<Chief
complaint>
Intermittent palpitations for one year.
<Case
presentation>
A 19-year-old girl had been healthy before until one year
ago when she suffered from intermittent palpitations. The
paroxysmal episodes were accompanied by profuse sweating and
headache, the duration and frequency of which were about 20-30
minutes and 2-3 times per day, respectively. There was no
known precipitating factor. She visited a hospital where
hypertension was noted (BP about 170/100 mmHg). Secondary
hypertension was impressed, and she underwent serial studies
to search the underlying causes.
Twenty-four-hour urine level of vanillylmandelic acid
(VMA) reported 182 £gmole/day (normal < 31).
Pheochromocytoma was highly suspected, but both computer
tomography of the adrenal glands and
131I-metaiodobenzylguanidine (MIBG) scan were
negative. She took doxazosin (1.5 mg Q.D.) and nifedipine if
needed but the discomfort was only slightly alleviated. So she
visited a medical center for second opinion and was admitted
for further evaluation and management.
She was a student and denied smoking, alcohol consumption,
other systemic diseases and a family history of hypertension.
Physical examination revealed a 63 kg, 163 cm-tall girl
without a round face, acne or truncal obesity. Her temperature
was 36.9¢XC, pulse rate 76/min, respiratory rate 20 /min and
her blood pressure was 160/90 mmHg. Her conjunctivae were
pink, sclerae were anicteric and pupils were isocoric with
prompt light reflex. Her neck was supple without a goiter,
engorged jugular veins, or lymphadenopathy. The thoracic,
abdominal, and neurologic examinations were normal. There was
no gynecomastia, galactorrhea, buffalo hump or purple
striae.
<Laboratory
data>
Table1. Endocrine test
Renin |
Aldosterone |
Cortisol (8AM) |
Cortisol (4PM) |
DHEA-SO4* |
ng/mL/hr (1-5) |
ng/dL (5-30) |
£g g/dL (5-25) |
£g g/dL (2.5-12.5) |
£g mol/L (4.6-15.4) |
6.40 |
66.75 |
10.3 |
6.87 |
4.7 | Table2. 24 HR urine catecholamine
Dopamine |
Epinephrine |
Norepinephrine |
VMA |
£g g/24h
(50-450) |
£g g/24h
(< 22.4) ƒÝ |
£g g/24h
(12.1-85.5) |
mg/24h (1-7) |
361.4 |
15.2 |
1173.2 |
18.1 |
*DHEA-SO4: dehydroepiandrosterone
sulfate
<Course and
treatment>
She
discontinued all medications for two weeks. Complete blood
counts and biochemical studies, including serum electrolytes
were within normal ranges. Both 24-hr urine levels of VMA and
norepinephrine increased. Other endocrine studies were within
normal limits except serum rennin activity and aldosterone
level elevated. Computer tomography of chest, abdomen and
pelvis revealed a heterogeneous and moderately enhanced mass
about 4 cm around the pancreatic head (Fig 1
). Bilateral adrenal glands were
normal in size. Previous 131I-MIBG scan was negative, so she
received 2-[Flurine-18] fluoro-2-deoxy-D-glucose positron
emission tomography (PET) of whole body. The image showed a
large FDG hypermetabolic ring lesion at right sub-hepatic
region (Fig 2).
She got a magnetic resonance image to exclude vascular
invasion. She took phenoxybenzamine for pre-operative blood
pressure control and propranolol for tachycardia. She
successfully accepted an operation for tumor excision. No
vascular invasion was noted. The tumor was soft and well
encapsulated and the pathology confirmed the diagnosis of
paraganglioma (Fig 3
& 4
). Post-operative 24-hour urine
levels of VMA and norepinephrine decreased apparently. She
never had palpitation and hypertension after removal of the
tumor and needed no antihypertensive drugs, either. She
followed up regularly at
OPD.
