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Year : 2015  |  Volume : 11  |  Issue : 4  |  Page : 1024

A rare case of carcinosarcoma of breast: Coexistence of mucinous carcinoma and malignant fibrous histiocytoma

1 Department of Radiation Oncology, The First Hospital, Jilin University, Changchun, China
2 Department of Cancer Center, The First Hospital, Jilin University, Changchun, China

Date of Web Publication15-Feb-2016

Correspondence Address:
Lihua Dong
Department of Radiation Oncology, The First Hospital, Jilin University, 71 Xinmin Street, Changchun - 130021
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.147723

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 > Abstract 

Carcinosarcoma (CS) of breast is a rare disease. Published reports provided little consensus about its clinical characteristics and optimal treatment protocols. Here we present a patient with CS of breast (mucinous carcinoma and malignant fibrous histiocytoma) and review related literature. Combined modality treatment (CMT) brings at least 2 year disease-free survival (DFS). Our case highlights the possibility of breast CS and helps to expand our understanding of this distinct breast malignancy.

Keywords: Combined modality treatment, breast carcinosarcoma, metaplastic breast carcinoma

How to cite this article:
Liu M, Liu B, Song Y, Dong L. A rare case of carcinosarcoma of breast: Coexistence of mucinous carcinoma and malignant fibrous histiocytoma. J Can Res Ther 2015;11:1024

How to cite this URL:
Liu M, Liu B, Song Y, Dong L. A rare case of carcinosarcoma of breast: Coexistence of mucinous carcinoma and malignant fibrous histiocytoma. J Can Res Ther [serial online] 2015 [cited 2020 Nov 24];11:1024. Available from: https://www.cancerjournal.net/text.asp?2015/11/4/1024/147723

 > Introduction Top

Metaplastic breast carcinoma (MBC) is a distinct entity identified by the coexistence of two or more different neoplasms.[1] Carcinosarcome (CS) is a subgroup of MBC with biphasic epithelial and mesenchymal malignancies,[1] representing 11.7% of MBC,[2] less than 0.2% of breast neoplasms.[3],[4],[5] The most common carcinomatous and sarcomatous components are invasive ductal carcinoma and fibrosarcoma, respectively.[6] The coexistence of mucinous carcinoma and malignant fibrous histiocytoma is extremely rare. To our knowledge, this is the first report of breast CS of such pathological combination. Combined modality treatment (CMT) offers benefits of both survival and life quality.

 > Case Report Top

A 51-year-old female presented in February, 2012 with a 2-month history of painless and rapidly-growing mass of left breast from 1.0 × 0.8 cm to 2.5 × 2.5 cm. On physical examination, in the outer upper quadrant of left breast, an irregular lump of approximately 2.5 × 2.5 cm was palpable. There was no change of the breast skin and nipple. No enlarged lymph nodes were palpated in bilateral supra and subclavicular regions and axillas. Her past history was unremarkable.

The breast ultrasound showed a 2.47 × 1.21 cm hypoechoic lump with a few blood flows in the outer upper quadrant of left breast and a 0.54 × 0.34 cm hypoechoic nodule at the 12 o'clock of right breast. Mammography also revealed a 2.5 × 2.0 cm irregular mass of high density with calcifications [Figure 1]a and [Figure 1]b.
Figure 1: Mammography of left breast: (a) Craniocaudal view depicted a 2.5 cm × 2.0 cm high-density mass in the upper outer quadrant without clear boundary. (b) Mediolateral oblique view demonstrated 3 calcifications inside this irregular mass

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Then Mammotome needle biopsy of the lump of the left breast was performed and the pathologic diagnosis was mucinous carcinoma. Immunohistochemical examination showed ER (-), PR (-), Her-2 (-) and Ki-67 (70% +) [Figure 2]a. The following abdominal ultrasound and thoracic computed tomography (CT) excluded metastasis. Then left breast-conserving surgery and lump resection of right breast with Mammotome technique were performed. The final pathologic results showed: Carcinosarcoma of left breast [Figure 2]b. The tumor volume was 1.6 cm × 1.4 cm × 1.2 cm. The carcinomatous part accounting for one-third of the total was mucinous carcinoma with a MBNG of 3. The sarcomatous part representing two-third was malignant fibrous histiocytoma. All the borders were negative and there was no invasion of vessels or nerves. 28 excised left axillary lymph nodes proved negative for metastases. Thus, stageᴵ (T1N0M0) was diagnosed. The final immunohistochemical results were as follows: CK (carcinoma +, sarcoma -) [Figure 2]c, CK5/6 (carcinoma +, sarcoma -), EGFR (carcinoma +, sarcoma -) [Figure 2]d, ER(-), PR(-), Her-2(-), Syn (-), Vimentin (+). The lesion of right breast was benign.
Figure 2: (a) Ki-67 immunochemistry showing 70% positive. b) Hematoxylin–eosin stained section revealing the coexistence of both sarcomatous (upper part) and carcinomatous (lower part) components in the operative specimen. (c) Immunochemistry examination for CK in the operative specimen (carcinoma +, sarcoma-). (d) Immunochemistry examination for EGFR in the operative specimen (carcinoma +, sarcoma-)

