Table of Contents
1
UI - 11840397
AU - Chauveau D; Burckle C; Beroud C; Correas JM; Duclos JM; Michel P;
TI -
Richard S; Grunfeld JP
Diagnosis of pheochromocytoma and laparoscopic adrenalectomy in two
anephric patients with von Hippel-Lindau disease.
SO - Am J Kidney Dis 2002 Feb;39(2):E6
AD - Service de Nephrologie and INSERM U 507, Hopital Necker, Paris, France.
dominique.chauveau@nck.ap-hop-paris.fr
This study reports the first two cases of laparoscopic treatment of
functional pheochromocytomas in patients with von Hippel-Lindau (VHL)
disease receiving regular dialysis treatment. The genetic changes
predisposing to pheochromocytoma, diagnostic procedures, and surgical
management were analyzed. Both patients were considered at low risk of
developing pheochromocytoma because they belonged to VHL families with
10 and 25 affected relatives without pheochromocytoma (VHL type 1
families). The mutation responsible for the disease was a stop codon in
one case and a missense change in the other. Multiple renal cell
carcinomas had required removal of the kidneys at age 37 in one patient
and age 25 in the other patient. Computed tomography scan was crucial
for diagnosis, showing a unilateral enlarged adrenal gland after 3 and 6
years on regular dialysis. No change in blood pressure was observed.
MIBG scintigraphy was negative. Plasma epinephrine in one case and
dopamine in the other were increased compared with patients on
hemodialysis without pheochromocytoma. Pheochromocytomas were removed
successfully by laparoscopic adrenalectomy. Lifelong follow-up of all
affected tissues is required in all VHL patients, and pheochromocytoma
should be screened for even in the absence of family history in large
kindreds. Computed tomography scan allows early recognition, and
patients on hemodialysis are responsive to laparoscopic adrenalectomy.
Copyright 2002 by the National Kidney Foundation, Inc.
2
UI - 11377298
AU - Klingler HC; Klingler PJ; Martin JK Jr; Smallridge RC; Smith SL; Hinder
TI -
RA
Pheochromocytoma.
SO - Urology 2001 Jun;57(6):1025-32
AD - Department of Urology, University of Vienna, Vienna, Austria.
3
UI - 11805353
AU - Misra AK; Agarwal G; Mishra A; Agarwal A; Bhatia V; Mishra SK
TI -
Pheochromocytoma in children and adolescents: an institutional
experience.
SO - Indian Pediatr 2002 Jan;39(1):51-7
AD - Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of
Medical Sciences, Lucknow 226 014, India. skmishra@sgpgi.ac.in
4
UI - 11861444
AU - Taylor RL; Singh RJ
TI -
Validation of liquid chromatography-tandem mass spectrometry method for
analysis of urinary conjugated metanephrine and normetanephrine for
screening of pheochromocytoma.
SO - Clin Chem 2002 Mar;48(3):533-9
AD - Department of Laboratory Medicine and Pathology, Mayo Clinic and
Foundation, Rochester, MN 55905, USA.
BACKGROUND: Metanephrines are biochemical markers for tumors of the
adrenal medulla (e.g., pheochromocytoma) and other tumors derived from
neural crest cells (e.g., paragangliomas and neuroblastomas). We
describe a liquid chromatography-tandem mass spectrometry (LC-MS/MS)
method for the measurement of urinary conjugated metanephrines. METHODS:
We added 250 ng of d3-metanephrine (d3-MN) and 500 ng of
d3-normetanephrine (d3-NMN) to 1 mL of urine samples as stable isotope
internal standards. The samples were then acidified, hydrolyzed for 20
min in a 100 degree C water bath, neutralized, and prepared by
solid-phase extraction. The methanol eluates were analyzed by LC-MS/MS
in the selected-reaction-monitoring mode after separation on a
reversed-phase amide C16 column. RESULTS: Multiple calibration curves
for the analysis of urine MN and NMN exhibited consistent linearity and
reproducibility in the range of 10-5000 microg/L. Interassay CVs were
5.7-8.6% at mean concentrations of 90-4854 microg/L for MN and NMN. The
detection limit was 10 microg/L. Recovery of MN and NMN (144-2300
microg/L) added to urine was 91-114%. The regression equation for the
LC-MS/MS (x) and colorimetric (y) methods was: y = 0.81x - 0.006 (r =
0.822; n = 110). The equation for the HPLC (x) and LC-MS/MS (y) methods
was: y = 1.09x + 0.05 (r = 0.998; n = 40). CONCLUSIONS: The sensitivity
and specificity of the MS/MS method for urinary conjugated metanephrines
offer advantages over colorimetric, immunoassay, HPLC, and gas
chromatography-mass spectrometry methods because of elimination of drug
interferences, high throughput, and short chromatographic run time.
