A report of two children with Gorham-Stout disease

Mario Edgar Tena-Sanabria,Félix Alejandro Álvarez-Martínez,Juan Carlos Núñez-Enríquez,Gabriela Fuentes-Herrera,Larisa Yarindy Jesús-Mejenes,Nora Patricia Victorio-García

doi:10.1186/s12887-019-1561-0

Mario Edgar Tena-Sanabria, Félix Alejandro Álvarez-Martínez + Show 4 more

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https://doi.org/10.1186/s12887-019-1561-0

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Abstract

BackgroundGorham-Stout disease is a rare condition characterized by unifocal and massive type IV osteolysis (variant of idiopathic nonhereditary osteolytic disease) with a slow progression, which is self-limiting for some years. It is characterized by recurrent vascular tumors with disruption of the anatomical architecture and intraosseous proliferation of vascular channels that leads to the destruction and resorption of the bone matrix. The aim of this study is to present the clinical features of this disease, as well as the importance of prompt diagnosis and treatment, with a review of the reported cases.Case reportsWe describe two cases of Gorham-Stout disease between 2013 and 2017 with surgical interventions, follow-up and results. Case one involves an 11-year-old male with involvement of the left iliac bone, with adequate evolution after a surgical procedure with a lyophilized cadaveric tricortical bone allograft. Case two involves a 6-year-old male with cervical spine C1-C3 repercussion; in the protocol for surgical treatment, he presented with signs of spinal cord compression and died.ConclusionDiagnosis of Gorham-Stout disease is made by exclusion, and its clinical presentation varies widely, from spontaneous remission to a fatal outcome.

Highlights

Gorham-Stout disease is a rare condition characterized by unifocal and massive type IV osteolysis with a slow progression, which is self-limiting in some years
It is characterized by recurrent vascular tumors with disruption of the anatomical architecture and intraosseous proliferation of vascular channels that leads to the destruction and resorption of the bone matrix [1, 2]
The clinical course varies from being self-limiting in some years, with mild manifestations, to a fatal outcome such as the second case study we presented here, where the patient died as a consequence of spinal cord compresion [22]

Summary

Introduction

Gorham-Stout disease is a rare condition characterized by unifocal and massive type IV osteolysis (variant of idiopathic nonhereditary osteolytic disease) with a slow progression, which is self-limiting for some years It is characterized by recurrent vascular tumors with disruption of the anatomical architecture and intraosseous proliferation of vascular channels that leads to the destruction and resorption of the bone matrix. Gorham-Stout disease is a rare condition characterized by unifocal and massive type IV osteolysis (a type of idiopathic nonhereditary osteolytic disease) with a slow progression, which is self-limiting in some years It is characterized by recurrent vascular tumors with disruption of the anatomical architecture and intraosseous proliferation of vascular channels that leads to the destruction and resorption of the bone matrix [1, 2]. Histopathological findings include lymphatic and vascular tissue in the bone and D2–40 immunohistochemistry positivity, with a sensitivity of 92.6% and specificity of 98.8% [9]

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