Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 

Ahead of print publication  

Mammary not otherwise specified-type sarcoma with CD10 expression

1 Department of Pathology, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, India
2 Department of Surgery, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, India

Date of Submission18-Mar-2021
Date of Decision24-May-2021
Date of Acceptance06-Jun-2021
Date of Web Publication11-Jan-2022

Correspondence Address:
Nageswar Sahu,
Department of Pathology, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar -751 024, Odisha
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.jcrt_459_21

 > Abstract 

Although mammary carcinoma is one of the most common malignancies among women, sarcoma taking origin from the breast tissue is extremely rare. Most of the mammary sarcomas represent a specific entity such as malignant phyllodes tumor, liposarcoma, or angiosarcoma. However, some cases do not fit into any specific category of sarcoma. These cases are diagnosed with breast sarcoma–not otherwise specified (NOS) type. They constantly express CD10 and are called as NOS type sarcoma with CD10 expression. Herein, we report a case of primary mammary sarcoma-NOS type with CD10 expression in an 80-year-old male. It was misdiagnosed with carcinoma breast on fine-needle aspiration. However, on histology, it was a high-grade tumor without any specific differentiation. Immunohistochemical results showed diffuse strong expression of vimentin and CD10, whereas pancytokeratin, desmin, and CD34 were negative. These tumors are considered a variant of sarcoma with myoepithelial differentiation.

Keywords: CD10, mammary sarcoma, not otherwise specified

How to cite this URL:
Sucharita S, Sahu N, Giri R, Sahu SK. Mammary not otherwise specified-type sarcoma with CD10 expression. J Can Res Ther [Epub ahead of print] [cited 2022 Dec 4]. Available from: https://www.cancerjournal.net/preprintarticle.asp?id=335483

 > Introduction Top

Primary breast sarcoma excluding malignant phyllodes tumor (MPT) is extremely rare constituting <1% of all breast malignancies and <5% of all soft-tissue sarcomas with an annual incidence of 4.6 cases per 1,000,000 women.[1],[2],[3] Most of them are usually angiosarcoma or liposarcoma.[4],[5] Primary breast sarcoma without specific differentiation on morphology and immunohistochemistry are called as not otherwise specified (NOS) type mammary sarcoma.[6] This type of sarcoma is extremely rare. Most of these cases were previously diagnosed with pleomorphic sarcoma or malignant fibrous histiocytoma.[4],[7] It can be easily confused with mammary adenocarcinoma both clinically, radiologically, or even on histological examination. Largest series of NOS type mammary sarcoma with seven cases was reported by Leibl and Moinfar.[8] Most significant feature in that series was consistent expression of CD10, hence considered as a variant with myoepithelial differentiation.

 > Case Report Top

An 80-year-old male presented to the surgical outpatient department with a painless mass in the right mammary region for 20 days. On examination, the mass was of 6 cm size, well circumscribed, and firm. The corresponding skin surface was normal. One right axillary lymph node was palpable. Fine-needle aspiration cytology was done from both the breast mass and axillary lymph node. Smears from breast mass were cellular showing mostly dyscohesive clusters of pleomorphic tumor cells. A cytodiagnosis of ductal carcinoma of the breast was made. Smears from the lymph node showed the features of reactive hyperplasia. Positron emission tomography scan showed a hypermetabolic heterogeneously enhancing lobulated mass in the right breast indicative of primary malignancy. Right axillary lymph nodes were looking benign. No other tumor foci were detected. Modified radical mastectomy was done, and the specimen was sent for histopathological study. Slicing of the breast tissue showed a well-circumscribed solid, soft, homogenous tumor of size 7.5 cm × 5 cm × 4.2 cm close to deep surgical surface [Figure 1]. Microscopy revealed a well-circumscribed tumor arranged in diffuse sheets and nests in a myxoid inflammatory background [Figure 2]. Cells were highly pleomorphic, round to oval to elongated with hyperchromatic nuclei, prominent nucleoli and pale eosinophilic cytoplasm. Tumor giant cells were seen. Mitotic count was high with atypical form [Figure 3]. Epithelium lined clefts or glandular components were not seen. Any specific lineage of mesenchymal differentiation was not appreciated. Differential diagnoses of metaplastic carcinoma and high-grade pleomorphic sarcoma were made. Extensive sampling failed to reveal any epithelial component. Adjacent muscle tissue was free of tumor. Lymph nodes were also free of tumor metastasis. On immunohistochemistry, tumor cells showed diffuse strong positivity for vimentin and CD10 [Figure 4] and [Figure 5], but were negative for panCK, CD34, and desmin [Figure 6]. A final diagnosis of primary mammary NOS type sarcoma with CD10 expression (NSCD10) was made.
Figure 1: A well-circumscribed tumor close to the deep surgical surface-Gross

Click here to view
Figure 2: Well-circumscribed tumor with tumor cells in sheets-(H and E, ×40)

Click here to view
Figure 3: Highly pleomorphic cells and numerous mitosis including atypical form (H and E, ×400)

Click here to view
Figure 4: Tumor cells showing immunoreactivity to Vimentin (×400)

Click here to view
Figure 5: Tumor cells showing immunoreactivity to CD10 (×400)

Click here to view
Figure 6: Tumor cells are negative for (a) pan cytokeratin, (b) desmin and (c) CD34 (×400)

