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Giant Cell Tumor of Skull Base: Management and Review of Literature
Abstract
Giant cell tumors (GCTs) of bone constitute 3%–5% of all the bone tumors. GCTs are uncommon, locally aggressive, osteolytic neoplasms commonly seen in early adulthood. Involvement of cranial and facial bones is rare and comprises 2% of all the cases. Sphenoid and temporal bones are the most common sites of GCTs in head and neck. Although complete resection may not be possible, feasibility of partial resection depending on the extent and involvement of critical structures should be the best alternative. GCTs, though historically considered radioresistant, have a dose-dependent effect. This is a case report of a 20-year-old male, who presented with headache, blurring of vision in both the eyes, and diplopia for 2 months. Magnetic resonance imaging (MRI) showed a 4.5 cm × 3.4 cm × 3.5 cm lesion involving superior body of clivus, sphenoid sinus, and floor of sella, adherent to anterior pituitary gland along the superior aspect, indenting optic chiasm with extrinsic compression and bulging into bilateral cavernous sinuses. The patient underwent endoscopic transnasal partial resection of the lesion. Histopathological examination showed tissue containing numerous osteoclasts such as giant cells separated by round to spindle-shaped mononuclear cells. On immunohistochemistry, the giant cells were positive for CD 68 with Ki 67 proliferation index about 15%–20% in highest proliferating areas. The patient underwent definitive radiation treatment to a radiation dose of 55 Gy in 31 fractions over 6 weeks. The patient tolerated treatment well with minimal toxicities, his vision improved by the end of treatment. The patient needs to be assessed for radiological response and late side effects. GCTs of the skull are rare benign osteolytic locally aggressive lesions generally seen in early adulthood. En bloc surgical resection is the treatment of choice but has high local recurrence rates. Radiation is the treatment of choice in unresectable, partially resected, and recurrent tumors. With the advent of advanced techniques such as intensity-modulated radiotherapy/volumetric-modulated arc therapy, better dose distribution can be achieved in the target minimizing dose to the critical structures. Imaging with daily kilovolt cone-beam computed tomography is essential in the treatment of tumors of the skull for precise treatment delivery.
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Giant cell tumor is a rare disease of the head and neck region. Predominantly giant cell tumor involves the end of long bones. Sometimes it occurs in the head and neck areas, predominantly in mandible and maxilla. The tumor arises from the nonosteogenic stromal cells of the bone marrow. Giant cell tumors are defined as either aggressive or nonaggressive lesions. An aggressive type tumor is large, rapidly growing, loose and displaced teeth. Nonaggressive type giant cell tumors do not exhibit these signs. Surgery is the most accepted treatment of giant cell tumor. Sometimes surgery can be performed in association with new approaches, in an attempt to avoid recurrence. Surgical treatment can be followed by local injection of steroids or antiangiogenic therapy using interferon alfa-2a. We report a 17-year-old girl with a large, slowly growing giant cell tumor of the maxilla. The patient was treated with surgical excision.
Giant cell tumour of soft tissue is an extremely rare tumour. It is thought to be the soft tissue counterpart of giant cell tumours of the bone
due to its histological and immunohistochemical resemblances. Almost 80% of these tumours occur in upper and lower extremities;
neck is a very rare location. Here we describe a case of primary soft tissue giant cell tumour in right submandibular region. A 35-yearold male patient presented with a swelling in right submandibular area. FNAC suggested a benign soft tissue neoplasm, comprising of
spindle cells and multinucleated giant cells. Histopathology of resected specimen showed spindle cell tumour with intricately mixed giant
cells, consistent with a primary giant cell tumour of soft tissue. Giant cells were CD 68 positive. Giant cell tumour of soft tissue is benign
tumour, though very rarely can show features of malignancy. We present this case for its rarity and morphological overlap with other soft
tissue neoplasms containing giant cells.
Giant cell tumour of bone (gctb) is one type of giant-cell-rich bone lesion characterized by the presence of numerous multinucleated osteoclast-type giant cells. Giant cells are known to express rankl (receptor activator of nuclear factor κB ligand) and are responsible for the aggressive osteolytic nature of the tumour. No available treatment option is definitively effective in curing this disease, especially in surgically unsalvageable cases. In recent years, several studies of denosumab in patients with advanced or unresectable gctb have shown objective changes in tumour composition, reduced bony destruction, and clinical benefit. Denosumab is a fully human monoclonal antibody that targets and binds with high affinity and specificity to rankl. Several large phase iii studies have shown that denosumab is more effective than bisphosphonates in reducing skeletal morbidity arising from a wide range of tumours and that it can delay bone metastasis. The relevant articles are reviewed here. The controversies related to the future use of denosumab in the treatment of gctb are discussed.
