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|LETTERS TO EDITOR
|Year : 2018 | Volume
| Issue : 3 | Page : 861-864
Giant cell tumor at the clivus: Not an area 51
Ayusman Satapathy, Manjul Tripathi, Ravi B Chauhan, Jenil Gurnaani, Sandeep Mohindra
Department of Neurosurgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
|Date of Web Publication||15-May-2018|
Dr. Sandeep Mohindra
Department of Neurosurgery, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
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
Primary giant cell tumor (GCT) or osteoclastoma of skull base is a rarely encountered neoplastic entity. Though considered histologically benign, they are locally aggressive with significant metastatic potential. Their relative rarity and radio-histologic mimicry with more common pathologies frequently leads to delay in the diagnosis. Radical excision with adjuvant focal radiation helps in their long-term control. Their definitive treatment still remains controversial. The authors describe their experience with GCT at the skull base originating from the clivus with multiple cranial nerves involvement. The present case enriches the existing literature with review of the treatment options and advancements [Table 1].,,,,,,,,,,
A 24-year old male patient presented with a history of diplopia on extreme gaze, progressive diminution of vision, and headache of 4-month duration. On examination, the visual acuity was no perception of light in the right eye and finger counting at 1m in the left eye. The visual deficits had started with an initial bitemporal hemianopia. Fundoscopy revealed primary optic atrophy. He had bilateral abducent nerve paresis. Computed tomography (CT) scan of the head showed an expansile lobulated mass involving the clivus and sella, destroying the adjacent sphenoid bone [Figure 1]a, [Figure 1]b, [Figure 1]c. Magnetic resonance imaging (MRI) showed a lobulated mass of size 5.7 × 4.5 × 5.7 cm, involving the sella and clivus, extending symmetrically to involve the sphenoid bone [Figure 1]d, [Figure 1]e, [Figure 1]f. An extended bifrontal craniotomy with total macroscopic excision of the tumor was achieved by an extradural approach. The tumor was greyish-white, moderately vascular, and firm in consistency. Piecemeal resection of the tumor was achieved by the extradural approach under microscopic guidance [Figure 2]a. The postoperative recovery was uneventful. The patient did not achieve any improvement in visual acuity but his diplopia improved, as observed at a 2 month follow-up. He subsequently received 60 Gy of radiation as adjuvant therapy.
|Figure 1: (a) Axial noncontrast CT scan showing a soft tissue mass at the skull base involving anterior cranial fossa floor; (b and c) coronal and sagittal images showing wide bony destruction with a soap bubble appearance; (d) axial; (e) coronal; and (f) sagittal contrast MRI images showing a large GCT centered on the clivus with a wide parasellar and suprasellar extension (CT, computed tomography; MRI, magnetic resonance imaging)|
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|Figure 2: (a) Postoperative CT scan axial image showing gross total tumor resection; (b) GCT with mononuclear background stromal cells and interspersed multinucleate giant cells (hematoxylin and eosin, ×200); (c) Vimentin immunostain highlighting background stromal cells with faint positivity in giant cells (×200); (d) CD68 immunostain highlighting multinucleated giant cells (×200)|
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The histopathology revealed a cellular tumor composed of osteoclastic giant cells and stromal cells [Figure 2]b, [Figure 1]c, [Figure 1]d without any significant atypia or mitosis. A fragment lined by respiratory epithelium with thickened basement membrane and underlying lymphoid follicle formation was also noted. Immunohistochemistry was positive for cluster of differentiation (CD)-68. The cellular morphology was sufficient for establishing the diagnosis of GCT. The immunochemistry is usually not essential in these cases for diagnostic purposes but helps in establishing the lineage using CD-68 immunostaining, which is a histiocytic marker.
The skull base GCT has a predilection for the sphenoid and temporal bones and constitutes 5% of all primary bone tumors. A clival-based GCT is very rare, and based upon its location, mimics a chordoma as a radiologic variant. GCT typically presents in female subjects in their second to third decade, who exhibit complaints of headache, ocular paresis and visual deterioration, and erosion of body of the sphenoid. The hormonal profile usually remains unremarkable. The plain radiograph and the CT scan show an expansible, lytic lesion with a classical “soap bubble” appearance. MRI usually reveals a hypointense mass with heterogeneous contrast enhancement, suggesting intermediary areas of necrosis. Expansion and extension through the bone with prominent bony trabeculation and loculation is often appreciated on the CT scan. Usually, GCT does not present with calcification on radiology. A similar lesion with calcification is usually suggestive of a chordoma, chondrosarcoma, or meningioma. The differential diagnosis of GCT includes giant cell reparative granuloma, chordoma, chondrosarcoma, osteoblastoma, chondroblastoma, brown cell tumor of the parathyroid gland, ossifying fibroma, fibrous dysplasia or aneurysmal bone cyst.
The most appropriate treatment of GCT in extracranial locations remains en bloc surgical excision, which is seldom possible in the skull base variants due to the involvement of neighboring neurovascular critical structures at traditionally remote areas of skull base., Tumor recurrence is dependent on the extent of tumor removal and adjuvant therapy [Figure 3]. A recurrence rate of 30% is noted in the cases managed purely by an intralesional surgical approach. GCT at extracranial sites present with recurrence rates as high as 60% with curettage alone, whereas total excision is associated with only a 7% recurrence rate. GCT is traditionally radioresistant, hence the efficacy and utility of radiotherapy after subtotal excision remains debatable, with the added risk of sarcomatous transformation (7–29%). Conventional radiation protocol involves 40–60 Gy over 3 to 6 weeks, yielding nearly 85% local control rate at 5 years. If used as a standalone procedure, primary radiotherapy has a higher recurrence rate of up to 63% rather than when it is used as an adjuvant therapy after surgical excision., Periodic follow-up is warranted to check for recurrence in a timely fashion. Anecdotal reports have mentioned the comparable utility of stereotactic radiosurgery as a boost radiation treatment for resistant cases. The pathological grading and nuclear atypia do not help in predicting subsequent tumor behavior and its potential for sarcomatous transformation.
|Figure 3: Management protocol for intracranial GCTs (CT, computed tomography; GCT, giant cell tumor; GTR, gross total resection; IMRT, intensity modulated radiotherapy; MRI, magnetic resonance imaging; RT, radiation therapy; SRS, stereotactic radiosurgery)|
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Various chemotherapeutic options (viz., vincristine, methotrexate, interferon alpha, etc.) have also been described as neoadjuvant and adjuvant modalities, with no definite survival benefit. Recent literature review has mentioned the role of denosumab, a monoclonal antibody against nuclear factor kappa-B ligand (RANKL), as a monotherapy for GCT. The authors have described complete remission and intense new cortical bone formation with restoration of bony integrity.
GCT at the skull base especially centered over the clivus remains a management challenge for the surgical fraternity [Figure 3]. Despite advances in microsurgical and radiation techniques, long-term control is still not achieved. With advancements in radiosurgical techniques and chemotherapeutic options, longer survival with a good functional outcome is expected.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]