Nipple Adenoma (Florid Papillomatosis of the Nipple)

Clinical Presentation

The nipple location as well as proliferative histopathology of florid papillomatosis gives rise to an interesting constellation of clinical manifestations. Almost all but a few examples from a supernumerary nipple and another from an axillary accessory nipple have originated in the nipple proper. Nipple discharge is the most common presenting symptom and is reported to occur in 65% to 70% of patients. The discharge is often bloody but can also be serous or serosanguineous. Nipple discharge can be intermittent or constant, with symptomatic exacerbation just before menses. In some instances, a mass or discrete indurated area in or under the nipple can be visibly appreciated or palpated. In one case report, a patient presented with a friable mass, projecting in an outward manner from the nipple. On palpation, the tumor is typically adherent to the overlying skin but freely movable from the underlying breast tissue. The nipple itself can appear enlarged, thickened, swollen, or, rarely, retracted. In conjunction with these findings, the overlying nipple skin may be eroded, ulcerated, reddened, scaly, crusty, and/or thickened. Such dermatoses of the nipple can be misdiagnosed as eczema or inflammatory skin disorders and, thus, initially (mis)treated with topical medication. If the surface of the nipple is clinically involved, the entity is usually mistaken for Paget’s disease. Alternatively, the skin can be intact but hyperplastic. When the surface of the skin is intact but a tumor is evident, the clinical suspicion is that of a papillary lesion (i.e., papilloma).

Patients can experience localized pain, itching, or a burning sensation. Coexisting axillary lymphadenopathy does not occur. Typically, florid papillomatosis of the nipple is not considered in the clinical differential diagnosis because even the most experienced clinicians will see it only a few times in their professional careers.

The majority of patients are women who are in their fifth decade of life at the time of diagnosis. However, florid papillomatosis of the nipple has been reported to occur in children as young as 5 months old, adolescents, as well as older patients, including an 89-year-old woman. This tumor uncommonly arises in men (less than 5%), and, in a minority of these cases, coexistent invasive and/or in situ ductal carcinoma has been reported arising within it. There is one report of a man developing florid papillomatosis after long-term treatment with diethylstilbestrol for prostatic carcinoma.

There is no predisposition in laterality. Bilateral or incidental cases are rare. Most individuals seek medical attention shortly (months) after developing clinical symptoms; however, some patients harbor the lesion for many (>10) years. Patients who present with nipple ulceration or erosion are more likely to seek medical attention sooner owing to the alarming nature of the symptoms. Likewise, clinicians are more suspicious of a malignancy (i.e., Paget’s disease) when patients present in this manner.

The etiology of florid papillomatosis is unknown and largely understudied. Possible causes, such as trauma, have been considered but are not confirmed. Some authors consider nipple adenoma in the same proliferative spectrum as complex sclerosing lesions, papillomas, and adenosis tumors differing only in location and the predominant growth pattern.

The overall incidence of florid papillomatosis of the nipple in the general population as well as in patients with breast cancer is unknown. Furthermore, this entity has not been found to be a proven risk factor for the development of carcinoma or more frequently found in those with a family history of breast cancer.

The coexistence of florid papillomatosis of the nipple and ipsilateral or contralateral mammary carcinoma has been reported in retrospective studies of breast specimens performed for carcinoma and range in frequency from 1.2% to 16.5% in these studies. These tumors were found to occur independently with sufficient distance and intervening breast parenchyma.

Even more uncommon is the occurrence of carcinoma arising from preexisting florid papillomatosis of the nipple. To date, there are fewer than 50 such cases reported, almost half of which occurred in men. ^aa

aa References .

The apparently high frequency of carcinoma associated with florid papillomatosis in men is most likely a result of their shared predisposition to arise in the central, subareolar region of the breast and, as such, the notion that florid papillomatosis has precancerous potential in men has not been substantiated. Interestingly, almost all of these patients had invasive or in situ carcinoma exclusively of the ductal type with or without concurrent ductal carcinoma in situ (DCIS) and/or Paget’s disease, except for three cases reported of spindle cell metaplastic carcinoma. An occasional case of closely approximated florid papillomatosis with severely atypical ductal hyperplasia and invasive ductal carcinoma has also been reported. Benign lesions have also been reported to occur concurrently with florid papillomatosis such as fibroadenoma and papilloma.