5
UI - 11823694
AU - Naguib M; Caceres M; Thomas CR Jr; Herman TS; Eng TY
TI -
Radiation treatment of recurrent pheochromocytoma of the bladder: case
report and review of literature.
SO - Am J Clin Oncol 2002 Feb;25(1):42-4
AD - Department of Radiation Oncology, University of Texas Health Science
Center San Antonio, 7979 Wurzbach Road, San Antonio, TX 78229, U.S.A.
Secondary to the paucity of pheochromocytoma, very limited data exist
regarding the optimal treatments of metastatic disease. Malignant
pheochromocytomas are often considered unresponsive to radiotherapy, but
this decision is based on the few case reports performed before 1970.
There have been a handful of reports about metastatic resolution and
palliation from radiation therapy. Nevertheless, radiotherapy is not
considered a mainstay of pheochromocytoma treatment. In this case
report, we describe a patient with a malignant extraadrenal
pheochromocytoma, metastatic to the right humerus, and her treatment
course of surgery and radiotherapy.
6
UI - 11821644
AU - Manger WM; Gifford RW
TI -
Pheochromocytoma.
SO - J Clin Hypertens (Greenwich) 2002 Jan-Feb;4(1):62-72
AD - Department of Medicine, National Hypertension Association, 324 East 30th
Street, New York, NY 10016, USA.
Pheochromocytoma, a relatively rare (<0.05% of hypertensives),
catecholamine-secreting tumor, is almost always lethal unless recognized
and appropriately treated. Clinical and biochemical manifestations are
mainly caused by excess circulating catecholamines and hypertension.
Manifestations mimic many conditions, which may result in erroneous
diagnoses and improper treatment. Sustained or paroxysmal hypertension
associated with headaches, sweating, or palpitations, occurs in 95% of
patients, but at least 5% are normotensive. All patients with
manifestations of hypercatecholaminemia or coexisting neoplasms should
be investigated for pheochromocytoma. Plasma free metanephrines and
fractionated urinary metanephrines are the most sensitive (about 100%)
chemical tests for diagnosing sporadic and familial pheochromocytomas;
plasma and urinary catecholamines and total metanephrines are fairly
sensitive for identifying sporadic cases but are less sensitive for
familial tumors. The clonidine suppression test helps exclude other
conditions that may elevate plasma and urinary catecholamines and their
metabolites. Magnetic resonance imaging is more sensitive than computed
tomography for localizing pheochromocytomas;
iodine-131-metaiodobenzylguanidine (131I-MIBG) tumor uptake confers
specificity. Surgical resection is successful in 90% of cases, but 10%
of tumors are malignant. Pheochromocytomas <5 cm in diameter can be
removed laparoscopically; larger tumors should be removed by open
surgery. Drug treatment prior to and during surgery is mandatory; drug
treatment, chemotherapy, and radiation therapy are used to treat
malignant lesions. (c) 2002 Le Jacq Communications, Inc.
