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CASE REPORT Table of Contents  
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Swiss cheese disease: A rare case of juvenile papillomatosis of the breast mimicking carcinoma in a young female

1 Department of Surgery, Mysore Medical College and Research Institute, Mysore, Karnataka, India
2 Department of Surgery, DM Wayanad Institute of Medical Sciences, Wayanad, Kerala, India

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Date of Submission13-Jun-2021
Date of Decision12-Dec-2021
Date of Acceptance22-Dec-2021
Date of Web Publication13-Jan-2023


Juvenile papillomatosis is a rare benign proliferative lesion of breast seen in young females. These patients have been reported to have a strong family history for carcinoma breast. It is rarely diagnosed preoperatively but has distinct histopathological features on postoperative examination. The subsequent treatment and prognosis vary accordingly. Here, we report a case of a 38-year-old female who was operated with a preliminary diagnosis of Mucinous carcinoma of left breast.

Keywords: Breast, cancer, juvenile papillomatosis

How to cite this URL:
Hameed R, Vettuparambil A. Swiss cheese disease: A rare case of juvenile papillomatosis of the breast mimicking carcinoma in a young female. Indian J Pathol Microbiol [Epub ahead of print] [cited 2023 Feb 2]. Available from:

   Introduction Top

Juvenile papillomatosis (JP) of the breast is a rare benign proliferative disease described in young women.[1],[2] Histopathologic features include atypical papillary duct hyperplasia and numerous cysts, hence known as Swiss Cheese Disease.[3] It has been observed that patients diagnosed with JP in the past develop breast carcinoma during follow-up. This report describes a case of a 38-year-old female with Swiss Cheese Disease.

   Case Report Top

A 38-year-old female presented with a lump in the left breast without pain for about 2 months. On palpation, there was a non-tender, hard elliptical lump of size 4 × 2 cm in the upper inner quadrant of left breast which moves with breast tissue. There were no signs of skin involvement or of an abscess. Opposite breast and both axillae were normal. Nipple and areola were normal. She was otherwise healthy and there was no family history of breast cancer and was not on any regular medications.

A solid lesion with cystic component in dimension of 4 × 2 cm was seen on breast ultrasonography. Fine needle aspiration cytology was reported as Mucinous carcinoma of left breast. Rest of the investigations were within normal limits. Owing to the clinical diagnosis and pathological findings, Mastectomy with axillary staging was done.

On macroscopic examination, Mastectomy specimen measuring 13 × 10 × 5 cm with attached skin bearing nipple-areola measuring 11 × 6 cm and unremarkable external surface was observed. On serial sectioning, mass identified in upper medial quadrant measuring 4 × 3 × 2 cm. All margins appeared to be uninvolved grossly. Serial sections taken from the mass proper showed multiple cystic formations resembling Swiss cheese [Figure 1]. Multiple sections were taken from superior, inferior, medial, lateral, and deep surgical margins along with nipple-areola complex and area adjacent to mass proper. Microscopic examination showed numerous cystically dilated ducts lined by bilayer epithelium. The epithelial cells show hyperplasia and papillomatosis (small papillae projecting into the lumen) [Figure 2], [Figure 3], [Figure 4]. Apocrine metaplasia of hyperplastic intraductal epithelium was also seen. No atypia was noted in the lining epithelium. These cystically dilated ducts were filled with foamy histiocytes admixed with amorphous eosinophilic secretions. Adjacent stroma showed fibro collagenous and fibroadipose tissue. These findings are suggestive of JP. There were no features suggestive of coexisting cancerous lesion. Patient was advised to follow-up regularly.
Figure 1: Macroscopic view of operated specimen

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Figure 2: Microscopic view of Juvenile papillomatosis of breast

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Figure 3: Microscopic view of Juvenile papillomatosis of breast

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Figure 4: Microscopic view of Juvenile papillomatosis of breast

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   Discussion Top

JP is a clinicopathological entity seen in young females which causes a lump in the breast.[1],[2] The macroscopic view of multiple cysts within a solid structure gave its nick name Swiss Cheese Disease. Ultrasonography of breast is helpful in differentiating the lesion from other tumors and lumps. Mammography and Magnetic Resonance Imaging (MRI) aids in the diagnosis.[4] The histopathologic criteria for diagnosis include duct papillomatosis with or without a degree of epithelial atypia, apocrine and non-apocrine cysts, papillary apocrine hyperplasia, sclerosing adenosis, and duct stasis.[1],[3] Constant features like cysts, duct hyperplasia, and papillary projections in to the lumen as observed in this case differentiate JP from other papillary lesions.