<Discussion>
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hypertension)¡C¶Ý»Ì²ÓM½F(pheochromocytoma)¬Oì¦]¤§¤@¡A90¢Hªº¶Ý»Ì²ÓM½F¦ì©óµÇ¤W¸¢¡A10¢H¦bµÇ¤W¸¢¥~¡AºÙ¬°°Æ¯«¸g¸`½F(paraganglioma)¡A«áªÌ±q¯«¸g¸`ªø¥X¡A¤j³¡¤À¥i¦b¸¡µÄ¤ºµo²{¡A¦ý¤]¥i¥H¦ì©ó«á¸¡µÄ¡B°©¬ÖµÄ¡B«e«áÁa¹jµÄ¡B¤ßŦ©Î¥D°Ê¯ß®Ç¡C³\¦h¯f¤H©ó¶EÂ_«e´N¦]¤ß«ß¤£¾ã©Î¤¤·¦º¤`¡A¤j¦h¼Æªº¯f¤H³£¦³°ª¦åÀ£¡BÀYµh¡B¤ß±ª¤Î«_¦½µ¥¯gª¬¡C15¢Hªº¶Ý»Ì²ÓM½F¬O´c©Êªº¡A¥i¥HÂಾ¨ìÀY°©¡B¦Ø°©¡B¯á´Õ¡B¯ÝºÞ¡B¸zô½¤©MªÍŦ¡F°Æ¯«¸g¸`½Fªº´c©Ê¾÷·|¸û°ª¡A¬ù30-50¢H¡C¤j¬ù30-60¢Hªº°Æ¯«¸g¸`½F¬O¦³¥\¯àªº(functional)¡A¥i¥H¤Àªcnorepinephrine¤Înormetanephrine¡A¦ý¬O¨Ã¤£·|¦]¦¹¼vÅT¥Í¦s¡C
°£¤FÁ{§É¯gª¬¥~¡A¤´»Ý¥Í¤ÆÀˬd»²§U¶EÂ_¡A¦p¦å¼ß©M§¿²G¤¤ªºcatecholamines¤Îmetanephrine
(catecholamineªº¥NÁª«)¡C¥Ø«e¥H´ú§¿²G¤¤ªºmetanephrineªº±Ó·P©Ê³Ì°ª¡A¦ý¬O°ò©ó¹êÅç«Çªº¨î¡A§Ú̪º¯f¤H¥u´ú¤G¤Q¥|¤p®É§¿²G¤¤ªºVMA¤Îcatecholamines¡CµM¦Ó¡A¦³¨ÇÃĪ«¡B¹ª«(©@°Ø¦]¡B»¿¼)¡B©Î¯e¯f¤]³£·|¼vÅT¦å¼ß©Î§¿²Gcatecholaminesªº¿@«×¡AÄ~¦Ó¤zÂZ§Ú̪º¶EÂ_¡A©Ò¥HÀ³¸Ó¥J²Ó°Ý¶E¡A¦pªG¤¹³\¡A©óÀˬd«eÀ³°±¤î©Ò¦³ÃĪ«¦Ü¤Ö¨â¬P´Á¡C¤]¥i¥H´úserum
chromogranin A
(CgA)¡A¥¿±`±¡ªp¤U¡ACgA¦¤W¤KÂI®É¦å¤¤¿@«×³Ì§C¡A¤U¤È¤Î±ß¤W11ÂI®É¿@«×³Ì°ª¡A¥BCgAªº¿@«×©M¸~½F¤j¤p¦³Ãö¡A©Ò¥H¬O¤@Ó¦³¥Îªº¸~½F¼Ð»x¡C¦]¬°catecholamines¥i¥H«P¶irenin¤Àªc¡A©Ò¥H¦³¨Ç¯f¤H¦å¤¤ªºrenin¤Îaldosterone¤]·|¤W¤É¡A¦p¦P§Ú̪º¯f¤H¡C
Á{§É¤W§Q¥Îmetaiodobenzylguanidine (MIBG)
scans©M¹q¸£Â_¼hÀˬd¨Ó©w¦ì¡CMIBGªº±Ó·P©Ê¬ù85¢H¡A¦ý¬O¹ï©ó°»´úµÇ¤W¸¢¥~¸~½F®É±Ó·P©Ê·|¤U°¡C§Ú̪º¯f¤H©óÁ{§É¤W(¥]¬A¯gª¬¤ÎÀËÅç)±j¯PÃhºÃ¬O¶Ý»Ì²ÓM½F¡A¦ý¬OµÇ¤W¸¢¹q¸£Â_¼hÀˬd¤ÎMIBG«o¨S¦³§ä¨ì¸~½F¡A¦¹®É´Nn¦Ò¼{°Æ¯«¸g¸`½F¡A¦]¦¹¶·¦h§@¯ÝµÄ¡B¸¡µÄ¤Î°©¬ÖµÄ¹q¸£Â_¼h¡C©ó¹q¸£Â_¼hÀˬd«e¡A¤@©wn¥ý±±¨î¦åÀ£¡A¦]¬°Åã¼v¾¯·|³y¦¨°ª¦åÀ£¦M¶H¡CÃh¥¥¯f¤Hº¿ïªºÀˬd¬O®ÖºÏ¦@®¶³y¼v¡A¦]¬°¤£»Ýn¥´Åã¼v¾¯¡A¤]¨S¦³¿ç®g½u¡F¥[§@¦åºÞÄá¼v¥i¥Hª¾¹D¬O§_¦³¦åºÞ«I¥Ç¡C¥¿¤lÄá¼v(positron
