title: Nodular Proliferation in Parkes Weber Syndrome.
abstract: Parkes Weber syndrome (PWS) is characterized by the association of high flow vascular malformation and overgrowth of a part of the body, usually a limb. In a previous review of 10 patients with PWS from our hospital we described a case of congenital short femur and four cases of severe lymphedema. We present a case of PWS associated with a nodular proliferative form not previously described. A 38 year old male with diagnosis of PWS with involvement of the right lower limb (RLL) was derived to our clinic. He complained about the appearance of painful nodular tumors in his RLL and some episodes of bleeding through the tumors. The physical examination revealed increased size of the RLL compared to left lower limb. Two nodular tumors were evident in his RLL. One located proximal in the leg and another one in ankle. The computed tomographic angiography revealed multiple arterio-venous shunts in the RLL. The tumors were not arterio-venous shunts, neither aneurysms. We decided to make surgical resection of the tumors. In the pathology analysis the tumors were positive for CD31, CD34 and negative for D240 markers. Eight months after surgery the patient had no recurrence of the tumors, and he is asymptomatic.The presence of nodular tumors in PWS has not been previously described. This makes us to think that these could be hamartomatous lesions similar to those of the CLOVES syndrome or a PIK3CA mutation.
authors: Department of Vascular Surgery, La Paz University Hospital, Madrid, Spain. Electronic address: doctorojas@gmail.com.
authors: Diego José
authors: DJ
authors: Rojas Esquivel
authors: Department of Vascular Surgery, La Paz University Hospital, Madrid, Spain.
authors: Elena
authors: E
authors: Marín Manzano
authors: Department of Vascular Surgery, La Paz University Hospital, Madrid, Spain.
authors: Nieves Aleicel
authors: NA
authors: Concepción
authors: Department of Pediatric Surgery, La Paz University Hospital, Madrid, Spain.
authors: Juan Carlos
authors: JC
authors: Lopéz Gutierréz
authors: Department of Vascular Surgery, La Paz University Hospital, Madrid, Spain.
authors: Luis
authors: L
authors: Riera de Cubas
mesh_terms: D000328:Adult; D058846:Antibodies, Monoclonal, Murine-Derived; D018952:Antigens, CD34 / Q000276:immunology; D001158:Arteries / Q000737:chemistry / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D001165:Arteriovenous Malformations / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D001706:Biopsy; D049109:Cell Proliferation; D000072226:Computed Tomography Angiography; D006801:Humans; D007150:Immunohistochemistry; D035002:Lower Extremity / Q000098:blood supply*; D008297:Male; D019408:Platelet Endothelial Cell Adhesion Molecule-1 / Q000032:analysis; D013341:Sturge-Weber Syndrome / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures; D014680:Veins / Q000737:chemistry / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D018952:Antigens, CD34; D019408:Platelet Endothelial Cell Adhesion Molecule-1; C500987:monoclonal antibody D2-40