< Chief
Complaint >
Intermittent palpitation and headache for two
months.
< Brief
History
>
This 54 year-old man, a boss of a factory, is a
patient with diabetes mellitus diagnosed for 1 year. He took
oral hypoglycemic agents (glimeperide 4 mg Q.D, metformin 500
mg T.I.D) and his average fasting plasma glucose level was
around 110 mg/dL. Two months prior to admission, he developed
intermittent palpitation and headache. Each episode of which
lasted several minutes and resolved spontaneously without
overt precipitating or aggravating factors. Occasionally the
episode happened at bedtime and was accompanied by pallor,
dizziness and perspiration. There was no chest tightness,
vomiting or abdominal pain. He had ever visited a local
medical clinic where hypertension was noted. Because these
episodes were paroxysmal, he refused to take any medication.
He denied a family history of hypertension.
Because the
frequency of headache increased recently, he underwent a
health examination. Whole body magnetic resonance imaging
revealed a left adrenal tumor measuring 3.2 cm in diameter
which presented with hyperintense signal on T2-weighted image
(fig
1& 2
). Whole body positron emission tomography (PET)
showed negative findings. Secondary hypertension which was
related to a pheochromocytoma was highly suspected and he was
admitted for further study.
<
Physical examination > Physical examination
revealed a 76 kg, 169 cm tall man without round face, acne or
truncal obesity. His blood pressure was 120/80 mmHg,
respiratory rate was 18/min, pulse rate was 88/min and his
temperature was 36.2¢J. His consciousness was clear,
conjunctivae were pink, and his sclerae were anicteric. The
pupils were isocoric with prompt light reflex. The neck was
supple without goiter, lymphadenopathy, engorged jugular veins
or carotid bruit. Chest, abdominal and extremity examinations
were normal. There was no gynecomastia, galactorrhea, buffalo
hump or purple striae.
<
Laboratory Examination >
Table1. Endocrine test
Renin |
Aldosterone |
Cortisol (8AM) |
Cortisol (4PM) |
DHEA-SO4* |
ng/mL/hr |
ng/dL |
£gg/dL |
£gg/dL |
£gmol/L |
3.48 (1-5) |
19.5 (5-30) |
10 (5-25) |
6 (2.5-12.5) |
4.2 (4.6-13.4) |
