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| CASE REPORT |
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| Year : 2014 | Volume
: 9
| Issue : 1 | Page : 48-49 |
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Giant cell tumor of the clivus with presence of epithelioid histiocytes
Amit Agrawal1, Rajsekhar Gali2, Vissa Shanthi3, Baddukonda Appala Ramakrishna3, Kuppili Venkata Murali Mohan3
1 Department of Neurosurgery, Narayana Medical College Hospital, Chinthareddypalem, India
2 Departments of Oral and Maxillofacial Surgery, Narayana Dental College Hospital, Chinthareddypalem, Nellore, Andhra Pradesh, India
3 Department of Pathology, Narayana Dental College Hospital, Chinthareddypalem, Nellore, Andhra Pradesh, India
| Date of Web Publication | 19-Apr-2014 |
Correspondence Address:
Amit Agrawal
Department of Neurosurgery, Narayana Medical College Hospital, Chinthareddypalem, Nellore - 524 003, Andhra Pradesh
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/1793-5482.131078
Giant cell tumor (GCT) is a benign neoplasm but locally aggressive tumor that uncommonly involves the skull bone. We report a case of a 62-year-old male presented with increasing headache and diplopia. Investigations were suggestive of an expanding mass lesion of the clivus. Histopathology was suggestive of diagnosed with GCT with abundant histiocytes. Keywords: Clivus, giant cell tumor, histiocytes, skull
How to cite this article:
Agrawal A, Gali R, Shanthi V, Ramakrishna BA, Mohan KM. Giant cell tumor of the clivus with presence of epithelioid histiocytes. Asian J Neurosurg 2014;9:48-9 |
How to cite this URL:
Agrawal A, Gali R, Shanthi V, Ramakrishna BA, Mohan KM. Giant cell tumor of the clivus with presence of epithelioid histiocytes. Asian J Neurosurg [serial online] 2014 [cited 2021 Jan 17];9:48-9. Available from: https://www.asianjns.org/text.asp?2014/9/1/48/131078 |
| Introduction | |  |
Giant cell tumor (GCT) is a benign neoplasm but locally aggressive tumor that uncommonly involves the skull bone (preferentially, the sphenoid and the temporal bones), [1],[2] with ooccasional instances of orbital, calvarial, and occipital bones involvement. [3] In the present article, we report a case of GCT of the clivus and review the relevant literature.
| Case Report | | |
A 62-year-old male underwent transphenoidal biopsy of the sellar mass lesion and it was diagnosed as GCT. Now, he presented with an increasing in headache and diplopia of 3 months duration. He was a known diabetic controlled with medication. There was no history of hypertension. His general and systemic examination was normal. Neurological examination was normal, except bilateral 6 th nerve paresis. Magnetic resonance imaging (MRI) of the brain showed a large well-defined hyperdense contrast-enhancing lesion involving the clivus. In view of the enlarging size of the lesion and increase in headache, the patient was planned for resurgery [Figure 1]. The patient underwent extended bifrontal craniontomy and orbitoomy, subfrontal approach, and decompression of the tumor. Postoperatively was uneventful, the headache improved but diplopia and 6 th nerve paresis was persisting. Microscopically, the lesion is composed of multinucleated giant cells admixed with mononuclear stromal cells. The stromal cells are polygonal, and some of them are elongated spindle-shaped. The cells have vesicular round to oval nuclei with prominent nucleoli in some of them. Increased vacularity was noted in some areas of tumor. Few foci showed sheets of clear histiocytes having small round nuclei and clear cytoplasm. Bony trabeculae rimmed by osteoblasts are noted in some foci [Figure 2] and [Figure 3]. | Figure 1: MRI of the brain showing extensive lesion involving the clivus
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 | Figure 2: (a) Sheets of histiocytes with small round nuclei and clear cytoplasm (H and E, ×100) and (b) Sheets of histiocytes with small round nuclei and clear cytoplasm (H and E, ×400)
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 | Figure 3: Tumor showing multiple osteoclast-like giant cells admixed with stromal cells (H and E, ×100)
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| Discussion | | |
Primary GCTs of the clivus are a rare lesion with only few reported cases in the literature. [4],[5],[6] The clinical features of these tumors depend on the location of cranial lesion and symptoms vary in accordance with their actual location. GCT of the sphenoid can present with headache, visual field defects, blindness, diplopia, second through 8 th cranial nerve dysfunction, endocrinopathy, and altered mental status; [4],[5],[6] on the contrary, temporal bone tumors can present with pain behind the ear, deafness, and facial weakness. [7] GCT is characterized by vascularized tissue that contains numerous cytologically benign multinucleated giant cells dispersed through plump, spindly, and/or ovoid cells. [1],[8] Nuclei of the cells are generally hypochromatic with inconspicuous nucleoli and uncommon mitotic figures. [9] Presence of epithelioid histiocytes is rare in GCT and it was described in case of GCT of the tendon sheath, which was largely composed of epithelioid; histiocytes are very rare and in the reported case, the tumor was composed of cellular and hypocellular zones, celluar zone was composed of spindle cells and osteoclast-like giant cells, and the hypocelluar zone was composed of epithelioid clear histiocytes, and it was suggested that the epithelioid histiocytes were the neoplastic cells. [10] GCTs needs to be differentiated from other lesion including chondrosarcoma and chordomas, aneurysmal bone cyst, giant cell reparative granuloma, "Brown tumor" of hyperparathyroidism, and fibrous dysplasia. [9] Skull X-rays and angiography have been the traditional investigations for the diagnosis of the GCTs of the skull. [3],[9] Recently, computed tomography (CT) and MRI have been increasingly used for the diagnosis of these lesions, and the CT appearance of GCT is that of a homogeneous hyperdense mass highly enhancing after contrast administration. [2],[11] Bony erosions can also be demonstrated by CT scan examination, [3],[11] and the bone adjacent the lesion can appear hyperplastic in some cases. [12] The treatment of choice of GCTs is complete surgical excision and if it can be achieved it is curative; however, as was seen in the present case, it is may not always be feasible. [9],[11],[12] Although controversial, in cases of unresectable tumors or with incomplete excision, radiotherapy remains the other option. [2],[4],[7],[9],[11]
| References | | |
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| 9. | Wolfe JT 3 rd , Scheithauer BW, Dahlin DC. Giant-cell tumor of the sphenoid bone. Review of 10 cases. J Neurosurg 1983;59:322-7.
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[Figure 1], [Figure 2], [Figure 3]
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The identification of H3F3A mutation in giant cell tumour of the clivus and the histological diagnostic algorithm of other clival lesions permit the differential diagnosis in this location |
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