Skip to Main Content


The disease processes discussed in this chapter are uncommon tumors: phyllodes tumors, sarcomas, lymphomas, melanomas, and metastases to the breast. As a group, these tumors are rare and heterogeneously diverse. Given their small numbers, there have been few large patient series or trials, and no large prospective trials will likely be performed. The majority of the literature consists of single-institution, retrospective studies/case reports with small numbers, varied follow-up, and inherent biases. Furthermore, these studies are difficult to compare as the definition, management, and treatment of these tumors have changed over time. Therefore, there are no established guidelines for the treatment of these uncommon tumors; patients with these rare tumors should be managed in a multidisciplinary fashion.


Phyllodes tumors represent a spectrum of fibroepithelial neoplasms that have biological behavior that is diverse and unpredictable. They account for less than 1% of all breast neoplasms.1,2 These tumors have also been referred to as cystosarcoma phyllodes, phylloides tumors, and periductalstromal tumors. The term cystosarcoma phyllodes was first introduced in 1838 by Johannes Mueller; "sarcoma" for the fleshy nature of the tumor and "phyllodes" for its leaf-like architecture.3 Mueller emphasized the benign nature of this tumor. The first report of histologically malignant features in these tumors was by Lee and Pack in 1931.4 In 1960, Lomonaco proposed the name tumor phyllodes to avoid any implications of biological behavior.5 The World Health Organization proposed phyllodes tumor to emphasize the putative origin of these tumors from specialized periductal stroma and to avoid the designation of sarcoma with its deceptive implication of malignancy for a majority of these tumors.6 These tumors can arise de novo, and less frequently from preexisting fibroadenomas or from the malignant transformation of benign phyllodes tumors.2


Clinical Presentation


Phyllodes tumors occur over a wide age range from adolescents to the elderly, with the majority of tumors occurring in women in their 40s to 50s.1,2,7,8 These tumors can occur in young children and men. Women usually present with a palpable, firm-hard, discrete, mobile mass with an average size of 4 to 5 cm. Most tumors are unilateral and painless. Some women may give a history of a stable mass that grows rapidly. Larger tumors may cause stretching of the overlying skin and ulceration. All of these findings can be seen in both benign and malignant tumors. Compared with fibroadenomas, phyllodes tumors are seen more frequently in older patients and those with a history of rapid tumor growth and/or larger tumors. Palpable axillary lymphadenopathy can be identified in up to 20% of patients but is usually due to reactive changes as metastatic involvement is rare.9-11 In some instances, tumors may be multifocal, bilateral, or occur in ectopic breast tissue. In 1% to 2% of cases, an in situ or invasive breast carcinoma occurs within a phyllodes tumor.12 Also, patients can have ...

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.


About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessSurgery Full Site: One-Year Subscription

Connect to the full suite of AccessSurgery content and resources including more than 160 instructional videos, 16,000+ high-quality images, interactive board review, 20+ textbooks, and more.

$995 USD
Buy Now

Pay Per View: Timed Access to all of AccessSurgery

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.