Introduction: Primitive neuroectodermal tumours (PNETs) of the chest wall are rare entities and little is known regarding its biological activity and prognostic factors. Two cases are reported and the available literature reviewed to highlight the presentation and management of these tumours.Clinical Features: We report 2 patients who were diagnosed with PNET of the chest wall in our centre. As there are no clinical features or basic diagnostic measures which are characteristic of these tumours, diagnosis is based on special tests. With the advent of newer immunohistochemical methods, it is now diagnosed more confidently. Treatment: Both patients received multidisciplinary modalities of treatment, comprising extensive surgical resection, chemotherapy and radiotherapy. Outcome: One patient succumbed to the disease one year after diagnosis and the other is currently disease-free, both clinically and radiologically at 24 months. Conclusion: Despite multidisciplinary modalities of treatment, the prognosis of PNET is still generally poor. Early diagnosis and treatment are important to improve the chances of survival.
Tumours of the chest wall are uncommon and are usually sarcomatous in nature. Primitive neuroectodermal tumours (PNETs) are rare and are thought to arise from neuroepithelial cells, possibly derived from the peripheral intercostal nerves. They could also arise from the neural crest cells, which have migrated alongside the peripheral nerve.
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