Phyllodes Tumors of The Breast: Natural History, Diagnosis, and Treatment

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Original Article

Phyllodes Tumors of the Breast: Natural


History, Diagnosis, and Treatment
Melinda L.Telli, MD;a Kathleen C. Horst, MD;b Alice E. Guardino, MD, PhD;a Frederick M. Dirbas, MD;c and
Robert W. Carlson, MD,a Stanford, California

Key Words unusual fibroepithelial lesions that account for less than
Phyllodes tumors, breast neoplasms, natural history, diagnosis, treat- 1% of all breast neoplasms.5,6 These tumors exhibit a wide
ment range of clinical behavior and represent a spectrum of
fibroepithelial neoplasms rather than a single disease
Abstract entity.7
Phyllodes tumors of the breast are unusual fibroepithelial tumors
that exhibit a wide range of clinical behavior. These tumors are cat-
egorized as benign, borderline, or malignant based on a combina-
tion of histologic features. The prognosis of phyllodes tumors is Pathologic Features
favorable, with local recurrence occurring in approximately 15% of Phyllodes tumors are characterized by the presence
patients overall and distant recurrence in approximately 5% to 10% of both epithelial and stromal elements, but the distin-
overall. Wide excision with a greater than 1 cm margin is definitive
primary therapy. Adjuvant systemic therapy is of no proven value.
guishing histologic features relate to the stroma.8
Patients with locally recurrent disease should undergo wide exci- Microscopically, these tumors contain elongated ductal
sion of the recurrence with or without subsequent radiotherapy. elements and papillary protrusions of connective tissue
(JNCCN 2007;5:324–330) lined by epithelium that produce the leaf-like appear-
ance. Marked stromal overgrowth and hypercellularity
are important features that distinguish these lesions from
Historical Overview the more common fibroadenomas. These lesions are cat-
In 1838, Johannes Müller gave a detailed description of egorized as benign, borderline, or malignant based on a
a large mammary tumor with a cystic appearance and combination of histologic features, including stromal cel-
leaf-like growth pattern which he named cystosarcoma lular atypia, mitotic activity, stromal overgrowth, type of
phyllodes.1 In 1951, Treves and Sutherland2 suggested that tumor margin (circumscribed vs. infiltrative), and tumor
benign, pre-malignant, and malignant forms of this dis- necrosis.9–11 Reported incidences of each subtype have
ease existed. In 1960, Lomonaco3 proposed the name tu- varied widely in the literature. On average, more than
mor phyllodes, thereby avoiding any implication of biologic 50% are categorized as benign and approximately 25% as
behavior. The World Heath Organization adopted the malignant in most large series.7,12 Despite these classifica-
terminology phyllodes tumor in 1981, and this name has tions, histologic grade has been found to correlate poorly
since gained widespread acceptance.4 Phyllodes tumors are with biologic behavior.13
The expression of estrogen and progesterone recep-
tors is common in the epithelial component but uncom-
From the Departments of aMedicine, bRadiation Oncology, and
mon in the stromal component of phyllodes tumors. In
c
Surgery, Stanford University, Stanford, California. one series, epithelial estrogen receptor expression was
Submitted November 2, 2006; accepted for publication November
20, 2006.
seen in 58% and progesterone receptor expression in
The authors have no financial interest, arrangement, or affiliation 75%.14 In this series, expression of hormone receptors was
with the manufacturers of any products discussed in the article or
their competitors.
related to histologic grade, with estrogen and proges-
Correspondence: Robert W. Carlson, MD, 875 Blake Wilbur Drive, terone receptor expression present in 67% and 77% of be-
Stanford, CA 94305. E-mail: rcarlson@stanford.edu
nign, 43% and 84% of borderline, and 47% and 47% of

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Phyllodes Tumors of the Breast

Table 1 Hormone Receptor Expression in Phyllodes Tumors


Estrogen Receptor Progesterone Receptor Androgen Receptor
Phyllodes Tumor Subgroup Epithelial Stromal Epithelial Stromal Epithelial Stromal
Benign 67% 3% 77% 1% 1% 1%
Borderline 43% 0% 84% 0% 3% 0%
Malignant 47% 5% 47% 5% 0% 5%

Adapted from Tse GM, Lee CS, Kung FY, et al. Hormonal receptors expression in epithelial cells of mammary phyllodes tumors
correlates with pathologic grade of the tumor: a multicenter study of 143 cases. Am J Clin Pathol 2002;118:522–526; used
with permission. ©2002 American Society for Clinical Pathology.

