scholarly journals Anti-PD-1 Immunotherapy Combined With Antiangiogenic Therapy as Salvage Therapy in a Patient With Refractory Metastatic Peripheral Primitive Neuroectodermal Tumor: A Case Report and Literature Review

Author(s):  
Qiaoyun Chen ◽  
Ronghui Jin ◽  
Xu Li ◽  
Qifan Wu ◽  
Gaili An

Abstract Background: Peripheral primitive neuroectodermal tumor (pPNET) is a relatively rare malignant neoplasm that usually occurs in children and young adults,associated with poor prognosis. However, standard treatment for refractory pPNET has not been determined.Case presentation: A case of a 38-year-old woman with pPNET on the right shoulder and back,she had gained up to seven-and-a-half years suvival undergoing these comprehensive treatment including surgery, radiotherapy, chemotherapy, antiangiogenic treatment. However, the tumor eventually progressed after receiving multiline treatment.With the patient's strong desire to receive immunotherapy, we finally adjusted the treatment plan to " anti-angiogenic tyrosine kinase inhibitor Lenvatinib combined with PD-1 inhibitor Toripalimab" for 2 cycles.Unfortunately,she developed grade 2-3 immune pneumonia after treatment.Conclusions: To our knowledge, this is the first report of immunotherapy combined with antiangiogenic therapy in pPNET,which provides new ideas for the treatment of refractory pPNET.

2004 ◽  
Vol 14 (2) ◽  
pp. 370-372 ◽  
Author(s):  
K.-J. Kim ◽  
B.-W. Jang ◽  
S.-K. Lee ◽  
B.-K. Kim ◽  
S.-L. Nam

Peripheral primitive neuroectodermal tumor (PNET) belongs to the PNET/Ewing's sarcoma family. PNET is a small round cell tumor of putative neuroectoderm origin and is the second most common sarcoma among children and young adults. It may occur anywhere in the body and within any age group; however, it is most likely to occur in the bone and soft tissues. There have been a small number of case reports of PNET arising in the ovary. We presented a case of PNET arising in the right ovary of an 18-year-old woman. The tumor was metastased to the lymph nodes of the pelvis and para-aorta at surgical staging. We had persecuted Taxol/carboplatin chemotherapy, pelvic cavity radiotherapy, and Vincristine/Actinomycink, Cyclophosphamide/Doxorubicin (VACA). She died after 10 months due to septic shock.


1992 ◽  
Vol 20 (4) ◽  
pp. 352-356 ◽  
Author(s):  
Cheryl M. Coffin ◽  
Teresa J. Vietti ◽  
Vita J. Land ◽  
William G. Kraybill ◽  
Louis P. Dehner

2003 ◽  
Vol 413 ◽  
pp. 255-260
Author(s):  
Juan L. Cebri??n ◽  
Aitor Ibarzabal ◽  
Rodrigo Garcia-Crespo ◽  
Fernando Marco ◽  
Luis Ortega ◽  
...  

2004 ◽  
Vol 16 (12) ◽  
pp. 1391-1400 ◽  
Author(s):  
Ralph Czekalla ◽  
Martin Fuchs ◽  
Angela St??lzle ◽  
Andreas Nerlich ◽  
Christopher Poremba ◽  
...  

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Eduardo Cambruzzi ◽  
Enilde Eloena Guerra ◽  
Hamilton Cardoso Hilgert ◽  
Herbert Jorge Schmitz ◽  
Vinícius Lopes Silva ◽  
...  

Primary liver sarcomas represent a rare group of neoplasias, with angiosarcoma being the most common histological type. Primitive neuroectodermal tumor (PNET) represents a high malignant neoplasia that usually affects the central nervous system and soft tissues. An 18-year-old male patient was admitted with clinical complains of pain in the right upper abdominal quadrant. The clinical evaluation revealed a solid mass in the right hepatic lobe. On the gross examination of the resected liver specimen, the right lobe of the liver was replaced by a yellow-red solid mass measuring 21 cm in its largest dimension. On the histopathology, a tumor composed of small round blue cells with little cytoplasm and round nuclei was identified. The lesion revealed positive immunoexpression for vimentin and CD99 and negative immunostaining for desmin, CD45, cytokeratin, and neuroblastoma protein, suggesting, then, the diagnosis of PNET. Although it is an unusual tumor, it should be considered in the differential diagnosis of liver masses, especially in young patients.


Sign in / Sign up

Export Citation Format

Share Document