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Then she received 6 cycles of adjuvant chemotherapy (Pirarubicin 75 mg (50 mg/m 2) d1, Cyclophosphamide 750 mg (500 mg/m 2) d1, Paclitaxel liposome 270 mg (175 mg/m 2) d2) followed by prophylactic radiotherapy to the whole left breast with a dose of 50Gy/25f and then a boost of 10Gy/5f to the tumor bed. To date, her condition remains stable and DFS has reached to 2 years.

 > Discussion Top

Breast CS is extremely rare which occurs in a wide spectrum of patients, aged from 16–82,[5],[7] commonly in middle-aged female. Rapid-growing breast lump is the most common clinical manifestation. Compared with breast cancer, axillary lymph node is less involved while hematogenous spread is more common. Pleural and lung metastasis are more frequent than bone, liver, brain.[8] MBC usually has a base-like immuno phenotype.[9] However, CS seems to have a better survival than other subtypes of MBC such as squamous and adenosquamous carcinoma.[7]

Preoperative diagnosis for breast CS is challenging. Breast CS have no specific manifestations by ultrasound and mammography. Similar to breast cancer, magnetic resonance image (MRI) of CS only demonstrated malignancy for the time versus signal intensity curve reached to plateau after rapid eclipse of contrast,[10] lacking satisfactory specificity for the diagnosis of breast CS.[11] Core needle aspiration biopsy can be misdiagnosed due to lack of adequate specimen. The accurate diagnosis usually depends on the postoperative pathology.[6],[11] In our case, the preoperative Mammotome needle biopsy only showed mucinous carcinoma element.

To date, the molecular pathogenesis of breast CS is still vague. Most studies suggest that it originates from myoepithelial cells because myoepithelial cells can differentiate into both epithelial and mesenchymal cells.[4],[12] Evgeny N et al. indicated biallelic inactivation of BRCA1 gene might contribute to its onset and rather dismal outcome.[13] Abnormality of PI3K/AKT and MARK pathway components was found to be involved in MBC.[14]

Given high aggressiveness of breast CS, CMT is crucial for the improvement of overall survival (OS).[6] Modified radical mastectomy was considered as the first option, especially for T2 and highly staged patients.[8],[15] For patients with breast-conserving surgery (BCS), the role of postoperative radiotherapy (RT) has been challenged by some reports.[8],[15] However, Warren H. Tseng et al. verified the OS benefit of RT for MBC patients after lumpectomy or mastectomy.[2] For individuals with BCS or those with either primary tumor ≥ 5 cm or over four metastatic axillary lymph nodes after mastectomy, RT is strongly indicated.[2] Early- staged patients prefer BCS because of increasing cosmetic awareness. Thus, postoperative RT is fundamental in local control.

In general, breast CS is chemo-resistant. Anthracycline-based regimen is superior to cyclophosphamide, methotrexate and fluorouracil (CMF) for anthracycline is effective in sarcoma.[8] Despite the popularity of paclitaxel in the chemotherapy of breast cancer, paclitaxel failed to bring a good response in the neoadjuvant chemotherapy of breast CS by previous reports.[16],[17] Anthracycline-taxane based chemotherapy showed less efficacy than their breast adenocarcinoma counterpart.[18] In the contrast, Hennessy et al. reported that sarcoma-type adjuvant chemotherapy contributed to relapse-free survival despite stable disease or poorer response in the neoadjuvant setting.[19] The role of chemotherapy with paclitaxel in breast CS needs to be further clarified by large-scaled clinical study in the future. MBC cells have a high frequency of activation or overexpression of components in PI3K/AKT and MARK pathway.[14] PI3K/AKT and MARK pathway inhibitors can resensitize MBC cells to conventional cytotoxic agents.[20] ER or PR expression is rather low in breast CS, thus hormonal treatment can benefit little proportion of patients. Her-2 is seldom expressed while Her-1(EGFR) is frequently expressed in MBC. Study of target therapy to EGFR has been initiated.[4],[21]

Breast CS is a unique clinicopathological entity. Compared with single modality, CMT can guarantee both a favorable local control and survival benefits. Successful published protocols using collaborative clinical trials are helpful for treatment strategy.

