8/2/13

1

Surgical Management of Sellar Lesions Sellar Anatomy

CN III

CN IV

CN VI

CN V1

CN V2

Single dural layer

two dural layers

Parasellar Anatomy Pituitary Mass Lesions

Pituitary Origin  Pituitary adenoma  Rathke’s Cleft Cyst

¬ Progressive mass lesion  Pars Intermedia Cyst

¬ Does not grow  Craniopharyngioma  Pituicytoma

¬ Grade I astrocytoma of the posterior lobe  Pituitary hyperplasia  Pituitary venous engorgement

8/2/13

2

Pituitary Mass Lesions Non-Pituitary Origin

  Langerhan’s histiocytosis X - Monoclonal macrophage tumor - Assoc. with eosinophilic granulomatus disease

  Lymphocytic hypophysitis - Third trimester of pregnancy - Posterior lobe (neuroinfundibulohypophysitis)

  Sarcoid   Tuberculoma   Germ cell (posterior lobe)

-  Pediatric tumor presenting with DI   Lymphoma

-  Can mimic other lesions -  Presents with cranial nerve palsy

Pituitary Mass Lesions Non-Pituitary Origin

  Meningioma (cavernous sinus, tuberculum sella) -  TS meningioma often mistaken for pituitary adenoma -  Cavernous sinus meningiomas can cause narrowing of

the ICA -  Firm, often vascular tumors

  Metastatic -  Breast

  Hemangioblastoma -  Highly vascular tumor

Pituitary Mass Lesions Non-Pituitary Origin

 Other mass lesions ¬  Colloid Cyst ¬  Arachnoid cyst ¬  Chordoma

- Presents with cranial nerve palsy ¬  Chondrosarcoma ¬  Optic nerve glioma ¬  Aneurysm

  Hypothalamic masses - Craniopharyngioma - Hamartoma - Glioma

-  pituicytoma

Presentation of Pituitary Lesions - 1

 Hormonal hypersecretion ¬ Cushing’s Disease ¬ Acromegaly/Gigantism ¬ Hyperprolactinemia (prolactinoma, stalk

effect) - Amenorrhea(hypogonadism)/galactorrhea

¬ Hyperthyroidism

8/2/13

3

Presentation of Pituitary Lesions - 2

 Glandular compression ¬ GH deficiency ¬ Hypogonadism ¬ Hyperprolactinemia (stalk effect) ¬ Hypothyroidism ¬ Hypocortisolism

¬ Pituitary adenomas do not cause DI, even when very large

Presentation of Pituitary Lesions - 3

 Optic nerve compression

Presurgical Considerations

  Careful review of signs/symptoms   Patients should have a complete w/u of

anterior lobe function ¬  GH, IGF-1, Prolactin, testosterone (men),

TSH, Free-T4, AM-cortisol

  Patients should undergo an MRI of sella with/without contrast ¬  Dynamic MRI should be done in patients

with small tumors

  Patients should have formal visual field studies if there is suprasellar extension

Surgical Considerations

  Assess MRI for: ¬  Carotid anatomy ¬  Cavernous sinus anatomy ¬  Tumor anatomy

8/2/13

4

Pituitary Adenoma (typical)

Partial hypopituitarism, no DI

Postoperative MRI – Pituitary Adenoma

Case Presentations

  34 yo female pediatrician with miscarriage at 10 wks after spontaneous pregnancy

  Work-up showed a prolactin level of 186 ng/dl   Repeat prolactin 3 months later was 154 ng/dl   MRI performed showing pituitary adenoma   Patient comes to discuss surgery vs. medical

therapy

PMH/ROS

 Menarche at age 13   Regular menses upto pregnancy   No history of infertility, galactorrhea,

weight change, change in libido   No allergies  MVI, no other medications

8/2/13

5

 Additional lab work ¬  TSH, Free T4-nl ¬ GH-nl ¬  LH slightly low, FSH-nl

Blood Work Post Contrast MRI Scan

Dynamic MRI of the Sella

8/2/13

6

Analysis   Clinical and MRI findings not consistent

with laboratory findings ¬  Elevated prolactin without clinical symptoms ¬  MRI not conclusive ¬  Testing for Macroprolactin performed

-  showed 80% of prolactin was complexed -  Free prolactin was 8.6

  Dx: Macroprolactinemia ¬  Believed to be caused by an antibody

against prolactin ¬  Antibody/prolactin complex increases half-

life of circulating hormone ¬  Complex not biologically active/bioavailable ¬  MRI normal in most cases

Case Presentation 2

  32 yo women, 33 wks pregnant with headaches and now blurry vision

  Normal menses upto pregnancy   No history of infertility, spontaneous pregnancy   Labs – Prolactin >200

