Targeting VEGF-VEGFR Pathway by Sunitinib in Peripheral Primitive Neuroectodermal Tumor, Paraganglioma and Epithelioid Hemangioendothelioma: Three Case Reports - PubMed (original) (raw)

Case Reports

Targeting VEGF-VEGFR Pathway by Sunitinib in Peripheral Primitive Neuroectodermal Tumor, Paraganglioma and Epithelioid Hemangioendothelioma: Three Case Reports

Tiziana Prochilo et al. Case Rep Oncol. 2013.

Abstract

Sunitinib malate (Sutent(TM); Pfizer Inc., New York, N.Y., USA) is a small molecule kinase inhibitor with activity against a number of tyrosine kinase receptors, including vascular endothelial growth factor receptors, stem-cell factor receptor, and platelet-derived growth factor receptors alpha and beta. Sunitinib, registered for the treatment of renal cell carcinoma and gastrointestinal stromal tumors, has recently been approved for the treatment of patients with advanced pancreatic neuroendocrine tumors. Peripheral primitive neuroectodermal tumor (pPNET), paraganglioma (PGL) and epithelioid hemangioendothelioma (EHE) are rare tumors in which there is an overexpression of pro-angiogenic factors and in which a high intratumoral microvessel density is a significant poor prognostic factor. On the basis of this preclinical rationale and the lack of effective treatments in pre-treated advanced stages of these rare diseases, we report our interesting experience of pPNET, PGL and EHE treatment with sunitinib.

Keywords: Antiangiogenic therapy; Epithelioid hemangioendothelioma; Paraganglioma; Peripheral primitive neuroectodermal tumor; Sunitinib.

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Figures

Fig. 1

Fig. 1

a CT scan shows a solid lesion with compression of the right-side profile of VI and VIII hepatic segments attributable to recurrent disease. b After 2 courses of sunitinib treatment, a CT scan documents a change in the consistency of the lesion, which appear predominantly fluid.

Fig. 2

Fig. 2

a PET scan pretreatment showing the appearance of a new right iliac bone lesion. b PET scan after treatment documents response to sunitinib.

Fig. 3

Fig. 3

a CT scan shows bilateral lung metastasis. b After 2 months of sunitinib treatment, a CT scan documents a partial response to treatment with the disappearance of some pulmonary nodules.

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