Hospital del Niño DIF Hidalgo. Pachuca, Hidalgo. México
Central Giant Cell Granuloma or CGCG is a benign bone injury of the maxillas of origin non-odontegenic. It has epidemiology, histology, clinical and radiological characteristics well defined, and spit it shares similarities with other pathologies, its difference requires an intensive clinical and lab analysis. Due to its locally destructive nature and its high recurrence, its treatment is complex. For this reason, it is necessary surgical procedures which are able to provoke important aesthetic and functional effects. In this article apart from collecting information about CGCG, it has presented the case of a 11-year old child who presents such injury in the upper maxilla giving his pathologic descriptions and the description of the studies in his treatment.
Keywords: Central Giant Cell Granuloma; Maxilla; Pediatric patient
Central Giant Cell Granuloma (CGCG) of the maxillas of a non odontogenic and non frequent injury. 1, 2. Defined by the World Health Organization (WHO) as an intraosea injury conformed by fibrous cellular tissue, which contains multiple focal hemorrhages, hemosiderin deposits, aggregate of multinuclear giant cells and sometimes trabeculaes of woven bone. 3, 4 CGCG was described as an affection of the maxillary bones by Jaffe in 1953, who proposed the term Giant Cell Reparative Granuloma (GCRG); however, due to the fact that the clinical behavior of many of these injuries is not compatible with the repair process, some authors prefer to omit the term “reparative” and use the term central giant cell granuloma 4 -6. This injury can appear at any age, although, it is observed a greater incidence in children and young adults, with predilection for females in a relation 2:1. This predilection has been suggested because of the the ovarian hormones such as estrogen and progesterone7. They are more common in the jaw, presenting in the maxilla in 30%. They affect the molar area, premolar area or the antero-inferior sector usually crossing over a mid line. Though, different cases of condilar affection have been described, they can involve the structure in the extramaxillar bones, more frequently being found in the crane region (orbit, nasal sinus, cranial vault, temporal bone, sphenoids and ethmoid bone), as well as the extracranial way in hand and foot bones 1, 4, 6-8
The pathogenesis of the CGCG is unknown, several authors have suggested that it is produced as a result of minor recurring or secondary hemorrhage to a trauma. The injury regarded would represent an abnormal healing process followed by repeatedly intramedullary haemorrhages9.
On the basis of its clinical and radiographic characteristics Choung et al10 classified the CGCG in two categories: 1.- NON-AGRESSVE: Injuries characterized by a minor sintomathology or its absence, slow growing, non production of perforations in the cortical bones or root absorption; 2.- AGGRESSIVE: Injuries with fast growing, they produce pain, paresthesia, root resorption, expansion and/or perforation of the cortical bone and high recurrence after the surgical curettage: 1, 6, 8, 10 . In 1987, Ficarra et al 11 confirmed that the severe injuries which have greater size and they present histologically huge areas occupied by giant cells, they are more frequent in young patients and constitute one of each 5 cases of CGCG1.
The majority of CGCG is presented as expansive injuries of slow, progressive, asynthompt growing and they are detected in a non-intentional way during an ordinary radiographic evaluation. In some cases at the intraoral level, it is observed a brownish-blue injury and depending on its size and location can produce movements and/or dental mobility. When the injuries have destroyed and have made thinner the cortical bone, it is observed in the mouth, a bluish-red on the alveolar ridge or on the vestibule-oral part12. The injury expansion in the maxilla can move up the sinus floor and sometimes it is necessary to remove it together with the injury, this expansion is faster due to its spongy nature and its thin cortical parts. Also, the orbit or nostrils can be involved.
Radiographically, CGCG produces injuries of the craniofacial bones which vary from a minor rounded or elliptic unilocular radiolucid injury to severe multilocular injuries to show a thin trabeculae of sharp edges with a minor sclerosing reaction. The bone can appear expanded with dental mobility, dental germs and root resorption, also there is an expansion of bones and occasionally with perforations on the cortical parts 1, 5, 13. Even though, the x-rays are useful, the images from computed tomography (CT) help to define in a better way the extent of the injury, in the case of the maxilla, the injury to the sinus is represented with the perforation of the upper maxilla, the zygomatic bone and the destruction of the medial and lateral bony wall of the sinus 4. The diagnosis is established during the clinical, radiological study, lab exams and in order to confirm, it is necessary an incisional biopsy. Nevertheless, in minor injuries is preferable to make the complete exeresis of the injury and eventually the histological study1.
