[Epithelioid malignant peripheral nerve sheath tumor. A case report]. / Tumeur maligne des gaines des nerfs périphériques type épithélioïde. A propos d'un cas.

ABSTRACT

Malignant peripheral nerve sheath tumors (MPNST) are exceptional and painful. The typical localization along a peripheral nerve is not always present. Prognosis is very poor. Diagnosis is particularly difficult because of the variable pathological presentation and unpredictable differentiation. We report an exceptional case of epithelioid differentiation in a 43-Year-old patient who complained of right thoracic pain for one and a half Years. Physical examination revealed weight loss and a paravertebral mass under the scapula. The chest x-ray showed a suprahilar opacity suggestive of parietal involvement and lysis of the sixth rib. Surgical biopsy of the parietal mass led to the diagnosis of epithelioid MPNST.