From Volume 15, Issue 1, January 2022 | Pages 35-39
This case report describes the diagnosis and management of a central giant cell lesion in a young, healthy patient. The article outlines the differences between central giant cell lesions and their counterparts, peripheral giant cell lesions. More crucially, it highlights the challenge in distinguishing these lesions, which are almost identical histopathologically.
Central giant cell lesions are benign, but sometimes aggressive, neoplasms of the jaws.1,2,3 They can cause cortical bone expansion, tooth displacement/mobility and root resorption.3 In comparison, peripheral giant cell lesions are benign reactive lesions that arise from the soft tissues, often in response to mucosal trauma.4
A 9-year-old female was referred to the oral and maxillofacial surgery (OMFS) department at the Royal Derby Hospital by her general dental practitioner (GDP). Medically, the patient was fit and well, the reason for referral being a possible haematoma in the lower right lateral incisor, canine and first premolar region (LR2–LR4).
On initial presentation, the patient had no complaints of pain or soreness. A detailed history revealed that the intra-oral swelling in the lower right quadrant had been present for approximately 12 months and had not changed size during this time period. There was no history of trauma or previous treatment to the area.
The extra-oral examination was unremarkable. Intra-orally, there was a soft, bluish, fluctuant swelling noted in the LR4 and LR3 region. As part of the assessment, a computed tomography (CT) Dentascan was taken to inform diagnosis and treatment planning. The scan suggested the presence of a follicular cyst associated with the crown of the LR3. The lingual cortex was seen to be intact. Subsequently the patient and her parents were informed of the results and advised that enucleation of the cyst should be undertaken under general anaesthetic. This would then be assessed histopathologically to help formulate a definitive diagnosis.
In addition to the swelling in the lower right quadrant, the patient also presented with a malocclusion. As the patient was due to undergo a general anaesthetic for enucleation of the cyst, it was appropriate that any other forms of treatment needed to aid future orthodontics, such as extractions or exposure of appropriate teeth, be carried out at the same time. It was agreed that assessment of the patient by the orthodontic team within the hospital setting was, therefore, appropriate. An orthodontic assessment revealed that the patient had Class I incisors on a mild Class II skeletal base with mild upper and lower arch crowding. This was complicated by a retained lower left lateral incisor and primary canine (LLB and LLC), with the lower left lateral incisor (LL2) distally positioned. The lower left permanent canine (LL3) was palpable buccally. At this stage, the LR3 and LR4 were both partially erupted. The molar relationship was Class 1/2 unit II on the right and Class I on the left, with no crossbites present (Figure 1).
The orthopantomogram (OPT) revealed the presence of all permanent teeth, excluding the third molars. In addition, a multilocular, diffuse lesion was evident in the LR2, LR3 and LR4 region (Figure 2).
Following consultation with both the OMFS and orthodontic team, a decision was taken that enucleation of the cyst would be performed under general anaesthetic along with extraction of the LLB, LLC (to aid alignment of the LL2), URC and ULC (to encourage eruption of the UR3 and UL3). A lower lingual arch and lower fixed appliance would be the used as an interceptive form of orthodontic treatment to help improve the alignment of the lower labial segment. The patient and her parents were informed that, from an orthodontic perspective, this form of treatment would only be interceptive in nature, and further orthodontic treatment may be required once the patient was further dentally developed. From an OMFS viewpoint, the patient and parents were warned at this stage that the risk of recurrence and need for future treatment would be high because the nature of the lesion was unknown, and a conservative surgical approach would be taken.
The enucleation of the cyst and planned extractions were undertaken uneventfully and the patient recovered well. The materials retrieved from the cyst were sent for histopathological assessment and the results confirmed a peripheral giant cell granuloma/peripheral giant cell epulis. There was no evidence of dysplasia or malignancy. The lesion was noted as reaching the excision margins. At the 4-week post-operative review appointment, there was a clinical suspicion that there may be some residual lesion present, mainly in the lower lingual aspect of LR3 and LR4 area. The parents were informed of this and it was explained that further surgery would be likely in the future. At this stage, it was decided that the lesion should be monitored for a period of 3 months, prior to any further interventions being instigated. As the prognosis and management of the lesion were uncertain, the planned interceptive orthodontic treatment was postponed.
At the 3-month follow-up appointment, there was obvious expansion of both the lingual and labial cortex extending from the LR3 across to the LL1 region (Figure 3). Owing to retirement, the surgical management was transferred to another maxillofacial consultant. A CT scan of the region was arranged to determine the extent of the recurrent giant cell granuloma and help assess its proximity to the local dentition. This confirmed extensive reoccurrence of the lesion (Figure 4). The patient and her parents were informed that further surgery was required to remove the lesion and that this may include removal of some of the teeth in the region. Given the extensive nature of this recurrence and its aggressive nature, a central giant cell lesion was suspected, and a number of blood investigations were carried out to rule out pathologies that may be related to bone metabolism, such as a Brown's tumour of hyperparathyroidism. These tests included serum calcium, parathyroid hormone (PTH), alkaline phosphatase and phosphorus levels; the results of which were all unremarkable.
