경희대학교 의과대학·의학전문대학원

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Pituitary Tumors

Division of Endocrinology & Metabolism

Sung-Woon Kim, MD

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Pituitary tumor -

Macroadenoma

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Pituitary tumors

Pituitary adenomas have specific signs and symptoms t

hat are primarily related to the endocrinopathies produ

ced by hypersecretion.

Prolactinoma

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Prolactinoma

The prolactin-secreting pituitary adenomas are the mos

t common, and account for approximately 30% of all p

ituitary tumors.

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She was well with Bromocriptine 2.5 mg (1/2 tab.) QD and had regular menstruation until Aug. 1997

오 O 숙 ( F/45 ) OPD : 98.2.10

She was OPD visit for evaluation of amenorrhea and g alactorrhea. (7-8 yrs duration)

She had no specific medical history and habits except ame norrhea & galactorrhea, but previously checked serum PRL showed over 236 ng/mL.

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Height 152.5 cm Weight 46kg BMI 20.63 kg/m2 Vital signs 120/80 -82 -20 -36.5

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Combined pituitary stimulation test

T3 97 ng/dL T4 6.4 ng/dL E2 122.5 pg/mL Progesterone 0.02 ng/mL Testosterone UD -30 0 30 60 90 120 Glucose 92 27 56 74 88 ACTH (15-90 pg/mL) 17.0 17.9 28.1 204.7 83.7 71.8 Cort (5-13 mg/mL) 8.8 10.9 16.5 26.0 27.5 28.8 GH ( <5 ng/mL) 0.2 0.1 0.3 10.2 7.7 3.6 FSH (1.4-9.6IU/L) 7.1 31.6 50.5 49.1 53.2 LH (0.8-26 IU/L) 11.0 63.7 65.9 61.0 42.9 PRL (2-15 ng/mL) >236 >236 >236 >237 >236

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Bromocriptine 5 mg bid.

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GH producing tumor

The endocrinopathy of excess growth hormone results i

n enlargement of the extremities, face and the soft tissu

es, producing a characteristic appearance called acrome

galy.

Acromegaly can be associated with hypertension, di

abetes mellitus and cardiovascular disease.

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His symptoms were aggravated and checked at local ENT and then, local ENT doctor recommended him to

endocrinologist for acromegalic features.

최 OO 용 (M/44)(M/44)

OPD : 98.2.10 He was well until 20 days before admission, but he had a bout of headache, nausea and vomiting.

He had adult type DM for 5 years, but had ignored to treatment.

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68 68

44 41 38 36 37

15

Review of System

He was suffered from fatigue, sweating, headache, dizziness, Heavy snoring, enlarging hands and feet and decreased libido.

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Physiacal Examination

He had prognathism, moist, warm, and fleshy skin. He had large tongue, widely spaced teeth

Height :173cm Weight: 75 kg BMI: 25.86 kg/m2 V/S : 130/90 mmHg - 80/min - 20/min - 36.6 C

He had been diabetic for 5 years, and his FBS

showed 146mg/dL and postprandial 2 hour glucose, 267 mg/dL

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진단은 ?

1. Acromegaly

2. type 2 DM

R/O secondary D

M

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Dynamic GH profiles

Basal GH secretion

8:30 9:00 9:30 10:00 10:30 11:00

GH 8.9 8.9 9.1 8.9 7.6 8.6

GH suppression test by octreotide

-30 0 30 60 90 120

GH 15 17.1 2.6 1.8 0.7 0.7

GH stimulation test by GHRH

-30 0 30 60 90 120

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Acromegaly

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Cushing’s disease

Cushing's syndrome results from hypercortisolism and is chara cterized by distinctive clinical features.

Patients may develop obesity, hirsutism (abnormal hair distribut ion), purple striae (stripes) on the skin, mental problems, poor wound healing and muscle wasting.

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She was well with antihypertensive regimen for

The last 5 years. One month ago, she had generalized

edema(?), but local doctor suggested Cushing’s syndrome. 김 O 순 ( F/60 )

Adm : 98.7.20 She was suffered from generalized weakness for a

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Systemic Review

She had 5 kg of weight gain since last year (59 to 64 kg), easy fa tigability, generalized and facial edema, easy bruisibility, oligome norrhea and menstrual irregulality.

