skin malignancies

Question 1

dermatofibrosarcoma have a translocation of which chromosomes?

Answer 1

t (17,22)

Question 2

whats the most common skin malgnancy in white people?

Answer 2

BCC

70% present on face and head
90% locally invasive only
don’t occur on palm of hand, plantar foot or mucosa

Question 3

what is the mutation seen in basal cell nevus syndrome (gorlin syndrome)?

Answer 3

germline mutation of the human patched gene 1 (PTCH1)

Major dx criteria: >2 confirmed BCC or 1 bcc <20yrs
keratocystic odontogenic tumors of jaw
>3 palmar and plantar pits
1st degree relative
bimallelar ca of falx cerebri <20yrs
fused, bifid or splayed ribs

multiple BCC few to thousands on face,chest, head flesh colored papules to ulcerating plaques
avoid radiotherapy and sun exposure
deformed w/ macrocephaly, hypertelorism, bifid ribs, palmar and plantar pitting, bone cysts in mandible, flax cerebri calcification, medulloblastoma in demsoplastic subtype 3-5%

Question 4

what % of BCC have abnormal hedgehog pathway signaling?

Answer 4

90%

hedgehog is targeted in therapy for this reason

Question 5

What is the surgical margin for BCC <2cm and nonagressive?

Answer 5

4mm

Question 6

What is the margin for BCC that is >2cm, aggressive, or recurrent?

Answer 6

10mm and CCPDMA + mohs

Question 7

what does CCPDMA stand for?

Answer 7

complete circumferential peripheral and deep margin assessment