Pazopanib Confers a Progression-free Survival in a Patient with Ewing's Sarcoma/Primitive Neuroectodermal Tumor of the Lung

Abstract

Ewing's sarcoma (ES)/primitive neuroectodermal tumors (PNETs) are highly malignant neoplasms that usually affect the bones and soft tissues in children and young adults. ES/PNET of the lung is very rare and is associated with a poor prognosis. We herein report a case of ES/PNET of the left lung in a 45-year-old man. He was treated with neoadjuvant chemotherapy and pneumonectomy, but unfortunately his disease recurred 1.5 months after surgery. He was started on pazopanib, which resulted in a five-month progression-free survival. To our knowledge, this is the first demonstration of pazopanib efficacy in ES/PNET of the lung.

Application of Anlotinib Combined With Neoadjuvant Chemotherapy in Primary EWS/PNET of Lung: A Case Report

“…PET-CT of Ewing’s sarcoma in lung often presents with large volume, smooth margins, and high fluorodeoxyglucose uptake, SUV MAX10-30.3. In addition, fluorodeoxyglucose positron emission tomography (PET) can help to rule out the possibility of pulmonary metastasis by extra-pulmonary Ewing’s sarcoma ( 4 , 6 – 8 , 16 , 17 ).…”

“…Recent studies have shown that anlotinib and pazopanib have been used in the treatment of PPES and achieve incredible results,but are not considered standard treatment ( 4 , 8 ). Takigami reported that a case of PPES received neoadjuvant chemotherapy and total pneumonectomy, recurred 1.5 months after surgery, and achieved a progression-free survival of 5 months after taking pazopanib ( 4 ). Zhang reported that a case of PPES received 5 cycles of chemotherapy, but he did not benefit unambiguously.…”

Primary EWS/PNET of the lung with TP53 germline and SKT11 somatic mutation: A case report and review of the literature

“…Therefore, TP53 testing is necessary, and if possible, radiotherapy and genotoxic chemotherapy should be avoided in carriers. Recent studies have shown that tumor angiogenesis can promote the growth and metastasis of solid tumors, and anlotinib and pazopanib have been reported to be effective in the treatment of PPES, but they are not considered standard treatment 6,8 . Anlotinib was selected for subsequent treatment after multidisciplinary discussion.…”

“…PPES is rare, and the clinical symptoms of cough, phlegm, chest pain, hemoptysis or fatigue, etc are nonspecific. The disease has been found to occur at any age, mostly in children and adolescents, and peaks at the age of 20–30 years old, without any difference in gender 5–7 …”

Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature

“…Regimens of VAC/IE or VDC/IE are commonly used in chemotherapy, but their chemotherapeutic effects are not always satisfactory. Takigami et al[12] reported on a lung PNET case that had been treated with VDC/IE chemotherapy for 5 mo before surgery; after the tumor was resected, it recurred 1.5 mo later. In this case, adriamycin was replaced with actinomycin-D due to a cumulative dose of doxorubicin near 500 mg/m 2 .…”