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Primary breast sarcomas are rare tumors that originate from
the mesenchymal tissue of the breast. In a study conducted at
the Mayo Clinic, the prevalence of primary breast sarcomas
among breast cancers was found to be 0.0006% . Primary
leiomyosarcoma of the breast is more rarely observed, and only
24 cases have been reported in the English medical journals. In
the present study, we present a case involving primary leio-
myosarcoma of the breast and we discuss optimal treatment
A 48-year-old female patient was referred to our hospital
because of pain and a mass in the breast. She noticed a lump
in her right breast 8 months earlier. In the following months,
this lump had grown rapidly and led to a tightening of the skin
of the right breast.
A physical examination revealed a tender mass that was 10
cm in size and extended from the upper outer quadrant of the
right breast to the lower outer quadrant. There was neither
retraction of the nipple nor palpable axillary or supraclavicular
lymphadenopathy. The contralateral breast was normal. Mam-
mography and ultrasonography revealed a well-circumscribed
tumor mass that was 8×6 cm in size and located in the upper
and lower outer quadrants of the right breast (Figure 1).
Because of evidence of malignancy in the fine needle aspiration
biopsy, the patient underwent total mastectomy of the right
breast, total excision of the pectoralis major muscle due to inva-
sion in the pectoral muscle, and axillary lymph node dissection.
In the macroscopic pathological examination of the mastec-
tomy specimen, a necrotic tumoral mass that was 10×9×6 cm
in size was observed and invasion into the overlying breast skin
and pectoralis major muscle was evident (Figure 2). Micro-
scopically, the tumor was composed of pleomorphic spindle
cells showing frequent mitoses and necrosis. Tumor cells dem-
onstrated diffuse immunohistochemical staining with smooth
muscle actin (SMA) (Figures 3-5). No staining was observed
with desmin, S100, CD117. According to the histopathological
and immunohistochemical analysis, the tumor was diagnosed
None of the 31 excised lymph nodes exhibited metastasis,
and the surgical margins showed no trace of tumor tissue.
A follow-up thoracoabdominal computed tomography scan
and bone scintigraphy performed 1 month after the operation
Primary leiomyosarcoma of the breast is rare and is consid-
ered an uncommon breast tumor. Only around 30 cases have
been reported in English medical journals. Because of the rarity
Primary Leiomyosarcoma of the Breast: A Case Report
Zülfikar Karabulut, Hampar Akkaya1, Gökhan Moray
Departments of Surgery and 1Pathology, Baskent University Medical Faculty, Ankara, Turkey
J Breast Cancer 2012 March; 15(1): 124-127 http://dx.doi.org/10.4048/jbc.2012.15.1.124
Primary leiomyosarcoma of the breast is an extremely rare tumor,
accounting for less than 1% of all breast tumors and only 24
cases have been reported in the English medical journals. It is
quite difficult to diagnose leiomyosarcoma preoperatively. Estab-
lishing an accurate diagnosis is very important in planning treat-
ment. When preoperative diagnosis can be achieved before or
during the operation, wide resection should be performed. There
is no need for axillary lymph node dissection. In this case report,
we present a case involving primary leiomysarcoma of the breast
in a 48-year-old woman and we discuss optimal treatment options.
Key Words: Breast, Leiomyosarcoma, Mastectomy
Correspondence: Zülfikar Karabulut
Department of Surgery, Baskent University Medical Faculty, Ankara, Turkey
Tel: +90-312-327-27-27, Fax: +90-312-327-12-76
Received: April 15, 2011 Accepted: October 24, 2011
Primary Leiomyosarcoma of the Breast
of the tumor more patient data should be presented for the
development of a proper treatment strategy.
Leiomysarcoma of the breast may originate from the mus-
cular blood vessels or from the smooth muscle of the nipple
[2,3]. It is quite difficult to diagnose leiomyosarcoma preopera-
Differantial diagnosis includes leiomyoma, spindle cell myo-
epithelioma and spindle cell sarcomatoid carcinomas as seen in
spindle cell purely epithelial metaplastic carcinomas (spindle
cell squamous metaplastic carcinoma and adenocarcinoma
with spindle cell differentiation). In leiomyomas the tumor
cells bland, without evidence of atypia and mitoses. Tumoral
necrosis is also not observed. Malign myoepithelioma often
lacking significant atypia, few mitosis, and at the periphery of
the lesion, more epitheloid cell population is evident emanating
Figure 2. Macroscopically, a lobulated elastic tumor that was, 10×9×
6 cm in size was totally excised.
Figure 3. Histopathology of leiomyosarcoma. Immunostaining for smooth
muscle actin (SMA) was positive in the malign spindle cells (SMA, ×200).
