Case Reports:

Benefit of Sunitinib in the treatment of pulmonary primitive neuroectodermal tumors: a case report and literature review

Chunhui Zhang, Jingchun Zhang, Guangyu Wang, Jiajia Xu, Yanlin Li, Qing Guo, Tongsen Zheng and Yanqiao Zhang _

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Oncotarget. 2016; 7:87543-87551. https://doi.org/10.18632/oncotarget.13896

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Chunhui Zhang1, Jingchun Zhang1, Guangyu Wang1, Jiajia Xu2, Yanlin Li2, Qing Guo2, Tongsen Zheng1 and Yanqiao Zhang1

1 Department of Gastrointestinal Medical Oncology, The Affiliated Tumour Hospital of Harbin Medical University, Harbin, Heilongjiang, China

2 Institute of Precision Medicine, 3D Medicines Inc, Shanghai, China

Correspondence to:

Yanqiao Zhang, email:

Keywords: pulmonary primitive neuroectodermal tumor, next-generation sequencing, von Hippel-Lindau, sunitinib

Received: April 25, 2016 Accepted: November 21, 2016 Published: December 10, 2016


Primitive neuroectodermal tumor (PNET) is a highly aggressive small round celltumor but is extremely rare in the lung. Next-generation sequencing (NGS) has led to breakthroughs for genetic analyses and personalizedmedicine approaches for cancer treatment.We report the case of a 30-year-old woman with an advanced pulmonary PNET treated with multiple chemotherapeutic regimens, and achieved a partial response (PR) as a best response. However, there was a disease progression after these treatment regimens.The NGS revealed the presence of a copy number loss (CNL) of Von Hippel-Lindau (VHL), CDKN2A/B and TP53 genes. The specific VHL CNL has not previously been associated with PNET, but has been reported in other tumors and has been associated with response to Sunitinib. Sunitinibwas then instituted for this patient and resulted in PR after the failure of multiple chemotherapeutic regimens. To our knowledge, this is the first report of pulmonary PNET with CNL of VHL gene that benefits from Sunitinib treatment. This case illustrates the potential of clinicalNGS to open unexpected avenues for treatment and thereby improve patient outcomes.

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