Germ cell tumors (GCTs) most often arise in the gonads but some develop extragonadally. The aim of this study was to examine sex- and race-specific trends in incidence and survival of gonadal (GGCTs) and extragonadal GCTs (EGCTs) in the US from 1973 to 2007. We also examined the topographic distribution of EGCTs by race and sex.
We estimated age-specific and age-standardized incidence rates and 5-year relative survival rates (RSR) of GCTs using the Surveillance, Epidemiology, and End Results (SEER) Program (SEER 9 registries). GCTs and their topographic sites were identified using ICD-O morphology and topography codes.
Of 21,170 GCTs among males, 5.7% were extragonadal (whites 5.5%; blacks 16.3%). Of 2,093 GCTs among females, 39.3% were extragonadal (whites, 36.9%; blacks 51.0%). The incidence of GGCT was much higher among white (56.3/1,000,000) than black males (10.0/1,000,000) while there was no difference in incidence between white and black females (3.2/1,000,000). The rates of EGCT among men and women of both races were similar (range:1.9 – 3.4/1,000,000). The most frequent extragonadal sites were mediastinum among males and placenta among females. The 5-year RSR of testicular GCT was higher among whites (97%) than blacks (90%), as was the 5-year RSR of ovarian GCT (whites, 92%; blacks 85%). In general, the 5-year RSRs of EGCTs were lower than the 5-year RSRs of GGCTs.
The different incidence trends of GGCTs and EGCTs and distinct age-specific incidence patterns by anatomic site of EGCTs suggests that GGCTs and EGCTs may have different etiologies.
testicular neoplasms; ovarian neoplasms; incidence; time trends; germ cell tumors; extragonadal germ cell tumors
Intracranial germ cell tumors (GCTs) are relatively rare. Their incidence has been considered to be higher in East Asia than in the United States. This study estimates the incidence of CNS GCTs in Japan and the United States, investigates gender discrepancies in each country, and describes treatment outcomes. Data on primary CNS GCTs from 4 databases were utilized: population-based malignant incidence data from (1) the Japan Cancer Surveillance Research Group (2004–2006; 14 registries), malignant and nonmalignant incidence data from (2) the Surveillance, Epidemiology, and End Results Program (2004–2008; 17 registries), and hospital-based observed survival data from (3) the Brain Tumor Registry of Japan (1984–2000) and (4) the US National Cancer Data Base (1990–2003). Incidence rates per 100 000 for malignant GCTs were not statistically significantly different between Japan (males = 0.143, females = 0.046) and the United States (males = 0.118, females = 0.030). The malignant incidence-rate ratio was higher for pineal GCTs versus nonpineal (ie, the rest of the brain) GCTs in Japan (11.5:1 vs 1.9:1, respectively) and the United States (16.0:1 vs 1.7:1, respectively). In general, 5-year survival estimates were high: over 75% for all GCTs, and over 81% for germinomas, regardless of the type of treatment in either Japan or the United States. The incidence of primary GCTs is similar between Japan and the United States and has the same gender-based patterns by location. High rates of survival were observed in both countries.
brain tumor; epidemiology; germ cell tumors; germinoma; mixed germ cell tumors; pineal gland; teratoma; tumor registry
Giant cell tumor (GCT) is a relatively rare neoplasm. In GCT, the bone affection of the axial skeleton is extremely rare. Most GCT arises in the meta-epiphyseal ends of the long bones. Its peak incidence is between 30 to 40 years of age. GCT is usually classified as benign, but shows locally aggressive behavior and may occasionally undergo a malignant transformation. The patients with GCT in the spine often complain of the lower back pains, as the tumors primarily involve the sacrum. We report a case of an adolescent female complaining of the upper back pain with a sudden weakness of the lower extremities, later diagnosed with the GCT of the T2 vertebra. The present patient showed American Spinal Injury Association Impairment Scale (AIS) D before the surgery, which changed to AIS E after the treatments including the surgery, radiation therapy and rehabilitation.
