Surgical Treatment and Targeted Therapy for a Large Metastatic Malignant Peripheral Nerve Sheath Tumor: A Case Report and Literature Review
Neurofibromatosis type 1 (NF1) significantly increases the risk of malignant peripheral nerve sheath tumors (MPNST), a rare and aggressive malignancy for which treatment is clinically challenging. This paper presents the case of a 24-year-old male with an NF1 who developed MPNST with lung metastases...
Saved in:
| Main Authors: | , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2024-12-01
|
| Series: | Life |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2075-1729/14/12/1648 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1850050964563689472 |
|---|---|
| author | Patryk Skórka Dawid Kordykiewicz Andrzej Ilków Konrad Ptaszyński Janusz Wójcik Wiktoria Skórka Małgorzata Edyta Wojtyś |
| author_facet | Patryk Skórka Dawid Kordykiewicz Andrzej Ilków Konrad Ptaszyński Janusz Wójcik Wiktoria Skórka Małgorzata Edyta Wojtyś |
| author_sort | Patryk Skórka |
| collection | DOAJ |
| description | Neurofibromatosis type 1 (NF1) significantly increases the risk of malignant peripheral nerve sheath tumors (MPNST), a rare and aggressive malignancy for which treatment is clinically challenging. This paper presents the case of a 24-year-old male with an NF1 who developed MPNST with lung metastases. Due to the limited effectiveness of systemic therapy in the treatment of MPNST, the patient underwent radical surgical resection and radiotherapy. Pathological evaluation confirmed high-grade MPNST, and PET-CT imaging revealed further metastatic progression. The treatment results for our patient are compared with those of other patients with NF1 who also developed MPNST with lung metastases in the literature. The findings suggest the need for further research into personalized treatment strategies that may improve prognosis and overall survival in patients with NF1 and MPNST, with immunotherapy being a promising therapeutic option. |
| format | Article |
| id | doaj-art-a1ff826d461142e9b4533a6f666ec2d3 |
| institution | DOAJ |
| issn | 2075-1729 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Life |
| spelling | doaj-art-a1ff826d461142e9b4533a6f666ec2d32025-08-20T02:53:18ZengMDPI AGLife2075-17292024-12-011412164810.3390/life14121648Surgical Treatment and Targeted Therapy for a Large Metastatic Malignant Peripheral Nerve Sheath Tumor: A Case Report and Literature ReviewPatryk Skórka0Dawid Kordykiewicz1Andrzej Ilków2Konrad Ptaszyński3Janusz Wójcik4Wiktoria Skórka5Małgorzata Edyta Wojtyś6Department of Thoracic Surgery and Transplantation, Pomeranian Medical University in Szczecin, Alfreda Sokołowskiego 11, 70-891 Szczecin, PolandDepartment of Thoracic Surgery and Transplantation, Pomeranian Medical University in Szczecin, Alfreda Sokołowskiego 11, 70-891 Szczecin, PolandDepartment of General, Vascular and Oncological Surgery, Provincial Hospital, Mikołaja Kopernika, Tytusa Chałubińskiego 7, 75-581 Koszalin, PolandDepartment of Pathology, University Hospital of Pomeranian Medical University in Szczecin, Unii Lubelskiej 1, 71-252 Szczecin, PolandDepartment of Thoracic Surgery and Transplantation, Pomeranian Medical University in Szczecin, Alfreda Sokołowskiego 11, 70-891 Szczecin, PolandDepartment of Thoracic Surgery and Transplantation, Pomeranian Medical University in Szczecin, Alfreda Sokołowskiego 11, 70-891 Szczecin, PolandDepartment of Thoracic Surgery and Transplantation, Pomeranian Medical University in Szczecin, Alfreda Sokołowskiego 11, 70-891 Szczecin, PolandNeurofibromatosis type 1 (NF1) significantly increases the risk of malignant peripheral nerve sheath tumors (MPNST), a rare and aggressive malignancy for which treatment is clinically challenging. This paper presents the case of a 24-year-old male with an NF1 who developed MPNST with lung metastases. Due to the limited effectiveness of systemic therapy in the treatment of MPNST, the patient underwent radical surgical resection and radiotherapy. Pathological evaluation confirmed high-grade MPNST, and PET-CT imaging revealed further metastatic progression. The treatment results for our patient are compared with those of other patients with NF1 who also developed MPNST with lung metastases in the literature. The findings suggest the need for further research into personalized treatment strategies that may improve prognosis and overall survival in patients with NF1 and MPNST, with immunotherapy being a promising therapeutic option.https://www.mdpi.com/2075-1729/14/12/1648case reportNF1MPNST1lung metastasis |
| spellingShingle | Patryk Skórka Dawid Kordykiewicz Andrzej Ilków Konrad Ptaszyński Janusz Wójcik Wiktoria Skórka Małgorzata Edyta Wojtyś Surgical Treatment and Targeted Therapy for a Large Metastatic Malignant Peripheral Nerve Sheath Tumor: A Case Report and Literature Review Life case report NF1 MPNST1 lung metastasis |
| title | Surgical Treatment and Targeted Therapy for a Large Metastatic Malignant Peripheral Nerve Sheath Tumor: A Case Report and Literature Review |
| title_full | Surgical Treatment and Targeted Therapy for a Large Metastatic Malignant Peripheral Nerve Sheath Tumor: A Case Report and Literature Review |
| title_fullStr | Surgical Treatment and Targeted Therapy for a Large Metastatic Malignant Peripheral Nerve Sheath Tumor: A Case Report and Literature Review |
| title_full_unstemmed | Surgical Treatment and Targeted Therapy for a Large Metastatic Malignant Peripheral Nerve Sheath Tumor: A Case Report and Literature Review |
| title_short | Surgical Treatment and Targeted Therapy for a Large Metastatic Malignant Peripheral Nerve Sheath Tumor: A Case Report and Literature Review |
| title_sort | surgical treatment and targeted therapy for a large metastatic malignant peripheral nerve sheath tumor a case report and literature review |
| topic | case report NF1 MPNST1 lung metastasis |
| url | https://www.mdpi.com/2075-1729/14/12/1648 |
| work_keys_str_mv | AT patrykskorka surgicaltreatmentandtargetedtherapyforalargemetastaticmalignantperipheralnervesheathtumoracasereportandliteraturereview AT dawidkordykiewicz surgicaltreatmentandtargetedtherapyforalargemetastaticmalignantperipheralnervesheathtumoracasereportandliteraturereview AT andrzejilkow surgicaltreatmentandtargetedtherapyforalargemetastaticmalignantperipheralnervesheathtumoracasereportandliteraturereview AT konradptaszynski surgicaltreatmentandtargetedtherapyforalargemetastaticmalignantperipheralnervesheathtumoracasereportandliteraturereview AT januszwojcik surgicaltreatmentandtargetedtherapyforalargemetastaticmalignantperipheralnervesheathtumoracasereportandliteraturereview AT wiktoriaskorka surgicaltreatmentandtargetedtherapyforalargemetastaticmalignantperipheralnervesheathtumoracasereportandliteraturereview AT małgorzataedytawojtys surgicaltreatmentandtargetedtherapyforalargemetastaticmalignantperipheralnervesheathtumoracasereportandliteraturereview |