¼±Åà - È­»ìǥŰ/¿£ÅÍŰ ´Ý±â - ESC

 

Pheochromocytoma

º¸Åë adrenal medulla¿¡¼­ ¹ß»ýÇÏÁö¸¸ chromaffin cell ¶Ç´Â sympathetic ganglia

(extraadrenal pheochromacytoma or paragangliomas)¿¡¼­ ¹ß»ýÇÒ¼öµµ ÀÖ´Ù.

HypertensionÀÌ °¡Àå ÈçÇÑ ÀÓ»ó¼Ò°ßÀ̸ç hypertensive paroxysm or crisis°¡ ȯÀÚÀÇ

¹Ý À̻󿡼­ »ý±ä´Ù. pheochromocytoma´Â Àüü °íÇ÷¾ÐÀÇ 0.1%¸¦ Â÷ÁöÇÏÁö¸¸ ±³Á¤°¡´ÉÇÑ

Áß¿äÇÑ ¿øÀÎÀÌ´Ù.

1. º´¸®

1) À§Ä¡ ¹× ÇüÅÂ

80%°¡ unilateral & solitaryÀ̰í 10%´Â bilateral, 10%´Â extraadrenal¿¡ À§Ä¡ÇÑ´Ù.

¾ÆÀÌ¿¡¼­´Â 1/4(25%)ÀÌ bilateralÀ̰í 1/4(25%)Àº extraadrenalÀÌ´Ù.

solitary lesionÀÏ °æ¿ì¿£ right side¿¡ ´õ Àß »ý±ä´Ù.

Å©±â´Â 3kgÀÌ»ó ÀÚ¶ó±âµµ ÇÏÁö¸¸ ´ëºÎºÐÀº 100g ¹Ì¸¸À̰í Á÷°æÀº 10cm ¹Ì¸¸ÀÌ´Ù.

Á¾¾çÀº highly vascularÇÏ´Ù.

10%¹Ì¸¸ÀÌ malignant

´Ù¸¥ endocrine tumoró·³ pheochromocytomaµµ Á¶Á÷ÇÐÀû ¸ð¾çÀ¸·Î ¾ç¼º°ú ¾Ç¼ºÀ»

±¸º°ÇÒ¼ö ¾ø°í local invasion or distant metastasis°¡ ÀÖÀ» ¶§ ¾Ç¼ºÀ» ÀǹÌÇÑ´Ù.

2) extraadrenal pheochromocytoma

20-40g, 5cm¹Ì¸¸ÀÌ´Ù.

´ëºÎºÐÀº º¹°­³»¿¡ À§Ä¡ÇÑ´Ù: celiac, superior & inferior mesenteric ganglia

´ë·« 10%°¡ thorax¿¡ À§Ä¡Çϸç, UB¿¡´Â 1%, neck¿¡´Â 3%¹Ì¸¸ÀÌ »ý±ä´Ù.

ÀÌ·±°ÍµéÀº ÈçÈ÷ sympathetic ganglia³ª 9th, 10th cranial nerveÀÇ extracranial branch¿¡

»ý±ä´Ù.

3) catecholamine synthesis, storage, and release

pheochromocytoma´Â catecholamine¿Ü¿¡µµ ´Ù¾çÇÑ peptides¸¦ ºÐºñÇϴµ¥ ¿©±â¿¡´Â

endogenous opioids, adrenomedulin, endothelin, erythropoietin, PTH-related protein,

neuropeptide Y, and chromagranin A °°Àº °ÍµéÀÌ ÀÖ´Ù.

* epinephrine, norepinephrine, and dopamine

´ëºÎºÐÀÇ pheochromocytoma´Â norepinephrine, epinephrine µÑ´Ù ºÐºñÇÑ´Ù.

normal adrenal¿¡¼­´Â norepinephrineÀÌ epinephrineº¸´Ù ´õ ¸¹´Ù.

´ëºÎºÐÀÇ extraadrenal pheochromocytoma¿¡¼­´Â norepinephrine¸¸À» ºÐºñÇϸç

epinephrine¸¸À» ºÐºñÇÏ´Â °æ¿ì´Â °ÅÀÇ ¾ø´Ù.