Age of the diagnosis ranged from 10 to 48 years with mean age of 23 years. Majority of the patients are Caucasians and are younger than 20 years.[1] Cases in Asians are very rare and thus fewer reports exists.[5] Studies showed a large percentage of JP patients to have a family history for breast carcinoma in their first and second-degree relatives.[6] JP patients are at a risk of developing breast cancer in the future.[3],[6],[7] Coexisting carcinoma with JP has been reported, and secretory carcinoma of breast being the most common.[8] JP patients with bilateral, recurrent or multifocal lesions, or with a family history of carcinoma breast are at a higher risk; this demands a need for long-term follow-up.[9] No relation between development of JP and hormonal influences were described.[6] The surgical management of JP is a complete excision with histologic confirmation.[7],[10]

   Conclusion Top

Swiss cheese disease of the breast, named JP, is a rare and benign lesion that may develop in young women. The lesion can mimic benign lesions like fibroadenoma and even carcinoma as in this case, influencing the mode of treatment for the patient. Complete excision of the lesion should be done given the fact that recurrence and multicentricity has been noted. The association of JP with family history of breast carcinoma, occurrence of coexisting carcinomatous lesions in these patients, and the probability to develop breast cancer in future warrants close follow-up of these patients to ensure early and better care when chances of cure are at its best.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Rosen PP, Cantrell B, Mullen DL, DePalo A. Juvenile papillomatosis (Swiss Cheese Disease) of the breast. Am J Surg Pathol 1980;4:3-12.  Back to cited text no. 1
Rosen PP, Lyngholm B, Kinne DW, Beattie EJ Jr. Juvenile papillomatosis of the breast and family history of breast carcinoma. Cancer 1982; 49: 2591-5.  Back to cited text no. 2
Gill J, Greenall M. Juvenile papillomatosis and breast cancer. J Surg Educ 2007;64:234-6.  Back to cited text no. 3
Ohlinger R, Schwesinger G, Schimming A, Köhler G, Frese H. Juvenile papillomatosis (JP) of the female breast (Swiss Cheese Disease) -Role of breast ultrasonography. Ultraschall Med 2005;26:42–5.  Back to cited text no. 4
Wang T, Li YQ, Liu H, Fu XL, Tang SC. Bifocal juvenile papillomatosis as a marker of breast cancer: A case report and review of the literature. Oncol Lett2014;8:2587-90.  Back to cited text no. 5
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;93:599-603.  Back to cited text no. 6
Mario T, Donatella S, Guiseppe M, Mazzoleni G, Rossati U, Giosa F, et al. Juvenile papillomatosis of the breast: Amultidisciplinary study. PatholAnnu 1991;1:25-35.  Back to cited text no. 7
Tokunaga M, Wakimoto J, Muramoto Y, Sato E, Toyohira O, Tsuchimochi A, et al. Juvenile secretory carcinoma and juvenile papillomatosis. Jpn J Clin Oncol 1985;15:457-65.  Back to cited text no. 8
Viswanathan K, McMillen B, Cheng E, D'Alfonso T, Patel A, Hoda SA. Juvenile papillomatosis (Swiss-Cheese Disease) of breast in an adult male with sequential diagnoses of ipsilateral intraductal, invasive, and widely metastatic carcinoma: A case report and review of the disease in males. Int J Surg Pathol 2017;25:536–42.  Back to cited text no. 9
Kafadar MT, Anadolulu Z, Anadolulu Aİ, Tarini EZ. Juvenile papillomatosis of the breast in a pre-pubertal Girl: An uncommon diagnosis. Eur J Breast Health 2018;14:51–3.  Back to cited text no. 10

Correspondence Address:
Ajith Vettuparambil,
Department of Surgery, DM Wayanad Institute of Medical Sciences, Wayanad, Kerala - 673577
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_597_21


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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