CGCG consists in a fibroblast stroma constituted by multinucleate giant cells, which have variable shapes and sizes, distributed forming small scattered accumulations. The vascular density is prominent dinstinguised with the marked extravasation of erythrocytes and hemosiderin deposits5-8. The stroma varies from laxo to fibrous, having the possibility to appear dystrophic calcification and metaplastic ossification focals located especially on the periphery of the injury. There are focal hemorrhages with distinct age, which periphery can be surrounded by giant cells, the quantity of their cores varies from few to several dozens. There is scientific evidence which suggests that the giant cells represent osteoclasts6. It has been suggested that the injuries which present a great amount of multinucleate giant cells uniformly distributed into a predominantly stroma cell, can be clinically more aggressive and with a major tendency to determine surgery as a necessary treatment.
It has been planned the differential diagnosis with another type of process which presents a hospital and a similar aspect5, 8, 9. 1. Apical cyst: It appears at the root of an erupted tooth, which its pulp has been devitalized because of tooth decays or dental trauma, it has a variable size, but generally it measures 1 cm in diameter. The light of the cyst contains liquid, giving it a serous microscopic appearance (humoral) contrary to the CGCG which has a fibrous aspect. Radiographicaly, it is presented in a well circumscribed rounded raduolucid zone, 2. Giant cell tumor of bone (GCT): It is a benign growth, but locally aggressive, made up of rounded, ovoid or extended mononuclear neoplastic cell sheet and spit there are abundant mononuclear giant cells, they are distributed uniformly; they are big and contain from 50 to 100 cores; usually they appear on the epiphyses of the large bones; rarely they have hemosiderin deposits, fibrosis or bleeding zones 5, 7., 3. Brown tumor of hyperparathyroidism: it is the excessive production of the parathyroid hormone because of the parathyroid glands, which control the levels of calcium, phosphorus and vitamin D in blood and bones. This tumor is clinically, radiographically and histologically similar to the CGCG; therefore, it is necessary biochemical studies to determine the levels of calcium, which normal values ignore such diagnosis 5, 12, 4. Cherubism: It is a dominant and self-limited hereditary autosomal disorder which provokes a progressive, symmetric and bilateral increase of the maxilla and/or jaw. Histologically, it is different from the CGCG since it presents multinucleate giant cells usually scarce, scattered around the blood capillaries and they tend to form small forces adopting a patched pattern 3, 7, 9,. 5. Metastasis: It is easy to ignore when there are not anaplasia signals, epithelial added or figures of abnormal metastasis.
The habitual and traditional treatment of the CGCG of the maxillas is the surgical exeresis and can vary from the simple curettage, surgical usage of the surgical wound, resection of the injury with periphery osteotomy up to the resection in the block of the same one; in those cases, patients have important effects such as loose of dental germs, loose of the adjacent teeth to the injury or to the extense maxillary zones which produces defects in shape and function of the maxillas2, 6, 8. Over time, this has meant aesthetic, functional and emotional problems. The usage of the reconstruction technique using fibular graft has demonstrated excellent results since it not only provides continuity and reestablishes the jaw contouring, it also can be placed into the undertow, dental implants which improves the subsequent prosthetic restoration14,. The surgical management with an aggressive curettage consists of the curttetage of the tumoral mass followed by the surgical removal of peripheral bony edges (peripheral osteotomy), these injuries are the ones with major frequency of recurrence 8,. Eisebund et al. 13, reports that on the superior maxilla, a simple curettage is engaging for presenting a high rate of recurrence; however, due to the fact that the anatomical characteristics of it (its thin cortical parts and the fact that the maxillary sinus is involved with frequency) the peripheral ostotomy become a difficult procedure; for instance, in this case is profitable a curettage instead of a partial maxillectomy 8. The CGCG is an injury gratefully vascularized. Thus, the access to this injury should be made carefully and a contingency plan should be ready in case of presenting severe haemorrhage8. The alternative of the surgical treatment is the application of intralesional injections of corticosteroid, which are applied weekly in order to obtain imaging evidence of an important decrease of the size of the injury until its involution 6, 8. Other alternatives are the administration of calcitocin, even when several factors can affect the results such as the form of administration (injections under the skin or nasal spray) or the different types of calcitonin (human or of salmon), as well as the variable expressions of the receptors of calcitocin 17. Another alternative is the use of injections under the skin of the interferon alpha, despite the adverse effects include fever, symptoms similar to flu, lethargy, runny nose, rash and loss of hair 15, , it can present spastic diplegia, neutropenia, thrombocytopenia and high levels of liver transaminases, these are signals that determine if the therapy stops or the dose of interferon is reduced 16,. The factors that can mean a major risk of recurrence are: The clinical activity of the injury (there is major recurrence on its aggressive way), young age, presence of perforation of the cortical bone and the size of the tumor (injuries with a diameter upper than 3.0 cm) 7, 12.