Physical Examination

Height 153 cm, Weight 64 kg, BMI : 27.3 kg/m2

Vital signs BP 150/90 mmHg TPR 36-74-20

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Impression :

Cushing’s syndrome

Diagnostic plan :

1. 24hr urine free cortisol, plasma ACTH level

2. Screening of 1mg dexamethasone suppression test 3. Confirmatory test

low-dose dexamethasone suppression test high-dose dexamethasone suppression test 4. Pituitary imaging - Sella MRI

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Bone densitometry ; within normal limits.

외부 Abdomen CT ; both renal cysts

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5 Day Dexamethasone suppression test (98-4-18)

Days PlasmaACTH (pg/mL) Serum Cortisol (g/dL) 24 hr. urine 17OHCS 17KS fCORT Cr. 1 Basal 8 am 54.8 13.6 11.4 12.8 222.9 0.6 4 pm 85.9 17.0 3 Low Dose 8 am 53.5 7.1 11.4 7.5 54.6 1.2 4 pm 31.3 2.4 5 High Dose 8 am 21.5 2.3 6.6 2.4 19.2 0.9

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Oral glucose tolerance Test (98-4-24)

0 30 60 90 120

Glucose (mg/dL) 97 195 257 273 273

Insulin (U/mL) 10.1 25.9 44.4

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Inferior Petrosal Sinus Sampling (98-4-28) ACTH (pg/mL) 0 3 6 8 10 Left 28.0 46.3 73.5 82.1 81.9 Right 200.5 >2175 >2175 >2175 >2175 Pph 29.0 33.7 66.2 64.5 74.4 Lt pph. 0.9 1.3 1.1 1.2 1.1 CRH 100 CRH 100 g iv.g iv.

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Diagnosis :

Cushing’s disease Microadenoma

Therapeutic plan :

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Overnight dexamethasone suppression test (98-8-22)

Days PlasmaACTH (pg/mL) Serum Cortisol (g/dL) 24 hr. urine 17OHCS 17KS fCORT Cr. 1 Basal 8 am 15.6 9.3 7.5 6.7 12.2 0.6 4 pm 9.9 3.1 2 Over-night 8 am 6.3 1.7 4.9 2.6 1.7 0.6 4 pm 3.5 1.1

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Oral glucose tolerance Test (98-8-25) 0 30 60 90 120 Glucose (mg/dL) 91 134 153 166 149 Insulin (U/mL) 12.8 28.7 34.8 C-peptide (ng/mL) 4.3 5.1 HbA1C : 6.0%,

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Combined pituitary stimulation test

(98-4-30)

-30 min 0 min 30 min 60 min 90 min 120 min

glucose (mg/dL) 111 48 63 81 84 ACTH (pg/mL) 42.1 38.3 37.2 21.6 20.5 28.1 Cortisol (ug/dL) 13.6 17.0 7.1 2.4 1.3 1.0 GH (ng/mL) 0.7 2.4 1.8 1.2 1.0 1.8 FSH ( IU/L ) 84.7 101.9 113.1 116.3 133.7 LH ( IU/L ) 11.7 63.5 60.2 51.5 26.7 PRL (ng/mL) 19.6 122.8 112.8 79.3 56.7 T3 61 ng/dL T4 4.9 ug/dL TSH 0.12 uU/mL

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MEN 1

Multiple endocrine neoplasia type

1 (MEN1) is an inherited disorder

that affects the endocrine glands.

It is sometimes called multiple end

ocrine adenomatosis or Wermer's

syndrome, after one of the first d

octors to recognize it.

MEN1 is quite rare, occurring in a

bout 3 to 20 persons out of 100,0

00. It affects both sexes equally a

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MEN 2

1. medullary cell carcinoma.(MCT, malignant)

2. phaeochromocytoma.

MEN 2A : 1 + 2 + parathyroid hyperplasia

MEN 2B : 1 + 2 +

neurofibromatosis =MEN 3

In familial MTC, people tend to get medullary thyroid

cancer only.

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