pazopanib and Hemangioendothelioma--Epithelioid

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.. 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.. The current case is the first to report objective, long-lasting response to pazopanib.

Topics: Angiogenesis Inhibitors; Antineoplastic Agents; Female; Hemangioendothelioma, Epithelioid; Humans; Immunohistochemistry; Indazoles; Lung Neoplasms; Middle Aged; Neoplasm Metastasis; Positron-Emission Tomography; Protein Kinase Inhibitors; Pyrimidines; Receptors, Vascular Endothelial Growth Factor; Sulfonamides; Tomography, X-Ray Computed; Treatment Outcome

Pazopanib is a multitargeted tyrosine kinase inhibitor approved for the treatment of patients with selective subtypes of advanced soft tissue sarcoma (STS) who have previously received standard chemotherapy including anthracyclines. Data on the efficacy in vascular sarcomas are limited. The main objective of this study was to investigate the activity of pazopanib in vascular sarcomas.. A retrospective study of patients with advanced vascular sarcomas, including angiosarcoma (AS), epithelioid hemangioendothelioma (HE) and intimal sarcoma (IS) treated with pazopanib in real life practice at EORTC centers as well as patients treated within the EORTC phase II and III clinical trials (62043/62072) was performed. Patient and tumor characteristics were collected. Response was assessed according to RECIST 1.1. and survival analysis was performed.. Fifty-two patients were identified, 40 (76.9%), 10 (19.2%) and two (3.8%) with AS, HE and IS, respectively. The response rate was eight (20%), two (20%) and two (100%) in the AS, HE and IS subtypes, respectively. There was no significant difference in response rate between cutaneous and non-cutaneous AS and similarly between radiation-associated and non-radiation-associated AS. Median progression-free survival (PFS) and median overall survival (OS; from commencing pazopanib) were three months (95% CI 2.1-4.4) and 9.9 months (95% CI 6.5-11.3) in AS, respectively.. The activity of pazopanib in AS is comparable to its reported activity in other STS subtypes. In this study, the activity of pazopanib was similar in cutaneous/non-cutaneous and in radiation/non-radiation-associated AS. In addition, pazopanib showed promising activity in HE and IS, worthy of further evaluation.

Topics: Adult; Aged; Aged, 80 and over; Angiogenesis Inhibitors; Female; Follow-Up Studies; Hemangioendothelioma, Epithelioid; Hemangiosarcoma; Humans; Indazoles; Lymphatic Metastasis; Male; Middle Aged; Neoplasm Staging; Neoplasms; Prognosis; Pyrimidines; Retrospective Studies; Sulfonamides; Survival Rate; Tunica Intima

Hepatic epithelioid hemangioendothelioma (EHE) is a rare malignant vascular tumor of endothelial origin with a highly variable clinical presentation and natural history. Given its vascular origin, new therapies with inhibitors of vascular endothelial growth factor (VEGF) have been introduced in the treatment of these patients and have shown promising results. Few reports have described the role of F-Fluorodeoxyglucose positron emission tomography/contrast-enhanced computed tomography (F-FDG PET/CT) in the evaluation of this tumor after treatment with anti-angiogenic agents. Our case reports how F-FDG PET-CT scan was critical in the assessment of this tumor after treatment with an anti-angiogenic agent, Pazopanib, demonstrating complete metabolic response.. A 30-year-old man with no previous significant medical history presented with pain in the right upper quadrant for over a year.. Multiple hepatic masses were found on abdominal ultrasound. Liver biopsy confirmed the diagnosis of epithelioid hemangioendothelioma. F-FDG PET/CT was performed for staging. Multiple FDG-avid hepatic, splenic, and lymph nodes lesions were detected on F-FDG PET/CT. A subsequent spleen biopsy confirmed splenic involvement. Immunohistochemistry was positive for CD31, CD34, and ERG, supporting the diagnosis of epithelioid hemangioendothelioma.. A 1-year cyclophosphamide treatment was provided followed by Pazopanib for 17 months.. Six years after the first F-FDG PET/CT, F-FDG PET/CT performed for restaging demonstrated complete metabolic response to therapy. Follow-up CT demonstrated no interval changes in size of some of the treated lesions.. F-FDG PET/CT is useful for baseline assessment and posttreatment follow-up of this rare cancer.

Topics: Adult; Angiogenesis Inhibitors; Antineoplastic Agents; Fluorodeoxyglucose F18; Hemangioendothelioma, Epithelioid; Humans; Indazoles; Liver Neoplasms; Male; Positron Emission Tomography Computed Tomography; Pyrimidines; Radiopharmaceuticals; Sulfonamides; Treatment Outcome