Practice guidelines for the evaluation and
treatment of pituitary incidentalomas
A case-based imaging review
Narayana S Mamillapalli MD; Marco Pinho MD; William A.
Moore MD; Thomas O’Neill MD; Abreu, Marconi MD
UT Southwestern Medical Center,
Dallas.
PURPOSE
•
To increase awareness within the
Neuroradiology community of the evidence
based current clinical guidelines for evaluation
and treatment of pituitary incidentalomas
provided by The Endocrine Society.
PART I – PRE TEST
Let’s try out a few cases first to evaluate your baseline knowledge (or
intuition) before reviewing the formal guidelines…
Pick the best option mentally and you’ll have a chance to review these
cases later with the correct answers…
Case 1
61 yr old female found to have a pituitary lesion during
imaging work-up for headaches
FINDINGS ?
What are the most likely diagnostic possibilities ?
Round, subcentimetric lesion within the left aspect of the pituitary gland, mildly deviating the
pituitary stalk to the right, demonstrating homogeneous marked hyperintensity on T2-weighted
images and no enhancement on T1-weighted post contrast images. The suprasellar cistern is
clear without contact on the optic chiasm.
What are the most likely diagnostic possibilities ?
These findings are most consistent with an intrasellar Rathke’s cleft cyst or cystic
microadenoma
What should be done next ?
A. Follow-up pituitary MRI in 6 months.
B. Clinical evaluation with H&P, physical exam and lab evaluation.
C. Clinical evaluation with H&P, physical exam, lab evaluation and visual field testing.
D. No follow up
Case 2
51 yr old female with headache and nausea found to have a
pituitary incidentaloma.
FINDINGS ?
What are the most likely diagnostic possibilities ?
Marked, mass-like diffuse enlargement of the pituitary gland extending cranially into the
suprasellar cistern and compressing the optic chiasm. Homogeneous enhancement and signal
intensity on T2-weighted images.
What are the most likely diagnostic possibilities ?
Main diagnostic possibilities include
pseudotumoral pituitary hyperplasia.
macroadenoma,
hypophysitis
and
What should be done next ?
A. Follow-up pituitary MRI in 6 months.
B. Follow-up pituitary MRI in 1 year.
C. Clinical evaluation with H&P, physical exam and lab evaluation(for hypersecretion
and hypopitutarism).
D. Clinical evaluation with H&P, physical exam, lab evaluation and visual field testing.
D. Transphenoidal biopsy
Case 3
45 yr old female with dull headache and visual changes
which became significantly worse in the past week.
FINDINGS ?
What are the most likely diagnostic possibilities ?
Large sellar mass with cystic,
septated
areas
of
T1
shortening which are not
suppressed on fat saturated
images
(methemoglobin)
extending into the suprasellar
cistern
with
marked
compression of the optic
chiasm. Focal areas of solid
enhancement
along
the
peripheral
non-hemorrhagic
mass components.
There is smooth remodeling of
the sella indicating a preexisting slow growing mass
lesion.
What are the most likely diagnostic possibilities ?
Findings are very suggestive of
hemorrhagic infarction of an
underlying
macroadenoma
(pituitary apoplexy). Apoplexy
can also rarely occur in healthy
glands.
Hemorrhagic Rathke’s cleft
cyst and craniopharyngeoma
are less likely diagnoses.
On further evaluation, patient was found to have no lab evidence of hypersecretion, mild
hypopituitarism and bilateral visual field defects.
What will be the most likely initial treatment approach?
A. Supportive care
B. Dopamine agonists
C. Surgical decompression
PART II
ENDOCRINE SOCIETY
RECOMMENDATIONS
Introduction
In 2011, The Endocrine Society published clinical practice guidelines* to help clinician
manage pituitary incidentalomas, a modern clinical issue which became reality due to
the sensitivity of cross sectional imaging studies such as CT and MRI.
The Endocrine Society defines a pituitary incidentaloma as an "unsuspected pituitary
lesion that is discovered on an imaging study for an unrelated lesion". By definition,
the imaging study is not done for a symptom such as visual loss, clinical
manifestation of hypopituitarism or hormone excess.
The prevalence of incidentalomas varies among studies from 420% on CT and 10-38% on MRI. **, ***
The guidelines were formulated by a task force based on
systematic review of evidence and expert consensus. Evidence
was developed using the Grading of Recommendations,
Assessment, Development and Evaluation (GRADE) system to
describe the strength of recommendations and quality of
evidence.
