Q85.82

Billable

Other Cowden syndrome

HCC Category Mapping

V28HCC 23 — Melanoma and Other Skin Cancers

0.251

V24HCC 12 — Breast, Prostate, and Other Cancers and Tumors

0.150

ESRDHCC 12 — Breast, Prostate, and Other Cancers and Tumors

0.000

RxHCCHCC 22 — Cancer, Other Specified Sites

0.000

What This Code Means

Cowden syndrome is an inherited genetic disorder characterized by multiple benign skin growths and increased risk of certain cancers.

Coding Tips

•Document specific manifestations such as trichilemmomas, breast lesions, or thyroid involvement
•Link with cancer codes if malignancies have developed

Clinical Significance

Cowden syndrome is a specific subset of PTEN hamartoma tumor syndrome characterized by distinctive mucocutaneous features and very high cancer risks, particularly breast and thyroid cancers, requiring aggressive surveillance starting in young adulthood. The condition affects both men and women but has particularly high implications for women's reproductive health decisions.

Documentation Requirements

✓PTEN gene mutation or clinical diagnosis criteria
✓Pathognomonic mucocutaneous features (trichilemmomas, etc.)
✓Personal or family history of associated cancers
✓Macrocephaly measurement and documentation
✓Thyroid abnormalities documented
✓Breast examination and surveillance protocols
✓Endometrial surveillance in women
✓Genetic counseling and family screening

Commonly Confused Codes

Q85.81 — PTEN hamartoma syndrome (broader category)C50.919 — Breast cancer (complication, not underlying syndrome)C73 — Thyroid cancer (complication, not underlying syndrome)Q87.89 — Other congenital malformation syndromes

Code Hierarchy

└Q85Phakomatoses, not elsewhere classified └Q85.8Other phakomatoses, not elsewhere classified └Q85.82Other Cowden syndrome

└Q85.82Other Cowden syndrome