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Phyllodes tumors of the breast

Authors: Ana M Grau, MD, FACS, A Bapsi Chakravarthy, MD, FASTRO, Rashmi Chugh, MD
Section Editors: Lori J Pierce, MD, Daniel F Hayes, MD, Anees B Chagpar, MD, MSc, MA, MPH, MBA, FACS, FRCS(C)
Deputy Editor: Wenliang Chen, MD, PhD

All topics are updated as new evidence becomes available and our peer review process is complete.

Literature review current through: Dec 2020. | This topic last updated: May 09, 2019.

INTRODUCTION

Phyllodes tumors are uncommon fibroepithelial breast tumors that are capable of a diverse
range of biologic behaviors. In their least aggressive form, phyllodes tumors behave like benign
fibroadenomas, although with a propensity to recur locally following excision without wide
margins. At the other end of the spectrum, other phyllodes tumors can metastasize distantly,
sometimes degenerating histologically into sarcomatous lesions that lack an epithelial
component [1,2].

The term "phyllodes," which means leaf-like, describes the typical papillary projections that are
seen on pathologic examination. Although they were originally called "cystosarcoma phyllodes"
by Johannes Müller in 1838 [2], phyllodes tumors only occasionally have cystic components and
are not true sarcomas by either cellular origin or biologic behavior. The terminology has since
evolved, with over 60 synonyms having been applied to this entity before the term "phyllodes
tumors" was adopted by the World Health Organization [3,4].

The clinical presentation, diagnosis, treatment, and prognosis of breast phyllodes tumors are
discussed in this topic. Other benign (eg, fibroadenoma) or malignant lesions of the breast are
discussed elsewhere. (See "Overview of benign breast disease" and "Clinical features, diagnosis,
and staging of newly diagnosed breast cancer" and "Overview of the treatment of newly
diagnosed, invasive, non-metastatic breast cancer".)

EPIDEMIOLOGY AND RISK FACTORS

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Phyllodes tumors account for less than 1 percent of all breast neoplasms [4,5]. Given their rarity,
epidemiologic data are scant. In a study from Los Angeles county over a 17 year period, the
average annual incidence rate of malignant phyllodes tumors was 2.1 per million women, and
the incidence was higher in Latina whites than in non-Latina whites, Asians, and African
Americans [6].

The vast majority of phyllodes tumors occur in women, with a median age of presentation of 42
to 45 years (range 10 to 82 years) [3,6-8]. Higher-grade tumors are more common in older
patients [9]. In men, phyllodes tumors usually occur in association with gynecomastia [3].

Phyllodes tumors have been associated with Li-Fraumeni syndrome, a rare autosomal dominant
condition that is characterized by the development of multiple tumors [10]. No other etiologic or
predisposing factors have been linked to phyllodes tumors. (See "Overview of hereditary breast
and ovarian cancer syndromes associated with genes other than BRCA1/2" and "Li-Fraumeni
syndrome".)

CLINICAL PRESENTATION

Phyllodes tumors are usually identified as a breast mass or an abnormal mammographic or

sonographic finding.

On examination, most patients have a smooth, multinodular, well-defined, firm mass that is
mobile and painless [2,7]. Tumor size is variable, ranging from 1 to 41 cm (average 4 to 7 cm)
[4,8]. Shiny, stretched, and attenuated skin may be seen overlying a large tumor [2]. Nipple
retraction, ulceration, chest wall fixation, and bilateral diseases are rare but have been
described for phyllodes tumors [2,11].

Approximately 20 percent of phyllodes tumors present as a nonpalpable mass identified on

screening mammography [12]. The typical appearance of a phyllodes tumor on mammography
is a smooth, polylobulated mass resembling a fibroadenoma [2]. (See "Breast imaging for
cancer screening: Mammography and ultrasonography" and "Diagnostic evaluation of women
with suspected breast cancer".)

Phyllodes tumors may grow slowly or rapidly or exhibit a biphasic growth pattern. As they grow
larger, phyllodes tumors can form a visible mass that distorts the contour of the breast or even
cause pressure necrosis of the overlying skin.

Although palpable axillary lymphadenopathy can be identified in up to 20 percent of patients,

most are reactive; metastatic involvement of lymph nodes with phyllodes tumor is rare [4,7].

