Case report: Primary pleural giant extraskeletal Ewing sarcoma in a child

Abstract

Primary extraskeletal Ewing sarcoma (EES) is a rare small round cell malignancy that accounts for less than 1% of all sarcomas. It is found most commonly in the trunk and lower limbs and very rarely in the pleura and can be easily misdiagnosed in clinical practice. This study presents the case of an 11-year-old boy who presented to our hospital with no apparent cause of left shoulder pain for 6 months. On physical examination, tenderness was noted in the left chest wall and shoulder joint, which had a limited range of motion. Computed tomography (CT) and magnetic resonance imaging (MRI) of the chest revealed an irregular soft tissue mass in the upper left thorax, with a wide base attached to the adjacent pleura and bone destruction of the adjacent left first rib. The patient’s bone scan showed a dense focus of increased radiotracer accumulation in the left first rib. A subsequent CT-guided aspiration biopsy of the left pleural mass with histomorphology and immunohistochemical phenotyping led to a diagnosis of extraskeletal Ewing sarcoma. To inhibit tumor growth, alternating systemic chemotherapy cycles of vincristine, doxorubicin, and cyclophosphamide (VDC) and isocyclophosphamide and etoposide (IE) were administered at 3-week intervals. After completing three VDC and two IE cycles, the child’s condition was well and the pain in the left shoulder joint was relieved. However, a repeat MRI of the chest showed that the mass did not shrink.

Rare Presentation of Primary Pleural Ewing Sarcoma With a Mass Extending Into the Right Ventricle: A Case Report

“…If the primary tumor is in the bone symptoms usually include bone pain that worsens at night. Of note, the presence of constitutional symptoms like weight loss and fever indicates advanced disease [1,[5][6][7].…”

“…It is challenging to accurately diagnose a patient with radiological imaging only, as it might overlap with other malignancies such as primary lung cancer. PET scans may be useful to monitor the disease activity and for surveillance purposes [6,7].…”

“…A combination of contrast-enhanced CT and MRI confirmed the presence of a pleural-origin tumor invading the left first rib. CT-guided biopsy was performed with histopathological analysis showing strong reactivity to CD99 and NKX2.2 Systemic chemotherapy was started with a combination of vincristine, cyclophosphamide, isocyclophosphamide, etoposide, and doxorubicin alternating regimens [7].…”