National Cancer Institute®
Ultima Vez Modificado: 1 de julio del 2002
UI - 10927994
AU - Prys-Roberts C
TI - Phaeochromocytoma--recent progress in its management.
SO - Br J Anaesth 2000 Jul;85(1):44-57
AD - Sir Humphry Davy Department of Anaesthesia, Bristol Royal Infirmary, UK.
UI - 11914474
AU - McNichol AM
TI - Differential diagnosis of pheochromocytomas and paragangliomas.
SO - Endocr Pathol 2001 Winter;12(4):407-15
AD - University Department of Pathology, Royal Infirmary, Castle Street, Glasgow G4 0SF, Scotland, United Kingdom. A.M.McNicol@clinmed.gla.ac.uk
Paragangliomas are of two types, sympathetic and parasympathetic, depending on the type of paraganglion in which they arise. The term pheochromocytoma is reserved for tumors arising in the adrenal medulla. These tumors are usually fairly easy to diagnose. However, several areas are the subject of debate, including the identification of malignant potential, the diagnosis of medullary hyperplasia, and the recognition of composite tumors. Some histologic features can cause problems in differential diagnosis. Paragangliomas may have spindle cell morphology or contain pigment, requiring distinction from mesenchymal tumors and melanoma, respectively. Extensive degenerative change in pheochromocytomas may mimic adrenal cortical tumors. This short review addresses the diagnosis of pheochromocytomas and paragangliomas and discusses useful approaches in the aforementioned problem areas.
UI - 11963757
AU - Lehnert H; Hahn K; Dralle H
TI - [Benign and malignant pheochromocytoma]
SO - Internist (Berl) 2002 Feb;43(2):196, 199-209
AD - Klinikum der Otto-von-Guericke-Universitat Magdeburg, Klinik fur Endokrinologie und Stoffwechselkrankheiten, Leipziger Strasse 44, 39120 Magdeburg. firstname.lastname@example.org
UI - 12028627
AU - Al-Sobhi S; Peschel R; Zihak C; Bartsch G; Neumann H; Janetschek G
TI - Laparoscopic partial adrenalectomy for recurrent pheochromocytoma after open partial adrenalectomy in von Hippel-Lindau disease.
SO - J Endourol 2002 Apr;16(3):171-4
AD - Department of Surgery, King Faisal Specialist Hospital & Research Centre, Riyadh, Kingdom of Saudi Arabia. email@example.com
BACKGROUND AND PURPOSE: Unilateral total adrenalectomy is the standard treatment for benign unilateral adrenal tumors. Partial adrenalectomy has to be considered for bilateral adrenal tumors. Recently, our group has reported the feasibility of unilateral and bilateral partial adrenalectomy by means of laparoscopy. Now, we present a case of recurrent pheochromocytoma after open bilateral adrenalectomy and demonstrate that laparoscopic cortex-sparing surgery is feasible for a recurrence after open surgery. Case Report: At the age of 10 years, a boy was found to have bilateral adrenal pheochromocytoma and was treated by open bilateral partial adrenalectomy. Eight years later, the patient presented with palpitation, sweating, and severe hypertension. Investigations biochemically and radiologically demonstrated functional recurrent pheochromocytoma in the left adrenal gland. Genetic studies confirmed mutation of the von Hippel-Lindau (VHL) gene. The patient was prepared for surgery preoperatively by phenoxybenzamine and metropolol. The surgery was performed as planned, and the normal adrenal tissue was spared. The patient had an uneventful postoperative course. His blood pressure was normal on the day of discharge. CONCLUSIONS: In a specialized center with experienced laparoscopic surgeons, laparoscopic partial adrenalectomy for recurrent pheochromocytoma is feasible even after previous open surgery on the ipsilateral adrenal gland. Adrenal-sparing surgery is indicated in hereditary syndromes such as VHL and MEN II to avoid the problems of life-long steroid replacement. Recurrences have to be expected, but further surgery may be less difficult by the previous laparoscopic approach.