MEN¿¡¼­´Â epinephrine¸¸À» ºÐºñÇÏ´Â °æ¿ì°¡ ¸¹´Ù.

2. Familial pheochromocytoma

5%°¡ autosomal dominant·Î À¯ÀüµÇ´Âµ¥ pheochromocytoma´Üµ¶ ¶Ç´Â MEN type

2a(Sipple's syndrome) or type 2b(mucosal neuroma syndrome), von Hippel-Lindau's

retinal cerebellar hemangioblastomosis, or von Recklinghausen's neurofibromatosis¿Í

°°ÀÌ »ý±ä´Ù.

bilateral adrenal pheochromocytoma´Â familial syndrome¿¡¼­ ÈçÇÏ´Ù.

MEN¿¡¼­ÀÇ pheochromocytoma´Â ¹ÝÀÌ»óÀÌ bilateralÀÌ´Ù. µû¶ó¼­ bilateral

pheochromocytoma°¡ ÀÖ´Â °æ¿ì´Â familial syndromeÀ» °í·ÁÇØ¾ß ÇÑ´Ù.

3. Genetic considerations

¨ç MEN 2A and 2B syndrome : chromosome 10¿¡ À§Ä¡ÇÑ RET protooncogeneÀÇ ÀÌ»ó°ú

°ü·Ã.

RET protooncogeneÀÇ Æ¯Á¤ºÎÀ§ mutationÀº pheochromocytoma¸¦ highly prediction

ÇÒ¼ö ÀÖ´Ù.

¨è von Hippel-Landau(VHL) syndrome : VHL tumor suppressor gene mutation

MEN 2 syndrome¿¡Ã³·³ VHL mutationµµ pheochromocytoma¿Í ¹ÐÁ¢ÇÑ °ü·ÃÀÌ ÀÖ´Ù.

Èï¹Ì·Î¿î »ç½ÇÀº RET protooncogene°ú VHL mutationµÑ´Ù sporadic

pheochromocytoma¿¡¼­ somatic mutationó·³ ÈçÇÏ°Ô ¹ß»ýÇÏÁö´Â ¾Ê´Â´Ù´Â »ç½ÇÀÌ´Ù.

4. ÀÓ»ó Æ¯Â¡

young to midadult life¿¡ °¡Àå ÈçÈ÷ ¹ß»ýÇÏ°í ¿©ÀÚ¿¡¼­ ¾à°£ ´õ ºóµµ°¡ ³ô´Ù.

´ëºÎºÐÀº hypertensive crisis, seizure disorder or anxiety attackÀ» ÀǽÉÇÒ Á¤µµÀÇ

paroxysmal symptoms, º¸ÅëÄ¡·á¿¡ Àß ¹ÝÀÀÇÏÁö ¾Ê´Â °íÇ÷¾ÐÀ¸·Î º´¿øÀ» ã°Ô µÈ´Ù.

1) hypertension

60%´Â sustained hypertensionÀ» °¡Áö¸ç ÀÌÁß ¹ÝÀº crisis or paroxysmÀ» °æÇèÇÑ´Ù.

³ª¸ÓÁö 40%´Â attack½Ã¿¡¸¸ Ç÷¾Ð»ó½ÂÀÌ ÀÖ´Ù.

2) paroxysms or crisis(1/2)

ÈçÇϰųª sporadicÇÏ°Ô ¹ß»ýÇÒ¼öµµ ÀÖ°í, ¼öÁÖ ³»Áö ¼ö°³¿ù°£°ÝÀ¸·Î ¹ß»ýÇÒ¼öµµ ÀÖ´Ù.

