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| CASE REPORTS |
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| Year : 2021 | Volume
: 4
| Issue : 1 | Page : 22-24 |
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Giant cell tumor of the clivus: A case report
Renuka Masodkar1, Mano Bhadauria1, Rajendra Prasad2
1 Department of Radiation Oncology, Indraprastha Apollo Hospital, New Delhi, India
2 Department of Neurosurgery, Indraprastha Apollo Hospital, New Delhi, India
| Date of Submission | 26-Nov-2020 |
| Date of Acceptance | 16-Feb-2022 |
| Date of Web Publication | 12-Apr-2022 |
Correspondence Address:
Dr. Renuka Masodkar
Department of Radiation Oncology, Indraprastha Apollo Hospital, E-102 Yamuna Apartments, Alaknanda, New Delhi 110019.
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/IJNO.IJNO_25_20
Giant cell tumors arising from the skull base are rare. We present a case of a giant cell tumor arising from clivus, which was managed by surgery, radiotherapy, and denosumab therapy. Keywords: Clivus, giant cell tumor, steoclastoma
How to cite this article:
Masodkar R, Bhadauria M, Prasad R. Giant cell tumor of the clivus: A case report. Int J Neurooncol 2021;4:22-4 |
| Introduction | |  |
Giant cell tumor (GCT) arising from the clivus is a rare entity. These are generally benign tumors with occasional malignant potential. GCTs of the skull base are uncommon and constitute <1% of all bone GCTs. These tumors preferentially involve the sphenoid and temporal bones.[1] There have been very few cases of GCT of the clivus reported in the literature. We report a case of GCT of the clivus that was managed with surgery, radiotherapy, and denosumab therapy.
| Case report | | |
A 21-year-old man presented with complaints of diplopia since 3 months. He also complained of global headache for the last 4 months.
Magnetic resonance imaging (MRI) brain [Figure 1] revealed a large well-defined expansile irregular heterogenously enhancing lobulated altered signal intensity mass lesion (5.2 cm × 3.2 cm × 4.7 cm) involving the body of the clivus and body of the sphenoid extending into the sphenoid sinus showing enhancement of the soft-tissue component within it and peripheral enhancement of the cystic component. | Figure 1: Axial, sagittal, and coronal views of contrast-enhanced MRI brain showing expansile mass lesion arising from the body of clivus
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He then underwent transnasal endoscopic decompression of the clival tumor.
Histopathology showed GCT of bone.
Postoperative MRI done 1 month postsurgery showed a 5.8 cm × 4.9 cm × 5.8 cm lesion involving the clivus and extending into the sphenoid sinus causing sellar widening and superior displacement of pituitary. Inferiorly the lesion was extending into the bilateral sphenoid sinuses with erosion of the body of sphenoid and clivus. Superiorly it was extending into the suprasellar cistern with indistinct planes with the pituitary gland. Laterally the lesion reached up to orbital apex bilaterally. Anteriorly the lesion was extending up to the nasal septum.
He then received fractionated stereotactic radiotherapy to a dose of 50.4 Gy in 28 fractions with daily exactrac guided image guidance on Novalis Tx linear accelerator.
He received loading doses of injection Denosumab 120 mg given subcutaneously on Days 1, 8, and 15 during the first month of therapy followed by injection Denosumab 120 mg subcutaneously every 4 weekly. Oral Vitamin D and calcium supplementation were given along with Denosumab.
He tolerated the treatment well and is symptom free 6 months post-treatment.
| Discussion | | |
GCTs are also known as osteoclastoma. Although they are classified as benign, malignant transformation may occur in 1%–3% of the cases.
They are known to arise from the epiphyses of long bones, particularly the distal femur, proximal tibia, distal radius, and proximal humerus. Skull base GCTs are rare and primarily involve the sphenoid and temporal bones.[2] Primary GCT of the clivus is extremely infrequent. As the number of reported cases in the literature is few, there are no standard guidelines regarding the management of GCT of the clivus.[3]
Clinical manifestation of GCTs arising from the skull base comprises headache, visual field defect, diplopia, ophthalmoplegia, endocrinopathy, and multiple cranial nerve involvement.[4]
X-ray and the computed tomography (CT) scan show an expansible, lytic lesion with a classical “soap bubble” appearance. MRI reveals a hypointense mass with heterogeneous contrast enhancement, and the soft-tissue extension of the tumor.