  Exam – VF-Superior quadrantanopsia

  MRI

8/2/13

7

Analysis

 MRI did not show expected findings of a macroadenoma ¬  Enlarged sella ¬  Absence of posterior lobe from chronic

compression

  Dx: Lymphocytic hypophysitis ¬  Started on steroids ¬  4 days later, vision improved ¬  F/U MRI scan 1 month later, dramatic

reduction in pituitary size

Case 3

  24 yo daughter of a neurologist presenting with severe headaches

 MRI scan performed  Menses normal, hormonal w/u normal   Visual fields normal

  Headaches were worse with exercise, Headaches resolved with lying down

  Dehydration worsened headaches   Dx: Spontaneous intracranial hypotension

¬  Can cause venous engorgement and pitutiary enlargement

¬  Diffuse dural enhancement ¬  “brain sag”

  Patient was treated with a large volume epidural blood patch with 80% improvement of symptoms.

Analysis

8/2/13

8

Case Presentation

58 yo with bitemporal hemianopsia

Diagnosis: Tuberculum sella meningioma

Postop MRI, visual fields improved at 6 month f/u

Cavernous sinus meningioma

Narrowing of ICA

Stalk Lesion

46 yo male presenting with DI and hypogonadism

Diagnosis: Langerhans Histiocytosis X

8/2/13

9

Diagnosis?

Apoplexy Craniopharyngioma

Epidermoid Cyst

Pituitary Apoplexy

  Hemorrhage or Infarction of a pituitary tumor

  Increased sellar pressure leads to absence of contrast enhancement

 Often associated with sphenoid sinus inflammation

Pituitary Apoplexy (Infarction) Postop MRI

8/2/13

10

Dx - Pituicytoma

Vision loss Panhypopituitarism

No DI

Stalk is anteriorly displaced and

midline

Postop: Pituicytoma

Stalk is anteriorly displaced and midline

Surgical Consideration

  Vascular anatomy ¬  understand carotid anatomy

Ectatic right ICA

64 yo man with headaches and hypogonadism

8/2/13

11

Carotid Anatomy

Ectatic R ICA, tumor cyst shows a fluid/fluid level

51 yo male with acromegaly

Inter-carotid distance = 4mm

Postop MRI scan

Postop GH: 0.8

Cavernous Sinus Invasion?

  Cavernous sinus invasion is the main reason for incomplete resection of pituitary tumors

 When tumor extends lateral to carotid artery, the diagnosis is made

  In all other cases, visualization/inspection of the cavernous sinus wall is important to achieve optimum results

8/2/13

12

Preop

Endonasal Transsphenoidal Resection of Giant Adenoma

? Invasion into right cavernous sinus

3 mo postop

Pitutiary Adenoma Anatomy

Variations in tumor anatomy may warrant an extended approach

Unusual Tumor Anatomy Suprasellar Tumor

40 yo female with bitemporal hemianopsia, normal pituitary function

8/2/13

13

Extended Transsphenoidal Approach with Endoscope assist

Giant Adenoma (5.5 cm)

72 yo male with bitemporal vision loss, headache, panhypopituitarism

Postop – Extended endoscopic approach Lessons learned

  1500 consecutive endonasal transsphenoidal surgeries from 2000-2012

  Diagnosis ¬  Pituitary Adenoma – 77% ¬  Rathke’s Cleft Cyst – 13% ¬  Craniopharyngioma – 3% ¬  Chordoma – 1% ¬  CSF leak repair – 1% ¬  Meningioma – 0.6%

8/2/13

14

Lessons learned

  Diagnosis (cont’d) ¬  Lymphocytic hypophysitis – 6 ¬  Langerhans – 5 ¬  Arachnoid Cyst – 5 ¬  Epidermoid Cyst – 4 ¬  Metastatic tumors - 4 ¬  Optic nerve tumor – 3 ¬  Germ cell tumor - 3 ¬  Colloid Cyst – 2 ¬  Hypothalamic mass – 2 ¬  Plasmacytoma – 1 ¬  Cholesterol granuloma – 1 ¬  Hemangioblastoma – 1

Conclusions:

  Patterns for presentation are important to recognize for pituitary pathology

  Consider all possible diagnoses if the clinical or radiographic presentation does not fit the “picture”

  Complete workup includes ¬  MRI scan ¬  Anterior lobe hormonal workup

-  TSH, Free T4, Prolactin, Testosterone (men), GH, IGF-1, am cortisol

¬  Visual field testing (if suprasellar extension present)

Acknowledgements

Department of Neurosurgery Manish Aghi, MD Gwen Stanhope, PA Lewis Blevins, MD

Division of Endocrinology Lewis Blevins, MD Blake Tyrell, MD

Division of Neuroradiology William Dillon, MD Chris Hess, MD

Division of Neuropathology Andrew Bollen, MD Tarik Tihan, MD Arie Perry, MD