It is presented a 11-year-1-month male patient apparently healthy, who is dispatched to the Department of Maxillofacial Surgery Services of the Children Hospital DIF Hidalgo (Spanish: Servicio de Cirugía Maxilofacial del Hospital del Niño DIF Hidalgo) in order to examine him since he has presented an increase of the volume in the facial region.
The patient’s mother says the beginning of the symptoms of 5 months of evolution, observing a facial asymmetry in relation to the right zygoma regions which increased since the beginning until now. With an extraoral exploration is observed in a frontal and caudocephalic projection and an increase in the volume in the right zygoma regions with colored teguments similar to the contralateral left side face, the bases of the alas of the nose are slightly elevated and nasolabial furrow are not very marked. (Images 1 and 2)
Intraorally, we can observe: a wound in the right dental reach with a movement of the vestibular corticals and palatine covering from the canine tooth until the mandibular second premolar, a gingival tissue in bright-red-rash color without reports of active bleeding or secretion of any kind of important movement of lateral dental organs and first right maxillary molar, and absence of right canine tooth.
In the imaging tests of Computed Axial Tomography (CAT) of solid facial saggital axial sections, coronal part and three-dimensional reconstruction, we can observe a hipodense zone in relation to a right side portion of a maxilla covering the canine tooth up to the first ipsilateral premolar level observing maxillary canine tooth moved to the region of the floor of the right nostril which is approximately 4.13 x 2.93 cm in the axial projection. At its media-lateral limit, we found that the wound is invading the floor of the right nostril of a one-third inferior of the Piriformis cutout on the same side, maxillary zygomatic buttress and a third of cleft palate of the right maxilla. (Image 4).
It was decided to do a biopsy of maxillary injury to start the protocol of the study. Under general balanced anesthesia and endotracheal intubation proceeds an insicional biopsy with the design of trapezoidal flap in relation of right vestibular relations, finding later it with abundant vascularity and the invasion of it to a right nostril and perforation of a cortical palatine ipsilateral. Colgajo is readjusted with 3-0 vicryil and it is sent a sample of the anatomopathological study.
In the next 7 days we found that the patient presents an adequate recovering of the healing process of the surgical wound in optimal conditions (image 6) and we were reported in the anatomopathological diagnosis of the Central Giant Cell Granuloma. (Image 7) The patient is sent to a third level for an adequate treatment.
The central giant-cell granuloma or CGCG of the jaws is a benign bone lesion of the maxillary bones of which origin is not odontogenic. It contains epidemiological, histological, well-defined clinical and radiological features and despite it shares similarities with other pathologies, its difference requires a deep analysis of both clinical and laboratory aspects. The biggest problem with this wound, apart from its diagnosis, is the fact that it is related to the treatment, but as well as other pathological conditions, the risk-benefit of using a particular therapy should always be evaluated in each patient.
This article states that the CGCG has a local destructive capacity and has shown a recurrence of 11-49%, which leads to repetitive surgical procedures whose benefits have been questioned since they leave important aesthetic and functional consequences such as the loss of the bone structure and teeth and/or dental germs. These consequences are probably of major importance when the injury affects children and teenage population. Currently, alternative non-surgical treatment is being studied; however, its results in success are highly variable, such as: intralesional corticosteroid injections, administration of calcitonin, or the use of subcutaneous injections of interferon alpha15-17. In our institution, we do not have experience in the use of surgical and non-surgical treatments in the management of these injuries, so we decided to send the patient to a third level for proper monitoring.
Image 1: Frontal photograph of the patient.
Image 2: Caudo-cephalic photograph of the patient
Image 3: Axial Tomography of the patient by computer.
Image 4: Caudo-cephalic view of a three-dimensional reconstruction.
Image 5: Intraoral Transsurgery photograph of the patient.
Image 6: Post-surgical control of the patient 7 days after surgery
Figure 7: Multicored Osteoclast-type giant cells in patient’s biopsy.
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