Table of Contents
1
UI - 11967710
AU - Frezza EE; Ikramuddin S; Gourash W; Schauer P
TI -
Laparoscopic resection of a large periadrenal nonmalignant
pheochromocytoma.
SO - Surg Endosc 2002 Feb;16(2):362-3
AD - Loyola University of Chicago, 2160 South First Avenue, Maywood, Il
60153, USA. efrezza@lumc.edu
This is, to our knowledge, the first case description of an extraadrenal
pheochromocytoma located in the periadrenal fat. Pheochromocytoma is a
tumor that originates in the chromaffin tissue. Extraadrenal
pheochromocytomas have been described commonly in locations such as the
organ of Zuckerkandle (29%); the bladder (12%); the sacrum, testis,
rectum, and pelvic floor (2%); the upper abdomen in association with
celiac, superior mesenteric, and inferior mesenteric ganglia (43%); the
thorax (12%); and the neck (2%), most commonly in association with the
ninth or tenth cranial nerve ganglion. Our patient was a 40-year-old
woman known to have had an adrenal mass for the last 4 years. She was
referred for surgery because of an increase in the size of the mass to
11 cm. Laparoscopic adrenalectomy was performed via a posterior flank
approach. The pathology report was of periadrenal fat pheochromocytoma,
with positive staining for synaptophysin, chromogranin, and vimentin.
The patient was discharged on postoperative day 3. The unique feature in
this case was the uncommon location of the extraadrenal tumor: the
supraadrenal fat. The other unique finding in this case was that the
pheochromocytoma was neither symptomatic nor malignant, common features
of extraadrenal masses.
2
UI - 11980085
AU - Koh LK; Francis IC; Kappagoda MB; Hooper MJ; Filipic M
TI -
Multiple endocrine neoplasia type two B. A case report with light and
electron microscopic and immunohistochemical correlation.
SO - Aust N Z J Ophthalmol 1995 Nov;23(4):343-50
AD - Department of Ophthalmology, Prince of Wales Hospital, Sydney.
PURPOSE: To describe a case of multiple endocrine neoplasia type two B
(MEN IIB) where ocular and systemic clinicopathological findings are
correlated, in association with light and electron microscopic and
immunohistochemical findings. METHODS: A 23-year-old man presented with
mucosal neuromas of the lips, tongue and eyelids, a Marfanoid habitus
and prominent corneal nerves. These findings led to the diagnosis of
multiple endocrine neoplasia type two B. The patient subsequently
developed phaeochromocytoma and metastatic medullary thyroid carcinoma
(MTC) which led to his demise. Correlation of light and electron
microscopic and immunohistochemical findings with the systemic and
ocular findings is presented to emphasise the aggressiveness of MTC in
MEN IIB. Clinicopathological correlation was obtained by examining the
eyes post mortem. RESULTS AND CONCLUSIONS: Three new findings in MEN IIB
have been established by this study. The enlarged corneal nerves can now
be regarded as ganglioneuromas. Medullary thyroid carcinoma metastases
were found in the choroid. Light and electron microscopic examination of
the eye showed ganglioneuromas of the nerves in the limbus, trabecular
meshwork, uveal tract and posterior ciliary nerves; this finding may
account for the glaucoma occasionally seen in patients with MEN IIB.
3
UI - 11969054
AU - Zerhouni H; Kaddouri N; Abdelhak M; Benhmamouch N; Barahioui M
TI -
[Pheochromocytoma in children. Two case reports]
SO - Ann Urol (Paris) 2002 Mar;36(2):87-94
AD - Clinique chirurgicale infantile A, hopital d'enfants, CHU Ibn Sina,
Rabat, Maroc. hzerhouni@hotmail.com
Severe sustained hypertension occurs in only 0.1% of the pediatric
population and only about 2% of these patients will have an underlying
endocrine cause. Pheochromocytoma as a catecholamine secreting tumour
causing severe hypertension is exceedingly rare in children. A high
index of suspicion and an awareness of the clinical spectrum are
therefore necessary to make the diagnosis. We report two cases of
pheochromocytoma occurring in two a 12 year-old boys who's presented
with sustained hypertension, headache, sweating, and visual blurring.
