• Unusual clinical manifestation of pheochromocytoma in a MEN2A patient.

• [Biochemical diagnosis of pheochromocytoma and neuroblastomas]

• Giant cystic pheochromocytoma located in the renal hilus.

• Phaeochromocytoma and acute myocardial infarction.

• Occurrence of pheochromocytoma in a MEN2A family with codon 609 mutation of the RET proto-oncogene.

• Images in cardiology: Preoperative treatment with phenoxybenzamine restores ECG to normal in a woman with pheochromocytoma.

• The urinary metanephrine-to-creatinine ratio for the diagnosis of pheochromocytoma.

• Plasma or urinary metanephrines for the diagnosis of pheochromocytoma? That is the question.

• A systems approach to surgical safety.

• Phaeochromocytoma diagnosed during labour.

• Paroxysmal hypertension, pheochromocytoma, and pregnancy.

• Sipple's syndrome presenting acutely as severe heart failure.

• von Hippel-Lindau disease complicated by pheochromocytoma.

• 5'-End RET splicing: absence of variants in normal tissues and intron retention in pheochromocytomas.

• From eyelid bumps to thyroid lumps: report of a MEN type IIb family and review of the literature.

• Germ-line mutations in nonsyndromic pheochromocytoma.

• Safe retroperitoneal endoscopic resection of pheochromocytomas.

• [Pheochromocytoma and von Recklinghausen's disease]

• A case of metastatic extra-adrenal pheochromocytoma 12 years after surgery.

• Haemodynamic changes during retroperitoneoscopic adrenalectomy for phaeochromocytoma.

• Retroperitoneoscopic excision of phaeochromocytoma.

• Sequential ACTH and catecholamine secretion in a phaeochromocytoma.

• [Clinical features of pheochromocytoma and anesthetic management during perioperative period]

• [Pheochromocytoma in children]

• How to detect pheochromocytomas?--the diagnostic relevance of plasma free metanephrines.

• Synchronous presentation of primary non-small cell lung carcinoma and pheochromocytoma.

• Adrenal incidentaloma--experience of a standardized diagnostic programme in the Swedish prospective study.

1
UI - 11883866
AU - Guerrieri M; Filipponi S; Arnaldi G; Giovagnetti M; Lezoche E; Mantero
TI - F; Taccaliti A Unusual clinical manifestation of pheochromocytoma in a MEN2A patient.
SO - J Endocrinol Invest 2002 Jan;25(1):53-7
AD - Clinics of General Surgery, University of Ancona, Italy. guerrieri@freefast.it
A case of unusual clinical manifestation of pheochromocytoma in a type 2A multiple endocrine neoplasia (MEN2A) patient is presented. A 27-year-old man affected by MEN2A syndrome, complaining of anxiety and depression, was admitted in our Division. Past medical history included a total thyroidectomy for medullary carcinoma in 1985, and left adrenalectomy for pheochromocytoma in 1994. Blood pressure was 130/ 85 mmHg without orthostatic hypotension and pulse rate was 72 beats/min. Laboratory data revealed thyroid hormones and carcinoembryonic antigen (CEA) in the normal range and high basal serum calcitonin levels (158 pg/ml). Plasma catecholamines and vanillylmandelic acid resulted in normal levels but epinephrine/norepinephrine ratio was elevated (0.65). The glucagon stimulation test showed positive clinical and biochemical response. Magnetic resonance imaging (MRI) and meta-iodobenzylguanidine (MIBG) scintiscan confirmed the presence of bilateral adrenal masses. Bilateral adrenalectomy by laparoscopic anterior approach was performed. Histology was consistent with adrenal pheochromocytomas. After surgical approach, psychiatric findings disappeared and did not recur at follow-up in spite of no medication for two years. In conclusion, bilateral pheochromocytoma is more frequent in MEN2A syndrome and probably understimated if the follow-up is not prolonged. In these cases clinical features are often aspecific and basal hormonal data may be normal in a great number of patients. Therefore long-term observation is justified in these patients. Pheochromocytoma was described as the "great mimic" for the numerous subjective manifestations. Differential diagnosis among typical features of neuropsychiatric disorders and pheochromocytoma must be considered.

