title: Long-term durable response to lenalidomide in a patient with hepatic epithelioid hemangioendothelioma.
abstract: Epithelioid hemangioendothelioma (EH) is a rare tumor arising from the vascular endothelial cells of soft tissue or visceral organs. The most common visceral site is the liver, where it is often involved in a multifocal manner known as hepatic EH (HEH). Surgical resection with curative intent represents the gold standard therapy. When surgery is not feasible, or in cases of metastatic disease, no standard medical treatment is currently indicated. In small series, drugs with anti-angiogenic activity (such as bevacizumab, sorafenib, thalidomide, and lenalidomide) have been proposed with promising results. We describe a 73-year-old man with multifocal non-resectable HEH treated with lenalidomide. Disease status was evaluated by abdominal ultrasound and magnetic resonance every four months. The patient was treated for a total of 39 mo with prolonged disease stabilization and, at the time of writing, is still under treatment with a good tolerance profile. During a short period of treatment discontinuation, the disease showed slight progression that immediately resolved after the reintroduction of lenalidomide. Lenalidomide may represent a valid treatment option for HEH due to its anti-angiogenic and antineoplastic activities. This preliminary result merits further study in a large series.
authors.affiliation: Maria Caterina Pallotti, Margherita Nannini, Anna Mandrioli, Cristian Lolli, Maristella Saponara, Guido Biasco, Maria Abbondanza Pantaleo, Department of Specialized, Experimental, and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40138 Bologna, Italy.
authors.forename: Maria Caterina
authors.initials: MC
authors.lastname: Pallotti
authors.affiliation: Maria Caterina Pallotti, Margherita Nannini, Anna Mandrioli, Cristian Lolli, Maristella Saponara, Guido Biasco, Maria Abbondanza Pantaleo, Department of Specialized, Experimental, and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40138 Bologna, Italy.
authors.forename: Margherita
authors.initials: M
authors.lastname: Nannini
authors.affiliation: Maria Caterina Pallotti, Margherita Nannini, Anna Mandrioli, Cristian Lolli, Maristella Saponara, Guido Biasco, Maria Abbondanza Pantaleo, Department of Specialized, Experimental, and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40138 Bologna, Italy.
authors.forename: Claudio
authors.initials: C
authors.lastname: Agostinelli
authors.affiliation: Maria Caterina Pallotti, Margherita Nannini, Anna Mandrioli, Cristian Lolli, Maristella Saponara, Guido Biasco, Maria Abbondanza Pantaleo, Department of Specialized, Experimental, and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40138 Bologna, Italy.
authors.forename: Simona
authors.initials: S
authors.lastname: Leoni
authors.affiliation: Maria Caterina Pallotti, Margherita Nannini, Anna Mandrioli, Cristian Lolli, Maristella Saponara, Guido Biasco, Maria Abbondanza Pantaleo, Department of Specialized, Experimental, and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40138 Bologna, Italy.
authors.forename: Valerio Di
authors.initials: VD
authors.lastname: Scioscio
authors.affiliation: Maria Caterina Pallotti, Margherita Nannini, Anna Mandrioli, Cristian Lolli, Maristella Saponara, Guido Biasco, Maria Abbondanza Pantaleo, Department of Specialized, Experimental, and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40138 Bologna, Italy.
authors.forename: Anna
authors.initials: A
authors.lastname: Mandrioli
authors.affiliation: Maria Caterina Pallotti, Margherita Nannini, Anna Mandrioli, Cristian Lolli, Maristella Saponara, Guido Biasco, Maria Abbondanza Pantaleo, Department of Specialized, Experimental, and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40138 Bologna, Italy.
authors.forename: Cristian
authors.initials: C
authors.lastname: Lolli
authors.affiliation: Maria Caterina Pallotti, Margherita Nannini, Anna Mandrioli, Cristian Lolli, Maristella Saponara, Guido Biasco, Maria Abbondanza Pantaleo, Department of Specialized, Experimental, and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40138 Bologna, Italy.
authors.forename: Maristella
authors.initials: M
authors.lastname: Saponara
authors.affiliation: Maria Caterina Pallotti, Margherita Nannini, Anna Mandrioli, Cristian Lolli, Maristella Saponara, Guido Biasco, Maria Abbondanza Pantaleo, Department of Specialized, Experimental, and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40138 Bologna, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Pileri
authors.affiliation: Maria Caterina Pallotti, Margherita Nannini, Anna Mandrioli, Cristian Lolli, Maristella Saponara, Guido Biasco, Maria Abbondanza Pantaleo, Department of Specialized, Experimental, and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40138 Bologna, Italy.
authors.forename: Luigi
authors.initials: L
authors.lastname: Bolondi
authors.affiliation: Maria Caterina Pallotti, Margherita Nannini, Anna Mandrioli, Cristian Lolli, Maristella Saponara, Guido Biasco, Maria Abbondanza Pantaleo, Department of Specialized, Experimental, and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40138 Bologna, Italy.
authors.forename: Guido
authors.initials: G
authors.lastname: Biasco
authors.affiliation: Maria Caterina Pallotti, Margherita Nannini, Anna Mandrioli, Cristian Lolli, Maristella Saponara, Guido Biasco, Maria Abbondanza Pantaleo, Department of Specialized, Experimental, and Diagnostic Medicine, S. Orsola-Malpighi Hospital, University of Bologna, 40138 Bologna, Italy.
authors.forename: Maria Abbondanza
authors.initials: MA
authors.lastname: Pantaleo
mesh_terms: D000368:Aged; D020533:Angiogenesis Inhibitors / Q000627:therapeutic use*; D018952:Antigens, CD34 / Q000032:analysis; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D018323:Hemangioendothelioma, Epithelioid / Q000098:blood supply* / Q000737:chemistry / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D000077269:Lenalidomide; D008113:Liver Neoplasms / Q000098:blood supply* / Q000737:chemistry / Q000188:drug therapy* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D013792:Thalidomide / Q000031:analogs & derivatives* / Q000627:therapeutic use; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D020533:Angiogenesis Inhibitors; D018952:Antigens, CD34; D014408:Biomarkers, Tumor; D013792:Thalidomide; D000077269:Lenalidomide
keywords: Abdominal ultrasound; Hepatic epithelioid hemangioendothelioma; Lenalidomide; Liver; Magnetic resonance