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Diagnosis and treatment of pheochromocytoma
Abstract
Early detection of a pheochromocytoma is necessary to avoid the cardiovascular complications of hypertension and to recognize the 10% of malignant neoplasms. Hypertensive changes, particularly hypertensive spikes and stable hypertension are the most common clinical features. A pheochromocytoma must be suspected in presence of various symptoms of which cephalea, tachycardia, diaphoresis are the most frequently encountered. Plasma catecholamines and 24-hours urine metabolites measurements have 60-100% and 72-99% sensitivity respectively. Radiologic procedures commonly used to locate a pheochromocytoma include CT and RM: they achieve 100% sensitivity for unilateral adrenal lesions and respectively 64% and 88% sensitivity for extra-adrenal lesions. Scintigraphic localization with MIBG provides functional informations and is recommended for follow-up of the recurrent or metastatic pheochromocytoma. Preoperative medical treatment using alpha and beta blocking agents or calcium channel blockers has contributed to reduce perioperative morbidity and mortality. Anterior transabdominal approach is advocated as the standard operative procedure; this approach allows ready access to any site where tumors are obviously present and permits thorough exploration of the abdominal cavity for additional contralateral adrenal or extra-adrenal lesions. An accurate preoperative localization of a sporadic, unilateral, of less than 7-10 cm, benign neoplasm constitutes the indication for the laparoscopic adrenalectomy. Pheochromocytoma-free survival were 92% and 80% at 5 and 10 years respectively; in living patients without recurrence, hypertension-free survival was 74% and 45% at 5 and 10 years respectively. Extra-adrenal pheochromocytomas are frequently malignant (36%) and are associated with a high incidence of persistent or recurrent disease (32%).
... Every incidentally discovered adrenal mass should be investigated for pheochromocytoma since it accounts for about 20% of the adrenal mass [1] and approximately 10% of it is malignant. [2] It can metastasize to lymph nodes, bone, liver, lung, etc., Pheochromocytoma can synthesize and secrete catecholamine automatically. Its secretion is not regulated by the nervous system. ...
Functional malignant pheochromocytoma is a rare tumor. Also, liver metastatic pheochromocytoma is deemed to be incurable because of its poor prognosis. Surgical debulking and removal of primary and metastases tumors, as well as radiosurgical or surgical treatment are recommended for patients. We treated a patient with multiple liver metastasis of functional malignant pheochromocytoma 4 years after the adrenal primary lesion was resected. The liver metastases were successfully removed by surgical resection. During the operation, the blood pressure increased quickly when the tumor was held between the fingers, reached the highest (201/110 mmHg), and then quickly declined to normal when the pressure was relieved. The patient is alive for more than 5 years after the operation. Thus, we believe that functional liver metastases of malignant pheochromocytoma may be curable by surgical resection with careful monitoring of anesthesia and blood pressure. Every effort should be taken to avoid direct stimulation of the tumor in the operation.
Adrenal masses discovered by imaging techniques for reasons unrelated to adrenal diseases are called incidentalomas with pheochromocytomas accounting for about 20%. The aim of this study was to report on our experience of 5 patients operated for incidentally discovered adrenal pheochromocytoma and update the literature concerning this subject.
From September 1976 to December 2000 we operated on 28 for adrenal incidentaloma, and pheochromocytoma was detected in 5 patients (19%). Average age was 61.4 years (range 54-65). All patients underwent adrenal hormonal as well as imaging investigation. Preoperative care for pheochromocytoma was performed in 3 patients. All patients were followed up.
Average tumor diameter, as detected by CT, was 5.5 cm (range 3-10). Urinary 24/h excretion of VMA resulted elevated in 2 cases (40%). Two patients during adrenalectomy developed paroxysmal hypertension which was readily controlled by intravenous administration of nitroprussiate and trandate. Postoperative outcome was uneventful. Average tumor size, as detected by pathology, resulted 8.9 cm (range 3-12). Mean follow-up was 60.4 months (range 32-122) and overall survival rate was 60%. Three patients are alive and disease-free and 2 died after a follow-up of 32 and 36 months for reasons unrelated to the primary disease.
Every incidentally discovered adrenal mass should be investigated for pheochromocytoma since accounting for about 20%. Undetected pheochromocytomas undergoing surgery without preoperative care for avoiding cardiovascular disturbances confer a high risk for morbidity and mortality, especially in pregnancy and childhood. Nonfunctional and small tumors sized 3 cm or lesser may be closely followed up by imaging and hormonal investigation. Surgery is indicated when tumors are functional or larger than 3 cm. Actually, laparoscopy is the new gold-standard in treating adrenal pheochromocytomas. Adrenal sparing surgery as well as autotransplantation of adrenal tissue are both effective and safe in treating patients with bilateral pheochromocytomas, thus improving quality of life since avoiding lifelong steroid dependency. When managed appropriately, the outlook for pheochromocytoma is excellent with a free survival rate of 92% and 80% at 5 and 10 years respectively. Unfortunately, malignant pheochromocytomas are still difficult to treat.
