Rare presentation in a rare case of pancreatic extraosseous Ewing’s sarcoma: A case report

Abstract

Rationale: Extraosseous Ewing’s sarcoma is a rare tumor which is aggressive with poor prognosis; it can occur anywhere in the body, but scantily in the pancreas. Pancreatic Ewing’s sarcoma is not reported commonly, with inconsistent clinical manifestations. In this regard, early recognition of this disease is very important for the patient’s sake. Patient concerns: A 16-year-old boy presented with left lower quadrant abdominal pain for 2 months, and left flank pain with dysuria for 1 month. Diagnosis: Abdominal and renal ultrasonography found a mass between the spleen and left kidney as well as left renal pelvic dilatation. Abdominal computed tomography found a heterogenous mass derived from the tail of the pancreas. Serial examinations revealed that the mass was a pancreatic Ewing’s sarcoma. Furthermore, no metastasis was documented. Interventions: The tumor was totally excised after 6 months of chemotherapy, which included 10 courses of neoadjuvant chemotherapy with vincristine, epirubicin, and cyclophosphamide, alternating with ifosfamide and etoposide. The patient completed consolidation chemotherapy with vincristine, epirubicin, and cyclophosphamide, alternating with ifosfamide and etoposide for 5 courses. Radiotherapy was applied to the tumor-involved region and tumor bed. Outcomes: To date, the malignancy has not recurred since the treatment was completed 4 years ago. There are no complications from the treatment for the patient. Lessons: The pancreas is a very rare extraosseous location for Ewing’s sarcoma. Pancreatic extraosseous Ewing’s sarcoma should be regarded as a differential diagnosis of non-urinary originated left flank pain with dysuria in adolescents.

Extraosseous Ewing sarcoma of the pancreas: a case report

“…Immunohistochemical and cytogenetic analyses are vital for distinguishing ESFTs from other small round blue cell tumors. ESFTs express CD99, a cell surface glycoprotein derived from the MIC2 gene, which distinguishes them from other entities [ 4 , 8 ]. The hallmark genetic abnormality in ESFTs is the EWSR1 gene rearrangement, particularly EWS-FLI1 , found in over 85% of cases, driving tumorigenesis through apoptosis and transcription deregulation [ 5 , 7 ].…”

“…Only few cases with pancreas involvement have been reported globally [ 3 ]. Patients are often diagnosed during adolescence, with a median age of 20, and may present with non-specific symptoms like abdominal pain, nausea, and weight loss [ 3 , 4 , 8 ]. Our patient was 4 years old at the time of diagnosis and her only presenting complaint was abdominal pain.…”

“…The primary approach combines surgery and chemotherapy and/or radiation therapy [ 3 , 5 ]. The standard chemotherapy regimen includes doxorubicin, cyclophosphamide, vincristine, ifosfamide, and etoposide [ 3 , 4 ]. Ewing sarcoma of the pancreas is associated with poor prognosis, primarily due to advanced disease at diagnosis and aggressive behavior, with 5-year survival rates ranging from 61% to 80% in localized cases but dropping below 30% in metastatic disease [ 3 , 4 , 7 , 9 ].…”

“…EES shares histopathological features and a common genetic abnormality, EWSR1 gene rearrangement, with other ESFTs. Due to its variable clinical presentation, which often includes non-specific symptoms like abdominal pain and weight loss, and its aggressive nature, EES necessitates a multidisciplinary treatment approach, incorporating chemotherapy, surgery, and radiation therapy [ 3 , 4 ]. The prognosis remains unfavorable due to late-stage diagnosis and frequent recurrence and metastasis [ 3 , 5 ].…”