emission
tomography¡APET)¹ï©w¦ì´c©Ê¶Ý»Ì²ÓM½FÂಾªº±Ó·P©Ê¤£¿ù¡A¦ý¬O¯S²§©Ê¸ûMIBG®t¡A¦n³B¬O¥i¥H¥ß§Y§@¤£»Ý¨Æ«e·Ç³Æ¡C
ªvÀø¥H¤â³N¤Á°£¬°¥D¡A¤ºµøÃèµÇ¤W¸¢¤Á°£(laparoscopic
adrenectomy)¬O¥Ø«eªº¥D¬y¡CµM¦Ón°µ¦n³N«e·Ç³Æ¡A¥H¨¾°ª¦åÀ£¦M¶H©Î¥ð§J¡F«Øij¨Ï¥Î£\-blockers(¦pphenoxybenzamine¡Bdoxazosin©Îprazosin)¡A¦ý¬O·|¼W¥[catecholamines©Mmetanephrineªº¦X¦¨¡A¨Ï±o¤ß¸õ¥[§Ö¡A¦¹®É¥i¥H¨Ö¥Î£]-
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enzyme (ACE)ªºµ²¦X¦ì¸m¡A©Ò¥H¥i¥H¦P®É¨Ï¥ÎACE inhibitors©Îangiotensin receptor
blockers (ARBs)¡A¦ý¬O¥¥°ü¸T¥Î¡A¦]¬°·|P·îL¡C
¸~½F¤Á°£«á¤´¦³25¢Hªº¯f¤H·|¦³°ª¦åÀ£¡C¨}©Ê¶Ý»Ì²ÓM½F5¦~¦s¬¡²v¬°96¢H¡A´c©Ê¸~½F¥u¦³44¢H¡C¤â³N«á2¬P´Á¦A°lÂܧ¿²Gcatecholamines¤Îmetanephrine¡A¤§«á¤]n©w´Á°lÂÜ¡C
<References>
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- Hoegerle S et al: Pheochromocytomas:
detection with 18F DOPA whole body PET-initial results.
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- Combemale F et al: Exclusive use of
calcium channel blockers and cardioselective beta-blockers
in the pre- and peri-operative management of
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- Winfield HN et al: Technique of
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- Tagaya N et al: Laparoscopic resection of a functional
paraganglioma in the organ of Zucherkandl. Surg Endosc
2002;16:219.
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