Successful chemotherapy with continuous immunotherapy for primary pulmonary endovascular epithelioid hemangioendothelioma: A case report

Abstract

Rationale: Epithelioid hemangioendothelioma (EHE) is a rare, low to moderate-grade malignancy, even less in pulmonary endovascular neoplasm. Patients with pulmonary EHE have no optimal treatment, resulting in poor prognoses. Patient concerns: We reported a 42-year-old man with multiple mild metabolic uptakes in pulmonary endovascular filling defect with a maximum standardized uptake value of 4.5 by 18-fluorodeoxyglucose/fibroblast associated protein inhibitor-positron emission tomography/ computed tomography. Anticoagulant treatment was not effective with the diagnosis of acute pulmonary embolism. Diagnoses: A primary endovascular EHE pulmonary endovascular epithelioid hemangioendothelioma was diagnosed by endovascular biopsy with positive stains for molecular CD31, CD34 and CAMTA1, and it had low proliferative capacity characterized by Ki-67 of 5%. The mutation gene MSH2 (p.Y656 in exon 12) (mutation abundance of 0.07%) from peripheral blood indicates the potential benefit of an immune checkpoint inhibitor, pembrolizumab. Interventions and outcomes: The patient was treated with tri-weekly paclitaxel (175mg/m2) and carboplatin (AUC 5) chemotherapy regimen. He exerted a remarkable response after 5 cycles (21 days per cycle) and Pembrolizumab (200mg once monthly) as maintenance treatment. Lessons: This case highlights the diagnostic challenge of differentiating endovascular lesions and optimal therapy for pulmonary EHE. Importantly, it indicated that the mutation gene MSH2 (p.Y656) might influence the pathogenesis of EHE.

An extremely rare case of pulmonary epithelioid hemangioendothelioma

“…Other treatments include chemotherapy, radiotherapy, steroids, and low dose interferon has been used in several patients. Very limited clinical trials have been done using chemotherapy and radiotherapy; therefore, the effectiveness of these methods remain unclear 13 . In our case, as the mass was in the left upper lobe, we considered lobectomy to extirpate the lesion completely and that extirpation by VATS might be a better option to treat this condition.…”

Dual immunotherapy alternating with anti-PD-1 antibody plus liposomal doxorubicin show good efficacy in prostate epithelioid hemangioendothelioma: a case report

“…It should be noted that some studies have reported that immunotherapy for prostate cancer was not significantly effective ( 31 , 32 ). However, treatments involving pembrolizumab ( 33 ) and toripalimab ( 34 ) have demonstrated benefits in epithelioid hemangioendothelioma, making immunotherapy a viable alternative strategy for patients with widespread metastatic epithelioid hemangioendothelioma. The difference in treatment between the two may be due to the differing pathological types, as well as the low incidence of hemangioendothelioma, resulting in limited data on the immunotherapy research.…”

Predicting biomarker for the acute pulmonary embolism by using gene ontology and machine learning

“…Early gene expression studies have provided evidence that PE is reflected by alterations in the whole blood transcriptome correlated with pathological pathways. [9][10][11] The integration of multi-layer biological data enriched with clinical annotations through advanced machine learning now enables the development of highly potent diagnostic tools applicable at the point of care. Prospective studies involving gene expression profiling in larger patient cohorts, functional validations, evaluation in real-world settings, and comparison with current methods are warranted before biomarkers can be confidently applied in clinical practice.…”