Evaluation of Sellar Masses - Lieberman's...

Where Has My Vision Gone? Where Has My Vision Gone? Evaluation of Evaluation of SellarSellar LesionsLesions

Caleb Caleb StowellStowell, HMS III, HMS IIIGillian Lieberman, MDGillian Lieberman, MD

November 2008November 2008

ObjectivesObjectives• Present a case highlighting the clinical presentation and

evaluation of a sellar lesion

• Review the anatomy of the sella

• Discuss the differential diagnosis of sellar lesions and show radiological examples

Index PatientIndex PatientHPI:• M.C. is a 56 year old woman with a history of diabetes,

hypertension, and glaucoma who presented to the ophthalmology clinic for interim evaluation of her of glaucoma and diabetic retinopathy

• On routine exam, new onset visual acuity loss and bitemporal field cuts were noted

• The patient was referred for imaging

Visual PathwaysVisual Pathways

Netter’s Anatomy, 4th Ed.www.netteranatomy.com

• The temporal portions of the visual fields pass through the pupil and are projected onto the nasal portion of the retina

Visual PathwaysVisual Pathways• The temporal portions of the visual

fields pass through the pupil and are projected onto the nasal portion of the retina

• The neuronal impulses from the nasal retinas exit the eye via the optic nerve and proceed to the optic chiasm, where they cross en route to the opposite visual cortex

• This crossing point, the optic chiasm, is the only point where both temporal fields can be affected simultaneously in isolation of the rest of the eye.

• This localizes our patient’s symptoms to the optic chiasm

The The SellaSella (Saddle)(Saddle)

Image courtesy Dr. Moonis

• The optic nerves exit the orbit via the optic canal

• The nerve fibers then cross at the optic chiasm which lies above a bony valley termed the sella (saddle)

• The majority of pathologies that affect the optic chiasm arise from anatomic structures in and around the sella

Anatomy of Anatomy of SellaSella -- SagittalSagittal

• Some important anatomical structures include:– Optic Chiasm

• Some important anatomical structures include:– Optic Chiasm– Hypothalamus

• Some important anatomical structures include:– Optic Chiasm– Hypothalamus– Infundibulum

• Some important anatomical structures include:– Optic Chiasm– Hypothalamus– Infundibulum– Adenohypophysis

• Some important anatomical structures include:– Optic Chiasm– Hypothalamus– Infundibulum– Adenohypophysis– Neurohypophysis

• Some important anatomical structures include:– Optic Chiasm– Hypothalamus– Infundibulum– Adenohypophysis– Neurohypophysis– Mamillary body

• Some important anatomical structures include:– Optic Chiasm– Hypothalamus– Infundibulum– Adenohypophysis– Neurohypophysis– Mamillary body– Frontal Lobe

Anatomy of Anatomy of SellaSella -- CoronalCoronal• Other important

structures include: – Internal carotids

structures include: – Internal carotids– Cavernous sinus

structures include: – Internal carotids– Cavernous sinus– Cranial nerves

structures include: – Internal carotids– Cavernous sinus– Cranial nerves– Sphenoid sinus

DdxDdx of of SellarSellar LesionsLesions

• The differential diagnosis of sellar lesions is broad, but can be organized into 4 categories:– Congenital– Neoplastic– Vascular– Inflammatory/Infectious

DdxDdx of of SellarSellar LesionsLesions• Congenital

– Rathke’s cleft cyst– Arachnoidal cyst– Ectopic posterior lobe

• Neoplasms– Pituitary adenoma– Meningioma– Craniopharyngioma– Germ cell tumor– Schwannoma/Neurinoma– Hypothalamic hamartomas– Metastases

• Vascular– Aneurysm– Cavernous sinus thrombosis– Infarction/Hemorrhage

• Inflammatory/Infectious– Sarcoidosis– Lymphocytic hypophysitis– Granulomatous hypophysitis– Pituitary abcess– Dermoid and epidermoid

tumors– Tolosa-Hunt syndrome

Presentation of Presentation of SellarSellar LesionsLesions

• Because the differential diagnosis of sellar lesions is broad, it is important to use clinical clues to narrow the diagnosis before imaging

• Sellar lesions typically present clinically in one of three ways:– Hormonal abnormalities (functional tumors)– Neurologic symptoms (mass effect of non-functional

tumors)– Incidental findings

• Hormonal abnormalities: hyper or hypo hormonal states– Hyper: usually due to functional adenoma

• Hyperprolactinemia - lactotroph neoplasia or stalk compression leading to loss of dopamine inhibition

• Hyperthyroidism – thyrotroph neoplasia• Gigantism/Acromegaly – somatotroph neoplasia• Cushing’s disease – corticotroph neoplasia