2
UI - 11830391
AU - Candito M; Billaud E; Chauffert M; Cottet-Emard JM; Desmoulin D; Garnier
TI - JP; Greffe J; Hirth C; Jacob N; Millot F; Nignan A; Patricot MC; Peyrin L; Plouin PF [Biochemical diagnosis of pheochromocytoma and neuroblastomas]
SO - Ann Biol Clin (Paris) 2002 Jan-Feb;60(1):15-36
AD - Laboratoire de biochimie, Hopital Pasteur, 30, avenue de la Voie-Romaine, BP 69, 06002 Nice cedex 1.
Pheochromocytoma and neuroblastoma are distinct tumours, but their biological diagnosis is based on secretion increase of one or several catecholamines. Assays have to be very sensible and specific for an early diagnosis. 24 hours urinary catecholamines and metabolites are currently measured, but technical improvements permit plasma metanephrine assay, an excellent indicator of pheochromocytoma. HPLC coupled to electrochemical detection represents the most efficient methodology. After a review of urinary and plasma assay methods, the authors show usual values of catecholamines, metanephrines, HVA and VMA, according to ages, and give examples of results encountered in classical or not tumours and in falsely positive cases. Urinary metanephrine assay is the most sensible and specific in biological diagnosis of pheochromocytoma, while catecholamines and VMA assays lack of sensibility. Results have to be given by 24 hours and by creatinine ratio. Metanephrine assay can be performed also in plasma and exhibits the same interest. However, in urine as in plasma, in case of renal failure, results cannot be interpreted. Neuroblastoma biological diagnosis is based classically on HVA, VMA, and dopamine assays, nowadays only in 24 hours urine (or in urinary micturition for screening), and results are also expressed as creatinine ratio. But even if several assays are advisable, 5% of the neuroblastoma cases do not produce increased catecholamine values. In some cases, metanephrine assay could be of interest. After the age of 12 months, clinical expression of neuroblastoma is dramatic in 70% of cases. So, a biological screening has been experimented in several countries including France. A French translation of the consensus conference report (1998) is appended, which shows the complexity of neuroblastoma screening. Now, there is no evidence that early tumour detection by screening lessens the mortality rate, but a weak benefit is not excluded.

3
UI - 11928862
AU - Melegh Z; Renyi-Vamos F; Tanyay Z; Koves I; Orosz Z
TI - Giant cystic pheochromocytoma located in the renal hilus.
SO - Pathol Res Pract 2002;198(2):103-6; discussion 107-8
AD - Department of Molecular Pathology, National Institute of Oncology, Budapest, Hungary.
A malignant tumor in the past medical history of a patient often makes the differential diagnosis of a second tumor more difficult, especially if one of the tumors does not show its characteristic features. The authors report a case of a 55-year-old male who presented with a malignant melanoma on his left shoulder. A retroperitoneal giant cystic mass, 200 mm in diameter, was found incidentally. Adrenal origin was ruled out by imaging techniques. The absence of typical clinical symptoms made a correct preoperative diagnosis unlikely, and severe cardiovascular complications set in during surgery. Considering the characteristics of the cutaneous malignant melanoma, the metastatic origin of the giant retroperitoneal tumor was not likely either. During surgery the left kidney, with a cystic tumor located in the hilus, was removed. The postoperative pathologic diagnosis was pheochromocytoma located in the hilus of the left kidney.

4
UI - 11939446
AU - Biccard BM; Gopalan PD
TI - Phaeochromocytoma and acute myocardial infarction.
SO - Anaesth Intensive Care 2002 Feb;30(1):74-6
AD - Department of Anaesthetics, University of Natal, Congella, South Africa.
Phaeochromocytoma is uncommonly associated with myocardial infarction. We present a patient who, despite established alpha adrenoceptor blockade, sustained an acute myocardial infarction and was found to have coronary artery disease. Indications for coronary revascularization were not met, and adrenalectomy was successfully performed four weeks later. Factors contributing to the myocardial infarction, the role of beta adrenoceptor blockade, the timing of adrenalectomy and the place of coronary revascularization are discussed.

5
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

6
UI - 12117853
AU - Tsioufis CP; Stefanadis CI; Toutouzas PK
TI - Images in cardiology: Preoperative treatment with phenoxybenzamine restores ECG to normal in a woman with pheochromocytoma.
SO - Heart 2002 Aug;88(2):186
AD - tsioufis@otenet.gr