malignant phyllodes tumors, respectively (Table 1). Phyllodes tumors occur at a median age of 45 years,7
Expression of androgen receptors was low in all tu- but cases in adolescents and the elderly have been well
mors. described.11,13,20,21,25 Recent Surveillance, Epidemiology,
The frequency of expression and role of the tu- and End Result (SEER) data indicate that the mean age
mor suppressor gene p53 in phyllodes tumors is highly at diagnosis is 50 years among patients with malignant
variable across series. In some but not all series, p53 phyllodes tumors.26 Bilateral phyllodes tumors are re-
expression increases across the spectrum of benign to ported to occur rarely,21,23,24 and a case of a phyllodes
malignant phyllodes tumors.15,16 tumor arising in an accessory breast has been reported.21
Clonal analysis of phyllodes tumors documents Additionally, phyllodes tumors associated with gyneco-
that the epithelial component is polyclonal, whereas mastia have been reported in men,27 and a bilateral
the stromal component is monoclonal, showing that phyllodes tumor arising in ectopic axillary breast tis-
the stroma represents the neoplastic component of sue has been described in a man.28
phyllodes tumors.17,18

Diagnosis
Clinical Presentation Unfortunately, triple assessment using clinical, radi-
The most common presentation of a phyllodes tumor ologic, and histologic examination results in low di-
is a palpable breast mass that may be rapidly growing. agnostic accuracy in phyllodes tumors.8,29 The
These tumors may exhibit biphasic growth and pa- deficiencies of this approach relate to the fact that
tients may present to medical attention only after phyllodes tumors have overlapping characteristics with
noting rapid growth in a lesion that had been stable benign breast disease in all 3 categories. Because the
for years.19 Rarely, patients present with a mass de- surgical management of these lesions differs from that
tected on routine screening mammography.13 Phyllodes of a fibroadenoma, accurate preoperative diagnosis
tumors can reach enormous proportions; tumors greater is optimal. Often, however, local excision of the mass
than 20 cm have been reported in multiple series.5,13,19–22 is required for diagnosis, and patients then require fur-
Many series have failed to show a correlation between ther surgery to ensure adequate tumor-free margins.
tumor size and histologic subtype, because even benign Clinically, the diagnosis of a phyllodes tumor
phyllodes tumors can reach great size.19,20 should be considered in the differential diagnosis of a
On examination, most patients have a smooth, large fibroadenoma. A history of rapid growth and at-
round, well-defined, firm mass that is mobile and pain- tainment of large size can be suggestive, but these fea-
less. Large lesions may be associated with dilated veins tures cannot reliably differentiate phyllodes tumors
visible over the skin, which may be stretched and at- from other benign breast disease. On mammogram,
tenuated. Nipple retraction,19,23 skin ulceration,5,19,21 phyllodes tumors appear as well-defined oval, round,
invasion of the chest wall,5,19,21 and bloody nipple dis- or lobulated masses that mimic the radiographic ap-
charge21 have been reported but are rare. Palpable pearance of a fibroadenoma.30 Associated coarse
axillary lymphadenopathy can be identified in up to microcalcifications are occasionally seen. On ultra-
20% of patients, but metastatic involvement of axil- sound, these lesions appear as solid, hypoechoic, well-
lary lymph nodes is rare.19,24 circumscribed masses.31 The presence of a cystic area

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Table 2 Paddington Clinicopathologic Table 3 Prognostic Factors Implicated in the


Suspicion Score Risk for Local and Distant Recurrence
Clinical Findings in Phyllodes Tumors
• Sudden increase in size in a long-standing breast lesion Implicated in Implicated in
Risk for Local Risk for Distant
• Apparent fibroadenoma > 3 cm in diameter or in a
Prognostic Factor Recurrence Recurrence
patient > 35 years
Tumor size No Yes
Imaging Findings
Histologic grade Unclear Yes
• Rounded borders/lobulated appearance at mammography
Positive margin Yes
• Attenuation of cystic areas within a solid mass on
ultrasonography Stromal overgrowth Yes
Fine Needle Aspiration Findings Prior local recurrence NA No

