Tumours of the sellar region Flashcards

Gruppnamngivning enligt WHO 2021.

1
Q

Where are neurohypophyseal tumors situated?

A

In the neurohypophys - posterior part of the pituitary gland

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2
Q

What is the Hardy system?

A

An anatomical classification of pituitary adenomas

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3
Q

What is the only pituitary tumor that is not treated surgically 1st hand?

A

Polactinomas

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4
Q

How are prolactinomas treated?

A
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5
Q

What are the 2 most common tumor/lesion of the sellar region?

A
  1. pituitary adenoma
  2. meningioma
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6
Q

Describe 4 steps of anatomic radiology approach to differential diagnostics in the sellar/parasellar region

A
  1. identify the pituitary gland and sella turcica
  2. Determine epicentre of the lesion - in? Above? below? lateral ? to the sella (+ is the sella enlarged?)
  3. Analyse the lesion
    -signal?
    -cystic?
    -solid?
    -flow void?
    -calcification?
  4. differential diagnostics
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7
Q

What are the three most common abnormalities in the pituitary gland?

A
  • Pituitary adenoma
  • Rathkes cleft cyst
  • Craniopharyngeoma
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8
Q

What is the pituitary stalk derived from embryologically?

A

From Rathkes cleft epithelium.

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9
Q

In the pituitary stalk, there are a few unusual things to be considered for children. Which?

A
  • germinomas
  • eosiniphilic granulomas
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10
Q

What can arrise in adults that is usually not seen in children in the pituitary stalk?

A
  • metastases
  • lymphoma
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11
Q

differential diagnostics for the pituitary stalk (mixed adult and children)

A
  • Rathces cleft cyst
  • craniopharyngeoma
  • germinoma
  • eosinophilic granuloma
  • metastasis
    *(lymphoma)
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12
Q

Where in the sellar region is the optic chiasm localised?

A

It is in the suprasellar cistern.

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13
Q

Differential diagnostics of the optic chiasm itself

A
  • gliomas
  • MS
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14
Q

The base of the brain lies cephalad to the optic chiasm. What region is this?

A

Hypothalamus.

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15
Q

What are the differential diagnostics in the hypothalamus?

A

No1 = Gliomas.
In children:
* Hamartomas
* Germinomas
* Eosinophilic granuloma

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16
Q

The cavernous sinus lies just lateral to the hypophysis. What is the segment of ICA just cephalade to the cavernous sinus called? And what structures in the sellar/suprasellar region is it close to?

A

It is the supracavernous segment. It lies just lateral to the pituitary stalk.

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17
Q

What happens after the supracavernous segment of carotis interna?

A

It bifurcates and the ACA passes cranially laterally to the optic chiasm. The MCA runs laterally.

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18
Q

What are artery caused differentials in this area?

A
  • Aneurysms
  • Ectasias
  • Anomalies
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19
Q

What structures run in the cavernous sinus?

A

CN III, CN IV, CN VI and V1 (these 4 then exit through supraorbital fissure) AND V2 (that exit through rotundum and then infraorbital fissure).

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20
Q

Which of the CN is located more medially in the sinus and run just caudal to the carotid artery?

A

Abducens. CN VI.

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21
Q

Differential diagnostics of the cavernous sinus

A
  • Schwannomas!
  • Inflammation that might lead to cavernous sinus thrombophlebitis and Thrombosis.
  • Carotud-cavernous fistulas - communication between ICA and the veins of the cavernous sinus.
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22
Q

The cavernous sinus is actually an extracephal structure (but intracranial). It is covered by meninges that are thicker laterally and superiorly (the pituitary gland but not the stalk will be covered in this tent).

  • What is the 2 most common tumors in this area and What is the most common inflammatory pathology infectious and non-infectios?
A

Tumor: ‘1. Meningioma’2. Metastasis
Inflammatory pathology: Tuberculous meningitis and non infectious: Sarcoidosis.

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23
Q

What is situated inferior to the pituitary gland?

A

The sphenoid sinus.

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24
Q

What is situated posterior to the sphenoid sinus?

A

Clivus.

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25
Q

What pathologies might occur in the clivus area?

A
  • Chordomas
  • Chondrosarcoma
  • Osteosarcoma
  • metastases can occur anywhere.
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26
Q

What is the normal pathway for bacteria and fungal infections to spread from the sphenoid sinus intracranially?

A

Via the cavernous sinus.

