Article

Bilateral ewing sarcoma/primitive neuroectodermal tumor of the breast: a very rare entity and review of the literature.

Department of Medical Oncology, National Institute of Oncology, Rabat, Morocco.
Case reports in oncological medicine 01/2013; 2013:964568. DOI: 10.1155/2013/964568
Source: PubMed

ABSTRACT Peripheral primitive neuroectodermal tumors (PNET) are rare malignant tumors, affecting mostly children and adolescents and have been described in breast in eight case reports only. In this paper, we present a case of bilateral mammary ES/PNET where distinction between primary and metastatic diseases was discussed through a literature review. The aim of this work is to demonstrate that although rare, the possibility of PNET should be kept in mind while evaluating a palpable breast abnormality in a young female.

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    ABSTRACT: Background: The peripheral primitive neuroectodermal tumor (pPNET) is a rare malignant tumor originating from neuroectoderm. The accurate diagnosis is essential for the treatment of pPNET. Methods: we performed the largest cases of retrospective analysis thus far to review the unique computed tomography (CT), magnetic resonance imaging (MRI), and clinicopathological features of pPNET. The tumor location, morphological features, signal intensity, contrast enhancement characteristics, and involvement of local soft tissues of 36 pPNETs were assessed. Results: Our results showed that there were more men (25/36) than women pPNETs patients. Unenhanced MRI (16 cases) showed that 14 cases were isointense and 2 cases were hypointense on T1WI. Nine cases were isointense and 7 were hyperintense on T2WI. Most pPNETs had heterogeneous signal intensity with small necrosis (CT: 31/36; MRI: 14/16) as well as heterogeneous enhancement (CT: 34/30; MRI: 15/16). The tumors usually had ill-defined borders and irregular shapes (CT: 30/36; MRI: 15/16). Pathologic exam showed small areas of necrosis in all tumors. Conclusions: The diagnosis of pPNET should be suggested in young men when the imaging depicts a single large ill-defined solid mass with small area of necrosis, especially for those whose images show iso-intense on T1WI and T2WI and have heterogeneous enhancement.
    Oncotarget 12/2014; · 6.63 Impact Factor
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    ABSTRACT: Ewing's sarcoma/primitive neuroectodermal tumors (EWS/PNET) are rare malignant and aggressive tumors, usually seen in the trunk and lower limbs of children and young adults. They are uncommon in the breast. We report a case of a 43-year-old woman who developed a painless breast mass. An initial core needle biopsy concluded to a fibrocystic dystrophy contrasting with a rapidly growing mass; thus a large lumpectomy was done. Diagnosis of primary PNET of the breast was established, based on both histopathological examination and immunohistochemical findings. Surgical margins were positive, therefore, left modified radical mastectomy with axillary lymph nodes dissection was performed. The patient was given 6 cycles of adjuvant chemotherapy containing cyclophosphamide, adriamycin and vincristine. Twenty months later, she is in life without recurrence or metastasis. EWS/PNET may impose a diagnostic challenge. Indeed, mammography and ultrasonography features are non specific. The histopathological pattern is variable depending on the degree of neuroectodermal differentiation. Immuno-phenotyping is necessary and genetic study is the only confirmatory tool of diagnosis showing a characteristic cytogenetic anomaly; t (11; 22) translocation.
    06/2014; 4(2):659. DOI:10.4081/cp.2014.659

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