Modalities of treatment include enbloc resection, curettage, curettage, and adjuvant chemotherapy (doxorubicin, cisplatin, methotrexate, zolendronate, and raloxefine), adjuvant radiotherapy, and Denosumab therapy.[5]
Radical surgery is the treatment of choice. However, complete removal of the tumor is difficult due to the anatomical location and the involvement of adjacent neurovascular structures. Surgical approaches consist of endonasal endoscopic transsphenoidal approach, frontal craniotomy, and transmaxillary approach.[6]
The extent of resection and adjuvant therapy determines the risk of recurrence.[7] Adjuvant treatment has a role as complete surgical removal of the tumor is often not possible.
Radiotherapy is used for unresectable as well as incompletely resected tumors. Postoperative radiotherapy to a dose of 45–50 Gy is recommended. For tumors that are unresectable, the primary treatment modality is radiotherapy. Intensity-modulated radiotherapy helps in delivering high dose to the tumor while decreasing the dose to the adjacent organs at risk. In a study by Roeder et al.,[8] intensity-modulated radiotherapy with a mean dose of 57.6 Gy was used for sphenoid lesion with good tumor control and no major toxicities.
GCTs are known to be abundant in stromal cells that express RANKL, which is an important mediator of osteoclast activation. Hence, monoclonal antibodies against RANKL, such as Denosumab, are also useful. Denosumab acts by blocking the RANKL–RANK interaction between stromal cells and osteoclastic giant cell precursors, thus inhibiting their maturation and bone resorption. Denosumab also has an antiangiogenic activity and decreases the content of proliferative tumor stromal cells.[9] Denosumab has shown to decrease the tumor burden by 90% while having minimal side effects.[10] It may be used as adjuvant treatment or as a neoadjuvant treatment to downstage the tumor prior to surgery. Long-term treatment protocols with the use of Denosumab are yet to be established.
| Conclusion | | |
Clival GCTs are extremely rare. Surgical resection is the primary modality of choice. Chances of incomplete resection due to location of the tumor are high. Hence, adjuvant treatments such as local radiotherapy and Denosumab therapy are essential for increasing the local control and decreasing the possibility of recurrence. To the best of our knowledge, this is the first case in India in which Denosumab has been used for the giant cell of the clivus.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Wolfe JT 3rd, Scheithauer BW, Dahlin DC Giant-cell tumor of the sphenoid bone: Review of 10 cases. J Neurosurg 1983;59: 322-7.  |
| 2. | Pai SB, Lalitha RM, Prasad K, Rao SG, Harish K Giant cell tumor of the temporal bone: A case report. BMC Ear Nose Throat Disord 2005;5:8. |
| 3. | Zhao J, Qian T, Zhi Z, Li Q, Kang L, Wang J, et al. Giant cell tumor of the clivus: A case report and review of the literature. Oncol Lett 2014;8:2782-6. |
| 4. | Iacoangeli M, Di Rienzo A, Re M, Alvaro L, Nocchi N, Gladi M, et al. Endoscopic endonasal approach for the treatment of a large clival giant cell tumor complicated by an intraoperative internal carotid artery rupture. Cancer Manag Res 2013;5:21-4. |
| 5. | Patibandla MR, Thotakura AK, Rao MN, Addagada GC, Nukavarapu MC, Panigrahi MK, et al. Clival giant cell tumor: A rare case report and review of literature with respect to current line of management. Asian J Neurosurg 2017;12:78-81. [ PUBMED] [Full text] |
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| 7. | Satapathy A, Tripathi M, Chauhan RB, Gurnaani J, Mohindra S Giant cell tumor at the clivus: Not an area 51. Neurol India 2018;66:861-4. |
| 8. | Roeder F, Timke C, Zwicker F, Thieke C, Bischof M, Debus J, et al. Intensity modulated radiotherapy (IMRT) in benign giant cell tumors: A single institution case series and a short review of the literature. Radiat Oncol 2010;5:18. |
| 9. | Bardakhchyan S, Kager L, Danielyan S, Avagyan A, Karamyan N, Vardevanyan H, et al. Denosumab treatment for progressive skull base giant cell tumor of bone in a 14 year old female: A case report and literature review. Ital J Pediatr 2017;43:32. |
| 10. | Thomas D, Henshaw R, Skubitz K, Chawla S, Staddon A, Blay JY, et al. Denosumab in patients with giant-cell tumour of bone: An open-label, phase 2 study. Lancet Oncol 2010;11:275-80. |
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