Malignant giant cell tumor (GCT) of bone is a rare tumor with debilitating consequences. Patients with GCT of bone typically present with mechanical difficulty and pain as a result of bone destruction and are at an increased risk for fracture. Because of its unusual occurrence, little is known about the epidemiology of malignant GCT of bone. This report offers the first reliable population-based estimates of incidence, patient demographics, treatment course and survival for malignancy in GCT of bone in the United States. Using data from the National Cancer Institute's Surveillance, Epidemiology and End Results (SEER) program, we estimated the overall incidence and determinants of survival among patients diagnosed with malignant GCT of bone from 1975-2004. Cox proportional hazards regression was used to evaluate demographic and clinical determinants of survival among malignant GCT cases. Based on analyses of 117 malignant GCT cases, the estimated annual incidence in the United States was 1.6 per 10,000,000 persons per year. Incidence was highest among adults aged 20 to 44 years (2.4 per 10,000,000 per year) and most patients were diagnosed with localized (31.6%) or regional (29.9%) disease compared to distant disease (16.2%). Approximately 85% of patients survived at least 5 years, with survival poorest among older patients and those with evidence of distant metastases at time of diagnosis. The current study represents the largest systematic investigation examining the occurrence and distribution of malignancy in GCT of bone in the general U.S. population. We confirm its rare occurrence and suggest that age and stage at diagnosis are strongly associated with long-term survival.
Giant cell tumors are rare neoplasms, representing less than 5% of all bone tumors. The vast majority of giant cell tumors occurs in extremity sites and is treated by surgery alone. However, a small percentage occurs in pelvis, spine or skull bones, where complete resection is challenging. Radiation therapy seems to be an option in these patients, despite the lack of a generally accepted dose or fractionation concept. Here we present a series of five cases treated with high dose IMRT.
From 2000 and 2006 a total of five patients with histologically proven benign giant cell tumors have been treated with IMRT in our institution. Two patients were male, three female, and median age was 30 years (range 20-60). The tumor was located in the sacral region in four and in the sphenoid sinus in one patient. All patients had measurable gross disease prior to radiotherapy with a median size of 9 cm. All patients were treated with IMRT to a median total dose of 64 Gy (range 57.6 Gy to 66 Gy) in conventional fractionation.
Median follow up was 46 months ranging from 30 to 107 months. Overall survival was 100%. One patient developed local disease progression three months after radiotherapy and needed extensive surgical salvage. The remaining four patients have been locally controlled, resulting in a local control rate of 80%. We found no substantial tumor shrinkage after radiotherapy but in two patients morphological signs of extensive tumor necrosis were present on MRI scans. Decline of pain and/or neurological symptoms were seen in all four locally controlled patients. The patient who needed surgical salvage showed markedly reduced pain but developed functional deficits of bladder, rectum and lower extremity due to surgery. No severe acute or late toxicities attributable to radiation therapy were observed so far.
IMRT is a feasible option in giant cells tumors not amendable to complete surgical removal. In our case series local control was achieved in four out of five patients with marked symptom relief in the majority of cases. No severe toxicity was observed.
Objective:
The current literature regarding cranial giant cell tumor (GCT) management includes scattered case reports and small case series. We present a comprehensive literature review and meta-analysis on this subject, along with a case report describing our management of a temporal GCT.
Methods:
A systematic literature review of all reports on GCTs of the skull was performed followed by a meta-analysis examining the effect of radiation and degree of resection on tumor recurrence.
Results:
Fifty-nine abstracts (1945-2015), reporting 110 GCT cases were reviewed. After exclusions were applied 31 manuscripts (67 patients) were selected for meta-analysis. Average age was 33.7 years old with 1:1 gender ratio. Tumor location: temporal (37), sphenoid (20), occipital (6), frontal (2), TMJ (2). Treatment methods: surgery + radiation (25), surgery alone (41) or radiation alone (1). Degree of resection for 66 surgical patients was: 34 gross total (GTR), 31 subtotal (STR), 1 not recorded. Mean f/u time: 36 months. Recurrence occurred in 8.8% of the GTR group and 32.3% of the STR group (OR= 0.203, 95% CI: 0.033-0.937, p= 0.018). Odds ratio for STR alone compared to STR + radiation was 14.01 (p=0.0038).
Conclusion:
GCTs of the skull commonly affect young adults with an equal gender distribution and are often centered in temporal bone. GTR results in lowest recurrence rates and should be the goal of treatment. If GTR cannot be achieved, the combination of STR and radiation results in similar recurrence rates. With the advent of Denosumab, there is now a role for chemotherapy in the treatment of GCTs.
