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NCI CANCERLIT® Search: Pheochromocytoma - July 2002
National Cancer Institute®
Last Modified: July 1, 2002
- Phaeochromocytoma--recent progress in its management.
- Phaeochromocytoma-recent progress in its management.
- Differential diagnosis of pheochromocytomas and paragangliomas.
- [Benign and malignant pheochromocytoma]
- Laparoscopic partial adrenalectomy for recurrent pheochromocytoma after open partial adrenalectomy in von Hippel-Lindau disease.
- Occurrence of pheochromocytoma in a MEN2A family with codon 609 mutation of the RET proto-oncogene.
- Simultaneous development of pheochromocytomas in prepubertal siblings with von Hippel-Lindau syndrome.
- Spontaneous rupture of adrenal pheochromocytoma.
- Pheochromocytoma in a pregnant woman with multiple endocrine neoplasia type 2a.
- Pheochromocytomas and paragangliomas in von Hippel-Lindau disease: a role for laparoscopic and cortical-sparing surgery.
- Images in cardiovascular medicine. Cyclic tachycardia and hypotension.
- Pheochromocytoma.
- Diminished circadian blood pressure rhythm in patients with asymptomatic normotensive pheochromocytoma.
- Anesthesia for one-stage bilateral pheochromocytoma resection in a patient with MEN type IIa: attenuation of hypertensive crisis by
- [Does the size of pheochromocytoma influence the results of its laparoscopic excision?]
- Early identification of patients with von Hippel-Lindau disease at risk for pheochromocytoma.
- Imaging vs biochemical testing for pheochromocytoma.
- Cystic phaeochromocytoma presenting as an acute abdomen with shock.
- Metastases of predominantly dopamine-secreting phaeochromocytoma that did not accumulate meta-iodobenzylguanidine: imaging with whole body
- [Clinical analysis of 17 patients with adrenal "incidentaloma"]
- Laparoscopic resection of large adrenal tumors.
- Phaeochromocytoma diagnosed during labour.
- Phaeochromocytoma diagnosed during labour.
- [Synchronous renal oncocytoma and asymptomatic pheochromocytoma. Report of a case]
- [Pheochromocytoma discovered in the preoperative period: analysis of 3 cases]
- [Bilateral adrenal pheochromocytomas in von Hippel-Lindau disease]
Table of Contents
1
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.
2
UI - 11573642
AU - James MF
TI -
Phaeochromocytoma-recent progress in its management.
SO - Br J Anaesth 2001 Apr;86(4):594-5
3
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.
4
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. hendrik.lehnert@medizin.uni-magdeburg.de
5
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. saifalsobhi@hotmail.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.
6
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
7
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.
8
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.
9
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.
10
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.
11
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.
12
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.
13
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. tzeli@lfl.cuni.cz
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.
14
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.
15
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.
lbrunaud@planete.net
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.
16
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.
17
UI - 12117391
AU - Neumann HP
TI -
Imaging vs biochemical testing for pheochromocytoma.
SO - JAMA 2002 Jul 17;288(3):314-5; discussion 315
18
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.
19
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.
kmtani@bekkoame.ne.jp
20
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.
21
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. mesurg@poa.mmc.org
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.
22
UI - 11573678
AU - Bullough AS; Watters M
TI -
Phaeochromocytoma diagnosed during labour.
SO - Br J Anaesth 2001 Feb;86(2):288; discussion 288-9
23
UI - 11573679
AU - Fawcett WJ; Edkins CL
TI -
Phaeochromocytoma diagnosed during labour.
SO - Br J Anaesth 2001 Feb;86(2):288; discussion 288-9
24
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.
25
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.
26
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.
The above citations and abstracts reflect those newly added to CANCERLIT for the month and topic listed in the title. The citations have been retrieved from CANCERLIT using a predefined search strategy of indexed subject terms. Although the search strategy has been refined as best as possible, citations may appear that are not directly related to the topic, and occasionally relevant references may be omitted.
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