Primary breast leiomyosarcoma: case report and literature review

doi:10.4236/health.2011.310104

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Vol.3, No.10, 620-622 (2011) Health

doi:10.4236/health.2011.310104

Primary breast leiomyosarcoma: case report and

literature review

Fatima Zahra EL M’ra bet1,2*, Hanane El kac emi1, Omar El Mesbahi2, Khal id El Hasso uni1,

Brahim khalil El Gu eddari1

1Radiotherapy unit, National Institute of Oncology, Rabat, Morocco; *Corresponding Author: [email protected]

2Departement of medical oncology, university hospital Hassan II, Fez, Morocco.

Received 4 April 2011; revised 16 May 2011; accepted 29 May 2011.

ABSTRACT

PURPOSE: Leiomyosarcoma of the breast is

extremely rare. To date, the factors that are

predictive of patient prognosis have not been

identified. To clarify the nature of leiomyosa-

rcoma of the breast, and also to establish the

proper treatment strategy, we report this case

We report this case while discussing The Cli-

nical presentation, diagnosis, therapy and path-

ologic feature. INTRODUCION: Sarcomas com-

prise less than 1% of all primary breast neo-

plasms and only a minority of these are leio-

myosarcomas. There were only 35 cases being

reported in English literature. The mainstay of

treatment is surgical excision with clear mar-

gins and longterm followup is essential. Its pro-

gnosis is better compared to other breast sar-

coma. CASE PRESENTATION: We report a case

of primary leiomyosarcoma of the breast occ-

urring in young female patient which was succ-

essfully treated by surgery and radiotherapy.

CONCLUSION: According to literature data,

primary leiomyosarcoma is characterized by a

better prognosis compared with other breast

sarcomas, To date, the factors that are predic-

tive of patient prognosis have not been iden-

tified. The rarity and diagnostic difficulty impo-

sed a multidisciplinary approach.

Keywords: leiomyosarcoma; Breast;

Immunohistochemistry; Mastectomy

1. INTRODUCTION

Sarcomas of the breast account for less than 1% of all

malignant tumors of the breast. Leiomyosarcomas bel-

ong to a less common subgroup (5% to 10%) of sar-

comas of the breast [1]. There were only 35 cases being

reported in English literature. Confirming the diagnosis

on either immunohistochemistry is important as it is

sometimes difficult to differentiate these pathologically

from poorly differentiated sarcomatoid carcinomas. To

date, the factors that are predictive of patient prognosis

have not been identified. [2]

In this report, we describe case with this uncommon

Tumor, which was treated successfully in The Institute

of National Oncology Rabat, Morocco.

2. CASE REPORT

A 40-year-old female patient, without any significant

history, who presented a left breast lump, it has been pre-

sent for 17 months. There was no other breast complaint

and no family history of breast cancer. Physical exam-

ination found a tumor of 6 cm in diameter in the lower

inner quadrant of her right breast with ipsilateral axillary

lymph node. The left breast was unremarkable. Mam-

mography showed a dense, well circumscribed mass

with little evidence of local invasion and the lump was

easily detectable by ultrasound. An incisional biopsy was

performed, the histological exam showed a sarcomatous

proliferation, Immunohistochemically, the tumor cells

were diffusely and strongly positive for α-smooth mus-

cle actin and vimentin (Figure 1), while negative for

cytokeratin, CD34 and S-100 protein ,which concludes

with primary breast leiomyosarcoma .

Investigations including Liver echography, chest scan

and bone scan were showed no evidence of another

primary tumor or metastatic disease. The patient under-

went a radical mastectomy with lymph node dissection.

Additional immunohistochemical examen routinely perf-

ormed in breast carcinomas (oestrogen, progesteron rec-

eptors, Her 2) also proved to be negative. Tumor cells

showed strong positivity for vimentin, desmin and smoo-

th muscle actin (SMA). Axillary lymph nodes were free

of metastases Axillary. Subsequently the patient had rec-

eived adjuvant radiotherapy to 50 Gray, the patient is in

good control until now. A follow-up examination revea-

led no evidence of distant metastasis or local recurrences

8 years after the treatment.