½Ã°£ÀÌ °¥¼ö·Ï ºóµµ, Áö¼Ó±â°£, ½ÉÇÑÁ¤µµ°¡ Áõ°¡ÇÑ´Ù.

attackÀÌ ÀÖÀ¸¸é ¼öºÐ¿¡¼­ ¼ö½Ã°£ÀÌ»ó±îÁö Áö¼ÓÇϴµ¥ headache, profuse sweating,

palpitation, and apprehensionÀÌ »ý±ä´Ù.

paroxysmÀº º¹°­³» ³»¿ë¹°ÀÇ À§Ä¡¸¦ À̵¿½ÃŰ´Â activity¿¡ ÀÇÇØ À¯¹ßµÈ´Ù.

attack½Ã anxiety°¡ µ¿¹ÝµÇÁö¸¸ mental or psychological stress°¡ crisis¸¦ À¯¹ßÇÏÁö´Â

¾Ê´Â´Ù.

3) other distinctive clinical features

* orthostatic hypotension

plasma volume°¨¼Ò ¹× blunted sympathetic reflexesÀÇ °á°ú·Î »ý±ä´Ù.

surgery or traumaµ¿¾È hypotension or shockÀÌ ¿¹»óÄ¡ ¾Ê°Ô ¹ß»ýÇÒ¼ö Àִµ¥ ¿©±â¿¡

À§ÀÇ µÎ°¡Áö ÀÎÀÚ°¡ À¯¹ß½ÃŲ´Ù. ÀϺÎȯÀÚ¿¡¼­´Â hypotensive peptide adrenomedullinµµ

ÀúÇ÷¾Ð¿¡ ±â¿©ÇÑ´Ù.

¨ç cardiac manifestations

i) arrhythmia : sinus tachycardia, sinus bradycardia, supraventricular arrhythmia, PVC°¡

º¸°íµÇ¾ú´Ù.

ii) angina and acute MI : coronary artery disease°¡ ¾ø´õ¶óµµ »ý±æ¼ö ÀÖ´Ù.

catecholamineÀÌ myocardial oxygen comsumptionÀ» Áõ°¡½Ã۰í

coronary spasmÀÌ ischemic event¿¡ ±â¿©Çϱ⠶§¹®ÀÌ´Ù.

iii) cardiomyopathy

¨è carbohydrate intolerance(1/2)

¨é hematocrit: plasma volumeÀÇ °¨¼Ò·Î 2Â÷ÀûÀ¸·Î Hct»ó½Â

¨ê other manifestations

PTH-related proteinºÐºñ·Î ÀÎÇÑ hypercalcemia

IL-6ºÐºñ·Î ÀÎÇÑ fever, ESRÁõ°¡

catecholamine-mediated increases in metabolic rate & heat dissipationÀ¸·Î ÀÎÇÑ

temperature»ó½Â

polyuria, rhabdomyolysis

¨ë pheochromocytoma of the UB

micturitionµ¿¾È¿¡ typical paroxysm¹ß»ý

hematuria°¡ ¹Ý¼ö¿¡¼­ º¸À̸ç cystoscopy·Î bladderº®¿¡ ºÙ¾îÀÖ´Â tumor¸¦ °üÂûÇÒ ¼ö

ÀÖ´Ù.

4) adverse drug reaction

i) opiates, histamine, ACTH, saralasin, and glucagon¿¡ ÀÇÇØ fatal paroxysmÀÌ À¯¹ßµÉ ¼ö

ÀÖ´Ù.: tumor¿¡ Á÷Á¢ ÀÛ¿ëÇÏ¿© catecholamineÀ» À¯¸®ÇÔ

ii) methyldopa: nerve endings³»¿¡ ÀúÀåµÈ catecholamineÀ» À¯¸®ÇÏ¿© Ç÷¾Ð»ó½Â

iii) TCA or guanethidine: neuronal uptake¸¦ block½ÃÄÑ circulating catecholaminesÀÇ

effect¸¦ Áõ°¡½ÃÅ´

5) associated disease

¨ç medullary carcinoma of the thyroid: MEN syndrome type 2a and 2b

¨è hyperparathyroidism : MEN 2a

¨é von Hippel-Lindau disease

pheochromocytoma¿Í neurofibromatosisÀÇ µ¿¹ÝÀº ÈçÄ¡ ¾ÊÁö¸¸ neurofibromatosisÀÇ

incomplete formÀÌ pheochromocytoma¿Í µ¿¹ÝµÉ¼ö ÀÖÀ¸¹Ç·Î hypertensionȯÀÚ¿¡¼­

cafe au lait spot, vertebral abnormality, or kyphoscoliosis¿Í °°Àº minor manifestation

ÀÌ º¸À̸é pheochromocytoma¸¦ ÀǽÉÇÑ´Ù.

von Hippel-Lindau disease¿¡¼­ pheochromocytomaÀÇ µ¿¹Ý·üÀº 10-25%³ª µÈ´Ù.

