Primary renal ewing sarcoma in a 15-year-old female: Case report

@#Extraskeletal Ewing sarcoma (EES) is a rare tumor that primarily affects children and lacks specific clinical signs. Diagnosis is confirmed through imaging techniques, histology, and molecular diagnostics. Treatment typically involves surgical intervention and chemotherapy. We present the case of a 15-year-old female with a history of abnormal mass enlargement in the right flank area. An initial diagnosis of Wilms tumor was made, and the patient underwent a right open radical nephrectomy. However, the tumor recurred eight months after nephrectomy, necessitating a metastasectomy. Chemotherapy was started to immediately target the tumor recurrence. Next-generation sequencing done on the open radical nephrectomy and metastasectomy samples revealed the presence of the EWSR1-FLI1 fusion gene in both specimens, confirming the final diagnosis to be primary renal Ewing sarcoma. Despite undergoing a right open radical nephrectomy for the primary tumor site, a metastasectomy during tumor recurrence, and chemotherapy, the patient’s prognosis remained poor.

Extraskeletal Ewing sarcoma of thyroid gland: A case report / 中南大学学报(医学版)

Extraskeletal Ewing sarcoma is a rare event, and extraskeletal Ewing sarcoma of the thyroid gland is even rarer. It has non-specific clinical manifestation and difficulty in early diagnosis. The diagnosis mainly depends on histology and immunohistochemistry. It possesses the features of high malignancy, high rate of local recurrence, and distant metastasis. Currently, the aggressive multimodal treatment contains surgery, chemotherapy, and radiotherapy. This study presented a case of extraskeletal Ewing sarcoma arising in the thyroid gland of a 30-year-old woman, who presented with supraclavicular mass and sense of dysphagia obstruction in Department of Otolaryngology, Head and Neck Surgery, Second Xiangya Hospital, Central South University in 2018. Imaging studies demonstrated a cystic-solid mass in inferior of the left leaf of thyroid gland and in the posterior of the trachea and esophagus. The patient underwent localized tumor resection. The pathological diagnosis revealed that it was a small round cell tumor, and the immunohistochemistry results were considered to be extraskeletal Ewing sarcoma. Subsequently, the patient was given chemotherapy and local radiation therapy. There was no evidence of tumor recurrence or metastasis.

Imaging Features of Primary Tumors and Metastatic Patterns of the Extraskeletal Ewing Sarcoma Family of Tumors in Adults: A 17-Year Experience at a Single Institution

OBJECTIVE: To comprehensively analyze the spectrum of imaging features of the primary tumors and metastatic patterns of the Extraskeletal Ewing sarcoma family of tumors (EES) in adults. MATERIALS AND METHODS: We performed a computerized search of our hospital's data-warehouse from 1996 to 2013 using codes for Ewing sarcoma and primitive neuroectodermal tumors as well as the demographic code for > or = 18 years of age. We selected subjects who were histologically confirmed to have Ewing sarcoma of extraskeletal origin. Imaging features of the primary tumor and metastatic disease were evaluated for lesion location, size, enhancement pattern, necrosis, margin, and invasion of adjacent organs. RESULTS: Among the 70 patients (mean age, 35.8 +/- 15.6 years; range, 18-67 years) included in our study, primary tumors of EES occurred in the soft tissue and extremities (n = 20), abdomen and pelvis (n = 18), thorax (n = 14), paravertebral space (n = 8), head and neck (n = 6), and an unknown primary site (n = 4). Most primary tumors manifested as large and bulky soft-tissue masses (mean size, 9.0 cm; range, 1.3-23.0 cm), frequently invading adjacent organs (45.6%) and showed heterogeneous enhancement (73.7%), a well-defined (66.7%) margin, and partial necrosis/cystic degeneration (81.9%). Notably, 29 patients had metastatic disease detected at their initial diagnosis. The most frequent site of metastasis was lymph nodes (75.9%), followed by bone (31.0%), lung (20.7%), abdominal solid organs (13.8%), peritoneum (13.8%), pleura (6.9%), and brain (3.4%). CONCLUSION: Primary tumors of EES can occur anywhere and mostly manifest as large and bulky, soft-tissue masses. Lymph nodes are the most frequent metastasis sites.

Ewing’s Sarcoma of Zygomatic Bone.

Ewing’s sarcoma is an uncommon malignancy usually arising from the skeletal system particularly pelvic bones and long bones of lower extremities and mostly affecting children and young adults. Its occurrence in the maxillofacial region with soft tissue involvement is struck with rarity. It carries a poor prognosis due to its uncontrolled potential for metastatic spread; therefore an early and integrated treatment approach must be adopted to improve patient’s long term survival.

Cutaneous Extraskeletal Ewing's Sarcoma / 대한피부과학회지

Extraskeletal Ewing's sarcoma is an extremely rare malignant soft tissue tumor, which presents the exact same histopathologic features with Ewing's sarcoma of bone. Tumor composed of small, blue, round cells with vesicular nuclei and scant cytoplasm is the characteristic histopathology of the disease. Extraskeletal Ewing's sarcoma is known to occur usually in the deep soft tissues of the extremities. We report a case of 22-year-old boy with solitary extraskeletal Ewing's sarcoma on his back without any evidence of bone involvement. The patient went through wide excision with meticulous lymph node dissection followed by chemotherapy. For the 15 months of follow up, there is not a sign of recurrence or metastasis observed.