UI - 12050290
AU - Igaz P; Patocs A; Racz K; Klein I; Varadi A; Esik O
TI - Occurrence of pheochromocytoma in a MEN2A family with codon 609 mutation of the RET proto-oncogene.
SO - J Clin Endocrinol Metab 2002 Jun;87(6):2994
UI - 12032534
AU - Franklin SL; Eisenhofer G; Geffner ME
TI - Simultaneous development of pheochromocytomas in prepubertal siblings with von Hippel-Lindau syndrome.
SO - J Pediatr 2002 May;140(5):625-8
AD - Division of Endocrinology, Department of Pediatrics, Mattel Children's Hospital at UCLA, Los Angeles, California 90095, USA.
We report 2 siblings with a known von Hippel-Lindau mutation who simultaneously developed asymptomatic pheochromocytomas at significantly younger ages than are typically seen in this setting.
UI - 12050522
AU - Sumino Y; Tasaki Y; Satoh F; Mimata H; Nomura Y
TI - Spontaneous rupture of adrenal pheochromocytoma.
SO - J Urol 2002 Jul;168(1):188-9
AD - Department of Urology, Oita Medical University, Oita, Japan.
UI - 11826768
AU - Martinez Brocca MA; Acosta Delgado D; Quijada D; Navarro Gonzalez E;
TI - Soto Moreno A; Gonzales Duarte D; del Valle Lopez de Ayala A; Astorga Jimenez R Pheochromocytoma in a pregnant woman with multiple endocrine neoplasia type 2a.
SO - Gynecol Endocrinol 2001 Dec;15(6):439-42
AD - Department of Endocrinology, Hospital Universitario Virgen del Rocio, Avenida Manuel Siurot s/n, 41013 Seville, Spain.
Pheochromocytoma is a rare cause of hypertension. Its coexistence with pregnancy is exceptional and laparoscopic removal has rarely been reported. We describe the case of a 34-year-old woman with multiple endocrine neoplasia type 2a (MEN 2a) with adrenal pheochromocytoma diagnosed in the 6th week of pregnancy. After pretreatment with phenoxybenzamine, a successful transperitoneal laparoscopic adrenalectomy was performed in the twentieth week of gestation. The management of pheochromocytoma in pregnancy and the indications for laparoscopic surgery in pregnant patients are discussed.
UI - 12049539
AU - Baghai M; Thompson GB; Young WF Jr; Grant CS; Michels VV; van Heerden JA
TI - Pheochromocytomas and paragangliomas in von Hippel-Lindau disease: a role for laparoscopic and cortical-sparing surgery.
SO - Arch Surg 2002 Jun;137(6):682-8; discussion 688-9
AD - Department of Surgery, Mayo Clinic and Mayo Foundation, 200 First St SW, Rochester, MN 55905, USA.
HYPOTHESIS: Von Hippel-Lindau disease (VHL) is an autosomal-dominant disorder characterized by benign and malignant tumors involving the central nervous system, kidneys, pancreas, adrenal glands, and paraganglia. Appropriate management of pheochromocytomas and paragangliomas in VHL is evolving as we better understand the genetics and natural course of the disease and master advanced surgical techniques for adrenalectomy. DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: A total of 109 patients identified at the Mayo Clinic, Rochester, Minn, with VHL (60 males and 49 females) between January 1, 1975, and June 30, 2000. Seventeen patients (16%) had an identifiable adrenal mass and 3 patients had paragangliomas. Follow-up was complete in all but 2 patients. MAIN OUTCOME MEASURES: Clinical presentation, preoperative evaluation, surgical management, and outcome. RESULTS: Three patients with paragangliomas and 13 of 17 patients with adrenal masses underwent surgical resection. Median age at time of diagnosis was 30 years (range, 16-47 years); 8 (40%) were asymptomatic. Fractionated urinary catecholamine and metanephrine concentrations were normal in one third of patients. Computed tomographic scanning identified 20 (83%) of 24 tumors. Adrenalectomies were performed as unilateral or bilateral, open or laparoscopic, and, finally, total or cortical-sparing. Seven (50%) of the patients underwent other concurrent abdominal procedures. There were no deaths, with an overall operative morbidity of 2 patients (14%). Only the 2 patients in whom bilateral total adrenalectomies were performed became corticosteroid dependent. No recurrences have been noted to date. CONCLUSIONS: A multidisciplinary approach is imperative for proper examination and monitoring of patients with VHL. Evaluation should begin early in life and always before elective surgery and childbirth. All adrenal masses in patients with VHL should be thoroughly evaluated and most should be resected. Early intervention and advanced surgical techniques better allow for cortical-sparing and laparoscopic procedures. With low recurrence rates, corticosteroid independence can be maintained for prolonged periods.
