Key result
Pazopanib treatment resulted in a long-lasting objective response, with complete metabolic response in the lungs and stable disease in the liver for over two years.
Case Report (n=1)
No
Pazopanib may be a suitable treatment option for metastatic pulmonary epithelioid hemangioendothelioma, yielding long-lasting objective response.
May support pazopanib consideration in this rare malignancy; hypothesis-generating and requires prospective validation before practice change.
BACKGROUND: Epithelioid hemangioendothelioma is a rare vascular tumor of borderline or low-grade malignancy. The lungs and liver are the two common primary organs affected. Metastatic disease was reported in more than 100 cases in the literature. However, no firm conclusions can be determined for recommended treatment options. CASE PRESENTATION: The current case presents a patient with metastatic pulmonary epithelioid hemangioendothelioma to the cervical and mediastinal lymph nodes, lungs and liver that has been treated with pazopanib for more than two years with PET avid complete metabolic response in the mediastinum and lungs, and long-lasting stable disease. Target therapies that block VEGFR have a logical base in this rare malignancy. CONCLUSIONS: The current case is the first to report objective, long-lasting response to pazopanib.
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Semenisty et al. (2015) conducted a case report in Metastatic pulmonary epithelioid hemangioendothelioma (n=1). Pazopanib was evaluated on Tumor response. Pazopanib treatment resulted in a long-lasting objective response, with complete metabolic response in the lungs and stable disease in the liver for over two years.
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