 > References Top

Kang Y, Kang S, Li Q, Zheng X. Mixed epithelial and mesenchymal metaplastic carcinoma (carcinosarcoma) of the breast: A case report. Eur J Med Res 2014;19:14.  Back to cited text no. 1
Tseng WH, Martinez SR. Metaplastic breast cancer: To radiate or not to radiate? Ann Surg Oncol 2011;18:94-103.  Back to cited text no. 2
Gogas JG, Kotsianos G, Gogas KG. Sarcoma of the breast. Am Surg 1976;42:812-6.  Back to cited text no. 3
Pai BS, Agarwal S, Srinivasan G, Rao L, Rao AC. Metaplastic carcinoma breast (carcinosarcoma variant). Indian J Surg 2010;72:357-8.  Back to cited text no. 4
Wernert R, Yazbek G, Voisin-Rigaud C, Ducarme G. Six-year follow-up without recurrence after a carcinosarcoma of the breast: Case report. Eur J Gynaecol Oncol 2011;32:226-7.  Back to cited text no. 5
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Esbah O, Turkoz FP, Turker I, Durnali A, Ekinci AS, Bal O, et al. Metaplastic breast carcinoma: Case series and review of the literature. Asian Pac J Cancer Prev 2012;13:4645-9.  Back to cited text no. 7
Varlatzidou A, Mastoraki A, Kefala M, Safioleas P, Sakorafas G, Safioleas M. Carcinosarcoma of the breast: Report of a case and review of the literature. Gynecol Surg 2011;8:201-5.  Back to cited text no. 8
Cakir A, Gönül II, Uluoğlu O. Metaplastic breast carcinomas and their relationship with basal-like phenotype. Turk Patoloji Derg 2012;28:134-41.  Back to cited text no. 9
Aritas Y, Bedirli A, Karahan OI, Okten T, Sakrak O, Ince O. Carcinosarcoma of the breast: Clinicopathologic and radiologic findings in an unusual case. Breast J 2003;9:323-4.  Back to cited text no. 10
Tian W, Xu D. Diagnosis and management of multiple carcinosarcoma of the breast in a young Chinese patient. Breast Care (Basel) 2012;7:147-9.  Back to cited text no. 11
Gogas J, Kouskos E, Markopoulos C, Mantas D, Antonopoulou Z, Kontzoglou K, et al. Carcinosarcoma of the breast: Report of two cases. Eur J Gynecol Oncol 2003;24:93-5.  Back to cited text no. 12
Suspitsin EN, Sokolenko AP, Voskresenskiy DA, Ivantsov AO, Shelehova KV, Klimashevskiy VF, et al. Mixed epithelial/mesenchymal metaplastic carcinoma (carcinosarcoma) of the breast in BRCA1 carrier. Breast Cancer 2011;18:137-40.  Back to cited text no. 13
Tampaki EC, Tampakis A, Agrogiannis G, Kavantzas N, Kontzoglou K, Kouraklis G. A case of positive mixed epithelial/mesenchymal metaplastic breast carcinoma (Carcinosarcoma). towards novel therapeutic targets: Case report. Onkologie 2013;36:506-9.  Back to cited text no. 14
Foscini MP, Dina RE, Eusebi V. Sarcomatoid neoplasms of the breast: Proposed definitions for biphasic and monophasic sarcomatoid mammary carcinomas. Semin Diagn Pathol 1993;10:128-36.  Back to cited text no. 15
Cornette J, Tjalma WA, Buytaert P. Biphasic sarcomatoid carcinoma or carcinosarcoma of the breast: Prognosis and therapy. Eur J Gynaecol Oncol 2005;26:514-6.  Back to cited text no. 16
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Buzdar AU, Valero V, Theriault RL, Frye D, Green M, Booser D, et al. Pathological complete response to chemotherapy is related to hormone receptor status. Breast Cancer Res Treat 2003;85:2.  Back to cited text no. 18
Hennessy BT, Giordano S, Broglio K, Duan Z, Trent J, Buchholz TA, et al. Biphasic metaplastic sarcomatoid carcinoma of the breast. Ann Oncol 2006;17:605-13.  Back to cited text no. 19
Hennessy BT, Smith DL, Ram PT, Lu Y, Mills GB. Exploiting the PI3K/AKT pathway for cancer drug discovery. Nat Rev Drug Discov 2005;4:988-1004.  Back to cited text no. 20
Leibl S, Moinfar F. Metaplastic breast carcinomas are negative for Her-2 but frequently express EGFR (Her-1): Potential relevance to adjuvant treatment with EGFR tyrosine kinase inhibitors? J Clin Pathol 2005;58:700-4.  Back to cited text no. 21


  [Figure 1], [Figure 2]


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