Extraskeletal Ewing Sarcoma in a Young Patient During Pregnancy

Background: Ewing’s sarcoma is usually identified as a primary malignancy of the bone affecting children and young adults. It is associated with soft tissue extension in 90% of cases. Primarily extraskeletal Ewing’s sarcoma is a rare subtype, and very few data are available concerning optimal treatment modalities. Case Presentation: We report a case of an 18 year-old female from Cabo Verde in her 34th week of pregnancy presenting with a rapid growing cervical tumor. It had initially started as an indolent swelling at the left cervico-posterolateral region. CT had shown involvement of both superficial and deep cervical fascia. At time of presentation, the histologic diagnosis of a peripheral Primitive Neuroectodermal Tumor/Extraskeletal Ewing’s sarcoma had already been provided. To reduce risk of fetal metastasis, the child was delivered by caesarean section. A chest CT of the patient revealed pulmonary metastasis and compressive atelectasis of the left lung caused by the tumor. Three weeks post partum, she presented with loss of muscular function of the extremities as well as parestesia and bladder dysfunction. A repeat CT confirmed medullar compression at the level of C5-C6, C6-C7 and C7-D1 by the tumor without signs of bone metastasis. 5 sessions of palliative radiotherapy were performed that led to parcial recovery of muscular function of the upper right extremity. Palliative chemotherapy was refused, so that the patient was released from hospital. No metastasis had been detected in the child so far. Conclusion: Extraskeletal Ewing’s sarcoma can manifest as a rapidly growing localized mass causing local compression symptoms. Palliative radiotherapy can achieve local tumor reduction and symptom relief.