Phyllodes Tumor in Ectopic Breast Tissue of the Vulva: A case report

PHYLLODES TUMOR IN ECTOPIC BREAST TISSUE OF
THE VULVA: A CASE REPORT
Ahmad Abu-Alsamen MD*, Ahlam Awamleh MD*, Mahmud Abdallat MD**
ABSTRACT
Phyllodes tumor is a rare fibroepithelial neoplasm of the breast. The majority of Phyllodes tumors occur in
women between the ages of 35-55 years. Phyllodes tumors arising in aberrant breast tissue in the vulva are a
very rare occurrence. We report a case of a 34 year-old, single woman, diagnosed to have this tumor on the left
labium majus. Excision was carried out, and no recurrence has been detected at 3, 9 and 18 months.
JRMS April 2008; 15(1): 58-60
Introduction
Phyllodes tumor is the term now recommended by
the World Health Organization for the tumor
previously called “Cystosarcoma Phyllodes”. The
later term was used to indicate large fleshy tumor
with a leaf-like appearance on the cut surface and is
not originally intended to denote malignancy. (1)
Phyllodes tumor is a rare fibroepithelial neoplasm
of the breast that accounts for about 0.5% of breast
tumors in females and tends to behave in a benign
fashion, but rarely may undergo sarcomatous
transformation. (2) It is a disease almost exclusively of
women, and occurs most often in the third and fourth
decades of life. In men it is very rare and only few
cases have been described. (3) There are reports of
occurrence in adolescents, as well as in elderly
women. (4,5)
Phyllodes tumor a peculiar tumor that produces a
spectrum of diseases ranging from benign, yet with
the risk of local recurrences, to malignant, sometimes
with a rapidly progressive course. (3) In the past, no
fewer than 62 different definitions were used to
define this particular tumor of the breast. (6) Because
most tumors are benign, the term “Cystosarcoma
Phyllodes” may be misleading, thus the favored
terminology is now phyllodes tumor. (4) Surgery
remains the main primary treatment and an adequate
resection margin must be obtained in all cases. (5)
*From the Department of Histopathology, Queen Alia Military Hospital, (QAMH), Amman-Jordan.
** Public Health and Preventive Medicine Division, Royal Medical Services, Amman-Jordan
Correspondence should be addressed to Dr. A. Abu-Alsamen, P.O. Box 390 Amman 11953 Jordan
Manuscript received October 30, 2004. Accepted June 16, 2005
Aberrant breast tissue may occur anywhere along
the embryonic milk ridge or milk line, (7) which runs
from the anterior axillary folds to the inner thighs.
Most cases of aberrant breast tissue are found above
the umbilicus. (8) The occurrence of actual breast
tissue in the vulva is very rare, (9) with only a couple
of cases reporting phyllodes tumor in the vulva. (10,11)
Case report
A 34-year-old single woman presented to the
Gynecology clinic at Prince Hashem Military
Hospital complaining of a mass on the vulva. She
stated that this mass had been present for 12 years.
She had noted no changes in its size and denied any
acute pain, redness, drainage, or purulent discharge.
Her chief complaint was pruritus and irritation. She
reported no other medical or surgical problems.
Physical examination disclosed a 5x3x2-cm wellcircumscribed,
mobile, nontender soft tissue
fungating mass of the left labium majus. The
remainder of her pelvic examination showed normal
results. Simple excision of the mass was carried out
under local anesthesia. At surgery, the mass was
noted to be well circumscribed and was easily
removed. The patient did well postoperatively and
was discharged home the next day.
Histopathological examination of the excised mass
showed a well-circumscribed lesion composed of
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mesenchymal and epithelial elements. The epithelial
component is characterized by elongated leaf-like
epithelial proliferation. The mesenchymal component
showed increased stromal cellularity mainly in periductal
regions. There was no evidence of cellular
atypia, necrosis, or high mitotic activity. These
features are diagnostic for benign phyllodes tumor
(Fig 1, 2, and 3). No evidence of recurrence was
noted after 18 months follow up.