Giant cell tumors (GCT) of the bone are uncommon primary bone neoplasms that occur mainly in the epiphyses of long bones. However, GCT with tumoral growth in the cranium is rarely encountered, particularly in the temporal bone. GCT demonstrates benign histological features but is locally aggressive, and surgical excision is the treatment of choice. The authors describe a 31-year-old man who presented with trismus and an extensive GCT, which originated in the petrous and squamous portions of the temporal bone and extended into the left mandibular ramus and middle ear. He was treated by en bloc resection of the tumor by left temporal craniectomy using an extended pterional approach and canal wall down mastoidectomy. In addition to the description of this unusual case of GCT of the temporal bone presenting with trismus, a review of the related literature is included.
Objective:
To study the clinical features, tumor characteristics and outcomes of giant cell tumors (GCTs) in the skull base based on long-term follow-up. We also report the largest series of GCTs in the temporal bone and the lateral skull base.
Materials and methods:
A retrospective study was conducted of all GCTs managed at the Gruppo Otologico, a quaternary referral skull base institute, in Italy from 1993 to 2013. The clinical features, investigations, surgical management and follow-up were recorded. The surgical approaches used were infratemporal fossa approach (ITFA) type B and D and middle cranial fossa (MCF) approaches.
Results and observations:
A total of 7 patients with GCTs of the skull base were treated at our institution. The principal complaints were hearing loss reported in 6 (85.71%) patients, tinnitus in 5 (71.43%) and swelling in 3 (42.9%). Pure-tone audiometry showed conductive hearing loss in 5 (71.43%) patients. High-resolution CT scan and MRI with gadolinium enhancement were done in all patients. Radiology showed involvement of the ITF and middle ear in 6 (85.71%) patients each, temporomandibular joint in 4 (57.14%) patients, invasions of the squamous part of the temporal bone, mastoid, MCF and greater wing of sphenoid in 3 (42.9%) patients each and the petrous bone in 2 (28.6%) patients. ITFA type B was applied as an approach for tumor removal in 5 (71.43%) patients, including a case where an additional MCF approach was employed, and ITFA type D and the transmastoid approach were applied in 1 (14.3%) patient each. Total tumor removal and successful cure was achieved in 6 (85.71%) patients. Subtotal removal leading to recurrence and eventual mortality was the result in 1 (14.3%) patient.
Conclusions:
A thorough knowledge of the anatomy of the skull base and the various skull base approaches is necessary to tackle GCTs. ITFA type B and D combined with MCF approaches provide good exposure of the tumor with minimal postoperative sequelae and good locoregional control. Recurrence due to either subtotal removal or suboptimal treatment may have disastrous consequences for the patient.
Giant cell tumors (GCTs) are generally benign, locally aggressive lesions mostly located in the metaphysis of long bones. GCTs of the skull are rare and the majority of the cases have been presented as case reports. The authors retrospectively reported 18 patients with GCTs of the skull at a single institution from April 1994 to February 2012 and summarized the clinical, radiological, pathological characteristics and management of the disease. Meanwhile, a systematic review of 94 case reports of GCTs of the skull was performed. Headache and symptoms related to the involvement of intracranial nerves were the most common symptoms. Over 90 % of the tumors originated from sphenoid and temporal bones. On MRI, very low signal on T2-weighted images were found highly indicative of GCTs of the temporal bone. Univariate analysis revealed that extent of tumor resection and post-operative radiation therapy (RT) were prognostic factors significantly influencing the survival of the patients. We concluded that complete tumor resection is the optimal goal in treating this disease and adjuvant RT should be given once tumor residual is inevitable.
To assess the outcomes of radiotherapy, in terms of local control and treatment complications, of advanced or difficult giant cell tumors of bone (GCTB) that could not be treated by surgery.
Among 122 consecutive patients with confirmed GCTB from 1985 to 2007, 77 patients were treated by megavoltage radiotherapy because they were inappropriate candidates for surgery. We have performed analysis of all data in terms of progression-free survival (PFS) and treatment morbidity. Median follow-up time was 58 months.
In the irradiated group, maximal tumor size ranged from 5 to 18 cm (median, 8.5). Anatomic distribution was as follows: femur, 27 cases; tibia, 19; radial/ulnar bone, 12; sacrum, 9; pelvic bones, 5; other, 5. Twenty-one patients (27%) were referred for local recurrence after ≥1 other treatment procedures. The radiation doses ranged from 26 to 89 Gy (median, 56; administered 1.8-2.0 Gy/fraction with average total duration of treatment of 5-7 weeks); 8 patients (10%) received <50 Gy. All patients tolerated treatment well without acute or late complications. All patients except two are alive. Local control was achieved in 65 patients (84%; bone recalcification/restitution of joint functions), 12 patients showed signs of local progression, all within irradiated fields (9 were treated successfully with salvage surgery). Five- and 10-year local PFS were 83% and 73%, respectively. Three patients developed lungs metastases. Malignant transformation of GCTB occurred in two patients.
