A Primary Care Provider’s Primer for Evaluation of

Pituitary LesionsNicholas B. Levine, MD

Associate Clinical Professor

Department of Neurosurgery

UCSF Fresno

Disclosures

None

Sellar Tumors

• Adenomas most common

• 10-15% of brain tumors

• Incidental imaging finding for sinusitis, headaches and trauma• 0.15-0.3%

• Female predominance in 3rd-6th decade of life

• Rare in children• Craniopharyngioma• Functional and more aggressive

• Ethnic incidences• 2.92/100,000 person years (African American)• 3.55/100,000 person years (Hispanic)• 1.82/100,000 person years (Caucasian)

• Identified in 10% of autopsies

Tumors

• Tumors of the anterior pituitary (adenohypophysis) primarily adenomas

• Rare tumors of posterior pituitary gland (neurohypophysis)

• Other tumors:• Craniopharyngiomas

• Meningiomas

• Metastases

• Lymphoma

• More to come…

Adenomas

• Functional vs. Nonfunctional

• Functional • Prolactinoma (PRL)

• Acromegaly (GH and IGF-1; prolactin)

• Cushing’s (ACTH)

• TSH producing

• 1/3 of adenomas of no clinical or biochemical evidence of hormone excess

• FSH, LH, null cell adenomas and silent adenomas

Presentation

• Local mass effect• Headaches

• Visual deficits

• Cavernous sinus CN deficits

• Stalk effect (hyperprolactinemia)

• Systemic symptoms due to hormone production

• Apoplexy

• Hypophysitis

Vernacular

• Size matters• Microadenoma <1 cm• Macroadenoma >1 cm• Giant Macroadenoma >4 cm

• Growth patterns• Suprasellar extension• Cavernous sinus invasion (relation to carotid)• Sinus

• Pathology• Gross- pink, purple, tan, brown tissue• Histology

• Diffuse, papillary, trabecular, acidophilic, basophilic, chromophobic

• Immunochemistry classification

Occurence

• Sporadic

• Minority are hereditary• MEN-1

• Carney Complex

• McCune-Albright Syndrome

• Rare familial syndromes

Adenoma subtypes

• PRL-secreting• Prolactinomas

• Constitute 80% of functioning adenomas

• 40-50% of all adenomas

• Treat with dopamine agonists

• Bimodal occurrence• Microadenomas in females during reproductive years (oligo and amenorrhea)

• Macroadenomas in men and elderly

• Mass effect with Has and VF deficits

• Men experience decreased libido and impotence

Adenoma subtypes

• GH secreting• 20% of functioning

• Acromegaly and Gigantism (adolescences)

• Similar incidence in women and men

• 40-45 years

• GH and IGF-1 levels increased• 30-50% have cosecretion of Prolactin

• Delay in diagnosis up to 10 years

• Treat with somastatin receptor ligands • Octreotide

• Pegvisemont

Adenoma subtypes

• ACTH secreting• 10-15% of adenomas

• Cushing’s Disease

• 30-40 year old

• 3.5:1 female to male ratio

• Majority are microadenomas

• Petrosal sinus sampling, etc.

Adenoma subtypes

• TSH secreting• Increased TSH levels• Hyperthyroidism or clinically euthyroid patients• Invasive

• FSH and LH• Clinically nonfunctional• 20% of all adenomas• 6th decade of life

• Silent adenomas

Adenoma subtypes

• Atypical adenomas• Ki-67 >3%

• Overexpression of p53

• Pituitary carcinoma• <1% of all tumors

• Metastatic spread by definition

Posterior gland tumors

• Pituicytomas• 5th-6th decade of life

• Male predominance

• Rare DI

• Granular cell tumors

• Infundibular location

Other

• Craniopharyngiomas• 1-2% of intracranial neoplasms• 10% of all sellar/extrasellar tumors

• Inflammatory Lesions• Lymphocytic hypophisitis

• Late pregnancy or postpartum

• Rare in men

• Auto-immune

• Concomitant endocrine or immune problems in 20% of patients

• Expanding mass with stalk effect

• Posterior gland involvement resulting in DI

• Granulomatous hypophysitis• Middle age or elderly women

• Hypopituitarism

• Lymphocytes and plasma cells

• Fibrosis

• Xanthomatous hypophysitis

• Chemotherapy induced hypophysitis in melanoma• Ipilimumab

• Nivolumab

What can I do to facilitate the work-up?• Endocrinological Review of Systems

• Endocrine panel• Prolactin• LH• FSH• TSH• GH• IGF-1• Cortisol (AM)• ACTH

• Referral to an endocrinologist

Ophtahalmologic evaluation

• In 1983, Montreal study found 60% of macroadenomas had visual field deficits

• Lower today due to earlier identification

• With intervention, substantial improvement • Normalization of VF

• Anatomically persistent optic atrophy and optical coherence tomography changes

• Document pre and post treatment vision

Ophthalmologic evaluation

• Evaluation to include:• Quantitate psychophysical acuity and visual fields• Functional APD and VEP• Anatomic disc appearance and OCT

• Automated static perimetry• Subtle bitemporal VF defects• Homonymous defects• Infrequent arcuate defects

• Bedside VF testing is good starting point (Do it, please!)

• Recovery of vision related to duration of visual symptoms• > 4 months of visual changes have decreased chance of recovery• Greatest recovery in first 3 months after treatment with improvement up to a

year

Neurosurgical referral

• Surgical management • Endoscopic transphenoidal resection

• Sublabial

• Transnasal• Speculum and microscope

• Team sport• ENT or OMFS

• Neurosurgeon

Monitoring

• Postoperative imaging with Neurosurgery follow-up• Immediate• 3-6 months• Annually for first 3 years• Biannually at year 4

• OMFS/ENT (sinus and smell/taste)

• Endocrine follow-up

• Ophthalmology follow-up

• 50% of patients report complete resolution of headaches• Multifactorial

Recurrences

• Observation

• Repeat surgery vs. radiation

• Radiation to include SRS, SRT and XRT• ~50% develop degree of hypopituitarism

Pick up the phone

• Team of phsysicians to assist in work-up, management, and maintenance