Rapidly fatal Askin’s tumor: a case report and literature review

Abstract

An 18-year-old male presented with a mass in the right anterior chest wall. Chest Computed tomography revealed a heterogenous mass of 19X13 cm in the right hemithorax with areas of necrosis. There was associated pleural effusion and infiltration of the soft parts of the chest wall. Bronchoscopy showed a tumor in middle lobe bronchus. CT guided biopsy of the mass was performed. Histological examination showed small round tumor cells with scanty cytoplasm, the nuclei are large and hyperchromatic. The tumor cells were positive for CD99 and neuron specific enolase, negative for cytokeratin, leukocyte common antigen and myogenin. Based on these histologic and immunohistochemical findings, the diagnosis of askin's tumor was made. The extension assessment was negative and the patient was given chemotherapy. Two months later, our patient died. Askin's tumor is a rare, highly malignant tumor affecting children and young adults. It is classified as primitive neuroectodermal tumor of the thoracopulmonary region. Prognosis remains poor. In our case, several prognostic factors may explain the shirt ‘term survival, despite no distant metastasis were found: important tumor size, impossibility of surgical treatment and pleural effusion.

Multimodal treatment of pediatric patients with Askin’s tumors: our experience

“…The typical histological feature is the presence of small round cells, with Homer-Wright rosettes and immunohistochemical positivity for several neural markers, such as NSE and CD99. The identification of chromosomal translocation t(11;22)(q24,q12) and the detection of proto-oncogenes (n-myc, c-myb, c-ets-1) may be additional diagnostic criteria [ 14 ].…”

Askin tumor in the chest wall

“…Despite the poor prognosis, treatment of Askin's tumor requires discussion by multidisciplinary tumor working groups. Aggressive treatment include surgery, chemotherapy, and radiation should be undertaken, and complete surgical resection is recommended for a definitive diagnosis and individual treatment 5 …”

Interdisciplinary Radical “En-Bloc” Resection of Ewing Sarcoma of the Chest Wall and Simultaneous Chest Wall Repair Achieves Excellent Long-Term Survival in Children and Adolescents

“…Unspecific symptoms such as fever, enlarged superficial lymph nodes, decreased general condition and nocturnal sweat may occur ( 6 ). Rarely, patients initially present with pathological fracture or metastasis related symptoms ( 7 ). Diagnostics reveal location and volume of the primary tumor as well as infiltrations e.g., into vertebral column, lung, pericardium, diaphragm or esophagus.…”