Abe 

et al. surg case rep (2020) 6:308

https://doi.org/10.1186/s40792-020-00986-8

CASE REPORT Open Access

Malignant phyllodes tumor of the breast

with rapid progression: a case report
Hajime Abe1*  , Atsuko Teramoto1, Yumiko Takei1, Yoshihito Tanaka2 and Genichiro Yoneda3

Abstract 
Background:  Malignant phyllodes tumors (PTs) of the breast occur infrequently and are difficult to treat with adju-
vant therapy. Here, we present a case of a female patient with a huge malignant PT with rapid progression in a short
period.
Case presentation:  A 44-year-old woman presented to our hospital with a rapid growth mass in her right breast,
measuring 20 cm. She was initially diagnosed as having a borderline phyllodes tumor by core needle biopsy and
underwent total mastectomy and artificial dermis was grafted, 20 days later, latissimus dorsi muscle flap and free skin
grafting were performed. Two courses of doxorubicin–ifosfamide therapy were administered because of recurrence,
but the patient died 4 months after the mastectomy.
Conclusions:  A standard therapeutic strategy for malignant PTs is needed in urgently to reduce the risk of tumor
recurrence.
Keywords:  Malignant phyllodes tumor, Breast, Mastectomy, Chemotherapy, Doxorubicin–ifosfamide

Background the borderline and malignant subgroups. Malignant PTs

Phyllodes tumors (PTs) of the breast are extremely rare, are more readily characterized by stromal pleomorphism
globally accounting for 0.3% to 1% of breast tumors [1]. and overgrowth, frequent mitoses and infiltrative borders
Their name is derived from the Greek phyllon (leaf ) [8]. Lymph node metastasis is rare, and the metastatic
because of its lobed histological appearance. It is also path relies mainly on the blood. Surgical removal is the
known as cystosarcoma phyllodes, adenomatous myx- primary treatment for PT, given that adjuvant treatments
oma, and pseudosarcoma adenoma [1–3]. It usually play a poorly efficient role in this malignancy. Here, we
occurs in middle-aged women (age, 35–55  years) [4]. report the case of a female patient with a huge malignant
Clinically, the size of the tumor varies between 4 and PT with rapid progression in a short period.
7 cm on average [5], but about one-fifth of PTs are called
giant PT tumors because of their uncommon diameter of Case presentation
more than 10  cm [6]. Depending on the histopathologi- A 44-year-old woman presented to our hospital with a
cal features, PTs are categorized into three grades with rapid growth mass in her right breast, measuring 20 cm.
different proportions, benign (60%–75%), borderline The breast skin appeared dark with ulceration (Fig.  1).
(13%–26%), and malignant (10%–20%) [7]. Patients suf- Her family history and past history were unremarkable.
fering from PTs have no specific clinical manifestations, One year prior to admission, she incidentally palpated
and it is difficult to distinguish the benign subgroup from the tumor, and noted gradual growth 2 months prior to
the hospital visit. Computed tomography and magnetic
resonance imaging revealed a hemorrhagic giant mass
*Correspondence: abe@belle.shiga‑med.ac.jp with a well-defined border. The axillary and supraclav-
1
Breast Center, Bell Land General Hospital, 500‑3 Higashiyama, Naka‑ku, icular lymph nodes were swollen, but there was no sign
Sakai, Osaka 599‑8247, Japan of chest wall invasion or distant metastasis (Fig.  2). A
Full list of author information is available at the end of the article

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Abe et al. surg case rep (2020) 6:308 Page 2 of 5

histopathological examination demonstrated that the

tumor was composed of atypical spindle-shaped cells
with enlarged nuclei and exhibited a fibrosarcoma-
like morphology which included ill-defined invasion
into the adjacent breast tissue, the overlying skin, and
the pectoral muscle (Fig.  4a). Numerous mitoses were
noted (10/10 high-power fields) and necrosis had
occurred (Fig.  4b). A degenerated leaf-like structure
was observed in the center of the lesion (Fig. 4c). Three
lymph node metastases with a maximum diameter of
25  mm. were noted. Based on these findings, a final
diagnosis of malignant PT was established.
Because 1 month after the mastectomy the tumor had
re-grown in the surrounding skin graft and right pleural
effusion had appeared (Fig.  5), as an alternative treat-
ment, we administered two courses of doxorubicin–
ifosfamide (AI) therapy (30  mg/m2 doxorubicin on
days 1–2 and 2 g/m2 ifosfamide on days 1–5) including
Fig. 1  Huge phyllodes tumor with ulceration in the right breast
Mesna (sodium 2-mercaptoethane sulfonate) and suf-
ficient infusion volumes to prevent ifosfamide-related
hemorrhagic cystitis. Grade 4 neutropenia and anemia
(as defined by the Common Terminology Criteria for
diagnosis of PT of borderline malignancy was estab- Adverse Events) occurred during AI therapy, thus, to
lished on the basis of the core needle biopsy findings. prevent the advancement of neutropenia, filgrastim, a
Subsequently, the patient underwent mastectomy of granulocyte-colony-stimulating factor, was adminis-
the right breast and axillary lymph node dissection. tered, and transfusion was performed. The local tumor
During surgery, the surgeons detected invasion of the was temporarily reduced after one cycle of AI therapy
pectoralis major; thus, the partial muscle adhering to (Fig.  6); however, after two cycles of AI therapy, the
the tumor was resected. Because the skin defect was chest wall recurrence and pleural dissemination pro-
large, artificial dermis [9] was grafted to remedy for gressed rapidly (Fig. 7). The patient died 4 months after
the defect (Fig.  3). Twenty days later, latissimus dorsi the mastectomy because of respiratory failure.
muscle flap and free skin grafting were performed. The