UI - 12093786
AU - Magnano AR; Bai D; Bloomfield DM
TI - Images in cardiovascular medicine. Cyclic tachycardia and hypotension.
SO - Circulation 2002 Jul 2;106(1):e1-2
AD - Department of Medicine, Division of Cardiology, Columbia University College of Physicians and Surgeons, New York, NY 10032, USA.
UI - 12057112
AU - Eigelberger MS; Duh QY
TI - Pheochromocytoma.
SO - Curr Treat Options Oncol 2001 Aug;2(4):321-9
AD - Department of Surgery, University of California, San Francisco/Mt. Zion Medical Center, University of California, Campus Box 1674, San Francisco, CA 94143-1674, USA.
The only definitive therapy for patients with pheochromocytoma is surgical resection [1,2**]. Advances in preoperative medical management of hypertension/hypovolemia and improved intraoperative anesthetic care have reduced the operative mortality rate for pheochromocytoma to less than 5% in most series. In addition, accurate preoperative localization studies have eliminated the need for extensive exploratory laparotomy. Focused approach and laparoscopic resection have become the new "gold standard," with a reduced morbidity [4**]. Large or locally invasive pheochromocytomas may still require open resection.
UI - 11829326
AU - Zelinka T; Widimsky J; Weisserova J
TI - Diminished circadian blood pressure rhythm in patients with asymptomatic normotensive pheochromocytoma.
SO - Physiol Res 2001;50(6):631-4
AD - Third Medical Department, General Faculty Hospital, Charles University, Prague, Czech Republic. firstname.lastname@example.org
In our study, the circadian blood pressure (BP) rhythm was studied in subjects with asymptomatic and normotensive pheochromocytoma. We have therefore performed 24-hour BP monitoring not only in 6 subjects with asymptomatic pheochromocytoma, but also in 33 patients with symptomatic pheochromocytoma and in 10 normotensive subjects, who served as a control group. Circadian BP rhythm was expressed by assessing a relative night-time BP decline. We found a similar BP rhythm, catecholamine excretion and tumor size in subjects with both forms of pheochromocytoma. Subjects with asymptomatic pheochromocytoma had a significantly lower night-time systolic BP decline (P=0.01) and diastolic BP decline (P=0.006) than normotensive controls. We conclude that the attenuated night-time BP decline in normotensive and asymptomatic subjects with pheochromocytoma might be a possible sign of partial desensitization of the cardiovascular system to catecholamines.
UI - 12075713
AU - Niruthisard S; Chatrkaw P; Laornual S; Sunthornyothin S; Prasertsri S
TI - Anesthesia for one-stage bilateral pheochromocytoma resection in a patient with MEN type IIa: attenuation of hypertensive crisis by magnesium sulfate.
