• [Pheochromocytoma--rare and often undiagnosed condition]

• Neurofibromin and NF1 gene analysis in composite pheochromocytoma and tumors associated with von Recklinghausen's disease.

• Germ-line mutations in nonsyndromic pheochromocytoma.

• Utility of plasma free metanephrines for detecting childhood pheochromocytoma.

• Pheochromocytoma multisystem crisis in a patient with multiple endocrine neoplasia type IIB and pyelonephritis.

• [Case of pheochromocytoma with metastases into the lung and mediastinum]

• Ectopic adrenocorticotropic hormone production by a noncatecholamine secreting pheochromocytoma.

• [Changes of blood volume and blood propofol concentration during the anesthetic management of a pheochromocytoma patient]

• [Use of magnesium sulfate during resection of pheochromocytoma]

• [Low-symptomatic pheochromocytoma]

• Minimally invasive adrenalectomy for pheochromocytoma during pregnancy.

• Laparoscopic adrenalectomy for pheochromocytoma: morbidity compared with adrenalectomy for tumors of other pathology.

• [Hypertension emergency during surgery of renal carcinoma associated with non-diagnosed pheochromocytoma]

• Hepatic 'pseudotumours': right upper quadrant masses mimicking intrahepatic tumours.

• Choice of biochemical test for diagnosis of pheochromocytoma: validation of plasma metanephrines.

• Alterations of the SDHD gene locus in midgut carcinoids, Merkel cell carcinomas, pheochromocytomas, and abdominal paragangliomas.

1
UI - 12025214
AU - Manhem P; Wahrenberg H
TI - [Pheochromocytoma--rare and often undiagnosed condition]
SO - Lakartidningen 2002 Mar 19;99(14):1585-9
AD - Endokrinologiska kliniken, Universitetssjukhuset MAS, Malmo.
Phaeochromocytoma is a rare but important cause of secondary hypertension that can prove fatal when undiagnosed. Several autopsy studies have reported that a large number of undiagnosed tumours occur during life. This review discusses the diagnostic guidelines for phaeochromocytoma. Factors such as hypertension, sustained or paroxysmal, in association with the most common symptoms of headache, sweating and palpitations, can be used to provide a suspicion of phaeochromocytoma. Although measures of plasma methoxylated catecholamines may provide a promising diagnostic method, the cornerstone for diagnosing phaeochromocytoma remains 24 h or overnight sampling of urinary free catecholamines and methoxylated catecholamines.

2
UI - 11904334
AU - Kimura N; Watanabe T; Fukase M; Wakita A; Noshiro T; Kimura I
TI - Neurofibromin and NF1 gene analysis in composite pheochromocytoma and tumors associated with von Recklinghausen's disease.
SO - Mod Pathol 2002 Mar;15(3):183-8
AD - Department of Pathology and Laboratory Medicine, Tohoku Rosai Hospital, Sendai, Japan. nkimura-path@tohokuh.rofuku.go.jp
Composite tumor of pheochromocytoma and neuroblastoma, or ganglioneuroma, or ganglioneuroblastoma (composite pheochromocytoma), also known as mixed neuroendocrine and neural tumor, are sometimes combined with neurofibromatosis type 1 (NF1). To better understand the relationship between NF1 and composite pheochromocytoma, an immunohistochemical study using anti-neuro-fibromin that is an NF1 gene product and DNA sequence of NF1 Exon 31 were carried out in five cases of composite pheochromocytoma and in various tumors from five patients with NF1. Neurofibromin was not expressed in Schwann cells and sustentacular cells of composite pheochromocytomas and was very weakly or negatively expressed in neurofibroma of NF1 patients. However, it was strongly expressed in ganglionic cells and pheochromocytoma cells of the composite pheochromocytomas and also in mucosal ganglioneuromas, a gangliocytic paraganglioma, and in pheochromocytomas from the patients with NF1. Although there was no mutation in NF1 Exon 31, it could not be ruled out that there were mutations in other sites of the NF1 gene. Neurofibromin insufficiency may induce abnormal proliferation of Schwann cells in composite pheochromocytomas as well as in neurofibromatosis.

