Peripheral primitive neuroectodermal tumor (PNET) of the right hemithorax

Case contributed by Vijay Mistry , 10 Mar 2018
Diagnosis certain
Changed by Bruno Di Muzio, 10 Apr 2018

Updates to Case Attributes

Presentation was changed:
7 weeks of cough with clinically reduced air entry on the right side.
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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 tumours are a rare type of tumortumour, and are treated along the lines of a an Ewing’s sarcoma. In this case, the disease arose in the right pleural space and was fairly extensive at diagnosis.

  • -<p>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 tumours are a rare type of tumor, and are treated along the lines of a Ewing’s sarcoma. In this case the disease arose in the right pleural space and was fairly extensive at diagnosis.</p>
  • +<p>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 tumours are a rare type of tumour, and are treated along the lines of an <a href="/articles/ewing-sarcoma">Ewing sarcoma</a>. In this case, the disease arose in the right pleural space and was fairly extensive at diagnosis.</p>
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Chest radiograph

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CT Chest
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Large right pleural effusion with complete collapse of the right lung.  Associated Associated mediastinal shift to the left and inferior displacement of the right hemidiaphragm and right abdominal organs.  Adherent 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 The left lung remains clear.

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Image 2 CT (C+ portal venous phase) ( update )

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Image 3 CT (lung window) ( update )

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Image 4 CT (lung window) ( update )

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Large right-sided medial irregular intensely avid pleural mass with circumferential moderate pleural uptake in keeping with malignancy,  associated 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.

Updates to Freetext Attributes

Description was changed:

Subsequent biopsy confirmed round cell malignant tumortumour, favouring primitive neuroectodermal tumour/extraskeletal ewing/extraosseous Ewing sarcoma.

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