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Background
This is a rare but important tumor that arises primarily in adolescent girls. There is controversy over whether this disease is a marker for later development of breast cancer or if it is a preneoplastic lesion itself. These tumors most commonly present as a solitary, unilateral mass.
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Swiss cheese disease AGE RANGE-MEDIAN 50% are diagnosed F overwhelmingly
DISEASE ASSOCIATIONS CHARACTERIZATION Breast cancer A history of breast cancer in first degree relatives is found in 50% of cases
LABORATORY/RADIOLOGIC CHARACTERIZATION Juvenile papillomatosis of the breast: sonographic appearance.
Kersschot EA, Hermans ME, Pauwels C, Gildemyn G, Chabeau P, De Vos L, Van Hoorde P.
Department of Radiology, St Vincent Clinic, Gent, Belgium.
Radiology 1988 Dec;169(3):631-3 Abstract quote
Three patients with juvenile papillomatosis of the breast are described. The lesions were apparent clinically as a palpable mass. At mammography, juvenile papillomatosis was not depicted in two patients and was seen as a poorly defined mass in one case.
In all patients, high-frequency ultrasound examination showed an ill-defined, inhomogeneous mass with one or several small (up to 0.4 cm), rounded, relatively echo-free areas, which were seen mainly or exclusively near the borders of the lesion.
MR imaging of juvenile papillomatosis of the breast.
Mussurakis S, Carleton PJ, Turnbull LW.
Centre for MR Investigations, University of Hull, UK.
Br J Radiol 1996 Sep;69(825):867-70 Abstract quote
Juvenile papillomatosis of the breast is a benign, localized proliferative condition encountered mainly in young women.
We describe the contrast-enhanced MR imaging findings, including dynamic data, of a patient with histologically proven juvenile papillomatosis of the breast. The pre-contrast T1 weighted sequence showed a hypointense lobulated mass which enhanced markedly in the post-contrast images. In the dynamic sequence the lesion showed a benign enhancement profile.
The most specific feature was the presence of multiple small internal cysts, best shown on the T2 weighted images. This constellation of findings should suggest juvenile papillomatosis.
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION General Well circumscribed tumors averaging 4 cm
Cross sections reveal multiple cysts separated by fibrous septaVARIANTS GIANT Giant juvenile papillomatosis of the breast: report of two cases.
Farid MK, Sarma HN, Ramesh K, al Fituri O, Visveswara RN.
Department of Pathology, Faculty of Medicine, Al Arab Medical University, Beaghazi, Libya.
East Afr Med J 1997 Feb;74(2):116-7 Abstract quote
Two cases of giant juvenile papillomatosis of the breast, a rare disease occurring in adolescent girls, are reported from Libya. Both cases had been initially misdiagnosed as phylloides tumour of the breast due to the large size of the tumours.
Juvenille papillomatosis should be included in the differential diagnosis of a large breast mass, particularly in young girls.
MALE BREAST A case of juvenile papillomatosis of the male breast.
Sund BS, Topstad TK, Nesland JM.
Department of Pathology, National Hospital, Oslo, Norway.
Cancer 1992 Jul 1;70(1):126-8 Abstract quote
BACKGROUND. Juvenile papillomatosis is a proliferative epithelial lesion of the female breast, and Rosen et al. introduced it in 1980 as an entity.
RESULTS. The authors present a case consistent with juvenile papillomatosis of the male breast, occurring in a 33-year-old man, who had noticed a swelling of his right breast for approximately 17 years. On review of the literature regarding epithelial proliferations of the male breast, the authors found three cases with similar histologic features.
CONCLUSIONS. The case studied indicates a benign behavior of this lesion in the male breast, although no data concerning long-term prognosis are available.
Juvenile papillomatosis of the breast in male infants: two case reports.
Rice HE, Acosta A, Brown RL, Gutierrez C, Alashari M, Mintequi D, Rodriguez A, Chavarrfa O, Azizkhan RG.
Department of Pediatric Surgery, Children's Hospital of Buffalo, and the State University of New York at Buffalo, USA.
Pediatr Surg Int 2000;16(1-2):104-6 Abstract quote
Juvenile papillomatosis of the breast ("Swiss cheese disease") is a benign localized proliferative condition of the breast which occurs almost exclusively in young adult women. Patients with this lesion often have a family history of breast carcinoma, and rarely carcinoma may coexist with the lesion at the time of diagnosis.
We present two cases of male infants with juvenile papillomatosis of the breast. The pathology and clinical management of this novel lesion is discussed.
A case of breast cancer associated with juvenile papillomatosis of the male breast.