3
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.

4
UI - 11994324
AU - Weise M; Merke DP; Pacak K; Walther MM; Eisenhofer G
TI - Utility of plasma free metanephrines for detecting childhood pheochromocytoma.
SO - J Clin Endocrinol Metab 2002 May;87(5):1955-60
AD - Developmental Endocrinology Branch, National Institute of Child Health and Human Development, Bethesda, Maryland 20892, USA.
Measurements of plasma free metanephrines, normetanephrine (NMN) and metanephrine (MN), provide a sensitive test for diagnosis of pheochromocytoma in adults but have not been evaluated in children. We therefore established reference ranges for plasma and urinary metanephrines and the catecholamines, norepinephrine (NE) and epinephrine (E), in 86 healthy children (age 5-17). A group of 158 healthy adults (age 18-72) served as a comparison group. Pediatric reference ranges were applied to examine the diagnostic utility of the various tests in 45 children evaluated for pheochromocytoma (age 8-17; 38 with von Hippel-Lindau syndrome), with tumors found on 12 occasions. Upper reference limits for E and MN were higher and those for NE and NMN lower in children than in adults. Boys had higher plasma levels of E and MN and higher urinary excretion of all four amines than girls. Plasma free metanephrines provided a diagnostic test with values for sensitivity (100%) and specificity (94%) that were equal to or higher than those of other tests. In two children screened for pheochromocytoma on multiple occasions, use of pediatric reference ranges for plasma free metanephrines indicated the tumor a year earlier than indicated using adult reference ranges. The findings indicate that plasma free metanephrines provide a sensitive tool for detection of pheochromocytoma in children. Age appropriate reference ranges should be used and gender differences should be considered.

5
UI - 12046054
AU - Caputo C; Fishbane S; Shapiro L; Courgi RG; Kostadinov S; Donovan V;
TI - Epstein D Pheochromocytoma multisystem crisis in a patient with multiple endocrine neoplasia type IIB and pyelonephritis.
SO - Am J Kidney Dis 2002 Jun;39(6):E23
AD - Division of Nephrology, and Department of Pathology, Winthrop-University Hospital, Mineola, NY, USA.
A patient with pyelonephritis developed multiorgan failure resulting in death. Clinical findings were consistent with multiple endocrine neoplasia type II, with bilateral pheochromocytomas identified by computed tomography scan. We hypothesize that either the infection or the administration of radiocontrast media led to a massive release of catecholamines from the pheochromocytomas. As a result, tissue perfusion was severely compromised, and multiorgan failure developed. This exceedingly rare complication of pheochromocytoma has been termed pheochromocytoma multisystem crisis. Copyright 2002 by the National Kidney Foundation, Inc.

6
UI - 11767396
AU - Kobeleva GV; Kopylova IF
TI - [Case of pheochromocytoma with metastases into the lung and mediastinum]
SO - Probl Tuberk 2001;(8):53-4

7
UI - 11992070
AU - Alvarez P; Isidro L; Gonzalez-Martin M; Loidi L; Arnal F; Cordido F
TI - Ectopic adrenocorticotropic hormone production by a noncatecholamine secreting pheochromocytoma.
SO - J Urol 2002 Jun;167(6):2514-5
AD - Departments of Endocrinology, Urology and Pathology, Hospital Juan Canalejo, Universidad La Coruna, La Coruna, Spain.

