Vaginal Ewing Sarcoma: An Uncommon Clinical Entity in Pediatric Patients

Abstract

Ewing sarcoma, including classical Ewing sarcoma of the bone and primitive neuroectodermal tumors arising in bone or extraosseous primary sites, is a highly aggressive childhood neoplasm. We present two cases of Ewing sarcoma arising from the vagina in young girls. Previously reported cases in literature focused on their pathologic rather than radiographic features. We describe the spectrum of multimodality imaging appearances of Ewing sarcoma at this unusual primary site. Awareness of vaginal Ewing tumors may facilitate prompt diagnosis and lead to a different surgical approach than the more commonly encountered vaginal rhabdomyosarcoma.

Primary vaginal Ewing sarcoma with uterine fibroid

“…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. [ 4 , 21 ] As to the present case, the patient was treated by hysterectomy, bilateral salpingo-oophorectomy and tumors excision, with the subsequent treatment of chemotherapy. The patient had remained free of local recurrence and metastasis for >1-year.…”

“…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.…”

“…[ 11 ] Pathologically, these tumors are often well demarcated with early involvement of adjacent soft tissues, in which necrosis and hemorrhage are common. [ 4 ] As hormone-dependent tumor, uterine fibroids are benign tumors of smooth muscle cells separated by fibrous tissue. [ 12 ] Up to 20% to 50% of uterine fibroids are symptomatic with symptoms, depending on the size, location, and degenerations.…”

“…On MRI examination, the tumors may be T2 isointense to hyperintense, T1 isointense to muscle, with heterogeneous contrast enhancement, while the tumor could demonstrate a heterogeneous ultrasonic appearance with few Doppler color flow. [ 4 ] During ultrasound examination, fibroids usually appear as well-defined, solid, concentric, hypoechoic masses, with a variable amount of acoustic shadowing. [ 7 ] MRI and computer tomography might be helpful in the diagnosis of uterine fibroid.…”

“…[ 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.…”