Fig. 1. Squamous epithelium of the vulva with
underlying tumor (H&E, Low power)
Fig. 2. Leaf-like pattern with cellular stroma (H&E,
medium power)
Fig. 3. Bland epithelium surrounded by a cellular
stroma (H&E, high power)
Discussion
The first description of aberrant breast tissue in the
vulva of human is attributed to Hartung in 1872, who
reported a case of fully formed mammary gland
tissue in the left labium majus of a 30-year old
woman. (9) Deaver and Macfarland noted only 2 cases
of vulvar breast tissue in their review of almost
11,000 examples of extramammary breast tissues. (9)
Since Hartung’s account, 36 cases of aberrant breast
tissue in the vulva have been reported. It is estimated
that aberrant breast tissue occurs in 1-6% of the
general population. (8) This tissue is capable of
behaving like normally situated breast tissue and
responds to physiologic stimulation, such as
lactation. (12)
Histologically, aberrant breast tissue is composed
of large ducts without fully developed lobules. This
tissue is subject to the same pathologic changes as
the normally situated breast, including the occurrence
of tumors such as fibroadenoma, carcinoma, or
phyllodes tumors. (9,10) Most lesions remain
undetected until stimulated by hormones or until a
pathologic process affects them. Most cases of
aberrant breast tissue in young females are usually
benign. In contrast, cases seen in older patients have
a high risk of neoplasia. (8)
Phyllodes tumor is a rare breast tumor, and is even
much rarer in extramammary locations such as the
vulva, axillae, and perineum. (8,10,11,13) There is a 20-
40% risk of local recurrence, and if malignant may
cause metastasis. Malignant lesions range from 3% to
45% of reported cases. (14) Phyllodes tumors are best
regarded as a spectrum of fibroepithelial neoplasms:
malignant tumors may grow very rapidly and cause
metastatic spread. In contrast, benign tumors behave
similar to fibroadenomas, and can be cured by simple
excision. (14)
The histological criteria given for the diagnosis of
phyllodes tumor include a well-developed, leaf-like
growth pattern and hypercellular stroma that may
have poorly cellular or sclerotic areas. Similar to
fibroadenomas, phyllodes tumors are composite
lesions of proliferating stroma and epithelium.
However, phyllodes tumor is distinguished by its
typical growth pattern, the tendency to become
cystic, and by the greater cellularity of its stroma. (1,15)
The diagnosis of phyllodes tumor is mainly
dependent on its leaf-like growth pattern. The
hypercellularity is prognostically very important, but
its presence does not make the diagnosis in the
absence of the leaf-like configuration. (10) It is usually
difficult to make the diagnosis of phyllodes tumors
by fine needle aspiration biopsy as it may not reveal
the leaf-like configuration. (14) However, the presence
of cohesive stromal cells, isolated mesenchymal
cells, clusters of hyperplastic duct cells, foreign body
giant cells, bipolar naked nuclei, and the absence of
apocrine metaplasia are highly suggestive of a
phyllodes tumor.
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Based on the histological characteristics of the
tumor, including its margin (pushing or infiltrating),
stromal cellularity (slight or severe), stromal
overgrowth (absent, slight, or severe), tumor necrosis
(present or absent), cellular atypia (absent, slight, or
severe), and the number of mitoses per high power
field, they can be classified into “benign”,
“borderline”, and “malignant” categories. (5,10)
The histological features in our case are those of a
benign tumor, as there was no stromal overgrowth,
no cellular atypia, no tumor necrosis, and no increase
in mitotic activity. This is supported by the absence
of recurrence 18 months after excision. In our case,
the diagnosis of phyllodes tumor of the vulva was not
suspected from the clinical or operative findings as
this type of tumor is very rare in this location, and
fine needle aspiration cytology was not performed
prior to surgery. Therefore, it was only possible to
reach the diagnosis of this rare entity by histological
examination.
It is recommended at the time of surgery to excise
the lesion with adequate margins to prevent local
recurrence. (14) Anecdotal cases have suggested that
adjuvant radiotherapy and chemotherapy may be
beneficial in tumors with poor prognostic
histological features (hypercellular stroma, high
nuclear pleomorphism, high mitotic rate, infiltrating
margins, the presence of necrosis and increased
vascularity within the tumor). (6)
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2. Sawyer EJ, Hanby AM, Ellis P, et al. Molecular
analysis of phyllodes tumors reveals distinct
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