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DIAGNOSTIC EVALUATION

Phyllodes tumors should be suspected when a patient presents with a large (>3 cm), rapidly
growing breast mass that is usually palpable. Although imaging features of a phyllodes tumor
can be suggestive of fibroadenoma, the large size and history of rapid growth indicate
otherwise. Breast masses suspicious for a phyllodes tumor should undergo biopsy or surgical
excision and are usually diagnosed on pathology.

Imaging — Phyllodes tumors can present as a palpable breast mass or an abnormal finding on

screening mammography. Suspicious lesions should be examined with breast ultrasound.
Breast magnetic resonance imaging (MRI) may also be useful in select patients.

Mammography — Approximately 20 percent of phyllodes tumors present as a nonpalpable

mass identified on screening mammography [12]. The typical appearance of a phyllodes tumor
on mammography is a smooth, polylobulated mass resembling a fibroadenoma [2]. (See "Breast
imaging for cancer screening: Mammography and ultrasonography" and "Diagnostic evaluation
of women with suspected breast cancer".)

Ultrasonography — Patients with a palpable breast mass or suspicious lesion on

mammography should have an ultrasound examination. Phyllodes lesions are primarily solid,
hypoechoic, and well circumscribed on ultrasonography. Suspicion for a phyllodes tumor, rather
than a fibroadenoma, is based on clinical features such as large tumor size at presentation and
rapid growth [2]. Although not always present, cystic areas within the mass on ultrasonography
may increase the level of suspicion for phyllodes tumors [13].

Magnetic resonance imaging — Phyllodes tumors and fibroadenomas cannot be reliably

differentiated by breast MRI [14]. However, when a phyllodes tumor has been diagnosed on
core biopsy, breast MRI may help determine the extent of disease and resectability in selected
cases [15]. However, the routine use of breast MRI in surgical planning for phyllodes tumors is
controversial and not supported by data.

Malignant phyllodes tumors are seen as well-circumscribed tumors with irregular walls, high
signal intensity on T1-weighted images, and low signal intensity on T2-weighted images [16].
Cystic change may be seen as well. A rapid enhancement pattern is seen more commonly with
benign rather than malignant phyllodes tumors, which is the opposite of the pattern seen with
adenocarcinomas of the breast [16,17].

Biopsy — Breast lesions suspicious for phyllodes tumors should undergo core biopsy, which is
typically diagnostic. Compared with core biopsy, fine needle aspiration (FNA) is less accurate. If

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the core biopsy results are indeterminate or if there is clinical-pathologic discordance, an

excisional biopsy should be performed.

Fine needle aspiration — In general, FNA has been associated with a high false negative
rate and low overall accuracy for the diagnosis of phyllodes tumors [18]. Some experts,
however, advocated paying special attention to three major cytological features (fibromyxoid
stromal fragments with spindle nuclei, fibroblastic pavements, and spindle cells of fibroblastic
nature) to improve the accuracy of FNA [19].

Core needle biopsy — On core needle biopsy, additional features can help distinguish
phyllodes tumors from fibroadenomas. Such features include increased cellularity, mitosis,
stromal overgrowth, and fragmentation (stroma with epithelium at one or both ends of the
fragment). (See 'Histologic' below.)

Excisional biopsy — If the result of a core biopsy is indeterminate, an excisional biopsy is

required. A core biopsy diagnosis of "cellular fibroadenoma," "cellular fibroepithelial lesion," or
"fibroepithelial lesion with cellular stroma" should prompt surgical excision of the lesion,
regardless of its appearance on imaging studies. Further pathologic characterization of the
lesion may reveal a phyllodes tumor. Additionally, core biopsy has a 25 to 30 percent false
negative rate when used to diagnose phyllodes tumors [20-22]. Thus, if a solid mass has a
benign core biopsy but subsequently grows rapidly or becomes symptomatic, an excisional
biopsy is also indicated.

Pathology

Gross — Grossly, phyllodes tumors may be indistinguishable from fibroadenomas. They are

round to oval multinodular masses with a grayish-white appearance that resemble the head of a
cauliflower ( picture 1) [2]. Phyllodes tumors grow radially, creating a pseudocapsule through
which tongues of stroma may protrude and grow into adjacent breast tissue [23]. Necrosis and
hemorrhage can occur in larger tumors [2].

Microscopic — Microscopically, the range of appearances covers the spectrum from

resembling a benign fibroadenoma to a high-grade sarcoma. The characteristic leaf-like
architecture consists of elongated cleft-like spaces that contain papillary projections of
epithelial-lined stroma with varying degrees of hyperplasia and atypia ( figure 1) [3]. The
stromal elements are a key component in differentiating phyllodes tumors from fibroadenomas
and in differentiating a benign tumor from a malignant one [23]. (See 'Histologic' below.)