Figure 1. Preoperative mammogram. (A) A 8×6 cm well-circumscribed
tumor mass is located in the upper and lower outer quadrants of the
right breast. (B) The left breast is normal.
Figure 5. Smooth muscle actin (+) spindle tumour cells (×400).
Figure 4. Spindle shaped tumour cells and mitosis (H&E stain, ×200).
Zülfikar Karabulut, et al.
from the myoepithelial cell layer of the entrapped ductules. In
myoepithelioma tumors cells are SMA (+) and S-100 (+). Meta-
plastic carcinomas of the breast is a general term referring to a
heterogeneous group of neoplasms. Leiomyosarcoma of the
breast should be distinguished from spindle cell purely epithe-
lial metaplastic carcinomas (spindle cell squamous metaplastic
carcinoma and adenocarcinoma with spindle cell differentia-
tion). In spindle cell squamous metaplastic carcinomas, tumor
cells are epithelial in origin and positive for cytokeratins and
negative for SMA. In adenocarcinoma with spindle cell meta-
plasia, the tumor is composed of tubules admixed with neo-
plastic spindle cells. These spindle cells immunoreact with
epithelial markers including Cytokeratin 7 and are negative
for SMA. In these case the tumor cells demonstrated diffuse
immunohistochemical staining with SMA. No staining was
observed with desmin, S100, CD117, pancytokeratin, and
cytokeratin 7. According to the histopathological appearance
and immunohistochemical staining, the tumor was diagnosed
Establishing an accurate diagnosis is crucial in planning
treatment. To date, this type of tumor has been observed to
affect middle-aged women (mean age, 56 years) and manifests
as a mass in their breast over a long period of time. The average
tumor size is 4.7 cm [2,4]. Because two of the reported cases
were in males, the involvement of endocrine factors in this
tumor appears to be unlikely . This type of tumor tends to
show local recurrence and distant metastasis has been observed
in 25% of patients [5,6]. Metastatic spreading usually occurs
via the hematogenous route. Tumor size is believed to have no
significant correlation with metastasis . The studies published
to date show that the tumor presents itself as a long-standing
and slow-growing mass. The duration of symptoms varies
between 2 weeks and 5 years, confirming those characteristics
. Local recurrence and distant metastases can arise even after
15-20 years . Chen et al.  and Nielsen  lost their patients
16 and 20 years, respectively, after the operation due to hepatic
and multiple metastases.
The tumor is usually observed as a dense, well-circumscribed,
fibroadenoma-like mass. It is difficult to reach a diagnosis based
on fine needle aspiration (FNA) cytology. However, preopera-
tive diagnosis can be achieved using a combination of FNA
cytology and immunohistochemistry [3,10].
Leiomyosarcoma is characterized by spindle-shaped cells
with pleomorphic, hyperchromatic and elongated nuclei;
eosinophilic cytoplasm; large nucleoli; and significant mitoses.
Definitive diagnosis is established through histological exami-
nation, in which positive staining is observed immunohisto-
chemically with desmin, vimentin, and muscle-specific actin,
whereas negative staining is seen with cytokeratin, myoglobin,
and S-100 . Because leiomyosarcoma often invades periph-
eral tissues, such as the skin and fascia, curative surgery requires
a wide resection. Because there has been no reported cases of
lymph node metastasis in which dissemination occurred via
the hematogenous route, axillary lymph node dissection is
unnecessary if leiomyosarcoma diagnosis can be achieved
before or during the operation [4-6]. In the present case, there
was no axillary lymph node involvement. Wide resection was
performed due to the invasion of the pectoralis major muscle
by the tumor. The benefits of chemotherapy and radiotherapy
have not yet been confirmed [3,5].
The prognosis of leiomyosarcoma is better than that of other
breast sarcomas . The prognostic factors are not fully known
because of the limited number of studies. However, large tumors
with frequent atypia and mitoses appear to be malignant .
Based on the published case reports, the characteristics of
primary leiomyosarcomas of the breast are well-circumscribed,
slow-growing tumors showing a tendency for skin and muscle
invasion. In cases where diagnosis can be achieved before or
during the operation, wide resection should be performed. There
is no need for axillary lymph node dissection. They require
long-term follow-up because local recurrence and distant
metastasis can occur long after the operation. The benefits of
chemotherapy, radiotherapy, and hormonal therapy are still
controversial. It has a better prognosis than other breast sarco-
mas. However, there is a need for further studies to determine
the prognostic factors.
CONFLICT OF INTEREST
The authors declare that they have no competing interests.
Primary Leiomyosarcoma of the Breast