¨ê cholelithiasis(10-20%)

¨ë Cushing's syndrome: pheochromocytoma¿¡¼­ ectopic ACTH secretionÀ¸·Î ÀÎÇÏ¿©

»ý±ä´Ù.

5. Áø´Ü

1) Biochemical test

: vanillylmandelic acid(VMA), metanephrine, free catecholamine

VMA´Â metanephrineÀ̳ª catecholamineº¸´Ù ƯÀ̵µ ¹× ¹Î°¨µµ°¡ ³·´Ù.

À§ÀÇ 3°¡Áö Áß 2°¡Áö¸¦ »ç¿ëÇÏ¿´À» ¶§ Áø´ÜÀÇ Á¤È®µµ¸¦ ³ôÀϼö ÀÖ´Ù.

24½Ã°£ urine sampleÀÌ ³ª°¥ ¶§ collectionÀÇ ÀûÀý¼ºÀ» Æò°¡Çϱâ À§ÇØ creatinineµµ °°ÀÌ

½ÃÇàÇϵµ·Ï ÇÑ´Ù. °¡±ÞÀû ȯÀÚ°¡ È޽ĽÃ, ¾à¹°À» º¹¿ëÇÏÁö ¾Ê´Â »óÅ¿¡¼­, ÃÖ±Ù radiologic

contrast media¿¡ ³ëÃâµÇÁö ¾ÊÀº »óÅ¿¡¼­ ÇÏ´Â °ÍÀÌ ÁÁ´Ù.

¨ç free catecholamine: normal totol urinary catecholamineÀº 100-150 ug/24hrÀÌ´Ù.

pheochromocytomaȯÀÚÀÇ ´ëºÎºÐÀº 250 ugÀ» ³Ñ´Â´Ù.

epinephrineÀ» ÃøÁ¤ÇÏ´Â °ÍÀÌ À¯¿ëÇѵ¥ ±× ÀÌÀ¯´Â adrenal lesionÀÇ °æ¿ì¿¡ epinephrine

secretionÀÌ Áõ°¡Çϸç(>50ug/24hr), MEN°ú µ¿¹ÝµÈ °æ¿ì´Â epinephrine¸¸ Áõ°¡Çϱ⠶§¹®

ÀÌ´Ù.

* false-positive

methyldopa, levodopa, labetalol, sympathomimetic amines

-> 2ÁÖ±îÁö catecholamine excretionÀ» Áõ°¡½ÃŲ´Ù.

sympathoadrenal systemÀÇ ÀÚ±ØÀ¸·Î endogenous catecholamineÀÌ Áõ°¡ÇÒ¼ö ÀÖ´Ù.

ÀÌ¿Í °°Àº »óȲÀ¸·Î´Â hypoglycemia, strenuous exertion, CNS disease with increased

intracranial pressure, severe hypoxia, and clonidine withdrawal

¨è metanephrine & VMA

´ëºÎºÐÀÇ lab¿¡¼­ totoal metanephrineÀÇ normal upper limit´Â 1.3mgÀ̸ç, VMA´Â 7.0mg

ÀÌ´Ù.

pheochromocytoma ´ëºÎºÐÀÇ È¯ÀÚ´Â urinary metabolite°¡ »ó´çÈ÷ Áõ°¡ÇÏ¿© Á¤»óÀÇ 3¹è

ÀÌ»ó Áõ°¡ÇÑ´Ù. metanephrine excretionÀº exogenous and endoenous catecholamine

¹× MAO inhibitorÄ¡·á½Ã¿¡µµ Áõ°¡ÇÒ¼ö ÀÖ´Ù. VMA´Â endogenous and exoenous

catecholamine¿¡ ´ú ¿µÇâÀ» ¹ÞÁö¸¸ carbidopa¿Í °°Àº ¾à¹°¿¡ ÀÇÇØ Áõ°¡ÇÒ¼ö ÀÖ´Ù.