7
UI - 8678394
AU - Heron E; Chatellier G; Billaud E; Foos E; Plouin PF
TI - The urinary metanephrine-to-creatinine ratio for the diagnosis of pheochromocytoma.
SO - Ann Intern Med 1996 Aug 15;125(4):300-3
AD - Hopital Broussais, Paris, France.
OBJECTIVE: To compare the operating characteristics of two tests for diagnosing pheochromocytoma: 1) measurement of the ratio between urinary metanephrine and creatinine levels and 2) measurement of urinary metanephrine levels alone. A second objective was to ascertain the reasons for false-positive test results. DESIGN: Cross-sectional study. SETTING: Hypertension referral center. PATIENTS: 1013 patients referred for hypertension and tested for pheochromocytoma. MEASUREMENTS: 24-hour urinary levels of metanephrine (measured using liquid chromatography) and creatinine. The presence of pheochromocytoma was confirmed at surgery. In patients with positive test results, the absence of pheochromocytoma was documented by negative results of retests and imaging procedures. RESULTS: Of 58 patients with increased metanephrine levels or increased metanephrine-to-creatinine ratios, 20 had pheochromocytoma and 38 did not. Of the 38 patients without pheochromocytoma, 15 had high metanephrine levels but normal metanephrine-to-creatinine ratios. The respective operating characteristics of measurement of urinary metanephrine levels and measurement of the metanephrine-to-creatinine ratio were as follows: sensitivity, 95% and 100%; specificity, 98% and 98%; positive predictive value, 46% and 47%; and negative predictive value, 100% and 100%. In 13 of the 23 patients who had a high metanephrine-to-creatinine ratio, various acute events may have caused hypersecretion of catecholamines. CONCLUSIONS: Measurement of the metanephrine-to-creatinine ratio is a sensitive and specific test for pheochromocytoma. However, acute events may increase urinary metanephrine excretion to the level that occurs with tumors.

8
UI - 8678398
AU - Bravo EL
TI - Plasma or urinary metanephrines for the diagnosis of pheochromocytoma? That is the question.
SO - Ann Intern Med 1996 Aug 15;125(4):331-2

9
UI - 12000985
AU - Calland JF; Guerlain S; Adams RB; Tribble CG; Foley E; Chekan EG
TI - A systems approach to surgical safety.
SO - Surg Endosc 2002 Jun;16(6):1005-14; discussion 1015
AD - Surgical Technology and Safety Laboratory, University of Virginia, Post Office Box 800709, Charlottesville, VA 22908-0709, USA.

10
UI - 11064629
AU - Strachan AN; Claydon P; Caunt JA
TI - Phaeochromocytoma diagnosed during labour.
SO - Br J Anaesth 2000 Oct;85(4):635-7
AD - Department of Anaesthesia, Northern General Hospital, Sheffield, UK.
The diagnosis of phaeochromocytoma during pregnancy is rare. We present the management of vaginal delivery in a woman diagnosed with the condition during labour. A Medline search and follow-up of references failed to find any similar report in the last 30 years.

11
UI - 12002199
AU - Lyman DJ
TI - Paroxysmal hypertension, pheochromocytoma, and pregnancy.
SO - J Am Board Fam Pract 2002 Mar-Apr;15(2):153-8
AD - Department of Family Medicine, University of Tennessee, Jackson, USA.
BACKGROUND: Hypertension is the most common medical complication of pregnancy. Pheochromocytoma in pregnancy is rare, and if unrecognized, can cause serious perinatal morbidity and mortality. METHODS: A patient with severe hypertension, postpartum pulmonary edema, and a recognized pheochromocytoma is described. RESULTS: Abdominal palpation after vaginal childbirth reproduced the diagnostic triad of hypertension, headaches, and palpitations. Magnetic resonance imaging established the correct diagnosis before biochemical confirmation of excess catecholamine production. The patient responded to alpha-adrenergic receptor blockade with control of her severe hypertension and clearing of pulmonary edema. The best time to diagnose a pheochromocytoma is before delivery because vaginal childbirth stimulates the release of lethal amounts of catecholamines. CONCLUSIONS: The physician who delivers babies must distinguish between labile hypertension and paroxysmal hypertension. Most experts believe that a spontaneous vaginal delivery is contraindicated when the patient has a pheochromocytoma. Postpartum pulmonary edema associated with a pheochromocytoma is unusual. The profound pressor response elicited by palpation of the postpartum abdomen, the failure of medications usually effective in the treatment of a hypertensive crisis, and the use of magnetic resonance imaging to confirm a functioning adrenal adenoma are the features unique to this case.

