Roberto N. Gonzales Jr. MD.

Edgardo Penserga, M.D., FPCS, FPSGS

Reynaldo Joson, M.D., MHA, MHPEd, MS Surg

Department of Surgery

Ospital ng Maynila Medical Center

Key words : Carcinosarcoma

Reprint request : Roberto N. Gonzales Jr. M.D.

Department of Surgery, Ospital ng Maynila Medical Center

OMMC Surgery@yahoogroups.com

Abstract

A 27 y/o female Filipino patient presented with  a 4 x 4 cm palpable mass on the upper outer quadrant of her left breast which enlarged dramatically to 21 x 40 cm for the span of 7 months. Fine needle aspiration cytolology was done and had been considered highly suspicious for carcinoma. Initial diagnosis was breast carcinoma and was planned to undergo a modified radical mastectomy. Patient underwent modified radical mastectomy of left breast. Final histopathology report was a Metastatic carcinoma, CARCINOSARCOMA, Gr III, 20 cm in its greatest diameters w/ skin ulceration, w/ microscopically very near basal margin of the breast, left w/ metastasis to 16/18 ALN including 2 matted; clear surgical margins; Stage IV. Discussion centered on carcinosarcoma a very rare lesion of the breast which accounts for only 0.1% of all breast malignancy.

Introduction

            Carcinosarcoma (CaSa), as defined by the World Health Organization, is a tumour composed of intimately admixed malignant epithelial and stromal components .  It is considered a distinct form of metaplastic carcinoma of the breast. Secretory carcinomas of the breast are uncommon tumours, generally encountered in children and are therefore called by some authors as juvenile carcinomas. However, they have also been reported in older patients including post-menopausal women . We report a case of CaSa of the breast with an unusual secretory carcinoma as the malignant epithelial component.

            This is a case report of a female Filipino patient who was diagnosed to have carcinosarcoma of the breast.

            This case report is made to develop the awareness on carcinosarcoma of the breast

CASE REPORT

A 27 year old lady presented with a painful lump in the left breast of seven months duration. The lump was small initially and gradually increased in size. There was no past history of surgery / radiotherapy / chemotherapy. Clinically an irregular, firm and fixed mass was felt beneath the nipple and areola in the left breast. The nipple was drawn outwards. The skin was ulcerated with foul smelling discharges. Axillary nodes were palpable. The right breast and systemic examination revealed no abnormality.

She was diagnosed as Malignant neoplasm of the breast [ st IIIB, T4N2M0], left and underwent a  modified radical mastectomy.

Gross examination of the specimen showed a 22.0 x 20.0 x 15.0 cm with axillary fat pad measuring 8 x5 x 3 cm. The areola and nipple measures 4 cm and 1.5 cm in widest diameter. At the center of skin ellipse is a single ulcerating mass measuring 20 x 17 x 15 cm extending up to the areola.

Sections show a well defined gray white, doughy to friable mass with some areas of necrosis. A total of 18 lymph nodes were recovered including 2 matted measuring 3 cm in tis widest diameter.. Microscopic examination of sections of the breast tumour showed a malignant tumour with carcinoma and sarcoma elements. The carcinomatous component was a secretory type of carcinoma with a tubulo-alveolar pattern, lined by cells with vacuolated cytoplasm. The carcinomatous component was intimately admixed with a fusiform spindle cell sarcoma at places . The sarcomatous areas showed  mitotic count of 10/10 HPF. Areas of necrosis were seen. No osseous / cartilagenous matrix / osteoclastic giant cells/ squamous cell carcinoma areas were noted.