Munitiz V, Illana J, Sola J, Pinero A, Rios A, Parrilla P.
Department of Surgery, Virgen de la Arrixaca University Hospital, Murcia, Spain.
Eur J Surg Oncol 2000 Nov;26(7):715-6 Abstract quote
Juvenile papillomatosis of the breast (JPB) was first described in 1980 and is occasionally associated with breast cancer. The literature reports only four cases of JPB in males; none of them associated simultaneously with breast cancer.
We present a case of a male with JPB associated with a ductal carcinoma in the same gland.
HISTOLOGICAL TYPES CHARACTERIZATION General Multiple cysts lined by papillary ductal hyperplasia and low cuboidal epithelium
Secretions may be presentJuvenile papillomatosis (Swiss cheese disease) of the breast.
Rosen PP, Cantrell B, Mullen DL, DePalo A.
Am J Surg Pathol 1980 Feb;4(1):3-12 Abstract quote
Thirty-seven cases of juvenile papillomatosis of the breast were reviewed. The average age was 19 years (range 10-44 years). On examination the patients usually had a localized, multinodular mass that was interpreted as a fibroadenoma.
Microscopically, typical lesions featured papillomatosis, sometimes with severe atypia, cysts with or without apocrine metaplasia, duct stasis, and sclerosing adenosis. In one case the breast with papillomatosis adenosis. In one case the breast with papillomatosis has remained free of carcinoma, but at the time of biopsy the patient had secretory carcinoma of the other breast. Papillomatous changes were not observed in the breast with carcinoma. Another woman had lobular carcinoma in situ coexisting with juvenile papillomatosis. After an average follow-up of 8 years, no subsequent carcinoma has been found.
Thus, there appears to be little risk for early onset of carcinoma, but without additional follow-up the long-term assessment of this disorder remains uncertain. We recommend that a patient with juvenile papillomatosis have a thorough clinical examination of the breasts at least once a year.
SPECIAL STAINS/IMMUNOHISTOCHEMISTRY CHARACTERIZATION Juvenile secretory carcinoma and juvenile papillomatosis: diagnosis and treatment.
Ferguson TB Jr, McCarty KS Jr, Filston HC.
J Pediatr Surg 1987 Jul;22(7):637-9 Abstract quote
Carcinoma of the breast in the child is a rare pathologic entity, constitutes less than 1% of all breast lesions in this age group. The case of a girl with a left breast mass first noticed at 4.5 years of age is presented. An eccentric subareolar mass was excised at 6 years of age, sparing her breast bud. This biopsy contained juvenile secretory carcinoma, with additional pathologic changes characteristic of juvenile papillomatosis.
Both the juvenile papillomatosis component as well as the juvenile secretory carcinoma component were devoid of estrogen and progesterone receptors in contrast to nondiseased breast tissue. The assay for these receptors was performed using a new antireceptor monoclonal antibody technique.
Simple mastectomy was performed after completion excision demonstrated tumor at the pectoralis major fascia.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES DUCTAL PAPILLOMATOSIS Extreme duct papillomatosis of the juvenile breast.
Kiaer HW, Kiaer WW, Linell F, Jacobsen S.
Acta Pathol Microbiol Scand [A] 1979 Sep;87A(5):353-9 Abstract quote
Three cases of peculiar and very severe ductal papillomatosis of the breast are reported. This ductal papillomatosis was unusual, both in its histological architecture and in occurring in juveniles, viz. girls aged 11, 14, and 17 years.
In two of the patients breast cancer was diagnosed 27 and 11 years later. In one of them the ductal papillomatosis must be presumed to have been precancerosis, while in the other patient there was possibly from the outset an unusually papilliferous, intraductal carcinoma of low malignancy.
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSIS GENERAL Juvenile papillomatosis of the breast and family history of breast carcinoma.
Rosen PP, Lyngholm B, Kinne DW, Beattie EJ Jr.
Cancer 1982 Jun 15;49(12):2591-5 Abstract quote
Eighty-four patients included in the Juvenile Papillomatosis Registry were reviewed.
The age of diagnosis ranged from 10-44 years (mean, 21). Sixty-nine percent of patients were less than or equal to 20 years of age. The left breast was affected slightly more often (52%) than the right (48%) when the lesion was unilateral. Two patients had bilateral lesions. Patterns of menarche, marital history, parity, and use of birth control pills were not exceptional for women in this age group. No instance was found of maternal use of estrogens during pregnancy. There were no instances of carcinoma developing after biopsy of juvenile papillomatosis, but a newly diagnosed patient had secretory carcinoma arising from juvenile papillomatosis. Two other examples of concurrent carcinoma were previously reported (ipsilateral lobular carcinoma in situ and contralateral secretory carcinoma).
