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Primary Multiple Pulmonary Primitive Neuroectodermal Tumor: Case Report and Literature Review
Ming Dong1, Jinghao Liu, Zuoqing Song
1From the Department of Lung Cancer Surgery, Tianjin Lung Cancer Institute (MD, JL, ZS, XL, DR, JC); Department of Pathology; Tianjin Medical University General Hospital, Heping District, Tianjin, China (TS, DW).
Medicine
|July 14, 2015
Summary
Primitive neuroectodermal tumors (PNETs) of the lung are rare and aggressive. Targeted therapy with Avastin and Cetuximab showed promise as a new treatment direction for this challenging PNET diagnosis.
Area of Science:
- Oncology
- Thoracic Surgery
- Molecular Pathology
Background:
- Primitive neuroectodermal tumors (PNETs) originating in the lung are exceptionally rare and aggressive neoplasms.
- While thoracic PNETs typically involve the chest wall, primary lung parenchymal PNETs are infrequently reported.
Observation:
- A case of a 16-year-old male with primary pulmonary PNET is presented.
- Diagnosis was confirmed via histologic and immunohistochemical examination of a video-assisted thoracic surgical biopsy.
- The patient's condition was initially poor, with delayed diagnosis and poor prognosis despite aggressive multimodal therapy.
Findings:
- High-throughput sequencing was employed to explore targeted pharmacotherapy options.
- Treatment with Avastin and Cetuximab was investigated as a potential therapeutic strategy.
- This targeted approach demonstrated potential as a novel treatment direction for PNET.
Implications:
- Primary pulmonary PNETs, especially those with Ewing sarcoma-like features, require consideration in differential diagnoses.
- Targeted therapy represents a promising avenue for managing these rare and aggressive lung neoplasms.
- Further research into targeted pharmacotherapy may significantly improve outcomes for patients with PNETs.

