Received: January 3, 2016; Accepted: February 27, 2016

AbstractMammary sarcomas are a heterogeneous group of malignant neoplasms that arise

from the mammary stroma. Primary sarcomas of the breast are extremely rare andcomprise less than 0.1% of all malignant tumors of the breast. We present the case ofa 56-year-old female diagnosed as stromal sarcoma, not otherwise specified - a veryrare entity.

Keywords: Breast sarcoma, Undifferentiated

IntroductionMammary sarcoma or stromal

sarcoma arises from interlobularmesenchymal elements whichcomprise the supporting mammarystroma. Mammary sarcomas mostlyshow features of liposarcomas orangiosarcomas and rarelypleomorphic sarcoma or malignantfibrous histiocytoma. Undifferenti-ated mammary sarcoma or mammarysarcoma - not otherwise specified(NOS) is an extremely rare findingand diagnosis is made after exclusionof all other malignant spindle celltumors in the breast.1

Case ReportA middle aged female presented

with a slowly growing mass in the left

breast of one year duration. Onexamination it was firm to hard inconsistency in the inner quadrant andmeasured 6×5×3 cm with a skinnodule size of 3×2×1 cm. Biopsyshowed slender to plump spindlecells arranged in sheets and fascicleswith nuclear pleomorphism andabundant mitoses (4-6/HPF) withvarying amounts of collagen (Figures1, 2). A diagnosis of mammarysarcoma was made. She underwent amodified radical mastectomy and thespecimen was solid, greyish whitein color. The specimen was sectionedwidely to search for a glandularepithelial component which wasabsent. Immunostaining wasperformed using pan cytokeratin(panCK), vimentin, CD34, S100,BCL2, estrogen receptor (ER),

♦Corresponding Author: Sohaila Fatima, MDDepartment of Pathology, KingKhalid University, Abha, KSATel: +966-502184094

Email: sohailafatima@gmail.com

Case ReportMiddle East Journal of Cancer; July 2016; 7(3): 169-172

Mammary Sarcoma - Not OtherwiseSpecified: A Case Report

Sabah Nayef Nemri*, Nazima Haider**, Sohaila Fatima**♦

*Department of Laboratory Medicine, Aseer Central Hospital, Abha, Kingdom of Saudi Arabia

**Department of Pathology, King Khalid University, Abha, Kingdom of Saudi Arabia

Sabah Nayef Nemri et al.

progesterone receptor (PR), Ki 67, smooth muscleactin (SMA), desmin, vimentin, and leukocytecommon antigen (LCA). The tumor was stronglypositive for vimentin (Figure 3) and negative forall other markers (Figure 4). CD10 was performedlater which was moderately positive (Figure 5). Adiagnosis of mammary sarcoma - NOS was made.For the past six months, the patient has been seenfor follow up visits without any evidence ofrecurrence. The patient remains on a follow upschedule.

DiscussionPrimary sarcomas of the breast that arise from

mesenchymal tissue of mammary glands are veryrare. The term stromal sarcoma includes allsarcomas other than cystosarcoma phyllodes.2Usually stromal sarcomas are subclassifiedaccording to the histological description of the cellof origin. Some of the subtypes reported as caseseries and case reports are malignant fibroushistiocytoma (MFH), fibrous sarcoma,angiosarcoma, spindle cell sarcoma,leiomyosarcoma, liposarcoma, rhabdomyosarco-ma, osteosarcoma, chondrosarcoma, and synovialsarcoma.1

Primary sarcoma of the breast with no specificdifferentiation based on morphology or immuno-histochemistry has been described as an NOStype of mammary sarcoma.2 This is a very uniqueentity, not much has been described in the

literature. Liebl and Moinfar described thissarcoma in 2006.3 There were seven cases ofmammary sarcoma which did not fit into anyspecific soft tissue category. Histologically thesewere composed of spindle cells with pleomorphicnuclei and abundant mitosis with collagenousstroma. A similar case of undifferentiatedmammary sarcoma has been reported in a youngfemale which was a recurrence of a benignphyllodes tumor.4

The diagnosis of stromal sarcoma NOS typeshould be made after taking into consideration allother tumors with similar histological features.Sarcomatous overgrowth in a high grade phyllodestumor, metaplastic carcinoma, fibrosarcoma,leiomyosarcoma, and MFH of the breast are to beruled out completely. Dermatofibrosarcomaprotuberans, fibromatosis, and myoepithelialcarcinoma of the breast are other important tumorsfor differential diagnoses.1,2 A thorough, adequatesampling and immunohistochemistry is needed tocarefully exclude these differentials.2,3 Tumorcells of mammary sarcoma-NOS type are negativefor most the immune markers. These includeseveral epithelial markers such as pancytoker-atins, CK 5/6, CK 14, CK17, CK 34βE12, CD34,SMA, desmin, h caldesmon, ER, and PR.2,3

Vimentin is strongly positive in all cases ofmammary sarcoma-NOS. Striking immunefeatures of these tumors are their positivity forCD10 and epidermal growth factor receptor

Middle East J Cancer 2016; 7(3): 169-172170

Figure 1. Tumor cells in sheets and fascicles under the skin in thebreast. (Hematoxylin and eosin, 10×).