Measurement of 24 hour urinary catecholamines showed a marked increase
and localization of adrenal unilateral pheochromocytoma was determined
by ultrasonography and computed tomography. The approaches to the
adrenal gland were transverse transperitoneal and the excision of the
tumors was followed of return to the normal of tensional numbers.
4
UI - 11961615
AU - Kercher KW; Park A; Matthews BD; Rolband G; Sing RF; Heniford BT
TI -
Laparoscopic adrenalectomy for pheochromocytoma.
SO - Surg Endosc 2002 Jan;16(1):100-2
AD - Department of General Surgery, Carolinas Medical Center, Charlotte, NC
28232, USA.
BACKGROUND: Laparoscopic adrenalectomy for Conn's syndrome, Cushing's
disease, cortisol-producing adenomas, and nonfunctioning adenomas has
been well established. This study was intended to evaluate the clinical
outcomes of patients undergoing laparoscopic adrenalectomy for
pheochromocytoma, and to assess the efficacy and safety of a minimally
invasive approach. METHODS: Data were collected prospectively on all
patients undergoing laparoscopic adrenalectomy for pheochromocytoma over
a 5-year period. RESULTS: In this study, 39 consecutive patients
underwent laparoscopic resection of a pheochromocytoma: 38 adrenal (23
left, 15 right) and 1 extraadrenal paraganglioma. There were no
conversions to open surgery. The mean tumor size was 5.2 cm (range,
2-12.1 cm). Average operative time was 159 min (range, 100-265 min), and
average estimated blood loss was 72 ml (range, 30-350 ml).
Intraoperative hypertension (systolic blood pressure > 170 mmHg)
occurred in 67% of the patients, and hypotension (systolic blood
pressure < 90 mmHg) in 39% of the patients. The mean length of stay was
2.1 days (range, 1-4 days). There were three minor postoperative
complications. During a mean follow-up period of 14 months, there were
no mortalities or recurrences of endocrinopathy. CONCLUSIONS:
Laparoscopic resection of pheochromocytomas can be accomplished safely
despite frequent episodes of hemodynamic variability equal to those of
historic open control subjects. A short hospital stay with expedient
recovery,minimal wound complications, and lack of endocrinopathy
recurrence makes a minimally invasive approach the procedure of choice
for the management of pheochromoctyoma.
5
UI - 11953123
AU - Peng Y; Li Y; Song H; Gao G; Huang C; Hu R; Han Z; Chen J
TI -
[Novel full-length cDNA cloning from normal adrenal gland and
pheochromocytoma and functional prediction]
SO - Zhonghua Yi Xue Za Zhi 2002 Jan 10;82(1):31-4
AD - Shanghai Institute of Endocrinology, Ruijin Hospital, Shanghai Second
Medical University, Shanghai 200025, China.
OBJECTIVE: To investigate methodology of cloning full-length cDNA from
tissues of normal adrenal gland and pheochromocytoma and predict their
function. METHODS: 104 samples from normal adrenal gland and 22 samples
from pheochromocytoma were examined by expressed sequence tags (EST)
sequencing, bioinformatics analysis, in silico cloning, rapid
amplification of cDNA ends and RT-PCR. RESULTS: Among the 126 samples of
novel full-length cDNA cloning 104 were from normal adrenal gland, and
22 from pheochromocytoma (PC). Among the novel genes 50 were cloned by
direct sequencing of clones, 74 by in silico cloning, and 2 by rapid
amplification of cDNA ends, and the localization of chromosomes was made
with UniGene searching and radiation hybrid (RH) procedure. Comparison
of homology and analysis of motif or domain showed that some genes may
be important functional genes, such as those coding signaling proteins,
proteins associated with ion channel, enzymes involved in hormone
production, important transcription factors, translation initiation
factors, etc. 7 of the novel full-length cDNAs are new isoforms produced
by alternative splicing. CONCLUSION: 126 novel full-length cDNAs have
been cloned from adrenal tissues by large-scale EST sequencing and
bioinformatic tools in a rather short time, some of which may play an
important role in regulating adrenal functions and tumorigenesis of
adrenal gland.
