Spindle cell (sarcomatoid) carcinoma of the breast: A clinicopathologic and immunohistochemical analysis of 29 cases

Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA 02115, USA.
American Journal of Surgical Pathology (Impact Factor: 4.59). 04/2006; 30(3):300-9. DOI: 10.1097/01.pas.0000184809.27735.a1
Source: PubMed

ABSTRACT Spindle cell (sarcomatoid) carcinoma of the breast is a rare variant of breast cancer that has been classified under the broad rubric of metaplastic carcinoma. Because the term "metaplastic carcinoma" comprises a heterogeneous group of tumors, it has been difficult to reliably predict biologic potential or to determine optimal therapy. To better characterize the spindle cell subset of metaplastic breast carcinomas, we reviewed 29 cases. All patients were adult females ranging from 40 to 96 years of age (median, 68 years). Tumor size ranged from 1.5 to 15 cm (median, 4 cm). Treatment was by excision and/or mastectomy with axillary node evaluation in most cases, often combined with postoperative radiation and/or chemotherapy. All cases were clinically of breast origin, showed >or=80% spindled/sarcomatoid morphology, and demonstrated keratin positivity and/or close association with ductal carcinoma in situ. Immunohistochemical studies showed evidence suggesting myoepithelial differentiation as exhibited by immunoreactivity for smooth muscle actin, cytokeratin 14, and p63 in a subset of cases (39%). Twenty-seven cases exhibited pure spindled or sarcomatoid morphology of variable appearance and nuclear grade, whereas 2 contained high-grade invasive ductal carcinoma comprising <or=20% of the tumor mass. Two cases exhibited heterologous elements (1 rhabdomyosarcoma and 1 with both chondrosarcoma and osteosarcoma) and 4 were associated with ductal carcinoma in situ. Follow-up data were available on 24 of 29 patients (range, 1-120 months; median, 20 months). Of 20 cases in which axillary nodes were biopsied, definitive nodal metastases were identified in only 1 (5%), and this was in a case with a significant component of invasive ductal carcinoma. Three patients developed local recurrences. Extranodal metastases occurred in 11 of 24 patients (46%), most commonly to the lungs. Ten of 24 patients (42%) died of disease at a median interval of 11.5 months (range, 1-46 months) and 3 patients were alive with metastatic disease. Eight patients were alive with no evidence of recurrent or metastatic disease (median, 29.5 months). Based on this series, spindle cell/sarcomatoid carcinoma of the breast is a highly aggressive neoplasm with a high rate of extranodal metastases. Purely spindled/sarcomatoid tumors have a significantly lower rate of nodal metastases than conventional ductal and lobular breast carcinomas.