Fig. 2  Computed tomography showed a 20 cm heterogeneous mass in the right breast and axillary and supraclavicular lymph nodes swelling
(arrow)
 Abe et al. surg case rep (2020) 6:308 Page 3 of 5

Fig. 3  Right mastectomy with axillary lymph node dissection

covered with artificial skin

Discussion
Malignant PT is rare lesion of the breast that can mimic
benign masses such as fibroadenomas, on clinical diag-
nosis, but is characterized by a typical rapid growth. PTs
usually occur in middle-aged women ranging in age from
35 to 55  years, with an average presentation at 45  years
[4]. PTs are composed of epithelial elements and a con-
nective tissue stroma with higher stromal cellularity. A
malignant PT is distinguished from a benign/borderline
PT by the presence of marked stromal cellularity, cellular
atypia and mitotic activity in at least 10/10 high-power
fields [10].
The clinical presentation and the radiographic findings
of malignant PT are strikingly similar to those of benign
lesions, such as fibroadenoma, or even benign PT, thus,
making it quite challenging for clinicians to diagnose or
even to suspect the disease at an early stage. Although
routine breast biopsy may not be warranted, it is crucial
for clinicians to consider and include PT in their differen-
tial diagnosis. Moreover, it is also evident that clinicians
cannot rely completely on radiographic findings.
According to the National Comprehensive Cancer
Network (NCCN) guidelines for breast cancer, the man-
agement of PTs with a size > 3.0  cm is surgical excision
with clean margins (≥ 1.0  cm) without axillary staging,
regardless of whether the tumor is benign, borderline, or
Fig. 4  The histopathological findings revealed atypical
malignant [11]. Many other studies have supported the spindle-shaped cells with enlarged nuclei and exhibited a
contention that margins that are ≤ 1.0 cm are associated fibrosarcoma-like morphology (a) (HE stain, × 2). Numerous mitoses
with a higher recurrence rate, ranging from 16.7 to 40% were noted (b) (HE stain, × 40). A degenerated leaf-like structure was
[4, 12]. found in the center of the lesion (c) (HE stain, × 10)
The prognosis of PTs is variable, with local recur-
rence rates ranging from 10 to 40% (average 15%) and
distant metastases occurring in 10% of all PTs and up to survival ranging from 4 to 17  months, with large vari-
20% of malignant PTs [13]. Survival after metastatic dis- ability based on the site of the metastatic disease [14].
ease is poor, with various case series reporting a median Other large prospective studies have reported 5-year
Abe et al. surg case rep (2020) 6:308 Page 4 of 5

disease-free survival rates of 96% for benign PTs and

66% for malignant PTs [15]. Most sarcomas metastasize
hematogenously, and the incidence of axillary lymph
node involvement in malignant PTs ranges from 1.1 to
3.8% [16].
There is currently no consensus regarding the recom-
mendations for radiotherapy, hormonal therapy, and
systemic chemotherapy for malignant PTs. To date, no
double-blinded, multicenter study has been performed
on this subject. Most case reports and studies describe
treating these tumors exclusively with wide local exci-
sion, in according with the current NCCN guidelines.
As PTs are considered as soft-tissue sarcoma, adjuvant
chemotherapy with doxorubicin plus dacarbazine may
provide some benefits to patients with large (> 5.0  cm),
Fig. 5  Computed tomography revealed regrowth of tumor in the high-risk tumors [17]. A deeper investigation of the addi-
right chest, right lung and right pleural effusion tion of adjuvant therapy for large aggressive malignant
cases of PT may prove to be fruitful [18]. Recently, doxo-
rubicin and ifosfamide therapy has been reported to be
effective to treat metastases of malignant PTs [19, 20]. In
accordance with previous reports [19, 20], we adminis-
tered AI therapy using 60 mg/m2 doxorubicin and 10 g/
m2 ifosfamide in each course.
Because there are not many reports of malignant cases
of PT, the data available are insufficient to calculate fully
the statistics of the survival rate associated with these
tumors. Past case reports and studies have suggested that
the prognosis of malignant PT of the breast is usually
poor, while the overall prognosis of benign PT is good
[14, 21].
The early diagnosis and staging of PTs are pivotal not
only for improving the overall outcome of the disease
Fig. 6  Computed tomography showed a mild reduction in chest wall after treatment, but also to promote the quality of life
tumor and pleural dissemination after one cycle of AI therapy of the patient by causing less disfiguration. While the
breast cancer screening guideline suggests that women
over 40 years of age should begin routine mammograms
to detect the presence of breast cancers, PTs can occur a
decade before this minimum screening age as they occur
during the third or fourth decades of life. Moreover, if the
patient suspects that the lesion exhibits growth within
6 months to a year of initial detection, it should be con-
sidered for further workup.

Conclusions
In conclusion, malignant PTs are rare entities with dis-
tinct clinicopathological features. These tumors should
be accurately recognized and effectively treated at first
diagnosis, as they have a high risk of recurrence. There
is no established consensus regarding the optimal type of
surgery and indications for radiotherapy and chemother-
apy regimens in these cases. The establishment of stand-
Fig. 7  Computed tomography showed a rapid increase in right chest ard therapeutic strategy for malignant PTs is needed
wall tumors and pleural dissemination
urgently to reduce the risk of tumor recurrence.
 Abe et al. surg case rep (2020) 6:308 Page 5 of 5

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