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First case description of a highly aggressive thoracopulmonary primitive neuroectodermal tumor in a Moroccan adult
Youssef Ait Takniouine1, Leila Afani1, Manal Aboussad1
1Medical Oncology Department, Mohammed VI University Hospital of Marrakech, Marrakech, Morocco.
Abstract:
Askin's tumor is a rare, highly aggressive thoracopulmonary malignancy within the Ewing sarcoma family of tumors, predominantly affecting children and young adults, and is associated with a poor prognosis. We present a case of a 40-year-old male who is a current smoker with a 7-month history of a painful left anterior chest wall mass. Contrast-enhanced CT showed a large chest-wall tumor with rib lysis and pleural involvement. Histopathology confirmed a primitive neuroectodermal tumor, with immunohistochemistry positive for CD99, S-100, and synaptophysin. The patient was treated with VAC-IE poly-chemotherapy but experienced rapid radiological progression with extensive metastatic disease despite full-dose treatment. In the absence of an effective treatment for an aggressive disease, early intervention and multidisciplinary management are essential for timely control, prolonging survival while preserving quality of life.