Histologic — Histologically, phyllodes tumors are classified as benign, borderline, or

malignant based upon the assessment of four features [2,4,6,7,24-27]:

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● The degree of stromal cellular atypia

● Mitotic activity
● Infiltrative or circumscribed tumor margins
● Presence or absence of stromal overgrowth (ie, presence of pure stroma devoid of
epithelium)

Benign tumors are characterized by increased stromal cellularity with mild-to-moderate cellular
atypia, circumscribed tumor margins and low mitotic rate (less than 4 mitoses per 10 high-
power fields), and lack of stromal overgrowth.

Borderline tumors have a greater degree of stromal cellularity and atypia, a mitotic rate of 4 to
9 mitoses per 10 high-power fields, microscopic infiltrative borders, and lack of stromal
overgrowth.

Malignant tumors are characterized by marked stromal cellularity and atypia, infiltrative
margins, high mitotic rate (more than 10 mitoses per 10 high-power fields), and the presence of
stromal overgrowth [4,7,26-28].

Of the four histologic features listed above, stromal overgrowth is most consistently associated
with aggressive (metastatic) behavior [1,29]. Most clinically malignant/metastatic phyllodes
tumors have had overgrowth of one or several sarcomatous elements. These elements include
liposarcoma, rhabdosarcoma, chondrosarcoma, and undifferentiated/unclassified sarcoma
[30,31].

Undifferentiated/unclassified sarcoma was previously included within the terms of "malignant

fibrous histiocytoma (MFH)" and "undifferentiated pleomorphic sarcoma (UPS)" but is now a
separate soft tissue sarcoma category with an undifferentiated pleomorphic variant [32]. Close
examination of the stroma is imperative as the sarcomatous component may be present in only
a small portion of the tumor [33]. (See "Clinical presentation, histopathology, diagnostic
evaluation, and staging of soft tissue sarcoma", section on 'Histopathology'.)

Although the exact figures vary, more than 50 percent of phyllodes tumors are classified as
benign and 25 percent as malignant in most large studies [1,2,34,35].

Histologic grades of phyllodes tumors have clinical implications. Patients with benign tumors
generally have better local control and disease-free survival compared with those with
borderline or malignant tumors [36]. Benign and borderline phyllodes tumors rarely recur
following wide excision [37].

Clinical behaviors of phyllodes tumors can also be influenced by factors other than histologic
grades [1,8]. In a study of 605 cases of phyllodes tumors, stromal atypia, mitoses, overgrowth,
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and surgical margin status (defined as complete, focally involved, and diffusely involved) were
shown to be independent predictors of recurrence, with surgical margin status being the most
important. A nomogram was developed by the use of a mathematical formula that could be
applied to an individual patient to guide clinical decision making [38].

Molecular — In phyllodes tumors, the status of tumor markers, such as p53, Ki-67,
epidermal growth factor receptors, c-kit, and platelet-derived growth factor, has failed to predict
outcomes [33]. The expression of estrogen and progesterone receptors is common in the
epithelial but not the stromal component of phyllodes tumors [11].

On the molecular level, genetic heterogeneity has been observed intratumorally, with increasing
numbers of aberrations coinciding with increased malignant potential [39]. Some believe that
phyllodes tumors arise from benign epithelial fibroadenomas due to their close histologic
resemblance and molecular similarities [2,3,40]. As an example, molecular analysis showed high
frequencies of MED12 (mediator complex subunit 12) mutations in both fibroadenomas and
phyllodes tumors [41]. In one study of 112 breast phyllodes tumors, MED12 mutations were
detected in 65, 66, and 43 percent of benign, borderline, and malignant phyllodes tumors,
respectively [42]. In another study using targeted next-generation sequencing, malignant
phyllodes tumors were shown to harbor additional mutations in key tumor suppressor genes
and oncogenes, such as TP53, RB1, NF1, and CAN [43]. Although controversial, some thought
that the acquisition of additional mutations explains the aggressive biological behaviors of
malignant phyllodes tumors.

