Giant cell reparative granuloma (GCRG) is a rare fibroosseous lesion uncommonly seen in the orbital area. Although benign, it is known to be recurrent and locally destructive. We report two cases of GCRG of the orbit. In both cases, computed tomography revealed a heterogeneously growing well-defined mass, arising from the roof of the orbit, affecting the cortex, and invading the orbit. In the first case, the mass extended into the anterior cranial fossa. Magnetic resonance imaging with gadolinium showed, in both cases, a cystic character of the lesion with fluid levels. The surgical treatment was performed via an upper crease incision. An ultrasonic aspirator system was used to remove the tumor tissue and its extension into cranial fossa. Careful histopathologic analysis established the diagnosis of GCRG. Symptoms resolved completely with no evidence of recurrence after a follow-up of 18 and 14 months, respectively. We present the clinicopathological and radiological findings, and we describe the surgical approach. As a rare entity, GCRG of the orbit should be considered in differential diagnosis of fibroosseous orbital masses. Complete surgical excision carries a low risk of recurrence.
Giant cell reparative granuloma (GCRG) is an uncommon osteolytic lesion that typically develops in the jawbone [
It was Jaffe [
It is included within a group of orbital fibroosseous lesions, among which are the osteoma, ossifying fibroma, fibrous dysplasia, osteoblastoma, osteoclastoma, brown tumor of hyperparathyroidism, and the aneurysmal bone cyst [
We have found 10 cases of GCRG with orbital involvement published in the medical literature [
We present two cases of orbital GCRG, one of them with extension into the anterior cranial fossa. We describe the clinical, histological, and radiological characteristics and the surgical approach to removing them using an ultrasonic surgical aspirator device.
A 37-year-old male presented with proptosis, fullness of the right upper eyelid, and inferior displacement of the right eye that had progressed gradually over several months. There was no history of pain, loss of visual acuity (VA), or diplopia. Ocular motility examination revealed a slight limitation of motion in supraduction. The patient reported no previous episodes of inflammation, sinus infection, or trauma. Fundus examination was normal. General physical examination and routine blood tests were within normal limits. Contrast-enhanced coronal and sagittal computed tomography (CT) images showed a lytic lesion arising from the roof of the right orbit, with heterogeneous captation, relatively hypocaptant with hypodense areas, and small foci of mineralization within the lesion. The mass had a thinning effect on the superior wall of the roof, without breaking it, and extended over the anterior cranial fossa. It also disrupted the inferior wall of the roof and invaded the orbit, displacing the superior rectus muscle and the globe inferiorly (Figures
(a, b) Contrast-enhanced coronal and sagittal CT images, demonstrating an expansive lesion arising from the roof of the right orbit with a heterogeneous enhancement and soft tissue attenuation. Inside view of the lesion reveals several small foci of mineralization (arrow). The lesion shows the osseous expansive changes, with thinning of superior and inferior wall of the roof which leads to an invasion over the frontal sinus and into the orbit. (c) Coronal T2-weighted MRI shows a well-defined lesion with a low-signal-intensity margin representing either osseous sclerosis or a pseudocapsule. The lesion shows a multilobulated lytic pattern which reveals markedly increased signal intensity, reflecting the expansive cystic component, and low signal intensity in the small solid regions. (d) Sagittal postcontrast T1-weighted MR image shows a well-encapsulated mass with homogenous contrast enhancement.
(a) Surgical approach through an upper eyelid crease incision to access the orbital roof. Intraoperative photograph showing bone destruction in the roof of the orbit and the presence of a red-yellowish mass, with evidence of dark coagulated blood and fragments of bone within the soft tissue. The tumor extends into the anterior cranial fossa. (b) The handpiece of SONOPET® ultrasonic aspirator used for aspiration and emulsification of the tumor tissue in the superior orbit and its extension into cranial cavity. (c) Resection of abnormal tissue from the upper orbit and its extension into anterior fossa.
A 39-year-old male was referred to our oculoplastic service complaining of gradually progressive proptosis, and right upper eyelid swelling. The VA and ocular motility examination were normal. He had previous episodes of sinusitis and endoscopic surgical repair of the right frontal sinus. Orbital CT revealed a cystic mass measuring
(a, b) Coronal and sagittal CT images, demonstrating an expansive lesion arising from the roof of the right orbit with soft tissue attenuation. Small foci of mineralization (arrow) into the lesion are presented. The lesion protrudes into the orbit. (c) Coronal T2-weighted MR image shows a well-defined lesion arising from the bone with an extraosseous component. The majority of the tumor has high signal intensity on the T2-weighted image, with low signal areas which represent the solid component of the tumor. (d) Volume-rendered 3D-CT reconstruction images show lytic areas with bone destruction in the roof of the orbit.