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27
Q

Difine a pituitary microadenoma

A

Less than 10mm

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28
Q

What is the first radiological diff diagnosis of microadenoma?

A

Rathkes cleft cysts.

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29
Q

What is the most common problem for patients with microadenoma?

A

Hyperprolactinemia

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30
Q

What is usually the treatment of microadenoma?

A

Treatment of the hyperprolactinemia only

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31
Q

What is then the purpous of an MRI scan for patients with hyperprolactinemia?

A

To rule out large lesions.

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32
Q

When is MRI with contrast needed?

A
  • Pt with failed medical therapy
  • Pt with disease not amenable to medical therapy such as Cushings disease.
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33
Q

Sign to differentiate a pituitary macroadenoma from a meningioma?

A

Macroadenomas are usually soft solid lesions that grow slowly from the pituitary. Expand the sella turcica but usually get a “waist” at the diaphragm sella - A snowman configuration on coronal scan.

Both the snowman appearance and the enlargement of sella usually only appear in tumors originating in the pituitary macroadenomas that originates in the sella (in contrat to the meningioma)

34
Q

What is a normal maximal level of prolactin?

A

25.

35
Q

What is the treatment of Prolactinoma?

A

Bromocriptine

36
Q

What can make something bright on an unenhanced T1 weighted MRI?

A
  • Blood or Proteinacious fluid
  • Fat
37
Q

Does normal pituitary gland and normal pituitary stalk enhance with Gd contrast?

A

Yes they do. They are both extracerebral masses (extraaxial).

38
Q

Why is it important to see MRI both w/o and with Gd in a tumor in the sella area?

A

Because the Gd images might lead us to think that a mass is situated both in the stalk and in the gland as the gland enhances with Gd both with and without malignancies.

39
Q

What three pathologies might be derived from Rathkes cleft epithelium?

A
  • Rathkes cleft cysts
  • Craniopahryngeoma
40
Q

How are Craniopharyngeomas usually behaving?

A
  • Benign but locally invasive.
  • Cyst with Thick walls (unlike rathkes cysts) and multiple nodules
    *
41
Q

How is surgery for Cranipharyngeomas?

A

Difficult. They grow multiple nodules along the skull base and sinuate aling the fissures.
Often incomplete resections.

42
Q

Typical radiographic presentation of craniopharyngeoma ? (50% of cases)

A

*Compressed pituitary gland
* Large intra and suprasellar mass with cystic and enhancing components as well as calcifications.
* If found in a child it is VIRTUALLY PATHOGNOMONIC for craniopharyngeoma

43
Q

How many % of meningiomas present in the skull base?

A

20%

44
Q

How does meningioma usually enhance?

A

Uniformely as a rule.

45
Q

What to look for in a suprasellar/sellar meningioma?

A
  • Dural enhancement? (tail)
  • Is the suprasellar part larger then the pituitary compound?
  • Is there no evident midja?
46
Q

How are hormones produced in the hypothalamus transfered to the anterior lobe of the pituitary gland?

A

Via a portal vein system

47
Q

What hormones are produced in the anterior lobe of the pituitary gland?

A
  • TRH
  • GnRH
  • GHRH
  • CRH
  • Prolactine
48
Q

Which of the hormones of the hypothalamus works inhibitory on the anterior lobe?

A

Dopamine. It inhibits the production of Prolactine in the anterior lobe.

49
Q

What effect does a compression of the stalk have on production of hormones in the anterior piuitary lobe?

A

Decreased production of all hormones except Prolactine that is increased.

50
Q

So name 2 obvious differential diagnostic of inadecuate prolaktin production

A
  1. microadenoma -prolactinoma
  2. Large stalk-compressing mass inhibiting the portal system.
51
Q

Is it possible to distinguish an aneurysm from a meningima on CT images?

A

No. Not in the sellar region.

52
Q

Who gets an Hamartoma?

A

Almost exclusively young children.

53
Q

What is the most common location for a Hamartoma?

A

The floor of the third ventricle.

54
Q

In what cistern might a hamartoma hang down?

A

The suprasellar sistern. ( de ser ut som droppar från 3e ventrikeln på en coronarbild. Som en extra hypofys. På en sagittalbild ser det mer ut som en extra bit hjärna bakom hypofysstjälken, hängande ner från tuber cinereum och ovan/framför mammilarkropparna. Man ser tydligt att det inte är en “extrahypofys” iallafall på exempelbilderna är hamartomet mycket större.