– Hypo: usually tumor mass effect causing glandular compression

• Ranges from loss of single hormone – usually LH/FSH first – to panhypopituitaryism

• Neurologic Symptoms: due to mass effect of lesion– Visual field cuts, visual acuity loss, headaches– Principal presenting feature of all lesions besides

functional adenomas• Incidental Findings

– Picked up on CT/MRI done for another reason

Menu of TestsMenu of Tests• MRI: Primary diagnostic tool

– Multiplanar – can acquire in coronal, sagittal, and transverse planes

– High contrast of soft tissues that lie in and surround the sella

• CT: Useful as adjunct– Look for calcifications, bony erosions– Evaluate bony structure in preparation for surgery

TodayToday’’s Focuss Focus• Due to time constraints, I will focus my discussion to

three of the most common sellar lesions:– Pituitary Adenomas– Meningiomas– Craniopharyngiomas

• Our index patient has one of these three diagnoses, and hopefully after our discussion, you will be able to tell which one!

Pituitary AdenomaPituitary Adenoma• Most common sellar pathology (estimated incidence of

27% at autopsy)• Classified by

– Size: micro <10 mm or macro >10 mm– Functionality: hormone producing or not

• Functional adenomas usually present early (microadenoma) due to hormonal abnormalities

• Non-Functional adenomas typically present late (macroadenoma) with neurologic symptoms caused by mass effect

• Non-functional and prolactinoma most common (30% each) followed by GH, ACTH, TSH, LH, FSH (last 3 very rare)

Companion Pt #1:Companion Pt #1: Pituitary Pituitary MicroadenomaMicroadenoma on MRIon MRI

• Note the round 9 mm by 9 mm pituitary microadenoma in the left side of the pituitary

• Distinguishing features of pituitary microadenomas include:– Hypo or isointense to rest of

pituitary on T1

T1 CoronalImage courtesy Dr. Moonis

• Note the round 9 mm by 9 mm pituitary microadenoma in the left side of the pituitary

• Distinguishing features of pituitary microadenomas include:– Hypo or isointense to rest of

pituitary on T1

– Decreased contrast uptake relative to rest of pituitary

T1 Coronal Post Contrast

• Notice the beautiful nearby anatomy that is relatively unaffected by the small adenoma– Optic chiasm

• Notice the beautiful nearby anatomy that is relatively unaffected by the small adenoma– Optic chiasm– Internal carotids

Companion Pt #2: Companion Pt #2: CraniopharyngiomaCraniopharyngioma

• Neoplasia of squamous epithelium of Rathke’s pouch

• Bimodal distribution:– 5-15 years old – typically present

with growth restriction due to mass effect causing panhypopituitarysm

– 60 + – typically present with neurologic symptoms due to mass effect

• Two histological sub-types:– Adamantinomatous

• cholesterol rich cysts (resemble “crank oil” on pathology)

• calcifications– Papillary

• Lack calcifications or cysts

http://www.med.mun.ca/anatomyts/head/pit.htm

Companion Pt #2: Companion Pt #2: CraniopharyngiomaCraniopharyngioma on MRIon MRI

• Note the large supersellar mass compressing the hypothalamus above and the pituitary below

• Distinguishing features of craniopharyngiomas include:– Suprasellar in origin

Sagittal T1

Companion Pt #2: Companion Pt #2: CraniopharyngiomaCraniopharyngioma on CTon CT

• Distinguishing features of craniopharyngiomas include:– Suprasellar in origin– Calcifications on CT

Axial CT

Companion Pt #2: Companion Pt #2: CraniopharyngiomaCraniopharyngioma on MRIon MRI

• Distinguishing features of craniopharyngiomas include:– Suprasellar in origin– Calcifications on CT– Cystic and solid

components– Enhances with contrast Transverse T1 Post Contrast

MeningiomaMeningioma• Typically benign neoplasm arising from meningothelial

cell of arachnoid matter

• Sellar region accounts for 20-30% of all intracranial meningiomas

Companion Pt #3: Companion Pt #3: MeningiomaMeningioma on MRIon MRI

• Note the large suprasellar mass

• Distinguishing features of meningiomas include:– Typically suprasellar

Sagittal T1

• Distinguishing features of meningiomas include:– Typically suprasellar– Rim of CSF

Sagittal T1

• Distinguishing features of meningiomas include:– Typically suprasellar– Rim of CSF– Dural tail (not seen here)

Sagittal T1

• Distinguishing features of meningiomas include:– Typically suprasellar– Rim of CSF– Dural tail (not seen here)– Sella turcica only mildly

enlarged

Sagittal T1

• Distinguishing features of meningiomas include:– Typically suprasellar– Rim of CSF– Dural tail (not seen here)– Sella turcica only mildly

enlarged– Early contrast

enhancementTransvere T1 Post Contrast

Back to Our PatientBack to Our Patient……

• Now that we’ve seen some examples of sellar neoplasms, let’s return to our patient and see if we can figure out what’s causing her visual symptoms