7
UI - 11688394
AU - Baccari P; Colombo R; Spotti D; Giovannelli M; Corlatti M; Staudacher C
TI -
Multidisciplinar surgical treatment in a case of von Hippel-Lindau with
CNS hemangioblastoma, bilateral adrenal pheochromocytoma and multiple
bilateral renal cell carcinoma--a case report.
SO - Contrib Nephrol 2001;(136):281-3
AD - Division of Emergency Surgery, IRCCS San Raffaele Hospital, University
of Milan, Italy. Baccari.Paolo@hsr.it
8
UI - 11688395
AU - Baccari P; Corlatti M; Staudacher C
TI -
Laparoscopic bilateral adrenalectomy in patients with pheochromocytoma
and von Hippel-Lindau disease.
SO - Contrib Nephrol 2001;(136):284-91
AD - Division of Emergency Surgery, IRCCS San Raffaele Hospital, University
of Milan, Italy. Baccari.Paolo@hsr.it
9
UI - 11768644
AU - Yoshida S; Hatori M; Noshiro T; Kimura N; Kokubun S
TI -
Twenty-six-years' survival with multiple bone metastasis of malignant
pheochromocytoma.
SO - Arch Orthop Trauma Surg 2001 Nov;121(10):598-600
AD - Department of Orthopaedic Surgery, Tohoku University School of Medicine,
Sendai, Japan. yoshida@gonryo.med.tohoku.ac.jp
The prognosis of metastatic pheochromocytoma is poor in general. There
have been few instances of long-term survival reported. We report a case
of a 44-year-old woman who has survived for 26 years after bone
metastasis. She was diagnosed as having pheochromocytoma arising in the
left adrenal medulla in 1974. Metastasis of pheochromocytoma in the
first and third lumbar vertebrae and the right ilium was observed at the
same time. The primary lesion was removed, and posterior lumbar spinal
fusion was performed for immobilization. The metastatic lesion in the
ilium was left untouched. After 26 years, she is well despite a
recurrence of the tumors in the skull and a new metastasis in the left
abdomen.
10
UI - 11790269
AU - Bravo EL
TI -
Pheochromocytoma.
SO - Cardiol Rev 2002 Jan-Feb;10(1):44-50
AD - Department of Nephrology and Hypertension, Cleveland Clinic Foundation,
Cleveland, OH 44195, USA.
Pheochromocytomas cause the most dramatic, life-threatening crises in
all of endocrinology. Pheochromocytoma is an explosive clinical syndrome
characterized by severe hypertension associated with cardiac
complications, hypotension, or even shock and sudden death. The key to
diagnosing pheochromocytoma is to suspect it, then confirm it. The cases
reported in this review illustrate how the diagnosis can be easily
missed and definitive treatment delayed. An appreciation of the wide
range of clinical manifestations, based on clear understanding of the
mechanisms of catecholamine action and the pathophysiology of
pheochromocytoma, and the availability of simple and accurate diagnostic
tests should lead to earlier detection of these tumors. Advances in
localization techniques and availability of various treatment modalities
have made successful management more promising than ever before.
11
UI - 11808220
AU - Tokunaga H; Tomita K
TI -
[Pheochromocytoma]
SO - Nippon Rinsho 2001 Dec;59 Suppl 8():149-57
AD - Third Department of Internal Medicine, Kumamoto University School of
Medicine.
12
UI - 11740055
AU - Candanedo-Gonzalez FA; Alvarado-Cabrero I; Gamboa-Dominguez A;
TI -
Cerbulo-Vazquez A; Lopez-Romero R; Bornstein-Quevedo L; Salcedo-Vargas M
Sporadic type composite pheochromocytoma with neuroblastoma:
clinicomorphologic, DNA content and ret gene analysis.
