|Year : 2020 | Volume
| Issue : 1 | Page : 60-62
Temporal bone giant-cell tumor with secondary aneurysmal bone cyst: A rare case report and review of literature
Venkateswara Rao Kommu1, Sudha S Murthy2, Rashmi Sudhir3, Swarna Ch Kumari4
1 Department of Neurosurgery and Neuro-Oncology, Basavatarakam Indo-American Cancer Hospital and Research Institute, Hyderabad, Telangana, India
2 Department of Laboratory Medicine, Basavatarakam Indo-American Cancer Hospital and Research Institute, Hyderabad, Telangana, India
3 Department of Radiodiagnosis, Basavatarakam Indo-American Cancer Hospital and Research Institute, Hyderabad, Telangana, India
4 Department of Radiation Oncology, Basavatarakam Indo-American Cancer Hospital and Research Institute, Hyderabad, Telangana, India
|Date of Submission||24-Jan-2020|
|Date of Acceptance||12-Apr-2020|
|Date of Web Publication||2-Jul-2020|
Dr. Venkateswara Rao Kommu
Department of Neurosurgery and Neuro-Oncology, Basavatarakam Indo-American Cancer Hospital and Research Institute, Hyderabad, Telangana
Source of Support: None, Conflict of Interest: None
Giant-cell tumor (GCT) and aneurysmal bone cyst (ABC) are locally aggressive and benign in nature. They usually occur in the extremities of the long bones. Involvement of the skull base or cranial vault is very rare. Both are different in pathologically and cytogenetically. GCT can be associated with ABC, which are likely reactive in nature. To the best of our knowledge, only two cases are reported coexisting GCT with ABC in the cranial vault. Here, we report the third case of coexisting GCT secondary ABC of the temporal bone and the first case of temporal bone coexisting GCT with ABC.
Keywords: Aneurysmal bone cyst, giant-cell tumor, temporal bone tumors
|How to cite this article:|
Kommu VR, Murthy SS, Sudhir R, Kumari SC. Temporal bone giant-cell tumor with secondary aneurysmal bone cyst: A rare case report and review of literature. Int J Neurooncol 2020;3:60-2
| Introduction|| |
Giant-cell tumor (GCT) and aneurysmal bone cyst (ABC) are benign bone tumors with aggressive behavior. Only 2% GCT occur in the craniofacial region and most commonly involves the middle cranial fossa bones. The common clinical symptoms of temporal bone GCT include otalgia, aural fullness, conductive or sensory neural deafness, localized temporal bone or preauricular swelling, and temporomandibular joint dysfunction. Up to 50% cases may present with local recurrence, and in some cases, lung and lymph nodal metastasis was also described. Giant-cell tumors are one of the most common causes of the secondary aneurysmal bone cyst, which are most common in the long bones, but very rare in the skull bones. Secondary ABC in GCT comprises 14% of all GCTs. Other causes of the secondary ABC include osteoblastoma, chondroblastoma, fibrous dysplasia, and osteosarcoma. To the best of our knowledge, GCT- and ABC-associated presentations in the skull base or calvarium are only two cases in the literature; ours is the third case.
| Case Report|| |
A 34-year-old male patient presented with gradually progressive swelling just above the right ear tragus associated with occasional pain in the swelling. No history of hearing difficulty, tinnitus, and facial weakness was noted. On examination, small swelling just above the right ear tragus skin over the swelling is pinchable, hard in consistency, immobile, and no tenderness over the swelling. Computed tomography (CT) showed osteolytic lesion involving temporal bone with hyper dense soft tissue compressing the temporal lobe. A well-defined expansile lytic lesion mass measuring 6.5 cm × 4.4 cm × 4.5 cm predominantly hypointense on T1W imaging with small hyperintense area (hemorrhagic component) and heterogeneously hyperintense on T2W imaging with thin hypointense internal septate and thick peripheral hypointense rim is seen involving squamous and mastoid parts of the right temporal bone indenting over temporal lobe without invasion/edema [Figure 1]. On postcontrast imaging, the lesion shows heterogeneous enhancement, suggestive of hemangiopericytoma right temporal bone [Figure 2]. The rest of the skull bones are normal. The patient underwent right temporal craniectomy. Intraoperatively, the temporal bone is completely destroyed [Figure 3]. The outer layer of the dura was infiltrated by the tumor. On histopathological examination, sections studied show multiple fragments of bony spicules and fibrous tissue, harboring an infiltrating cellular lesion arranged in nests and sheets among the extensive areas of hemorrhage. The cells are oval having a moderate amount of cytoplasm, hyperchromatic nucleus, and prominent nucleoli in few. Focal area shows bundles of spindle cells having elongated hyperchromatic nucleus with prominent nucleoli lobules of cartilage are noted. Interspersed thin-walled dilated blood vessels of varying size are noted. Stroma shows extensive areas of hemorrhage with multiple osteoclasts-like giant cells. Status of margins cannot be commented as the tissue is received in fragments. On immunohistochemistry, CD31 and CD34 are negative, in the tumor cells, P53 was mutant and Ki67 was 6-8%. These features in correlation with morphology and radiologic findings were consistent with Giant cell tumor with Aneurysmal bone cyst [Figure 4].
