Giant cell tumor of the clivus with presence of epithelioid histiocytes

Agrawal, Amit; Gali, Rajsekhar; Shanthi, Vissa; Ramakrishna, Baddukonda Appala; Mohan, Kuppili Venkata Murali

doi:10.4103/1793-5482.131078

Cited by 6 publications

(6 citation statements)

References 9 publications

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“…We summarize 9 clival GCTs including our case [Tables 1 and 2 ]. [ 1 5 6 7 9 10 11 13 ] The patients’ age at diagnosis ranged from 9 to 62 years, with 5 male and 4 female patients. Major symptoms included headaches owing to high intracranial pressure and diplopia caused by abducens nerve palsy.…”

Section: Discussionmentioning

confidence: 99%

Giant cell tumors of the clivus: Case report and literature review

Shibao¹,

Toda²,

Yoshida³

2015

Surg Neurol Int

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Background:Clival giant cell tumors (GCTs) are extremely rare with only eight cases reported to date, and malignant transformation is quite rare. Herein, we report a case of an uncontrolled clival GCT, which was transformed malignant, and review the literature.Case Description:A 25-year-old man experienced double vision for 1 month. Computed tomography and magnetic resonance imaging revealed a clival tumor. The endonasal endoscopic transsphenoidal approach (EEA) was used, and partial resection was performed because of massive bleeding. Histological examination showed a GCT. After radiation therapy, the tumor recurred; the EEA and the anterior transpetrosal approaches were used to perform second and third operations, respectively. The MIB-1 index increased from 4.2% to 26.3%.Conclusions:GCTs are difficult to treat because of their location, vascularity, and the potential for malignant transformation.

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Section: Discussionmentioning

confidence: 99%

Giant cell tumors of the clivus: Case report and literature review

Shibao¹,

Toda²,

Yoshida³

2015

Surg Neurol Int

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“…Regarding treatment, data were available for all but 2 patients whose information about adjuvant treatment and follow-up were lacking 15,16. Overall, 29 tumor resections were performed: all the patients underwent surgery as initial treatment and 4 needed a further resection.…”

Section: Review Of the Literaturementioning

confidence: 99%

“…15 , 16 Overall, 29 tumor resections were performed: all the patients underwent surgery as initial treatment and 4 needed a further resection. The surgical approaches used were endoscopic endonasal approach, frontal or temporal craniotomy, 4 , 16 transmaxillary, 18 and retromastoid 10 with endoscopic endonasal approach, the most commonly used (68%, 17/25 patients). The majority of patients were treated with subtotal or near-total resection.…”

Section: Review Of the Literaturementioning

confidence: 99%

“…14,25 Regarding treatment, data were available for all but 2 patients whose information about adjuvant treatment and follow-up were lacking. 15,16 Overall, 29 tumor resections were performed: all the patients underwent surgery as initial treatment and 4 needed a further resection. The surgical approaches used were endoscopic endonasal approach, frontal or temporal craniotomy, 4,16 transmaxillary, 18 and retromastoid 10 with endoscopic endonasal approach, the most commonly used (68%, 17/25 patients).…”

Section: Review Of the Literaturementioning

confidence: 99%

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Unresectable Clival Giant Cell Tumor, Tumor Control With Denosumab After Relapse: A Case Report and Systematic Review of the Literature

Pionelli¹,

Asaftei²,

Tirtei³

et al. 2022

Journal of Pediatric Hematology/Oncology

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Giant cell tumors (GCTs) of the skull base are rare entities. Although considered histologically benign, GCTs are locally aggressive with a high rate of local recurrence. The present case describes a 14-year-old girl with a clival GCT who underwent long-term therapy with denosumab after local relapse. To our knowledge, it is the second case described with a follow-up term > 2 years from the start of denosumab and who did not receive any other adjuvant treatment besides denosumab. The patient achieved a local control of the disease. According to the few available data, radical excision with adjuvant therapy helps in long-term control in uncommon sites, such as the skull. However, the definitive treatment is still controversial because of their rarity and few follow-up data. The present case highlights the benefit of denosumab and its safety as long-term therapy and contributes to the existing literature with analysis and evaluation of the management strategies and prognosis.

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“…Albeit histologically benign, Clival GCT can be clinically devastating because of its anatomical location and destruction of vital structures [ 15 ]. The tumour also shows a high tendency to local recurrence, thus making the total surgical resection essential [ 15 , 19 ]. However, the complete removal is not always feasible and an adjuvant treatment (chemotherapy or radiotherapy) is often used [ 15 – 17 , 20 , 24 ].…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2018

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BackgroundGiant Cell Tumour of Bone (GCT) is a locally aggressive primary bone tumour that usually occurs at the epiphyses of the long bones of the appendicular skeleton with a tendency to recurrence. Recurrent somatic H3F3A mutations have been described in 92% of GCT cases. GCTs involving the Clivus are extremely rare lesions and less than 15 cases are described in the literature. They represent a surgery challenge and are easily misdiagnosed. Our aim was to reveal if the genetic bases underlying Clival GCTs were the same of GCTs of long bones to improve the diagnosis and treatment.MethodsThe targeted somatic sequencing of GCT-related genes (H3F3A, H3F3B, IDH1, IDH2 and ZNF687) was performed on Clival GCT biopsies of two different cases. Histological analyses on the same tissues were used to detect the neoplastic population and its expression profile.ResultsSanger sequencing revealed that both patients were positive for the p.Gly34Trp mutation in the H3F3A gene. Immunofluorescence assay using monoclonal antibody, specifically detecting the mutant H3.3, highlighted that the mutation only involved the mononuclear cell population and not the multinucleated giant cells. Moreover, immunohistochemistry assay showed that RANKL was highly expressed by the stromal cells within Clival GCT, mimicking what happens in GCT of the long bones. In addition, systematic literature review allowed us to generate a histology-based diagnostic algorithm of the most common clival lesions.ConclusionsWe conclude that the Clival GCT is genetically defined by somatic mutation in the H3F3A gene, linking it to the GCT of long bones. The similarity with GCTs of long bones let us to hypothesize the utility of Denosumab therapy (already effective for GCTs) in these surgically challenging cases. Moreover, H3F3A genetic screening can be combined to the histological analysis to differentiate GCTs from morphologically similar giant cell-rich sarcomas, while the histological diagnostic algorithm could help the differential diagnosis of other clival lesions.

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Giant cell tumor of the clivus with presence of epithelioid histiocytes

Cited by 6 publications

References 9 publications

Giant cell tumors of the clivus: Case report and literature review

Giant cell tumors of the clivus: Case report and literature review

Unresectable Clival Giant Cell Tumor, Tumor Control With Denosumab After Relapse: A Case Report and Systematic Review of the Literature

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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