Particularly noteworthy is the finding that 26% of patients wih information available reported that breast carcinoma had occurred in one or more female relatives. The major were older, secondary relatives (grandmothers, great aunts), although maternal breast carcinoma (five cass) was also reported. The mothers of both girls who had secretory carcinoma associated with juvenile papillomatosis had been treated for breast cancer.
These findings, based on incomplete reporting, are cause for concern in two respects: the findings suggest that juvenile papillomatosis may be a marker for breast cancer for the patient's family, and they indicate a need for continued long-term follow-up of juvenile papillomatosis patients who may themselves be at increased risk.
Juvenile papillomatosis and breast carcinoma.
Rosen PP, Holmes G, Lesser ML, Kinne DW, Beattie EJ.
Cancer 1985 Mar 15;55(6):1345-52 Abstract quote
Juvenile papillomatosis (JP) is a breast tumor of young women featuring atypical papillary duct hyperplasia and numerous cysts, first described as a clinicopathologic entity in 1980.
The disease is of interest because of the youth of these patients and the fact that the pathologic elements resemble those considered to be precancerous in older women. This report summarizes the findings in 180 patients enrolled in the Juvenile Papillomatosis Registry. The mean age at diagnosis was 23 years (range, 12-48 years; median, 21 years; mode, 18 years). No relationship was found with parity, age of menarche, or use of birth control pills. Fifty patients (28%) reported that one or more relatives had had breast cancer. Twelve (7%) patients reported a first degree relative (mother, 10; sister, 2), whereas in 38 (21%) breast cancer was limited to one or more secondary relatives, most often maternal (23/39 or 59%). Persistent (or recurrent) JP was found in 25 patients who had a subsequent biopsy, and 8 patients had bilateral JP. Pathologic analysis revealed focal epithelial necrosis, a feature often associated with carcinoma, in 30 (17%) cases predominantly in those with the most severe cytologic atypia of hyperplastic duct lesions (77%). Positive family history was reported by 33% (10/30) of women with epithelial necrosis and 27% (40/150) without this finding. Seven patients (4%) had breast carcinoma diagnosed concurrent with JP (ipsilateral, 5 cases; contralateral, 2 cases). A previously unreported finding is the diagnosis of carcinoma subsequent to JP in two cases (contralateral intraductal and ipsilateral microinvasive duct, respectively) with intervals of 8 and 9 years, respectively. The nine patients with carcinoma and JP tended to be slightly older at the time JP was diagnosed (mean, 27 years) and had a higher frequency of positive family history (56%) than those without breast cancer (mean age, 23 years; positive family history, 26%).
These findings confirm earlier evidence that JP is a marker for families at risk and now indicate that the patient with JP may herself be in jeopardy of developing breast cancer coincidentally or at a later date. Wide local excision is adequate to control the lesion in most cases. Careful clinical surveillance is indicated for any woman who has juvenile papillomatosis and for her female relatives. The early age of onset of this unusually florid proliferative disease, and family history association, suggest there may be an underlying hormonal disturbance or genetic abnormality.
Juvenile papillomatosis (epitheliosis) of the breast. A clinical and pathologic study of 13 cases.
Bazzocchi F, Santini D, Martinelli G, Piccaluga A, Taffurelli M, Grassigli A, Marrano D
Am J Clin Pathol 1986 Dec;86(6):745-8 Abstract quote
The clinical and pathologic findings of 13 patients with juvenile papillomatosis (JP) (epitheliosis) of the breast are analyzed. The average age was 25 years, with a range from 15 to 42. Family history of breast carcinoma was observed in 33%. Carcinoma did not develop in any patient after the diagnosis of JP. A co-existing carcinoma was present in 15% of the patients.
This study confirms that JP may be related to family history of breast carcinoma and that the patients themselves may be at increased risk for the development of cancer. A long-term follow-up for these patients and relatives is needed so that additional information and better knowledge of this entity can be obtained.
Juvenile papillomatosis of the breast. A follow-up study of 41 patients having biopsies before 1979.
Rosen PP, Kimmel M.
Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York 10021.
Am J Clin Pathol 1990 May;93(5):599-603 Abstract quote
Juvenile papillomatosis (JP) is a benign proliferative breast tumor rarely found in women older than 30 years of age that may exhibit considerable histologic atypia. Often these findings would be regarded as precancerous in older patients. Rarely, carcinoma and JP have been coexistent at diagnosis, but little is known about the risk for development of carcinoma after excisional biopsy of JP.
