Breast sarcoma is a malignant non-epithelial neoplasm of the breast characterized by rapid growth and desemination, an unfavorable course. Sarcoma is manifested by the presence of a dense lumpy tumor in the mammary gland, thinning and hyperemia of the skin over the formation, expansion of the network of subcutaneous veins. Detection is performed on the basis of a complex of echographic, radiological, tomographic, cytological data. For breast sarcoma, radical surgical treatment (extended mastectomy) with radiation and chemotherapy is indicated.
Meaning
In mammology and oncology, breast sarcoma refers to rare types of malignant neoplasms, ranging from 0.6 to 2% among them. Disease can develop in women and men; histologically, this type of neoplasm is identical to similar soft tissue tumors found in other anatomical areas. Most often, sarcoma develops against the background of a leaf-shaped tumor of the breast. Breast sarcoma is prone to rapid growth and metastasis, has an extremely malignant course and an unfavorable prognosis.
Classification
The structure of sarcoma lacks epithelial and glandular tissue. Most often, sarcomas originate from the stromal component — striated or smooth muscles, adipose, nervous, bone tissue, etc. In the mammary gland, the development of various histological types of sarcomas is possible. The size of breast sarcoma varies in the range from 1.5 to 15 cm.
According to the morphological variant, fibrosarcoma, liposarcoma, chondrosarcoma, angiosarcoma, leiomyosarcoma, rhabdomyosarcoma, osteosarcoma, malignant fibrous histiocytoma, etc. are distinguished. Histological examination often reveals fibrosarcomas (about 30%) originating from connective tissue fibers, which reach large sizes and are not accompanied by ulceration.
Liposarcomas are malignant mesenchymal tumors from adipose tissue, also characterized by rapid progression, but they often ulcerate and synchronously affect both mammary glands. Rhabdomyosarcomas, represented by elements of striated muscles, are characterized by extreme malignancy, rapid growth with infiltration of tissues. This type of breast sarcoma affects mainly young women under the age of 25.
Angiosarcomas formed by vascular endothelial cells are fast-growing tumors characterized by persistent recurrent course; they are detected mainly at the age of 35-45 years. Chondrosarcomas and osteosarcomas of extra-skeletal localization account for 0.25% of cases of all breast sarcomas, differ in a pronounced degree of malignancy; they occur in women over 55 years of age. By origin, there are primary breast sarcoma and secondary, developing from initially benign formations (for example, leaf-shaped tumors).
Causes
The true causes of breast sarcoma have not been determined. Among the factors contributing to the development of sarcoma, chemical carcinogens, radiation, injury to the breast, burdened family history of cancer can act.
Quite often, the appearance of sarcoma is preceded by insufficiently radical sectoral resection of the breast for some benign neoplasm – fibroadenoma, leaf—shaped or spindle-cell tumor. Breast sarcoma can be combined with fibroadenoma or cancer of the opposite gland, cervical and vaginal cancer, rectal cancer.
Symptoms
The development of sarcoma begins with the appearance of a dense nodular formation in the mammary gland, which has clear contours and a bumpy surface. As the tumor node grows, the skin above it becomes thinner, acquires a bluish-purple hue, against which an expanded subcutaneous venous pattern stands out.
Tumor growth leads to an increase in the volume of the affected breast, breast asymmetry, soreness, ulceration of the skin over the tumor. In advanced stages, a decaying tumor can bleed. With the rapid growth of sarcoma, a pronounced inflammatory reaction sometimes develops, simulating a breast abscess.
The rate of local spread of breast sarcoma can be slow, progressive, abrupt and violent. It is noted that high-grade sarcomas develop rapidly or abruptly, in a few months; tumors with a relatively favorable prognosis – slowly, sometimes for many years. Breast sarcomas are characterized by a hematogenic pathway of metastasis to the lungs and skeleton; involvement of regional lymph nodes is rare.
Diagnostics
Clear criteria for the diagnosis of breast sarcoma in mammology have not been developed due to the rare occurrence of pathology and a large range of histological variability. A mammologist-oncologist is engaged in the diagnosis and treatment of breast sarcoma. Palpation in the mammary gland determines a large, elastic, mobile tumor with a heterogeneous consistency; when examined, attention is drawn to puffiness and hyperemia of the skin, areas of ulceration.
The data of review mammography and ultrasound of the mammary glands in sarcoma are non-specific. In the course of the study, shadows are revealed in the form of a conglomerate of nodes with lumpy contours that push aside the surrounding tissues. Thinning of the skin over the tumor and dilated subcutaneous veins of the breast are well defined. Additionally, MRI or breast scintigraphy with technetium-99 can be performed.
Finally, the diagnosis of breast sarcoma can be confirmed only with the help of morphological and cytological examination of tumor samples obtained by fine needle or trepan biopsy. Microscopically, sarcoma is represented by stroma elements, cellular atypia with pronounced polymorphism and enlarged nuclei, and the absence of an epithelial component.
Treatment
An adequate volume of intervention in the detection of breast sarcoma is mastectomy. Less often, with highly differentiated sarcomas, radical resection is performed. Lymphadenectomy is indicated for the detection of metastases in regional lymph nodes or in the case of a sarcoma close to lymphatic structures, especially during the collapse of the tumor.
A comprehensive approach to the treatment of breast sarcoma can improve long-term results. Therefore, in the postoperative period, chemotherapy with anthracycline antibiotics is performed. The need for radiation therapy is considered only in cases of questionable radicalism of surgical intervention.
Forecast
Prognostic factors affecting the duration of the relapse-free period and survival are the histotype of breast sarcoma, the degree of differentiation, and the size of the tumor. The best survival rates are observed in highly differentiated sarcomas and the absence of metastasis to regional lymph nodes.
Breast sarcoma is prone to local recurrence in the area of postoperative scars, breast stump, as well as distant metastasis to lung and bone tissue. The question of the expediency of reconstructive mammoplasty is debatable.
Literature
- Zeng W., Styblo T.M., Li S. et al. Breast angiosarcoma: FDG PET findings. Clin Nucl Med 2009;34(7):443-5. link
- Silverman L.R., Deligdisch L., Mandeli J., Greenspan E.M. Chemotherapy for angiosarcoma of the breast: case report of 30 year survival and analysis of the literature. Cancer Invest 1994;12(2):145-55.
- Georgiannos S.N., Sheaff M. Angiosarcoma of the breast: a 30 year perspective with an optimistic outlook. Br J Plast Surg 2003;56(2):129-34. link
- Johnson C.M., Garguilo G.A. Angiosarcoma of the breast: a case report and literature review. Curr Surg 2002;59(5):490-4.
- Casper E.S., Waltzman R.J., Schwartz G.K. et al. Phase II trial of paclitaxel in patients with soft-tissue sarcoma. Cancer Invest 1998;16(7):442-6. link
