Giant cell tumor of the frontal sinus: case report

INTRODUCTION

GCT is an uncommon, very challenging, locally aggressive, benign primary bone tumor with risk metastasis and malignant transformation.¹ GCT was first described by Cooper and Travers in 1818.² It is usually encountered by orthopedic surgeons as a long bone tumor.³ It is very rarely encountered in skull bones where it predominantly affects temporal and sphenoid bones.⁴ Therefore it is extremely rare to present as a frontal bone mass. There are only 3 cases reported in the English literature as a GCT of frontal bone in adult patients and 1 pediatric patient.^5-7 Only one of them was a frontal sinus occupying mass.⁵ None of these case reports included a complete clinical, radiological and histopathological demonstration.

CASE REPORT

A 49 year old male, presented to rhinology clinic with a 3 year history of a large, gradually progressive left supraorbital mass. The mass is painless and is not associated with any nasal secretions or external discharge, or other nasal or visual symptoms. He is known to have HTN and Bronchial Asthma for several years. On presentation the mass was very obvious on left supraorbital and frontal area about 5 x 3 cm with normal covering skin and normal eye movement and vision (Figure 1). Other otorhinolaryngology and neurological evaluations were unremarkable.

Radiological evaluation done including computed tomography (CT) and magnetic resonance imaging (MRI). CT showed isodense lesion with areas of calcifications in the left frontal sinus destructing the anterior table of the sinus and significant enhancement with the IV contrast (Figure 2). On MRI the lesion was isointense to the muscle on both T1 and T2 with central hyperintesity, and significant enhancement with intravenous contrast, and no invasion to skin or meningies (Figure 3).

The patient underwent complete excision of the tumor with the involved parts of the anterior table of left frontal sinus through an external approach. Histopathologically the tumor is composed of extensive amount of giant cells and consistent with GCT (Figure 4). The patient followed up in the rhinology clinic and showed no recurrence over 6 months of follow up.

DISCUSSION

GCT is a benign primary bone tumor, thought to be derived from hematopoietic system monocytic and histiocytic cell. This explains why in some references it is called osteoclastoma.⁸ It was firstly described by Cooper and Travers in 1818.² It is considered the most challenging benign bone tumor because of it is aggressive behavior, difficult complete excision and risk of recurrence, metastasis and malignant transformation. It represents about 5% of primary bone tumor and commonly affects the epiphysis of long bones.³ It is relatively more predominant in female.⁸

Primary bone tumors in the skull are generally uncommon and account for 2.5% only, of these tumors is the GCT, which thereby is considered a rare tumor in the skull bones, and when it occurs it prefers the sphenoid and temporal bones.⁴

In the English literature, only three cases reported of such tumor involving the frontal bone in adults and one pediatric patient.^5-7 Only one of these cases was filling the frontal sinus.

Hlavácek et al. described their first 2 cases report of frontal giant cell tumor as a polyostotic form of the disease. The first patient present with disease involving the acromion and the frontal sinus, and in the other patient it was extending from frontal to ethmoid labyrinth. Both cases were treated surgically and showed no recurrence within 2 years of follow up.¹⁰

The approach to patient with suspected GCT should include radiological work up in the form of CT and MRI to assess the extent of the tumor and exclude other possible diagnoses.¹⁰ The tumor usually enhances with the intravenous contrast.

The treatment of choice for GCTs is surgical excision. Radiotherapy role in the treatment of GCT is still controversial. So, it is reserved for unresectable tumors, and in general it showed a good outcome.⁹

The malignant transformation of GCT occurs in 5-10% of cases.^11,12 For diagnosis of malignant transformation of GCT, the following criteria has to be considered.¹³

• • The number of giant cells and respective nuclei.
• • The mitotic index.
• • The Osteoid development.
• • The presence of cell atypias or metaplasias.

In our patient, it was isolated to the frontal sinus. The presenting symptom was mainly the swelling, which is a usual presenting symptom. But with no pain as what is usually the most presenting symptom in GCTs.⁴ The CT scan showed a bony destructive mass with contrast enhancement as the usual with GCT, and the MRI ruled out any intracranial extension of the tumor.¹⁰ The patient underwent only excisional biopsy of the mass which established the diagnosis of GCT, and, in 6 months follow up, no further management for the primary bone disease was required as there was no recurrence.

CONCLUSION

GCT is one of the differential diagnoses of paranasal sinus tumor. It should be considered preoperatively so prompt surgical excision to be achieved, which usually is enough as a primary management. There is a relatively high risk of recurrence as well as malignant transformation. Therefore, regular long term follow up is advised to avoid missing such sequelae.

Funding: No funding sources

Conflict of interest: None declared

Ethical approval: Not required

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