- Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston
- Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston
- Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, Boston
Osama A. Jamil
Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston
DOI:10.4103/2152-7806.103878Copyright: © 2012 Jamil OA. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Jamil OA, Lechpammer M, Prasad S, Litvack Z, Dunn IF. Giant cell reparative granuloma of the sphenoid: Case report and review of the literature. Surg Neurol Int 27-Nov-2012;3:140
How to cite this URL: Jamil OA, Lechpammer M, Prasad S, Litvack Z, Dunn IF. Giant cell reparative granuloma of the sphenoid: Case report and review of the literature. Surg Neurol Int 27-Nov-2012;3:140. Available from: http://sni.wpengine.com/surgicalint_articles/giant-cell-reparative-granuloma-of-the-sphenoid-case-report-and-review-of-the-literature/
Background:Giant cell reparative granulomas (GCRGs) are rare lesions in the cranial bones. We present a case of this rare lesion emanating from the clivus and replacing the sphenoid sinus, a highly unusual location for this entity.
Case Description:The case and clinical course of a 29-year-old female who presented with a large sphenoid mass are described here. The patient presented with symptoms of severe headache and diplopia; imaging demonstrated a large sphenoid mass which was completely resected via an endoscopic endonasal approach. It was based on the clivus and was shown to be a GCRG.
Conclusion:GCRGs are benign granulomatous lesions which should be considered in the differential in the setting of a sphenoid mass.
Keywords: Clivus, diplopia, giant cell reparative granuloma, giant cell tumor, neurofibromatosis-1
Giant cell reparative granulomas (GCRGs) are benign osteolytic non-neoplastic granulomatous lesions of bone which occur most commonly in the maxilla and mandible in children and young adults.[
This entity has to be pathologically distinguished from a giant cell tumor (GCT) which is a true neoplasm. Other bone lesions to be considered in the cranial and facial bones include aneurysmal bone cyst (ABC), fibrous dysplasia, chondroblastoma, osteosarcoma, cherubism, and brown tumor of hyperthyroidism. Management options in the literature have included gross total resection, curettage, radiation, and calcitonin therapy.[
We present a very unusual case of a 29-year-old female presenting with severe headache and diplopia found to have GCRG based on the clivus and involving the entire sphenoid sinus.
A previously healthy 29-year-old female developed two months of progressively worsening headaches. She had been treated with amitriptyline and sumatriptan, and with antibiotics for presumed sinusitis. Ten days before presentation, she developed horizontal binocular diplopia, initially occurring at the end of the day, and then becoming more persistent. She did not describe visual loss in either eye. She was evaluated in our emergency room.
Her neurologic examination was notable for her eye examination. On examination, the visual acuity without correction was 20/20. Color vision and confrontation visual fields were normal. The pupils reacted normally without anisocoria or an afferent pupillary defect. There were slight bilateral abduction deficits, greater on the left. Alternate cover testing revealed a 6 prism diopter esophoria in primary gaze which increased to 8 prism diopters in right gaze and 10 prism diopters in left gaze. The abducting saccades were slowed bilaterally, greater on the left. There was no nystagmus. Examination of the fundus revealed normal optic nerves without pallor or swelling. In summary, the patient had partial bilateral sixth nerve palsies causing binocular horizontal diplopia.
Her laboratory panel was normal, showing no abnormalities of calcium metabolism or pituitary hormones.