Giant cell tumor; Spinal cord injuries
Central nervous system (CNS) germ cell tumors (GCT) have not been epidemiologically well described. Our study describes 2 population-based series of nonpineal CNS GCT. Data on all primary (malignant and nonmalignant) CNS (ICD-O-3 sites: C70.0–C72.9, C75.1–C75.3) GCT diagnosed between 2000 and 2004 from the Central Brain Tumor Registry of the United States (CBTRUS) and on all malignant GCT diagnosed between 1992 and 2005 from the Surveillance, Epidemiology, and End Results (SEER) were analyzed. Of 234 nonpineal GCT in CBTRUS, the most common site was brain, NOS (31.6%). Males had a greater frequency (59.7%) than females (40.3%). However, by age group, the male-to-female incidence rate ratio (IRR) differed: children (0–14 years) had an IRR of 1.1, young adults (15–29 years) an IRR of 2.3, and adults (aged 30+) an IRR of 1.0. For children and young adults, most tumors were malignant (86.8% and 89.0%, respectively), whereas for adults, more than half were nonmalignant (56.8%). Germinoma was the most frequent diagnosis (61.5%). In SEER, the frequency of malignant GCT in the CNS (2.5%) was greater than that in the mediastinum (2.1%). Of 408 malignant CNS GCT, 216 (52.9%) were nonpineal. The male-to-female IRR was 1.5. Overall relative survival for nonpineal CNS malignant GCT was 85.3% at 2 years, 77.3% at 5 years, and 67.6% at 10 years. Previous studies of GCT that have not stratified by site have suggested greater gender disparity. Nonpineal CNS GCT show no significant gender preference, yet have outcomes similar to pineal GCT.
brain tumor; epidemiology; germ cell tumors; germinoma; teratoma
Giant cell tumour of bone (GCT) is a relatively rare benign bone tumour more frequent in young people (20–40 years). Histologically, two cell types are represented, stromal cells of osteoblastic origin and a distinctive osteoclast-like population probably of monocytic origin. GCTs can be aggressive and they recur locally in up to 50% of cases; up to 5% of GCTs metastasise to the lungs and spontaneous transformation to a high-grade malignancy occurs in 1–3% of patients. The aetiology of GCT is not known, and no risk factors have been recognised, although familial clustering of both Paget’s disease and GCT has been reported.
GCTs account for approximately 3–5% of primary bone tumours. GCT is rarely multicentric and usually occurs at the epiphyses of long bones, but may also affect other bones.
There are few randomised, prospective clinical trials available to guide clinical management of GCT. Recent developments have led to evaluation of newer therapeutic agents, including biphosphonates and denosumab, with encouraging results. We report the case of a 66-year-old woman affected by GCT. In 1985 the patient, then 41 years old, presented a cystic lesion on her left tibia, which was removed surgically. This lesion relapsed two years later. Therefore the patient was hospitalised and received a diagnosis of “multicentric giant cell bone lesions” (limb-girdle, sternum, mandible, ribs), confirmed by histological examination. These lesions showed hyperactivity on bone scintigraphy. Plain radiographs demonstrated destructive lytic lesions. Blood and urinary examinations showed markedly elevated levels of bone alkaline phosphatase and urine pyridinoline and there was persistent bone pain. In 1993 normocalcaemic primary hyperparathyroidism was diagnosed and an adenoma was removed, with no relapse of the disease. Subsequently the patient started clodronate therapy, i.v., followed by alendronate-neridronate per os and clodronate i.m. for about nine years. Biphosphonate therapy caused a modest and transient decrease in bone indexes. Initial bone lesions were unchanged on computed tomography 25 years after diagnosis, but new bone lesions had appeared. MEN1 gene and CasR analyses were negative.
This is a rare case of a patient affected by multicentric giant cell tumours with a 25-year follow up. A slow progression of the lesion is documented, as well as the absence of significant effect of biphosphonate therapy.
Giant cell tumor (GCT) of bone is a locally destructive tumor that occurs predominantly in long bones of post-pubertal adolescents and young adults, where it occurs in the epiphysis. The majority are treated by aggressive curettage or resection. Vascular invasion outside the boundary of the tumor can be seen. Metastasis, with identical morphology to the primary tumor, occurs in a few percent of cases, usually to the lung. On occasion GCTs of bone undergo frank malignant transformation to undifferentiated sarcomas. Here we report a case of GCT of bone that at the time of recurrence was found to have undergone malignant transformation. Concurrent metastases were found in the lung, but these were non-transformed GCT.
