| Abstract|| |
Malignant mesenchymal tumors of the breast other than angiosarcoma are extremely rare and comprise <0.5% of breast tumors. Primary chondrosarcoma of the breast is an extremely rare entity and only 10 cases are reported as single case reports in literature until date. A diagnosis of primary mammary sarcoma can be established only after excluding metaplastic carcinomas and malignant phyllodes by extensive sampling for evidence of in situ or invasive carcinoma. Here, we report a primary chondrosarcoma of breast in a 55-year-old lady diagnosed precisely on fine-needle aspiration cytology and confirmed by histopatholigcal examination after total mastectomy. We emphasize on diagnostic difficulties encountered in cytology smears and discuss differential diagnoses.
Keywords: Breast, chondrosarcoma, cytology, histopathology
|How to cite this article:|
Sinhasan SP, Bharathi KV, Bhat RV, Dasiah SD. Primary chondrosarcoma of breast - cytology with histopathological correlation: A rare case report with review of literature. Indian J Pathol Microbiol 2014;57:311-3
|How to cite this URL:|
Sinhasan SP, Bharathi KV, Bhat RV, Dasiah SD. Primary chondrosarcoma of breast - cytology with histopathological correlation: A rare case report with review of literature. Indian J Pathol Microbiol [serial online] 2014 [cited 2019 Jun 25];57:311-3. Available from: http://www.ijpmonline.org/text.asp?2014/57/2/311/134727
| Introduction|| |
Mammary sarcomas are a heterogenous group of malignant neoplasms that arise from the supporting interlobular mammary stroma.  A diagnosis of primary mammary sarcoma can be established only after excluding metaplastic carcinoma by extensive sampling for evidence of in situ or invasive carcinoma. This distinction is important for treatment as well as for prognosis.  The precise diagnosis of breast sarcoma on cytology smears is challenging entity for cytopathologist. Here, we are reporting a case presenting as huge breast lump clinically diagnosed as breast carcinoma. We discuss the cytology findings with emphasis on diagnostic difficulties encountered with differential diagnoses and correlation with histopathology findings after total mastectomy.
| Case report|| |
A 55-year-old female patient presented to the surgical out-patient department with the complaints of lump in the left breast of 4 months duration. The lump gradually increased in size and attained present size. We received the requisition for cytological examination with clinical probable diagnosis of carcinoma breast. On inspection, entire left breast was massively enlarged and measured 15 cm × 12 cm, with overlying shiny-stretched out skin [Figure 1]a]. Nipple and areola appeared normal. On palpation, lump was soft to firm with variable consistency. There was no axillary lymphadenopathy. Fine-needle aspiration was carried out from two different areas. The cytology smears revealed cell rich aspirate containing pleomorphic tumor cells scattered in singles and clusters against extensive chondroid and chondromyxoid background [Figure 2]a]. High power examination showed tumor cells displaying hyperchromatic nucleus enclosed in a lacunae around them [Figure 2]b]. Scattered multinucleated tumor giant cells were also seen. When slides reviewed by our faculties in department, we received various differential diagnoses like infiltrating ductal carcinoma-metaplastic type with chondroid differentiation, malignant phyllodes tumor, chondroid syringoma and chondrosarcoma. In view of presence of characteristic chondroblasts in lacunae [Figure 2]b], and absence of convincing ductal component, we offered primary chondrosarcoma of breast as a final diagnosis and requested the surgeon for biopsy for further confirmation of this rare tumor and to rule-out metaplastic carcinoma/malignant phyllodes tumor. Meanwhile, ultrasound examination of breast revealed the tumor with heterogeneous echogenicity with an anterior halo and posterior shadowing and enhancement. There were no areas of calcification or lymphadenopathy. Radiologist offered diagnosis of malignant phyllodes tumor. This was followed by total mastectomy. Intra-operatively surgeon confirmed that it is not originating from underlying rib bones or cartilage and completely removed the tumor with clearance of surgical margins.
Grossly, the total mastectomy measured 17 cm × 13 cm × 7 cm covered by elliptical bit of skin bearing nipple and areola. Cut section showed fairly well-circumscribed tumor nodule measuring 10 cm × 7 cm. The tumor was lying 0.5 cm from overlying skin and 1.4 cm from deep resection margin. The tumor was seen extending right up to the lateral surgical margins on both the sides sparing 1 cm margin. The cut surface of the tumor was grey white, homogeneous, lobulated and gelatinous with extensive myxoid areas with foci of hemorrhages and cystic spaces [Figure 1]b]. Extensive sampling of the tumor was done taking sections at every 1 cm interval. No lymph nodes were identified in the attached fibrofatty tissue.
|Figure 1: (a) Entire left breast was massively enlarged with overlying shiny-stretched out skin, (b) Total mastectomy specimen showing well-circumscribed tumor with homogeneous, gelatinous areas with foci of hemorrhages|
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Histopathological examination revealed well-circumscribed tumor with nodular, multilobulated [Figure 3]a] infiltrative growth pattern of varying cellularity, composed of round to slightly elongated cells. The individual tumor cells displayed hyperchromatic nuclei and prominent elongated nucleoli with abundant deeply eosinophilic cytoplasm enclosed within lacunae reminiscent of chondroblasts [Figure 3]b]. These cells were arranged in short anastomosing cords, strands and lace like patterns. The background stroma showed extensive chondroid and chondromyxoid areas entrapping the tumor cells. The mitotic figures were seen at a range of 1-2/hpf in cellular areas. Meticulous search for the ductal epithelial cells doesn't show either benign or malignant component. Blocks were sent to Immunohistochemistry at the private center (additional material sent) and received a report as sarcoma due to positivity for vimentin and negativity for cytokeratin and ER. Hence, the final diagnosis of primary chondrosarcoma of breast was offered. Six months postoperative follow up of the patient is uneventful. However, she is instructed for a long time follow-up.