SO - Endocr Pathol 2001 Fall;12(3):343-50
AD - Department of Pathology, Oncology Hospital, Centro Medico Nacional Siglo
XXI, IMSS. Mexico, D.F. Mexico. fcandanedo@hotmail.com
Composite pheochromocytomas (CP) account for only 3% of all
pheochromocytomas. We analyzed the clinical, immunohistochemical,
ultrastructural, DNA content, and 634 ret mutation features in a
56-year-old Mexican woman with CP localized in the right adrenal gland
and associated to a blood pressure of 140/90 mmHg. Clinical symptoms
were absent after surgery. The tumor showed pheochromocytoma and
neuroblastoma components. This dual phenotype was supported by light
microscopy and corroborated by immunohistochemistry and ultrastructural
findings. Flow cytometric analysis showed that both components were
diploid. A genetic mutational analysis of the ret oncogene in exon 11
showed no 634 mutation. This case demonstrates the indolent behavior of
neuroblastoma associated to a sporadic-type CP in an adult patient.
13
UI - 11899980
AU - Pocaro AB; Cavalleri S; Ballista C; Righetti R; Ficarra V; Malossini G;
TI -
Tallarigo C
Modern imaging methods and preoperative management of pheochromocytoma:
review of the literature and case report.
SO - Arch Esp Urol 2000 Oct;53(8):749-53
AD - Cattedra e Divisione Clinicizzata di Urologia, Ospedale Policlinico,
Universita degli Studi di Verona, Italy.
OBJECTIVES: To discuss the modern imaging techniques and preoperative
management of pheochromocytoma and to report on one additional case.
METHODS: A 66-year-old male with an incidentally discovered left adrenal
mass is described. The adrenal medulla strongly accumulate 131
I-metaiodobenzylguanidine (MIBG). The patient underwent left
adrenalectomy after preoperative therapy with alpha and beta-blockers.
The recent literature on pheochromocytoma modern imaging techniques and
preoperative management is reviewed. RESULTS: MIBG scintigraphy
diagnosed a benign functioning adrenal pheochromocytoma, allowing
preoperative medical management. Postoperative workup was unremarkable.
Diagnosis of pheochromocytoma was confirmed by immunohistopathology. At
18 months follow-up, the patient is alive and disease-free. CONCLUSIONS:
Incidentally discovered adrenal masses have to be investigated to detect
malignancy and subtle hormonal overproduction. MIBG scintigraphy has a
high specificity (100%) in detecting pheochromocytoma, metastasis,
surgical residual tumor, local relapse and other adrenal crest tumors.
Positive results of octreotide scintigraphy in detecting malignant
pheochromocytoma have been reported. Currently, pheochromocytoma removal
is a safe operation with mortality rates ranging from 0 to less than 3%.
Preoperative alpha adrenergic blockage with phenoxybenzamine or prazosin
is important in decreasing the operative risk. Beta-blockers may be
necessary for cardiac arrhythmia. Intraoperative invasive monitoring of
hemodynamic variables may be both diagnostic and therapeutic of
inadequate preoperative management. Lifelong follow-up for patients with
pheochromocytoma is important.
14
UI - 11716230
AU - Hartley A; Spooner D; Brunt AM
TI -
Management of malignant phaeochromocytoma: a retrospective review of the
use of MIBG and chemotherapy in the West Midlands.
SO - Clin Oncol (R Coll Radiol) 2001;13(5):361-6
AD - Cancer Centre at the Queen Elizabeth Hospital, Birmingham, UK.
Metastatic malignant phaeochromocytoma is a rare disorder, with no
randomized and few prospective data to facilitate choice between the two
main treatment modalities, chemotherapy and radiolabelled
metaiodobenzylguanidine (MIBG). In the last decade the latter modality
has been preferred and radiological response rates of 30% have been
reported. There are fewer patients described in the literature who have
received chemotherapy but one prospective trial of chemotherapy reported
radiological response rates of 57%. A recent prospective trial combining
the two modalities has been disappointing with only one patient
completing the treatment schedule. We present six patients with
malignant phaeochromocytoma or paraganglioma who received MIBG therapy.
Four patients also received chemotherapy. A retrospective review of the
case notes was performed. Radiological and hormonal responses were
determined and the time to progression after each modality was
calculated. One partial hormonal response was seen with MIBG treatment.