DISCUSSION

Carcinosarcoma (CaSa) of the  breast accounts for only 0.1% of all cases . CaSa, as applied to the breast, has been used as a generic term for biphasic neoplasms having both malignant epithelial (carcinoma) and malignant stromal (sarcoma) components . Metaplastic carcinoma is a generic term for tumours of ductal type in which the predominant component has an appearance other than epithelial and glandular and more in keeping with another cell type. The term is too encompassing, imprecise and includes sarcomatoid carcinoma, CaSa, spindle cell carcinoma, carcinoma with osteoclastic giant cells and squamous cell carcinoma . The term CaSa is used where the sarcoma component resembles fibrosarcoma (FS), malignant fibrous histiocytoma  (CS), osteogenic sarcoma (OS), rhabdomyosarcoma (RMS), angiosarcoma (AS) or a combination of these various patterns, and when present with a carcinomatous component. The carcinomatous component usually is  the typical ductal type of carcinoma. The term CaSa has not been applied consistently in literature. Some authors use the term CaSa only for those biphasic tumours where at least 50% of the tumour is composed of a cellular malignant appearing spindle cell component with the carcinoma component being contiguous or admixed with the sarcomatous component. The stroma, by definition, had to appear sarcomatous. Other authors maintain that the term CaSa is applicable only to those neoplasms composed of epithelial and mesenchymal cancers without evidence of transition between the two . The presence of a carcinoma not contiguous or admixed with an existing sarcoma is viewed as two separate tumours. Some authors have characterized these biphasic lesions according to the immunohistochemical reactivity of the mesenchymal elements; if positive immunostaining with CK markers is seen they would classify the tumour as metaplastic carcinoma and if none is present, the tumour would be grouped as ‘CaSa’. The main differential diagnosis of CaSa is an infiltrating carcinoma with formation of a pseudosarcomatous stroma. Sarcomatoid carcinomas often show separation, albeit a gradual transition between the epithelial and the sarcoma-like component. The sarcoma like element, here, is also CK positive. In our case, the carcinomatous and sarcomatous areas were intimately admixed. FS, MFH, OS and stromal sarcoma were ruled out because of the presence of an overt malignant glandular component. Cystosarcoma phylloides with malignant transformation of the epithelial component was ruled out because of the absence of curved elongated ducts and stromal leaf-like projections and the presence of an extensive secretory carcinoma component. Matrix producing carcinoma was excluded because of the lack of chondroid and/or osseous stromal matrix. A squamous metaplastic component was not seen. Secretory carcinoma is rare in adults. Even though axillary lymph nodal metastasis are seen, systemic metastasis is rare in a pure secretory carcinoma . There is no known hormonal abnormality that would explain the  heterogeneous group of neoplasms with CaSa being a distinct form and also the most aggressive form in keeping with its high-grade stroma. These tumours commonly occur in older individuals and display aggressive behaviour. CaSa is reported to have a 5 year survival of 49%. Although controversy still exists regarding their exact histogenesis and their classification remains obscure, pathologists should recognize and diagnose CaSa to assign the correct prognosis and ensure appropriate treatment to the patient.

References :

1. Wicks MR, Swanson PE. Carcinosarcomas : current  perspectives and a historical review of nosologic concepts.

 Semin Diagn Pathol 1993;10:118-27.

2. Rosen PP, Cranor ML. Secretory carcinoma of the breast.

Arch Pathol Lab Med 1991;115:141-4.

3. Wada H, Enomoto T, Tsujimoto M, Nomura T, Murata Y, Shroyer KR.

            Carcinosarcoma of the breast. Molecular biological study for analysis of    histogenesis. Hum Pathol 1998;29(1):1324-8.

4. Silverberg SG, Masood S. Breast. In: Silverberg SG, Delellis RA, Frable WJ, editors. Principles and Practice of Surgical Pathology and Cytopathology. 3rd ed,

New York. Churchill Livingstone 1997:575-673.

5. Rosai J. Breast. In: Rosai J, editor. Ackerman’s Surgical Pathology, 8thed,

St.Louis. Mosby 1996:1565-1660.

6. Azzopardi JG. Special Problems in Breast Pathology. In: Bennington JL, editor. Major Problems in Pathology-Vol 11,London: WB Saunders 1979:258 333.