SO - J Med Assoc Thai 2002 Jan;85(1):125-30
AD - Department of Anesthesiology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
Multiple endocrine neoplasia (MEN) type IIa, manifesting as an autosomal dominant trait, consists of medullary thyroid carcinoma, parathyroid adenoma or hyperplasia, and pheochromocytoma. We report our experience of a 42-year-old woman, MEN type IIa with a large bilateral pheochromocytoma, who underwent one-stage bilateral tumor resection under a combined continuous epidural technique with 0.25 per cent bupivacaine and general anesthesia using vecuronium, fentanyl, nitrous oxide, and isoflurane. An initial intra-operative hypertensive response was acceptably controlled by nitroprusside and a beta-blocker but during tumor handling the hypertensive crisis worsened and she developed acute pulmonary edema despite a continuing high dose of nitroprusside infusion. After receiving intermittent i.v. MgSO4 up to 3 g in 15 min, her condition gradually improved and the cardiovascular response was under control throughout the period of tumor handling. Hypotension encountered post-pheochromocytoma resection was treated by volume replacement, metaraminol, CaCl2, and dopamine infusion. The patient's post-operative course was uneventful.
UI - 12094419
AU - Brunaud L; Cormier L; Ayav A; Klein M; Roumier X; Zarnegar R; Bresler L;
TI - Boissel P [Does the size of pheochromocytoma influence the results of its laparoscopic excision?]
SO - Ann Chir 2002 May;127(5):362-9
AD - Service de chirurgie digestive, endocrinienne et viscerale, CHU Nancy-Brabois, 11 allee du morvan, 54511 Vandoeuvre les Nancy, France. email@example.com
BACKGROUND: The tumor size is considered as a limitation for laparoscopic adrenalectomy. The aim of this study was to assess diagnostic characteristics, hemodynamic modifications, and outcome of intraperitoneal laparoscopic adrenalectomy according to the size of pathologic study (< or > or = 5 cm). RESULTS: Forty two patients underwent laparoscopic adrenalectomy during this period. Among them, 11 (26%) were operated on for adrenal pheochromocytoma (6 patients < 5 cm and 5 patients > or = 5 cm). Preoperative systolic hypertension was 138 mmHg and 178 mmHg respectively (p = 0.01). Urinary metanephrine/normetanephrine rate was 1.259 and 0.268 respectively (p = 0.08). Capsular tumor effraction rate was 27%. Tumor size was 37% larger than that estimated by CT scan (24% versus 52%). Mean hospital stay was 10 days and 8 days respectively. Morbidity and mortality were 18% and 0%. All these criteria were not statistically significant among the two groups of patients. CONCLUSIONS: Pheochromocytoma size > or = 5 cm do not modify outcome of patients but is responsible for preferential noradrenaline secretion and stronger hemodynamic modifications. Tumor fragmentation rate and CT scan size underestimation seem important in this group of patients. These characteristics have to be integrated to improve laparoscopic adrenalectomy outcome of patients with pheochromocytoma > or = 5 cm.
UI - 12084291
AU - Maranchie JK; Walther MM
TI - Early identification of patients with von Hippel-Lindau disease at risk for pheochromocytoma.
SO - Curr Urol Rep 2001 Feb;2(1):24-30
AD - Urologic Oncology Branch, National Cancer Institute, 10101 Grosvenor Place, #518, North Bethesda, MD 20852, USA. Maranchj@mail.nih.gov
von Hippel-Lindau disease (VHL) is an autosomal dominant familial syndrome that predisposes to the formation of tumors in multiple organ systems, including adrenal and extra-adrenal pheochromocytomas. However, fewer than 30% of VHL families develop pheochromocytomas. In recent years, this clinical heterogeneity has been correlated with missense mutations. The VHL patient requires vigilant, lifelong biochemical and radiographic screening for pheochromocytoma. Half of VHL pheochromocytomas present bilaterally, and there is a high incidence of recurrence after surgery. Because of the morbidity of bilateral total adrenalectomy with subsequent steroid replacement therapy, the recent therapeutic trend has been toward observation and minimally invasive adrenal-sparing procedures.