One complete and one partial hormonal response and one partial
radiological response were seen with chemotherapy. The median time to
disease progression from commencement of MIBG was 12 months (range 3-44)
and from commencement of chemotherapy used as first or second line
treatment was 22.5 months (range 7-25). Chemotherapy may be a more
active modality in this disease than previously considered. MIBG uptake
may increase after a partial radiological response to chemotherapy,
enabling subsequent MIBG therapy. Researchers carrying out future trials
on combined therapy should consider administering chemotherapy prior to
MIBG for the reasons that we outline in this article.
15
UI - 11809538
AU - Vierhapper H
TI -
Adrenocortical tumors: clinical symptoms and biochemical diagnosis.
SO - Eur J Radiol 2002 Feb;41(2):88-94
AD - Department of Internal Medicine III, Division of Endocrinology and
Metabolism, University of Vienna, Wahringer Gurtel 18-20, A-1090,
Vienna, Austria.
Patients who are suspected clinically to suffer from hypersecretory
disorders of their adrenal(s) should undergo an appropriate
endocrinological investigation to confirm or exclude the presence of
such disorders prior to any radiological investigation. In those
patients in whom an adrenal mass is found 'incidentally' on imaging
clinical symptoms of hormonal activity should be carefully followed up.
Asymptomatic patients should be screened biochemically for latent
hormonal hypersecretion syndromes including pheochromocytoma (urine
catecholamine excretion), hypercortisolism (overnight dexamethason
suppression test) and aldosteronism (blood pressure and serum
potassium). These investigations are mandatory in all patients scheduled
for surgery. The decision to refer patients with inactive adrenal tumors
to surgery is, in the absence of valid biochemical markers of
malignancy, mainly influenced by tumor size but remains arbitrary.
Patients who are not at first treated by surgery should be operated if
follow-up indicates a progression in tumor size.
16
UI - 11865592
AU - Golkowski F; Trofimiuk M; Huszno B; Szybinski Z; Nowak K; Popiela T
TI -
[An oligosymptomatic case of pheochromocytoma]
SO - Pol Arch Med Wewn 2001 May;105(5):399-402
AD - Katedra i Klinika Endokrynologii Collegium Medicum Uniwersytetu
Jagiellonskiego.
A case of 34-year old female with incidentally diagnosed adrenal tumour
is discussed. The patient complained only of mild headaches and heart
palpitations and was not previously treated for hypertension. A
diagnosis of pheochromocytoma was made. The diagnostic controversies
arose because of subclinical course of the disease, slightly elevated
biochemical markers of pheochromocytoma (catecholemines urinary
excretion) and non-characteristic result of glucagon stimulation test
results. The diagnosis was confirmed by histologic examination of tumour
tissue. Presented case indicates the need for thorough clinical and
hormonal evaluation of patients with incidentaloma (particularly, when
adrenal tumour diameter is larger than 3 cm) to avoid serious
complication of surgery treatment in case of misdiagnosis.
17
UI - 11882600
AU - Higashi Y; Sasaki S; Nakagawa K; Kimura M; Sasaki S; Noma K; Matsuura H;
TI -
Hara K; Goto C; Oshima T; Chayama K
Excess norepinephrine impairs both endothelium-dependent and
-independent vasodilation in patients with pheochromocytoma.