12
UI - 12131643
AU - Gomez Ramos MJ; Gonzalez Valverde FM; Sanchez Alvarez MC
TI - Sipple's syndrome presenting acutely as severe heart failure.
SO - Eur J Emerg Med 2002 Jun;9(2):171-4
AD - Department of Intensive Care, Hospital General Universitario, Servicio De Medicina Intensiva, Pza. Cruz Roja, 1, 30.003, Murcia, Spain.
We describe the case of a 56-year-old man who developed acute hypertension leading to cardiac insufficiency, arrhythmia, severe heart failure and death. The autopsy revealed Sipple's syndrome (multiple endocrine neoplasia syndrome type IIa) and catecholamine-induced cardiomyopathy. This man had received a false diagnosis of primary hypertension 1 year before. The prime objective of this report is to call attention to the necessity of an in depth diagnosis of labile and paroxysmal hypertension. The clinical diagnostic features of phaeochromocytoma as well as the main therapeutic approaches suggested in the literature are commented on.

13
UI - 12151681
AU - Kashyap AS; Kashyap S
TI - von Hippel-Lindau disease complicated by pheochromocytoma.
SO - Postgrad Med J 2002 Jul;78(921):443

14
UI - 12187076
AU - Le Hir H; Charlet-Berguerand N; de Franciscis V; Thermes C
TI - 5'-End RET splicing: absence of variants in normal tissues and intron retention in pheochromocytomas.
SO - Oncology 2002;63(1):84-91
AD - Centre de Genetique Moleculaire, Laboratoire associe a l'Universite Pierre et Marie Curie, Gif-sur-Yvette, France.
The Ret tyrosine kinase is implicated in neuronal cell survival, kidney development and tumorigenesis. Several 3' and 5' transcript variants have been described resulting from alternative splicing of the RET pre-mRNA. The 3' variants code for three C-terminal isoforms, RET51, RET9 and RET43. The 5' variants RET2/4, RET2/5 and RET2/6 result from skipping exons 3, 3-4 and 3-5, respectively. These variants code for putative Ret proteins differing in their extracellular ligand-binding domains, and their expression is strongly regulated during kidney development. Here we analyzed the presence of these RET 5' variants in normal tissues and in MEN2 and sporadic pheochromocytomas. In all tissues examined, the abundance of these transcripts remained extremely low (less than 1% of all RET transcripts) thus indicating these species as rare variants with little biological meaning. On the other hand, in tumors, the 5' RET splicing pattern differed from that of normal tissues. Indeed, we identified a RET-derived transcript that results from the aberrant retention of intron 2. This transcript is enriched in tumor samples of both familial and sporadic origin, and indicates RET as a target for RNA splicing deregulation in tumor cells. Copyright 2002 S. Karger AG, Basel

15
UI - 11388386
AU - Jacobs JM; Hawes MJ
TI - From eyelid bumps to thyroid lumps: report of a MEN type IIb family and review of the literature.
SO - Ophthal Plast Reconstr Surg 2001 May;17(3):195-201
AD - Department of Ophthalmology, University of Colorado Health Sciences Center, Denver, USA.
PURPOSE: We present a two-generation family with multiple endocrine neoplasia (MEN) type IIb diagnosed by their ophthalmologists based on characteristic ophthalmic findings. METHODS: A family consisting of a 33-year-old female proband and her 8- and 7-year-old children had prominent corneal nerves; eyelid, lip, and tongue nodules; and a characteristic facies. A polymerase chain reaction-based genetic assay was obtained to detect the genetic mutation most commonly associated with MEN type IIb. Serum calcitonin and urine catecholamine studies were obtained. RESULTS: Molecular genetic studies detected in all 3 patients a mutation at codon 918 of the RET proto-oncogene known to be present in 95% of the cases of MEN type IIb. Serum calcitonin was elevated in the proband and her son. Urine catecholamine levels were elevated in the proband. Surgical treatment and histologic analysis confirmed pheochromocytoma and medullary thyroid carcinoma (MTC) in the proband. Surgical exploration revealed the MTC to be metastatic to the liver. CONCLUSIONS: This family demonstrates the characteristic findings of MEN type IIb: prominent corneal nerves in a clear stroma and multiple submucosal neuromas of the conjunctiva, eyelids, lips, and tongue. Ophthalmologists have a critical role to play in recognizing these signs, because the early diagnosis of medullary thyroid carcinoma and pheochromocytoma may be life saving.

