The exploration of surgical outcomes in patients with giant cell tumor of the tendon sheath in spine: An epidemiological, radiological, and follow-up investigation.

PURPOSE: This study aimed to characterize giant cell tumor of the tendon sheath (GCTTS) in spine more fully and further validate the therapeutical effect of surgcial resection for treating this neoplasm. METHODS: Patients diagnosed with spinal GCTTS and received surgical resection in our hospital between January 2009 and September 2021 were identified retrospectively. The clinical data and radiological images were summarized and the clinical outcomes of patients with a follow-up period of more than 12 months were analyzed. RESULTS: Thirty patients with benign GCTTS and one with malignant GCTTS were included. Preoperative radiological images were available in 28 of 30 benign cases. Benign lesions were revealed as soft tissue masses centering on the facet joint with osteolytic bone destruction in 26 patients on CT, and as prevertebral or intramuscular masses without bone erosion in 2. MRI showed the signal of isointensity or hypointensity on T1 weighted images (T1WI) in 25 patients and slightly hyperintense in three. On T2 weighted images (T2WI), 17 lesions displayed homogeneous hypointense signal, and eight lesions possessed heterogeneous signals. The remaining three lesions featured slightly hyperintense signal on T2WI. Follow-up data were available in 23 of 30 benign cases treated with gross-total resection, and two patients experienced recurrence. CONCLUSIONS: Spinal GCTTS should be suspected in cases with features such as the mass mainly involving the posterior bone elements, the lack of intralesional calcification, T2-weighted dark signals, and free of any cancer. Gross-total resection is an effective means for treating spinal GCTTS.

Índice de recurrencia de tumor de células gigantes con curetaje y cementación / Recurrence rate of giant-cell tumor treated by curettage and cementation

OBJETIVO Identificar la tasa de recurrencia de tumor de células gigantes (TCG) en pacientes tratados con curetaje y cementación con seguimiento mínimo de tres años. MATERIALES Y METODOS Cohorte retrospectiva y observacional de pacientes con diagnóstico de TCG en estadios 1 y 2 de Enneking tratados con curetaje intralesional y cementación entre 1981 y 2011. Se registraron edad, sexo, región anatómica, y tiempo de recurrencia. Se utilizó estadística descriptiva con medidas de tendencia central y medidas de dispersión (desviación estándar) para variables cuantitativas, y porcentajes para variables cualitativas. RESULTADOS Entre 1981 y 2011, se identificaron 375 casos de TCG, de los cuales 141 (37,6%) fueron tratados con este método. El seguimiento fue de 48 a 240 meses, y la edad, de 27 9 años; 45% de los pacientes eran mujeres, y 55%, varones, con una relación mujer:hombre de 1,2:1. El hueso más afectado fue la tibia (38%), seguida del fémur (32%), del húmero (16%), y del radio (10%). En 88,6%, la resección fue curetaje intralesional, y el resto, marginal. Hubo 15,7% de casos de TCG con fractura, y recidiva en 12,7%. DISCUSIÓN Se ha demostrado que este método de tratamiento reduce el riesgo de recurrencia por los efectos adyuvantes locales de la cementación acrílica. La recurrencia ocurre en los dos primeros años de seguimiento. Sin embargo, hay autores que respaldan que el margen quirúrgico es el único factor que influencia el riesgo de recurrencia local. La extensión extraósea del TCG no es contraindicación para curetaje intralesional y adyuvante con metilmetacrilato. CONCLUSIONES Reportamos tasa una de recurrencia similar a la de la literatura, siendo un recurso factible de reconstrucción de miembros

OBJETIVE To identify the recurrence rate of giant-cell tumor (GCT) in patients treated by curetage and adjuvant therapy with polymethil metacrylate with a minimum followup of 3 years. MATERIALS AND METHODS Observational and retrospective cohort with patients with diagnoses of Enneking stages 1 and 2 GCT treated through intralesional curetage and cementation between 1981 and 2011. Age, gender, anatomic location and relapse period were recorded. The data was analyzed with measures of central tendency and dispersion (standard deviation) for the quantitative variables, percentages for the qualitative variables. RESULTS Between 1981 and 2011 375 cases of GCT were identified, 141 (36.7%) of which were treated by this method. The follow-up ranged from 48 to 240 months, the age was of 27 9 years, 45% of the patients were female, and 55%, male, with a female: male ratio of about 1.2:1. The tibia was the most frequent affected bone (38%), followed by the femur (32%), the humerus (16%), and the radius (10%). The resection thecnique was intralesional curetage in about 88.6% of the cases, and marginal resection in the remaining cases. Pathologic fracture was present in approximately 15.7%, and recurrence occurred in approximately 12.7%. DISCUSSION We demonstrated that this treatment method decreases the risk of recurrence due to the local adjuvant effects of acrylic cementation. Recurrence events occur in the first two years after resection. However, some authors defend that the surgical margin is the only factor that influences the risk of local recurrence. The extraosseus extension of GCT is not a contraindication to perform intralesional curetage or to prescribe the adjuvant treatment with polymethyl metacrylate. CONCLUSIONS We reported a recurrence rate similar to that of the literature, and this is a feasible resource for limb reconstruction

Circulating Candidate Biomarkers in Giant Cell Tumors of Bone.