TREATMENT

Phyllodes tumors should be completely excised; axillary lymph node dissection is not necessary.
Adjuvant radiation therapy may benefit borderline or malignant, but not benign, tumors.
Chemotherapy is reserved for highly selected patients with large, high-risk, or recurrent
malignant phyllodes tumors. Hormonal therapy is not used to treat phyllodes tumors. Given the
rarity of the disease, treatment principles are based mainly on retrospective series and case
reports.

Surgical resection

Breast surgery — A complete surgical excision is the standard of care for phyllodes tumors.
Phyllodes tumors should be completely excised because most [37,44-46], but not all [47],
studies have associated positive margins with unacceptably high local recurrence rates. In a
multivariate survival analysis that included 172 patients with phyllodes tumors, a positive

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surgical margin was associated with an almost fourfold higher risk of a tumor-related event
such as local recurrence or distant disease (hazard ratio [HR] 3.9, 95% CI 1.1-14.3) [45].

Positive margins often occur when phyllodes tumors are misdiagnosed as fibroadenomas and
either enucleated or locally excised without attention to margins [4,5,12,13,24,36,37,46,48-51]. A
positive margin requires re-excision.

The minimal acceptable margin beyond "clear," however, is controversial and also depends on
tumor grade [12,44,45,52]. While we aim for a 1 cm margin for phyllodes tumors, we will accept
a narrower but clear margin for benign, but not borderline or malignant, phyllodes tumors:

● Emerging evidence suggests that a positive surgical margin of benign phyllodes tumors
may not be related to local recurrence [53]. A 2019 meta-analysis of 54 observational
studies also found that a positive margin only correlated with a higher local recurrence risk
for malignant, but not for benign and borderline, phyllodes tumors [28]. Consequently, it
has become controversial whether a negative margin should be strictly obtained for a
benign phyllodes tumor [54,55]. Regardless of the ongoing debate, however, it appears safe
to accept a clear, but narrower than 1 cm, margin rather than mandating re-excision for
benign phyllodes tumors.

● Surgical margins of ≥1 cm have been associated with a lower local recurrence rate in
borderline and malignant phyllodes [1,4,49]. In a retrospective review of 48 women with
high-grade malignant phyllodes tumors, 10 patients were treated with local excision
(margins <1 cm), 14 with wide local excision (margins ≥1 cm), and 24 with mastectomy [46].
At a median follow-up of nine years, the local recurrence rate was higher after local excision
with narrow margins than after wide local excision (60 versus 28 percent). Local recurrence
and cancer-specific survival were related to tumor size and excision margins. The average
tumor size was 7.8 cm in this study.

As long as adequate margins can be achieved, breast-conserving surgery and mastectomy are
equally effective in treating malignant phyllodes tumors [1,4,12,56]. In a study of 821 women
with malignant phyllodes tumors from the Surveillance, Epidemiology, and End Results (SEER)
database, mastectomy and wide local excision were performed in 52 and 48 percent,
respectively [12]. Compared with mastectomy, wide local excision was associated with
equivalent or improved cause-specific survival regardless of tumor size.

Mastectomy is generally not indicated for benign phyllodes unless negative margins cannot be
achieved and/or if the tumor is so large that breast-conserving surgery would result in
suboptimal cosmetic outcomes.

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Despite best surgical efforts, phyllodes tumors are known to recur locally with rates that vary
with tumor grade. In earlier reports, benign, borderline, and malignant phyllodes tumors had
local recurrence rates of 8, 21, and 36 percent, respectively [37,49]. More contemporary series
reported lower rates of local recurrence [57]. As an example, a 2019 meta-analysis of 54
retrospective studies reported an overall local recurrence rate of 12 percent (95% CI 10-14), and
pooled local recurrence rates of 8, 13, and 18 percent for benign, borderline, and malignant
tumors, respectively [28]. Recurrent phyllodes tumors require surgical and/or radiation therapy
as discussed below. (See 'Local recurrence' below.)

Axillary surgery — Axillary lymph node involvement by phyllodes tumors is rarely reported,

even when tumors are malignant [5,7,12,50,58]. In the SEER database study cited above, only 8
of 498 women with known lymph node status had involved nodes [12]. Thus, axillary surgery is
rarely indicated in patients diagnosed with phyllodes tumors.

Radiation therapy — When adequate surgical margins cannot be achieved because of tumor

location, adjuvant radiation therapy (RT) should be administered, even after mastectomy.
However, when adequate surgical margins can be achieved, there is less agreement about the
need for adjuvant RT. We base our decision about adjuvant RT on tumor grade:

● We do not suggest adjuvant RT for patients with benign phyllodes tumors that are widely
excised.