Table 2. 24 HR urine catecholamines
Dopamine |
Epinephrine |
Norepinephrine |
VMA** |
£gg/24h |
£gg/24h |
£gg/24h |
mg/24h |
286.72 (50-450) |
213.37 (< 22.4) |
175.93 (12.1-85.5) |
8.25 (1-7)
|
*DHEA-SO4: dehydroepiandrosterone sulfate;
**VMA: vanillylmandelic acid ƒÝ
< Course and Treatment
>ƒÝ During
hospitalization, he experienced another two episodes of
palpitation and headache which were accompanied by concomitant
elevation of blood pressure (systolic/diastolic 170/100 mmHg).
Complete blood counts and biochemical studies, including serum
electrolytes, were within normal ranges except for an
overnight fasting glucose of 130 mg/dL. Twenty-four-hour urine
exam revealed elevated VMA (8.25 mg) and catecholamine levels
(epinephrine 213.37£gg, norepinephrine 175.93£gg). Plasma renin
activity, aldosterone and cortisol levels were all within
normal limits. The laboratory test confirmed the diagnosis of
pheochromocytoma. Doxazosin 2 mg daily initially was
prescribed and the dose was gradually increased to 16 mg daily
for better blood pressure control. He received laparoscopic
adrenalectomy smoothly. The pathology proved the diagnosis of
pheochromocytoma (fig 3
&
4
). Both his blood pressure and
fasting blood glucose levels gradually returned to normal
ranges without necessitating any medications. He was
discharged and regularly followed up at out-patient
department. ƒÝ
< Discussion
>
¶Ý»Ì²ÓM½F(pheochromocytoma)¬O¤@ºØ¤Ö¨£ªº¸~½F¡A¨C¦~¨C¤@¦Ê¸U¤H¬ù¦³¨â¦ì¡A¬O³y¦¨Äòµo©Ê°ª¦åÀ£(secondary
hypertension)ªºì¦]¤§¤@¡A¦û©Ò¦³°ª¦åÀ£¤Ö©ó0.1¢H¡C¨S¦³©Ê§O¤Wªº®t²§¡A¥i¥Hµo¥Í¦b¥ô¦ó¦~¬ö¡A¤×¨ä±`¨£©ó30¦Ü40·³¡C90¢Hªº¦¨¤H¤Î70¢Hªº¤p«Ä¦ì©óµÇ¤W¸¢¡A¥k°¼¤S¤ñ¥ª°¼±`¨£¡A¥k°¼ªº¶Ý»Ì²ÓM½F¸û±`¥X²{°}µo©Ê(paroxysmal)°ª¦åÀ£¡A¬ù10¢Hªº¦¨¤H¤Î35¢Hªº¤p«Ä¨â°¼µÇ¤W¸¢¬Ò¦³¸~½F¡C¤j³¡¤À°¸µo©Ê¶Ý»Ì²ÓM½F¬O¦³¥]½¤ªº(encapsulated)¡A«Ü¤Ö·|«I¥Ç©PÃä²Õ´¡A¦ý¬O¦³®É¸~½F·|¶i¤JµÇ¤W¸¢ÀR¯ß¡B¤UµÄÀR¯ß¡A³y¦¨ªÍ®ê¶ë¡C¶Ý»Ì²ÓM½Fªº¤j¤pÅܲ§«Ü¤j¡A¥i¥H«Ü·L¤p(microscopic)¤]¥i¥H¤j¦Ü3600§J¡A¥§¡ª½®|¬ù4.5¤½¤À¡A«¬ù100§J¡CParagangliomas¬OµÇ¤W¸¢¥~ªº¶Ý»Ì²ÓM½F(extra-adrenal
pheochromocytomas)¡A±q¯«¸g¸`ªø¥X¡A¦û¦¨¤H¶Ý»Ì²ÓM½F¬ù10¢H¡A¨ä¤¤85¢H¥i¥H¦b¸¡³¡µo²{¡C
¤j³¡¤Àªº¶Ý»Ì²ÓM½F¬O°¸µoªº(sporadic)¡A¦ý¬O¶W¹L10¢H¥i¥H¬O¿ò¶Çªº¡A³o¨Ç¯f¤H»Ýn§@°ò¦]¿zÀË¡A¦pmultiple