Wir berichten über den Fall einer 73jährigen Patientin, die elektiv an einem Phäochromozytom operiert wurde. Eine präoperative alpha-Rezeptoren-Blockade mit Phenoxybenzamin konnte nicht durchgeführt werden, da sie von der Patientin abgelehnt wurde. Die präoperative antihypertensive Therapie erfolgte daher mit dem kompetitiven alpha-Rezeptor-Antagonisten Prazosin und dem beta-Rezeptoren-Blocker Metoprolol. Intraoperativ wurde zur Blutdruckkontrolle Natriumnitroprussid eingesetzt. Nach Entfernung des Primärtumors und Absetzen des Natriumnitroprussids kam es noch intraoperativ zu rezidivierenden Blutdruckspitzen, woraus sich die Verdachtsdiagnose eines multilokulären Phäochromozytoms ergab. Ein im makroskopisch unauffälligen Pankreasschwanz liegender Tumor konnte lediglich durch Blutdruckanstieg bei Palpation lokalisiert werden. Nach vollständiger Resektion aller Tumorherde traten keine weiteren hypertensiven Phasen mehr auf. Der postoperative Verlauf war unauffällig.
Malignant pheochromocytoma is a rare cause of hypertension and still has a high mortality rate. The most accurate way to localize a malignant pheochromocytoma is by a combination of scans, both CT and scintigraphy. Selective sampling of venous blood from multiple sites for plasma catecholamine levels is a safe and reliable technique and may be used successfully in some patients. A case is presented where venous sampling proved to be useful in preoperatively localizing a malignant pheochromocytoma and its metastatic lesions which both CT and ultrasound had failed to demonstrate.
An adrenal pheochromocytoma producing somatostatin (SRIF) and vasoactive intestinal polypeptide (VIP) in a 17-year-old boy is presented. High concentrations of immunoreactive (IR)-SRIF were found in plasma taken from the antecubital vein (31.0-33.0 pg/ml) and the inferior caval vein near the tumor (54.6 pg/ml), but after removal of the tumor the values became normal (11.0-15.2 pg/ml). In two portions of the resected tumor, considerable but different amounts of IR-SRIF (151.7 and 12.1 ng/g wet wt) and IR-VIP (13.0 and 5.5 ng/g wet wt) were demonstrated with size heterogeneities. Immunohistochemically, many IR-SRIF cells and a few IR-VIP cells were observed, but no cell reacting with both anti-SRIF and anti-VIP sera was found. Electronmicroscopically, many tumor cells had catecholamine-like granules (250-350 nm in diameter) while some others had VIP-like granules (110-140 nm in diameter). However, no granules resembling the SRIF granules seen in the pancreatic D cells were found. This seems to be the first report of an adrenal pheochromocytoma that produces SRIF and VIP simultaneously. It provides information on the histogenesis of hormone-producing neurogenic tumors.
One-thousand computed tomograms of the abdomen, chest, and pelvis in oncologic patients were reviewed to determine the incidence of serendipitous findings and their impact on clinical management. The incidence of serendipitous findings was 30% in thorax, 24% in both genitourinary and osseous structures, 23% in the abdomen, and 21% in the retroperitoneum. These serendipitous abnormalities changed clinical management or required further diagnostic procedures (thus, they were considered significant) in less than 1% of the abdominal to 12% in osseous findings.
Operative treatment is the method of choice for pheochromocytomas. The best success of the operation requires both good operative technique and effective preoperative medication. Phenoxybenzamine hydrochloride, an alpha-receptor blocker, has been our traditional premedication. Since 1988 we have used it in a combination with alpha-methylthyrosine, a catecholamine-synthesis blocker.
To evaluate different preoperative medications.
Between 1984 and 1994 22 patients were operated on for pheochromocytomas in Tampere University Hospital. Five patients received alpha-receptor blocker, three patients received catecholamine-synthesis blocker, and 14 patients received their combination for a median of 33 days prior to the operation.
Three of the five patients receiving alpha-receptor blocker, two of the three patients receiving catecholamine-synthesis blocker, and four of the 14 patients receiving combination therapy demonstrated high systolic blood pressure during the operation. Three patients, one in the alpha-receptor blocker group and two in the combination therapy group, demonstrated a short hypotensive period during the operation. None of the patients experienced severe perioperative cardiac arrhythmias. Mild sedation occurred similarly in either premedications, but the treatment did not need to be discontinued due to adverse reactions. There was no hospital mortality. Two patients had postoperative complications.
Our initial experience with the combination therapy with alpha-receptor blocker and catecholamine-synthesis blocker is encouraging.