Treatment of giant cell tumor of bone (GCT) often is complicated by local recurrence. Intralesional curettage is the standard of care for primary GCTs. However, there is controversy whether intralesional curettage should be preferred over wide resection in recurrent GCTs.
We investigated the rerecurrence-free survival after surgical treatment of recurrent GCTs to determine the influence of the surgical approach, adjuvant treatment, local tumor presentation, and demographic factors on the risk of further recurrence.
Patients and Methods
We retrospectively reviewed the medical records of 46 patients with recurrent GCTs of long bones treated with wide resection or intralesional curettage and compared these cohorts. Recurrence rates, risk factors for recurrence, and the development of pulmonary metastases were determined. The minimum followup was 37 months (mean, 134 months; range, 37–337 months).
The rate of rerecurrence after wide resection was 6%. Intralesional curettage showed an overall rerecurrence rate of 32%. Implantation of polymethylmethacrylate (PMMA) instead of bone grafting was associated with a lower risk of subsequent recurrence in intralesional procedures (14% versus 50%). Extracompartmental disease did not increase the risk of rerecurrence. Pulmonary metastases occurred in seven patients and appeared independent of the surgical treatment modality chosen.
Intralesional curettage with methylmethacrylate for recurrent GCT provided equivalent tumor control compared with resection in this retrospective study. If joint salvage is possible, we advocate this treatment over resection in recurrent GCTs to preserve the native joint articulation.
Level of Evidence
Level III, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.
Background and purpose
Giant cell tumor (GCT) of the small bones (small-bone GCT) is usually rare and considered somewhat different from conventional GCT. The purpose of this study was to investigate and report the clinicopathological features of 11 cases with small-bone GCT.
Materials and methods
Patient information was obtained with the help of questionnaires. X-rays and paraffin blocks obtained from several institutions were clinically, radiographically, and histologically evaluated.
Small-bone GCT was observed in younger patients compared to conventional GCT; 5 of the 11 (45%) patients were below 20 years of age, whereas the corresponding figure for all GCT patients is 16% in Japan. Excessive cortical bone expansion is a special feature. There were two cases of recurrence and one case of lung metastasis; the primary lesion was in the hand for all three cases. In contrast, no primary lesion of the foot recurred or metastasized. Varying degrees of positive p63 immunostaining were observed in all examined cases (n = 9) of small-bone GCT but were negative in case of giant cell reparative granuloma (GCRG) and solid variant of aneurysmal bone cyst (ABC). One case that demonstrated high-intensity positive staining had two episodes of recurrence.
Small-bone GCT tends to develop in younger patients than does conventional GCT. Primary GCTs of the hand may be biologically more aggressive than those of the feet. The p63 immunostaining may be useful not only for differential diagnosis but also for prognostication of small-bone GCT.
clinicopathological study; giant cell tumor; p63 immunostaining; small bone
Many surgeons treat giant cell tumor of bone (GCT) with intralesional curettage. Wide resection is reserved for extensive bone destruction where joint preservation is impossible or when expendable sites (eg, fibular head) are affected. Adjuvants such as polymethylmethacrylate and phenol have been recommended to reduce the risk of local recurrence after intralesional surgery. However, the best treatment of these tumors and risk factors for recurrence remain controversial.
We evaluated the recurrence-free survival after surgical treatment of GCT to determine the influence of the surgical approach, adjuvant treatment, local tumor presentation, and demographic factors on the risk of recurrence.
We retrospectively reviewed 118 patients treated for benign GCT of bone between 1985 and 2005. Recurrence rates, risk factors for recurrence and the development of pulmonary metastases were determined. The minimum followup was 36 months (mean, 108.4 ± 43.7; range, 36–233 months).
Wide resection had a lower recurrence rate than intralesional surgery (5% versus 25%). Application of polymethylmethacrylate decreased the risk of local recurrence after intralesional surgery compared with bone grafting; phenol application alone had no effect on the risk of recurrence. Pulmonary metastases occurred in 4%; multidisciplinary treatment including wedge resection, chemotherapy, and radiotherapy achieved disease-free survival or stable disease in all of these patients.
We recommend intralesional surgery with polymethylmethacrylate for the majority of primary GCTs. Because pulmonary metastases are rare and aggressive treatment of pulmonary metastases is usually successful, we believe the potential for metastases should not by itself create an indication for wide resection of primary tumors.