• Presence of hypercellular stromal fragments


• Indeterminate features
approximately 25% of phyllodes tumors are catego-
Any 2 features mandate core biopsy
rized as malignant and more than 50% are character-
Adopted from Jacklin RK, Ridgway PF, Ziprin P, et al. ized as benign.7,12 Benign lesions have the potential to
Optimising preoperative diagnosis in phyllodes tumour of recur locally and rarely at distant sites,19 and cases of
the breast. J Clin Pathol 2006;59:454-459; with permission benign tumors transforming into higher-grade lesions
from BMJ Publishing Group Ltd.
at recurrence have been reported.19,21,23,32 Despite hav-
ing an increased risk for distant spread, malignant le-
within a solid mass on ultrasound suggests the diagno- sions follow an indolent clinical course in most patients
sis of phyllodes tumor.30 with 15-year cause-specific survival rates of approxi-
Using fine needle aspiration (FNA) to diagnose mately 89%.26 Because of the unpredictable nature of
a phyllodes tumor is associated with an excessive rate these lesions, experts have suggested that all phyllodes
of false-negative results and an overall accuracy esti- tumors be regarded as having malignant potential.7
mated at 63%.8 Accuracy of FNA is often compro- Many studies have attempted to identify histo-
mised by inadequate sampling, because these tumors logic and clinical prognostic factors implicated in the
tend to have a very heterogeneous composition. Core risk for local and distant recurrence. This is difficult
biopsy, although subject to the same potential for sam- because of the rarity of this tumor, differences in patho-
pling error, is associated with higher accuracy than logic interpretation, and selection bias for the type of
FNA. In a recent article, Jacklin et al.8 concluded that treatment provided in various series. Despite these
core biopsy is potentially the most useful method of impediments, several important observations have
preoperatively diagnosing phyllodes tumor. To help been made (Table 3). The literature does not suggest
clinicians select patients for core needle biopsy, they a correlation between tumor size and increased risk
formulated a set of criteria that they refer to as the for local recurrence,11,13,21,29 although size has been im-
Paddington Clinicopathologic Suspicion Score (Table plicated in the risk for developing distant disease.29 A
2). The intraoperative diagnosis of phyllodes tumor us- positive surgical margin is the most powerful predic-
ing frozen section is often inaccurate, as is the case tor of local recurrence.13,22,29 Local recurrence does not
with intraoperative frozen section diagnosis of other correlate with an increased risk for distant disease21,29
breast masses. Chen et al.21 reported an accurate diag- and does not seem to affect survival.11 The literature
nosis using frozen section in only 41.6% of cases in is divided on the relationship between histologic grade
their series of 172 patients with phyllodes tumor. and risk for local recurrence. Some series suggest an
increase in local recurrence among borderline and ma-
lignant lesions,11 but this has not been found in other
Prognostic Factors large series.13,19–21,29 In contrast, the development of
The clinical course of phyllodes tumors can be unpre- metastatic disease has been shown to correlate with
dictable, although in most patients the prognosis is grade,19,20,29 and experts have estimated that 20% of
favorable. Poor correlation exists between histologic patients with malignant tumors will develop metasta-
features and biologic behavior. In most large series, tic disease.7 Stromal overgrowth has been identified as

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Phyllodes Tumors of the Breast

