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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 53  |  Issue : 4  |  Page : 785-786
Primary osteogenic sarcoma of the breast


Department of Pathology, Christian Medical College & Hospital, Ludhiana, India

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Date of Web Publication27-Oct-2010
 

   Abstract 

Primary osteogenic sarcomas of the breast are extremely rare with less than 100 cases reported in literature. They generally present in middle aged and older women and are highly aggressive. These tumors need to be differentiated from osteoid/bone- producing metaplastic carcinomas as the two differ in their biologic behavior and treatment protocols. A 60-year-old lady presented with a short history of a rapidly enlarging breast lump. Histopathogical examination revealed a richly cellular, pleomorphic tumor with abundant osteoid. The tumor cells were immunoreactive for vimentin and negative for cytokeratin and estrogen and progesterone receptors.

Keywords: Breast, breast sarcoma, osteosarcoma

How to cite this article:
Jacob S, Japa D. Primary osteogenic sarcoma of the breast. Indian J Pathol Microbiol 2010;53:785-6

How to cite this URL:
Jacob S, Japa D. Primary osteogenic sarcoma of the breast. Indian J Pathol Microbiol [serial online] 2010 [cited 2020 Nov 24];53:785-6. Available from: https://www.ijpmonline.org/text.asp?2010/53/4/785/72090



   Introduction Top


Extra-skeletal osteosarcomas (OS) are uncommon, comprising less than 1% of all soft tissue sarcomas. [1] They have been reported at many sites but their occurrence in the breast is extremely unusual. [2] With less than a hundred cases of primary mammary OS reported in literature , this report documents one more case of this rare entity. [3]


   Case Report Top


A 60-year-old lady presented with a 4-month history of a rapidly enlarging painless lump in the right breast. There was no history of local trauma. Clinical examination revealed a firm to hard, nontender, slightly mobile breast lump measuring 7x5 cm. The tumor was not fixed to the skin or underlying ribs. Nipple, areola, and skin were unremarkable. There was no axillary lymphadenopathy. A plain chest X-ray showed clear lung fields and uninvolved underlying ribs. A right simple mastectomy was done.


   Pathology Top


Serial cut sections of the mastectomy specimen showed a well-circumscribed firm to hard tumor measuring 7x6 cm, with a variegated grayish white, dark brown, and hemorrhagic appearance [Figure 1]. The nipple, areola, and skin were unremarkable. Microscopic examination disclosed a highly cellular tumor composed of sheets of cells. The neoplastic cells were spindle, ovoid to polyhedral with abundant eosinophilic cytoplasm, and moderately pleomorphic nuclei having prominent nucleoli and increased mitotic activity. Interspersed amidst these was numerous osteoclast type of multinucleated tumor giant cells. Abundant osteoid was noted either in the form of fine ramifying lace-like pattern or irregular islands [Figure 2]. Cartilaginous differentiation was not present. Osteoid and bone were more prevalent in the center of the lesion with hypercellular tissue at the periphery (reversed zonation pattern). Dilated vascular spaces rimmed by neoplastic cells "telangiectatic pattern" were also present [Figure 3]. Extensive sampling and a thorough search ruled out any foci of epithelial differentiation, phyllodes tumor, or fibroadenoma. The tumor was strongly positive for vimentin and nonreactive for cytokeratin (CK), and estrogen (ER) and progesterone (PR) receptors. A diagnosis of primary osteosarcoma of the breast was made.
Figure 1 :Mastectomy specimen with a circumscribed growth (serially sectioned) having a variegated cut surface. Arrow points to the location of the nipple

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Figure 2 :Sheets of malignant osteoblasts, lace-like osteoid, and osteoclastic multinucleated giant cells (H and E, ×200)

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Figure 3 :(a) Partly calcified tumor osteoid infiltrating amidst mammary acini (H and E, ×200). (b) Telangiectatic pattern (H and E). (c-f) Tumor cells express strong positivity for vimentin and are immunonegative for CK, ER, and PR

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   Discussion Top


Breast cancer is the commonest cancer that affects females worldwide. [2] Of all the cancers of the breast, carcinoma forms the overwhelming majority while sarcomas are of negligible incidence. [1],[2],[4],[5] The more common breast sarcomas include fibrosarcomas, malignant fibrous histiocytoma, angiosarcoma, and liposarcoma. [1],[6] Primary OS of breast are distinctly rare with published references generally limited to occasional case reports. The largest series reported is a clinicopathological analysis of 50 cases, seen over a 38-year period at the Armed Forces Institute of Pathology, Washington. [1]