Level of Evidence
Level III, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.
The distal end of the radius is one of the common sites of involvement in giant cell tumors (GCTs) with reportedly increased propensity of recurrence. The objective of the present analysis was to study the modalities of management of the different types of distal end radius GCTs so as to minimize the recurrence rates and retain adequate function.
Materials and methods:
Twenty-four patients of distal end radius GCTs treated between January 2000 and December 2004 were retrospectively reviewed. Nineteen cases were available for follow-up with an average follow-up of 37.5 months. There was one Campanacci Grade 1 lesion, nine Grade 2 and 14 Grade 3 lesions. Thirteen (54%) of these patients were treated elsewhere earlier and presented with recurrence. The operative procedures that were performed were: curettage and cementing (five), curettage and bone grafting (seven), excision and proximal fibular arthroplasty (two), excision and wrist arthrodesis (nine) and excision of soft tissue recurrence (one).
Functional status was evaluated using Musculo Skeletal Tumor Society scoring system which averaged 78%. The recurrence rate was 32%. Complications included local recurrence (six), nonunion at the graft bone junction (one), infection (one), deformity (two), stiffness (two), subluxation (two) and bony metastasis (one).
The majority of patients undergoing curettage were either Campanacci Grade 1 or 2. Patients undergoing curettage and reconstruction had a better functional result (82%) as compared to arthrodesis or fibular arthroplasty (69%). Previous intervention did not appear to increase the recurrence rates. Even though complications occur, judicious decision-making and an appropriate treatment plan can ensure a satisfactory outcome in the majority of cases.
Campanacci grade; distal end radius; function; giant cell tumor; recurrence
A giant cell tumour (GCT) is a benign tumour that commonly arises in the distal end of the long bones. Extraosseous GCTs have been reported in a number of organs, but it is rare for a GCT to present in the parotid gland. Therefore, primary GCTs of the parotid gland (GCTPs) are extremely rare. Although GCTPs have been identified as benign soft-tissue tumours, they have a highly malignant potential and poor prognosis. In the present case, we report a 58-year-old male patient presenting with non-tender mass over the left preauricular area for 11 months. The final pathology report revealed a rare case of a GCTP that was treated by parotidectomy and adjuvant radiation therapy. The patient had no recurrence after 2 years of follow-up.
giant cell tumour; parotid gland tumours
Twenty-nine patients with
malignant giant-cell tumours of bone (GCT) were followed- up for
between 6 months and 18 years. Seventeen of the tumours were
primary and 12 were due to malignant degeneration of initially
benign lesions. The clinical features did not differ from those
of benign GCT, except for a higher incidence in the distal tibia
and sacrum. Anaplastic GCTs were included in the study because
their clinical and radiographic features and prognosis were no
different from those of the GCT grade III of Jaffe. Eighteen of
the tumours were grade III, and 11 were anaplastic. This
retrospective study was intended to assess the effects of
chemotherapy and surgery for malignant GCT. Three treatment
groups were selected, in which treatment was either by surgery
alone, surgery plus chemotherapy, or radiotherapy alone. – The
prognosis was poor and the 5 year tumour-free survival rate in
the series was 50%. The prognosis was the same for primary as
for secondarily malignant tumours. There was no statistical
difference in survival between malignant grade III and
anaplastic malignant tumours. The one-year survival rate for
patients treated by chemotherapy and surgery was statistically
test) than for persons treated by surgery alone. However, the
five-year survival rate and the actuarial survival curves were not
statistically different in the two groups (log rank test). –
Chemotherapy appears to be of some value in the treatment of these
malignant tumours but a larger series is required to confirm the
efficacy of this approach.
Giant cell tumor (GCT) is usually a benign but locally aggressive primary bone neoplasm in which monocytic macrophage/osteoclast precursor cells and multinucleated osteoclast-like giant cells infiltrate the tumor. The etiology of GCT is unknown, however the tumor cells of GCT have been reported to produce chemoattractants that can attract osteoclasts and osteoclast precursors. Rarely, GCT can originate at extraosseous sites. More rarely, GCT may exhibit a much more aggressive phenotype. The role of chemotherapy in metastatic GCT is not well defined.
