- Department of Neurosurgery, Oakland University William Beaumont School of Medicine, Royal Oak, Michigan, USA
- Department of Plastic and Reconstructive Surgery, Oakland University William Beaumont School of Medicine, Royal Oak, Michigan, USA
- Department of Pathology, Oakland University William Beaumont School of Medicine, Royal Oak, Michigan, USA
Daniel K. Fahim
Department of Neurosurgery, Oakland University William Beaumont School of Medicine, Royal Oak, Michigan, USA
DOI:10.4103/2152-7806.151393Copyright: © 2015 Le J. 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: Le J, Chaiyasate K, Donev K, Fahim DK. A rare case of giant cell tumor involving the clivus resected through Le Fort I Osteotomy and median maxillotomy. Surg Neurol Int 13-Feb-2015;6:26
How to cite this URL: Le J, Chaiyasate K, Donev K, Fahim DK. A rare case of giant cell tumor involving the clivus resected through Le Fort I Osteotomy and median maxillotomy. Surg Neurol Int 13-Feb-2015;6:26. Available from: http://sni.wpengine.com/surgicalint_articles/a-rare-case-of-giant-cell-tumor-involving-the-clivus-resected-through-le-fort-i-osteotomy-and-median-maxillotomy/
Background:Giant cell tumors (GCTs) are bone tumors that seldom involve the skull. Skull GCTs preferentially occur in the sphenoid and temporal bones with few reported cases involving the clivus. Due to the rarity and complex location, surgical management is not well established for clival GCTs.
Case Description:A 49-year-old male presented with headaches and blurred vision in the right eye for 2 weeks. Computed tomography (CT) with contrast revealed a sellar mass eroding through the sphenoid sinuses with compression of optic chiasm. Biopsy was consistent with GCT. Patient underwent tumor resection by Le Fort I Osteotomy and median maxillotomy for an extended transsphenoidal approach. Upon discharge, patient showed no neurological deficits and intact cranial nerves.
Conclusion:This case contributes to the limited amount of skull-based GCT cases worldwide. Additionally, the extended transoral approach can be performed safely in the context of a GCT within the clivus with acceptable morbidity and cosmesis.
Keywords: Clivus, giant cell tumor, Le Fort osteotomy, skull-based neoplasm
Giant cell tumors (GCTs) comprise 5% of skeletal tumors.[
A 49-year old male presented to the emergency department with headaches and blurred vision in the right eye for 2 weeks. Computed tomography (CT) with contrast revealed a 4.9 × 3.2 cm sellar mass with extension through the sphenoid sinus and posterior ethmoid air cells, compression of the optic chiasm and right optic nerve, and bony destruction of the clivus [Figures
Management and outcome
Given the atypical appearance of this lesion, a transnasal endoscopic transsphenoidal biopsy was performed for tumor diagnosis. The transsphenoidal biopsy was technically challenging due to the patient sustaining five previous nasal fractures and associated septal deviation. Biopsy revealed neoplasm composed of polygonal mononuclear cells with intermixed uniformly distributed giant cells (some containing more than 40 nuclei). Mitotic count was low (less than 5 mitoses per 10 high power fields) with no identifiable atypical mitoses. There was no tumor necrosis or vascular invasion in form of intravascular plugs by tumor cells. Morphologic findings were consistent with bone GCT.[
Patient underwent a tracheostomy followed by Mayfield head-holder immobilization and registration of frameless stereotactic neuronavigation (Medtronic Stealth System). A full-thickness palatal split was performed with median incision spanning from the left of the uvula to between the incisors. Bilateral gingival buccal sulcus incisions were made that extended into the subperiosteal plane and subsequently the bilateral pterygoid plates were dissected.
Prior to osteotomy, plates were preregistered along the nasomaxillary, zygomaticomaxillary buttresses and across the maxilla inferior to the anterior nasal spine. A standard LFO was performed with midline maxillotomy to separate the maxilla between the incisors [
Following exposure of the sella turcica and inferior clivus, microdissection and stereotactic navigation was used to dissect the tumor off the medial walls of the cavernous sinuses bilaterally, optic chiasm and optic nerves superiorly, and the dura behind the clivus [Figures
Prior to completion, stereotactic neuronavigation was used to check the margins of the gross total resection including inferior margin (a), superior margin (b), and posterior margin (c). The medial aspect of the right carotid was inspected for sufficient decompression and decompression of the optic nerve (d)
The patient was monitored for 2 days in the intensive care unit and transferred to the floor where his diet was advanced 7 days after surgery. Postoperative imaging was obtained [
After surgery, he remained neurologically intact without cranial nerve abnormalities and reported vision improvement, confirmed by Humphrey Visual Fields testing. Histological examination revealed tumor morphologically identical to the previous biopsy [
Our case contributes to the few reported skull-based GCT cases worldwide. As demonstrated in previous case reports, the typical patient with skull GCT is a young female, while our patient was a 49-year-old male.[
EEA is a newer, less invasive approach for clival GCTs, with reported advantages of better visualization by placing the lens and light source closer to the mass and lateral visualization with angled endoscopes.[
The transoral approach for clival lesions allows exposure alongside the midline of the inferior third of the clivus, the cervicovertebral junction and the C1/C2 complex. This allows a direct extradural approach without brain retraction. The extended transoral approach is used when the lesions extend beyond the exposure limits of a standard transoral approach and involves additional incisions and facial osteotomies to mobilize structures that may obstruct visualization of the lesion. Accurate reconstruction of the maxillofacial osteotomies is essential to achieve excellent cosmesis and avoid malocclusion. The following approaches allow for more superior exposure of the upper and middle clivus and sphenoid sinus: Transmaxillary (Le Fort I maxillotomy), transmaxillary palatal split, or the transpalatal. The LFO with MM approach was optimal for our exposure. Gupta et al. reported utilizing this approach for resection of a clival GCT in a 17-year-old female, but did not describe the surgical details.[
Our case report demonstrates that the extended transoral approach can be performed safely in the context of a clival GCT with acceptable morbidity. Also, this case underscores the importance of multidisciplinary collaboration between craniofacial surgeons and neurosurgeons to optimize patient outcomes.
The authors would like to thank Dr. Lori Stec and Dr. Robert Granadier from the Department of Opthalmology at Beaumont Hospital, Royal Oak, MI for assistance with the interpretation of the Humphrey Visual Fields Test results.
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