Authors : Hajar Ben Ataya; Laila Herrak; Aziza Rhanim; Asmaa Jniene; Mustapha Ftoh; Leila Achachi
Volume/Issue : Volume 6 - 2021, Issue 6 - June
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Primary peripheral neuro-ectodermal tumor
(pPNET) is a rare and very aggressive tumor that
belongs to a small round cell tumor, and is most often
located in the chest wall.
PNET of the chest wall belongs to the Ewing
sarcoma family because of their genotypic and
phenotypic appearance, It can affect several organs,
Pleural localization remains rare and little described in
the literature. Here, we report a case of a particular
localization of a pPNET. This is a 30-year-old patient
who presented with chest pain on the right side,
posterior irradiation, stage III dyspnea of MMRC and a
dry cough, all progressing in a context of apyrexia and
significant weight loss. .The chest X-ray showed
complete opacification of the right hemi-thorax with
deviation from the midline to the left side. Thoracic
computed tomography reveals a large right pleural mass
of cystic density containing multiple raised septa after
injection of the PDC, pushing back the mediastinum to
the left, the liver below, and the vascular structures of
the neck above and inside which, however, remain
permeable, associated a pleural effusion of moderate
abundance, without other secondary localizations. The
diagnosis of pleural Pnet was made on an echo-guided
biopsy of the necrotic pleural mass. The patient was then
transferred to the cancer center to start chemotherapy.
We insist through this work on the rarity of the
pathology, the diagnostic difficulties and the reserved
prognosis.
Keywords : Peripheral primitive neuro-ectodermal tumor (PNET) Pleural space Necrotic pleural mass