● We suggest adjuvant RT for patients with borderline or malignant phyllodes tumors

following surgical excision.

Adjuvant RT has been shown to reduce local recurrence of borderline or malignant phyllodes
tumors [26,27,35,36,49,58,59]. In a meta-analysis of eight observational studies, adjuvant RT
clearly reduced local recurrences of borderline or malignant phyllodes tumors after breast-
conserving surgery (HR 0.31, 95% CI -0.10 to 0.72) but had no effect on overall or disease-free
survival [60]. The effect of adjuvant RT on local recurrences after mastectomy was less
pronounced (HR 0.68, 95% CI -0.28 to 1.64).

In clinical practice, the utilization of adjuvant RT for phyllodes tumors appears to be modest. In
a retrospective review of the National Cancer Database that included 3120 patients with
malignant phyllodes tumors, only 14 percent received adjuvant RT [61]. Patients were more
likely to receive radiation therapy if they were diagnosed later in the study, were 50 to 59 years
old, had tumors >10 cm, or had lymph nodes removed. In adjusted models, adjuvant radiation
reduced local recurrence but did not impact survival after a median follow-up of 53 months.

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Chemotherapy — Based on limited data, the role of systemic chemotherapy in phyllodes

tumors is limited. Patients with benign or borderline phyllodes tumors are usually cured with
surgery and should not be offered chemotherapy unless they develop unresectable metastases.

The role of adjuvant chemotherapy in malignant phyllodes tumor is controversial. Malignant

phyllodes tumors are pathologically and clinically most similar to soft tissue sarcomas; however,
localized malignant phyllodes tumors have a better prognosis than most high-grade sarcomas
of similar stage. Thus, systemic chemotherapy should be considered for malignant phyllodes
tumors with even more caution.

There have been no randomized studies of adjuvant chemotherapy specifically for phyllodes
tumors. In a small observational study, 28 patients with malignant phyllodes tumors were
treated with either adjuvant doxorubicin plus dacarbazine or observation alone after surgical
resection based upon patient preference [62]. Although the five-year relapse-free survival was
not different between the two groups, the study was retrospective, nonrandomized, and
underpowered. It did not utilize ifosfamide in combination with doxorubicin (which is superior
to dacarbazine plus doxorubicin in other soft tissue sarcomas).

Based on experience and limited data, we recommend adjuvant chemotherapy only to a small
minority of patients with high-risk (>10 cm) or recurrent malignant phyllodes tumors who have
excellent functional status and minimal comorbidities, and only after a thorough discussion
about the risks, benefits, and controversial nature of such treatment. When systemic
chemotherapy is indicated, malignant phyllodes tumors should be treated according to
protocols designed for soft tissue sarcoma, rather than breast cancers. (See "Systemic
treatment of metastatic soft tissue sarcoma".)

For patients with metastatic disease, systemic treatments used are the same as those used for
soft tissue sarcoma but often have limited or short-lived benefit. Given the paucity of efficacious
agents in the metastatic setting, patients should be referred for investigational treatments on
clinical trials.

Hormone therapy — Hormone therapy is not effective against phyllodes tumors [11,63].

Despite the presence of hormone receptors in the epithelial component of some phyllodes
tumors, the stromal component is the principle neoplastic cell population responsible for the
metastatic behavior. The stromal component primarily expresses estrogen receptor beta
instead of estrogen receptor alpha, which is expressed by breast cancer [64,65].

POST-TREATMENT SURVEILLANCE

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Given the small number of patients with malignant phyllodes tumors of the breast, there are no
evidence-based recommendations for surveillance after therapy [66,67]. The following follow-up
guidelines are adapted from those for soft tissue sarcoma (see "Overview of multimodality
treatment for primary soft tissue sarcoma of the extremities and chest wall", section on
'Posttreatment cancer surveillance'):

● Since most recurrences occur in the first two years after treatment [49], we perform a
history and physical examination every six months for the first two years, then annually.
(See 'Local recurrence' below.)

● Patients who have not had mastectomy should resume surveillance with mammography
annually. If suspicious lesions are found on mammography or breast examination, further
imaging and/or biopsy may be required. (See 'Imaging' above.)