To examine this issue, the authors reviewed a series of 41 patients with JP who had biopsies between 1960 and 1978 with a median follow-up of 14 years. Age at diagnosis ranged from 15 to 35 years (median, 19 years), whereas at last follow-up the patients were 22-48 years old (median, 36 years). Overall, 21 (58%) reported a positive family history for breast carcinoma, with mothers and maternal aunts being at highest risk. Six had bilateral JP. Subsequent breast carcinoma was diagnosed in four (10%) of the patients with JP. All of these patients were unusual because they had recurrent and bilateral JP. In two instances the changes were multifocal. Each had a positive family history for breast carcinoma.
In this series, none of the patients with nonrecurrent unilateral, unicentric JP has thus far had subsequent mammary carcinoma develop. When counseling the JP patient one should take care not to exaggerate her near term risk for development of carcinoma. The present data suggest that concern should be greatest for women with a positive family history and recurrent bilateral JP.
A Case of Multiple Juvenile Papillomatosis of the Breast and Its Immunohistochemical Pathology.
Nio Y, Minari Y, Hirahara N, Takamura M, Sasaki S, Iguchi C, Tamura K.
First Operation of Surgery, Shimane Medical University, 89-1 Enya-cho, Izumo 693, Japan.
Breast Cancer 1998 Apr 25;5(2):187-193 Abstract quote
Juvenile papillomatosis(JP)is a benign proliferative disease of the breast affecting young women. Some patients with JP develop breast carcinoma.
We recently treated and followed a case of JP in a 16-year-old female, and studied its immunohistochemical characteristics. The patient noticed a small tumor, 5 mm in diameter, in the periareolar region on the medial side of the right breast. She underwent an excisional biopsy and pathology demonstrated JP. However, 6 months later, two new lesions were discovered by routine echogram examination, and were later excised. Immunohistochemistry demonstrated that the JP tumors were negative for estrogen receptor, but positive for progesterone receptor, epidermal growth factor receptor and erbB-2. In addition, tests for p53 were negative and Rb protein was normally expressed. Immunohistochemical analysis suggested that an abnormality in estrogen receptor is important in the pathogenesis of JP, and that the patient had a relatively high risk of developing breast carcinoma, since her JP is multicentric and recurrent.
The present study also suggests that immunohistochemical pathology may be beneficial in assessing the malignant potential of JP.
RECURRENCE May occur with incompletely excised tumors SECRETORY CARCINOMA Juvenile secretory carcinoma and juvenile papillomatosis.
Tokunaga M, Wakimoto J, Muramoto Y, Sato E, Toyohira O, Tsuchimochi A, Funasako S, Tachiwada W.
Jpn J Clin Oncol 1985 Jun;15(2):457-65 Abstract quote
Two cases of juvenile secretory carcinoma associated with juvenile papillomatosis are reported. One patient's disease was initially diagnosed at the age of 13 and she died 12 years later with extensive metastases. The contralateral breast at autopsy showed findings indicative of juvenile papillomatosis. The second case is that of an 18-year-old girl who had multiple juvenile secretory carcinomas in the left breast associated with juvenile papillomatosis.
Secretory carcinoma of the breast associated with juvenile papillomatosis in a 12-year-old girl. A case report.
Nonomura A, Kimura A, Mizukami Y, Nakamura S, Ohmura K, Watanabe Y, Tanimoto K, Ikegaki S.
Department of Internal Medicine, Kanazawa University Hospital, School of Medicine, Japan.
Acta Cytol 1995 May-Jun;39(3):569-76 Abstract quote
A rare case of juvenile secretory carcinoma (SC) associated with juvenile papillomatosis (JP) is described.
Tumor biopsy and fine needle aspiration cytology (FNAC) were performed in a 12-year-old girl who had noticed a painful right breast mass. One of the two types of tumor cells found on FNAC smears formed mainly sheets or clusters, intercellular spaces within the sheets and intracytoplasmic vacuolation. These cells, a few of which had a signet-ring cell appearance with completely disordered and randomly arranged nuclei, were considered to be SC cells. Cells of the other type were found in scattered papillary clusters and had well-defined cytoplasmic borders, uniform cell and nuclear size, and an orderly nuclear arrangement, suggesting a benign papillary lesion.
Histologic examination of the tumor biopsy and breast quadrantectomy specimens revealed SC with JP. The cytologic, histologic and electron microscopic features of SC are described, and the relationship between SC and JP is discussed.
TREATMENT Complete excision Am J Clin Pathol 1990;93:599-603
Semin Diagno Pathol 1999;16:235-247.
Last Updated 2/21/2002
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