general knowledge - tumors Flashcards

1
Q

What NF diagnosis is associated to meningiomas?

A

NF2

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2
Q

4 types of metastases that are not very radio-therapy sensitive?

A

Renal cell carcinoma
Colon cancer
some type of breast cancer
?

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3
Q

What does SEGA stand for?

A

subependymal giant cell astrocytoma. Grade II

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4
Q

Two types of craniopharyngeoma

A
  • adamantinoumatous
  • papillary
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5
Q

What cromosome has deletion in about 35-50% of medullobasltomas?

A

17p

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6
Q

Vad är Forbes-Albright syndrome?

A

Amenorrhea och Galactorrhea associated to prolactinoma

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7
Q

Name a few familial syndromes associated to paragangliomas and pheochromocytomas

A

MEN2 (A, and B - RET genetic dist)
von Hippel Lindau disease
NF-1

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8
Q

What is paragangliomas?

A

They are tumors that arise from neuroendocrine tissues symmetrically distributed along the spine from the base pf the skull to the pelvis.

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9
Q

What is Li-Fraumeni syndrome?

A

Its a cancer predisposition syndrome associated with high risks for a diverse spectrum of childhood and adult onset malignancies. Life time risk of malignancy for men is more than 70% and more than 90% for women.

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10
Q

What gene is most ogften associated to Li-Fraumeni syndrome?

A

p53.

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11
Q

What are the clinical signs of Foster-Kennedy syndrome and what is the cause (if not pseudo-foster Kennedy)

A
  • one sided optic atrophy
  • central scotoma in the ipsilateral eye
  • papilledema in the OTHER (contralateral) eye. !!
    Due to an ipsilateral frontal mass. Usually an olfactory groove meningioma.

Historically ipsilateral anosmia was thought to be associated too, but is now understood not to be strictly associated.

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12
Q

What two types of tumors are the most common to cause Foster Kennedy syndrome?

A
  • Meningioma
  • Plasmocytoma (rare solitary tumor of neoplastic monoclonal plasma cells in either bone or soft tissue (extramedullary (as in bone marrow medulla))
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13
Q

How common are pineal tumors in adults?

A

1% of all brain tumors.

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14
Q

There are two types of cranipharyngeoma. One is rather common in children, and one is rare and found in adults. What are they called?

A

Pediatric: Adamantinomatous craniopharyngeoma
Adult: Papillary craniopharyngeoma

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15
Q
A

26-50%!!

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16
Q

What grades might Astrocytoma IDH mutant have?

A

1,3 and 4.

17
Q

What grades might Oligodendroglioma, IDH-mutant and 1p/19q-codeled have?

A

2 and 3.

18
Q

What grades might glioblastoma ICH wt have?

A

4

19
Q

What grades might diffuse midline glioma, H3, K27 altered have?

A

4

20
Q

What grade might diffuse hemispheric glioma, H3 G34 mutant have?

A

4

21
Q

What grades might pilocytic astrocytoma have?

A

1

22
Q

What grades might pleomorphic xanthoastrocytoma have?

A

2 and 3

23
Q

What grades might embryonal tumours have?

A

4

24
Q

What grades might myxopapillary ependymoma have?

A

2

25
Q

What grades might Meningioma have?

A

1,2 and 3

26
Q

What grades might solitary fibrous tumour have?

A

1,2 and 3.

27
Q

What are the diagnostic criteria of NF1?

A

2 or more of the following:
* At least 6 cafe aulait macules
* Freckling in axillary or inguinal regions
* Optic glioma
* At least 2 Lisch nodules (iris hamartomas)
* At least two neurofibromas or one plexiform neurofibroma
* A distinctive osseus lesion (sphenoid dysplasia pr tibial pseudoarthrosis)
* A first degreee relative with NF1

28
Q

What is a Lisch nodule

A
29
Q

What is a plexiform neurofibroma?

A
30
Q

Main Diagnostic criteria for NF2

A
  1. Bilateral vestibular schwannomas
    or
  2. 1st degree relative + Unilateral vestibular schwannoma OR 2 of:
    meningioma, glioma, schwannoma or juvenile posterior lenticular opacities
31
Q

What is the chromosome location and name of protein mutated in NF2?

A

22q12 - Merlin

32
Q

What is the name of the mutated protein in NF1?

A

Neurofibromin.