● Patients with large (≥5 cm) or malignant phyllodes tumors are at higher risk of developing
metastatic disease. For such patients, surveillance may be performed more frequently and
with chest radiograph or chest computed tomography (CT) as recommended by the
National Comprehensive Cancer Network (NCCN) on soft tissue sarcomas [68]. (See
'Metastatic disease' below.)

RECURRENT AND METASTATIC DISEASES

Local recurrence — When phyllodes tumors recur, they typically recur locally within two years
of the initial excision [4,11]. Some series have found that the time to local recurrence was
shorter for malignant than for benign or borderline tumors [4,33,37].

Although recurrences typically have the same grade as the original tumors, there have been
several case reports of benign tumors transforming into malignant ones upon recurrence
[1,8,35,69]. In one series of 293 phyllodes tumors, for example, six benign tumors recurred
locally as malignant tumors [8]. A systematic review of 54 retrospective studies reported some
26 percent (range 13 to 38) of benign and 21 percent (8 to 33 percent) of borderline tumors that
recurred underwent upgrade [28].

Recurrent phyllodes tumors are treated with surgery and/or radiation, with the goal of avoiding
re-recurrence and the need for additional surgical intervention. Resectable recurrent disease is
treated with either re-excision with wide margins or mastectomy, followed by radiation therapy
(RT). Unresectable recurrences are treated with palliative radiation alone [13]. (See 'Surgical
resection' above and 'Radiation therapy' above.)

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Metastatic disease — Metastatic disease has been reported in 13 to 40 percent of patients with

phyllodes tumors [1,2,4,18]. The mean overall survival is 30 months in patients who develop
metastatic diseases [70].

Phyllodes tumors metastasize most often to the lungs. Tumors that metastasize are typically
large (≥5 cm) or have malignant histologic features (see 'Histologic' above):

● In a series of 101 patients, eight developed distant metastases, seven of whom had
malignant tumors and one benign [1]. All tumors had stromal overgrowth, and six were ≥5
cm in size.

● In another retrospective review of 293 phyllodes tumors treated between 1954 and 2005,
five patients developed distant disease. All tumors that metastasized had one or more
malignant histologic features such as infiltrative borders, marked stromal overgrowth,
marked stromal cellularity, high mitotic count, and necrosis and were ≥7 cm in size [8].

As with other soft tissue sarcomas, pulmonary metastases of phyllodes tumors should be
resected when technically feasible. (See "Surgical treatment and other localized therapy for
metastatic soft tissue sarcoma".)

For patients with metastatic disease, chemotherapy may be administered based upon
treatment guidelines for soft tissue sarcomas [4,71]. The choices of chemotherapeutic agents
for this purpose are discussed elsewhere. (See "Systemic treatment of metastatic soft tissue
sarcoma".)

SURVIVAL

The majority of patients with benign and borderline phyllodes tumors are cured by surgery. The
survival rate for malignant phyllodes tumors is approximately 60 to 80 percent at five years
[1,12,48].

● The impact of histology on survival was explored in the SArcoma and PHYllode
Retrospective (SAPHYR) Study, a retrospective study of 70 patients with primary breast
sarcomas and phyllodes tumors treated from 1966 to 2004 [48]. The overall three-year
survival rate for combined benign and borderline tumors was 100 percent. The overall
three-year survival rate for malignant phyllodes tumors was 54 percent, similar to that of
nonangiosarcoma primary breast sarcomas (60 percent).

● Similarly, in a retrospective study of 101 patients treated between 1944 and 1998, the five-
year overall survival rate for patients with benign/borderline and malignant tumors was 91

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and 82 percent, respectively [1].

SOCIETY GUIDELINE LINKS

Links to society and government-sponsored guidelines from selected countries and regions
around the world are provided separately. (See "Society guideline links: Evaluation of breast
problems".)

SUMMARY AND RECOMMENDATIONS

● Phyllodes tumors are uncommon fibroepithelial breast tumors that are capable of a diverse
range of biologic behaviors. Most phyllodes tumors present as smooth, multinodular,
painless breast lumps. While they can be confused for fibroadenomas, phyllodes tumors
typically have larger sizes and grow rapidly. Twenty percent of phyllodes tumors present as
abnormal findings on screening mammography. (See 'Introduction' above and 'Clinical
presentation' above.)

● The typical appearance of a phyllodes tumor on mammography is a smooth, polylobulated

mass. On ultrasound examination, these lesions are solid, hypoechoic, and well
circumscribed. The presence of cystic areas within the mass may increase the level of
suspicion for phyllodes tumors. (See 'Imaging' above.)