Imaging revealed a large mass occupying the sella turcica, sphenoid sinus and encroached upon the prepontine cistern in displacing the clival dura posteriorly. Computed tomography (CT) revealed a heterogeneous lesion causing bony erosion of the dorsum sella and clivus. The infundibulum was minimally deviated to the right and normal pituitary appeared elevated and was seen underneath the optic chiasm. On magnetic resonance imaging (MRI), the lesion was T1 isointense with moderate contrast enhancement [Figures
She underwent an endoscopic endonasal transsphenoidal resection of this lesion so that a diagnosis could be established, and symptomatic relief was provided by complete resection. A mass emerging from the right sphenoid ostium was immediately appreciated during the sphenoidotomy. Similar findings were observed in the left sphenoid ostium, though the face of the sphenoid had not been eroded. The mass, however, filled the entire sinus. A frozen section suggested a reactive and non-neoplastic process. Therefore, it was felt that surgical resection should be undertaken in this young patient for immediate symptomatic improvement and removal of the offending process. It was highly vascular and was dissected from the roof, walls, and floor of the sphenoid sinus. The sellar floor, superior clivus, and posterior clinoids had been partially eroded and the mass was highly adherent to the clival dura; the tumor did not appear to be emanating from the pituitary as the sellar dura was intact. The mass ultimately was entirely extradural, with no dural violation and no intradural cerebrospinal fluid (CSF) leak. It was most adherent to the clival dura. Macroscopically, a gross total resection was achieved as the tumor was resected off the dura. The MRI showed gross total resection [Figures
Postoperatively, she recovered well and her headaches and diplopia resolved within a month after surgery.
Histopathological examination demonstrated a large number of osteoclast-like, multinucleated giant cells within a background of mononuclear stromal cells and spindle-shaped fibroblasts associated with areas of hemorrhage. The final histopathological diagnosis was consistent with GCRG [
Histological features of giant cell reparative granuloma; a: Multinucleated giant cells dispersed within fibroblastic matrix, hematoxylin and eosin (H and E)-stained section; original magnification ×100; b and C Osteoclast-like giant cells , H and E-stained section and smear; original magnification ×200; bar =200 um (panels B and C)
Her symptoms resolved soon after surgery. She was lost to follow-up in the short term, but we were able to see her again at six months. Although the patient was asymptomatic, we performed a routine surveillance MRI six months after surgery, given the rarity of the lesion, which showed a recurrence of the mass [
GCRG is a benign granulomatous lesion of the bone. It is a locally aggressive lesion. This entity occurs most commonly in children or in young adults aged 10-30, with an increased preponderance for females. It was initially described by Jaffe in 1954.[
The etiology is of GCRG is unclear. Jaffe, who initially described the lesion, believed that this lesion was caused by trauma,[
Symptoms depend on the location. GCRG in the cranial bones presents symptomatically as hearing loss, tinnitus, facial weakness, and dysphagia. The temporal bone is the most common cranial site for GCRG; lesions here may present with headache, a palpable mass, tinnitus, vertigo, localized pain, or hoarseness, dysphagia, and conductive hearing impairment. Sphenoid bone lesions present with diplopia, vision changes, and headache.[
Radiological findings are nonspecific and it is difficult to distinguish between GCT and GCRG; CT scan will reveal lytic areas. MRI reveals an area of low intensity on both T1- and T2-weighted images which indicate fibrosis or hemosiderin.[
The management of GCRG is usually surgical. The rate of recurrence after gross total resection is about 10-20%.[
The diagnosis of GCRG is a pathologic one; other lesions in its differential diagnosis include true GCT, brown tumor of hyperparathyroidism, ABC, chondroblastoma, fibrous dysplasia, osteosarcoma, and cherubism.[
It is particularly important to exclude GCT. GCTs are benign locally aggressive tumors. They have an incidence of 3-7%. Only 2% involve the skull.[
GCRG is a benign non-neoplastic granulomatous lytic lesion of the bone. Its etiology is clearly unknown but maybe traumatic with some role for infection or inflammatory processes. Differential diagnosis includes GCT, ABC, chondroblastoma, and brown cell tumor of the bone. Management includes surgical (either as gross total resection or curettage) or nonsurgical (radiation, calcitonin, and anti-inflammatory drugs) modalities. Nonsurgical modalities should be considered for lesions that cannot be completely resected. Although the recurrence rate after total resection is modest (10-20%), close follow-up is warranted. To our knowledge, this is the first known case of GCRG based in the clivus.
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