Presentation and management of pituitary lesions

1. L E W I S S . B L E V I N S , J R . , M . D .
C A L I F O R N I A C E N T E R F O R P I T U I T A R Y
D I S O R D E R S A T U C S F
Presentation and Management
of Pituitary Lesions

2. Pituitary Lesions
 14-27% of autopsied pituitaries demonstrated adenoma
 Most under 4mm
 40% prolactinomas
 18 per 100,000 will present clinically with a pituitary
adenoma
 I believe a great proportion of those undetected “did” as
well
 Radiographic presentations are the same as above
 22% incidental lesion
 Some of these Rathke’s cleft cysts, pars intermedia cysts
 About 20 per 100,000 with macroadenomas

3. Pituitary Lesions
 What determines clinical significance?
 Hormone hypersecretion
 Hypopituitarism
 Mass effects
 Other effects on patient and contacts

4. Sellar Masses

5. Differential Diagnosis of Sellar and Parasellar Masses
 Pituitary Adenoma
 Craniopharyngioma, Meningioma, Pituicytoma,
Germinoma
 Metastases (breast, lung, melanoma, GBM)
 Rathke’s Cleft Cyst, Arachnoid Cyst, Pars Intermedia
Cyst
 Langerhans Cell Histiocytosis, Sarcoidosis
 Hypophysitis: Lymphocytic, Granulomatous, IgG4-
mediated

6. What are the potential consequences of a sellar mass?
 Mass effects
 Headache
 Visual compromise
 CN palsies
 Epistaxis
 Rhinorrhea
 Abnormalities of pituitary hormone secretion
 Excess
 Deficiency
 Normal pituitary function
 Diabetes insipidus
 Suggests a disease process other than a pituitary adenoma

7. Pituitary Excess
 Hyperprolactinemia
 Acromegaly
 Cushing’s Syndrome
 Hyperthyroidism
Each of these disorders are associated with important
co-morbidities that often require evaluation.

8. Hyperprolactinemia
 Serum PRL level
 Macroadenoma and PRL > 200 ng/mL is almost
always a prolactinoma
 Atypical prolactinomas often associated with PRL <
200 ng/mL
 Microadenomas can be associated with any degree of
PRL elevation
 Drugs and stalk effect
PRL usually <150

9. Hyperprolactinemia
 Medical Management with Dopamine Agonists
 Bromocriptine
 Cabergoline
 90% normalization of PRL
 Tumor regression
 Surgical intervention
 Success dependent on tumor size and invasion
 95% remission in microadenomas
 50-75% remission in macroadenomas

10. Acromegaly
 IGF-1 level almost always elevated
 GH levels not diagnostic but indicative of disease
activity
 Oral glucose suppression test occasionally used to
confirm abnormal GH secretion

11. Management of Acromegaly
 Surgical intervention
 Success dependent on tumor size and invasion
 95% remission in microadenomas
 50-75% remission in macroadenomas
 Debulking can improve situation and lead to better responses to XRT
and medications
 Radiotherapy
 50-75% control in 7-15 yrs
 99% control of tumor growth
 Medical management
 Dopamine Agonists- 10-40% control
 Somatostatin Receptor Agonists- 40-50% control
 GH Receptor Antagonists- 69-94% control

12. Cushing’s Syndrome
 24-h Urine Free Cortisol
 Plasma ACTH level
 Other tests under guidance of an Endocrinologist

13. Management of Cushing’s Syndrome
 Surgical intervention
 Success dependent on tumor size and invasion
 95% remission in microadenomas
 50-75% remission in macroadenomas
 Debulking can result in clinical improvement and responses to XRT
and medical therapy
 Radiotherapy
 50% control in 3-10 years
 Medical Management
 Somatostatin Receptor Agonist
 Adrenal Biosynthesis Inhibitors
 Glucocorticoid Receptor Antagonist

14. Hyperthyroidism
 TSH, free T4 and T3 levels
 TSH usually elevated but may be inappropriately
“normal” in setting of hyperthyroidism

15. Management of TSH–secreting Adenomas
 Surgical intervention
 Success rates depend on size and invasiveness of tumor
 Most are invasive macroadenomas
 Radiotherapy
 Useful to prevent tumor growth
 Slow and low rates of hormonal control
 Medical Therapy
 Somatostatin Receptor Agonists control 90% of patients
 Rarely… beta-blocker and methimazole also required

16. Rathke’s Cleft Cysts
 Surgery indicated when….
 Lesions >1cm
 Lesions associated with mass effects including loss of pituitary
function
 Suspicion of infection
 Surgical intervention
 9% recurrence non-infected cysts
 31% recurrence infected cysts not treated with antibiotics
 13% recurrence infected cysts treated with antibiotics

17. Craniopharyngiomas
 Intrasellar, suprasellar and hypothalamic locations
 Subtotal resection with XRT is favored to spare
neural tissue and especially in children
 Not unusual to require multiple treatments
 Hormone replacement and management of DI are
essential to QoL

18. Pituitary Insufficiency
 Partial or complete loss of one or more anterior pituitary
hormones
 Presentation can range from asymptomatic to severe
hyponatremia and prostration
 Pituitary deficiency leading to target gland deficiencies
 T4 and T3 (Central Hypothyroidism)
 Cortisol (Central Adrenal Insufficiency)
 Sex Steroids (Central Hypogonadism)
 GH deficiency
 PRL deficiency
 Preoperative therapy guided by needs

19. Suggested Tests for any Pituitary Lesion
 PRL
 IGF-1, GH
 TSH, free T4, T3
 Cortisol, ACTH
 24-h Urine Free Cortisol if Cushing’s suspected
 LH and Testosterone in men
 FSH and Estradiol in women with absent or
abnormal menses
 Serum sodium and urine osmolarity if DI suspected

20. California Center for Pituitary Disorders at UCSF
 Multidisciplinary team approach
 Endocrinology, Neuro-ophthalmology, Neuroradiology,
Radiation Oncology, etc
 Surgeons
 Sandeep Kunwar
 Manish Aghi
 Philip Theodosopoulos
 Busiest and most active center in the US
 260-280 surgical cases annually
 Thousands of patient visits annually

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