GCTB can be safely and effectively treated with megavoltage radiotherapy with local control rate >80% at 5 years. Our study confirms that radiotherapy of GCTB offers an alternative to difficult or complex surgery and may be an option of choice in the treatment of inoperable patients.
All seventy-five cases recorded as giant-cell tumor of bone in the Swedish Cancer Registry for the years 1958 through 1968 were analyzed. At reexamination, fifty-three cases constituted genuine giant-cell tumor of bone and twenty cases were so-called "giant-cell variants". The genuine giant-cell tumors showed a significantly higher incidence in the urban than in the rural population. The recurrence rate was 42 per cent. Patients under the age of twenty-five rarely had recurrences. A high recurrence rate was found among patients with tumors located in the distal end of the femur and the proximal end of the tibia. Tumors penetrating through the bone cortex were more aggressive than those located entirely within bone, regardless of tumor size and presence or absence of spontaneous fracture. A malignant course was found in 11.3 per cent of cases, predominantly in patients with tumors in the femur. Histopathological grading was of no prognostic value. Primary en bloc resection with or without prosthetic replacement is recommended in patients over the age of twenty-five.
Giant cell tumors are uncommon primary bone tumors. They primarily occur in the long bones. Giant cell tumors are extremely rare in the skull and head and neck. When it does occur, the maxilla and mandible are the common sites to be involved. We described two cases of giant cell tumor in the temporal bone. In the noncontrast enhanced CT, the lesion presents as a soft tissue density mass with expansion of the bone. The bony cortex is usually intact. The adjacent soft tissues and cerebral parenchyma show no infiltration or edema. The post contrast scan reveals homogenous enhancement of the mass.
For giant cell tumors of bone, does radiotherapy provide a safe and effective treatment? This retrospective review includes 24 patients with 26 histologically diagnosed tumors treated with megavoltage radiotherapy between March 1972 and July 1996. Of the 10 recurrent tumors, five had an intralesional resection, two had a biopsy, and three had no biopsy before radiotherapy. Of the 16 previously untreated tumors, one was irradiated after a marginal resection, five after an intracapsular resection, and 10 after biopsy alone. The total doses ranged from 35 to 55 Gy (median, 43 Gy) in fractions of 1.67 to 2.33 Gy per day. Twenty of 26 tumors (77%) were controlled locally. All of the local recurrences occurred within the irradiated field. Five of six patients with local recurrence were treated successfully with additional surgery. Salvage surgery after local recurrence required amputation of an extremity in three patients and a total knee replacement in one patient. The ultimate local control rate was 96% with one patient alive with progressive disease. Lung metastases in one patient were treated successfully with surgery, chemotherapy, and radiotherapy. In one patient a radiation-induced sarcoma developed 22 years after treatment. The authors conclude that radiation therapy is a safe and effective treatment option for benign giant cell tumors of bone. A total dose greater than 40 Gy is the only variable found to significantly influence local control.
Giant cell tumors of bone are known for their unpredictable behavior characterized occasionally even by metastases. Most metastases lodge in the lungs but other rare sites are regional lymph nodes, mediastinum, skin, scalp and the pelvis. In this case report we document a case of giant cell tumor of the patella in which, associated with local recurrence, there were simultaneous metastases to lymph nodes and lungs.
The giant cell tumour of bone (GCT) is a locally aggressive intraosseous neoplasm of obscure biological behaviour. Although well defined in clinical, radiological and histological terms, detailed information on its biological development is still relatively incomplete. The tumoral tissue consists of three cell types--the neoplastic giant cell tumour stromal cells (GCTSC), representing the proliferative fraction, secondarily recruited mononuclear histiocytic cells (MNHC) and multinuclear giant cells (MNGC). These cellular components interact together with factors that have a role in regulating osteoclast function in normal bone tissue (e.g. RANK, RANKL, OPG, M-CSF). Recent publications suggest that the neoplastic stromal cells express differentiation features of mesenchymal stem cells. Further research of the pathogenesis of GCT as well as the complex interactions of its cellular populations may provide the knowledge necessary for developing approaches for a biological-based therapy of this neoplasm.
Giant-cell tumor: A study of 195 cases
- Dahlin
Derivation of the mammalian skull vault
- Morriss-Kay