Our Patient:Our Patient: SellarSellar Mass on MRIMass on MRI

PACS, BIDMCSagittal T1

• Note the sellar mass with the following distinguishing features:

• Note the sellar mass with the following distinguishing features:– Sellar enlargement

Our Patient:Our Patient: SellarSellar Mass on CTMass on CT

PACS, BIDMC

Sagittal CT

• Note the sellar mass with the following distinguishing features:– Sellar enlargement

• Note the sellar mass with the following distinguishing features:– Sellar enlargement– Isointense to brain– No cysts

PACS, BIDMC

Sagittal T1 Post Contrast

• Note the sellar mass with the following distinguishing features:– Sellar enlargement– Isointense to brain– No cysts– Heterogenous uptake of

contrast

Our Patient:Our Patient: Pituitary Mass on MRIPituitary Mass on MRI

PACS, BIDMC

• These findings are classic for a pituitary macroadenoma, here’s why:– Sellar enlargement – suggests sellar origin of mass

that has caused remodeling of the bony sella due to chronic pressure

– Isointense to brain – suggests soft tissue pathology– No cysts – suggests against craniopharyngiomas or

rathke’s cleft cysts– Heterogenous uptake of contrast – suggests

intermixing of neoplastic hypovascular tissue with normal vascular pituitary parenchyma

Our Patient: Our Patient: What Next?What Next?

• Now that we have a preliminary diagnosis, we can move to laboratory studies to assess functionality of the pituitary adenoma

• Labs:– Prolactin – 182 (nl <25)– TSH nl– Free T4 low– GH, ACTH, IGF-1 nl

• Our patient’s laboratory abnormalities are relatively non-specific– Prolactin >200 is usually functional adenoma– Prolactin < 200 can be functional or due to stalk compression– Subclinical hypothyroidism (nl TSH, low T4 could be advancing age or

due to compression of pituitary)

Our Patient:Our Patient: Treatment CourseTreatment Course

• Considering the possibility of a functional prolactinoma, the pt was placed on Bromocriptine for 3 weeks– Repeat prolactin – 1.4– Substantial improvement in vision noticed

• However, a repeat MRI 4 months later showed no change in the size of the adenoma

• Trans-sphenoidal resection was offerred and accepted• After surgery, the patient remarkably reported complete

return of vision and no longer needed to wear glasses• She also reported cessation of headaches that she had

long attributed to something else

Our Patient:Our Patient: PostPost--Surgery MRISurgery MRI

PACS, BIDMCCoronal T1

• Note the following findings after surgery:– Hypointense mass in the

left pituitary

PACS, BIDMCCoronal T1

• Note the following findings:– Hypointense mass in the

left pituitary– Decompression of the

cavernous sinus and optic chiasm

PACS, BIDMC

• Note the following findings:– Hypointense mass in the

left pituitary– Decompression of the

cavernous sinus and optic chiasm

– Hypointense post contrast due to decreased vascularity

Coronal T1 Post Contrast

SummarySummary• Sellar lesions are common and typically present with:

– hormonal abnormalities due to functional adenomas or stalk compression

– neurological symptoms due to mass effect

– neurological symptoms due to mass effect• The differential diagnosis of sellar lesions include:

– congenital– neoplastic– vascular– inflammatory/infectious pathologies

• MRI imaging coupled with laboratory studies can help differentiate sellar pathology

• Medical treatment can be successful in select cases (prolactinomas), but surgery is often required for lesions causing symptoms by mass effect

AcknowledgementsAcknowledgements

• Dr. Gillian Lieberman• Dr. Gul Moonis• Dr. Brian Callahan• Maria Levantakis

ReferencesReferences• Snyder, Peter J et al. Causes, presentation, and evaluation of sellar

masses. UpToDate. Accessed on 11/7/08.• Synder, Peter J et al. Clinical Manifestations and Diagnosis of

Hyperprolactinemia. UpToDate. Accessed on 11/15/08.• Kumar, Jyoti et al. Magnetic Resonance Imaging of Sellar and

Suprasellar Pathology: A Pictorial Review. Curr Probl Diagn Radiol. 2007; 36:227-36.

• Rennert, Janine et al. Imaging of sellar and parasellar lesions. Clinical Neurology and Neurosurgery. 2007; 109:111-124.

• Kumar, Vinay et al. Robbins and Cotran Pathologic Basis of Disease, 7th Edition. Philadelphia, PA: Elsevier, 2005.

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