8
UI - 12058430
AU - Kimura M; Nakagawa I; Hamada H; Uesugi F; Sakai A
TI - [Changes of blood volume and blood propofol concentration during the anesthetic management of a pheochromocytoma patient]
SO - Masui 2002 May;51(5):489-92
AD - Department of Anesthesia, Hiroshima Municipal Funairi Hospital, Hiroshima 730-0844.
A 44-year-old man with right pheochromocytoma was scheduled for tumor resection. General anesthesia was maintained with nitrous oxide (66%), oxygen (33%), continuous infusion of propofol (4 mg.kg-1.hr-1), and continuous epidural block (1.5% mepivacaine 4 ml.hr-1). During operation, cardiac output and blood volume (BV) were measured by pulse dye-densitometry using DDG-2001 (Nihon Kohden, Japan), and arterial and mixed venous blood concentrations of propofol were measured by high performance liquid chromatography (HPLC). After tumor resection, cardiac output increased without change in BV, on the other hand, both arterial and mixed venous blood propofol concentrations clearly decreased. In conclusion, during the anesthetic management with propofol in case of pheochromocytoma, attention should be paid to depth of anesthesia because of changing propofol blood concentration.

9
UI - 12058437
AU - Masamune T; Ishiyama T; Kawamura A; Suzuki M; Oguchi T; Kashimoto S;
TI - Kumazawa T [Use of magnesium sulfate during resection of pheochromocytoma]
SO - Masui 2002 May;51(5):516-7
AD - Department of Anesthesiology, Yamanashi Medical University, Yamanashi, 409-3098.
A 75-year-old woman was scheduled to undergo resection of pheochromocytoma under general and epidural anesthesia. Continuous infusion of magnesium sulfate was initiated at the time of tracheal intubation and was terminated at the tumor resection. Intraoperative blood pressure and heart rate were stable, but blood pressure rose above 160 mmHg when the tumor was handled. Hypertension caused by the tumor manipulation was successfully treated with intravenous nicardipine. Following the tumor removal, reduced blood pressure was treated with dopamine and norepinephrine. After the operation, spontaneous respiration did not appear until 120 minutes following the last vecuronium injection. Although neuromuscular blockade was reversed with neostigmine and atropine, muscle tone was not restored and satisfactory spontaneous respiration was not obtained. One hour later the patient was extubated. Intraoperative use of magnesium sulfate provides adequate hemodynamic stability for resection of pheochromocytoma, but may cause prolonged neuromuscular blockade. Monitoring of neuromuscular function should be essential and reduction of ve curonium dose should be considered on using magnesium sulfate intraoperatively.

10
UI - 11957807
AU - Madej A; Czarkowski M; Skorski M; Gornicka B; Cieszanowski A
TI - [Low-symptomatic pheochromocytoma]
SO - Pol Merkuriusz Lek 2002 Jan;12(67):61-4
AD - amadej@amwaw.edu.pl
We present the patient with 1-month history of mild hypertension, who was admitted to our department with suspicion of coronary heart disease. He had family history of hypertension and coronary heart disease, hyperlipidaemia and he has been smoker for several years. During the diagnostic exertion test chest pain together with an unusual high blood pressure was found (240/140 mmHg). The results of catecholamines and their metabolites in 24-urine collection, abdominal ultrasonography and NMR revealed pheochromocytoma in the left adrenal gland, which was removed successfully. Particular clinical examination allowed to include the patient to the group with low-symptomatic pheochromocytoma. Abdominal USG, which had been done prior to the exertion test, didn't reveal any incidental tumor in the adrenal glands. The abnormal hypertensive reaction during exertion test was the main decision of the urine catecholamines and their metabolites determination. Pheochromocytoma should be included to the differential diagnosis in all patients with chest pain and high blood pressure during diagnostic exertion test.

11
UI - 11948301
AU - Pace DE; Chiasson PM; Schlachta CM; Mamazza J; Cadeddu MO; Poulin EC
TI - Minimally invasive adrenalectomy for pheochromocytoma during pregnancy.
SO - Surg Laparosc Endosc Percutan Tech 2002 Apr;12(2):122-5
AD - Center for Minimally Invasive Surgery, St. Michael's Hospital, University of Toronto, Ontario, Canada.
Pheochromocytoma during pregnancy is a very rare condition; fewer than 200 cases have been reported in the literature. We present the case of a 24-year-old pregnant woman found to have a pheochromocytoma during investigation of abdominal pain. This is the second reported case of laparoscopic adrenalectomy for pheochromocytoma detected during pregnancy. After appropriate radiologic investigation and medical management, a laparoscopic left adrenalectomy was performed at the beginning of the second trimester. There were no complications, and she was delivered of a healthy baby at term. We review the management of pheochromocytoma in pregnant patients and discuss the role of laparoscopy.