*Journal of Clinical Endocrinology & Metabolism, 96(4):894–904, 2011.
** Ann Intern Med, 15;120(10):817-20, 1994.
*** Ann Intern Med, 15;120(10):817-20, 1994.
GRADE SYSTEM
The GRADE working group published in 2008* a standardization method to rate the
quality of evidence available and strengths of recommendation in clinical guidelines.
For the quality of evidence, GRADE uses a system of four cross-filled circles:
•“+000 “ denotes very low quality evidence
• “++00 “ low quality
• “+++0” moderate quality
• “++++” high quality
For the strength of recommendation, GRADE uses two categories:
•Strong recommendation = “we recommend” and the number 1
•Weak recommendation = “we suggest” and the number 2
Recommendations listed on this presentation will be annotated according to
the GRADE system.
*BMJ. 2008 Apr 26; 336(7650): 924–926
INITIAL EVALUATION OF A PATIENT WITH A
PITUITARY INCIDENTALOMA
Which is the initial clinical evaluation recommended for all
patients presenting with a pituitary incidentaloma ?
The Endocrine Society recommends:
- History and physical examination
- Initial lab evaluations for hormone hypersecretion (1, +++0) and hypopituitarism
(1,+++0)
- Those with lesions abutting the optic nerves or chiasm on MRI should also undergo
formal visual field examination (1,++++).
IMAGING EVALUATION OF INCIDENTALOMAS
Which is the acceptable imaging modality to initially
characterize and follow-up pituitary incidentalomas?
The Endocrine Society recommends:
- MRI with contrast is recommended to all patients (if the lesion was initially diagnosed
by CT) to better delineate the nature and extent of incidentalomas (1,++++).
- CT with contrast is the second option in case of contraindications for MR.
SURGICAL CONSIDERATIONS FOR
PITUITARY INCIDENTALOMAS
When should patients be referred to surgery for a clinically
non-functioning incidentaloma?
The Endocrine Society recommends surgical consideration for (1,++++):
- Lesions causing visual field defects.
- Other visual abnormalities, such as ophthalmoplegia or neurological compromise due
to compression by the lesion.
- Lesion abutting or compressing the optic nerves or chiasm on MRI – an imaging
diagnosis. In this case the age of the patient and other factors needs to be taken in
consideration.
- Pituitary apoplexy with visual disturbance.
SURGICAL CONSIDERATIONS FOR
PITUITARY INCIDENTALOMAS
When should surgery be considered for clinically
hyperfunctioning incidentalomas ?
The Endocrine Society recommends surgical consideration for (1,++++):
- Hypersecreting tumors other than prolactinomas as recommended by other
guidelines of The Endocrine Society and The Pituitary Society.
- Macroprolactinomas that failed medical therapy (continues to grow, demonstrate
significant mass effect, visual defect, loss of endocrine function)
SURGICAL CONSIDERATIONS FOR
PITUITARY INCIDENTALOMAS
In which situations should surgery be considered ?
The Endocrine Society suggests that surgical may be considered if (2,++00):
- Clinically significant growth of the pituitary incidentaloma.
- Loss of endocrinological function.
- A lesion close to the optic chiasm and a plan to become pregnant.
- Unremitting headache.
IMAGING FOLLOW-UP OF NONSURGICAL
PATIENTS
In patients not meeting criteria for surgical treatment, how should
follow-up be performed?
The Endocrine Society recommends:
- Periodic clinical assessments.
- MRI every six months for macroincidentalomas (> 1 cm). (1, ++00)
- MRI every year for microincidentalomas (< 1 cm). (1, ++00)
- If unchanged, repeat MRI every year for macroincidentalomas and every 1–2 yr in
microincidentalomas, for the following 3 yr and gradually less frequently thereafter (2,
++00)
- Visual field testing in patients with a pituitary incidentaloma that enlarges to abut or
compress the optic nerves or chiasm on a follow-up imaging study (1, ++++).
BIOCHEMICAL FOLLOW UP OF
NON-SURGICAL PITUITARY INCIDENTALOMAS
The Endocrine Society recommends:
•
Patients with hypopituitarism should be performed 6 months after the initial testing
and yearly thereafter in patients with a pituitary macroincidentaloma (1, ++00).
•
Do not need to test for hypopituitarism in patients with pituitary microincidentalomas
whose clinical picture, history, and MRI do not change over time (2,++00).