|Figure 1: (a) Axial T1-weighted magnetic resonance imaging showing an extraaxial mass in the squamous part of the right temporal bone and zygomatic arch of mixed signal intensity with focal hyperintense areas suggesting hemorrhage. (b) Axial T2-weighted magnetic resonance imaging showing mixed signal-intensity mass with fluid-filled multiloculated cysts, multiple internal septations and peripheral hypointense rim around the mass. (c) Coronal T2-fluid-attenuated inversion recovery magnetic resonance imaging showing increased signal intensity of the mass with multiple internal septations and peripheral hypointense rim. The mass is compressing the brain parenchyma with mild midline shift|
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|Figure 2: (a-c) Axial, coronal, and sagittal sections of postcontrast T1-weighted magnetic resonance imaging showing heterogeneous enhancement with nonenhancing cystic and hemorrhagic areas with peripheral bony rim|
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|Figure 3: (a and b) Showing solid tumor mass lesion coming out of the cranial cavity by the destroyed temporal bone (c) tumor involved the outer layer of dura mater with normal brain parenchyma|
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|Figure 4: (a) H and E × 100-fragmented tissue bits with extensive areas of hemorrhage (b) bony spicules and lesion comprising multinucleated osteoclastic type of giant cells with stromal cells in the spatial arrangement (c) extensive areas of hemorrhage and tumor (d) immunohistochemistry for p53 is mutant (90%)|
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| Discussion|| |
Sir Astley cooper first describe the GCT in 1818 and its malignant potential by Virchow. GCT of the temporal bone was first described by Doderlin in 1913. GCT is locally aggressive and invasive benign tumor, comprising 4%–9.5% of all bone tumors and 18%–23% of all benign bone tumors. In the skull, sphenoid bone is the most common site followed by nonsquamous portion of the temporal bone. In the literature, a total of 110 cases of GCT were reported. Out of 110 cases, 37 were arising from the temporal bone, 20 patients of the sphenoid bone, six patients of the occipital bone, and two patients of the frontal bone. Out of 110 cases, no case has secondary ABC. It is slightly common female patients, with peak prevalence in the third decade of life. Stroma of the most GCT contains lot of vascular channels with hemorrhages which are associated with secondary ABC formation. Nearly 14% of GCT associated with secondary ABC formation. Differential diagnoses of temporal bone GCT include chondrosarcoma, chondroblastoma, giant-cell reparative granuloma, Brown tumor of hyperparathyroidism, ABC, and osteolytic bone metastasis. Fifty percent of reported cases of ABC are secondary to preexisting tumors such as GCT, chondroblastoma, chondromyxoidtumor, osteosarcoma, chondrosarcoma, fibrousdyplasia, and osteoblastoma. GCT arises from the nonosteogenic stromal cells of the bone marrow of epiphysis of long bone ends. Radiographically, it appears as radiolucent without sclerotic changes. On CT scan, no special appearance usually expanding lytic destructive lesion with cortical thinning and penetration and periosteal reaction. Magnetic resonance imaging (MRI) is the ideal test to determine the soft-tissue extension. On MRI, usually appear as hypointense on T1- and T2-weighted images with heterogeneous enhancement on contrast. In bone scintigraphy, both GCT and ABC revealed increased uptake around the periphery with central photogenic region (doughnut sign).. Histopathologically, it resembles the chondroblastoma, giant-cell repatative granuloma, aneurismal bone cyst, and fibrous dysplasia. Histopathology of GCT has characterized by gray-to-yellow brown with cystic areas macroscopically. Microscopically, it has three types of cells that include (1) multinucleated giant cells, (2) round monocyte resembling cells, and (3) spindle-shaped fibroblast-like stromal cells. Biopsy of the solid component only reveals the diagnosis. GCT stromal cells secrete receptor activator of nuclear factor kappa B ligand (RANKL), which has a main role in bone destruction. The detection of RANKL factor in bony tumors, which is a key factor in osteoclast biology, used as a good therapeutic target agent in chemotherapy. Complete surgical excision of tumor is the treatment of choice, but the presence of the important neurovascular structures hampers the en bloc resection. Bone curettage was associated with a high recurrence rate. Radiotherapy is reserved for incompletely excised tumors and the patients who are not fit for the surgery. Irradiation-induced malignant transformation is the described complication. Denosumab is the inhibitor of the RANKL, which is the drug of choice for recurrent and unresectable tumors. High-dose dexamethasone therapy associated with decreased size of the tumor, but discontinuation of the steroids associated with the tumor regrowth.
| Conclusion|| |
Although GCT is a common cause of ABC, the cooccurrence of these lesions in the skull is rare. According to our review of the literature, this is the third case of skull base or calvarium reported in the literature. All skull lesions looking like ABC should have a high index suspicion of underlying primary pathology. Exact diagnosis can only be made histopathologically. Gross total excision of the tumor is the most effective treatment of choice. Subtotal resection followed by radiotherapy will provide acceptable tumor control. The role of chemotherapy with denosumab is not completely established, but shows decreased volume of the tumor size and surgical morbidity.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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