16
UI - 12000816
AU - Neumann HP; Bausch B; McWhinney SR; Bender BU; Gimm O; Franke G;
TI - Schipper J; Klisch J; Altehoefer C; Zerres K; Januszewicz A; Eng C; Smith WM; Munk R; Manz T; Glaesker S; Apel TW; Treier M; Reineke M; Walz MK; Hoang-Vu C; Brauckhoff M; Klein-Franke A; Klose P; Schmidt H; Maier-Woelfle M; Peczkowska M; Szmigielski C; Eng C; The Freiburg-Warsaw-Columbus Pheochromocytoma Study Group Germ-line mutations in nonsyndromic pheochromocytoma.
SO - N Engl J Med 2002 May 9;346(19):1459-66
AD - Department of Nephrology and Hypertension, Albert Ludwigs University, Freiburg, Germany. neumann@mm41.ukl.uni-freiburg.de
BACKGROUND: The group of susceptibility genes for pheochromocytoma that included the proto-oncogene RET (associated with multiple endocrine neoplasia type 2 [MEN-2]) and the tumor-suppressor gene VHL (associated with von Hippel-Lindau disease) now also encompasses the newly identified genes for succinate dehydrogenase subunit D (SDHD) and succinate dehydrogenase subunit B (SDHB), which predispose carriers to pheochromocytomas and glomus tumors. We used molecular tools to classify a large cohort of patients with pheochromocytoma with respect to the presence or absence of mutations of one of these four genes and to investigate the relevance of genetic analyses to clinical practice. METHODS: Peripheral blood from unrelated, consenting registry patients with pheochromocytoma was tested for mutations of RET, VHL, SDHD, and SDHB. Clinical data at first presentation and follow-up were evaluated. RESULTS: Among 271 patients who presented with nonsyndromic pheochromocytoma and without a family history of the disease, 66 (24 percent) were found to have mutations (mean age, 25 years; 32 men and 34 women). Of these 66, 30 had mutations of VHL, 13 of RET, 11 of SDHD, and 12 of SDHB. Younger age, multifocal tumors, and extraadrenal tumors were significantly associated with the presence of a mutation. However, among the 66 patients who were positive for mutations, only 21 had multifocal pheochromocytoma. Twenty-three (35 percent) presented after the age of 30 years, and 17 (8 percent) after the age of 40. Sixty-one (92 percent) of the patients with mutations were identified solely by molecular testing of VHL, RET, SDHD, and SDHB; these patients had no associated signs and symptoms at presentation. CONCLUSIONS: Almost one fourth of patients with apparently sporadic pheochromocytoma may be carriers of mutations; routine analysis for mutations of RET, VHL, SDHD, and SDHB is indicated to identify pheochromocytoma-associated syndromes that would otherwise be missed.

17
UI - 12098038
AU - Berends FJ; Harst EV; Giraudo G; Terkivatan T; Kazemier G; Bruining HA;
TI - De Herder WW; Bonjer HJ Safe retroperitoneal endoscopic resection of pheochromocytomas.
SO - World J Surg 2002 May;26(5):527-31
AD - Department of Surgery, University Hospital Dijkzigt, Dr Molewaterplein 40, 3015 GD Rotterdam, The Netherlands.
Although endoscopic adrenalectomy is advocated for small adrenocortical tumors, questions remain about the safety of endoscopic retroperitoneal resection of pheochromocytomas. In this study we evaluated the outcome of retroperitoneal endoscopic adrenalectomy for pheochromocytoma. endoscopic adrenalectomies for a pheochromocytoma or paraganglioma. All patients received adequate alpha-adrenergic blockade. The adrenal vein was ligated at the end of the procedure. Operative blood pressure values were recorded and evaluated. Altogether 15 patients (11 women, 4 men; mean age 47.2 years) were operated on for 17 pheochromocytomas and 1 extraadrenal tumor (4 right, 11 left, 3 bilateral). One female patient was operated on at 13 weeks' gestation. Hypertensive episodes at operation were seen in 4 (26.7%) patients, and tachycardia occurred in 5 (33%). Hemodynamic changes could be corrected in all cases using simple measures without morbidity or detrimental effects. The mean operating time was 125 minutes (80-180 minutes), and the conversion rate was 5.6% (1/18). The median hospital stay was 5 days (3-28 days). Morbidity was 20% (3/15). Endoscopic retroperitoneal adrenalectomy for pheochromocytoma is safe and effective, and it is associated with limited morbidity.