12
UI - 12042109
AU - Gotoh M; Ono Y; Hattori R; Kinukawa T; Ohshima S
TI - Laparoscopic adrenalectomy for pheochromocytoma: morbidity compared with adrenalectomy for tumors of other pathology.
SO - J Endourol 2002 May;16(4):245-9; discussion 249-50
AD - Department of Urology, Nagoya University School of Medicine, Nagoya, Japan. gotoh@med.nagoya-u.ac.jp
PURPOSE: We report our experience with laparoscopic adrenalectomy in nine patients with pheochromocytoma and compare the morbidity with that of laparoscopic adrenalectomy for tumors of other pathology. PATIENTS underwent laparoscopic surgery for pheochromocytoma via a transperitoneal approach. Of the patients, eight had solitary tumors, and one presented with bilateral pheochromocytomas. The mean size of the tumors was 5.4 cm. The surgical outcomes of the 9 patients were compared with those of 28 patients with adrenal tumors of other pathology (primary aldosteronism in 15 patients, Cushing syndrome in 6, and nonfunctioning adenoma in 7) who underwent transperitoneal laparoscopic adrenalectomy during the same period. The mean size of the adrenal tumors of other pathology was 2.4 cm. RESULTS: In eight of the nine patients with pheochromocytoma, laparoscopic adrenalectomy was successful. The procedure was converted to open surgery in the patient with bilateral tumors because of uncontrollable hemorrhage. A hypertensive crisis with the systolic blood pressure >200 mm Hg occurred in 6 patients (67%), but the episode could be controlled by temporary discontinuation of tumor manipulation, administration of drugs, or both. In adrenalectomy for pheochromocytoma, the mean operative time was longer (199 v 177 minutes) and the mean estimated blood loss was greater (360 v 54 mL) than for tumors of other pathology. Blood transfusion was given to two patients with pheochromocytoma but to no patient with tumors of other pathology. The patients with adrenal tumors of other pathology could resume normal activity earlier (mean 18 v 26 days) than those with pheochromocytoma. CONCLUSION: The operation is more difficult and the morbidity is higher in laparoscopic adrenalectomy for pheochromocytoma than that for tumors of other pathology. An experienced team of surgeons with advanced laparoscopic skills and anesthesiologists is mandatory. In large tumors, great caution should be taken for intraoperative complications. Nevertheless, laparoscopic adrenalectomy is not contraindicated for pheochromocytoma and can be performed safely.

13
UI - 11692436
AU - Egea Camacho J; Fernandez del Busto E; Gonzalez de Zarate J; Trueba
TI - Arguinarena FJ; Sanz Lucas FJ; Pascual Samaniego M [Hypertension emergency during surgery of renal carcinoma associated with non-diagnosed pheochromocytoma]
SO - Arch Esp Urol 2001 Sep;54(7):707-11
AD - Servicio de Urologia Hospital Clinico Universitario C/Ramon y Cajal s/n 47005 Valladolid, Espana.
OBJECTIVE: To present an uncommon association of renal carcinoma with an undiagnosed ipsilateral pheochromocytoma that caused severe cardiovascular disorders during surgical treatment of the renal carcinoma, and emphasize the need for careful assessment of these adrenal nodules before resection although they may appear to be clinically irrelevant. METHODS: A 61-year-old male with mild arterial hypertension controlled with drug therapy for 40 years was diagnosed as having a right renal hypernephroma and a probable adrenal adenoma by IVP, abdominal ultrasound, CT, bone scintiscan and renal arteriography. Preoperative values were within the normal limits. The patient developed severe hypertension intraoperatively, which was controlled by administration of lidocaine, nitroglycerine, sodium nitroprusside and labetalol. Surgery was interrupted and completed when the patient was stable. RESULTS: The pathological analysis showed clear cell renal adenocarcinoma and pheochromocytoma. Postoperative evaluation showed dopamine, adrenaline, noradrenaline and vanilmandelic acid were normal. A 131 MIBG scan was normal. CONCLUSION: Although an adrenal mass may appear to be clinically irrelevant, a careful study should be performed to discard a functioning tumor and to avoid the serious complications that might arise during surgery.