VMA excretionÀº MAO inhibitor¿¡ ÀÇÇØ¼­ °¨¼ÒÇÑ´Ù.

¨é plasma catecholamine

plasma catecholamineÀº Á¦ÇÑÀûÀ¸·Î »ç¿ëµÈ´Ù. urinary catecholamine ÃøÁ¤ÀÌ ´õ

¸¸Á·½º·´´Ù.

ÀÓ»óÁõ»óÀº pheochromocytoma°¡ Àǽɵdzª uriary assay´Â borderlineÀÏ ¶§ plasma

catecholamineÃøÁ¤ÀÌ °¡Ä¡ÀÖ´Ù. Å©°Ô Áõ°¡ÇÑ total catecholamineÀº Áø´Ü¿¡ µµ¿òÀÌ µÈ´Ù.

sympathetic system activity¸¦ ¾ïÁ¦ÇÏ´Â ¾àÁ¦¿¡ ÀÇÇØ °Ë»çÀÇ À¯¿ë¼ºÀÌ Áõ°¡ÇÑ´Ù.

clonidine & ganglionic blocker´Â Á¤»óÀΰú essential hypertension¿¡¼­ plasma

catecholamine levelÀ» °¨¼Ò½ÃŰ³ª pheochromocytomaȯÀÚ¿¡¼­´Â catecholamine level

¿¡ °ÅÀÇ ¿µÇâÀ» ³¢Ä¡Áö ¾Ê´Â´Ù.

elevated or borderline catecholamine level ȤÀº clonidineÅõ¿©ÈÄ plasma or urinary

catecholamineÀÌ ¾ïÁ¦µÇÁö ¾Ê´Â °ÍÀº pheochromocytomaÁø´Ü¿¡ µµ¿òÀ» ÁØ´Ù.

¨ê pharmacologic test

i) phentolamine : Åõ¿© 2ºÐÈÄ Àû¾îµµ 35/25 mmHgÀÇ Ç÷¾ÐÀÌ °¨¼ÒÇÏ¿© 10-15ºÐ°£ Áö¼ÓÇÑ´Ù.

ÀÌ·¯ÇÑ ¾à¹°ÀÇ ¹ÝÀÀÀº Áø´ÜÀûÀÌÁö ¸øÇϸç biochemical confirmationÀÌ Áø´Ü¿¡ ÇʼöÀûÀÌ´Ù.

ii) provocation test in normotensive patient

: potentially dangerous and rarely indicated

±×·¯³ª glucagon provocative test´Â aroxysmal hypertension ¹× nondiagnostic basal

catecholamine levelÀ» °¡Áö´Â ȯÀÚ¿¡¼­ »ç¿ëµÉ¼ö ÀÖ´Ù.

glucagonÀº Á¤»ó ȤÀº °íÇ÷¾Ð ȯÀÚ¿¡¼­ BP or plasma catecholamine level¿¡ °ÅÀÇ ¿µÇâÀÌ

¾ø´Ù.

±×·¯³ª pherochromocytoma¿¡¼­´Â BP or circulating catecholamine level¸ðµÎ¸¦

Áõ°¡½ÃŲ´Ù.

6. °¨º°Áø´Ü

¨ç pressor crisis d/t clonidine withdrawal & cocaine abuse or MAO inhibitor

Áø´ÜÀÌ ¾î·Á¿î °æ¿ì¿¡ clonidine suppression & glucagon stimulation test±î À¯¿ëÇϰÔ

»ç¿ëµÉ ¼ö ÀÖ´Ù. clonidine withdrawal & cocaine or MAO inhibitor»ç¿ë°ú °ü·ÃÇÏ¿© pressor

crisis°¡ ¿Ã ¼ö Àִµ¥ ÀÌ´Â pheochromocytomaÀÇ paroxysm°ú ºñ½ÁÇÏ´Ù.

