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27 Cards in this Set

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Name 5 Cancer Syndromes that involve stomach cancer
Stomach Cancer:

*Hereditary Diffuse Gastric Cancer
*HNPCC
*LFS
*FAP
*PJS
What is the 5 year survival of stomach cancer?
5 year survival of stomach cancer

5-15%
What kind of cancer is stomach cancer?
Stomach Cancer

95% adenocarcinoma (Papillary/ tubular/ mucinous/ signet ring)

The other 5% are lymphoma, leiomyosarcoma, carcinoid, squamous cell
What are the 2 major types of Gastric Cancer
2 major types of Gastric Cancer

*Diffuse Variant
*Intestinal Variant
What do you see in the diffuse variant of gastric cancer
Diffuse Variant
*younger pts
*worse prognosis
*assoc with Barrett's epithelium
*PRoximal
*Linitis plastica = just a thickening of the stomach lining - more "diffuse" not a specific defined area
What gene causes Hereditary Diffuse Gastric Cancer?
Hereditary Diffuse Gastric Cancer

CDH1 gene (E-cadherin)
What kind of mutation happens in the CDH1 Gene to cause Hereditary Diffuse Gastric Cancer
mostly frameshift, truncating
What are the clinical criteria to diagnose Hereditary Diffuse Gastric Cancer?
the clinical criteria to diagnose Hereditary Diffuse Gastric Cancer

2+ cases of diffuse gastric cancer in 1st or 2nd deree relatives, one being <50

3+ cases of diffuse gastric cancer in 1st or 2nd degree relatives at any age

Any individual with diffuse gastric cancer <35

Any family with history of both diffuse gastric cancer and lobular breast cancer
Do we test minors for Hereditary Diffuse Gastric Cancer?
Yes - earliest onset of Hereditary Diffuse Gastric Cancer is age 14
With a CDH1 gene mutation what is the lifetime risk for gastric cancer (males and females)
With a CDH1 gene mutation the lifetime risk for gastric cancer is

Male = 67%
Female = 83%
What kind of breast cancer is associated with CDH1 gene mutation (Hereditary Diffuse Gastric Cancer)?
associated with CDH1 gene mutation (Hereditary Diffuse Gastric Cancer)

Lobular Breast Cancer 20 - 40% lifetime risk
How do you maanage someone with Hereditary Diffuse Gastric cancer?
Mgt Hereditary Diffuse Gastric Cancer

EGD every 6-12 months
Mammo + MRI alternating 6 months
PET?
Prophylactic total gastrectomy?
Chemoprevention with NSAIDS?
What kind of Syndromes are seen with liver cancer (hepatoblastoma)
Syndromes causing Liver Cancer:

* FAP
* Overgrowth (Beckwith-Wiedemann/ Simpson-Golabi-Behmel)
Name four syndromes caused by chromosomal breakage?
Chromosomal Breakage

*Ataxia telangiectasia
*Bloom Syndrome
*Fanconi Anemia
*Xeroderma Pigmentosum
What gene causes Ataxia Telangiectasia and how is it inherited?
Ataxia Telangiectasia

ATM gene
Autosomal Recessive
Ataxia Telangiectasia - What kind of cancers do you see?
Ataxia Telangiectasia Cancers:

Non-hodgkins lymphoma
leukemia
gastric cancer
medulloblastoma
Basal Cell carcinoma
Glioma
Uterine cancer
What is the gene that causes Bloom Syndrome and how is it inherited?
Bloom Syndrome

BLM gene - Autosomal Recessive
What cancers are seen in Bloom Syndrome (BLM gene)
cancers seen in Bloom Syndrome (BLM gene)

*leukemia
*lymphoid neoplasma
*Wilms tumor
*tongue/ larynx/ lung/ esophagus/ colon/ breast/ cervix
What other conditions are seen in Bloom Syndrome (BLM gene)
Bloom Syndrome (BLM gene)

*immunodeficiency
*high pitched voice
*small jaw
*skin lesions
*low IQ
*DM
*Pulmonary disease
Fanconi Anemia B has what type of inheritance?
Fanconi B (FANCB) X-Linked
What kind of cancers are seen with Fanconi Anemia?
Fanconi Anemia Cancers:

*acute Myeloid Leukemia
*Myelodysplactic Syndrome
*tumors of head/ neck/ skin/ GI/genital/ liver
Xeroderma Pigmentosum - what is the gene and the inheritance
Xeroderma Pigmentosum:

Many genes

Autosomal Recessive
Xeroderma Pigmentosum

What are some of the cancers?
Xeroderma Pigmentosum Cancers

non-melanoma skin cancer < 10 y.o.
glioma
solid tumors (lung/ uterus/ breast/ pancreas/ stomach/ kidney)
Xeroderma Pigmentosum

What are some of the eye abnormalities?
Xeroderma Pigmentosum / Eye abnormalities

photophobia
keratitis of eyelids
Pigmented eye lids
loss of lashes
Xeroderma Pigmentosum

CNS abnormalities
Xeroderma Pigmentosum -CNS abnormalities

acquired microcephaly
diminished deep tendon reflexes
What is the 2009 Chompret Criteria
Chompret Criteria:

Criteria for testing LiFraumeni
What is LFL Li-Fraumeni Like Syndrome
If the p53 testing is negative but they meet clinical features then they have LFL syndrome