•
Patients who develop any signs or symptoms potentially related to the
incidentaloma or who show an increase in size of the incidentaloma on MRI should
undergo more frequent or detailed evaluations as indicated clinically (1,++00).
FLOW DIAGRAM FOR THE EVALUATION AND
TREATMENT OF PITUITARY INCIDENTALOMAS
PART III – POST TEST
Now let’s review the initial 3 cases and review a few more to check how
much of the recommendations you were able to incorporate into your
working knowledge.
We’ll also try out some of your neuroradiology skills…
Case 1
61 yr old female found to have a pituitary lesion during
imaging work-up for headaches
What should be done next ?
A. Follow-up pituitary MRI in 6 months.
B. Clinical evaluation with H&P, physical exam and lab evaluation.
C. Clinical evaluation with H&P, physical exam, lab evaluation and visual field testing.
D. No follow up
What should be done next ?
Best option is B, which is the recommended initial follow-up
for incidentalomas not compressing the optic chiasm.
This patient had normal clinical evaluation and labs.
A. Follow-up pituitary MRI in 6 months.
B. Clinical evaluation with H&P, physical exam and lab evaluation.
C. Clinical evaluation with H&P, physical exam, lab evaluation and visual field testing.
D. No follow up
What follow up is recommended
A. MRI in 3 months
B. MRI in 6 months
C. MRI in 1 year
D. No follow-up necessary.
What follow up is recommended
Best option is C, which is the recommended initial follow-up
for microincidentalomas.
Review of follow up recommendations for nonsurgical
lesions:
- Periodic clinical assessments.
- MRI every six months for macroincidentalomas (> 1 cm).
- MRI every year for microincidentalomas (< 1 cm).
- If unchanged, repeat MRI every year for macroincidentalomas
and every 1–2 yr in microincidentalomas, for the following 3 yr
and gradually less frequently thereafter.
A. MRI in 3 months
B. MRI in 6 months
C. MRI in 1 year
D. No follow-up necessary.
What follow up is recommended
This lesion was stable for 4 years on MRI follow up and
most likely represents a Rathke’s cleft cyst.
A. MRI in 3 months
B. MRI in 6 months
C. MRI in 1 year
D. No follow-up necessary.
Case 2
51 yr old female with headache and nausea found to have a
pituitary incidentaloma.
What should be done next ?
A. Follow-up pituitary MRI in 6 months.
B. Follow-up pituitary MRI in 1 year.
C. Clinical evaluation with H&P, and lab evaluation.
D. Clinical evaluation with H&P, lab evaluation and visual field testing.
D. Transphenoidal biopsy
What should be done next ?
Best option is D, which is the recommended initial
follow-up for incidentalomas abutting or compressing
the optic chiasm.
A. Follow-up pituitary MRI in 6 months.
B. Follow-up pituitary MRI in 1 year.
C. Clinical evaluation with H&P, and lab evaluation.
D. Clinical evaluation with H&P, lab evaluation and visual field testing.
D. Transphenoidal biopsy
FOLLOW-UP
• Visual field testing: Normal
• Labs: TSH 256 (Normal: 0.40 - 4.50 mcIU/mL)
FT4: 0.1 (Normal: 0.8 - 1.8 ng/dL)
• Diagnosed with severe hypothyroidism and started on levothyroxine.
POST TREATMENT FOLLOW-UP
12/2012
03/2013
09/2014
Given the presentation and serial imaging + lab
findings, what is the correct diagnosis ?
12/2012
03/2013
09/2014
Labs (Normal)
12/2012
03/2013
09/2014
TSH (0.40 - 4.50 mcIU/mL)
236
2.2
0.13
Free T4(Normal: 0.8 - 1.8 ng/dL)
0.1
0.9
1.6
A. Thyrotropin secreting macradenoma
B. Null cell macroadenoma
C. Hypophysitis
D. Pseudotumoral Pituitary Hyperplasia
Given the presentation and serial imaging + lab
findings, what is the correct diagnosis ?
12/2012
03/2013
09/2014
Labs (Normal)
12/2012
03/2013
09/2014
TSH (0.40 - 4.50 mcIU/mL)
236
2.2
0.13
Free T4(Normal: 0.8 - 1.8 ng/dL)
0.1
0.9
1.6
A. Thyrotropin secreting macradenoma
B. Null cell macroadenoma
C. Hypophysitis
D. Pseudotumoral Pituitary Hyperplasia
Given the presentation and serial imaging + lab
findings, what is the correct diagnosis ?
