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

Zhang, Chunhui; Zhang, Jingchun; Wang, Guangyu; Xu, Jiajia; Liu, Yanlin; Guo, Qing; Zheng, Tongsen; Zhang, Yanqiao

doi:10.18632/oncotarget.13896

Cited by 6 publications

(6 citation statements)

References 31 publications

(32 reference statements)

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“…More recently, a retrospective study by Roma et al showed that 9 of 14 (64%) patients with VHL who received sunitinib treatment as a first-line therapy exhibited radiological response not only in renal and hepatic lesions but also in pancreatic nodules 12 . In addition, a case study reported that sunitinib may be effective in treating a patient with primitive neuroectodermal tumors that express a copy number loss of the VHL gene 13 .…”

Section: Resultsmentioning

confidence: 99%

A retrospective case study of sunitinib treatment in three patients with Von Hippel-Lindau disease

Yuan

Liu

Tong

et al. 2018

Cancer Biology & Therapy

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Von Hippel-Landau (VHL) disease is characterized by malignant and benign tumors in multiple organs. Sunitinib, a tyrosine kinase inhibitor, has been clinically available for treating sporadic patients with recurrent or unresectable and metastatic clear renal cell carcinomas (cRCCs) and metastatic lesions of the lung, but its effect on VHL disease-associated tumors remains poorly understood. This retrospective case series examined the effect of sunitinib on RCC, hemangioblastomas, pheochromocytomas, and pancreatic neuroendocrine tumors in patients with confirmed VHL. Of note, three patients with VHL disease who were treated with sunitinib were identified from a review of their medical records. The efficacy of sunitinib was evaluated by comparing computed tomography (CT) or magnetic resonance imaging (MRI) scans conducted before and after treatment. Adverse side effects associated with sunitinib were assessed and recorded. All three patients with VHL disease exhibited clinical improvement after treatment with sunitinib. Patient 1 exhibited a decrease in the size of both their pheochromocytoma and RCC after 19 months of sunitinib treatment. RCCs in Patients 2 and 3 exhibited stable response to sunitinib for approximately 1 and 6 years, respectively. All the patients reported tolerable side effects. Therefore sunitinib treatment was associated with either partial response or stable control of VHL-related RCCs, pheochromocytomas and pancreatic neuroendocrine tumor (NET) with acceptable side effects. Further evaluation of sunitinib in patients with VHL disease in larger prospective studies is warranted.

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Section: Resultsmentioning

confidence: 99%

A retrospective case study of sunitinib treatment in three patients with Von Hippel-Lindau disease

Yuan

Liu

Tong

et al. 2018

Cancer Biology & Therapy

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“…A recent report suggested that pulmonary PNET with copy number loss (CNL) of the Von Hippel-Lindau (VHL) gene benefits from sunitinib treatment; after a four-month treatment with sunitinib, the patient achieved partial response according to the Response Evaluation Criteria in Solid Tumors guideline and the quality of life has improved significantly. 8 …”

Section: Discussionmentioning

confidence: 99%

“…A recent report suggested that pulmonary PNET with copy number loss (CNL) of the Von Hippel-Lindau (VHL) gene benefits from sunitinib treatment; after a fourmonth treatment with sunitinib, the patient achieved partial response according to the Response Evaluation Criteria in Solid Tumors guideline and the quality of life has improved significantly. 8 For patients with pulmonary nodules who cannot undergo surgery due to disease stage or other contraindications, lung puncture can be used to detect the nature of the nodules. If the patient has pleural effusion or sputum, these specimens can be embedded in liquid paraffin and used for diagnosis.…”

mentioning

confidence: 99%

Rare Primary Pulmonary Primitive Neuroectodermal Tumor: A Case Report and Literature Review

Wang

Dong

et al. 2021

OTT

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“…In addition, tumor cells may be positive for other proteins related to neural differentiation, including neuron-specific enolase (NSE), S-100 protein, neurofilament, synaptophysin, and chromogranin A. NSE, CD99 and vimentin positive can help confirm the diagnosis. In addition, chromosome ectopic (11; 22) (q24; q12) is highly specific and it can have additional diagnostic standards (7)(8)(9)(10).…”

Section: Discussionmentioning

confidence: 99%

Primitive neuroectodermal tumors: a clinical and radiological analysis of six cases

Liu

Zhao

Shi

et al. 2019

Quant. Imaging Med. Surg

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Background: Primitive neuroectodermal tumor (PNET) is extremely rare and highly aggressive with poor prognosis. Few studies concerning PNET's the imaging features have been published.Methods: Six cases of PNET, all confirmed by pathology and immunohistochemical (IHC) examinations, were treated during January 2012 to December 2016. These cases' clinical course and imaging findings were retrospectively analyzed.Results: Among six PNET cases, one located in left superior abdomen, one case at posterior abdominal wall, one case in right orbit, one case in left frontal temporal lobe, one case in pelvic cavity, and one case located in left supraclavicular fossa. The tumor's density was uniform for small tumor and heterogeneous for large tumors on CT images, while the size of tumors differed during presentation depending on the location of the tumor. Marked enhancement was visualized after injection of contrast media. The demarcation between the lesion and adjacent tissues or organs tended to be unclear. On magnetic resonance imaging (MRI) images, the mass mainly showed heterogeneously long T1 and long T2 signal intensity, mixed high signal intensity on fluid-attenuated inversion recovery (FLAIR) image. In two cases maximum intensity projection image reconstruction demonstrates tortuous blood vessels within the tumor on enhanced CT images.Five cases were treated by surgical resection with two cases received adjuvant radiotherapy and three cases received adjuvant chemotherapy. Six patients were followed up with a mean period of 34.5 months (ranging from 6 to 55 months). Five patients survived and one died. Among the five patients undergoing surgeries, one patient presented pelvic and abdominal recurrence/metastasis 2 months after abdominal PNET resection. One patient had a recurrent lesion in the right orbit involving the right ethmoid sinus 6 months after right orbital PNET resection. One patient's pelvic tumor recurred 7 months after PNET operation, and this patient died after 1 year and 10 months of follow-up. During the follow-up period, the remaining three cases did not show obvious recurrence and/or metastasis.Conclusions: Overall, the imaging appearances of PNET lack characteristics. PNETs generally do not have clear boundary, or partially so, with its adjacent organs or tissues suggesting their invasive nature. Upon further validation, maximum intensity projection image reconstruction demonstrates tortuous blood vessels within the tumor on enhanced CT images may be valuable information for the diagnosis of PNET.

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

Cited by 6 publications

References 31 publications

A retrospective case study of sunitinib treatment in three patients with Von Hippel-Lindau disease

A retrospective case study of sunitinib treatment in three patients with Von Hippel-Lindau disease

Rare Primary Pulmonary Primitive Neuroectodermal Tumor: A Case Report and Literature Review

Primitive neuroectodermal tumors: a clinical and radiological analysis of six cases

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