Key result
Sintilimab plus platinum chemotherapy shows no benefit in a case of pulmonary epithelioid hemangioendothelioma.
Why the study?
Pulmonary epithelioid hemangioendothelioma is a rare vascular tumor with no established treatment protocol.
Does sintilimab plus chemotherapy improve outcomes in a patient with pulmonary epithelioid hemangioendothelioma harboring a germline PALB2 mutation and WWTR1-CAMTA1 fusion?
Case Report (n=1)
Does sintilimab plus chemotherapy improve outcomes in a patient with pulmonary epithelioid hemangioendothelioma harboring a germline PALB2 mutation and WWTR1-CAMTA1 fusion?
PEH patients with a germline PALB2 mutation and WWTR1-CAMTA1 gene fusion may respond poorly to PD-1 inhibitors plus chemotherapy.
Should not yet change practice in pulmonary epithelioid hemangioendothelioma; leaves open whether germline PALB2 and WWTR1-CAMTA1 predict PD-1 inhibitor resistance.
Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor with no established treatment protocol. The authors report the case of a young woman diagnosed with PEH. DNA and RNA analysis by next-generation sequencing was performed on the tumor tissue. A novel germline PALB2 mutation and classical WWTR1-CAMTA1 fusion were identified. She experienced a poor response to sintilimab (a PD-1 inhibitor) plus platinum-based chemotherapy as the first-line treatment. PEH patients harboring a germline PALB2 mutation and WWTR1-CAMTA1 gene fusion may respond poorly to treatment with PD-1 inhibitors plus chemotherapy.
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Zeng et al. (2023) conducted a case report in Pulmonary epithelioid hemangioendothelioma (PEH) (n=1). Sintilimab plus platinum-based chemotherapy was evaluated on Treatment response. Sintilimab plus platinum-based chemotherapy resulted in a poor response in a young woman with pulmonary epithelioid hemangioendothelioma harboring a germline PALB2 mutation and WWTR1-CAMTA1 fusion.
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