Histopathological and Clinical Characteristics of Surgically Removed Cavernous Venous Malformations (so-called Cavernous Hemangiomas) of the Orbit

Victor Vlad Costan,Andrei Ionut Cucu,Anca Sava,Gheorghe Raftu,Alexandru Carauleanu,Gabriela Dimitriu,Maria Silvia Stoicescu,Gabriela Florenta Dumitrescu,Nicoleta Dumitrescu,Claudia Florida Costea,Mihaela Dana Turliuc

doi:10.37358/rc.19.1.6914

Abstract

Cavernous venous malformation (CVM) is the most common primary vascular lesion of the orbit. The aim of this study is to present authors �experience regarding fourteen patients with orbital CVM (so-called cavernous hemangioma) and identify their clinical and histopathological particular characteristics. From 2010 to 2017 a total of 14 patients were diagnosed with orbital cavernous hemangiomas and treated by the authors. Patients� socio-demographic, clinical, radiological, and surgical findings were retrospectively retrieved from medical records and analyzed. Diagnosis of a cavernous hemangioma was confirmed histologically in all cases. Results: All 14 patients were females, with a mean age of 44.2 years old, ranging from 13 years to 57 years. The left orbit was affected in eleven (78.57%) cases. A painless, gradually progressive proptosis and visual disturbance were the main clinical signs. In four (28.57%) cases, the lesion was incidentally discovered at imaging evaluations for persistent headache. All patients were accurately diagnosed preoperatively based on computed tomography (CT) exam or magnetic resonance imaging (MRI). The tumors were removed successfully by anterior orbitotomy. Transconjunctival approach proved to be an important surgical procedure in their treatment, with excellent results and a low risk of complications. Histologically, all lesions revealed large vascular channels with endothelium lining and multilaminar smooth muscle layers in their walls, but also nests of small capillary channels in interstitium. No intravascular thrombosis or stromal lymphocytes could be seen. Orbital cavernous hemangiomas presented clinical, imaging and histological features of a benign lesion. Having to deal with extraconal lesions, we successfully used anterior orbitotomy, mainly the transconjuctival approach, with no complications or recurrences after a six-month follow-up period. Histologically, all lesions revealed images being most closely to dysplastic veins. So, we concluded that the so-called orbital cavernous hemangioma was rather a cavernous venous malformation.

Highlights

VICTOR VLAD COSTAN1, ANCA SAVA2,7*, ALEXANDRU CARAULEANU3*, CLAUDIA FLORIDA COSTEA4,6, ANDREI IONUT CUCU5, GABRIELA DIMITRIU6, GABRIELA FLORENTA DUMITRESCU7, NICOLETA DUMITRESCU1, MARIA SILVIA STOICESCU8, GHEORGHE RAFTU9, MIHAELA DANA TURLIUC5,10
At magnetic resonance imaging (MRI) the aspect of the tumor was similar to computed tomography (CT) images, but the signal in T1 was isointense compared to the muscle, and in T2 hyperintense compared to the muscle
Our series of cases demonstrated that orbital cavernous hemangiomas express clinical and imaging features of a displace, compress, or incorporate surrounding structures

Summary

Introduction

VICTOR VLAD COSTAN1, ANCA SAVA2,7*, ALEXANDRU CARAULEANU3*, CLAUDIA FLORIDA COSTEA4,6, ANDREI IONUT CUCU5, GABRIELA DIMITRIU6, GABRIELA FLORENTA DUMITRESCU7, NICOLETA DUMITRESCU1, MARIA SILVIA STOICESCU8, GHEORGHE RAFTU9, MIHAELA DANA TURLIUC5,10. Orbital cavernous hemangiomas presented clinical, imaging and histological features of a benign lesion. Osaki et al (2013) have shown these kind of vascular lesion are characterized by a virtual absence of Ki-67 positivity [2] This is a non-infiltrative, slowly progressive lesion, symptomatic in middle-aged patients, especially women [3], and the stimulation in their growth during pregnancy reported in studies [4- 6] seems to suggest hormonal influence. The location of most of these malformations is typically intraconal and lateral in location but rarely it can develop in extraconal areas [7-11] This malformation is a unique lesion, but Wang and Yan described four cases of multiple cavernous hemangiomas of the orbit in their series of 214 patients with this pathology [12]. Shields et al (2000) reported a case with bilateral cavernous haemangiomas of the orbit [13]

Objectives

Methods

Results

Conclusion