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Surgical Pathology Criteria

Juvenile Papillomatosis


  • A discrete multicystic breast lesion typically ocurring in young females

Alternate/Historical Names

  • Swiss cheese disease

Diagnostic Criteria

  • Discrete mass
    • Circumscribed but not encapsulated
    • Usually solitary
      • Rarely multicentric and bilateral
    • 1 to 8 cm diameter
  • Large numbers of cysts, up to 2 cm
    • "Swiss cheese disease"
    • Frequently contain foamy histiocytes
  • Cyst lining variably hyperplastic
    • Frequently papillary with epithelial bridges
    • Hyperplasia may be atypical in 10% of cases
    • Both epithelial and myoepithelial layers present
    • Apocrine metaplasia may be prominent
    • Occasionally contains sclerosing adenosis or radial scar
    • Necrosis and mitotic figures may be seen in rare cases
  • Typically in adolescent or young adult female

Richard L Kempson MD
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Original posting:: May 27, 2006

Supplemental studies


  • No reported studies, but myoepithelial layer would be expected to be positive for actin, calponin and p63

Differential Diagnosis

Fibrocystic Disease Juvenile Papillomatosis
Non-circumscribed Circumscribed
Uncommon under age 25 Mean age 20

Pubertal Macromastia Juvenile Papillomatosis
Diffuse Circumscribed
Massive enlargement Mean size 4 cm
Usually bilateral Usually solitary
Predominantly stromal process Prominent cysts

Juvenile Fibroadenoma Juvenile Papillomatosis
Cellular stroma Paucicellular stroma
Canalicular and tubular epithelium Prominent cysts


  • Median age 20
    • Reported from neonates to age 60
  • Most cases in females
    • Rare cases in males
  • May be a marker of a family at risk for breast carcinoma
    • Not clear if the lesion is a precursor
    • Patients with carcinoma or family history of carcinoma had multifocal and recurrent disease
    • Solitary, non-recurrent lesions not associated with carcinoma


Breast Tumors and Lesions Exhibiting Reactivity for Muscle/Myoepithelial Markers

(Most benign lesions with an epithelial component will have a myoepithelial cell layer)

Breast lesions preferentially presenting in juvenile/adolescent/pubertal breast

(These may, of course occasionally present at other ages and other lesions may rarely present at this age)


  • Rosen PP, Oberman HA. Tumors of the Mammary Gland, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 7, 1993
  • Pacilli M, Sebire NJ, Thambapillai E, Pierro A. Juvenile papillomatosis of the breast in a male infant with Noonan syndrome, cafe au lait spots, and family history of breast carcinoma. Pediatr Blood Cancer. 2005 Dec;45(7):991-3.
  • Nio Y, Minari Y, Hirahara N, Takamura M, Sasaki S, Iguchi C, Tamura K. A Case of Multiple Juvenile Papillomatosis of the Breast and Its Immunohistochemical Pathology. Breast Cancer. 1998 Apr 25;5(2):187-193.
  • Rosen PP, Kimmel M. Juvenile papillomatosis of the breast. A follow-up study of 41 patients having biopsies before 1979. Am J Clin Pathol. 1990 May;93(5):599-603.
  • Bazzocchi F, Santini D, Martinelli G, Piccaluga A, Taffurelli M, Grassigli A, Marrano D. Juvenile papillomatosis (epitheliosis) of the breast. A clinical and pathologic study of 13 cases. Am J Clin Pathol. 1986 Dec;86(6):745-8.
  • Rosen PP, Holmes G, Lesser ML, Kinne DW, Beattie EJ. Juvenile papillomatosis and breast carcinoma. Cancer. 1985 Mar 15;55(6):1345-52.
  • Rosen PP, Lyngholm B, Kinne DW, Beattie EJ Jr. Juvenile papillomatosis of the breast and family history of breast carcinoma. Cancer. 1982 Jun 15;49(12):2591-5.
  • Rosen PP, Cantrell B, Mullen DL, DePalo A. Juvenile papillomatosis (Swiss cheese disease) of the breast. Am J Surg Pathol. 1980 Feb;4(1):3-12.
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