18
UI - 12162190
AU - Rabii R; Fekak H; Moufid K; Joual A; Bennani S; el Mrini M; Benjelloun S
TI - [Pheochromocytoma and von Recklinghausen's disease]
SO - Ann Urol (Paris) 2002 Jul;36(4):254-7
AD - Service d'urologie, CHU Ibn Rochd, Casablanca, Maroc. rrabii@urologues.com
The association between von Recklinghausen's disease and pheochromocytoma is present about 10% of cases. We report a case of 49 years old women who presented with elevated blood pressure and von Recklinghausen's neurofibromatosis. Laboratory examination showed a marked level in the urinary excretion of cathecholamine. The computed tomography showed a right adrenal tumor suggesting a pheochromocytoma. The adrenalectomy was realised by transabdominal approach and the histological examination confirmed a benign pheochromocytoma. The authors discuss the pathogenetic hypothesis of this rare pathological association, the diagnostic methods and the therapeutic procedure.

19
UI - 11924720
AU - Mori S; Okura T; Kitami Y; Takata Y; Nakamura M; Watanabe S; Iwata T;
TI - Hiwada K A case of metastatic extra-adrenal pheochromocytoma 12 years after surgery.
SO - Hypertens Res 2002 Jan;25(1):141-4
AD - Second Department of Internal Medicine, Ehime University School of Medicine, Japan.
At the age of 53, a 65-year-old man had been diagnosed with extra-adrenal pheochromocytoma in the retroperitoneum and underwent total tumorectomy. Afterward, he had his serum catecholamine years after surgery, he complained of lower left abdominal pain. Computed tomography and magnetic resonance imaging revealed an osteolytic lesion in thoracic vertebrae 11Th (Th 11). Although his basal serum and urine catecholamines were at normal levels, glucagon injection increased blood pressure and plasma catecholamine levels. 131I-metaiodobenzylguanidine (MIBG) scintigraphy was specifically taken up to Th 11. By bone biopsy, the osteolytic lesion in Th 11 was finally diagnosed with metastasis of pheochromocytoma. For post-operative pheochromocytoma, long-term follow-up involving biochemical tests, including serum catecholamines, and MIBG is needed.

20
UI - 11575354
AU - Atallah F; Bastide-Heulin T; Soulie M; Crouzil F; Galiana A; Samii K;
TI - Virenque C Haemodynamic changes during retroperitoneoscopic adrenalectomy for phaeochromocytoma.
SO - Br J Anaesth 2001 May;86(5):731-3
AD - Department of Anaesthesiology, Rangueil Hospital, Toulouse University Hospitals, France.
Surgical removal of phaeochromocytoma may be accompanied by acute cardiovascular changes. We report the haemodynamic changes in seven patients with retroperitoneal laparoscopic adrenalectomy for phaeochromocytoma. Transient hypertension (systolic pressure (SBP) > 160 mm Hg) was observed in all patients during manipulation of the tumour, in two patients during pneumoretroperitoneum insufflation, and in one patient during intubation. Small doses of nicardipine were sufficient to control these episodes of hypertension. Transient hypotension (SBP < 100 mm Hg) was observed in two patients during exsufflation and in one patient during repositioning to the lateral position. Our observations suggest that this approach provides relative haemodynamic stability, especially during pneumoretroperitoneum insufflation.

21
UI - 12173237
AU - Subramaniam R
TI - Retroperitoneoscopic excision of phaeochromocytoma.
SO - Br J Anaesth 2002 Jul;89(1):188; discussion 188

22
UI - 12153741
AU - van Dam PS; van Gils A; Canninga-van Dijk MR; de Koning EJ; Hofland LJ;
TI - de Herder WW Sequential ACTH and catecholamine secretion in a phaeochromocytoma.
SO - Eur J Endocrinol 2002 Aug;147(2):201-6
AD - Department of Clinical Endocrinology, University Medical Center, Utrecht, The Netherlands. P.S.vanDam@digd.azu.nl
OBJECTIVE: We describe a patient with an ACTH-producing phaeochromocytoma who initially presented with hypercortisolism and normal catecholamine concentrations, followed by near-normalisation of ACTH secretion and massive catecholamine secretion. In vitro studies were carried out on the tumour to evaluate the interaction between the tumour cells and normal adrenal cortex. METHODS AND RESULTS: A 30-year-old man initially presented with severe hypercortisolism, biochemical evidence of ectopic ACTH production, a tumour in the right adrenal gland without a hyperintense signal on the T2-weighted images at magnetic resonance imaging (MRI) scanning, and normal urinary metanephrine concentrations. After 6 months, ACTH production had almost completely resolved, but the patient developed severe hypertension and excess catecholamines. At repeated MRI-scanning, the T2-weighted images showed a hyperintense signal, in agreement with the diagnosis of phaeochromocytoma. Although the initial T1-weighted images suggested bleeding in the adrenal tumour, no signs of bleeding were observed after surgical removal. The diagnosis of ACTH-producing phaeochromocytoma was histologically and immunohistochemically confirmed. Cultured cell suspensions of the tumour secreted ACTH, which stimulated cortisol production in the ipsilateral adrenocortical cells. CONCLUSION: This case demonstrates that the biological activity of an ACTH-producing phaeochromocytoma can vary significantly in time, which may be the consequence of different stages of tumour differentiation.

