Table of Contents
1
UI - 21281567
AU - Jacobs JM; Hawes MJ
TI -
From eyelid bumps to thyroid lumps: report of a MEN type IIb family and
review of the literature.
SO - Ophthal Plast Reconstr Surg 2001 May;17(3):195-201
AD - Department of Ophthalmology, University of Colorado Health Sciences
Center, Denver, USA.
PURPOSE: We present a two-generation family with multiple endocrine
neoplasia (MEN) type IIb diagnosed by their ophthalmologists based on
characteristic ophthalmic findings. METHODS: A family consisting of a
33-year-old female proband and her 8- and 7-year-old children had
prominent corneal nerves; eyelid, lip, and tongue nodules; and a
characteristic facies. A polymerase chain reaction-based genetic assay
was obtained to detect the genetic mutation most commonly associated
with MEN type IIb. Serum calcitonin and urine catecholamine studies were
obtained. RESULTS: Molecular genetic studies detected in all 3 patients
a mutation at codon 918 of the RET proto-oncogene known to be present in
95% of the cases of MEN type IIb. Serum calcitonin was elevated in the
proband and her son. Urine catecholamine levels were elevated in the
proband. Surgical treatment and histologic analysis confirmed
pheochromocytoma and medullary thyroid carcinoma (MTC) in the proband.
Surgical exploration revealed the MTC to be metastatic to the liver.
CONCLUSIONS: This family demonstrates the characteristic findings of MEN
type IIb: prominent corneal nerves in a clear stroma and multiple
submucosal neuromas of the conjunctiva, eyelids, lips, and tongue.
Ophthalmologists have a critical role to play in recognizing these
signs, because the early diagnosis of medullary thyroid carcinoma and
pheochromocytoma may be life saving.
2
UI - 21367648
AU - Winter C; Schmidt-Mutter C; Cuny R; Soulas S; Dupeyron JP; Steib A
TI -
Fatal form of phaeochromocytoma presenting as acute pyelonephritis.
SO - Eur J Anaesthesiol 2001 Aug;18(8):548-53
AD - Department of Anaesthesiology, Hopital Civil, Hopitaux Universitaires,
67091 Strasbourg Cedex, France.
We report the case of a young man who presented with a clinical picture
of acute pyelonephritis. Within 3 h of admission, the patient developed
acute respiratory distress associated with tachycardia and shock, and he
was transferred to the intensive care unit. Mechanical ventilation of
the lungs and symptomatic treatment were started immediately. Abdominal
ultrasound revealed the presence of an adrenal tumour with central
necrosis indicating a probable phaeochromocytoma. There was no sign of
pyelonephritis. Ventricular fibrillation followed by asystole occurred
soon after admission. The suddenness of the patient's death did not
allow time for further investigation and therapy. The severity of the
clinical signs was probably related to a massive release of
catecholamines because of necrosis of the tumour, which may have been
worsened by the diagnostic procedures performed to investigate the
clinical symptoms and signs of acute pyelonephritis.
3
UI - 21457581
AU - Bullough AS; Watters M
TI -
Phaeochromocytoma diagnosed during labour.
SO - Br J Anaesth 2001 Feb;86(2):288; discussion 288-9
4
UI - 21457582
AU - Fawcett WJ; Edkins CL
TI -
Phaeochromocytoma diagnosed during labour.
SO - Br J Anaesth 2001 Feb;86(2):288; discussion 288-9
5
UI - 20383129
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.
6
UI - 21268711
AU - Yeh CN; Jeng LB; Chen MF; Hung CF
TI -
Nonfunctioning malignant pheochromocytoma associated with
dermatomyositis: case report and literature review.
SO - World J Urol 2001 Apr;19(2):148-50
AD - Department of Surgery, Chang Gung Memorial Hospital, 5, Fu-Hsing Street,
Kwei-Shan, Taoyuan, Taiwan.
A 42-year-old man with dermatomyositis presented with
right-upper-quadrant dull pain and normal blood pressure for 10 days.
