Presentation
Left-sided lower chest and upper abdominal wall large swelling extending to the back, rapidly growing
Patient Data
MRI study shows a left postero-lateral lower chest/ upper abdominal huge soft tissue mass lesion measures 14x15x20 cm along its largest axial and cc dimensions.
- anterosuperior, the mass is seen exerting mass effect on the lower lung base and pleura smoothly displacing them. Associated minimal pleural effusion is noted
- postero-lateral: the mass is seen violating the lower chest and upper abdominal wall, bulging externally
- medially, the mass is seen displacing the spleen and reaching the left paraspinal region
- inferiorly, the mass is seen reaching the left lumbar region, indenting the lateral cortex of the left kidney
It elicits markedly heterogeneous signal intensity in T1 and T2 with internal hemorrhagic and cystic areas. Heterogeneous enhancement is noted in the post-contrast study
CT study shows erosive changes of the posterior ribs and right pulmonary two small nodules mostly metastatic deposits.
Histopathology:
Macroscopic: FNAC is done for the soft tissue mass and 11 slides are smeared, fixed in 95% alcohol, and stained by H&E stain.
Microscopic: Adequate smears show numerously scattered discohesive blue round cells with large hyperchromatic nuclei. The cytoplasm is scanty. All are in a background of RBCs, and Inflammatory cells.
Diagnosis: left postero-lateral chest soft tissue mass, FNAC shows a picture of small round blue cell tumor.
Case Discussion
Ewing sarcoma family of tumors (ESFT), also referred as Ewing sarcomas of the chest wall, are malignant tumors affecting children and young adults, originating either from the osseous structures or the soft tissues of the chest wall. On imaging, they are usually characterized as large extrapulmonary invasive soft tissue masses that are heterogeneous due to the presence of hemorrhage, necrosis, or cystic changes.
Small round blue cell tumors are a large number of tumors that share similar histology, composed of relatively uniform primitive small round blue cells. They also share many demographic, radiographic and clinical similarities.
Originally a small round blue cell tumor of the soft tissues of the chest was described and became known as an Askin tumor or peripheral primitive neuroectodermal tumor (pPNET). All three entities are now thought to belong to the same tumor family, now referred to as the Ewing sarcoma family of tumors (ESFT).
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