Giant cell tumor (GCT) of bone is a giant-cell-rich bony lesion associated with abundant multinucleated osteoclast-type giant cells. It is a primary neoplasm of bone with characteristic clinical, radiological, and pathological features. It is an expansive and lytic lesion without periosteal reaction and prominent peripheral sclerosis. Giant cells are also seen in other diseases like giant cell granuloma of the jaws, traumatic bone cyst, aneurysmal bone cyst, and jaw tumor of hyperparathyroidism. We present a unique case of GCT of palate in a 30-year-old female.
Giant cell tumor of bone or Osteoclastoma is a benign giant cell tumor characterized by mononuclear cells proliferation intermixed with multinucleated osteoclast-like giant cells. However, because of their unpredictable nature, these lesions are no longer termed as “Benign.” The mononuclear cells, although considered to be nonneoplastic and reactive in nature, they are seen in distant lung metastases [
A 30-year-old female patient reported to the Department of Oral Pathology, with a swelling over the left side of the palate. Past history revealed that the patient had initially noticed the swelling 6 weeks ago. She had consulted a private dentist when the swelling was approximately 1.5 × 1.5 cm in size and had no associated symptoms (Figures
Swelling in the left maxillary region.
Proliferative growth of size 6 cm × 5 cm with irregular margins, extending from the 24 region up to the posterior part of the hard palate crossing the midline.
Heterogenous, well-defined, intensely enhancing lesion measuring 3 × 4.1 × 4.3 cm (cc × ap × trans) seen involving left side of buccal mucosa and the hard palate with displacement of lingual septum to right.
H&E 10x view showing vascular stroma with proliferation of spindle cells intermixed with extravasated RBCs.
H&E 40x view showing anastomosing vascular channels lined by atypical endothelial cells.
Immunohistochemical staining: showing positivity for endothelial cells to CD34 and negativity for tumor cells.
Patient was placed in supine position and GA was administered. Right nasotracheal intubation was done. Considering the angiomatous nature of the lesion in maxilla, prior to Maxillectomy, the ECA was exposed and held for immediate ligation in case of untoward hemorrhage. The surgery was done as two stages: (1) neck and (2) maxilla.
Skin incision was placed on Resting Skin Tension Line on the left side of the neck, followed by layer-by-layer dissection. Weber-Ferguson incision was placed on the left side and layer-by-layer dissection done to locate the left maxillary buttress region. Osteotomy was done at Lefort I level from left pyriform aperture to maxillary tuberosity region. After complete excision of the lesion with adequate clearance, an obturator was placed over a Bismuth Iodide Paraffin Paste pack. The resected tumor was sent for histopathological examination.
Histopathological examination of the soft tissues revealed an encapsulated mass comprising stratified squamous epithelium and underlying richly cellular connective tissue stroma, containing plenty of multinucleated giant cells and dilated blood capillaries. H&E 40x view showed multinucleated giant cells with agglomeration of around 20–40 hyperchromatic nuclei in the center surrounded by clear cytoplasm and pleomorphic proliferating stromal cells. Some of the sections showed the increased vascularity with extravasation of red blood cells. Cellular pleomorphism and mitotic figures with an average of 4 per high power view were also seen which indicates local aggressiveness of this lesion (Figures
H&E 10x view vascular stroma with multinucleated giant cells.
H&E 10x view overlying epithelium, connective tissue capsule, neoplastic areas showing proliferation of stromal cells, and multinucleated giant cells.
H&E 40x view showing multinucleated giant cells with agglomerate of nuclei in the center with a clear cytoplasmic halo.
H&E 40x view showing cellular pleomorphism and mitotic activity.