(a, b) Histopathology (hematoxylin and eosin stain (40x) showing multinucleated foreign body giant cells (cholesterol crystals) with xanthomized cells, presence of hemosiderin deposition, and lymphocytic inflammatory infiltrate. Multinucleated giant cells (thick blue arrows), cholesterol crystals (short green arrows), xanthomized cells (wavy black arrows), and hemosiderin deposits (fine yellow arrow).
GCRG of the orbit is a benign fibrous lesion, although it can expand aggressively and can be locally destructive [
The etiology of this tumor remained unclear [
GCRG occurs more frequently in women and in the first two decades of life [
The clinical signs that our patients mainly manifested were proptosis, upper eyelid fullness, periorbital inflammation, lower displacement of the globe, and impaired extraocular movements; these signs and symptoms are in line with those described in the medical literature [
Most of the published cases of GCRG of the orbit affected the roof and/or the lateral orbital wall, as in our cases, but it can also affect the inferior, central, and medial portion of the orbit (Table
Giant cell reparative granuloma cases involving the orbit.
Authors | Number cases | Side, location in the orbit |
---|---|---|
Bengoa-González A et al., current study | 2 | Right, superior, one extending anterior cranial fossa |
2013, Chawla et al. [ | 1 | Right, lateral |
2005, Pherwani et al. [ | 1 | Left, superomedial |
2005, D’Ambrosio et al. [ | 1 | Left, optic strut, and anterior clinoid process |
2003, Font et al. [ | 1 | Bilateral, lateral, and inferior |
1999, Mercado et al. [ | 1 | Left, lateral and posterior, and sphenoid bone |
1988, Rootman et al. [ | 1 | No data, superior extending anterior cranial fossa |
1985, Sebag et al. [ | 1 | Right, superolateral, and roof |
1984, Scully et al. [ | 1 | Right, posterior, lateral, and central |
1981, Hoopes et al. [ | 1 | Right, superior, lateral, and posterior |
1967, Sood et al. [ | 1 | Left, medial, and ethmoid sinus |
Treatment of GCRG is surgical excision, which is usually done with total resection or local curettage [
Imaging findings of GCRG are not specific, thus making it very difficult to distinguish from other osteolytic bone lesions [
Given the location and extent of the mass, an upper eyelid crease approach was used to access the superior orbital space, the orbital roof, and cranial fossa extension. Intraoperatively, bone destruction of the orbital walls was noted, and detached bone fragments were observed among soft tissues.
On gross inspection, the tumor may appear as a friable red-bluish mass [
The GCGR of the orbit is very similar to the brown tumor of hyperparathyroidism, although in the latter the serum levels of calcium are usually high and those of phosphorus are low [
It is important to distinguish GCRG from other tumors, such as osteoclastoma, also known as giant cell tumor, for management and prognosis. Osteoclastoma is a giant cell tumor within the bone [
Eosinophilic granuloma is the bone variant of histiocytosis X or Langerhans cell histiocytosis and generally occurs in the first decade of life [
In both cases, an ultrasonic aspirator system was used to remove the tumor. The SONOPET® ultrasonic aspirator is a handheld surgical tool that allows access to small operative fields, such as the orbit [
This ultrasonic system allowed us to sculpt the bone precisely, when an improvement of visibility was needed, as in the first case presented, and to remove the abnormal tissue, regardless of its consistency, with no traction nor sharp excision. It enables work near areas such as the dura mater without damaging the adjacent tissues.
We think, as do other authors [
The recurrence rate of these tumors can be between 10 and 15% of the cases that have been incompletely removed, as has been seen in other published cases [
In conclusion, the orbital GCGR, although rare, should be included in the differential diagnosis of fibroosseous lesions or other orbital masses. Although it is more frequent in young or middle-aged patients it must be considered in older patients too. The surgical resection of this lesion is usually possible and can be curative, relieving ocular symptoms.
The data that support the findings of this study are available from the corresponding author, EM-G, upon reasonable request.
All procedures performed in this study involving human participants were in accordance with ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or compatible ethical standards.
Informed consent was obtained from all individual participants included in the study.
The authors have no financial or conflicts of interest to disclosure.