14
UI - 12028101
AU - Koea JB; Blumgart LH
TI - Hepatic 'pseudotumours': right upper quadrant masses mimicking intrahepatic tumours.
SO - ANZ J Surg 2002 May;72(5):371-2
AD - The Hepatobiliary Service, Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, USA. jonathank@ahsl.co.nz

15
UI - 12003709
AU - Eisenhofer G; Lenders JW; Pacak K
TI - Choice of biochemical test for diagnosis of pheochromocytoma: validation of plasma metanephrines.
SO - Curr Hypertens Rep 2002 Jun;4(3):250-5
AD - Building 10, Room 6N252, 10 Center Drive, MSC 1620, National Institutes of Health, Bethesda, MD 20892-1620, USA. ge@box-g.nih.gov
Pheochromocytomas, although a rare cause of hypertension, are dangerous tumors that require consideration among large numbers of patients. The subsequent low prevalence of the tumor among those tested and inadequacies of commonly used biochemical tests make excluding or confirming the tumor an often difficult and time consuming task. Recognition that catecholamines are metabolized to free metanephrines within pheochromocytoma tumor cells, and that this process is independent of catecholamine release, provides a rationale for use of these metabolites in the biochemical diagnosis of pheochromocytoma. Measurements of plasma concentrations of free metanephrines thereby promise more reliable and efficient diagnosis of pheochromocytoma than offered by conventional biochemical tests.

16
UI - 12007193
AU - Kytola S; Nord B; Elder EE; Carling T; Kjellman M; Cedermark B; Juhlin
TI - C; Hoog A; Isola J; Larsson C Alterations of the SDHD gene locus in midgut carcinoids, Merkel cell carcinomas, pheochromocytomas, and abdominal paragangliomas.
SO - Genes Chromosomes Cancer 2002 Jul;34(3):325-32
AD - Department of Molecular Medicine, Karolinska Hospital, Stockholm, Sweden.
Several types of endocrine tumors show frequent somatic deletions of the distal part of chromosome arm 11q, where the tumor-suppressor gene SDHD (succinate-ubiquinone oxidoreductase subunit D), constitutionally mutated in paragangliomas of the head and neck, is located. In this study, we screened 18 midgut carcinoids, 7 Merkel cell carcinomas, 46 adrenal pheochromocytomas (37 sporadic and 9 familial), and 7 abdominal paragangliomas for loss of heterozygosity (LOH) and/or mutations at the SDHD gene locus. LOH was detected in 5 out of 8 (62%) informative midgut carcinoids, in 9 out of 30 (30%) sporadic pheochromocytomas, in none of the familial pheochromocytomas (0%), and in 1 out of 6 (17%) abdominal paragangliomas. No sequence variants were detected in the pheochromocytomas or paragangliomas. However, two constitutional putative missense mutations, H50R and G12S, were detected in two midgut carcinoids, which were both associated with LOH of the other allele. The same sequence variants were also detected in two Merkel cell carcinomas. In addition, the S68S polymorphism was found to coexist with the G12S sequence variant in both cases. In conclusion, we show that alterations of the SDHD gene seem to be involved in the tumorigenesis of both midgut carcinoids and Merkel cell carcinomas. Copyright 2002 Wiley-Liss, Inc.

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