UI - 12117391
AU - Neumann HP
TI - Imaging vs biochemical testing for pheochromocytoma.
SO - JAMA 2002 Jul 17;288(3):314-5; discussion 315
UI - 11848243
AU - Mishra AK; Agarwal G; Agarwal A; Mishra SK
TI - Cystic phaeochromocytoma presenting as an acute abdomen with shock.
SO - Eur J Surg 2001 Nov;167(11):863-5
AD - Department of Endocrine Surgery, Sanjay Gandhi Institute of Medical Sciences, Lucknow, India.
UI - 11848244
AU - Taniguchi K; Ishizu K; Torizuka T; Hasegawa S; Okawada T; Ozawa T; Iino
TI - K; Taniguchi M; Ikematsu Y; Nishiwaki Y; Kida H; Waki S; Uchimura M Metastases of predominantly dopamine-secreting phaeochromocytoma that did not accumulate meta-iodobenzylguanidine: imaging with whole body positron emission tomography using 18F-labelled deoxyglucose.
SO - Eur J Surg 2001 Nov;167(11):866-70
AD - Department of Surgery, Hamamatsu Medical Center, Shizuoka, Japan. firstname.lastname@example.org
UI - 12016808
AU - Huang J; Zhou Z; Chao C
TI - [Clinical analysis of 17 patients with adrenal "incidentaloma"]
SO - Hunan Yi Ke Da Xue Xue Bao 1999;24(3):282-4
AD - Department of Endocrinology, Second Affiliated Hospital, Hunan Medical University, Changsha 410008.
This paper reports 17 cases of adrenal "incidentaloma", discovered by B-mode ultrasonography, computed tomography and/or magnetic resonance imaging scan and identified by operation and pathological examination. Seven of them were pathologically diagnosed as adrenocortical adenoma, 4 pheochromocytoma, 2 adrenal cyst, and 1 mixed cell adenoma, 1 leiomyoma, 1 adrenocortical carcinoma, and 1 gangliocytoma. Adrenal shows hormone-secreting function in 17 patients with adrenal "incidentaloma". The diagnosis and management of this disorder are discussed.
UI - 12052760
AU - MacGillivray DC; Whalen GF; Malchoff CD; Oppenheim DS; Shichman SJ
TI - Laparoscopic resection of large adrenal tumors.
SO - Ann Surg Oncol 2002 Jun;9(5):480-5
AD - Department of Surgery, Division of Surgical Oncology, Maine Medical Center, Portland, ME 04104, USA. email@example.com
BACKGROUND: The maximum size of adrenal tumors that should be removed with a laparoscopic approach is controversial. It has been suggested that laparoscopic adrenalectomy is appropriate only for adrenal tumors < 6 cm in size. We report our experience with laparoscopic adrenalectomy in patients with adrenal tumors of > or =6 cm compared with patients with smaller tumors. METHODS: We retrospectively reviewed a consecutive series of patients who had a laparoscopic adrenalectomy. Patients were considered candidates for laparoscopic adrenalectomy if their computed tomography (CT) scan showed a well-encapsulated tumor confined to the adrenal gland. RESULTS: Sixty laparoscopic adrenalectomies were performed in 53 patients. Twelve of the adrenalectomies (20%) were for tumors that were > or =6 cm (median, 8 cm; range, 6 to 12 cm). There have been no local or regional recurrences, but one patient with adrenocortical carcinoma developed pulmonary metastases. When the 12 patients with large tumors were compared with the 36 patients with tumors < 6 cm, the median operative time (190 vs. 180 minutes; P =.32), operative blood loss (100 vs. 50 mL; P =.53), and postoperative hospital stay (2 vs. 2 days; P = 1.0) were similar. CONCLUSIONS: The size of an adrenal tumor should not be the primary factor in determining whether a laparoscopic adrenalectomy should be performed. Large adrenal tumors that are confined to the adrenal gland on CT can be removed with a laparoscopic approach.
