Primary Fibrosarcoma of Breast: A Rare Case Presentation: Cash Report

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International Surgery Journal

Pothare AN et al. Int Surg J. 2015 Nov;2(4):721-724


http://www.ijsurgery.com pISSN 2349-3305 | eISSN 2349-2902

DOI: http://dx.doi.org/10.18203/2349-2902.isj20151113
Cash Report

Primary fibrosarcoma of breast: a rare case presentation


Amit Narayan Pothare, Vipul Versi Nandu*

Department of Surgery, SVNGMC, Yavatmal, Maharashtra, India

Received: 24 August 2015


Accepted: 07 October 2015

*Correspondence:
Dr. Vipul Versi Nandu,
E-mail: [email protected]

Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under
the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial
use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT

Primary Fibrosarcoma of breast is an extremely rare tumor accounting for <1% of all the primary breast malignancies
and <5% of all the sarcomas. Breast fibrosarcoma is a rare tumor characterized by marked rapidity of growth.
Diagnostic investigations like FNAC, mammography and USG are relatively insensitive and histopathological
diagnosis is mainstay and it is usually a diagnosis of exclusion. Surgical therapy is most effective treatment and
chemotherapy should be considered for large tumors with high mitotic rates. In all the diagnosis and management
should be multidisciplinary team approach. In this case report we are reviewing a case of primary Fibrosarcoma of
breast in a 45 years old female presenting with a rapidly growing painless mass in left breast since 3 months.
Repeated FNAC were inconclusive and hence incisional biopsy was done which revealed Fibrosarcoma. Modified
radical mastectomy was done with axillary clearance and histopathology reports further confirmed the diagnosis.
Being a very rare tumor, the primary aim of this presentation is to provide an overview of literature on the diagnosis
and management protocols for breast fibrosarcoma patients.

Keywords: Fibrosarcoma, FNAC, Incisional biopsy, Modified radical mastectomy

INTRODUCTION fibrosarcoma reported.1 Terrier et al, in his study of 33


cases of sarcoma of breast only 2 cases of fibrosarcoma
Sarcomas of breast are extremely rare breast tumors were reported.2 Blanchard et al, in his study of 55 cases
accounting for <1% of all the primary breast of sarcoma only 2 cases of fibrosarcoma were reported.11
malignancies and < 5% of all the sarcomas.1-3 They arise
from mesenchymal tissues of the breast. The annual The peak incidence of fibrosarcoma occurs in 5th – 6th
incidence is about 44.8 new cases per 10 million women.4 decade of life, but for fibrosarcoma of breast the peak
The exact definition of breast sarcoma is still not incidence is unknown. Roberson Et al reviewed the
uniform. Some authors excluded cystosarcoma phylloids literature of 49 reported patients with fibrosarcoma of
from their studies due to presence of epithelial breast and half of them were 41-60 years of age.12
components but others do not because of their similar
clinical and surgical course.1-3,5-9 Most publicized articles The most common presentation of fibrosarcoma of breast
are limited to small retrospective reviews and case is rapidly progressing painless lump in breast which
reports, making clinicopathological study difficult. attains a large size. Metastasis from breast fibrosarcoma
commonly spread haematogenously typically to lungs,
The overall incidence of primary fibrosarcoma of breast bone and liver.1-3,9 Lymph node metastasis is rare.3,13
is not known nor has it been reported in literature.10 In a
review conducted by Poland et al of 25 cases of primary The rarity of this pathology led to lack of consensus on
breast sarcoma, during 80 yrs. period, only 4 cases of the optimal treatment and hence a multidisciplinary

International Surgery Journal | October-December 2015 | Vol 2 | Issue 4 Page 721


Pothare AN et al. Int Surg J. 2015 Nov;2(4):721-724

approach is needed for diagnosis and treatment of


patients.3,14

CASE REPORT

Figure 2: Gross appearance of tumor.

Figure 1: Clinical photograph of fibrosarcoma breast.

