Giant Cell Tumor of the Skull: Review of the Literature

Abstract

Background?Giant cell tumors (GCTs) are rare in the skull. The present report describes a case with a primary GCT located in the temporal bone and reviews the relevant literature. We also propose a treatment strategy for GCT of the skull. Clinical Presentation?A 41-year-old man presented with headache and auditory disturbance. Radiologic images showed a lytic expansive extradural lesion originating primarily from the right temporal bone and expanding into the middle cranial fossa and the infratemporal fossa. A biopsy specimen of the lesion was obtained from the external auditory meatus. Total removal was performed with temporal craniectomy, mandibular condylar process removal, tympanoplasty, and mastoidectomy. Discussion?The rate of recurrence of GCTs is related to complete resection and location of the GCT rather than to the degree of invasiveness. Some of the mononuclear cells and stromal cells in GCT express receptor activator of nuclear factor ?-? ligand (RANKL). Because inhibition of RANKL and bisphosphonate therapy might eliminate giant cells, this approach might be useful for recurrent or unresectable GCTs of the skull. Conclusions?Preoperative diagnosis by biopsy is important in determining the therapeutic strategy of GCTs. Complete resection is important to reduce the recurrence rate of GCTs in the skull.

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

“…Moreover, we analysed 104 case reports of GCTs of the skull base from 1969 to 2017 and highlighted that the sphenoid and temporal bones were those preferentially involved by GCT, with a frequency of 47% and 28%, respectively [ 14 , 29 , 31 – 45 ]. Clivus as primary site of GCT was described in 12 out of 104 reports (12%), while occipital and frontal GCTs showed an incidence of 9% and 4%, respectively (Fig.…”

Giant Cell Tumor of the Temporal Bone and Skull Base

“…Giant cell tumors (GCTs) mainly originate in the epiphysis region of the long bones, especially in the distal femur, proximal tibia, and distal radius. 2,4 GCTs are rare primary bone neoplasms, representing only 5% of all bone tumors and 20% of benign bone neoplasms, and they are exceedingly rare in the skull. Only 2% of GCTs are found in the head and neck and the skull is affected in only 1% of cases, predominantly in the sphenoid and temporal bones.…”

“…Only 2% of GCTs are found in the head and neck and the skull is affected in only 1% of cases, predominantly in the sphenoid and temporal bones. 4,5 When the cranium is involved, GCTs most commonly arise from the floor of the middle fossa, specifically the temporal and sphenoid bones. 2 In temporal bone, it more commonly arises in the petromastoid part which develops from cartilage.…”

“…7 GCTs in the skull are locally aggressive, malignant phenotype exists in only 5 to 10% of cases. 4 As the name implies, GCTs feature giant cells on microscopic analysis, a finding that is not pathognomonic for this type of tumor. It should be noted that GCTs, though benign, may contain histologic features that are often associated with malignancy, such as abundant mitotic figures, areas of tumoral necrosis, and areas of tumoral bone formation.…”

Unique presentation of occipital condyle giant cell tumor as occipital condyle syndrome – A review

“…Recurrence rates are as high as 40–60% with subtotal resections. [ 14 ] As most of these data are available from surgical series of long bone GCTs, it is doubtful whether this can be extrapolated to similar lesions of the skull base. Moreover, due to rarity of these cases, an optimal treatment strategy for skull base GCTs has not been described in the literature.…”