F. Z. EL. M’rabet et al. / Health 3 (2011) 620-622

621621

Figure 1. The immunohistochemical stains for α-smooth muscle actin are diffusely and strongly positive in the tumor cells.

3. DISCUSSION

Sarcomas comprise less than 1% of all primary breast

neoplasms and only a minority of these are leiomy-

osarcomas [1] Leiomyosarcoma is not rare among soft

tissue tumors. It can exist in any part of the body.

However, its most common primary sites are the uterus,

retroperitoneum, subcutaneous tissues and gastrointes-

tinal tract [3]. Leiomyosarcomas of the breast are extre-

mely rare, either primary or metastatic from other sites.

The smooth muscle component might be originated from

the blood vessels and sarcomatous change is usually

from leiomyoma or other spindle cell tumors [4]. Our

presented case should be regarded as primary leiomyo-

sarcoma of the breast since there was no other primary

focus being found after staging. Confirming the diagno-

sis on either IHC or electron microscopy is important as

it is sometimes difficult to differentiate these patholo-

gically from poorly differentiated sarcomatoid carcino-

mas [5].

Diagnosis can be challenging because of the nonspe-

cific clinical and radiological findings. Clinically they

present with a progressively enlarging tumour. The pre-

sence of enlarged axillary nodes is rare making axillary

node staging unnecessary [6]. Our patient had a progr-

essively tumor evolving for 17 months, with ipsilateral

axillary lymphadenopathy nonspecific.

Radiological findings are also non specific. Leiom-

yosarcoma is observed as a dense, circumscribed, lobu-

lated mass on mammography, showing features similar

to phyllodes tumors or fi broadenomas as described in

most reports. Furthermore, on ultrasonography, as repor-

ted in all the reports cited, leiomyosarcomas are either

oval or lobulated tumors with a smooth clear border, mi-

micking a fi broadenoma, phyllodes tumor, or an intra-

cystic papilloma. Therefore, with an image analysis alo-

ne, differential diagnosis between a leiomyosarcoma and

a benign tumor can be extremely diffi cult [7,8].

Histologically, leiomyosarcoma is characterized by

interlacing bundles of spindle cells and the absence of

ductal or epithelial differentiation. Assessment, Immu-

nohistochemical staining is essential as adjuncts to diff-

erentiate leiomyosarcomas from other tumours and soft

tissue sarcomas. These tumours are usually positive for

desmin, smooth muscle actin, muscle specific actin and

negative for neural tumour marker (PS 100), cytoker-

atins, and epithelial markers. [2,9]

The principal treatment strategy is surgical resection.

The complete excision of the tumor by completely inclu-

ding the surgical margin should be recommended as the

F. Z. EL. M’rabet et al. / Health 3 (2011) 620-622

622

best treatment. Most authors advice either radical or sim-

ple mastectomy arguing that wide excision is associated

with higher rate of recurrence [2,5]. Though axillary

node dissection is not necessary, it has been performed

in many of the reported cases because of uncertain pre-

operative diagnosis or if there were clinically palpable

nodes [6]. In our case, the patient underwent a radical

mastectomy with lymph node dissection for the presence

of clinically palpable axillary lymph nodes, lymph nodes

were free of metastases Axillary. There is no evidence

describing whether radiation therapy and/or chemothe-

rapy improve the disease-free rate or overall survival [10].

In general, the prognosis of patients with leiomy-

osarcoma of the breast is considered to be better than

that for patients with other sarcomas of the breast [11]

4. CONCLUSIONS

Leiomyosarcoma of the breast is extremely rare. To

date, the factors that are predictive of patient prognosis

have not been identified. Since local recurrences and

distant metastases could occur after a relatively long

period, a long-term follow-up is necessary.

5. CONSENT

Written informed consent was obtained from the pa-

tient for publication of this case report.

6. COMPETING INTERESTS

The authors declare that they have no competing in-

terests.

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