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2014, Volume 30, Number 3, Page(s) 225-227
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DOI: 10.5146/tjpath.2014.01249 |
Primary Chondroblastic Osteosarcoma of the Breast |
Mohamed Reda EL OCHI1, Fouad ZOUAIDIA2, Rachad KABAJ1, Hafsa EL OUAZZANNI1, Mohamed ALLAOUI1, Mohamed OUKABLI3, Abderrahman AL BOUZIDI3, Basma EL KHANNOUSSI1 |
1Department of Pathology, National Institute of Oncology, RABAT, MAROC
2Ibn Sina Hospital, RABAT, MAROC
3Mohamed V Military Hospital, RABAT, MAROC |
Keywords: Breast, Osteosarcoma, Sarcoma |
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Pure sarcomas of the breast are uncommon, accounting for less
than 1% of primary breast malignant tumors. Mammary osteogenic
sarcomas are very rare and less than 100 cases have been reported
in literature. They mainly affect older and middle aged women and
are highly aggressive. We report an additional case in a 56-year-old
woman. Histological and immunohistological characteristics
were similar to those described in other localizations. Differential
diagnosis involves phyllodes sarcoma, breast metaplastic carcinoma
with chondroid and osteoid differentiation, osteosarcoma of the ribs,
and metastatic osteosarcoma. The prognosis is poor. |
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Extraskeletal osteosarcomas are uncommon neoplasms
representing less than 1% of soft tissue sarcomas. Mammary
sarcomas are also rare, accounting for less than 1% of all
primary breast malignant tumors. In this group, primary
osteosarcomas are one of the least common sarcomas of the
breast 1,2. Most cases have been reported in the literature as
case reports except Silver et al who reported a retrospective
analysis of 50 cases 3. With less than a hundred cases of
primary mammary osteosarcoma reported in the literature 4, we report an additional case with this unusual tumor. |
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Abstract
Introduction
Case Presentation
Disscussion
References
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A 56 year-old postmenopausal woman, without clinical
antecedent, presented with a breast lump that had rapidly
grown for 7 months. On examination, breast palpation
revealed a painless, mobile, hard, relatively regular shaped
and large mass of 11x8 cm, involving all four quadrants of
the right breast. There was no axillary lymphadenopathy.
Ultrasound showed a heterogenous hypoechoic solid mass.
Mammography showed a large hyperdense and round
mass with calcifications (Figure 1). Biopsy was performed
and showed a high-grade sarcoma with chondromatous differentiation. Preoperatively, the staging did not reveal
primary or metastatic extramammarian sarcomatous
tumor.
A mastectomy was performed. Grossly, a right mastectomy
specimen measuring 18x12x5cm was received. The tumor
was grey white, hard, with regular margins, measured
11x8.5x6 cm, and involved all four quadrants of the breast
with small areas of haemorrhage, cysts and necrosis.
Histological examination revealed chondrosarcomatous
proliferation, with foci of chondroid background containing
atypical neoplastic cells. Furthermore, there were solid and
compact sheets of spindle cells (Figure 2) producing an
osteoid and bony matrix (Figure 3). No areas of epithelial
component were identified despite achieving complete
section slices of the tumor. Nipple areolar areas, skin and
lymph nodes were free of tumor. On immunohistochemistry,
tumor cells displayed immunoreactivity for vimentin.
Positivity for S-100 and EMA was noted in chondroid areas
(Figure 4). The spindle cell component was positive for
smooth muscle actin but negative for desmin. Cytokeratin,
p63 and CD34 were all negative. Thus, a diagnosis of
primary chondroblastic osteosarcoma was made.
The search for a primary bone tumor, especially in the
rib cage, was negative. The patient presented with lung
metastasis 2 months after mastectomy. She died 3 months
later.
Click Here to Zoom |
Figure 4: On immunohistochemistry cells of chondromatous
component were positive for S-100 protein (S100; x40). |
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Abstract
Introduction
Case Presentation
Disscussion
References
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Pure sarcomas of the breast are uncommon, accounting for
less than 1% of primary breast malignant tumors 5,6. More
common breast sarcomas include fibrosarcomas, malignant
fibrous histiocytoma, angiosarcoma, and liposarcoma 3,7.
Primary mammary osteosarcoma of the breast represents
12.5% of breast sarcomas 5,8. Tumors with the following
criteria are considered ‘pure osteosarcoma’: absence of bone
origin, presence of osteoid or bony matrix, absence of an
epithelial differentiation, and absence of a benign tumor 9. The largest series, from the Armed Forces Institute of
Pathology, Washington in the U.S.A., reported 50 cases, 3.
Mammary osteosarcoma predominates in middle-aged
and older women3,9. Most often, they arise as de
novo conditions without predisposing factors especially
after radiation therapy3. The association between
osteosarcoma and fibroadenoma was previously reported4. The duration of symptoms is variable but most present
within few months3. All cases reported in literature had
unilateral disease5.
Clinical features, mammographic and macroscopic
findings are not specific10. Mammographically, these
tumors are usually dense and well-circumscribed with
focal or extensive coarse calcifications5,11. They may
radiologically simulate a benign tumor5.
Microscopic findings of primary mammary osteosarcoma
are similar to skeletal and other extraskeletal osteosarcomas.
Considerable diversity in morphological appearance
has been reported with variants like fibroblastic,
osteoblastic, osteoclastic, and chondroblastic; a variable
amount of osteoid tissue and bone are present in all5.
Chondrosarcomatous differentiation is unusual3,10 but
was extensive in our case.
The histogenesis of this entity is unclear. It probably develops
from totipotent stromal cells or a transformation from
pre-existing fibroadenoma or phyllodes tumor3,5. The
main differential diagnosis is malignant phyllodes tumor
with osteosarcomatous component but also metaplastic
carcinoma, osteogenic sarcoma arising from the underlying
ribs or sternum, and metastatic osteosarcoma3,9.
In localized forms, the treatment is based on surgical
excision with clear margins. Lymph node metastases are
exceptional so that lymphadenectomy is not indicated12,13. There is controversy regarding the use of
chemotherapy. For some authors, it is not recommended
especially in localized and well-resected low-grade tumors13. Regarding metastasizing tumors, treatment is based
mainly on chemotherapy using conventional drugs for
osteosarcoma (doxorubicine, ifosfamide, cisplatinium,
methotrexate)12,13.
The prognosis is poor and the five-year survival is 38%.
Recurrences are less frequent in patients treated with
mastectomy than those treated with local excision.
Metastases occur mainly in the lung; there is no axillary
node involvement in almost all cases9. Cases of death
occurring a few months after the diagnosis have been
reported1,4,6. |
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Abstract
Introduction
Case Presentation
Discussion
References
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breast. Breast. 2003;12:72-4. |
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Abstract
Introduction
Case Presentation
Discussion
References
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