6
UI - 11927767
AU - Prokhorova M; Fritz S
TI -
Case of a 73-year-old man with dementia and a likely pheochromocytoma
mistaken for an anxiety disorder.
SO - Psychosomatics 2002 Jan-Feb;43(1):82
7
UI - 11979086
AU - Thompson LD
TI -
Pheochromocytoma of the Adrenal gland Scaled Score (PASS) to separate
benign from malignant neoplasms: a clinicopathologic and
immunophenotypic study of 100 cases.
SO - Am J Surg Pathol 2002 May;26(5):551-66
AD - Department of Endocrine and Otorhinolaryngic-Head & Neck Pathology,
Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
thompsonl@afip.osd.mil
No comprehensive series has evaluated the histologic features of
pheochromocytoma to separate benign from malignant pheochromocytoma by
histomorphologic parameters only. Fifty histologically malignant and 50
histologically benign pheochromocytomas of the adrenal gland were
retrieved from the files of the Armed Forces Institute of Pathology. The
patients included 43 females and 57 males, with an age range of 3-81
years (mean 46.7 years). Patients usually experienced hypertension (n =
79 patients). The mean tumor size was 7.2 cm (weight was 222 g).
Histologically, the cases of malignant pheochromocytomas of the adrenal
gland more frequently demonstrated invasion (vascular [score = 1],
capsular [score = 1], periadrenal adipose tissue [score = 2]), large
nests or diffuse growth (score = 2), focal or confluent necrosis (score
= 2), high cellularity (score = 2), tumor cell spindling (score = 2),
cellular monotony (score = 2), increased mitotic figures (>3/10 high
power fields; score = 2), atypical mitotic figures (score = 2), profound
nuclear pleomorphism (score = 1), and hyperchromasia (score = 1) than
the benign tumors. A Pheochromocytoma of the Adrenal gland Scaled Score
(PASS) weighted for these specific histologic features can be used to
separate tumors with a potential for a biologically aggressive behavior
(PASS > or =4) from tumors that behave in a benign fashion (PASS <4).
The pathologic features that are incorporated into the PASS correctly
identified tumors with a more aggressive biologic behavior. Application
of these criteria to a large cohort of cases will help to elucidate the
accuracy of this grading system in clinical practice.
8
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; 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.
9
UI - 12000821
AU - Dluhy RG
TI -
Pheochromocytoma--death of an axiom.
SO - N Engl J Med 2002 May 9;346(19):1486-8
10
UI - 11891502
AU - Neumann HP; Hoegerle S; Manz T; Brenner K; Iliopoulos O
TI -
How many pathways to pheochromocytoma?
SO - Semin Nephrol 2002 Mar;22(2):89-99
AD - Nephrology Section, Department of Medicine, Albert-Ludwigs University,
Freiburg, Germany. neumann@mm41.ukl.uni-freiburg.de
Pheochromocytomas, like several other tumors, may be either sporadic or
the manifestation of a familial cancer syndrome. Recently, major
advances have occurred in both the understanding of diverse molecular
mechanisms leading to pheochromocytoma and the diagnostic modalities
available for detection of the disease. Familial pheochromocytoma may be
a manifestation of multiple endocrine neoplasia type 2 (MEN-2), von
Hippel-Lindau (VHL), or neurofibromatosis-1 (NF 1) disease.
Tumor-suppressor genes responsible for the familial occurrence of
extra-adrenal pheochromocytoma, called paraganglioma, have been
identified. This wealth of genetic information, coupled with the
availability of sensitive and specific biochemical tests as well as
imaging studies, allows for genetic screening and early diagnosis of
pheochromocytoma. In addition, genetic screening of relatives at risk is
now feasible. In this article, we review recent clinical and molecular
advances in our understanding of pheochromocytoma. Copyright 2002,
Elsevier Science (USA). All rights reserved.