PURPOSE: Approximately 5% of giant cell tumors (GCT) of bone develop pulmonary metastases. Although many biomarkers have been proposed, identification of circulating low abundance molecules may be useful to predict malignant progression. EXPERIMENTAL DESIGN: The hydrogel nanoparticle technique followed by MS was used to detect low molecular weight serum proteins or protein fragments in serum of 20 GCT patients with different clinical course and in ten healthy sera used as control. The most representative low-abundant de novo or differentially abundant proteins were submitted to String database that recognized interconnected activated pathways including protein activation cascade, wound healing, cell-substrate adhesion, and response to stress. Statistics were performed for identification of candidate prognostic factors. RESULTS: Proteome cluster analysis separated metastasis-free from metastatic GCT patients in two well-defined groups where serum levels of signaling transduction mediators and regulators of kinase activity presented a high discriminatory power. Increased expression of proteins STAT5B, GRB2, and OXSR1 was related to a higher probability of metastasis. Multivariate analysis demonstrated that tumor grade and STAT5B were independent prognostic factors. CONCLUSIONS AND CLINICAL RELEVANCE: By using a noninvasive technique, we identified differentially abundant serum candidate biomarkers, also providing prognostic information in patients with GCT of bone.

En Bloc Resection Versus Intralesional Surgery in the Treatment of Giant Cell Tumor of the Spine.

STUDY DESIGN: Multicenter, ambispective observational study. OBJECTIVE: The aim of this study was to quantify local recurrence (LR) and mortality rates after surgical treatment of spinal giant cell tumor and to determine whether en bloc resection with wide/marginal margins is associated with improved prognosis compared to an intralesional procedure. SUMMARY OF BACKGROUND DATA: Giant cell tumor (GCT) of the spine is a rare primary bone tumor known for its local aggressiveness. Optimal surgical treatment remains to be determined. METHODS: The AOSpine Knowledge Forum Tumor developed a comprehensive multicenter database including demographics, presentation, diagnosis, treatment, mortality, and recurrence rate data for GCT of the spine. Patients were analyzed based on surgical margins, including Enneking appropriateness. RESULTS: Between 1991 and 2011, 82 patients underwent surgery for spinal GCT. According to the Enneking classification, 59 (74%) tumors were classified as S3-aggressive and 21 (26%) as S2-active. The surgical margins were wide/marginal in 27 (36%) patients and intralesional in 48 (64%) patients. Thirty-nine of 77 (51%) underwent Enneking appropriate (EA) treatment and 38 (49%) underwent Enneking inappropriate (EI) treatment. Eighteen (22%) patients experienced LR. LR occurred in 11 (29%) EI-treated patients and six (15%) EA-treated patients (P = 0.151). There was a significant difference between wide/marginal margins and intralesional margins for LR (P = 0.029). Seven (9%) patients died. LR is strongly associated with death (Relative Risk 8.9, P < 0.001). Six (16%) EI-treated patients and one (3%) EA-treated patients died (P = 0.056). With regards to surgical margins, all patients who died underwent intralesional resection (P = 0.096). CONCLUSION: En bloc resection with wide/marginal margins should be performed when technically feasible because it is associated with decreased LR. Intralesional resection is associated with increased LR, and mortality correlates with LR. LEVEL OF EVIDENCE: 3.

Incidence and anatomical distribution of hand tumours: a Singapore study.

INTRODUCTION: Hand tumours are frequently encountered in clinical practice. A list of differential diagnoses of the most common hand tumours based on anatomical location would be helpful for clinicians. We aimed to determine the anatomical distribution of hand tumours seen at a hand surgery practice in Singapore. METHODS: The medical records of 50 men and 65 women (mean age 41.7 [range 17-74] years) who underwent excision of hand tumours between 1 June 2010 and 31 December 2012 were reviewed. The histological diagnoses and anatomical locations of the tumours were analysed. The locations were divided into three main groups: (a) distal to the metacarpophalangeal joints (MCPJs); (b) between the MCPJs and carpometacarpal joints (CMCJs); and (c) between the CMCJs and the radiocarpal joint (RCJ). RESULTS: Overall, the most common tumours excised from the hand were ganglions (n = 66/116, 56.9%) and giant cell tumours of the tendon sheath (GCTTSs; n = 11/116, 9.5%). However, distal to the MCPJs, GCTTSs (n = 11/39, 28.2%) were more common than ganglions (n = 7/39, 17.9%). Most of the ganglions (n = 59/66, 89.4%) arose from between the CMCJs and RCJ. CONCLUSION: Most hand tumours were benign. Ganglions were the most common tumours between the CMCJs and RCJ, while GCTTSs were the most common tumours distal to the MCPJs.