23
UI - 12133496
AU - Guo X; Luo A; Huang Y; Ren H; Ye T
TI - [Clinical features of pheochromocytoma and anesthetic management during perioperative period]
SO - Zhonghua Yi Xue Za Zhi 2002 Apr 25;82(8):523-6
AD - Department of Anesthesia, Peking Union Medical College Hospital, Beijing 100730, China.
OBJECTIVE: To investigate the clinical features of pheochromocytoma and summarize the experience of anesthetic management during perioperative period. METHODS: Two hundred and fifty eight medical records of patients who were diagnosed as pheochromocytoma in Peking Union Medical College Hospital were reviewed retrospectively for clinical features, anesthetic management and perioperative mortality. RESULTS: About 5.8% (15/258) of pheochromocytomas was an integral part of multiple endocrine neoplasia (MEN) type II or mixed type. Sixty percent (149/249) of the patients undergoing surgery possessed evidence of catecholamine cardiac toxicity preoperatively, including abnormal ECG, myocardial hypertrophy and decreased left ventricular ejective fraction. Impaired glucose tolerance was found in 59% (147/249) of patients before surgery. The volume infused during operation was significantly higher both in the epidural anesthesia group (3 474 ml +/- 624 ml, q(1) = 5.72, P < 0.01) and in the epidural plus general anesthesia group (3 654 ml +/- 475 ml, q(2) = 5.83, P < 0.01) than that in the general anesthesia group (2 534 ml +/- 512 ml). There were favorable hemodynamic characteristics before removal of the tumor in the epidural anesthesia group and epidural plus general anesthesia group, as compared with in the general anesthesia group. Perioperative mortality was significantly decreased from 8% (5/60) in period 1 (from 1955 to 1975) to 1.2% (1/75) in period 2 (from 1976 to 1994) (chi(2) = 4.05, P < 0.01). No perioperative death (0/111) occurred in period 3 (from 1995 to 2001). CONCLUSION: A good surgical outcome for the excision of pheochromocytoma depends on multiple factors, including careful assessment of potential end organ damages and restoration of blood volume by establishing alpha-blockade during the preoperative period, meticulous anesthetic management during surgery, and appropriate circulatory support after surgery.

24
UI - 11907949
AU - Hoeffel JC; Galloy MA; Hoeffel C; Mainard L
TI - [Pheochromocytoma in children]
SO - Ann Med Interne (Paris) 2001 Oct;152(6):363-70
AD - Service de Radiologie, Hopital d'Enfants, 5, allee du Morvan, 54511 Vandoeuvre-les-Nancy.
Pheochromocytoma is rare in children. A wide varieties of lesions are observed and diagnosis, often made late, is based on urinary catecholamine assay. Magnetic resonance imaging provides the best morphological information. The disease is usually benign and prognosis is good. Familial forms of pheochromocytoma and pheochromocytoma associated with ischemia-induced bone lesions must be emphasized.