11
UI - 11977772
AU - Harada T; Ohtaki E; Sumiyoshi T; Hosoda S
TI -
A 68-year-old female with repetitive palpitation. Adrenal
pheochromocytoma.
SO - J Cardiol 2002 Apr;39(4):233-6
AD - Department of Cardiology, Sakakibara Heart Institute, Yoyogi 2-5-4,
Shibuya-ku, Tokyo 151-0053.
12
UI - 11375294
AU - Grouzmann E; Fathi M; Gillet M; de Torrente A; Cavadas C; Brunner H;
TI -
Buclin T
Disappearance rate of catecholamines, total metanephrines, and
neuropeptide Y from the plasma of patients after resection of
pheochromocytoma.
SO - Clin Chem 2001 Jun;47(6):1075-82
AD - Division d'Hypertension, Centre Hospitalier Universitaire Vaudois, 1011
Lausanne, Switzerland. eric.grouzmann@chuv.hospvd.ch
BACKGROUND: Plasma free metanephrines are a more reliable analyte to
measure than catecholamines for the biochemical diagnosis of
pheochromocytomas. We hypothesized that the long persistence of total
(sulfate-conjugated plus free) metanephrines in the blood might have a
significant diagnostic value. METHODS: We measured plasma concentrations
of catecholamines and total metanephrines (sulfate-conjugated plus free
forms) by HPLC with amperometric detection, and neuropeptide Y (NPY) by
an amplified ELISA in seven patients before and after removal of their
pheochromocytomas. The results for catecholamine, total metanephrines,
and NPY in each patient were analyzed for up to 120 min, starting from
the time of tumor vessel clamping. The persistence of analytes was
quantified as the area under the concentration-time curve over 120 min.
RESULTS: On the basis of the upper reference limit for each variable,
plasma free norepinephrine (NE) and epinephrine (E) concentrations were
increased preoperatively in at least one sample in seven and six
patients, respectively. Total normetanephrine (NMN) and metanephrine
(MN) were increased in all samples in seven and six patients,
respectively. NPY was increased 2- to 465-fold. After removal of the
tumor, MN and NMN showed a higher average relative increase above the
upper limit of the reference interval than NE and E (P = 0.05), whereas
NPY was intermediate. The persistence of increased values was
significantly shorter for catecholamines than for metanephrines. The
half-life estimated by nonlinear regression was 12.3 +/- 7.8 min for
NPY. Significant correlations were observed among NE, E, NMN, MN, and
NPY concentrations, but parent markers (E and MN or NE and NMN) did not
appear significantly intercorrelated. CONCLUSIONS: A larger increase and
a longer persistence of total metanephrines (reflecting predominantly
sulfo-conjugated metanephrines) than catecholamines and NPY in plasma
may contribute to their greater diagnostic accuracy in pheochromocytoma.
13
UI - 11830291
AU - Blanco M; Garcia-Caballero T; Fraga M; Gallego R; Cuevas J; Forteza J;
TI -
Beiras A; Dieguez C
Cellular localization of orexin receptors in human adrenal gland,
adrenocortical adenomas and pheochromocytomas.
SO - Regul Pept 2002 Mar 15;104(1-3):161-5
AD - Department of Morphological Sciences, Faculty of Medicine, Clinical
University Hospital, University of Santiago de Compostela, c/ San
Francisco s/n, E-15782, Santiago de Compostela, Spain.
Orexin-A and -B are hypothalamic peptides derived from a precursor
called prepro-orexin and related with the regulation of the energy
balance and arousal. They act on G protein receptors named orexin
receptor 1 (OX1R) and orexin receptor 2 (OX2R). In the present study, we
used immunohistochemical techniques to detect the distribution of OXR in
normal human adrenal gland and adrenal tumours (adrenocortical adenomas
and pheochromocytomas). OX1R was expressed in the cortex of the normal
human adrenal gland (glomerulosa, fasciculata and reticular zones) and
OX2R was located in the medulla (epinephrine and norepinephrine cells).