endocrine neoplasia type 2 (MEN 2)¬O¦]¬°ret
proto-oncogene¬ðÅÜ¡A³y¦¨¶Ý»Ì²ÓM½F¡B¥Òª¬¸¢Åè½èÀù(medullary thyroid
carcinoma)¤Î°Æ¥Òª¬¸¢¾÷¯à¤®¶i(hyperparathyroidism)©ÎÂH½¤¯«¸g½F(mucosal
neuroma)¡F¤S¦pVon Hipple-Lindau disease¬O¦]¬°VHL tumor suppressor
gene¬ðÅܵ¥¡C¤j³¡¤À°¸µo©Ê¸~½F¬O¨}©Êªº¡A¦Ó¦³15¢Hªº¶Ý»Ì²ÓM½F¬O´c©Êªº¡A¥i¥HÂಾ¨ìÀY°©¡B¦Ø°©¡B¯á´Õ¡BªÍ¡B¯ÝºÞ¡B¸zô½¤©MªÍŦ¡CMEN
2ªº´c©Ê¾÷·|¸û°ª¡Aparagangliomas¦³30-50¢H¬O´c©Êªº¡CµÇ¤W¸¢¥~¶Ý»Ì²ÓM½F¡B¤j¸~½F(ª½®|6¤½¤À¥H¤W)¡B¸~½F¦³¿Ä¦X©ÊÃa¦º¡B¦åºÞ«I¥Ç©Î¼sªx©Ê§½³¡«I¥Ç«h´c©Ê¾÷·|¸û¤j¡C
ÁöµM¦³¨Ç¶Ý»Ì²ÓM½F¤£·|¤Àªccatecholamine¡A¦ý¬O¤j³¡¤À³£¥i¥H»s³ycatecholamine
¡A¬Æ¦Ü°ª©ó¥¿±`ªº27¿¡CµM¦Ó¦]¬°µLªk¦^õX§í¨î¡A©Ò¥H³y¦¨«ùÄò¥B¹L«×¤Àªc¡A¦Ó¼W¥[¤ßŦ¿é¥X¶q(cardiac
output)¡B¶gÃä¦åºÞªý§Ü(peripheral
resistance)¤ÎµÇ¯À(renin)¤Àªc¡AÄ~¦Ó³y¦¨°ª¦åÀ£¡C¦ý¬O¡A¦å¤¤catecholamineªº¿@«×©M¸~½F¤j¤p¤£¤@©w¬ÛÃö¡A¦]¬°¤j¸~½F¥i¯à·|¦³¤º³¡¥X¦å©ÊÃa¦º¡C¸~½F¤º¦Ûµo©Ê¥X¦å¡B¥Í²z©Ê¨ë¿E¦p±Æ§¿(micturation)¡B¨Åé¯kµh¡B¤ß·Ð·N¶Ã¡B´¡ºÞ¡B³Â¾K³£¥i¯à¤Þ°_°ª¦åÀ£¦M¶H(hypertensive
crisis)¡C°£¦¹¤§¥~¡A¶Ý»Ì²ÓM½FÁÙ·|¤Àªcneuropeptide Y
(NPY¡A«D±`±jªº¦åºÞ¦¬ÁY¾¯¡A¤Þ°_°ª¦åÀ£)¡Bneuron-specific enolase
(NSE¡A¨}©Ê¸~½F¦å¤¤¿@«×·|¥¿±`¡A¤@¥bªº´c©Ê¶Ý»Ì²ÓM½F¿@«×·|¤W¤É)¡BACTH(³y¦¨®wªY¯gÔ¸s)¡Berythropoietin(¬õ¦å²y¥Í¦¨¯À¡A³y¦¨¬õ¦å²y¹L¦h)¡Bparathyroid
hormone- related peptide (PTHrP¡A³y¦¨°ª¦å¶t)¡BIL-6 (µo¿N)¡C
¶W¹L¤T¤À¤§¤@ªº¯f¤H©ó¶EÂ_¥X¶Ý»Ì²ÓM½F«e´N¤w¸g¦]¬°¤ß«ß¤£¾ã©Î¤¤·¦º¤`¡C90%ªº¦¨¤H¦³°ª¦åÀ£¡AµM¦ÓµÇ¤W¸¢¯À¤]¥i¯à¤Þ°_°¸µo©Ê§C¦åÀ£¤Î©ü³Ö¡C80¢Hªº¯f¤H¦³ÀYµh¡A70¢H·|«_¦½¡A60¢H¦³¤ß±ª¡A50¢H·|µJ¼{¤£¦w¡A40¢H·|¤â§Ý¡A¬Æ¦Ü¥X²{·Pı²§±`¡B¤ßµ±µh¡B¸¡µh©Îµøı§ïÅÜ¡C¦ý¬O¡AMEN
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desensitization)³y¦¨¦åºÞ¤º®e¿n´î¤Ö·|¾ÉP«º¶Õ©Ê§C¦åÀ£¡F¦³¨Ç¯f¤H¦]¬°¥h±Ó·P¡B@¨ü(tolerance)¡B©Îtachyphylasix¡A§Y¨Ï¦å¤¤catecholamine¹L°ª¦åÀ£¤]¥¿±`¡C¦¹¥~¡A¦å¤¤catecholamine¿@«×¹L°ª¤]·|¾ÉP¥ª¤ß«ÇªÎ¤j¡B¤ß¦Ùª¢¡BÂX±i«¬¤ß¦Ù¯f(dilated
cardiomyopathy)¡A¦pªG¨S¦³³y¦¨¤ß¦ÙÅÖºû¤Æ¡A©ó¸~½F¤Á°£«á¥i¥H§¹¥þ«ì´_¡C
¥Í¤ÆÀˬd³q±`¬O´ú¦å¼ß©M§¿²G¤¤ªºcatecholamine¤Îmetanephrine(catecholamineªº¥NÁª«)¡C¥Ø«e¥H´ú§¿²Gmetanephrineªº±Ó·P©Ê³Ì°ª¡A¬°97¢H¡A¦ý¬O¦]¬°¹êÅç«Çªº¨î¡A§Ú̪º¯f¤H¥u¦³´ú§¿²Gnorepinephrine¡Bepinephrine¡BVMA¤Îdopamine¡A«o¤]¤w¸g¨¬°÷¶EÂ_¤F¡CMEN
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< References
>
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