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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.

journal_name

Ann Vasc Surg

journal_title

Annals of vascular surgery

authors

Rojas Esquivel DJ,Marín Manzano E,Concepción NA,Lopéz Gutierréz JC,Riera de Cubas L

doi

10.1016/j.avsg.2016.06.015

subject

Has Abstract

pub_date

2017-01-01 00:00:00

pages

321.e1-321.e4

eissn

0890-5096

issn

1615-5947

pii

S0890-5096(16)30712-9

journal_volume

38

pub_type

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