55
Q

What is good and what is bad with a Hamartoma?

A

They are benign lesions, but located badly.

56
Q

For what patientgroup is gliomas by the optic chiasm common?

A

NF 1 patients.

57
Q

How many percent of optic nerve gliomas does not enhance?

A

25%!!

58
Q

Germinomas are usually seen in children. They are also more common in another area. Which?

A

The epiphysial area - by the pineal gland.

59
Q

What is the most common lesion of the clivus?

A

Chordomas

60
Q

What would be the differential diagnosis if you find a mass behind the pituitary and infront of the mamillary bodies and perhaps also pons?

A
  1. Chordoma
  2. metastases
  3. chondrosarcoma
61
Q

What should be the normal signal intensity in a sagittal view of a normal clivus?

A

It should be high intensity due to normal fatty marrow.

Many tumors like small cell lung cancer mets, lymphoma, myeloma and diffuse bone marrow abnormalities may “eat” the fatty appearance and it will be low intensity on normal T1 weighted MRI.

62
Q

How often is increased levels of growth hormones due to pituitary adenoma?

A

more than 95%

63
Q

What diseases occur from increased growth hormon release?

A

Adults - Acromegali
Children - Gigantism

64
Q

What disease is caused by corticotropin releasing tumors of the pituitary?

A

Cushings disease.

65
Q

What is secondary hyperthyreoidism?

A

Its due to TSH producing pituitary tumors.

66
Q

What to suspect if there is hormone hyposecretion?

A

A large tumor compressing the gland or the stalk

67
Q

What to suspect if only one hormone is depressed?

A

Autoimmune hypophysitis

68
Q

What to ask if diabetes insipidus is seen preoperatively?

A
  • Autoimmune hypophysisis
  • Hypothalamic glioma
  • Supracellar germ cell tumor
69
Q

Signs of pituitary apoplexy?

A
  • sudden onset H/A
  • Visual disturbance
  • Opthalmoplegia
  • Reduced mental status

Corticosteroid treatment needs to be started immidiately.

Rapid decompression is needed if:
* severe restriction of visual field
* Severe deterioration of visual acuity
* Mental status changes due to hcph

70
Q

WHat is the Hardy system?

A

Anatomic classification of pituitary adenoma

71
Q

Describe the 4+4 descriptions of the Hardy system

A

1- no expansion
2- expanding into the suprasellar cistern
3- obliteration of the anterior recess of the third ventricle
4- displacement of the 3rd ventricle floor.

I- Intact sellar floor
II- Enlarged sella
III- Localized perforation of the sellar floor
IV- Diffuse destruction of sella floor

72
Q

What is the difference between Cushings disease and Cushings syndrome?

A

Cushings disease = due to hypersecretion of ACTH.
Cushings syndrome = the effect on the body from hypercortisonism.

73
Q

Treatment of ACTH hypersecreting adenomas?

A

Most = transsphenoidal surgery

Some = medical treatment with Ketoconazole

74
Q

What is the function of Ketoconazole?

A

Block adrenal steroid synthesis

75
Q

what is the cure rate from transsphenoidal surgery of ACTH expressing piuitary adenomas?

A

85% for the microadenomas and less for larger lesions

76
Q

What is the last resort treatment in non-resectable ACTH secreting piuitary adenomas?

A

Bilateral adrenalectomy.

77
Q

What syndrome is developed within 1-4 years from adrenalectomy in 10-30% of patients?

A

Nelson syndrome.

78
Q

What is Nelson syndrome?

A
  • increased ACTH
  • Hyperpigmentation - ACTH is similar to melanocyte stimulating hormone.
  • Enlarged pituitary tumor
79
Q

Which are the two most common tumors of the neurohypophysis and infundibulum?

A
  • metastases
  • granular cell tumor - mostly in the stalk.
80
Q

What surgical steps is needed in an expected neurohypophysis/infundibular tumor?

A

It needs to be approached cranially.

81
Q

The chiasm is located superior to the sella in 79%. But if not, its got special names and implications. What are these names?

A

Postfixed chiasm - more prone to optic chiasm compression—Ipsilateral loss of vision and contralateral quadrant anopsia.

Prefixed chiasm - compression of the optic tract when the chiasm is situated infront of the suprasellar area is homonymous hemianopsia.

82
Q

What is the classical visual field deficit in pituitary tumors?

A

Bitemporal hemianopsia