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Indian Journal of Cancer, Vol. 40, No. 2, (April - June 2003) , pp. 77-79 Case Report Primary Chondrosarcoma of the Breast: A Case Report Gupta S, Gupta V, Aggarwal PN, Kant R, Khurana N,* Mandal AK
Departments of *Pathology and Surgery, Maulana Azad Medical College & Associated
LN Hospital,
New Delhi-110002, India. Code Number: cn03011 ABSTRACT Mammary sarcomas are uncommon tumors. Of these pure chondrosarcoma without any other area of epithelial or mesenchymal differentiation feature as even rarer. This excludes tumors like malignant cystosarcoma phyllodes and metaplastic carcinoma where malignant cartilaginous areas may be present. Further primary chondrosarcoma arises from breast stroma and not from underlying bone or cartilage. Only five cases of pure and primary chondrosarcoma have been reported so far. The sixth case is reported here. Key Words: Chondrosarcoma, Breast, Sarcoma. Introduction Pure sarcomas are very uncommon tumors of the breast, representing about 0.5% of all mammary tumors.1 They represent a diverse and heterogenous group of neoplasms. Pure sarcomas of the breast lack epithelial component. Pure, primary and denovo chondrosarcoma features as one of the rarer types of sarcomas of the breast.2 A pre operative clinical and cytological diagnosis though possible in a few cases is usually not reached both due to marked similarity in clinical behavior and low index of suspicion. Case Report A 46-year-old postmenopausal female presented in the surgery out patient department (OPD) of Lok Nayak Hospital with a lump in left breast for the last 8 months. She noticed the lump after blunt trauma. A diagnosis of breast abscess had been made in another hospital, and an incision and drainage was attempted. Patient presented to us with non-healing wound. There was no history of nipple discharge or any other breast problem. Patient was married with three children. There was no family history of breast cancer. There was no history of exposure to radiation. On examination, a hard lump 14x15 cm size, occupying most of the breast was noted. It was not attached to underlying structures however overlying skin was ulcerated and covered with slough. A single 1x1 cm firm, mobile ipsilateral axillary lymph node was also clinically present. Contralateral breast and axilla were normal on clinical examination. A clinical diagnosis of carcinoma breast was proposed. FNAC done in our hospital was reported as infiltrating duct carcinoma with no evidence of metastasis to the axillary lymph node. No distant metastasis was detected on various investigations. Patient was given 3 cycles of chemotherapy preoperatively (CAFCyclophosphamide, Adriamycin and 5-FU). Tumor showed partial response to chemotherapy. Subsequently a modified radical mastectomy was performed. Histopathology The right mastectomy specimen including axillary dissection measuring 20x18x8 cm was received in the Department of Pathology, Maulana Azad Medical College. Overlying skin showed an area of ulceration covered with slough. Nipple and areola were unremarkable. On serial slicing a huge grey white growth involving all the four quadrants measuring 12x8x4 cm was seen. The tumor on cross section was firm grey white. There were no gritty, glistening or necrotic areas seen. Eleven lymph nodes ranging in size from 0.5 to 2 cm were isolated. Microscopically A well differentiated chondrosarcoma was diagnosed. A lobular tumor was seen with multiple chondroblasts in single lacunae. The chondroblasts were frequently multinucleated with plump nuclei. There were frequent mitoses at the margins of the lobules. The tumor cells were surrounded by breast ducts lined by normal looking epithelial cells (Figure 1). The overlying skin, nipple, areola and all eleven lymph nodes were free of tumor. The specimen was further extensively sampled to look for any other malignant stromal or epithelial component. None was found. A final diagnosis of chondrosarcoma of the breast was made. The tumor cells were negative for hormone receptors. Patient did not develop any post-operative complications. She has been on regular follow-up for over a year now with no complaints what-so ever. Discussion Primary breast sarcomas are a highly heterogenous group of tumors. Majority of these are malignant fibrous histiocytoma, fibrosarcoma, liposarcoma, and less commonly angiosarcoma, rhabdomyosarcoma, dermatofibrosarcoma, desmoid tumors etc. Previous radiotherapy to the breast increases the risk of angiosarcoma of the breast. True stromal sarcomas are still rare. Extra skeletal chondrosarcoma and osteogenic sarcoma are rare tumors. Most of the extra skeletal chondrosarcoma fit into the category of myxoid chondrosarcomas,3 and occur in extremities of adult patients. Primary chondrosarcoma occurring in the breast is extremely rare. It contains chondrosarcomatous areas throughout the tumor and arises from the breast itself rather than from underlying bone or cartilage. Only five cases of pure and primary chondrosarcoma of breast have been reported so far. Kennedy and Biggart4 reported one case in 1967, Beltaos and Banerjee2 reported two cases in 1978 and Thilagavathi et a15 reported fourth case in 1992. Last case was reported by Guymar et al6 in 2001. These tumors are usually large in size and occur in women more than 40 years old. Regional lymphadenopathy is expected in 14-29% of these cases, most of which are reactive hyperplasia.7 The present case substantiates the clinical findings of previously reported cases. These tumors usually do not invade the overlying skin.4 The present case although showed ulceration of skin, there was no tumor deposit on the skin. The ulceration was probably due to previous surgery. Microscopically the tumor shows chondroid areas with cellular atypia and pleomorphism. This has to be clearly differentiated from matrix producing metaplastic carcinoma8 and also firm malignant cystosarcoma phyllodes with chondroid differentiation.9 Differentiation from metaplastic carcinoma is possible by absence of direct transition between carcinomatous and mesenchymal component in the former. Further the sarcoma like elements in metaplastic carcinoma though acquire vimentin positivity, still retain epithelial markers.10 Differentiation form malignant cystosarcoma phyllodes with predominant chondrosarcomatoid component can be extremely difficult. Benign ductal elements interspersed - among sarcomatoid areas should be taken as evidence of former where as the present case displayed clear-cut demarcation between the two elements. Most mammary tumours with areas of chondroid metaplasia show benign histological appearance. Cystosarcoma phyllodes displaying a chondrosarcomatous element are very rare. Surgery remains the mainstay of treatment for most sarcomatoid tumors.11 Multimodality treatment may decrease local and systemic recurrence rates of somatic sarcomas, but results are inconclusive in patients with breast sarcomas.12 As only five cases of primary chondrosarcoma of the breast have been reported so far, role of radiotherapy or chemotherapy in this particular breast sarcoma is difficult to assess. As against this, the present case showed partial response to chemotherapy. Favorable prognostic factors like low degree of cellular atypia and mitotic rate could have had a bearing on this response to chemotherapy. The tumor was negative for any of the hormonal receptors. This supports the theory that adjuvant therapy with estrogen antagonists and other hormone manipulations have no role in treatment of mammary sarcomas. Conclusion Primary sarcomas of the breast are rare tumors and it is important that these be recognized as a separate entity from the more common breast carcinoma keeping in mind the difference in behavior of the two tumors while planning therapy. References
Copyright 2003 - Indian Journal of Cancer
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