Abdominal ultrasonography, abdominal computed tomography, and
angiography revealed a retroperitoneal tumor with direct invasion to the
liver and the diaphragm. The diagnosis of nonfunctioning malignant
pheochromocytoma was made on the basis of clinical evidence. The tumor
was removed en bloc with part of the diaphragm, the right lobe of the
liver, the right adrenal gland, and the right kidney. Pathologic
examination with immunohistochemical staining revealed a malignant
pheochromocytoma growing exophytically from the right adrenal gland and
invading the right lobe of the liver and the diaphragm. The
postoperative course has been uneventful and no recurrence has been
noted over a 6-month follow-up period.
7
UI - 21434076
AU - Wu KD; Chen YM; Chu TS; Chueh SC; Wu MH; Bor-Shen H
TI -
Expression and localization of human dopamine D2 and D4 receptor mRNA in
the adrenal gland, aldosterone-producing adenoma, and pheochromocytoma.
SO - J Clin Endocrinol Metab 2001 Sep;86(9):4460-7
AD - Department of Internal Medicine, National Taiwan University Hospital,
Taipei 100, Taiwan. kdw@ha.mc.ntu.edu.tw
Aldosterone secretion is evidently regulated by a dopaminergic
inhibitory mechanism. Pharmacological characterization and
autoradiographic studies revealed D2-like receptors in the adrenal
cortex, especially in the zona glomerulosa. However, the subtype of the
dopamine receptors involving this regulation has not been elucidated. To
investigate which subtype of receptors expresses in the adrenal cortex,
we examined the messages of D2-like receptors, D2, D3, and D4, by RT-PCR
and in situ hybridization of adrenal glands and adrenal neoplasm. Both
D2 and D4 receptors were expressed in normal adrenal glands,
pheochromocytoma, and aldosterone-producing adenoma. However, the D2
receptors were not universally expressed, in contrast with the D4
receptors that were detected in all cases of aldosterone-producing
adenoma and adrenal remnant. No D3 receptor message was detected by
RT-PCR in any adrenal sample. Both D2 and D4 receptors were expressed in
significant amounts in the adrenal medulla and pheochromocytoma. In the
adrenal cortex, the expression of the D2 receptors was in the zona
glomerulosa and zona reticularis, with no different signal intensities
between the two zones. D4 receptors were mainly localized in the zona
glomerulosa and, to a lesser extent, in the zona reticularis. Both
receptors were expressed at low levels in the zona fasciculata. In
aldosterone-producing adenoma, the expression of D2 and D4 was
especially found in nonzona fasciculata-like cells. To elucidate which
dopamine receptor regulates aldosterone secretion, the effects of
specific D2 and D4 antagonists, raclopride and clozapine, respectively,
were examined in cultured NCI-H295 cells. Dopamine further increased
angiotensin II-induced aldosterone secretion by 20%. In the presence of
1 microM dopamine and angiotensin II, 10(-5)-10(-7) M clozapine
decreased aldosterone levels by 40-55%. The decrease in aldosterone
secretion by clozapine was completely reversed when raclopride was added
simultaneously. These data suggest that dopamine exerts dual effects on
aldosterone secretion in NCI-H295 cells. Activation of D4 receptors can
increase aldosterone secretion, whereas an inhibitory effect is mediated
via D2 receptors. In summary, we demonstrated the existence of both D2
and D4 receptors in the human adrenal gland and adrenal neoplasm. Both
receptors play significant roles in the modulation of aldosterone
secretion, but in opposite directions.
8
UI - 21457545
AU - James MF
TI -
Phaeochromocytoma-recent progress in its management.
SO - Br J Anaesth 2001 Apr;86(4):594-5
9
UI - 21459805
AU - Giarelli E
TI -
Ethical issues in genetic testing. The experiences of one family
diagnosed with an inherited cancer syndrome.