We report a case of an aggressive GCT of the uterus with rapidly growing lung metastases, and its response to chemotherapy with pegylated-liposomal doxorubicin, ifosfamide, and bevacizumab, along with a review of the literature.
Aggressive metastasizing GCT may arise in the uterus, and may respond to combination chemotherapy.
Giant cell tumor (GCT) of bone is a benign but locally aggressive and destructive lesion generally occurring in skeletally mature individuals. Typically involving the epiphysiometaphyseal region of long bones, the most common sites include the distal femur, proximal tibia and distal radius. On radiographs, GCT demonstrates a lytic lesion centered in the epiphysis but involving the metaphysis and extending at least in part to the adjacent articular cortex. Most are eccentric, but become symmetric and centrally located with growth. Most cases show circumscribed borders or so-called geographical destruction with no periosteal reaction unless a pathological fracture is present. There is no mineralized tumor matrix. Giant cell tumor can produce wide-ranging appearances depending on site, complications such as hemorrhage or pathological fracture and after surgical intervention. This review demonstrates a spectrum of these features and describes the imaging characteristics of GCT in conventional radiographs, computerized tomography scans, magnetic resonance imaging, bone scans, positron emission tomography scans and angiography.
Giant cell tumor; imaging; magnetic resonance imaging
Patient: Male, 30
Final Diagnosis: Giant cell bone tumor
Symptoms: Bone swelling • pain
Clinical Procedure: —
Unusual clinical course
Cooper first reported giant cell tumors (GCT) in the 18th century. The clavicle is a rare site for tumors. Metastatic tumors are more common than benign. This is the first case of GCT lateral end of clavicle to be reported in the literature.
A 30-year-man was admitted with a 1-year history of progressively increasing swelling and pain over the left lateral end of the clavicle.
The plain radiograph and PET scan revealed an expansile radiolucent lesion in the lateral end of the clavicle. Swelling was epiphsio-metaphyseal in location. It demonstrated geographical type of destruction with a narrow zone of transition. There was no periosteal reaction or soft-tissue component. The mitotic activity was found to be 0–1/10 HPF. Diagnosis was confirmed histopathologically. A wide excision of the mass, including 3 cm of healthy tissue of the clavicle, was performed.
The presence of an expansile lytic lesion of the lateral end of the clavicle should be taken seriously and complete radiological and histopathological investigation should be done and giant cell tumor of the bone should be kept in mind despite its rarity.
giant cell tumour; expansile lytic lesion; clavicle; swelling
Giant cell tumor of bone (GCT) is a destructive neoplasm of uncertain etiology that affects the epiphyseal ends of long bones in young adults. GCT stromal cells (GCTSCs) are the primary neoplastic cells of this tumor and are the only proliferating cell component in long-term culture, which recruits osteoclast-like giant cells that eventually mediate bone destruction. The oncogenesis of GCT and factors driving the neoplastic stromal cells to proliferate extensively and pause at an early differentiation stage of pre-osteoblast lineage remain unknown. Overexpression of p63 was observed in GCTSCs and there is growing evidence that p63 is involved in oncogenesis through different mechanisms. This study aimed to understand the specific role of p63 in cell proliferation and oncogenesis of GCTSCs. We confirmed p63 expression in the mononuclear cells in GCT by immunohistochemical staining. By real-time PCR analysis, we showed a higher level (>15-fold) of TAp63 expression in GCTSCs compared to that in mesenchymal stem cells. Furthermore, we observed that knockdown of the p63 gene by siRNA transfection greatly reduced cell proliferation and induced cell cycle arrest at S phase in GCTSCs. We found that the mRNA expression of CDC2 and CDC25C was substantially suppressed by p63 knockdown at 24–72 h. Moreover, p63 was found to be recruited on the regulatory regions of CDC2 and CDC25C, which contain p53-responsive elements. In summary, our data suggest that p63 regulates GCTSC proliferation by binding to the CDC2 and CDC25C p53-REs, which may inhibit the p53 tumor suppressor activity and contribute to GCT tumorigenesis.