Click here to view

 > Discussion Top

Primary mammary sarcomas those arise from mammary mesenchymal tissue are a rare heterogeneous group of neoplasm.[2],[3],[4],[7] Most of the previously diagnosed cases of primary mammary sarcomas were later proven to be sarcomatoid metaplastic carcinoma on immunohistochemistry.[4] It is one of the important differential diagnosis because of its potential for lymphatic spread and thus the need of axillary lymph node dissection.[5],[8] We also thought of diagnosis of sarcomatoid metaplastic carcinoma. However, the absence of sheet like or acinar arrangement and tumor cell negative for pancytokeratin excludes the diagnosis of carcinoma. CD10 positivity is not helpful in excluding metaplastic carcinoma because 80% of them are positive for this immunomarker.[8]

Existing literature shows only small retrospective cases series and few cases reports of NSCD10 type of sarcoma. Leibl and Moinfar reported seven cases of mammary sarcoma those lacking any specific differentiation. These tumors composed of highly pleomorphic spindle cells with numerous mitosis in a collagenous or myxoid matrix. All of these cases were positive for CD10 hence diagnosed with NSCD10.[5],[8] Later on, few more cases of NSCD10 were reported having similar IHC pattern and fibroblast like ultrastructural features.[3],[4],[5] This entity was considered as a variant of sarcoma with myoepithelial differentiation.[5],[8] The histological picture and immunohistochemical pattern in the present case also fit to the diagnosis of NSCD10. MPT usually shows leaf like biphasic structure, although some cases with stromal overgrowth may not show any epithelial component. In these situations, CD34 is helpful as phyllodes tumors are frequently positive for this marker.[4],[8] The absence of epithelial component after extensive grossing and negativity for CD34 in our cases excluded the diagnosis of MPT. Males constitute only 1.0%–3.8% of all mammary sarcoma cases.[9],[10] However, our review of literature could not find a single case of NSCD10 type sarcoma affecting male. It was commonly seen in females with a wide age range from 23 to 88 years.[3],[4],[5],[7],[8]

One of the striking features of NSCD10 is the constant expression of CD10. It is a cell surface neutral endopeptidase, a member of metalloproteinase family which explains its role of facilitating malignant potential of a tumor. In the breast, it is used as a myoepithelial cell marker.[4],[5] CD10 has a role in the diagnosis and prognosis of phyllodes tumor. Increased expression of CD10 in mammary fibroepithelial lesion is associated with the ability to metastasize.[8] Among all types of fibroepithelial lesions of breast, CD10 expression is highest in MPT; positivity in around 20% of tumor cells with strong staining intensity. NSCD10 may rank the top of phyllodes tumor if they were seen as a continuous spectrum since there are increasing trend in CD10 expression with malignancy grade in fibroepithelial neoplasms.[4] Hence, NSCD10 type of sarcoma should be brought under differential diagnoses of primary malignant breast tumors not showing any specific differentiation and CD10 should be added to the immunohistochemical panel.

The origin of NSCD10 still remains unknown. The immunophenotype of this sarcoma with constant CD10 expression suggests that they represent a rare variant of mammary sarcoma with myoepithelial features, but the exact histogenesis remains unknown.[3],[4],[5],[8] Further molecular studies should be performed to clarify the origin of this tumor due to its close relationship with metaplastic carcinoma and phyllodes tumor.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

 > References Top

Adem C, Reynolds C, Ingle JN, Nascimento AG. Primary breast sarcoma: Clinicopathologic series from the Mayo Clinic and review of the literature. Br J Cancer 2004;91:237-41.  Back to cited text no. 1
Vergel JC, Osorio AM, Garcia Mora M, García Angulo á, Guzmán Abisaab L, Cervera-Bonilla S, et al. Breast sarcomas: Experience of a reference center in Colombia. Cureus 2019;11:e5078.  Back to cited text no. 2
Shruti S, Gupta P, Malik A, Bhatnagar A, Siraj F. Cluster of differentiation 10 positive stromal sarcoma of breast: A diagnostic challenge. Cureus 2019;11:e5143.  Back to cited text no. 3
Yang GZ, Li J, Jin H, Ding HY. Is mammary not otherwise specified-type sarcoma with CD10 expression a distinct entity? A rare case report with immunohistochemical and ultrastructural study. Diagn Pathol 2013;8:14.  Back to cited text no. 4
Hasbay B, Bolat FA, Aslan H, Aytaç HÖ. Not otherwise specified-type sarcoma of breast with CD10 expression: Case report. Eur J Breast Health 2019;15:268-71.  Back to cited text no. 5
Moinfar F. Mesenchymal Lesions/Tumors. In: Moinfar F, editor. Essentials of Diagnostic Breast Pathology: A Practical Approach. Berlin, Heidelberg: Springer; 2007. p. 389-90.  Back to cited text no. 6
Nemri SN, Haider N, Fatima S. Mammary sarcoma-Not otherwise specified: A case report. Middle East J Cancer 2016;7:169-72.  Back to cited text no. 7
Leibl S, Moinfar F. Mammary NOS-type sarcoma with CD10 expression: A rare entity with features of myoepithelial differentiation. Am J Surg Pathol 2006;30:450-6.  Back to cited text no. 8
Karlsson F, Granath F, Smedby KE, Zedenius J, Bränström R, Nilsson IL. Sarcoma of the breast: Breast cancer history as etiologic and prognostic factor-A population-based case-control study. Breast Cancer Res Treat 2020;183:669-75.  Back to cited text no. 9
Lee JS, Yoon K, Onyshchenko M. Sarcoma of the Breast: Clinical Characteristics and Outcomes of 991 Patients from the National Cancer Database. Sarcoma 2021:8828158. doi: 10.1155/2021/8828158. PMID: 33542674; PMCID: PMC7843167.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]


     Search Pubmed for
    -  Sucharita S
    -  Sahu N
    -  Giri R
    -  Sahu SK
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  >Abstract>Introduction>Case Report>Discussion>Article Figures
  In this article

 Article Access Statistics
    PDF Downloaded24    

Recommend this journal