Primary pulmonary extraskeletal Ewing sarcoma/Primitive neuroectodermal tumor: Two case reports

Eman Sobh,Wael H El-Sheshtawy,Shady E Anis

doi:10.4103/ejb.ejb_48_16

Eman Sobh, Wael H El-Sheshtawy + Show 1 more

Open Access

https://doi.org/10.4103/ejb.ejb_48_16

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Abstract

Primary pulmonary extraskeletal Ewing sarcoma (EES)/ primitive neuroectodermal tumor is an extremely rare tumor with only few cases reported in the literature. In this study, we present two cases of primary pulmonary EES diagnosed and treated at our institution. The median age was 20 years (range: 19–21). Cough, dyspnea, and hemoptysis were the predominant features at presentation, associated with a large lung mass on imaging. Image-guided core needle biopsies were the diagnostic modalities for both patients. Initial histopathology showed malignant small round cell tumor, which has been confirmed by immunohistochemistry as EES. Both patients received neoadjuvant chemotherapy followed by surgery; postoperative pathology for the first patient showed 98% tumor necrosis, whereas the second patient’s pathology showed no evidence of residual tumor after complete surgical excision.

Highlights

Ewing’s sarcoma family is a group of aggressive tumors, mostly arising from bone
Ewing sarcoma usually presents during the second decade of life with male predominance [3]
The patient left the hospital after surgery without complications and she is still under regular follow-up at our hospital with no relapse until now (35.5 months). Metastatic workup for both patients included Computed tomography (CT), bone scan, and bone marrow biopsy before treatment to rule out the possibility of metastasis and the presence of skeletal primary, and all were free from metastasis

Summary

Introduction

Ewing’s sarcoma family is a group of aggressive tumors, mostly arising from bone. It was first described in 1921 by American pathologist James Ewing [1]. Tumor cells showed negative staining for leukocyte common antigen (LCA), thyroid transcription factor 1 (TTF1), and pancytokeratin (AE1/AE3) (Fig. 2b); these findings were compatible with extraskeletal Ewing’s. The patient left the hospital after surgery without complications and she is still under regular follow-up at our hospital with no relapse until now (35.5 months) Metastatic workup for both patients included CT (neck, chest, abdomen, and pelvis), bone scan, and bone marrow biopsy before treatment to rule out the possibility of metastasis and the presence of skeletal primary, and all were free from metastasis

Discussion

Conclusion