By the double immunofluorescence techniques, we demonstrated that
virtually all medullar cells (epinephrine and norepinephrine cells)
expressed OX2R. As was expected, according to the results obtained in
normal tissues, cortical tumours (adrenocortical adenomas) were positive
for OX1R but not for OX2R and conversely, medullar tumours
(pheochromocytomas) expressed only OX2R.
14
UI - 11823392
AU - Chung PC; Li AH; Lin CC; Yang MW
TI -
Elevated vascular resistance after labetalol during resection of a
pheochromocytoma (brief report).
SO - Can J Anaesth 2002 Feb;49(2):148-50
AD - Department of Anesthesiology, Chang Gung Memorial Hospital, Tao-Yuan,
Taiwan. steve226@ms23.hinet.net
PURPOSE: To report a case of pheochromocytoma exhibiting an increase in
systemic vascular resistance index (SVRI) and decreased cardiac index
(CI) after use of labetalol. CLINICAL FEATURES: A 36-yr-old male
underwent adrenectomy for pheochromocytoma. Midazolam 5 mg, fentanyl 100
microg and labetalol 20 mg were administrated intravenously for
premedication upon arrival in the operating theatre. After induction of
anesthesia with fentanyl, thiopental and atracurium, 30 mg iv labetalol
was administered. The blood pressure gradually rose to 178/101 mmHg with
mildly increased SVRI (1958 dn-sec(-1).m(2).cm(5)) and stable CI (3.8
L.min(-1).m(2) ). The blood pressure reached 247/150 mmHg after
intubation with an increase in SVRI (3458 dn-sec(-1).m(2).cm(5)) and a
decrease in CI (3.6 L.min(-1).m(2)). The SVRI increased further to 4986
dn-sec(-1).m(2).cm(5) and CI declined to 2.4 L.min(-1).m(2) after the
administration of additional labetalol 20 mg. Sodium nitroprusside was
administered and the blood pressure declined immediately to 108/72 mmHg,
with a decreased SVRI (2526 dn-sec(-1).m(2).cm(5)) and stable CI (2.3
L.min(-1).m(2)). CONCLUSIONS: The elevated SVRI with low CI was
considered to result from increased alpha-adrenergic activity secondary
to ss-adrenergic blockade with labetalol. Clinicians should be aware of
the possibility of a hypertensive crisis after iv labetalol. We suggest
that labetalol should be replaced promptly with alpha-adrenergic
blockers or other vasodilators when such a condition arises.
15
UI - 11963212
AU - Bruch C; Dagres N; Wieneke H; Herborn C; Brandt-Mainz K; Erbel R
TI -
[Sinus node dysfunction with intermittent sinus arrest and AV-nodal
escape rhythm as initial manifestation of pheochromocytoma]
SO - Z Kardiol 2002 Jan;91(1):81-7
AD - Westfalische Wilhelms-Universitat Munster Innere Medizin C
Albert-Schweitzer-Str. 33 48129 Munster, Germany. bruchc@uni-muenster.de
A 25-year-old male student complained about episodic palpitations,
dizziness, nausea and headache 5 years prior to presentation. No
otorhinolaryngic, neurologic or gastrointestinal causes were identified.