Figure 2. Spindle shaped tumor cells with nuclear pleomorphism,mitotic activity, and interspersed collagen. (Hematoxylin andeosin, 40×)

Mammary Sarcoma- Not Otherwise Specified

(EGFR-1). CD29, SMA, p63, and calponin mayshow focal positivity in a few cases.2,3

In the breast, CD10 or the common acutelymphoblastic leukemia antigen (CALLA), a cellsurface neutral endopeptidase and member of themetalloprotease family is known as a marker formyoepithelial cells.5,6 Moderate to strongexpression of CD10 is also seen in cystosarcomaphyllodes.7 Malignant phyllodes tumors consist ofa predominant mesenchymal component and abenign epithelial component. The mesenchymalcomponent simulates pleomorphic sarcoma whichrequires an extended sampling to demonstratethe presence of ductal involvement.

Immunochemistry usually points out thepositivity of stromal cells for vimentin, actin,CD34 and Bcl-2.7,8 Myoepithelial lesions are alsopositive for SMA, S100, CK 5/6 and highmolecular weight keratin.2 Metaplastic carcinomais biphasic and contains both a carcinomatouscomponent and heterogeneous sarcomatouscomponent which show co-expression of S100,vimentin and cytokeratin.1,2 Fibrosarcoma is ahighly cellular spindle cell tumor that displays aninterdigitating fasciculated herringbone pattern.Leiomyosarcomas exhibit similar morphologicalfeatures but are usually positive for desmin, SMA,and h-caldesmon.1 MFH of the breast is extremelyrare and most cases of primary MFH in the breastmay actually represent undifferentiated sarcoma

of the breast that cannot be further classified.MFH is composed of pleomorphic spindle cellswith abundant mitosis and inflammatory cellsalong with histiocyte-like cells. Cells in MFHmay be actin, desmin, factor VIII, α1antitrypsin,and lysozyme positive.2 Dermatofibrosarcomaprotuberans is composed of relativelymonomorphic spindle cells that form a storiformpattern around the sweat glands of the deep dermisand subcutaneous fat vacuoles. The tumor cellsshow strong immunoreactivity for CD34.1

Fibromatosis consists of uniform, plumpproliferating spindle cells with low mitosis withvarying amounts of collagenization and focalmyxoid areas.1,2

Middle East J Cancer 2016; 7(3): 169-172 171

Figure 3. Immunohistochemical study showing positivity forvimentin in malignant cells. (Vimentin immunostain, 20×)

Figure 4. Immunohistochemical study of: A) smooth muscle actin(SMA), B) pan cytokeratin (panCK), C) CD34, and D) estrogenreceptor (ER) that indicated negativity in the malignant cells.(10×)

Figure 5. Immunohistochemical study of CD10 showing moderatepositivity for malignant cells. (CD10 immunostain, 40×).

Sabah Nayef Nemri et al.

Breast sarcomas differ totally from epithelialbreast cancers and require different management.The first-line treatment is surgical excision withadequate margins, with small survival advantagesshown for post-operative radiotherapy.9 HighEGFR-1 expression in these undifferentiatedsarcomas can be evaluated further for moleculartarget therapy. The prognosis of mammarysarcoma is difficult to determine because of thesmall number of reported cases. However a studyhas associated CD10 expression in stromal cellsin breast carcinoma with poor prognosis, estrogenreceptor negativity and high grade tumor.10

Conflict of InterestNo conflict of interest is declared.

References1. Rosen, PP. Sarcoma. In: Rosen, PP, editor. Rosen's

breast pathology. 3rd ed. Philadelphia: LippincottWilliams & Wilkins; 2009. p. 902-959.

2. Moinfar, F. Mesenchymal Lesions/Tumors. In: Moinfar,F, editor. Essentials of diagnostic breast pathology: apractical approach. Springer Verlag Berlin Heidelberg:2007. p. 379-390.

3. Leibl S, Moinfar F. Mammary NOS-type sarcomawith CD10 expression: a rare entity with features ofmyoepithelial differentiation. Am J Surg Pathol.2006;30(4):450-6.

4. Varma K, Gupta P, Das P, Singh P, Misra V. CD10positive recurrent undifferentiated mammary sarcomain a young female: A rare case report with brief reviewof literature. Rare Tumors. 2015;7(2):5737.

5. Moritani S, Kushima R, Sugihara H, Bamba M,Kobayashi TK, Hattori T. Availability of CD10immunohistochemistry as a marker of breastmyoepithelial cells on paraffin sections. Mod Pathol.2002;15(4):397-405.

6. Iwaya K, Ogawa H, Izumi M, Kuroda M, Mukai K.Stromal expression of CD10 in invasive breastcarcinoma: a new predictor of clinical outcome.Virchows Arch. 2002;440(6):589-93.

7. Tsai WC, Jin JS, Yu JC, Sheu LF. CD10, actin, andvimentin expression in breast phyllodes tumorscorrelates with tumor grades of the WHO gradingsystem. Int J Surg Pathol. 2006;14(2):127-31.

8. Moore T, Lee AH. Expression of CD34 and bcl-2 inphyllodes tumours, fibroadenomas and spindle celllesions of the breast. Histopathology. 2001;38(1):62-7.

9. Al-Benna S, Poggemann K, Steinau HU, SteinstraesserL. Diagnosis and management of primary breast

sarcoma. Breast Cancer Res Treat. 2010;122(3):619-26.

10. Desmedt C, Majjaj S, Kheddoumi N, Singhal SK,Haibe-Kains B, El Ouriaghli F, et al. Characterizationand clinical evaluation of CD10+ stroma cells in thebreast cancer microenvironment. Clin Cancer Res.2012;18(4):1004-14.

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