¨è factitious crisis´Â Á¤½Å°úÀûÀ¸·Î ¹®Á¦°¡ Àִ ȯÀÚ°¡ sympathomimetic aminesÀ» ÀÚ°¡

Åõ¿©ÇÔÀ¸·Î½á ¹ß»ýÇÏ´Â °ÍÀÌ´Ù.

¨é intracranial lesion(ƯÈ÷ posterior fossa tumor or SAH)ÀÌ hypertensionÀ» ÀÏÀ¸Å³ ¼ö

ÀÖÀ¸¸ç catecholamine ¹× catecholamine metabolites excretionÀÌ Áõ°¡µÉ¼ö ÀÖ´Ù.

7. Ä¡·á

1) preop management

ÀÏ´Ü Áø´ÜÀÌ È®Á¤µÇ¸é phenoxybenzamine(noncompetitive ¥á-receptor blocker)À¸·Î

Ä¡·á¸¦ ½ÃÀÛÇÑ´Ù. 10mg q 12hrÀ¸·Î ½ÃÀÛÇÏ¿© Ç÷¾ÐÀÌ Á¶ÀýµÇ°í paroxysmÀÌ ¾ø¾îÁú ¶§±îÁö

¼öÀϸ¶´Ù 10-20mg¾¿ Áõ·®ÇÑ´Ù. ´ëºÎºÐ ȯÀÚ´Â 40-80mg»çÀ̰¡ ÇÇ·áÇÏ´Ù.

¼ö¼úÀü¿¡ phenoxybenzamineÀ» Àû¾îµµ 10-14Àϰ£ Åõ¿©Çϵµ·Ï ÇÑ´Ù. phenoxybenzamine

°°Àº ¥á-blocker¸¦ ±¸ÇϱâÀü±îÁö oral prazocin or noncompetitive IV pentolamineÀ¸·Î

Ä¡·áÇÒ¼ö ÀÖ´Ù.

nitroprusside, calcium channel blocker´Â ¥á-blockade°¡ À¯µµµÈ ÈÄ¿¡¸¸ Åõ¿©ÇÒ¼ö Àִµ¥

¸¸¾à ¸ÕÀú Åõ¿©ÇÑ´Ù¸é beta-mediated vasodilationÀÌ ±æÇ׵Ǹ鼭 Ç÷¾ÐÀÌ paradoxicÇϰÔ

Áõ°¡ÇÒ¼ö Àֱ⠶§¹®ÀÌ´Ù.

¥â-blocker´Â ¥á-blockadeÈÄ¿¡ tachycardia¸¦ Á¶ÀýÇϱâ À§ÇØ ÈçÈ÷ »ç¿ëµÇ¸ç Àú¿ë·®À̸é

ÃæºÐÇÏ´Ù.

2) tumor localization

¨ç CT or MRI : intraadrenal lesionÀ» È®ÀÎÇϴµ¥ À¯¿ë

chest³»ÀÇ extraadrenal lesionÀ» È®ÀÎÇϴµ¥´Â chest CT»ç¿ë

¨è aortography: MRI°¡ º¹°­³»ÀÇ extraadrenal tumor¸¦ localizationÇϴµ¥ ½ÇÆÐÇÏ¿´´Ù¸é

aortography¸¦ ½ÃÇàÇØ º¼¼ö Àִµ¥ pheochromocytoma´Â ¸¹Àº aberrant artery·ÎºÎÅÍ

supply¸¦ ¹Þ±â ¶§¹®ÀÌ´Ù.

¨é venous sampling: CT, MRI, aortography°¡ ¸ðµÎ ½ÇÆÐÇÏ¿´´Ù¸é ¼­·Î ´Ù¸¥ level¿¡¼­

venous samplingÇÏ¿© catecholamine gradient¸¦ È®ÀÎÇÑÈÄ selective aortography³ª

CT, MRI¸¦ ´Ù½Ã °Ë»çÇØº¼¼ö ÀÖ´Ù.

¨ê 131I-MIBG scan

extraadrenal pheochromocytoma¸¦ localizationÇÏ´Â °Í»Ó¸¸ ¾Æ´Ï¶ó

biochemical confirmationÀÌ È®Á¤ÀûÀÌÁö ¾ÊÀ»¶§ CT¿¡¼­ ¹ß°ßÇÑ lesionÀ» Ư¼ºÀ» °áÁ¤

Áþ´Âµ¥ À¯¿ëÇÏ´Ù.

