Kazeev was considered to be one of the leading Russian endocrine surgeons. He performed more than 3 thousand operations on endocrine glands, had the largest experience in Europe in the surgical treatment of pheochromocytoma. K. N. Kazeev generously shared his experience, vast knowledge, paid much attention to pedagogical activity. Endocrine surgeons trained by him successfully work in different regions of the country. He was respected by colleagues and loved by patients. He always worked hard. Many thousands of patients owe their health to him.

Anesthesia for Surgical Treatment of Pheochromocytoma

Anesthesia for Surgical Treatment of Pheochromocytoma