Recurrent Giant Cell Tumour of Maxilla: A case report and review of literature

Abstract

Giant cell tumors (GCTs) are rare, locally aggressive neoplasms that commonly affect the long bones. However, GCTs can also occur in the craniofacial region, including the maxilla. This case report presents a rare case of recurrent giant cell tumor of the maxilla in a 32-year-old male patient. The patient underwent a total maxillectomy with clear margins, which resulted in successful local control. This report highlights the clinical presentation, diagnostic approach, treatment modalities, and favorable prognosis associated with recurrent giant cell tumors of the maxilla.

Introduction

Giant cell tumors constitute approximately 4–5% of all primary bone tumors [1]. They are primarily benign but occasionally can undergo malignant transformation. A substantial majority, more than 75%, tend to develop in the epiphyseal region of long bones. The most frequently observed locations, in descending order of occurrence, are the distal femur, proximal tibia, and distal radius [1]. GCTs of the maxilla are rare, accounting for approximately 1% of all giant cell tumors. To date, only a limited number of GCT originating from the craniofacial skeleton have been reported in the literature. This case report describes a patient with recurrent GCT of the maxilla, emphasizing the challenges faced in management and the importance of a multidisciplinary approach.

Case Report

A 32-year-old male presented with complaints of gradual swelling and pain in the left side of his face for the past eight months. The patient had a history of giant cell tumor resection from the left maxilla one year ago, treated with curettage and bone grafting. Histopathological examination at that time confirmed the diagnosis of a giant cell tumor.

On examination, a firm, non-tender, and expansile mass of size 6 × 5 cm was palpable in the left maxillary region, causing facial asymmetry. The overlying skin was unremarkable, and no neurological deficits were observed. Intra-oral examination revealed a hard bulge in the left side of the palate involving the alveolar margin and upper gingivobuccal sulcus. [Fig. 1] There were no vision abnormalities.

Fig. 1.

Pre operative image showing (a) tumor of left maxilla & (b) bulge over hard palate

Contrast-enhanced computed tomography (CT) scan revealed a well-defined, expansile, and enhancing soft tissue lesion of the left maxilla with multiple internal bony septations. Thinning and erosion of the surrounding bone were noted. The lesion was extending to involve the soft palate inferiorly and the floor of the orbit superiorly. The case was discussed in a multidisciplinary head and neck tumor clinic, including head and neck surgeons, maxillofacial surgeons, radiation oncologists, and radiologists. Considering the aggressive behavior of the recurrent tumor, the treatment plan involved total maxillectomy with interim obturator placement. Reconstruction of maxillectomy defect is deferred to the second stage to monitor recurrence during follow-up.

Under general anesthesia, an extended Weber-Ferguson incision was made to access the tumor adequately. The tumor, along with the whole left maxilla, was removed to achieve clear margins. [Fig. 2] An interim obturator was placed in the maxillectomy defect.

Fig. 2.

Image showing total maxillectomy specimen with tumor

Histopathological examination revealed a tumor comprising of evenly distributed numerous osteoclastic giant cells embedded in a stroma composed of mononuclear cells. [Fig. 3] All the resection margins were free from tumor infiltration. The features were consistent with the diagnosis of a giant cell tumor. He recovered well during the post-procedural period and was discharged on the fifth post-op day. He was explained about the diagnosis, potential chances of recurrence, and the need for close follow-up. He was in routine follow-up till 18 months without any evidence of recurrence. [Fig. 4].

Fig. 3.

Histopathological section showing multinucleated osteoclast like giant cells and mononuclear cells having oval to vesicular nuclei and eosinophilic cytoplasm (H&E, 400x) (black arrow)

Fig. 4.

Post operative follow up image showing (a) well healed facial scar & (b) obturator fitment of maxillectomy defect

Discussion

GCT originates from undifferentiated mesenchymal cells of the bone marrow [1]. Approximately 2% of all GCTs occur in the head and neck region, affecting the maxilla and mandible [2]. Giant cell lesions occurring in the craniofacial skeleton, apart from the jaws, are infrequent; with a predominant occurrence noted in the sphenoid, ethmoid, and temporal bones. They usually occur after the second decade of life. The tumor is usually slow-growing growing associated with pain. Giant cell tumors (GCTs) may at times, be linked to other medical conditions, including Von Recklinghausen's disease, Francescatti syndrome, hyperthyroidism, and Paget's disease. The occurrence of multiple GCTs is rare but can be associated with Paget's disease. Among all GCT cases, the malignant subtype accounts for approximately 5%-10% and is frequently a consequence of previous radiation treatment for a benign GCT [1].

GCT is identified by the widespread proliferation of stromal cells that are RANKL-positive (receptor activator of nuclear factor kappa-B ligand), along with osteoclast-like cells that are RANK-positive. The interaction between RANK and RANKL within GCT is believed to play a role in the expansion of the tumor cells [10].

Histopathologically, they are characterized by profuse multinucleate giant cells scattered throughout the stroma of mononuclear cells. These giant cells have histological similarities with osteoclasts hence, these tumors are also synonymized as osteoclastomas [3].

GCT shares numerous clinical and radiological characteristics with other jawbone lesions like giant cell granuloma, aneurysmal bone cyst, fibro-osseous lesions, cherubism, hyperparathyroidism-related brown tumors, and malignant jawbone neoplasms like sarcoma. It can be challenging to differentiate between these lesions, underscoring the importance of meticulous clinical, pathological, and radiological correlation.

The primary treatment modality for GCT involves surgical excision with clear margins. In cases requiring extensive removal, reconstruction employing autografts or metal prostheses is a common practice [5, 6].

The recurrence rate of GCT in the head and neck region is notably higher than in other sites, making long-term follow-up crucial. The reported recurrence rate after curettage stands at 35%, whereas it reduces to 7% following wide resection [4, 5]. In most cases, the postoperative recurrence occurs within 24 months after the surgery [7]. The risk of recurrence increases when any other treatment short of complete removal of the tumor with adequate bony margins is employed. Recurrent GCTs have the potential to transform into malignant sarcomas and lead to the development of lung metastases. These metastatic growths appear histologically benign, resembling the primary bone tumor's characteristics. In our case, the patient had undergone curettage of the lesion in the prior surgery, which led to recurrence within 6 months. Total maxillectomy with clear margins was performed to achieve complete disease clearance. Reconstruction of the defect was deferred to the second stage in order to monitor recurrence.

Non-surgical treatment modalities have also been reported in literature with limited success rates. These include bisphosphonates such as Zoledronate, which are thought to induce apoptosis in the multinuclear giant cell fraction, preventing tumor-induced osteolysis [8]. Recently, humanized monoclonal antibodies such as Denosumab targeting the RANK ligand (RANK-L) have also been employed in the treatment of non-resectable recurrent GCT [9]. Donesumab induces tumor ossification and inhibits tumor growth to a certain extent.

Conclusion

Recurrent giant cell tumor of the maxilla poses a challenge for clinicians due to its rarity, aggressive behavior, and potential for recurrence. Early diagnosis and wide surgical excision with clear margins play a pivotal role in achieving successful outcomes and reducing the risk of disease relapse. Long-term surveillance is essential for detecting any signs of recurrence promptly.

Funding

No sources of support or funding obtained.

Declarations

Conflicts of interest

The authors declare that they have no conflict of interest. Authors declare that the views expressed in this report are their own.

Human and/or Animals

Not applicable.

Informed Consent

Written Informed consent has been obtained from the patient.