SO - J Infus Nurs 2001 Sep-Oct;24(5):301-10
AD - University of Pennsylvania School of Nursing, 420 Guardian Drive,
Philadelphia, PA 19104, USA. IMLNG@aol.com
This article describes selected ethical issues faced by members of a
family with genetic predisposition to the cancer syndrome multiple
endocrine neoplasia type 2a. The bioethical principles of autonomy,
nonmaleficence, beneficence, and justice are the moral guides for nurses
and other health professionals, who may apply them to help patients,
family members and peers resolve ethical issues and moral dilemmas.
Sometimes, however, issues that are morally problematic cannot be
settled by simply referring to standards of practice and bioethical
norms. In these cases all stakeholders must struggle together to resolve
the conflict.
10
UI - 21290106
AU - Rizzo S; Bonomo S; Moser A; Bottura D; Castellini C; Mazzola F; Lauro E;
TI -
Vicenzi L; Betresini B; Angeli G; Brazzarola P; D'Azzo G; Rosa G
[Bilateral pheochromocytoma associated with duodeno-jejunal GIST in
patient with von Recklinghausen disease: report of a clinical case]
SO - Chir Ital 2001 Jan-Feb;53(2):243-6
AD - Dipartimento di Scienze Chirurgiche e Gastroenterologiche Cattedra di
Semeiotica e Metodologia Chirurgica, Universita degli Studi di Verona.
The authors present the case of a 60-year-old male patient suffering
from von Recklinghausen's disease (neurofibromatosis type I, NF1) with
bilateral pheochromocytoma and occasional intraoperative reports of
duodenojejunal GIST (GastroIntestinal Stromal Tumour). Through a review
of the literature the authors analyze the frequency and the features of
bilateral pheochromocytoma and its rare histological variant, the
so-called composite pheochromocytoma, characterized by the combination
of pheochromocytoma and ganglioneuroma or ganglioneuro-blastoma.
Bilaterality of pheochromocytoma is more frequent in patients with
familiarity for pheochromocytoma without NF1. Composite pheochromocytoma
accounts for about 3% of total pheochromocytomas. In addition, the
authors summarize the present knowledge about gastrointestinal stromal
tumours and investigate the possible association between them and NF1 or
pheochromocytoma, concluding that any such association is purely casual,
while confirming the well known, genetically determined association
between NF1 and pheochromocytoma.
11
UI - 20350009
AU - Sorva A
TI -
[A patient with previous myocardial infarction, now work-shy]
SO - Duodecim 1997;113(19):1943, 1947
12
UI - 21437013
AU - Takami H; Ikeda Y; Takayama J; Sasaki Y; Kan S; Niimi M; Inada E;
TI -
Kameyama K
Adrenal-sparing adrenalectomy in hereditary bilateral phaeochromocytoma.
SO - ANZ J Surg 2001 Oct;71(10):623-4
AD - First Department of Surgery and Department of Anesthesiology, Teikyo
University School of Medicine and Division of Diagnostic Pathology, Keio
University Hospital, Tokyo, Japan. takami@med.teikyo-u.ac.jp
13
UI - 21448255
AU - Pautler SE; Pavlovich CP; Mikityansky I; Drachenberg DE; Choyke PL;
TI -
Linehan WM; Wood BJ; Walther MM
Retroperitoneoscopic-guided radiofrequency ablation of renal tumors.
SO - Can J Urol 2001 Aug;8(4):1330-3
AD - Urologic Oncology Branch, National Cancer Institute and the Diagnostic
Radiology Department, Warren G. Magnuson Clinical Center, National
Institutes of Health, Bethesda, Maryland 20892, USA.
