Gorham-Stout disease with involvement of the jaws: a systematic review.

Abstract:

:The purpose of this study was to systematically review all published cases of Gorham-Stout disease (GSD) involving the jaws and to identify the clinico-radiological and histopathological features associated with persistence of the lesions, as well as the best treatment options available. An electronic search was undertaken in November 2018. Eligibility criteria included publications with sufficient information to confirm the diagnosis. Eighty-six publications reporting 89 cases were included. Features observed included symptomatic disease (51.1%), swelling (34.1%), pathological fracture (31.8%), history of previous trauma (32.1%), high alkaline phosphatase levels (24.3%), and predominance of vascular tissue (72.4%). Nearly a quarter of the patients were only followed up, with no treatment implemented. Most treatments consisted of some type of surgery with/without additional therapies (42.0%), drugs (20.5%), and radiotherapy (14.8%). Half of the cases were found to persist after some treatment modality, and five patients died. Among the variables investigated, only a lesion crossing the midline showed an association with persistence of the disease. There remains much to understand about GSD, a rare condition with no clear consensus on the aetiopathology, an unpredictable clinical course, and no standard treatment. The high rate of persistence after treatment was found to be associated only with the lesion crossing the midline.

authors

Chrcanovic BR,Gomez RS

doi

10.1016/j.ijom.2019.03.002

subject

Has Abstract

pub_date

2019-08-01 00:00:00

pages

1015-1021

issue

8

eissn

0901-5027

issn

1399-0020

pii

S0901-5027(19)30107-9

journal_volume

48

pub_type

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