A 15-year-old patient has been treated for bone ES of the left humerus at another facility. The chest computed tomography (CT)-scan, bone scintigraphy, and osteomedullary biopsy were negative. She had neoadjuvant chemotherapy based on six cycles of Vincristine, Ifosfamide, Doxorubicin, and Etoposide (VIDE). She had a tumor resection with reconstruction by cement. The anatomic pathology examination of the operative part concluded that there was no viable tumor outbreak with clear margins. The patient underwent adjuvant chemotherapy made of eight cycles of Vincristine, Adriamycin, and Cyclophosphamide (VAC).
The patient did not show up for 6 years.
Six years later, she consulted our institution for relapse in her left arm. The full workup based on CT scan showed a lytic tumor process of the first rib that invades the upper mediation, ensheathed the brachio-cephalic arterial trunk, and compresses the upper vena cava. We discovered on the CT scan a voluminous retro-gastric tumor process of 12 x 8 cm compressing the stomach, the tail of the pancreas, and the splenic vessels. The patient had no abdominal symptoms. Esophagogastroduodenoscopy was normal. A core biopsy was performed for both chest and abdominal lesions. The anatomic pathology examination concluded a relapse of her ES for both the sites (Figure 1). The fusion gene such as EWS-FLI-1, EWS-ERG, EWS-ETV1, and EWS-FEV was not performed in our institution due to the lack of materials. The diagnosis of ES was based on histopathology and immunohistochemistry. The multidisciplinary committee (MDC) recommended starting with chemotherapy. She received six cycles of Etoposide, Ifosfamide, and Cisplatin (VIP). Abdominal magnetic resonance imaging (MRI) was performed following chemotherapy treatment. MRI showed a retro-gastric mass that measured 4.41 x 1.51 cm in its greatest axis. This mass was in a hyposignal T1 and T2 with non-gadolinium-enhancing (Figures 2 and 3). These findings invoked a fibrous lesion. However, the local recurrence did not show any clinical response. The MDC decided to operate on the patient because of the persistence of the mass and the single character of the metastasis.
The patient underwent a median exploratory laparotomy. The tumor was located in the lesser sac and abutted the splenic hilum to the left and the posterior wall of the stomach anteriorly. The tumor was closely tied to the pancreatic tissue. We performed an excision of the tumor with distal pancreatectomy and splenectomy (Figure 4). Her postoperative recovery was uneventful. The anatomic pathology examination concluded a complete therapeutic effect within the resected mass (Figure 5). All the margins were free. The surgical margin was R0 according to the R classification. The patient was subsequently referred to a cardiothoracic surgery to operate the coastal mass.
She underwent a wide resection of the local recurrence. The histological exam showed the features of viable ES, with necrosis. The necrosis rate was estimated greater than 50%. The margins were free. Adjuvant radiotherapy was performed at the local recurrence site at the dose of 50 Gy.
The patient is doing well after 21 months of regular follow-up.