A 45 years old female patient, G3 P3 L3 A0 in her third


year of menopause presented with rapidly growing
painless lump in left breast since 5 months. The lump was
extending over whole of breast, measuring 20 x 15 x 10
cms in dimensions. It was firm to hard in consistency
with nodular surface with reddish brown discoloration of
overlying skin. There was fixity to skin but no fixity to
pectoralis major muscle. The mass was involving the
entire nipple-areola complex. There were no axillary
lymph nodes palpable.
Figure 3: Microscopic appearance of tumor.
Repeated 3 FNAC of tumor came to be inconclusive and
so incisional biopsy taken which was suggestive of
fibrosarcoma of breast. USG of breast reveals solid
hyperechoic mass with no cystic component. X-ray chest The second most proposed risk factor is chronic
and USG abdomen did not reveal any secondary deposits lymphedema of breast or arm.19 (Especially for
in chest and abdomen. Angiosarcoma)
After proper pre-anesthetic workup and fitness modified People with hereditary disease like neurofibromatosis or
radical mastectomy was done with primary closure of Li fraumani syndrome have an increased risk of
defect. Specimen sent for histopathology examination developing soft tissue sarcoma.20
confirming the diagnosis.
Clinical presentation
Grossly the tumor was big, nodular involving the whole
breast tissue. Histopathology examination shows tumor
The most common presentation of fibrosarcoma of breast
mass composed of spindled shaped cells arranged in
is rapidly progressing painless lump n breast which
whorls and band pattern. Nuclei are hyperchromatic,
attains a large size. Metastasis from breast fibrosarcoma
pleomorphic with prominent nucleoli. Mitosis rate is
commonly spread haematogenously typically to lungs,
increased. There was no duct like or epithelial gland like
bone and liver.1-3,9 Lymph node metastasis is rare.3,13
structure.
Diagnosis
No recurrence at operative site or distant metastasis
reported in follow up period of 6 months.
Diagnosis on basis of FNAC is often difficult to interpret
and has limited accuracy.21,22 So histopathological tissue
examination is must for clinical diagnosis. On
histopathological examination, breast sarcomas are

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Pothare AN et al. Int Surg J. 2015 Nov;2(4):721-724

divided several subtypes- Angiosarcoma, Malignant Radiotherapy


fibrous histiocytoma, Stromal sarcoma, Spindle cell
sarcoma and Fibrosarcoma. Among them Angiosarcoma In contrast to surgical therapy, there is widespread
is most common. disagreement for the benefits of radiotherapy in breast
sarcomas.31 Majority of studies did not find any benefit
Cellular pleomorphism, mitotic numbers, stromal atypical for adjuvant radiotherapy.1,7,11,32 But McGowan et al and
and infiltrating borders have been found to be main Johnstone et al demonstrated significant benefits for
prognostic factors.6,7,23 patients.3,33 They recommended post-operative irradiation
of at least 60 grays to whole tumor bed. It may help to
Mammography is nonspecific demonstrating reduce the local recurrence of tumor. Fibrosarcomas are
nonspiculated dense mass without microcalcification.21,24 relatively insensitive to radiotherapy.

On USG they appear as hyperechoic with no Chemotherapy


shadowing.25 MRI may display rapid enhancement with
washout characteristics of lobules.26 The role of chemotherapy for breast sarcomas remains
unclear, but adjuvant chemotherapy is worth a shot in
Staging patients with highly malignant sarcoma, with positive
surgical margins or post-operative recurrence.34 The most
Table 1: AJCC system for soft tissue sarcoma27 effective chemotherapy regimen is Adriamycin (ADM) +
Ifosfamide (IFO).
Location:
Histological Systemic
Stage Size Superficial Prognosis
grade metastasis
or Deep
<= 5 It is generally agreed that the prognosis of disease is
IA Low Superficial No
cm related with tumor cell differentiation, tumor size and
>5 surgical approaches.29 Large studies have demonstrated a
IB Low Superficial No 5 years disease free survival ranging from 44-66% and a
cm
>= 5 5 year overall survival ranging from 49-67%.2,3,5-7,9,13,35,36
II A Low Deep No
cm
<5 Superficial CONCLUSION
II B High No
cm or Deep
Breast fibrosarcoma is a rare tumor characterized by
>= 5
II C High Superficial No marked rapidity of growth. Diagnostic investigations like
cm
FNAC, mammography and USG are relatively insensitive
>= 5
III High Deep No and histopathological diagnosis is mainstay and it is
cm usually a diagnosis of exclusion. Surgical therapy is most
IV Any Any Any Yes effective treatment and chemotherapy should be
considered for large tumors with high mitotic rates. In all
Surgery: the diagnosis and management should be
multidisciplinary team approach.
For all breast sarcomas surgical resection is the first
modality of treatment. In comparison to wide local Funding: No funding sources
excision, mastectomy was considered gold standard but Conflict of interest: None declared
recent study conflict this comparison and considered both Ethical approval: Not required
of them equivocal.3,5,7,9,13 For smaller localized breast
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7. Cite this article as: Pothare AN, Nandu VV. Primary
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the pre-operative diagnosis of phyllodes tumours of Surg J 2015;2:721-4.

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