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< REVIEW > 다양한 임상양상을 보이는 Pituitary Gland Mass 내분비대사 내과 R 3 이 < REVIEW > 다양한 임상양상을 보이는 Pituitary Gland Mass 내분비대사 내과 R 3 이 윤 정

Normal pituitary gland Normal pituitary gland

Contents Pituitary gland mass Acromegaly Central diabetes insipidus Contents Pituitary gland mass Acromegaly Central diabetes insipidus

Classification n n n n Pituitary adenomas : most common cause of sellar masses Classification n n n n Pituitary adenomas : most common cause of sellar masses , 10 percent of all intracranial neoplasms q Microadenoma , Macroadenoma Pituitary hyperplasia Benign tumor : craniopharyngiomas and meningiomas Malignant tumors : germ cell tumors, sarcomas, chordomas, and lymphomas Metastatic disease Cysts : Rathke's cleft, arachnoid, and dermoid cysts Abscess Arteriovenous fistula of the cavernous sinus Lymphocytic hypophysitis

Pituitary adenomas <Harrison 16 th. > Pituitary adenomas

Clinical manifestation n n Neurologic symptoms : visual impairment or headache Abnormalities related to Clinical manifestation n n Neurologic symptoms : visual impairment or headache Abnormalities related to undersecretion or oversecretion of pituitary hormones Impacted Structure Clinical Impact Pituitary Hypogonadism Hypothyroidism Growth failure and adult hyposomatotropism Hypoadrenalism Optic chiasm Loss of red perception Bitemporal hemianopia Superior or bitemporal field defect Scotoma Blindness Cavernous sinus Opthalmoplegia ± ptosis or diplopia Facial numbness

Evaluation of sella mass n Radiologic procedures q Magnetic resonance imaging (MRI) q CT Evaluation of sella mass n Radiologic procedures q Magnetic resonance imaging (MRI) q CT scan : craniopharyngioma or meningioma is seen better by CT scan than by MRI n Hormonal hypersecretion q Hyperprolactinemia : elevated of prolactin q Acromegaly : measurement of serum insulin-like growth factor (IGF)-I, oral glucose tolerance test with GH q Cushing's syndrome : Elevated 24 hour urine cortisol excretion , highnormal or high ACTH concentration q Gonadotroph adenomas n Hormonal hyposecretion : Central diabetes insipidus , Hypopituitarism n Pituitary incidentaloma

Evaluation of sella mass <Harrison 16 th. > Evaluation of sella mass

Contents Pituitary gland mass Acromegaly Central diabetes insipidus Contents Pituitary gland mass Acromegaly Central diabetes insipidus

Cause of acromegaly <N Engl J Med 2006; 355: 2558 -73> Cause of acromegaly

Clinical feature of acromegaly <N Engl J Med 2006; 355: 2558 -73> Clinical feature of acromegaly

Diagnosis and treatment of acromegaly <N Engl J Med 2006; 355: 2558 -73> Diagnosis and treatment of acromegaly

Giant pituitary adenoma n Giant pituitary adenoma: 뇌하수체 선종의 6∼ 23%를 차지, 조직학적 으로 Giant pituitary adenoma n Giant pituitary adenoma: 뇌하수체 선종의 6∼ 23%를 차지, 조직학적 으로 양성으로 서서히 성장하지만 침습적 성향을 보임. n Yasagil의 분류 : Large pituitary adenoma를 종양의 크기에 따라 moderate(2∼ 4 cm), large(4∼ 6 cm), giant (>6 cm)으로 분류함. n 남녀의 비에서 차이가 없음, 주로 40∼ 50대에서 호발하며, 내분비학적 증상 없이 시야장애가 오는 것이 특징임. n 종양이 터어키안 밖으로 탈출되어 자라는 경우가 대부분이기 때문에 뇌하수체 기능 저하증의 기회는 많지 않음.

<J Korean Neurosurg Soc 28: 1606 -1612, 1999>

<Neurosurgery, Vol. 50, No. 6, June 2002>

Contents Pituitary gland mass Acromegaly Central diabetes insipidus Contents Pituitary gland mass Acromegaly Central diabetes insipidus

Diabetes insipidus of Clinical characteristics n n n Decreased secretion or action of AVP Diabetes insipidus of Clinical characteristics n n n Decreased secretion or action of AVP 24 -h urine volume is >50 m. L/kg body weight and the osmolarity is <300 mosmol/L. Polyuria produces symptoms of urinary frequency, enuresis, and/or nocturia, disturb sleep and cause mild daytime fatigue or somnolence. Thirst and a commensurate increase in fluid intake (polydipsia) Clinical signs of dehydration are uncommon unless fluid intake is impaired

<Pituitary 5: 119– 132, 2002>

1991. 8 ~ 1995. 12 25 subjects with CDI Age : 2 – 58 1991. 8 ~ 1995. 12 25 subjects with CDI Age : 2 – 58 yrs old <대한내분비학회지 제 11권 제 3호 1996 p 285 -292>

79 pts (1970 -1976) Mean 7 yrs old Duration of F/U 7. 6 yrs 79 pts (1970 -1976) Mean 7 yrs old Duration of F/U 7. 6 yrs

MRI scans of the hypothalamicpituitary region in a normal subjects < MRI findings of MRI scans of the hypothalamicpituitary region in a normal subjects < MRI findings of Central DI > 1. Thickened pituitary stalk → infiltrative disease : 29 pts (37%) 2. No hyperintense in post. Pituitary region : 74 pts (94%)

MRI scans of the hypothalamicpituitary region in Central DI subjects <N Eng J Med MRI scans of the hypothalamicpituitary region in Central DI subjects

<N Eng J Med , October 5, 2000>

<N Eng J Med 2000; 343: 998 -1007>

Algorithm for diagnosis and management of central diabetes insipidus Gd-DTPA = Gadolinium diethylenetriamine pentaacetic Algorithm for diagnosis and management of central diabetes insipidus Gd-DTPA = Gadolinium diethylenetriamine pentaacetic acid

Special remark n n n Consistent lack of posterior pituitary hyperintensity : cardinal feature Special remark n n n Consistent lack of posterior pituitary hyperintensity : cardinal feature of CDI Thickening of the pituitary stalk : second feature of CDI Anterior pituitary hormone deficits with idiopathic CDI : frequent Progressive reduction of anterior pituitary size : commonly associated with higher risk of additional endocrine defect Increased anterior pituitary size with stalk thickening : strongly associated with presence of germinoma < Horm Res 2003; 59(suppl 1): 42– 54>