UI - 11573678
AU - Bullough AS; Watters M
TI - Phaeochromocytoma diagnosed during labour.
SO - Br J Anaesth 2001 Feb;86(2):288; discussion 288-9
UI - 11573679
AU - Fawcett WJ; Edkins CL
TI - Phaeochromocytoma diagnosed during labour.
SO - Br J Anaesth 2001 Feb;86(2):288; discussion 288-9
UI - 11816608
AU - Rado Velazquez MA; Correas Gomez MA; Martin Garcia B; Hernandez
TI - Rodriguez R; Portillo Martin JA; Gutierrez Banos JL; del Valle Schaan JI; Roca Edreira A; Villanueva Pena A; de Diego Rodriguez E; Hernandez Castrillo A [Synchronous renal oncocytoma and asymptomatic pheochromocytoma. Report of a case]
SO - Arch Esp Urol 2001 Oct;54(8):816-9
AD - Servicio de Urologia, Hospital Universitario Marques de Valdecilla, Santander, Cantabria, Espana.
OBJECTIVE: To report a rare case of coexisting renal oncocytoma and pheochromocytoma. To our knowledge, only one such case has been reported in the literature. METHODS: The findings of the imaging studies of this rare case and the unsuspected anatomopathological findings are presented. RESULTS/CONCLUSIONS: Despite its very low frequency, coexisting renal ocnocytoma and pheochromocytoma should be taken into account in patients with synchronous renal and adrenal masses. Unfortunately, there are no radiological patterns that allow us to suspect this rare condition.
UI - 11761690
AU - Khaleq K; Miloudi Y; Idali B; Harti A; Barrou L
TI - [Pheochromocytoma discovered in the preoperative period: analysis of 3 cases]
SO - Prog Urol 2001 Sep;11(4):673-6
AD - Service d'Anesthesie-Reanimation Chirurgicale, CHU Ibn Rochd, Casablanca, Maroc. Khaleq20@caramail.com
Phaeochromocytoma is a rare tumour that may be discovered incidentally during a surgical operation that can sometimes cause fatal arrhythmias. The authors report 3 cases of phaeochromocytoma presenting in the form of hypertensive crisis and serious arrhythmias following manipulation of the tumour with a fatal outcome in 2 cases. These cases illustrate the severity and complexity of the problems encountered and the importance of preoperative detection of phaeochromocytomas, particularly at the pre-anaesthetic visit.
UI - 11774764
AU - Touiti D; Seket B; Deligne E; Badet L; Colombel M; Dawahra M; Martin X;
TI - Marechal JM; Dubernard JM [Bilateral adrenal pheochromocytomas in von Hippel-Lindau disease]
SO - Ann Urol (Paris) 2001 Nov;35(6):323-8
AD - Service d'urologie et de chirurgie de la transplantation, hopital Edouard Herriot, 5, place d'Arsonval, 69437 Lyon, France.
The pheochromocytoma is a medullo-adrenal tumor which develops at the cost of the chromaffin cells. It appears in 11-19% of cases of von Hippel-Lindau's disease (VHL), is often bilateral, and the symptomatology is often crude: arterial hypertension is frequently isolated and unstable, and the classic triad of headache, palpitations and sweating is quite rarely observed. We report four observations of bilateral pheochromocytomas in patients with von Hippel-Lindau's disease (three with phenotype IIA and one with phenotype IIB). The tumor was bilateral during the diagnosis in three cases; in the fourth patient, the attack on the contralateral adrenal gland came two years after the first adrenalectomy. All the patients had undergone an adrenalectomy by open surgery after a short preparation of 48 hours; replacement therapy was begun in each patient. Morbidity was low, and the patients submitted to a prolonged follow-up in order to screen for the onset of future lesions of VHL.
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