giant cell tumor of bone; p63; CDC2; CDC25C; cell proliferation; siRNA
The characteristic bone destruction in giant cell tumour of bone (GCT) is largely attributed to the osteoclast-like giant cells. However, experimental analyses of bone resorption by cells from GCT often fail to exclude the neoplastic spindle-like stromal cells, and several studies have demonstrated that bone resorption by GCT cells is increased in the presence of stromal cells. The spindle-like stromal cells from GCT may therefore actively contribute to the bone resorption observed in the tumour. Type I collagen, a major organic constituent of bone, is effectively degraded by three matrix metalloproteinases (MMPs) known as the collagenases: MMP-1, MMP-8 and MMP-13. We established primary cell cultures from nine patients with GCT and the stromal cell populations were isolated in culture. The production of collagenases by primary cultures of GCT stromal cells was determined through real-time PCR, western blot analysis and a multiplex assay system. Results show that the cells produce MMP-1 and MMP-13 but not MMP-8. Immunohistochemistry confirmed the presence of MMP-1 and MMP-13 in paraffin-embedded GCT tissue samples. Medium conditioned by the stromal cell cultures was capable of proteolytic activity as determined by MMP-1 and MMP-13-specific standardized enzyme activity assays. The spindle-like stromal cells from GCT may therefore actively participate in the bone destruction that is characteristic of the tumour.
PMID: 19442604 CAMSID: cams200
Giant cell tumour; Stromal cells; Collagen; Osteolysis; Matrix metalloproteinases
Giant Cell Tumor of Bone (GCT) is an aggressive skeletal tumor characterized by local bone destruction, high recurrence rates and metastatic potential. Previous work in our lab has shown that the neoplastic cell of GCT is a proliferating pre-osteoblastic stromal cell in which the transcription factor Runx2 plays a role in regulating protein expression. One of the proteins expressed by these cells is parathryroid hormone-related protein (PTHrP). The objectives of this study were to determine the role played by PTHrP in GCT of bone with a focus on cell proliferation and apoptosis. Primary stromal cell cultures from 5 patients with GCT of bone and one lung metastsis were used for cell-based experiments. Control cell lines included a renal cell carcinoma (RCC) cell line and a human fetal osteoblast cell line. Cells were exposed to optimized concentrations of a PTHrP neutralizing antibody and were analyzed with the use of cell proliferation and apoptosis assays including mitochondrial dehydrogenase assays, crystal violet assays, APO-1 ELISAs, caspase activity assays, flow cytometry and immunofluorescent immunohistochemistry. Neutralization of PTHrP in the cell environment inhibited cell proliferation in a consistent manner and induced apoptosis in the GCT stromal cells, with the exception of those obtained from a lung metastasis. Cell cycle progression was not significantly affected by PTHrP neutralization. These findings indicate that PTHrP plays an autocrine/paracrine neoplastic role in GCT by allowing the proliferating stromal cells to evade apoptosis, possibly through non-traditional caspase-independent pathways. Thus PTHrP neutralizing immunotherapy is an intriguing potential therapeutic strategy for this tumor.
Objective: To discuss the current management options for giant-cell tumors (GCTs) involving the temporal bone and present two case reports and a review of the literature. Method: In a tertiary-care academic medical center, two patients with GCTs of the temporal bone were evaluated and managed. The patients underwent gross total resection and curettage of GCTs involving the temporal bone. Afterward, both patients were evaluated for postoperative complications as well as for recurrence. Results: Two patients underwent operative excision using curettage. Clinical and radiographic studies demonstrated no evidence of recurrence with 3 years of follow-up in one patient and 10 years of follow-up in the second patient. Conclusion: Based on these results, we concluded that gross total removal and curettage of GCTs in the temporal bone is a viable treatment option. This finding is contrary to previous studies.
Temporal bone; giant-cell tumors; skull base
Giant cell tumour (GCT) or osteoclastoma is a benign locally aggressive tumour with a tendency for local recurrence. 85–90% of cases occur in long bones; the sites most commonly affected being lower end of femur, upper end of tibia, lower end of radius and proximal humerus in descending order of frequency. Only 2% of GCT occurs in hand. GCT of bone accounts for 5% of all primary bone tumour. 80% of patients are above the age of 18 years, and it occurs commonly in adults between ages of 20 and 40 years. The authors report a case of GCT of first metacarpal which is very rare site for such tumour and only few cases reported in literature so far.
