From Ataxia to Diagnosis of Askin Tumor – a Case Report

Abstract

Peripheral primitive neuroectodermal tumors (pPNET) are a group of extremely rare, aggressive, malignant tumors that are most often found in the thorax (Askin tumor), abdomen, pelvis, extremities and less frequently in the head and neck. The most important prognostic factor is the stage of the tumor. Significant progress both in surgery and in neoadjuvant and adjuvant chemotherapy and radiotherapy, as well as the improvement in diagnosis by cytogenetic and immunohistochemical analysis, should improve the survival rate. We report a case of a 14-year-old girl, with ataxic gait, cardiopulmonary compensated, without respiratory symptoms, who was referred to our hospital for further examination and treatment of newly discovered tumor of the left hemithorax. After a detailed radiological and laboratory investigation, next step was an extensive thoraco-neurosurgical surgery. After histopathological, cytological and molecular analysis, a diagnosis of Askin tumor was made.

Askin tumor in the chest wall

“…Primitive neuroectodermal tumor (PNET) including primitive neuroectodermal central nervous system tumor, neuroblastoma and peripheral primitive neuroectodermal tumor belongs to Ewing's sarcoma family of tumors which is a kind of aggressive tumor. Among them, peripheral PNET in the chest wall (Askin's tumor) is extremely rare, 1 and was first reported by Askin et al . in 1979 2 .…”

“…There may be systemic symptoms such as breathlessness, cough, and weight loss. Patients usually present with an asymptomatic mass on computed tomography (CT) scan, with or without pain 1 . To date, it remains a challenge to surgeons due to the absence of standard therapeutic guidelines on the diagnosis and treatment of Askin's tumor, leading to a poor prognosis with two‐ and six‐year survival rates of 38% and 14%, respectively 4 .…”