SO - Hypertension 2002 Feb;39(2 Pt 2):513-8
AD - First Department of Internal Medicine, Faculty of Medicine, Hiroshima
University, Japan. yhigashi@hiroshima-u.ac.jp
There is little information concerning the interaction of nitric oxide
and norepinephrine (NE) on endothelial function in humans. The purpose
of this study was to determine whether endothelial function is impaired
by NE secreted from patients with pheochromocytoma (pheo) and whether
surgical resection of the tumor improves endothelial function in these
patients. We evaluated the forearm blood flow (FBF) response to
acetylcholine (ACh), an endothelium-dependent vasodilator, and
isosorbide dinitrate (ISDN), an endothelium-independent vasodilator,
before and after adrenalectomy in 8 pheo patients, 20 normotensive
subjects, and 20 patients with essential hypertension. FBF was measured
using a mercury-filled silastic strain-gauge plethysmograph. The FBF
response to ACh was the greatest in normotensive subjects and the least
in pheo patients. The FBF response to ISDN was significantly less in
pheo patients than in the other 2 groups, which had similar responses to
ISDN. Adrenalectomy significantly decreased plasma and urinary NE,
systolic and diastolic blood pressures, heart rate, and forearm vascular
resistance. After adrenalectomy, FBF responses to both ACh and ISDN were
enhanced in all pheo patients. The ratio of maximal ACh-stimulated FBF
to maximal ISDN-stimulated FBF was significantly higher after
adrenalectomy than before adrenalectomy (2.1 +/- 0.4 versus 1.1 +/- 0.1;
P<0.05). The increase in maximal FBF response to ACh correlated
significantly with the decrease in urinary excretion of NE (r=-0.62,
P<0.01). These findings suggest that excess NE from pheo may
predominantly impair endothelium-dependent vasodilation in humans.
18
UI - 11903030
AU - Lenders JW; Pacak K; Walther MM; Linehan WM; Mannelli M; Friberg P;
TI -
Keiser HR; Goldstein DS; Eisenhofer G
Biochemical diagnosis of pheochromocytoma: which test is best?
SO - JAMA 2002 Mar 20;287(11):1427-34
AD - Department of Internal Medicine, St Radboud University Medical Center,
Geert Grooteplein Zuid 8, PO Box 9101, 6500 HB, Nijmegen, the
Netherlands. j.lenders@aig.azn.nl
CONTEXT: Diagnosis of pheochromocytoma depends on biochemical evidence
of catecholamine production by the tumor. However, the best test to
establish the diagnosis has not been determined. OBJECTIVE: To determine
the biochemical test or combination of tests that provides the best
method for diagnosis of pheochromocytoma. DESIGN, SETTING, AND
PARTICIPANTS: Multicenter cohort study of patients tested for
pheochromocytoma at 4 referral centers between 1994 and 2001. The
analysis included 214 patients in whom the diagnosis of pheochromocytoma
was confirmed and 644 patients who were determined to not have the
tumor. MAIN OUTCOME MEASURES: Test sensitivity and specificity, receiver
operating characteristic curves, and positive and negative predictive
values at different pretest prevalences using plasma free metanephrines,
plasma catecholamines, urinary catecholamines, urinary total and
fractionated metanephrines, and urinary vanillylmandelic acid. RESULTS:
Sensitivities of plasma free metanephrines (99% [95% confidence interval
[CI], 96%-100%]) and urinary fractionated metanephrines (97% [95% CI,
92%-99%]) were higher than those for plasma catecholamines (84% [95% CI,
78%-89%]), urinary catecholamines (86% [95% CI, 80%-91%]), urinary total
metanephrines (77% [95% CI, 68%-85%]), and urinary vanillylmandelic acid
(64% [95% CI, 55%-71%]). Specificity was highest for urinary
vanillylmandelic acid (95% [95% CI, 93%-97%]) and urinary total
metanephrines (93% [95% CI, 89%-97%]); intermediate for plasma free
metanephrines (89% [95% CI, 87%-92%]), urinary catecholamines (88% [95%
CI, 85%-91%]), and plasma catecholamines (81% [95% CI, 78%-84%]); and
lowest for urinary fractionated metanephrines (69% [95% CI, 64%-72%]).
Sensitivity and specificity values at different upper reference limits
were highest for plasma free metanephrines using receiver operating
characteristic curves. Combining different tests did not improve the
diagnostic yield beyond that of a single test of plasma free
metanephrines. CONCLUSION: Plasma free metanephrines provide the best
test for excluding or confirming pheochromocytoma and should be the test
of first choice for diagnosis of the tumor.
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