Several ECG recordings revealed sinus node dysfunction with intermittent
sinus arrest and AV-nodal escape rhythm. The patient was given a
permanent DDD-pacemaker. Six months later, the clinical symptoms were
unchanged. During an attack, physical examination revealed paleness,
diffuse sweating and an arterial blood pressure of 250/130 mmHg, which
decreased to 120/80 mmHg within a few minutes. Abdominal ultrasound and
abdominal computed tomographic scan demonstrated the presence of a large
(6.4 x 5.5 cm) left-sided adrenal mass. Two 24-h-urinary collections
demonstrated elevated noradrenaline (mean 315 micrograms/24 h, normal <
80 micrograms/24 h) and adrenaline (mean 268 micrograms/24 h, normal <
20 mg/24 h) levels. Blood samples, which were drawn during excessive
blood pressure rise, revealed elevation of plasma catecholamines (6.793
pg/ml for adrenaline (normal 50-150 pg/ml) and 10.424 pg/ml for
noradrenaline (normal 200-500 pg/ml), so that the diagnosis of
pheochromocytoma was considered established. The tumor was successfully
removed during laparascopic surgery. After surgery, the patient remained
well and normotensive. Three months later, several long-term ECG
recordings showed sinus arrhythmia with no evidence of sinus arrest or
AV-nodal escape rhythm, so that the DDD pacemaker was turned off. This
case underlines that sinus node dysfunction with intermittent sinus
arrest and AV-nodal escape rhythm is a potential early manifestation of
a pheochromocytoma. These changes seem to disappear after successful
removal of the tumor.
16
UI - 11967723
AU - Bemurat L; Gosse P; Ballanger P; Tauzin-Fin P; Barat P; Lacombe D;
TI -
Lemetayer P; Clementy J
Successful laparoscopic operation of bilateral pheochromocytoma in a
patient with Beckwith-Wiedemann syndrome.
SO - J Hum Hypertens 2002 Apr;16(4):281-4
AD - Hopital Saint Andre, service de Cardiologie/Hypertension arterielle,
Bordeaux, France.
We report the case of a 20-year-old female patient with
Beckwith-Wiedemann syndrome presenting with high blood pressure and
bilateral adrenal pheochromocytoma successfully removed with laparoscopy
in the same time. To our knowledge, the present case is the first
observation of a bilateral pheochromocytoma occurring in the
Beckwith-Wiedemann syndrome. It provides further support for a genetic
anomaly in this condition. Our case also indicates the interest of
laparoscopy for the surgical treatment of adrenal pheochromocytoma, even
in bilateral tumors.
17
UI - 10882262
AU - Lockhart ME; Smith JK; Canon CL; Morgan DE; Heslin MJ
TI -
Appendiceal ganglioneuromas and pheochromocytoma in neurofibromatosis
type 1.
SO - AJR Am J Roentgenol 2000 Jul;175(1):132-4
AD - Department of Radiology, University of Alabama at Birmingham,
35249-6830, USA.
18
UI - 11763822
AU - Gavrilenko AV; Sheremet'eva GF; Skrylev SI; Zolicheva NIu; Lisitskii DA;
TI -
Siniavin GV
[Surgical treatment of a patient with giant pheochromocytoma]
SO - Khirurgiia (Mosk) 2001;(10):51-2
19
UI - 11818620
AU - Hoegerle S; Nitzsche E; Altehoefer C; Ghanem N; Manz T; Brink I; Reincke
TI -
M; Moser E; Neumann HP
Pheochromocytomas: detection with 18F DOPA whole body PET--initial
results..
SO - Radiology 2002 Feb;222(2):507-12
AD - Divisions of Nuclear Medicine, Department of Radiology, Albert-Ludwigs
University, Freiburg, Germany. hoegerle@ukl.uni-freiburg.de
PURPOSE: To evaluate fluorine 18 ((18)F) dihydroxyphenylalanine (DOPA)
whole-body positron emission tomography (PET) as a biochemical imaging
approach for detection of pheochromocytomas. MATERIALS AND METHODS:
(18)F DOPA PET and magnetic resonance (MR) imaging were performed in 14
consecutive patients suspected of having pheochromocytomas (five
sporadic, nine with von Hippel-Lindau disease); metaiodobenzylguanidine
(MIBG) scintigraphy was performed in 12 of these patients. The
individual imaging findings were assessed in consensus by specialists in
nuclear medicine and radiologists blinded to the results of the other
methods. The findings of the functional imaging methods were compared
with those of MR imaging, the reference standard. Histologic
verification could be obtained in eight patients with nine tumors.