commonly used in the treatment of fibroadenoma, is


inadequate treatment for phyllodes tumors.11,20,33 In a
series of 101 patients, most of whom (99%) had patho-
logically negative margins after surgery, Chaney et al.20
reported a low actuarial 10-year rate of local recur-
rence (8%), suggesting that the risk for local recur-
rence can be significantly decreased but not eliminated
when negative margins are obtained.
Historically, mastectomy was the standard surgical
treatment for all phyllodes tumors.5 More recently,
breast-conservative approaches have been increasingly
Figure 1 Estimated cause-specific survival adjusted for age category used. In the series by Chaney et al.,20 approximately
(< 50, 50–59, 60–69, 艌70 years). Adapted from Macdonald OK,
Lee CM, Tward JD, et al. Malignant phyllodes tumor of the breast: half the patients were treated with breast conservation
association of primary therapy with cause-specific survival from the and half with mastectomy. Only 4 local recurrences
Surveillance, Epidemiology, and End Results (SEER) program. Cancer were seen, and these occurred equally in both groups.
2006;107:2127–2133; with permission from John Wiley & Sons, Inc.
Other large series have shown similar findings.19,25 A re-
cent analysis by Macdonald et al.26 used the SEER data-
an important independent histologic predictor of dis-
base to determine prognostic factors that predicted
tant recurrence.20
cause-specific survival among 821 patients with malig-
A multivariate analysis of 821 patients diagnosed
nant phyllodes tumor. In this retrospective analysis,
with malignant phyllodes tumors between 1983 and
wide excision was associated with equivalent or im-
2002 in the SEER program database assessed the
proved cause-specific survival relative to mastectomy
impact of age, race, year of diagnosis, size of primary
in a multivariate analysis. These data, similar to all ret-
tumor, and type of local therapy on cause-specific
rospective series, are potentially confounded by physi-
survival.26 Increased age was the only nontreatment
cian selection bias regarding the type of surgery
factor associated with decreased survival (Figure 1).
performed. However, given the overall low rate of lo-
Use of mastectomy plus external beam radiation ther-
cal failure with breast-conserving surgery, breast con-
apy was also associated with a poor prognosis, proba-
servation is the preferred primary therapy if negative
bly related to the advanced nature of tumors treated
margins can be obtained with an acceptable cosmetic
with both modalities.
result. In cases in which breast conservation is not pos-
sible because of large tumor size relative to breast vol-
Primary Surgical Management ume, total mastectomy is appropriate.13,19,20
When phyllodes tumor is diagnosed only after
Local recurrence occurs in approximately 15% of pa-
narrow margin excision, re-excision to obtain mar-
tients with phyllodes tumors and is more frequent af-
gins of greater than 1 cm is indicated.13,34 Simple mas-
ter inadequate excision.7 Distant failure occurs in
tectomy may be required to achieve this. The primary
approximately 5%–10% of cases overall and in ap-
mode of spread of phyllodes tumors is through
proximately 20% of malignant lesions.7, 12 Tumor biol-
hematogenous dissemination. Although axillary lym-
ogy seems to dictate metastatic potential, and this risk
phadenopathy is present in approximately 20% of
does not seem to be influenced by the choice of local
patients with phyllodes tumors, only approximately
treatment.33 Given these observations, the primary
5% of patients will have axillary metastases at lymph
surgical objective is to attain definitive local control.
node dissection.13 The current literature supports the
Wide local excision with margins of greater than
role of axillary lymph node staging only in cases where
1 cm is the preferred primary treatment.13,19–21,34
clinically pathologic nodes are found on examination.11,13
Microscopic projections of tumor often extend into
the pseudocapsule of normal compressed breast tissue
that surround these lesions.35 Therefore, achieving a
1-cm microscopic margin frequently requires remov- Role of Adjuvant Systemic Therapy
ing more tissue than would be expected based on gross The adjuvant use of chemotherapy or hormonal ther-
inspection. Simple enucleation of the tumor, as is apy has no proven role in treating phyllodes tumors.

