Juvenile Papillomatosis
Key Facts
Terminology
Rare mass-forming lesion of breast, composed of cysts and epithelial proliferation, occurring in young patients
Also termed “Swiss cheese” disease
Etiology/Pathogenesis
1/3-2/3 of women with JP report family history of breast cancer
1/2 of male infants with JP have neurofibromatosis 1
Clinical Issues
Rare: < 1% of all excised breast masses
Mean age: 23 (range: 12-48 years)
Occurs in both females and males
Presents as palpable, circumscribed, mobile mass
Can recur if not completely excised
˜ 10-15% of patients have concurrent cancer, and ˜ 10% may subsequently develop cancer
 Juvenile papillomatosis is also termed “Swiss cheese” disease due to its appearance on gross and microscopic examination as multiple cysts of varying sizes within a discrete mass-forming lesion. |
 Young women and men with juvenile papillomatosis generally present with a firm, palpable, circumscribed, mobile mass. The presence of multiple cysts  is clearly seen on ultrasound examination. |
TERMINOLOGY
Abbreviations
Juvenile papillomatosis (JP)
Synonyms
“Swiss cheese” disease
Definitions
Rare mass-forming lesion of breast, composed of cysts and epithelial proliferation, occurring in young patients
ETIOLOGY/PATHOGENESIS
Etiology
1/3-2/3 of women with JP report family history of breast cancer
Affected relatives are usually mothers and maternal aunts
1/2 of male infants with JP have neurofibromatosis 1
Suggests genetic basis for at least some cases
CLINICAL ISSUES
Epidemiology
Incidence
Rare: < 1% of all excised breast masses
Age
Mean age: 23 (range: 12-48 years)
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