Extraskeletal and Skeletal Ewing Sarcoma Family of Tumours in Adults: A Clinico Radiological Review of 50 Cases
ثبت نشده
چکیده
Ewing sarcoma (ES) and primitive peripheral neuroectodermal tumor (PNET) were originally described as distinct clinicopathologic entities by James Ewing in 1921 and Stout in 1918 respectively. At present, it is known that these entities comprise the same spectrum of tumours known as the Ewing sarcoma family of tumors (EFT) [1], which also includes malignant round-cell tumor of the thoracopulmonary region (Askin’s tumor), and atypical ES. The EFT can develop in nearly any bone or soft tissue, but the commonest site is in a flat or long bone. The typical patient presents with localized pain and swelling [2]. Although metastatic disease is found in lesser than 25 percent at presentation [3], subclinical metastatic disease is assumed to be present in almost all patients because of the 80 to 90 percent relapse rate in patients undergoing local therapy alone [4]. The diagnostic work-up is usually initiated with a plain radiograph of the affected region. Compared to plain radiographs, a CT scan better delineates the extent of cortical destruction and soft tissue involvement. CT imaging features include a heterogeneous appearing mass with poorly defined irregular margins, displacing surrounding tissues with mixed internal attenuation, similar to muscle with necrosis and/or calcification. However, definition of tumor size, local intraosseous and extraosseous extent, and the relationship of the tumor to fascial planes, vessels, nerves, and organs are best achieved by magnetic resonance imaging (MRI).
منابع مشابه
Ewing sarcoma of the testis: A case report and review of the literature
Solid tumours in the inguinal scrotal area (inguinal, testicular and para-testicular) are infrequent in the clinic. After its observation, depending on factors such as age, time of evolution, metastatic disease and blood markers at diagnosis, clinicians oriented to one origin or other. However, this approach may lead to a wrong diagnosis and treatment approach. In this publication, we report an...
متن کاملCytokeratin immunoreactivity in Ewing sarcoma/ primitive neuroectodermal tumour.
Ewing sarcoma (ES)/ primitive neuroectodermal tumour (PNET) is an aggressive malignant neoplasm affecting mainly children and young adults. The tumour is included with other primitive neoplasms under the category of small round cell tumour. Cytokeratin expression in ES/PNET has been described in sporadic case reports as well as a few systemic series. We studied this feature in Malaysian patient...
متن کاملExtraskeletal Ewing’s Sarcoma of the Little Finger, a rare case
When Ewing’s sarcoma arises in soft tissue rather than bone, it is referred to as extraskeletal Ewing’s sarcoma (ESS). It is generally accepted to be between 15% and 20% of that of Ewing sarcoma of bone. Extraskeletal Ewing sarcoma usually manifests in young patients, with 85% of cases detected between 20 months and 30 years of age. The most commonly reported locations of extraskeletal Ewing sa...
متن کاملExtra-skeletal Ewing's sarcoma in adults: presentation of two cases.
Extraosseous Ewing's sarcoma represents about 5% of the Ewing family of tumours. Two cases in adult patients are presented, emphasizing the complexity of a multi-modality treatment approach of this tumour. Clinical presentation, chemotherapeutical, surgical and radiotherapeutical approaches are discussed. A thorough literature search was done to correlate our therapeutic attitude with current k...
متن کاملSoft tissue and visceral sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment
Extraskeletal Ewing sarcoma is covered by other ESMO Guidelines: in general, the same principles for these tumours in children apply to adults. This is also the case for embryonal and alveolar rhabdomyosarcoma, which are exceedingly rare in adults. On the other hand, pleomorphic rhabdomyosarcoma is viewed as a high-grade adult-type soft tissue sarcoma. Gastrointestinal stromal tumours are cover...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره شماره
صفحات -
تاریخ انتشار 2017