Giant cell tumor is rare and benign tumor of bone. It occurs in approximately one person per million per year [
Based on clinical features and radiological and histological features, staging classification was initially proposed by Campanacci [
Giant cell tumor of bone causes localised severe intractable epistaxis, proptosis, visual defects, hearing loss, tinnitus, reduced joint mobility, and swelling [
The classic radiological findings of giant cell tumor often reveal a well-circumscribed lytic lesion enclosed by minimal or no sclerosis. Tumors may break through the cortex and invade the adjacent soft tissues. A CT scan of lesion shows soft tissue mass, bony destruction, perforation of cortex, extension toward adjacent anatomic structures, resorption of teeth, and perforation of bundle bone [
The appearance of gross findings of GCT of bone is variable. It is generally soft, purple-red to brown, and meaty and may be uniform or variegated in aspect, with small, spongy yellow foci or extensive areas of cystic changes [
Metastasis in GCTs ranges from 1 to 6%. Lung is the main site where metastasis usually occurs [
The level of serum alkaline phosphate in our case was 320 U/L (normal level: 45–129 U/L). Histochemical and quantitative chemical methods show high levels of alkaline phosphate in relation to osteogenic matrix of giant cell tumor [
Giant cells can also be found in certain other giant cell lesions such as central giant cell granuloma, brown tumors of hyperparathyroidism, and aneurysmal bone cysts [
Central giant cell granuloma is a proliferative lesion, which is usually seen in young females. Clinically, it appears that destructive lesion, definite loculations and histopathological presence of proliferating spindle-shaped fibroblasts, collagen fibers, deposits of hemosiderin, patchy distribution of multinucleated giant cells, and signs of bleeding into mass are present usually on maxilla followed by mandible, whereas in our case there was no definite loculations noted [
Brown tumors of hyperparathyroidism show bone cysts, bone resorption, and generalized osteopenia. The most common sites are ribs, clavicle, pelvic girdle, and mandible. Deposits of hemosiderin and vascularity and presence of hemorrhage are responsible for arriving at a diagnostic terminology as “brown tumor.” Histologically, these tumors are characterized by several osteoclast-like multinucleated giant cells interspersed with infiltration of hemorrhage and deposits of hemosiderin [
Aneurysmal bone cysts are usually seen in vertebral column and mandible. They consist of blood filled spaces separated by fibrous septa, multinucleated giant cells, and osteoid and presence of hemosiderin and bone formation. Conventional type of ABC shows soft tissue invasion, expansive and rapid growing destructive lesion causing cortical perforation, whereas in our case absence of blood filled spaces and hemosiderin pigments were seen [
Treatment of GCT usually consists of intralesional curettage with autograft reconstruction [
Literature review of previously reported cases of oral cavity with treatment aspects.
S. number | Authors | Year | Gender/age | Site | Follow-up | Recurrence | Treatment |
---|---|---|---|---|---|---|---|
1 | Koszel et al. [ |
2011 | 17 M | Maxillary alveolar process | 2 years | No recurrence | Surgical removal |
2 | Pradhan et al. [ |
2003 | 19/F | Jaw bones, orbit | Every 6 months | No recurrence | Subciliary, transperiosteal anterior orbitotomy |
3 | Giri et al. [ |
2015 | 12/F | Mandible | 3 years | No recurrence | Surgical resection |
4 | Anand et al. [ |
2001 | 20/M | Hard palate | Eight months | No recurrence | Surgical excision |
5 | Mishra and Shukia [ |
1999 | 6/M | Upper alveolus, cheek | 3 years | No recurrence | Surgical removal |
6 | Saha et al. [ |
2012 | 45/M | Maxilla | — | No recurrence | Partial anterolateral maxillectomy |
Alcohol, hydrogen peroxide, zinc chloride, and phenol are usually applied to the lesional site. Application of hydrogen peroxide raises the infiltration of phenol into adjacent tissues. Low recurrences rate has been related to chemical adjuvants. Embolisation can be achieved by polyvinyl alcohol particles, coils, and gelfoam. Serial embolisation in large cortical effects has reduction in morbidity rate, preserve function, and relieve pain [
Various bone tumors reveal multinucleated giant cells which often should be differentiated from GCT. Early diagnosis of GCT can be done with evaluation of all the radiographic, biochemical, and histopathological limits. To attain a proper diagnosis, careful histopathological assessment is mandatory. Our case describes the difficulty in diagnosing giant cell tumors from various other lesions with which they contribute to similar behaviour, histopathology, and prognosis.
The authors declare that there is no conflict of interests regarding the publication of this paper.