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    ABSTRACT: SUMMARY Introduction: Metaplastic breast carcinoma is charac- terized by the direct transition from an invasive duct carci- noma to metaplastic or/and sarcomatoid elements. As the epithelial component can be minimal or even absent, it may be difficult to differentiate it from sarcomatoid tumours. In these cases, immunohistochemical markers such as p63 and CD99 can be useful. We study the expression of these markers in a case of metaplastic carcinoma of matrix pro- ducing variant and make a thorough immunohistochemical characterization of the epithelial and metaplastic compo- nent. Material and method: The case of an 85-year-old woman with a 4,7-cm retroareolar mass is presented. Grossly, the specimen revealed a soft, whitish, lobulated mass of gelatinous appearance which microscopically exhi- bited multiple cellular patterns. Several foci of invasive duct carcinoma were observed although the tumour was composed predominantly of fascicles and chords of small round cells dispersed in a basophilic and myxoid stroma. Results: Immunohistochemical study revealed that the entire tumour stained positively with keratin AE1/AE3, p63, CD99, EGFR, EMA S-100 and progesterone. In addi- tion, the epithelial component exhibited keratin CAM 5.2 and e-cadherine. The metaplastic component was positive with keratin 5/6, keratin 14, high molecular weight keratin (34‚E12), actin and vimentin. Oestrogen receptors and Her2/neu were negative. It is a metaplastic carcinoma of matrix producing variant. Discussion: The metaplastic car- cinoma of matrix producing variant is positive for CD99 and it is the only subtype which expresses it. Therefore, it can be diagnostically useful, both in primary tumours and metastases. In this case, p63 expression is valuable in the differential diagnosis with other sarcomas and mixed tumours of the breast (fillodes tumour). This matrix-produ- cing carcinoma shows a myoepithelial or basal phenotype in the metaplastic component and exhibits immunohisto- RESUMEN Introducción: El carcinoma metaplásico de mama se caracteriza por la transición directa de un carcinoma ductal infiltrante a elementos metaplásicos y/o sarcomatoides. El componente epitelial puede ser escaso o estar ausente y entonces su diferenciación frente a otros tumores de hábito sarcomatoide resulta difícil. La utilización de marcadores inmunohistoquímicos como el p63 y CD99 puede ser eficaz. Estudiamos la expresión de estos marcadores en un caso de carcinoma metaplásico variante productor de matriz (CMPM), haciendo una descripción detallada del compo- nente epitelial y metaplásico. Material y método: Presenta- mos el caso de una mujer de 85 años que presenta una gran masa de 4,7 cm en localización retroareolar. Macroscópica- mente, se trata de una masa blanquecina, de aspecto gelati- noso, blando de contorno lobulado, que corresponde a un tumor con múltiples patrones celulares. Muestra varios focos de carcinoma ductal infiltrante, aunque el componente más abundante es de células pequeñas redondeadas con tenden- cia a la formación de hileras o cordones inmersos en un estroma basófilo y mixoide. Resultados: El estudio inmu- nohistoquímico muestra que la totalidad del tumor expresa queratina AE1/AE3, p63, CD99, EGFR, EMA, S-100 y pro- gesterona. El componente epitelial expresa además CAM 5.2 y e-caderina. El componente metaplásico resulta positivo para queratina 5/6, queratina 14, queratina de alto peso (34‚E12), actina y vimentina. Los receptores de estrógeno y Her2/neu son negativos. Se trata de un carcinoma metaplási- co variante «productor de matriz» (CMPM). Conclusiones: El CMPM resulta positivo para CD99, y es el único subtipo de carcinoma de mama que lo expresa. Por ello se constitu- ye en un marcador útil para su diagnóstico tanto en el tumor primario como en las metástasis. La expresión de p63 en nuestro caso, resulta útil para su diferenciación frente a otros sarcomas o tumores mixtos de mama (fillodes). Este CMPM muestra un fenotipo mioepitelial o basal en el componente
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    ABSTRACT: Metaplastic carcinoma breast is categorized as a rare heterogenous neoplasm generally characterized by a mixture of adenocarcinoma with dominant areas of spindle cell, squamous and/or other mesenchymal differentiation. To determine the epidemio-demographic and histopathologic characteristics of this rare entity a retrospective study was conducted to review all cases at the Aga Khan University Hospital (AKUH) Karachi, received during 1st January 2000 to 31st August 2005. Twenty-four patients were identified with a mean age at diagnosis of 46.4 (+/-SD 3.8) years, and an age range of 28-68 years. The mean tumor size was 7.9 cm, range 2.0-17.0 cms (+/-SD 4.77). The specimens were mostly obtained by modified radical mastectomy(54.2%) followed by biopsy (29.2%), lumpectomy (8.3%), and total mastectomy (4.2%). Skin ulceration was found in 37.5% cases. Component sub-categorization showed 13 (54.2%) cases of infiltrating ductal carcinoma with squamous metaplasia, followed by 2 (8.3 %) cases with heterologous elements, 4 (16.7%) cases with spindle cell component, 2 cases of matrix producing carcinoma and one case of squamous cell carcinoma. The malignancy was high grade, modified Bloom Richardson's grade III (54.2 %) and grade II (12.5%). Such grading was not applicable to 4 cases of spindle cell component and 1 case with extensive chondroid areas. Twelve patients had information available on the nodal status. Five (41.6%) were node-negative, four (33.33%) had 1-3 lymph node involvement positive, and three (12.5%) had more than 3 lymph nodes positive. The median 3 year Event Free Survival (EFS) was 10% and overall survival was 30%. MCB is an aggressive disease with a poor prognosis. This aspect appears bleaker in our population either due to the biological characteristic of the malignancy in a high risk group or the lack of availability and accessibility of health coverage, resulting in a delayed presentation. MCB is an uncommon breast malignancy and due to the lack of sufficiently large studies there is limited knowledge as to the pathogenesis, progress, best treatment protocols and prognosis. Collaborative studies are therefore recommended to allow for better understanding of this intriguing neoplasm.
    Asian Pacific journal of cancer prevention: APJCP 01/2006; 7(4):667-71. · 2.51 Impact Factor
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    ABSTRACT: Breast spindle cell tumours (BSCTs), although rare, represent a heterogeneous group with different treatment modalities. This work was undertaken to evaluate the utility of fine needle aspiration cytology (FNAC), histopathology and immunohistochemistry (IHC) in differentiating BSCTs. FNAC of eight breast masses diagnosed cytologically as BSCTs was followed by wide excision biopsy. IHC using a panel of antibodies against vimentin, pan-cytokeratin, s100, desmin, smooth muscle actin, CD34, and CD10 was evaluated to define their nature. FNAC defined the tumors as benign (n = 4), suspicious (n = 2) and malignant (n = 3), based on the cytopathological criteria of malignancy. Following wide excision biopsy, the tumors were reclassified into benign (n = 5) and malignant (n = 3). In the benign group, the diagnosis was raised histologically and confirmed by IHC for 3 cases (one spindle cell lipoma, one myofibroblastoma and one leiomyoma). For the remaining two cases, the diagnosis was set up after IHC (one fibromatosis and one spindle cell variant of adenomyoepithelioma). In the malignant group, a leiomyosarcoma was diagnosed histologically, while IHC was crucial to set up the diagnosis of one case of spindle cell carcinoma and one malignant myoepithelioma. FNAC in BSCTs is an insufficient tool and should be followed by wide excision biopsy. The latter technique differentiate benign from malignant BSCTs and is able in 50% of the cases to set up the definite diagnosis. IHC is of value to define the nature of different benign lesions and is mandatory in the malignant ones for optimal treatment. Awareness of the different types of BSCTs prevents unnecessary extensive therapeutic regimes.
    Diagnostic Pathology 02/2006; 1(1):13. DOI:10.1186/1746-1596-1-13 · 2.41 Impact Factor
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