QUIZ: No. 10, 04/05/2025

X Ray

ANS – Primary Pleural Ewings Sarcoma (Small blue round cell tumor)

Findings: Heterogeneously enhancing right upper thoracic mass with massive pleural effusion without signs of adjacent bone destruction and calcification. Similarly enhancing pleural nodules seen in the right hemithorax.

Small Blue Round Cell Tumors (SBRCTs) in pediatrics is challenging to diagnose radiologically. Extraskeletal Ewing's sarcoma (EES) is a small round cell malignant tumor of neuroectodermal origin.

EES occurs in the head and neck, retroperitoneum, omentum, paravertebral, orbit, skin, chest wall, pelvis, and lower limbs, as well as in the gastrointestinal tract.

Pleural EES is very rare. It is highly malignant, has a poor prognosis, and needs to be differentiated from other thoracic malignancies such as neuroblastoma, lymphoma, and embryonal rhabdomyosarcoma in this age group.

The best treatment in the early stages is surgical excision of the primary followed by local radiotherapy and systemic chemotherapy.

Take home points

·   Aggressive soft tissue mass with necrotic areas, heterogenous enhancement & massive pleural effusion are clues to narrow down to SBRCTs.