OBJECTIVE: Minimally invasive approaches to the management of renal
tumors are being studied intensively in urology. Herein, we describe the
use of multiple organ-sparing techniques for the management of tumors in
a patient with von Hippel Lindau disease (VHL). MATERIALS AND METHODS: A
42 year-old woman with VHL underwent a right partial adrenalectomy and a
left renal radiofrequency ablation (RFA) of two renal tumors. RESULTS: A
2.2 cm solitary right adrenal pheochromocytoma was resected using a
transperitoneal approach. A retroperitoneal approach to the left kidney
was performed and RFA of the two renal tumors completed using
sonographic guidance. On the 5-month follow-up CT scan, there was no
evidence of residual adrenal tumors and both renal lesions lacked
contrast enhancement. No complications occurred during the
post-operative recovery. CONCLUSIONS: Multiple organ-ablative
laparoscopic procedures may be performed in a single sitting.
Laparoscopic partial adrenalectomy is an effective technique in patients
with bilateral tumors or a familial syndrome predisposing to multiple
adrenal tumors. Further study of renal RFA is required to assess the
long-term durability of the procedure.
14
UI - 21464051
AU - Minei S; Yamashita H; Koh H; Satoh T; Kobayashi S; Furuhata M; Uchida T;
TI -
Baba S
[Giant cystic pheochromocytoma: a case report]
SO - Hinyokika Kiyo 2001 Aug;47(8):561-3
AD - Department of Urology, Kitasato University School of Medicine.
The patient, a 59-year-old woman, was referred to our University
Hospital for evaluation of a left mass. Ultrasonography revealed a left
adrenal cystic mass. On excretory urograms, the left kidney was pressed
downward by a suprarenal mass, and computerized tomography (CT) and
magnetic resonance imaging (MRI) confirmed an adrenal cyst.
131I-meta-iodo-benzylguanidine (MIBG) scintigraphy showed prominent
accumulation in the left adrenal mass and the capsule. Considering the
elevation of catecholamines in both blood and urine samples, we
performed a left adrenalectomy with a presumptive diagnosis of
pheochromocytoma (tumor size: 11.6 x 7.5 x 6.5 cm, tumor weight 720 g).
The subsequent pathological examination confirmed a left giant cystic
pheochromocytoma. 131I-MIBG scintigraphy was the most useful tool in the
diagnosis of the cystic pheochromocytoma.
15
UI - 21460223
AU - Akcay G; Akcay MN; Alici HA
TI -
The effects of diathermy on haemodynamic stability in phaeochromocytoma.
SO - Anaesthesia 2001 Oct;56(10):1015-6
16
UI - 21486587
AU - Mazzocchi G; Malendowicz LK; Aragona F; Rebuffat P; Gottardo L;
TI -
Nussdorfer GG
Human pheochromocytomas express orexin receptor type 2 gene and display
an in vitro secretory response to orexins A and B.
SO - J Clin Endocrinol Metab 2001 Oct;86(10):4818-21
AD - Department of Human Anatomy and Physiology, Section of Anatomy,
University of Padua, Via Gabelli 65, I-35121 Padua, Italy.
mazzocch@ux1.unipd.it
Orexins A and B are hypothalamic peptides, that act through two receptor
subtypes, called OX1-R and OX2-R. OX1-R selectively binds orexin A,
whereas OX2-R is nonselective for both orexins. High levels of OX1-R
mRNA and low levels of OX2-R mRNA have been previously detected in the
human adrenal cortex and medulla. Here we demonstrated by RT-PCR the
expression of the OX2-R, but not the OX1-R, gene in 10 benign secreting
pheochromocytomas. Both orexins A and B stimulated catecholamine
secretion from pheochromocytoma slices; the maximal effective
concentration was 10(-8) mol/liter. Orexins A and B (10(-8) mol/liter)
increased IP3, but not cAMP production, by tumor slices, and the effect
was blocked by the PLC inhibitor U-73122. The catecholamine response to
10(-8) mol/liter orexins A and B was abolished by either U-73122 or the
PKC antagonist calphostin C and was unaffected by the adenylate cyclase
inhibitor SQ-22536 and the PKA inhibitor H-89. Collectively, these
findings suggest that orexins stimulate catecholamine secretion from
human pheochromocytomas, acting through OX2-R coupled to the PLC-PKC
signaling pathway.
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