|Figure 2: (a) Fine-needle aspiration cytology smear showing cell rich aspirate containing pleomorphic tumor cells scattered in against extensive chondromyxoid background (Pap, ×10), (b) Cytology smear showing pleomorphic tumor cells with nucleus enclosed in a lacunae reminiscent of chondroblasts (Pap, ×40)|
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|Figure 3: (a) Histopathology section showing tumor with multinodular infiltrative growth pattern with chondroid stromal background (H and E, ×10), (b) Microscopy showing tumor cells with marked degree of pleomorphism with nucleus enclosed within lacunae suggestive of chondroblasts against chondromyxoid stroma (H and E, ×40)|
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| Discussion|| |
The term stromal sarcoma was introduced in 1962, the first case of breast angiosarcoma was reported in 1967 by Kennady and Biggart.  Until now only 10 case of primary chondrosarcoma of the breast are reported in literature as per the most recent article  and ours is 11 th case report.
A sarcomatous appearing tumor in the breast is far more likely to be a metaplastic carcinoma or a malignant phyllodes tumor than a primary sarcoma.  Therefore, before concluding that any breast tumor is a primary sarcoma, efforts should be made to exclude metaplastic carcinoma be extensively sampling the tumor to identify foci of conventional invasive mammary carcinoma and/or ductal carcinoma in situ.  As we did in our case, the tumor is dissected at every 1 cm interval and extensive sampling doesn't reveal any foci of ductal origin. The other possibility of phyllodes tumor is also excluded by extensively examining the lesion to identify the benign epithelial component that characterizes these lesions. Primary sarcomas of the breast other than angiosarcomas are extremely rare. Some sarcomas arise in the chest wall and secondarily involve the breast, particularly those that develop following irradiation; these may be mistaken for primary breast sarcomas.  After angiosarcoma, the other sarcomas in decreasing order of frequency are liposarcoma, fibrosarcoma, leiomyosarcoma, rhabdomyosarcoma, malignant peripheral nerve sheath, and osteosarcoma. Matrix-producing carcinoma of the breast is a unique subclass of metaplastic carcinoma, which is characterized by the existence of a ductal carcinomatous component with direct transition to areas showing cartilaginous or osseous differentiation, lacking an interspersed spindle cell component. 
The cytological diagnosis of primary sarcoma of the breast is not always easy. More common matrix-producing mammary carcinomas and phyllodes tumors have to be excluded by careful search for epithelial component.  When the tumor size is massive, multiple site aspirations yield better results. For this cytologist should gain patient's cooperation and take her confidence into account. Preparations of many thin smears stained with variety of stains are the key point in the success. Characteristic cytomorphological features include an abundant chondroid extracellular matrix to which chondroid type cells with variable degree of atypia. Giemsa-stained smears demonstrate the extracellular metachromatic stromal elements more clearly than Papanicolaou stained smears. Use of Diff quik stain can also be very useful in the identification of stroma in this neoplasm.
A review of the reported incidence of local recurrence in relation to primary treatment suggests that total mastectomy should be recommended for most sarcomas of the breast.  Axillary lymph node metastases are exceedingly uncommon at the time of primary therapy. Axillary dissection or sentinel lymph node sampling are not ordinarily indicated in breast sarcomas. 
The origin of primary sarcoma of the breast is obscure, and speculations abound. Misplaced mesenchymal rests, chondroid differentiation of tumors of mesenchymal origin, and pluripotential cells from periductal and acinar mesenchymal cells undergoing chondroblastic differentiation have been proposed. The study of the microenvironment of sarcomas reveals an interesting situation where cancer cells of mesenchymal origin are surrounded by stromal cells of the same origin. At present, the number of documented cases of breast chondrosarcomas is too small to permit a critical discussion of their origin. 
| Conclusion|| |
The cytological features of chondrosarcoma are characteristic and allow a conclusive diagnosis provided the smears are examined keenly bearing in mind the diagnostic traps that can mislead a cytopathologist. Preoperative cytological diagnosis is challenging as primary chondrosarcoma of breast usually will not be considered in the differential diagnoses. We offered the precise diagnosis in our case in view of presence characteristic chondroblasts enclosed within lacunae against chondromyxoid background. However, this particular finding may not be so evident in all cases, as it was in the present one. Histologically extensive sectioning of the tumor is very essential to rule-out metaplastic carcinoma and malignant phyllodes tumor. Even tiny foci of ductal component, whether benign or malignant, should strongly question the diagnosis. Careful search for ductal epithelial component is mandatory to confirm primary sarcoma of breast and immunohistochemistry plays a big role in such cases.
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Sankappa P. Sinhasan
Department of Pathology, Indira Gandhi Medical College and Research Institute, Puducherry Government Institutions, Puducherry - 605 009
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3]