Peripheral primitive neuroectodermal tumor (PNET) of the right hemithorax

Case contributed by Vijay Mistry
Diagnosis certain


7 weeks of cough with clinically reduced air entry on the right side.

Patient Data

Age: 26
Gender: Male

Complete white out of right lung with mediastinal shift to the left. Clear left lung field.

Large right pleural effusion with complete collapse of the right lung. Associated mediastinal shift to the left and inferior displacement of the right hemidiaphragm and right abdominal organs. Adherent to the diaphragmatic and posterior costal pleura are lobulated increased attenuation lesions, largest measuring 11.5 cm.  There is no thoracic or abdominal lymphadenopathy. The left lung remains clear.

Subsequent biopsy confirmed round cell malignant tumor, favoring primitive neuroectodermal tumor/extraosseous Ewing sarcoma.

Large right-sided medial irregular intensely avid pleural mass with circumferential moderate pleural uptake in keeping with malignancy, associated with a large right-sided effusion and collapse. Right-sided intercostal catheter, with partial drainage of right pleural effusion, compared to previous CT and new gas pockets within the effusion.  Associated surgical emphysema along the right chest wall, bilateral supraclavicular fossae and bilateral neck post ICC insertion. Intensely FDG-avid right-sided pleural malignancy with no distant metastatic disease identified.

Case Discussion

While the presentation of a pleural effusion is not uncommon, given the age and lack of previous medical history, in this case, provides concern for malignancy. Primitive peripheral neuroectodermal tumors are a rare type of tumor, and are treated along the lines of an Ewing sarcoma. In this case, the disease arose in the right pleural space and was fairly extensive at diagnosis.

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