¨ë percutaneous needle aspiration: ±Ý±â

3) surgery

surgical mortality < 2-3%

hypertension & cardiac arrhythmia´Â ¸¶ÃëÀ¯µµ, ±âµµ»ð°ü, tumor manipulation½Ã¿¡

°¡Àå Àß »ý±ä´Ù. IV phentolamineÀº BP controlÇϴµ¥ ÃæºÐÇÏÁö¸¸ ¶§·Î´Â nitroprusside°¡

ÇÊ¿äÇÒ ¼öµµ ÀÖ´Ù. tachydardia¿Í ventricular ectopy¸¦ controlÇϱâ À§ÇØ propranololÀ»

»ç¿ëÇÑ´Ù.

4) pheochromocytoma in pregnancy

ÀÓ½ÅÃʱ⿡´Â phenoxybenzamineÀ» ÁغñÇÑÈÄ °¡´ÉÇÑ Áø´ÜÈÄ »¡¸® ¼ö¼úÇÑ´Ù.

ÀϺη¯ À¯»ê½Ãų ÇÊ¿ä´Â ¾øÀ¸³ª ¼ö¼ú ÀÚü·Î ÀÎÇØ ÀÚ¿¬À¯»êµÉ¼ö ÀÖ´Ù.

3rd trimester¿¡¼­´Â adrenergic blocking agent°¡ »ç¿ëµÉ¼ö ÀÖ´Ù.

žư¡ ÃæºÐÈ÷ Å©¸é C-secÈÄ tumor extirpationÇÑ´Ù.

antepartum diagnosis½Ã »ê¸ðÀÇ »ç¸Á·üÀº nonpregnant pheochromocytomaº¸´Ù ³·Áö¸¸

žÆÀÇ »ç¸Á·üÀº ¿©ÀüÈ÷ ³ô´Ù.

5) unresectable and malignant tumors

malignant pheochromocytoma´Â ÁÖ·Î retroperitoneum¿¡ À§Ä¡Çϸç bone & lungÀ¸·Î

metastasis Àß ÇÑ´Ù. radiotherapy¿¡ resistantÇÏÁö¸¸ CTx¿Í combinationÇÏ¿© Á¦ÇÑÀûÀ¸·Î

¼º°øÀ» º¸À̱⵵ ÇÑ´Ù.

131I-MIBG´Â uptake°¡ Àß ¾ÈµÇ¹Ç·Î Ä¡·á¿¡ µµ¿òÀÌ ¾ÈµÈ´Ù.

8. Prognosis & follow-up

¼ö¼úÈÄ 5³â »ýÁ¸·üÀº 95%ÀÌ»óÀ̸ç Àç¹ß·üÀº 10% ¹Ì¸¸ÀÌ´Ù.

¼º°øÀûÀÎ Ä¡·áÈÄ catecholamine excretionÀº 2ÁÖ³» Á¤»óÈ­µÈ´Ù.

malignant pheochromocytomaÀÇ 5³â »ýÁ¸·üÀº 50% ¹Ì¸¸ÀÌ´Ù. ¿ÏÀüÇÑ ÀýÁ¦ÈÄ 3/4¿¡¼­

°íÇ÷¾ÐÀÌ ¿ÏÄ¡µÇ³ª ³ª¸ÓÁö 1/4¿¡¼­´Â °íÇ÷¾ÐÀÌ Àç¹ßÇÏÁö¸¸ ÀϹÝÀûÀÎ Ç×°íÇ÷¾Ð¾àÁ¦·Î Àß

Á¶ÀýµÈ´Ù. ÀÌ·± ȯÀÚµéÀº underlying essential hypertensionÀ̳ª catecholamine-induced

irreversible vascular damage°¡ ÀÖ¾î °íÇ÷¾ÐÀ» Áö¼ÓÀûÀ¸·Î ÀÏÀ¸Å°´Â °ÍÀÌ´Ù.