Primary breast chondrosarcoma

Last revised by Henry Knipe on 20 Jun 2018

Primary breast chondrosarcoma is a rare type of sarcoma that originates from the mammary stroma and not from the underlying bone or cartilage of the chest wall.

The prevalence of primary breast chondrosarcoma is reported to be 0.5-1%, they represent <5% of all sarcomas 1,14.

The clinical presentation of primary breast chondrosarcoma is varied, but they usually occur in females over 50 years old. It varies from a rapidly growing, mobile, well-circumscribed mass, that may be painful, to an irregular mass with lobulated margins.

Primary breast chondrosarcoma originates from mesenchymal cells of the mammary stroma.

Microscopically, primary breast chondrosarcoma is characterized by cellular atypia in chondroid lacunae and cellular pleomorphism 9,12. Pure chondrosarcomas without any area of epithelial differentiation are even rarer.

The role of imaging is to determine staging and prognosis, but the definitive diagnosis is made histopathologically.

Usually appear relatively benign as a hyperdense opacity with regular margins and a round shape.

A hyperechoic multilobed or a complex echoic mass can be observed.

MRI is used to looking for extension/invasion into adjacent tissues, skin or lymph nodes.

Gold standard treatment is surgical resection. Several effective mastectomy techniques have been reported, all with an adequate margins resection, in order to reduce the risk of recidivism. The excision of axillary lymph nodes is not recommended since these are rarely invaded and no improvement in prognosis has been reported.

In the case of adjuvant therapy, the literature reports a low response to both, chemotherapy and radiotherapy, which is why it is not recommended to use it as an adjuvant treatment.

Prognosis is usually favorable, but it depends on the size and extension of the lesion, resection of the tumoral margins and the histopathological characteristics of each tumor.

ADVERTISEMENT: Supporters see fewer/no ads