Primary Intradural Extraosseous Ewing's Sarcoma

Ewing's sarcoma usually arises from skeletal bone, but rarely may have an extraskeletal origin. However, Ewing's sarcoma that originates around the spinal column, especially, the intradural extramedullary type is extremely rare. We report a rare case of primary intraspinal extraskeletal Ewing's sarcoma.

Multimodal Treatment of Primary Extraskeletal Ewing's Sarcoma of the Chest Wall: Report of 2 Cases / Journal of the Korean Cancer Association, 대한암학회지

Extraskeletal Ewing's sarcoma (EES) is a type of Ewing's sarcoma that arises in soft tissue and is now regarded as a member of a family of small round cell neoplasms of bone and soft tissue, including primitive neuroectodermal tumors (PNETs). EES occurs predominantly in adolescents and young adults between the ages of 10 and 30 years. The disease follows an aggressive course with a high recurrence rate. The presence of a distant metastasis is also common. EES arises in the soft tissue of either the trunk or extremities. We recently experienced two cases of EES that occurred in the chest wall. The two patients underwent wide resection and combined radiochemotherapy. There was no evidence of disease 30 and 22 months, respectively, after surgery. Although extremely rare, EES should be considered in the differential diagnosis of chest wall tumors. We report two cases of EES with a brief review of the literature.

Primary Cutaneous Extraskeletal Ewing's Sarcoma / 대한피부과학회지

Extraskeletal Ewing's sarcoma has been recognized as being histologically indistinguishable from Ewing's sarcoma of bone. Histopathologically, Ewing's sarcoma consists of solid sheets of small round cells with vesicular nuclei and scant cytoplasm, and the cells are arranged in irregular masses separated by strands of fibrous tissue. Extraskeletal Ewing's sarcoma may arise virtually anywhere, but it is most common in the deep soft tissues of the extremities. We report here on a 27-year-old woman with cutaneous extraskeletal Ewing's sarcoma. She presented with a subcutaneous tumor of the right upper arm, and this was without osseous involvement. The patient underwent wide local excision and she received chemotherapy with vincristine, cyclophosphamide, etoposide and ifosfamide. There has been no evidence of recurrence or metastasis during 16 months of follow up.

A Case of Extraskeletal Ewing Sarcoma of Retroperitoneum / 대한소아혈액종양학회지

Ewing sarcoma is an uncommon bone malignancy of childhood. Although Ewing sarcoma is mostly a tumor of bone, it may also arise from soft tissues (extraskeletal Ewing sarcoma). We report a case of extraskeletal Ewing sarcoma that arose in the retroperitoneum of a 18-month-old girl and presented with right leg pain and gait disturbance. A brief review of related literatures was also made.

Extraskeletal Cervical Epidural Ewing's Sarcoma: Case Report and Review of the Literature

A patient with extraskeletal cervical epidural Ewing's sarcoma who presented with cervical radiculopathy is reported. A 58-year-old woman presented with right posterolateral neck pain and upper extremity radiculopathy. The computed tomography myelography showed epidural and paravertebral masses on right side, with widening of the ipsilateral neural foramina at the C2-C3 level. On magnetic resonance images, the mass was isointense to soft tissue on T1-weighted images, hyperintense on T2-weighted images, and showed diffuse enhancement on GdTA enhanced T1-weighted images. The staged, combined posterior and anterior approach were done and the tumor mass was subtotally removed. During postoperative adjuvant chemotherapy with Cytoxane, Adriamycin, Vincristine, Prednisolone regimen, the patient died of severe compression of upper cervical cord by recurrent, extended tumor mass in the spinal canal.

Extraskeletal Ewing's Sarcoma

A case of extrastkeletal Ewing's sarcoma on lower extremity in a 60-year-old male is reported. Extraskeletal Ewing's sarcoma is a rare malignant neoplasm that shows similar histologic and ultrastructural features to Ewing's sarcoma of bone. Histologically, the neoplasm was composed of small, round to oval cells with scant cytoplasm that were arranged in sheets with large area of necrosis. Electron microscopy of the neoplastic cells revaled scant cytopasm containing glycogen rosettes and a paucity of organelles. There is no ultrastructural evidence to indicate the origin of the cells.

Extraskeletal Neoplasm Resembling Ewing's Sarcoma: Case Report / 대한정형외과학회잡지

Ewing's sarcoma had never been described as a primary tumor outside bone, although other malignant skeletal tumors, such as osteogenic sarcoma & chondrosarcoma, are known to arise from extraskeletal soft tissues. In 1975, Angervall & F.M. Enzinger reported 39 cases of small, round or oval cell sarcomas occuring in the soft tissues and considered histologically indistinguishable from Ewing's sarcoma of bone. Recently, We experienced one case of extraskeletal neoplasm resembling Ewing's sarcoma of bone which it was located deeply in the calf area of young female patient and the case review has been followed until the death, approxlmately 10.5 months after removal.