Outstanding Response to Sorafenib in a Patient with Metastatic Gastrointestinal Stromal Tumour

Gastrointestinal stromal tumour (GIST) is the most common sarcoma and can be seen in any part of the gastrointestinal tract. The effect of tyrosine kinase inhibitors varies with mutation status in receptor tyrosine kinase KIT and in platelet-derived growth factor receptor A (PDGFRA). This case prese...

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Autores principales:	Charlotte Brinch, Marie Dehnfeld, Estrid Hogdall, Tim Svenstrup Poulsen, Anders Toxvaerd, Gina Al-Farra, Magnus Bergenfeldt, Anders Krarup-Hansen
Formato:	article
Lenguaje:	EN
Publicado:	Karger Publishers 2021
Materias:	gastrointestinal stromal tumour sorafenib pdgfra mutation multidisciplinary approach Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
Acceso en línea:	https://doaj.org/article/486d5e758ceb4949b7c1db703d5bedbf
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spelling	oai:doaj.org-article:486d5e758ceb4949b7c1db703d5bedbf2021-12-02T12:40:22ZOutstanding Response to Sorafenib in a Patient with Metastatic Gastrointestinal Stromal Tumour1662-657510.1159/000519747https://doaj.org/article/486d5e758ceb4949b7c1db703d5bedbf2021-11-01T00:00:00Zhttps://www.karger.com/Article/FullText/519747https://doaj.org/toc/1662-6575Gastrointestinal stromal tumour (GIST) is the most common sarcoma and can be seen in any part of the gastrointestinal tract. The effect of tyrosine kinase inhibitors varies with mutation status in receptor tyrosine kinase KIT and in platelet-derived growth factor receptor A (PDGFRA). This case presents a 61-year-old man, diagnosed with an 11-cm GIST located at the stomach with a high risk of recurrence. The patient showed intolerance to imatinib shortly after introduction and subsequently progressed on sunitinib and nilotinib. The patient started fourth-line treatment with sorafenib with an impressive response to a point at which metastases intra-abdominally and in the liver could be resected. After surgery, sorafenib was restarted. Due to toxicity, sorafenib dose was reduced over time. The dose was insufficient to control the disease since a new recurrence was detected. Mutation analyses revealed a GIST harbouring a deletion of codon p.I843_D846del, located at PDGFRA exon 18, right next to the codon D842 where mutations are known leading to imatinib resistance. In this case, the GIST was highly sensitive to sorafenib, and the response was dose related. It is mandatory to perform mutation analyses on primary tumour and at recurrence in the decision-making of the correct treatment for the patient. In March 2021, the patient had been in treatment with sorafenib for 12.5 years and was still without signs of recurrence. A multidisciplinary approach was essential for the long-term survival of the patient in this case.Charlotte BrinchMarie DehnfeldEstrid HogdallTim Svenstrup PoulsenAnders ToxvaerdGina Al-FarraMagnus BergenfeldtAnders Krarup-HansenKarger Publishersarticlegastrointestinal stromal tumoursorafenibpdgfra mutationmultidisciplinary approachNeoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENCase Reports in Oncology, Vol 14, Iss 3, Pp 1567-1573 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	gastrointestinal stromal tumour sorafenib pdgfra mutation multidisciplinary approach Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
spellingShingle	gastrointestinal stromal tumour sorafenib pdgfra mutation multidisciplinary approach Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282 Charlotte Brinch Marie Dehnfeld Estrid Hogdall Tim Svenstrup Poulsen Anders Toxvaerd Gina Al-Farra Magnus Bergenfeldt Anders Krarup-Hansen Outstanding Response to Sorafenib in a Patient with Metastatic Gastrointestinal Stromal Tumour
description	Gastrointestinal stromal tumour (GIST) is the most common sarcoma and can be seen in any part of the gastrointestinal tract. The effect of tyrosine kinase inhibitors varies with mutation status in receptor tyrosine kinase KIT and in platelet-derived growth factor receptor A (PDGFRA). This case presents a 61-year-old man, diagnosed with an 11-cm GIST located at the stomach with a high risk of recurrence. The patient showed intolerance to imatinib shortly after introduction and subsequently progressed on sunitinib and nilotinib. The patient started fourth-line treatment with sorafenib with an impressive response to a point at which metastases intra-abdominally and in the liver could be resected. After surgery, sorafenib was restarted. Due to toxicity, sorafenib dose was reduced over time. The dose was insufficient to control the disease since a new recurrence was detected. Mutation analyses revealed a GIST harbouring a deletion of codon p.I843_D846del, located at PDGFRA exon 18, right next to the codon D842 where mutations are known leading to imatinib resistance. In this case, the GIST was highly sensitive to sorafenib, and the response was dose related. It is mandatory to perform mutation analyses on primary tumour and at recurrence in the decision-making of the correct treatment for the patient. In March 2021, the patient had been in treatment with sorafenib for 12.5 years and was still without signs of recurrence. A multidisciplinary approach was essential for the long-term survival of the patient in this case.
format	article
author	Charlotte Brinch Marie Dehnfeld Estrid Hogdall Tim Svenstrup Poulsen Anders Toxvaerd Gina Al-Farra Magnus Bergenfeldt Anders Krarup-Hansen
author_facet	Charlotte Brinch Marie Dehnfeld Estrid Hogdall Tim Svenstrup Poulsen Anders Toxvaerd Gina Al-Farra Magnus Bergenfeldt Anders Krarup-Hansen
author_sort	Charlotte Brinch
title	Outstanding Response to Sorafenib in a Patient with Metastatic Gastrointestinal Stromal Tumour
title_short	Outstanding Response to Sorafenib in a Patient with Metastatic Gastrointestinal Stromal Tumour
title_full	Outstanding Response to Sorafenib in a Patient with Metastatic Gastrointestinal Stromal Tumour
title_fullStr	Outstanding Response to Sorafenib in a Patient with Metastatic Gastrointestinal Stromal Tumour
title_full_unstemmed	Outstanding Response to Sorafenib in a Patient with Metastatic Gastrointestinal Stromal Tumour
title_sort	outstanding response to sorafenib in a patient with metastatic gastrointestinal stromal tumour
publisher	Karger Publishers
publishDate	2021
url	https://doaj.org/article/486d5e758ceb4949b7c1db703d5bedbf
work_keys_str_mv	AT charlottebrinch outstandingresponsetosorafenibinapatientwithmetastaticgastrointestinalstromaltumour AT mariedehnfeld outstandingresponsetosorafenibinapatientwithmetastaticgastrointestinalstromaltumour AT estridhogdall outstandingresponsetosorafenibinapatientwithmetastaticgastrointestinalstromaltumour AT timsvenstruppoulsen outstandingresponsetosorafenibinapatientwithmetastaticgastrointestinalstromaltumour AT anderstoxvaerd outstandingresponsetosorafenibinapatientwithmetastaticgastrointestinalstromaltumour AT ginaalfarra outstandingresponsetosorafenibinapatientwithmetastaticgastrointestinalstromaltumour AT magnusbergenfeldt outstandingresponsetosorafenibinapatientwithmetastaticgastrointestinalstromaltumour AT anderskraruphansen outstandingresponsetosorafenibinapatientwithmetastaticgastrointestinalstromaltumour
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Outstanding Response to Sorafenib in a Patient with Metastatic Gastrointestinal Stromal Tumour

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