NBME 21 TP53 (NBME Answers)

NBME 21 Answers

nbme21/Block 4/Question#46 (reveal difficulty score)
A 14-year-old girl is brought to the ...
TP53 🔍 / 📺 / 🌳 / 📖
tags: process_of_elimination Li_Fraumeni p53

+4 upvote downvote
submitted by ∗neonem(629)

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Sounds like a case of Li-Fraumeni syndrome - since p53 is a tumor suppressor for a bunch of cell types, mutations in this gene (as in LFS) result in a myriad of familial tumor types.

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pparalpha Li-Fraunemi syndrome = SBLA (sarcoma, breast, leukemia, adrenal gland syndrome) and occurs because of an autosomal dominant inherited mutation of p53 APC: linked to FAP (colorectal cancer) RET: linked to papillary thyroid cancer, MEN 2A, MEN 2B RB1: retinoblastoma +11

privatejoker The thing that threw me off was that the only connection in her FH to the above SBLA reference was the mention of a paternal cousin with adrenocortical carcinoma. The other two mentioned had brain cancers, which seem completely outside the scope of the above mnemonic. Then again, as mentioned elsewhere, I suppose the best policy on these is just to rule out the absolute wrong answers. I swear, the NBME is lying when they tell us to choose the "best" answer on some of these. What they actually mean in practice is for us to choose the least shitty. +17

dbg ^ this guy cracked the code. nbme ur doomed. +10

cienfuegos @privatejoker: I feel the pain. Quick FYI: UW includes brain in the associated tumors. +3

hyperfukus we can just make her thing SBBLA and hopefully never get this wrong again +9

jakeperalta @privatejoker: according to UW, Li Fraumeni includes SABBB(sarcoma/adrenocortical/breast/brain/blood(leukemia)) +2

ac3 side note: RB1 = retinoblastoma with an increased risk of osteosarcoma +

lukin4answer TP53 associated with SBLA + Brain tumor + Anaplastic Thyroid ca + Transitional cell ca. -UW +

an1 Li Fraumeni in sketchy path includes brain, breast, bone and adrenals +

+2 upvote downvote
submitted by ∗hayayah(1212)

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Most human cancers are d/t a loss of function of TP53 gene.

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+2 upvote downvote
submitted by ∗sahusema(174)

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Associated neoplasms in Li-Fraumeni syndrome. Sarcomas, breast cancer, brain tumors, adrenocortical carcinoma, leukemia

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+1 upvote downvote
submitted by ∗mcl(670)

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Other way to get this question is by eliminating other options -- this figure is useful in listing some mutations and associated cancers.

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+0 upvote downvote
submitted by ∗kevin(52)

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Li Fraumeni, Tp53 mutation. SBBLA cancers

sarcoma
brain
breast
lung
adrenal

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kevin leukemia not lung, sorry +

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