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Chaney et al.20 proposed that adjuvant chemother- tients selected for radiotherapy were probably those
apy be considered for patients with histologic evi- with larger tumors with narrow tumor-free or involved
dence of stromal overgrowth, particularly when the margins.26
tumor size is greater than 5 cm, because these patients
seem to have the greatest risk for developing distant
disease. However, because systemic therapy has had Management of Locally
limited value in patients with metastatic phyllodes Recurrent Disease
tumors, it is not expected to be effective in the adju- In approximately 15% of cases of phyllodes tumor, the
vant setting, and no randomized data are available. Tse clinical course will be complicated by one or more lo-
et al.14 suggested that the potential role of endocrine cal recurrences.7 General consensus is that these recur-
therapy in treating phyllodes tumors is limited be- rences result from failure of primary surgical treatment,
cause hormone receptors are primarily expressed on although the biologic behavior of the tumor may be
the epithelial component of the tumors, their expres- an important determinant. de Roos et al.29 found that
sion is inversely related to the degree of malignancy, the development of one local recurrence seemed to
and the stromal component of phyllodes tumors is predispose to further episodes of local recurrence re-
the component implicated in metastatic spread. Thus, gardless of the histologic subtype or type of initial sur-
there is no role for measuring hormone receptors in gery. Most tumors recur within 2 years of initial
these tumors. surgery,19,25 although tumors recurring as soon as 1
month25 and as late as 17 years36 after initial surgery
have been reported. Some series have found that the
Role of Adjuvant Radiation Therapy time to local recurrence is shortest among patients
The role of adjuvant breast or chest wall radiotherapy with the malignant subtype compared with those with
is controversial. Most experience is based on case re- benign and borderline lesions.11 Generally, phyllodes
ports of patients with chest wall recurrences. Because tumors recur with the same histology as that of the
of the important relationship between excision mar- initial disease, but cases of transformation to more ag-
gin and the risk for local recurrence, some experts gressive tumors have been reported.20,21,23,25 In most pa-
have suggested that adjuvant radiation at doses of 50 tients, local recurrence is not associated with distant
to 60 Gy be considered in patients for whom negative metastases.21,29
surgical margins cannot be attained.13,20 The use of ad- Cases of successful treatment of patients with mul-
juvant radiation in patients with a resection margin tiple recurrent tumors have been reported.3 However,
of 1 cm or larger is not recommended.20,34 However, some authors believe that mastectomy should be con-
no high-level evidence supports the role of adjuvant sidered after the first recurrence of borderline or ma-
radiotherapy in phyllodes tumors.
lignant lesions,11 whereas others believe that
In a series of 37 patients with malignant phyllodes
mastectomy should be considered after any second re-
tumors, Pandey et al.22 administered 45 to 50 Gy of
currence.13 Chaney et al.20 suggest that adjuvant ra-
radiation to 69% of patients, including all patients
diotherapy may be appropriate after resection of
treated with wide excision alone and those experienc-
ing recurrent phyllodes tumors after resection of the recurrent disease. Mangi et al.13 reported a case of a pa-
recurrent disease. In this study, the 5-year disease-free tient with a high-grade tumor recurrence that could
survival rate was 61.2% among patients who under- not be fully excised surgically. This patient was treated
went adjuvant radiotherapy versus 51.4% among those with radiotherapy to the chest wall and remained free
who did not. Cox proportional hazard survival analy- of disease for 84 months after recurrence. The NCCN
sis, however, showed no statistically significant ad- Breast Cancer Clinical Practice Guidelines in
vantage to radiotherapy (P = .47). The recent analysis Oncology support re-excision with wide margins for
of SEER data found that the use of adjuvant radio- locally recurrent disease.34 Some members of the
therapy predicted a worse cause-specific survival com- NCCN guidelines panel recommend local radiation
pared with surgery alone among 821 patients with therapy to the remaining breast or chest wall after re-
malignant phyllodes tumors. In this series, however, section of a local recurrence, but this recommendation
only 9% of patients underwent radiotherapy, and pa- is controversial.

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Phyllodes Tumors of the Breast

Management of Metastatic Disease Summary


Based on data from large cases series, it is estimated that Phyllodes tumors of the breast are rare tumors that
approximately 5%–10% of patients with phyllodes present as rapidly growing breast masses and are often
tumors will develop distant disease, although the in- misdiagnosed as fibroadenomas. These tumors have
cidence of metastatic disease among patients with ma- both an epithelial and stromal component and may be
lignant tumors is estimated at approximately 20%.7,12 benign, borderline, or malignant in histology. They
Metastatic lesions histologically contain only stromal have a propensity for local recurrence, but systemic
elements and are devoid of epithelial elements.6 A metastasis occurs in only approximately 5% to 10% of
metastatic phyllodes tumor carries a poor prognosis, patients overall and approximately 20% of those with
with an average survival of less than 2 years.5 In a se- the malignant subtype. Long-term cause-specific sur-
ries of 170 patients, Reinfuss et al.19 found that among vival among patients with the malignant subtype is
patients with metastatic disease, 93% developed metas- approximately 90%. Local excision with a 1-cm or
tases within 3 years and the median survival was 4 larger tumor-free margin is definitive therapy after ini-
months. In this series, 3 patients initially diagnosed tial diagnosis. Local radiation therapy after breast-
with benign tumors developed metastatic disease, and conserving therapy or mastectomy may be considered
this phenomenon has been described in several other when adequate excision margins cannot be attained.
series.13,20,21 de Roos et al.29 reported a median survival Adjuvant systemic therapy has no proven value.
of 17 months, ranging from 12 to 145 months. The Patients experiencing local recurrences after ini-
most common sites of distant disease are the lung, tial therapy should undergo wide excision of the recur-
bone, and abdominal viscera,7 although metastases rence with or without subsequent local radiation
have been reported in the brain,19,20 heart,37 soft tis- therapy. Patients with metastatic disease should be
sues of the neck,21 thigh,6 pelvis,20 and oral cavity.38 managed, as appropriate, following treatment guide-
Clinical experience with chemotherapy for lines for patients with metastatic soft tissue sarcoma.
metastatic phyllodes tumors is limited. Multiple single-
agent and combination regimens have been used with
varying success. Responses, when they occur, are gen- References
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© Journal of the National Comprehensive Cancer Network | Volume 5 Number 3 | March 2007

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