RESULTS: Seventeen pheochromocytomas (11 solitary, three bifocal; 14
adrenal, three extraadrenal) were detected with MR imaging. (18)F DOPA
PET and MR imaging had concordant results in all 17 tumors. In contrast,
MIBG scintigraphy had false-negative results in four patients with three
adrenal tumors smaller than 2 cm and one extraadrenal tumor with a
diameter of 3.6 cm. On the basis of these data, sensitivities of 100%
for (18)F DOPA PET and of 71% for MIBG scintigraphy were calculated.
Specificity was 100% for both procedures. CONCLUSION: (18)F DOPA PET is
highly sensitive and specific for detection of pheochromocytomas and has
potential as the functional imaging method of the future.
20
UI - 11996444
AU - Agarwal G; Mishra AK; Kapoor A; Agarwal A; Bhatia E; Mishra SK
TI -
Reversal of catecholamine induced cardiomyopathy in a patient with
bilateral malignant pheochromocytoma.
SO - J Assoc Physicians India 2001 Dec;49():1193-6
AD - Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of
Medical Sciences, Lucknow, India.
A patient of catecholamine induced cardiomyopathy (CMP) due to bilateral
malignant adrenal pheochromocytoma (PCC), successfully reversed on
treatment is described here. To the best of our knowledge, this is first
report of catecholamine induced CMP due to bilateral malignant adrenal
PCC, which could be reversed after surgery.
21
UI - 11981218
AU - Brunt LM; Lairmore TC; Doherty GM; Quasebarth MA; DeBenedetti M; Moley
TI -
JF
Adrenalectomy for familial pheochromocytoma in the laparoscopic era.
SO - Ann Surg 2002 May;235(5):713-20; discussion 720-1
AD - Department of Surgery, Section of Endocrine and Oncologic Surgery,
Washington University School of Medicine, St. Louis, Missouri 63110,
USA. bruntm@msnotes.wustl.edu
OBJECTIVE: To report the results of treatment of patients with familial
pheochromocytomas in the laparoscopic era. SUMMARY BACKGROUND DATA: The
optimal surgical management of pheochromocytomas that arise in familial
neoplasia syndromes may be complicated by bilateral involvement and
associated endocrinopathies. METHODS: Twenty-one patients with familial
pheochromocytomas (15 with multiple endocrine neoplasia [MEN] 2A, 4 with
MEN 2B, 1 each with von Hippel-Lindau and neurofibromatosis type 1)
biochemical, and pathologic data were obtained by retrospective review
of perioperative medical records, postoperative biochemical testing, and
patient questionnaire. RESULTS: Mean age at diagnosis was 37 +/- 11
years. Twenty of the 21 patients had elevated urine catecholamines, and
all had radiographic evidence of an adrenal tumor or tumors.
Pheochromocytoma-related symptoms were present in 11 patients (52%). One
patient with MEN 2B underwent open adrenalectomy due to previous adrenal
surgery and megacolon. Laparoscopic adrenalectomy was attempted in the
remaining 20 patients (9 right, 11 left, 2 bilateral). Two patients
(9.1%) were converted to open adrenalectomy. Intraoperative hypertensive
episodes occurred in 15 patients (71%) and were easily controlled
medically. Mean operative time was 216 +/- 57 minutes, mean
postoperative length of stay was 3.1 +/- 1.3 days, and mean tumor size
was 3.1 +/- 1.0 cm. Minor complications occurred in three patients
(14.3%) and major complications in two patients (9.5%). During a mean
follow-up of 57 months, a contralateral pheochromocytoma developed in
four patients with MEN 2 (33%); three of them underwent adrenalectomy.
There have been no long-term complications related to hypertension or
adrenalectomy. CONCLUSIONS: This study is the largest series of patients
with familial pheochromocytoma undergoing adrenalectomy during the
laparoscopic era. The results suggest that the laparoscopic approach is
safe and effective for managing unilateral or bilateral adrenal
medullary disease in this population.
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