The precise frequency of primary osteosarcoma of breast is difficult to assess because of its infrequent occurrence and due to variation in nomenclature. [4] Collectively mammary sarcomas make up less than 1% of primary breast malignancies of which OS forms a mere 12.5%, reflecting its extreme rarity. [4],[5]

Mammary OS predominates in middle aged and older women. [1],[4],[7] The duration of symptoms is variable but most present within few months. [1],[8] Mammography may appear deceptively benign. All cases reported in literature had unilateral disease. [4] The tumor size ranged from 1.4 to 30 cm with mean of 4.6 cm. [1],[8]

Histologically primary OS of breast is indistinguishable from conventional OS of bone. Considerable diversity in morphologic appearance has been reported with variants like fibroblastic, osteoblastic, osteoclastic, and chondroblastic but in all variable amount of osteoid and bone are present. [4]

The histogenesis of primary mammary OS is not clear but an origin from toti potent mesenchymal stromal cells or a transformation from preexisting fibroadenoma or phyllodes tumor has been suggested. [2],[4],[7]

The main pathological differential diagnosis of primary mammary OS is metaplastic carcinoma containing osteoid and bone. This differentiation is of prime importance as the two entities have different histological behavior and require different treatment. [4] Metaplastic carcinoma is immunoreactive for CK, whereas OS is negative. The cells of OS also display negativity for EMA, ER, PR, and Her2, and are vimentin positive; areas of cartilaginous differentiation show S100 positivity. [1],[4] Although immunohistochemistry plays a major role in differentiating OS from sarcomatoid carcinoma, some sarcomas may display abundant CK expression. Therefore, extensive sampling of the specimen is still considered a crucial step. [1]

Overall primary mammary OS are highly aggressive with early recurrence and propensity for hematogenous spread, most frequently to lungs. [1] The 5-year survival rate is a dismal 38%. [1] Unlike carcinomas, OS of the breast rarely metastasizes to lymph nodes. Hence only a wide surgical excision of the tumor without axillary node dissection has been advocated. [1],[6] The response of the tumor to adjuvant chemotherapy is unclear and surgery remains the mainstay of treatment. [9]

To conclude, primary OS of the breast are exceedingly rare. It is of utmost importance to be aware of this unusual occurrence and to differentiate these from osteoid/bone-producing metaplastic carcinoma as the two differ in their biological behavior and treatment protocols.

 
   References Top

1.Silver SA, Tavassoli FA. Primary osteogenic sarcoma of the breast. A clinicopathologic analysis of 50 cases. Am J Surg Pathol 1998;22:925-33.  Back to cited text no. 1
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2.Ogundiran TO, Ademola SA, Oluwatosin OM, Akang EE, Adebamowo CA. Primary osteogenic sarcoma of the breast. World J Surg Oncol 2006;4:90.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Brustugun OT, Reed W, Poulsen JP, Bruland OS. Primary osteosarcoma of the breast. Acta Oncol 2005;44:767-70.  Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
4.Bahrami A, Resetkova E, Ro JY, Ibanez JD, Ayala AG. Primary osteosarcoma of the breast. Report of 2 cases. Arch Pathol Lab Med 2007;131:792-5.  Back to cited text no. 4
    
5.Blanchard DK, Reynolds CA, Grant CS, Donohue JH. Primary non-phylloides breast sarcomas. Am J Surg 2003;186:359-61.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Ciatto S, Bonardi R, Cataliotti L, Cardona G. Sarcomas of the breast: A multicenter series of 70 cases. Neoplasma 1992;39:375-9.  Back to cited text no. 6
[PUBMED]    
7.Anani PA, Baumann RP. Osteosarcoma of the breast. Virchows Arch A 1972;357:213-8.  Back to cited text no. 7
    
8.Brown AL, Holwill SDJ, Thomas VA, Sacks NP, Given-Wilson R. Case report: Primary osteosarcoma of the breast: Imaging and histological features. Clinical Radiology1998;53:920-2.  Back to cited text no. 8
    
9.Irshad K, Mann BS, Campbell H. Primary osteosarcoma of the breast. The Breast 2003;12:72-4.  Back to cited text no. 9
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Correspondence Address:
Sunitha Jacob
Professor, Department of Pathology, Christian Medical College & Hospital, Ludhiana-141 008, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.72090

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    Figures

  [Figure 1], [Figure 2], [Figure 3]

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