Primary Extraosseous Ewing Sarcoma of the Upper Limb: Report of a Rare Case

Introduction: Extraosseous Ewing sarcomas (EESs) are rare tumors that originate from soft tissues. Upper extremity EESs account for about 3% of all cases. Here we reported a case of ESS of the upper limb whose management became complicated due to the COVID-19 pandemic. Case Presentation: A 27-year-old female with EES of the right deltoid region presented after 3 months delay when the tumor had reached a huge size. Neoadjuvant therapy was initiated for her with acceptable results, however, her surgical treatment was postponed 3 times due to the cancellation of elective operations in the hospital as well as her involvement with COVID-19 infection. She developed multiple pulmonary metastases shortly after the surgery and passed away within a fortnight due to respiratory complications. Conclusions: Although not “emergent” by definition, surgical treatment of patients with cancer, especially those who suffer from malignancies with high metastatic potential such as Ewing sarcoma (including EES), should not be considered as “elective” since the disease may progress in a short time and become incurable.

Extraosseous Ewing sarcoma presenting as recurrent intergluteal pilonidal disease

Background Pilonidal disease classically presents as an abscess or soft tissue swelling which classically occurs in the intergluteal cleft, just above the anus. Risk factors for this disease include obesity, prolonged sitting, and abundance of gluteal hair. Treatment options are extensive but most often include incision and drainage with antibiotic treatment. Presentation of recurrent intergluteal pilonidal disease in a young, active female would then be unusual. We present a case of a 17-year-old female presenting with what appeared to be intergluteal pilonidal disease. Definitive excision with histopathology revealed a diagnosis of extraosseous Ewing sarcoma. Case presentation An otherwise healthy and active 17-year-old female presented to our Pediatric Oncology clinic with a 2-month history of recurrent painful soft tissue swelling of the intergluteal cleft. At that time, she had been diagnosed with pilonidal abscess and had already undergone three incision and drainage procedures. A definitive excision with pathology was performed within weeks of her initial presentation. Immunohistochemical evaluation confirmed a diagnosis of extraosseous Ewing sarcoma. Conclusion This unusual case underlies the importance of considering a broad differential when evaluating potential pilonidal abscess in a patient who otherwise has no risk factors. Additionally, definitive excision with pathology is critical in a patient with unusually recurrent disease as this can be crucial in the identification of an alternative, and potentially devastating, diagnosis.

Extraosseous Ewing Sarcoma of the Cervical Esophagus: Case Report and Literature Review

This study presents a case of Extraosseous Ewing sarcoma arising in the cervical esophagus of a 36-year-old woman who presented with cervical mass and dysphagia. Imaging studies demonstrated a well-defined, cystic-solid mass in the neck extending into the upper mediastinum, which results in deviation of the trachea and esophagus. The relationship between the tumor and the cervical esophagus was found in the operation. The patient underwent localized tumor resection without radical esophagectomy. The accurate diagnosis was obtained by the immunohistochemistry and molecular genetic assay. Although this is a rare site for this type of tumor to occur, extraosseous Ewing sarcoma should be considered in the differential diagnosis of small round cell tumors of the cervical esophagus. The postoperative chemotherapy and radiotherapy are of great value in improving the prognosis of cervical esophageal Ewing sarcoma.

Multi-modality management of extraosseous Ewing sarcoma: 10-year experience from a tertiary care centre

Aim: To analyse the presentation, diagnosis and patterns of care of extraosseous Ewing sarcoma treated at our institution between 2008 and 2018. Methods: Electronic medical records of extraosseous Ewing sarcoma patients treated at our institution between January 2008 and April 2018 were reviewed. Kaplan–Meier curves were plotted to assess the overall and disease-free survival with 95% confidence intervals. A univariate analysis was carried out to assess the impact of variables such as surgical excision, completeness of surgery, completeness of chemotherapy and addition of radiation therapy on the survivorship. Results: The records of 65 patients treated at our institution were available for review. The mean age was 26·4 years. The most frequent sites of extraosseous Ewing tumour were kidney—9/65 (13·8%) and brain—10/65 (15·4%). Sixteen (24·6%) patients presented with inoperable/metastatic disease at diagnosis. The other 49 (75·4%) had localised disease at presentation. The median overall survival of the 49 non-metastatic patients was 46 months, and the disease-free survival was 45 months. Conclusion: Extraosseous Ewing sarcoma is a rare and aggressive tumour diagnosed by molecular techniques. Multi-modality treatment including surgical resection with wide margins, adjuvant radiation when indicated and completion of systemic chemotherapy results in optimum outcomes.