The enucleation procedure was performed successfully and no teeth were removed. It was noted by the surgeon that the lesion was significantly more extensive than evident on the CT scan, extending from the LR4 to the LL3 region. It was very vascular in nature and adherent to the attached gingivae. A significant amount of bone associated with the LR1 and LR3 had been resorbed by the lesion, leaving these teeth slightly mobile. At the time of the operation, the surgeon noted that despite this bone loss, approximately 50% of the roots of both the LR1 and LR3 were still supported by surrounding bone. It was therefore decided that these teeth were to be kept in situ with the possibility that future bone development in the region could help improve their support. In addition to this, bone loss through removal of the lesion involved careful removal of all the residual soft tissue from the root surfaces of these teeth, invariably risking damage to the root surfaces and increasing the possibility of future ankylosis. Undoubtedly, the long-term prognosis of these teeth was uncertain, and following the procedure, the patient and parents were informed that although these teeth had been maintained for the present, they might be lost in the future.
At 3-month post-operative review, the patient showed good signs of healing (Figure 5) and no complications were reported. The final histological diagnosis of the lesion was reviewed at the multidisciplinary team meeting where a definitive diagnosis of a central giant cell lesion, also known as a central giant cell granuloma (CGCG), was determined. Owing to the aggressive nature of this lesion, it was decided that the patient be placed under regular clinical and radiographic review. An OPT radiograph was taken that showed good bony infiltration, with no evidence of recurrence (Figure 6).
The management of the lesion over an extended period of time has meant that the patient is now further dentally developed. At the time of writing, she was 11 years of age and presented with a retained URE, LRE, ULE and LLE, as well the LL2 that is now disto-lingually displaced. There is no evidence of recurrence of the lesion. The orthodontist recommended a 12-month review and reassessment, while awaiting eruption of the remaining permanent dentition. The latest OPT suggested that all the unerupted teeth were in a reasonable position for eruption, with the exception of the LR5 which appeared to be associated with a developmental anomaly (Figure 7). The development of this tooth would need careful monitoring. It would need surgical removal as part of the overall extraction pattern, and orthodontic treatment may need to be considered.
The patient continued to remain under regular review by the orthodontic and OMFS teams, with further a mandible CT approximately 9–12 months later to assess for healing or recurrence. Provided there was no untoward reoccurrence and there was evidence of continued bone development in the region, a definitive orthodontic treatment would be commenced.
The central giant cell lesion, otherwise known as the central giant cell granuloma (CGCG), is an uncommon, benign, neoplasm-like lesion that occurs in the craniofacial bones, more frequently in women than men.5 It has a predilection to the mandible with approximately 70% of giant cell lesions developing here.6,7 Most cases of CGCGs are seen in children and young adolescents, with approximately 75% reported before the age of 30.8 Giant cell lesions are predominantly slow growing; however, they can behave aggressively. Clinical features include cortical bone expansion, root resorption, mobility and migration of teeth. The lesions can be symptomless, but pain and/or paraesthesia has been noted in some severe cases.9
CGCGs are different to the peripheral giant cell lesion (PGCG), which predominantly occurs in the soft tissues. These lesions can, however, encroach into the cortical bone. The histological features of CGCGs and PGCGs are almost identical to each other, which can make diagnosis challenging.13 Therefore clinical and radiographic findings are essential in influencing the final diagnosis. Additionally, similar histological features are seen in a Brown's tumour, which is associated with hyperparathyroidism and cherubism.11,14 Therefore metabolic bone investigations must be undertaken, including blood tests for serum calcium, alkaline phosphatase and parathyroid hormone levels to rule out this condition. Multiple giant cell lesions can also be associated with fibrous dysplasia and fibro-osseous lesions.5
Surgical curettage stands to be the most common surgical method for removal of central giant cell granulomas.11 Recurrence rates after curettage vary between 13% and 49%.12
The orthodontic management of this patient was postponed until the lesion had been completely removed. Challenges faced included cortical bony expansion and displacement of teeth. The bone resorption caused by the lesion resulted in reduced support of the LR1 and LR3. These teeth were deemed to have a poor long-term prognosis, but were given the benefit of the doubt by the surgeon, and were to be monitored as healing of the bony support occurred. The teeth have remained in situ and symptomless. However, caution should be exercised when orthodontically moving these teeth in the future as they are at increased risk of loss of vitality, further mobility, root resorption and possible ankylosis. The patient and parent must be informed of these risks and the orthodontic treatment completed using light forces, minimum treatment duration, and careful clinical and radiographic monitoring of these teeth in particular.
Central giant cell lesions, or central giant cell granulomas (CGCG) are uncommon lesions of the jaw, and can often develop without symptoms. They are, however, invariably destructive to the tissues, and so a high degree of caution should be exercised by clinicians when diagnosing these lesions. Focus should be placed on primary care practitioners referring patients in a timely manner to avoid progression of a potentially aggressive lesion. Clinicians should note that uncommon lesions of the jaw may present at any age, and may well reoccur in a patient's lifetime. The orthodontic team should be aware of lesions that may occur in their patient cohort and the potential impact this may have on their future orthodontic treatment.
