NCCN Guidelines

Question 1

Breast Cancer

Answer 1

Personal hx:
dx < 50
dx any age AND:
TNBC, male BC, multiple primaries, lobular w/ phx/fhx gastric ca, AJ ancestry
>= CBR w/
- BC dx <= 50, male BC, OC, PA, PR (high risk), >3 total BC dx in pt or CBR, >2 CBR w/ BC or PR (any grade)

Fam hx only:
FDR or SDR meeting above criteria

Question 2

Colorectal Cancer (Lynch Syndrome)

Answer 2

Per hx:
- MMR def tumor at any age
- CRC or EC AND:
dx <50
also had/has LS-related cancer
1 FDR or SDR w/ LS-related cancer dx <50
>= 2 FDR or SDR w/ LS-related cancer any age

Fam hx only:
>= 1 FDR with CRC or EC <50
>= FDR w/ CRC or EC and hx LS-related cancer (synch or meta)
>= 2 FDR or SDR w/ LS-related ca including >= 1 dx <50
>= 3 FDR or SDR w/ LS-related ca any age

Question 3

Colorectal Cancer (Familial Adenomatous Polyposis (FAP))

Answer 3

Personal hx:
>= 20 cumulative adenomas
Multifocal or B/L CHRPE

Question 4

Ovarian Cancer

Answer 4

Per hx:
dx at any age (epithelial, fallopian tube, or peritoneal)

Fam hx:
FDR or SDR with OC at any age

Question 5

Pancreatic Cancer

Answer 5

Per hx:
dx at any age

Fam hx:
FDR with PA

Question 6

Peutz-Jeghers Syndrome (PJS)

Answer 6

Per hx:
>= 2 PJS type hamartomatous polyps
Mucocutaneous hyperpigmentation of mouth, lips, nose, eyes, genitalia, or fingers

Question 7

Juvenile Polyposis Syndrome

Answer 7

Per hx:
>= 5 juvenile polyps of colon
Multiple juvenile polyps of the GI tract

Question 8

Prostate Cancer

Answer 8

Per hx:
Mets or high/very-risk group
and dx BC
>= FDR with PR <= 60
>= 1 FDR with:
- BC < 50, TNBC, Male BC, OC, PA, PR (high risk) any age
>= 2 CBR with either BC or PR (any grade) any age
AJ ancestry

Fam hx only:
FDR meet above criteria

Question 9

Renal (RCC)

Answer 9

Per hx:
dx < 46y
B/L or multifocal
>= 1 FDR or SDR with RCC
Multifocal papillary
HLRCC-associated RCC
RCC with FH deficiency
Multiple chromophobe, oncocytoma, or oncocytic hybrid

Fam hx only:
>= 2 FDR with RCC
Any FDR who meets above criteria

Question 10

Gastric Cancer

Answer 10

Per hx:
dx < 40
dx < 50 AND 1 CBR with gastric ca
and BC with 1 of them dx < 50
dx at any age AND:
- >= 2 CBR with gastric ca
- fam hx BC dx < 50
- fam hx juvenile polyps or GI polyposis
- fam hx LS-related cancer

Fam hx only:
1 CBR dx < 40
3 CBR with 1 dx < 50
3 FDR
CBR with BC, with one dx < 50, juvenile polups, or GI polyposis

Question 11

Neuroendocrine (NET) and Adrenal Tumors

Answer 11

Per hx:
ACC
PGL/PCC
Gastrinoma
Multifocal pancreatic NETs
Parathyroid adenoma or primary hyperparathyroidism dx < 30
Multiple parathyroid adenomas
Multiglandular hyperplasia
Recurrent primary hyperparathyroidism

Fam hx:
FDR meeting above criteria

Question 12

Clinical suspicion for MEN1

Answer 12

Primary hyperparathyroidism, duodenal/pancreatic NET, pituitary adenoma, foregut carcinoid (bronchial, thymic, or gastric)

Question 13

Clinical suspicion for MEN2

Answer 13

Medullary thyroid cancer or other combo of features

Question 14

PTEN Hamartoma Tumor Syndrome

Answer 14

Per hx:
BRR Syndrome
ASD + macrocephaly
>= 2 trichilemmomas
Macrocephaly + major criteria
3 Major criteria
1 Major + 3 minor
>= 4 minor

Major criteria:
BC, EC, follicular thyroid, multiple HI hamartomas, macrocephaly, macular pigmentation of glans penis, mucocutaneous lesions

Minor criteria:
ASD, CRC, >= 3 esophageal glycogenic acanthoses, lipomas, RCC< thyroid lesions, 1 GI hamartoma, testicular lipomatosis, vascular anomalies

Question 15

Li-Fraumeni Syndrome

Answer 15

Class Crtiera:
Clinical dx < 45 with sarcoma AND FDR dx < 45 or SDR with cancer dx < 45 or sarcoma at any age

Chompret Criteria:
LFS tumor (Sarcoma, osteosarcoma, CNS, BC, ACC) dx < 46 AND FDR with LFS cancer except BC < 56 OR multiple primaries any age
- Multiple LFS tumors (besides BC) with first dx < 46
- ACC or choroid plexus carcionoma or rhabdomyosarcoma any age
- BC dx < 31
- pediatric hypodiploid acute lymphocytic leukemia