12/2012
03/2013
09/2014
Labs (Normal)
12/2012
03/2013
09/2014
TSH (0.40 - 4.50 mcIU/mL)
236
2.2
0.13
Free T4(Normal: 0.8 - 1.8 ng/dL)
0.1
0.9
1.6
Longstanding hypothyroidism can induce massive pituitary hyperplasia, which can
simulate a primary mass. Gland enlargement usually resolves after exogenous
administration of thyroid hormones, like in this case.
Case 3
45 yr old female with dull headache and visual changes
which became significantly worse in the past week.
On further evaluation, patient was found to have no lab evidence of hypersecretion, mild
hypopituitarism and acute bilateral visual field defects.
What will be the most likely initial treatment approach?
A. Supportive care
B. Dopamine agonists
C. Surgical decompression
On further evaluation, patient was found to have no lab evidence of hypersecretion, mild
hypopituitarism and acute bilateral visual field defects.
What will be the most likely initial treatment approach?
A. Supportive care
B. Dopamine agonists
C. Surgical decompression
On further evaluation, patient was found to have no lab evidence of hypersecretion, mild
hypopituitarism and acute bilateral visual field defects.
What will be the most likely initial treatment approach?
A. Supportive care
B. Dopamine agonists
C. Surgical decompression
Best option is C. Visual field
compromise is usually a formal
indication for surgical
decompression, specially in the
acute/subacute setting to
prevent permanent damage to
the optic apparatus.
Case 4
56 year old female, sellar mass found on head CT
after motor vehicle accident
What is the most likely diagnosis ?
A. Microadenoma.
B. Macroadenoma.
C. Craniopharyngeoma.
D. Rathke’s Cleft Cyst
What is the most likely diagnosis ?
A. Microadenoma.
B. Macroadenoma.
C. Craniopharyngeoma.
D. Rathke’s Cleft Cyst
What is the most likely diagnosis ?
Rathke’s cleft
cyst
Optic
chiasm
Intracystic
nodule
A well defined intracystic nodule within a cystic, nonenhancing sellar or
suprasellar mass is very suggestive of a Rathke’s cleft cyst with
intracystic nodule*,**.
*AJNR Am J Neuroradiol 21:485–488, March 2000
**Journal Neurosurgery, 103:837–840, 2005.
What should be done next?
A. Follow-up pituitary MRI in 6 months.
B. Follow-up pituitary MRI in 1 year.
C. Clinical evaluation with H&P, physical exam and lab evaluation.
D. Clinical evaluation with H&P, physical exam, lab evaluation and visual field testing.
D. Transphenoidal biopsy
What should be done next?
Best option is D, which is the
recommended initial follow-up for
incidentalomas abutting or
compressing the optic chiasm.
A. Follow-up pituitary MRI in 6 months.
B. Follow-up pituitary MRI in 1 year.
C. Clinical evaluation with H&P, physical exam and lab evaluation.
D. Clinical evaluation with H&P, physical exam, lab evaluation and visual field testing.
D. Transphenoidal biopsy
Case 5
41 yr male, suprasellar mass seen on routine
MR for lung cancer staging
What is the most likely diagnosis ?
A) Craniopharnygioma
B) Optic glioma
C) Aneurysm
D) Metastasis
What is the most likely diagnosis ?
A) Craniopharnygioma
B) Optic glioma
C) Aneurysm
D) Metastasis
What is the most likely diagnosis ?
•Partial thrombosed
aneurysm.
•There are pulsation
artifacts and flow voids
seen.
Pulsation
artifacts
Flow
voids
What should be done next?
A)
B)
C)
D)
H&P, labs for hypopituitarism and
visual field testing.
Neurosurgery consultation
Follow up in 6 months
No follow up
What should be done next?
A)
B)
C)
D)
•
H&P, labs for hypopituitarism and
visual field testing.
Neurosurgery consultation
Follow up in 6 months
No follow up
Best option is A. Patient should get
initial H&P, labs for hypopituitarism
due to compression on the pituitary
gland and check for visual field
defects due to compression on the
optic chiasm.
CONCLUSION
• Keeping abreast of existing clinical guidelines is vital for
Radiologists to effectively translate imaging findings into
best practices.
• Pituitary incidentalomas are very commonly encountered
in Neuroradiology clinical routine and awareness of
evidence-based guidelines is essential to increase the
value and impact of our practice.