Extraosseous Ewing sarcoma of the vagina: a rare entity

Abstract

Ewing sarcoma, a highly malignant neoplasm of the bone, usually occurs during childhood. About 15% are extraosseous. The Ewing family of tumors (EFTs) are extremely rare in the vagina. A 40-year literature review from 1970 to 2010 revealed only nine cases. A 32-year-old woman presented with a painless vaginal mass. A wide excision was performed. Histopathology, immunohistochemistry and molecular studies confirmed extraosseous vaginal Ewing sarcoma. Despite aggressive chemotherapy with a good initial response, she developed local recurrence and metastasis to the spine and pelvis and succumbed 22 months later. A previous infiltrating ductal breast cancer, treated and in remission complicated the picture. We present the tenth case of vaginal Ewing sarcoma and the fourth to be confirmed by molecular studies. We stress the importance of molecular techniques in definitely diagnosing EFTs, especially those arising at unusual sites, particularly in the context of a previous diagnosis of breast cancer.

Primary vaginal Ewing sarcoma with uterine fibroid

“…Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET) are considered as single tumor category known as Ewing family of tumors (EFTs), [ 2 , 8 ] which could be diagnosed in children and adults. [ 1 , 2 , 4 , 9 ] Extra osseous ES mainly affects the paravertebral region, lower extremity, chest wall, retroperitoneum, pelvis, and hip, [ 10 ] while the most common site of metastatic spread is lung. [ 11 ] Pathologically, these tumors are often well demarcated with early involvement of adjacent soft tissues, in which necrosis and hemorrhage are common.…”

“…[ 2 , 20 ] Wide local excision followed by adjuvant chemotherapy and/or radiotherapy is recommended. [ 1 ] The outcome for the tumor is primarily dependent on the presence of distant metastases. In the absence of detectable metastases, 70% of children and adolescents are cured with a combination of intensive, multiagent chemotherapy with surgery and/or radiotherapy.…”

“…As a highly malignant neoplasm of bones, Ewing sarcoma (ES) usually occurs during childhood, and nearly 15% of ES are extraosseous. [ 1 ] Extraosseous Ewing sarcoma (EES) grows rapidly, with round-cell malignancy of uncharacterized mesenchymal cell origin. [ 2 ] Virtually, all Ewing sarcomas share a common chromosomal translocation of the long arm of chromosome 11 and 22.…”

“…[ 3 ] The common translocation and a strong membranous expression of CD99 could unify the diagnosis of extraosseous Ewing sarcoma. [ 4 ] A 48-year literature review from 1970 to 2018 revealed that <30 cases of primary vaginal Ewing's sarcoma had been previously reported, [ 1 , 2 , 4 ] and few literatures had discussed the imaging findings of primary vaginal Ewing sarcoma. In addition, uterine fibroid is the most commonly benign tumor in female pelvis, and occurs in about 20% to 50% of women around the world, from which black women of reproductive age have the highest incidence.…”

Peripheral primitive neuroendocrine tumor of the chest wall—A case report with pathological correlation

“…EO ES remains rare. There have been case reports of EO ES occurring in the kidney [4] , [5] , [6] , GI tract [7] , [8] , [9] , thyroid [10] , [11] , vagina [12] , [13] , [14] , and other more unusual locations such as the orbit [15] , adrenal gland [16] , and tonsil [17] . Fewer are seen in the thoracic region, with 1 reported case in the supraclavicular fossa [18] , and a few seen in the lung [19] , [20] .…”