● Breast masses suspicious for phyllodes tumors should undergo core biopsy; fine needle
aspiration is less accurate. If the core biopsy results are indeterminate or if there is clinical-
pathologic discordance, an excisional biopsy should be performed. (See 'Biopsy' above.)

● For phyllodes tumors of the breast, we recommend wide excision with a margin of at least 1
cm, rather than excision with narrower margins or enucleation alone (Grade 1B). Positive
margins require re-excision. We will accept a narrower but clear margin for benign, but not
borderline or malignant, tumors. As long as adequate margins can be achieved, phyllodes
tumors can often be treated with breast-conserving surgery (partial mastectomy).
Mastectomy is only required if adequate margins cannot be achieved with breast-
conserving surgery.

● We recommend not performing axillary dissection for phyllodes tumors (Grade 1C). Axillary
lymph node involvement is rare, even with malignant tumors. (See 'Surgical resection'
above.)

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● For benign phyllodes tumors that are completely excised, we recommend not performing
adjuvant radiotherapy (Grade 1C). For borderline or malignant phyllodes tumors, we
suggest performing adjuvant radiotherapy, even after a complete excision (Grade 2C).
Radiation therapy substantially reduces the local recurrence rates of these tumors. (See
'Radiation therapy' above.)

● We suggest that adjuvant chemotherapy be reserved for highly selected patients with large,
high-risk, or recurrent malignant phyllodes tumors (Grade 2C). Chemotherapeutic agents
should be chosen based upon treatment guidelines for soft tissue sarcomas, rather than
breast cancer. (See 'Chemotherapy' above.)

● We recommend not using hormonal therapy for phyllodes tumors (Grade 1C). (See
'Treatment' above.)

● We perform a history and physical examination every six months for two years after the
initial treatment of phyllodes tumors, then annually. Patients at higher risk for developing
metastatic diseases (≥5 cm or malignant tumors) may require more frequent surveillance
with chest radiograph or chest computed tomography (CT) per guidelines for soft tissue
sarcoma. (See 'Post-treatment surveillance' above.)

● Locally recurrent phyllodes tumors that are resectable should undergo wide re-excision
followed by adjuvant radiotherapy. Unresectable recurrences are palliated with radiation
therapy. Resectable pulmonary metastasis should be resected; unresectable metastatic
phyllodes tumors are palliated with chemotherapy according to soft tissue sarcoma
protocols. (See 'Recurrent and metastatic diseases' above.)

● The majority of patients with benign and borderline phyllodes tumors are cured by surgery.
The survival rate for malignant phyllodes tumors is approximately 60 to 80 percent at five
years. (See 'Survival' above.)

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Topic 793 Version 23.0

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GRAPHICS

Cut surface of phyllodes tumor

Graphic 51479 Version 2.0

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Cellular stroma with leaf-like process

Graphic 78624 Version 1.0

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Contributor Disclosures
Ana M Grau, MD, FACS Nothing to disclose A Bapsi Chakravarthy, MD, FASTRO Nothing to
disclose Rashmi Chugh, MD Nothing to disclose Lori J Pierce, MD Nothing to disclose Daniel F Hayes,
MD Patent Holder: Immunicon Corporation [Inventor]; University of Michigan [Inventor]; University of
Michigan [Inventor]. Grant/Research/Clinical Trial Support: Menarini Silicon Biosystems, LLC [Breast
cancer]; Pfizer [Breast cancer]; AstraZeneca [Breast cancer]; Merrimack Pharmaceuticals, Inc [Breast
cancer]. Consultant/Advisory Boards: Cepheid [Breast cancer]; Freenome, Inc [Breast cancer]; Artiman
Ventures [Breast cancer]; CVS Caremark [Breast cancer expert panel]; Agendia, Inc [Breast cancer]; Lexent
Bio [Biomarkers]; EPIC Sciences, Inc [Liquid biopsies]; Salutogenic Innovations, LLC [Biomarkers]. Other
Financial Interest: Royalties from licensing of patent above to Menarini Silicon Biosystems [Breast cancer];
University of Michigan [Inventor]. Anees B Chagpar, MD, MSc, MA, MPH, MBA, FACS,
FRCS(C) Consultant/Advisory Boards: Protean BioDiagnostics [Breast cancer]. Wenliang Chen, MD,
PhD Nothing to disclose

Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must
conform to UpToDate standards of evidence.

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