Giant cell tumor (GCT) is classified as a benign bone tumor, and it is frequently identified at the epiphysis of long bones and relatively rare in the temporal bone. For orthopedists expert at recognizing bone and soft tissue tumors, the diagnosis of GCT is relatively easy; however, since head and neck surgeons experience few cases of GCT, it may be difficult to diagnose when it occurs in the temporal bone. A 32-year-old man complained of left hearing loss, aural fullness, and tinnitus. Examination of the ear revealed a bulging tumor. Audiologic examination demonstrated conductive hearing loss of the left ear. Computer tomograph of the temporal bone showed a soft-tissue-density specification indicating bone destruction at the left temporal bone. The tumor invaded the skull base. Imaging examinations using magnetic resonance imaging revealed a nonhomogenous isosignal intensity area on T1 at the left temporal bone. After intravenous gadolinium, the mass showed unequal enhancement. This patient subsequently underwent surgery to remove the lesion using transmastoid and middle fossa approach. Pathological examinations from specimens of the tumor revealed characteristic of GCT. No clinical or radiological evidence of tumor recurrence was detected for 4 years.
Background. We report on 19 cases of giant cell tumor of bone (GCT) affecting the spine or sacrum and evaluate the outcome of different treatment modalities. Methods. Nineteen patients with GCT of the spine (n = 6) or sacrum (n = 13) have been included in this study. The mean followup was 51.6 months. Ten sacral GCT were treated by intralesional procedures of which 4 also received embolization, and 3 with irradiation only. All spinal GCT were surgically treated. Results. Two (15.4%) patients with sacral and 4 (66.7%) with spinal tumors had a local recurrence, two of the letter developed pulmonary metastases. One local recurrence of the spine was successfully treated by serial arterial embolization, a procedure previously described only for sacral tumors. At last followup, 9 patients had no evidence of disease, 8 had stable disease, 1 had progressive disease, 1 died due to disease. Six patients had neurological deficits. Conclusions. GCT of the axial skeleton have a high local recurrence rate. Neurological deficits are common. En-bloc spondylectomy combined with embolization is the treatment of choice. In case of inoperability, serial arterial embolization seems to be an alternative not only for sacral but also for spinal tumors.
Giant cell tumor (GCT) of bone though one of the commonest bone tumors encountered by an orthopedic surgeon continues to intrigue treating surgeons. Usually benign, they are locally aggressive and may occasionally undergo malignant transformation. The surgeon needs to strike a balance during treatment between reducing the incidence of local recurrence while preserving maximal function.
Differing opinions pertaining to the use of adjuvants for extension of curettage, the relative role of bone graft or cement to pack the defect and the management of recurrent lesions are some of the issues that offer topics for eternal debate.
Current literature suggests that intralesional curettage strikes the best balance between controlling disease and preserving optimum function in the majority of the cases though there may be occasions where the extent of the disease mandates resection to ensure adequate disease clearance.
An accompanying treatment algorithm helps outline the management strategy in GCT.
Curettage; giant cell tumor; treatment
Giant cell tumor of bone (GCT) is a destructive and potentially metastatic bone tumour in which the characteristic giant cells have classically been considered the culprits in bone destruction. However, the neoplastic element of the tumour consists of propagative osteoblast-like stromal cells that may play a role in bone resorption. The objectives of this study were to determine the expression and activity of the gelatinases, matrix metalloproteinase (MMP)-2 and -9, in GCT stromal cells, and to determine if these cells have bone-resorbing capabilities. We performed immunohistochemistry on clinical specimens, and real-time polymerase chain reaction (PCR) and zymography on cell lysates and conditioned media from cultured clinical GCT specimens in order to evaluate the expression and activity of MMP-2 and-9 in GCT stromal cells. Our results support the fact that GCT stromal cells express MMP-2 and MMP-9 and are capable of gelatin degradation in vitro. These cells may therefore play a role in bone destruction in GCT.
PMID: 22287999 CAMSID: cams215
The majority of giant cell tumors (GCTs) occur in the ends of the long bones. The presence of more than one GCT in the axial skeleton is rare. A GCT is capable of remaining clinically latent following treatment and becoming active a number of years later. We report an extremely rare case of GCT occurring in the axial skeleton, involving the sacrum, thoracic spine and parieto-occipital skull in more than 15 years of follow-up.
giant cell tumor; sacrum; spine; skull