25
UI - 12089859
AU - Roden M
TI - How to detect pheochromocytomas?--the diagnostic relevance of plasma free metanephrines.
SO - Wien Klin Wochenschr 2002 Apr 15;114(7):246-51
AD - Division of Endocrinology and Metabolism, Department of Internal Medicine III, University of Vienna Medical School, Vienna, Austria. michael.roden@akh-wien.ac.at
Pheochromocytomas are chromaffin cell tumors, mostly originating from the adrenal medulla, and represent a rare cause of hypertensiondue to excessive production of catecholamines (norepinephrine and/or epinephrine). More than 10% occur in families with multiple endocrine neoplasia type II, von Hippel-Lindau disease, neurofibromatosis type I, and familial carotid body tumors. Since approximately half of the afflicted patients present without or with only episodic hypertension, detailed clinical evaluation and sensitive biochemical tests are mandatory for the diagnosis, which relies on the detection of increased catecholamine production. Commonly employed tests such as the measurement of free catecholamines in plasma and urine or of their metabolites, vanillylmandelic acid and total metanephrines (= free + conjugated normetanephrine and metanephrine) in urine, suffer from interference from external factors and sometimes low clinical sensitivity and/or specificity. Recent technical advances now allow us to measure plasma free (unconjugated) metanephrines, thus increasing clinical sensitivity and specificity to close to 100%. Plasma free metanephrines offer the following advantages for the detection of pheochromocytomas: (i) independence of short-term changes in catecholamine secretion which may result from change of posture, exercise or intraoperative stress, (ii) information on long-term increase of catecholamine production, (iii) tight correlation with tumor mass, and (iv) only minor interference from drugs. This method does not need time-consuming standardized procedures for blood sampling, which are a prerequisite for the determination of free catecholamines. In conclusion, it is therefore recommended to use plasma free metanephrines--after meticulous clinical screening--as the first-line biochemical test for detecting pheochromocytomas.

26
UI - 12238870
AU - Chen EP; Weber CJ; Smith CD; Miller JI Jr
TI - Synchronous presentation of primary non-small cell lung carcinoma and pheochromocytoma.
SO - Ann Thorac Surg 2002 Sep;74(3):924-6
AD - Division of Cardiothoracic Surgery and General Surgery, Emory University Affiliated Hospitals, Atlanta, Georgia, USA.
A 54-year-old man was diagnosed with a 3.2-cm primary right lower lobe non-small cell lung cancer and a synchronously present 2.5-cm right adrenal gland mass. Workup of the adrenal lesion revealed a pheochromocytoma. The patient underwent combined mediastinoscopy, laparoscopic right adrenalectomy, and right thoracotomy with right lower lobectomy. This case illustrates the importance of establishing a firm diagnosis for all synchronously present adrenal lesions that are discovered in the setting of primary lung carcinoma.

27
UI - 12270004
AU - Bulow B; Ahren B; The Swedish Research Council Study Group of Endocrine
TI - Abdominal Tumours Adrenal incidentaloma--experience of a standardized diagnostic programme in the Swedish prospective study.
SO - J Intern Med 2002 Sep;252(3):239-46
AD - Department of Medicine, Lund University, Lund, Sweden. b.bulow@telia.com
OBJECTIVE: To report the 5-year experience of a diagnostic programme for adrenal incidentaloma with special emphasis to diagnose hormonally active and malignant lesions. DESIGN: A prospective study in which new cases of adrenal incidentalomas in Sweden have been evaluated by a SETTING: Thirty-three different Swedish hospitals have contributed with cases. SUBJECTS: A total of 381 patients (217 females, 164 males) with adrenal incidentalomas were studied. INTERVENTIONS: Diagnostic procedures were undertaken according to a standardized programme. Operation was recommended if the incidentaloma had a size of more than 3-4 cm or if there was a suspicion of a hypersecreting tumour. MAIN OUTCOME MEASURES: The size of the incidentaloma, clinical characteristics of the patients and results of biochemical diagnostic tests were registered. RESULTS: The median age of the patients was 64 years (18-84 years), and the median size of the incidentalomas was 3 cm (1-20 cm). A total of 85(22%) patients were operated. Twenty of these patients were diagnosed with a benign hypersecreting tumour and 14 with a malignant tumour. Fourteen of 15 operated patients with diagnosed pheochromocytoma had elevated 24-h urinary noradrenaline and all of the patients operated because of a biochemical suspicion of aldosterone or cortisol hypersecretion (n = 6) were found to have adrenal adenomas. Of the 14 operated patients with malignant diseases, 10 were adrenal carcinomas (median size 10 cm; range 4-16 cm). In a multiple logistic regression model, incidentaloma size was significantly associated with the risk of a malignant tumour (P = 0.009), and there was a tendency of an association between age/male sex and the risk of a malignancy (both, P = 0.07). CONCLUSION: In this Swedish multicentre study of 381 cases with adrenal incidentalomas, 5% had benign hypersecreting tumours and nearly 4% had malignant tumours. The results of the biochemical diagnostic tests used had a high compatibility with the histological diagnosis found at operation in the patients with hypersecreting tumours. Tumour size, male gender and high age were predictive for the risk of a malignant tumour. A follow-up of the patients is warranted in order to establish whether there are undiscovered cases of malignant or hypersecreting tumours amongst the nonoperated patients.

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