Ewing’s Sarcoma: An Uncommon Breast Tumor

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.

Ewing’s Sarcoma of the Breast in a Young Woman: A Case Report and Review of the Literature

“…Nonetheless, imaging characteristics are non-specific and they rarely provide a definitive diagnosis. The first level breast imaging (mammography and ultrasound) has features not specific to breast Ewing’s sarcoma/pPNET and they can range from hypoechoic masses with posterior enhancement to heterogeneous masses with areas of necrosis ( 8 ). Fluorodeoxyglucose-positron emission tomography (FDG-PET) imaging and computed tomography (CT) scanning are used for detecting metastasis.…”

“…Table 1 summarizes the clinical features and the therapeutic course of primary and metastatic Ewing’s sarcomas/pPNET previously published, all presenting as a breast mass, including our case. In some cases, the mass was initially mistaken for a different type of disease ( 25 ), sometimes with a benign lump ( 8 , 15 ). Maxwell et al.…”

“…Table 1 summarizes the clinical features and the therapeutic course of primary and metastatic Ewing's sarcomas/pPNET previously published, all presenting as a breast mass, including our case. In some cases, the mass was initially mistaken for a different type of disease (25), sometimes with a benign lump (8,15). Maxwell et al (15) reported a case of a 14 mm breast lump presenting as well delimited and palpable, superficial, oval, isodense, and hypoechoic, that was initially considered for follow-up.…”

“…The mass was revealed to be a pPNET of the breast on the surgical specimen. Meddeb et al (8) described a case of a 30 mm lump mistaken at the core needle biopsy with fibrocystic dystrophy. The patient was scheduled to follow up, but the mass increased in a month, up to 130 mm.…”

Ewing sarcoma: A pictorial review of typical and atypical locations with reference to the updated 2020 WHO classification system

“…The most common sites are the chest wall, paravertebral region, retroperitoneal space, lower extremities and gluteal region 12 …”