Peripheral primitive neuroectodermal tumour of the chest wall invading lung with regional lymph node metastasis

Abstract

Here we present the case of a 26-year-old man in whom peripheral primitive neuroectodermal tumour of chest wall origin invaded the left lung with regional lymph node metastasis. He underwent initial resection of the left chest wall tumour with combined left lower lobectomy, left S5 segmentectomy, and lymph node dissection in order to facilitate a definitive diagnosis and also to obviate the risk of fatal bleeding due to tumour invasion of the pulmonary artery. Histological examination of the resected sample revealed small round cell proliferation with neural differentiation, and confirmed lymph node involvement within the left lower lobe. EWS/FLI-1 fusion gene transcripts were detected by the reverse-transcription polymerase-chain reaction. Diagnosis of peripheral primitive neuroectodermal tumour was confirmed. After surgery, combination chemotherapy with cyclophosphamide, vincristine, doxorubicin, ifosphamide, and etoposide was given. Five years after resection, the patient remains alive and well, with no signs of recurrence.

Treatment outcomes in 23 thoracic primitive neuroectodermal tumours: a retrospective study

“…All primary thoracic tumours require wide excision of a lesion with normal tissue margins such as ribs, vertebrae, pleura or pericardium etc. A primary tumour located in the lung parenchyma or a chest tumour invading the lung parenchyma mandates anatomical lung resections with hilar and/or mediastinal lymph node dissection as performed in primary lung carcinoma, though the propensity for lymph nodal involvement and its significance in the prognosis of lung PNET are currently not known [12].…”

“…The adjuvant chemotherapy and radiation ablates residual microscopic disease after surgical excision of a tumour and was also shown to improve the overall survivals and disease-progression-free survivals [11,13]. The surgical resection of a chest wall tumour before radiation also allows accurate marking of the area of involvement to permit limited radiation of the thorax [12,14].…”