A rare tumor of clivus masquerading as pituitary adenoma

Abstract

Giant cell tumors (GCT) are generally benign, commonly affecting young adults, with a slight preponderance in females. They are locally aggressive with a high rate of local recurrence. Most of them are found in the epiphysis of long bones, making the base of the skull a rare site. We report the case of a 35-year-old female, who presented with neurological symptoms of headache and diplopia. On magnetic resonance imaging, a space-occupying lesion was discovered in the clivus. Histopathology was diagnostic of a GCT. GCT arising from the clivus is extremely uncommon, with about 15 cases published in the literature. The present case highlights the rarity of this tumor and contributes to the existing literature with analysis and evaluation of the management strategies and prognosis.

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

“…Two patients were given denosumab for 4 and 5 years, respectively, by gradually extending the dosing intervals and without any serious side effects reported. 4,26 Furthermore, in our case, denosumab was used to treat relapse thus obtaining a long-term local benefit with radiologic and clinical stabilization at 2-year follow-up. The treatment continues to be maintained at this dosing interval for a total of 24 months from drug resumption.…”

“…A gradual extension appears to be recommended, for the adjustment of the dosing interval, as in 2 cases previously reported. 4,26 Moreover, our case contributes to the limited follow-up data of denosumab therapy, thereby contributing as an example of its effectiveness in treating relapses and long-term tumor control.…”

“…20 Less than 1% of GCTs occur in the skull and among those, primary GCTs involving the clivus are extremely rare. 26 Because of their expansive nature and proximity to the cavernous sinus, clival GCTs typically present with headache, diplopia, and cranial nerve palsies, of which the sixth is the most common involved followed by the third. In addition, a fifth cranial nerve palsy has been reported, 2,3 as shown in the present review.…”

“…They usually originate in the epiphyses of the long bones, such as the distal femur, proximal tibia, distal radius, and proximal humerus 20. Less than 1% of GCTs occur in the skull and among those, primary GCTs involving the clivus are extremely rare 26. Because of their expansive nature and proximity to the cavernous sinus, clival GCTs typically present with headache, diplopia, and cranial nerve palsies, of which the sixth is the most common involved followed by the third.…”

“…The treatment of recurrence, however, varied according to the case in question: 1 patient was treated with RT (external beam radiotherapy) 11 ; 1 patient had a second surgery and tomotherapy 25 ; the remaining 5 patients were prescribed denosumab treatment, and proton therapy was also given in just 1 case. 4,20,23,26 Two patients experienced a second recurrence (Table 1: cases 5 and 22): in patient 5, the tumor regrew 1 year after radiotherapy and a second limited resection was performed 11 ; patient 22 had a recurrence 2 years later and a further surgery was planned at the time of the reported case. 25 A malignant transformation was reported in 2 cases (Table 1: cases 7 and 15).…”