Treatment options and prognosis for repeatedly recurrent giant cell tumor of the spine.

BACKGROUND: Giant cell tumor of the spine has high rate of postoperative recurrence. There are not much published studies on repeatedly recurrent GCTS (RRGCTS). Also, there are controversies as to the prognostic factors and treatment options for RRGCTS. METHODS AND MATERIALS: A retrospective survival analysis between 2000 and September 2014 was performed on the 74 times of in-hospital treatment of the 21 patients. Recurrence-free survival was defined as the time between the date of surgery and the date of recurrence. Factors with P values <0.05 in univariate analysis were subjected to multivariate analysis by means of proportional hazard analysis. RESULT: A total of 21 patients comprising ten males and 11 females with a mean age of 29.7 years (range 15-49) were included, with a total of 74 times of surgery performed. Eighteen patients had no evidence of disease. Univariate and multivariate analysis suggested that total spondylectomy and bisphosphonate therapy were independent prognostic factors for better RFS in RRGCTS patients. CONCLUSION: Patients of RRGCTS are suitable for further surgical treatment with relatively good healing process and restoration of nervous function. Early diagnosis of recurrence may be associated with better prognosis. Total spondylectomy in combination with bisphosphonate therapy could reduce postoperative recurrence rate.

Giant-cell tumor: analysis on the importance of early diagnosis and the epidemiological profile / Tumor de células gigantes: análise sobre importância do diagnóstico precoce e perfil epidemiológico

This study aimed to ascertain the relationship between early diagnosis of giant-cell tumors (GCT) and their prognosis, by correlating the time of symptom onset with the staging of the injury (through the Campanacci classification at the time of diagnosis), and with the type of treatment. The secondary objective of the study was to outline the epidemiological profile of patients with GCT in the region where the data were gathered, and to compare them with data in the literature. METHODS: The authors present an evaluation on 61 patients diagnosed with bone GCT, with regard to the site of involvement, age, initial symptoms, time of symptom onset, classification and type of treatment, among patients attended between May 1994 and August 2009. RESULTS: The threshold indicated as the limit for Campanacci stage I tumors to be the commonest diagnosis, with a 98.2% chance that the treatment would be non-aggressive, was 2 months after symptom onset. This finding was statistically significant (p = 0.017). Every additional month increased the chance that a patient would be diagnosed with an advanced-stage tumor by 10.94%, in relation to the chances of having the other two stages of the tumor. CONCLUSION: The study result not only suggests that the alternative hypothesis that the earlier the diagnosis of GCT is, the less severe the lesion will be, has been confirmed; but also especially predicts the relationship between the time of symptom appearance and the severity of the tumor.

Presumir a relação entre o diagnóstico precoce do tumor de células gigantes (TCG) e o seu prognóstico, relacionar o tempo de surgimento dos sintomas com o estadiamento da lesão, por meio da classificação de Campanacci no momento do diagnóstico, e com tipo de tratamento. O objetivo secundário do estudo é traçar o perfil epidemiológico dos pacientes com TCG da região onde foram colhidos os dados e compará-lo com dados da literatura. MÉTODOS: Avaliação de 61 pacientes diagnosticados com tumor de células gigantes ósseo quanto ao local de acometimento, idade, sintomatologia inicial, tempo do surgimento dos sintomas, classificação e tipo de tratamento em pacientes atendidos entre maio de 1994 e agosto de 2009. RESULTADO: Aponta o marco de dois meses após o início da sintomatologia como data limite, quando seria mais comum o diagnóstico de tumor estágio I de Campanacci e com 98,2% de chance de ser tratado de modo não agressivo, dados com relevância estatística (p = 0,017). A cada aumento de um mês a chance de um paciente ser diagnosticado com tumor em estágio avançado é 10,94% maior do que em relação aos outros dois estágios do tumor. CONCLUSÃO: O resultado do estudo sugere não somente a confirmação da hipótese opcional de que quanto mais precoce o diagnóstico de TCG, menos grave é a lesão, mas, principalmente, prediz a relação do tempo de surgimento do sintoma com a gravidade do tumor.

Clinical characteristics and evolution of giant cell tumor occurring in Paget's disease of bone.

Patients with Paget's bone disease (PDB) have an increased risk of developing giant cell tumor (GCT). This study was performed to evaluate the clinical characteristics and evolution of GCT complicating PDB and to compare these clinical characteristics to those observed in two large PDB cohorts, the PDB Italian Registry and the United Kingdom's Multi-Centre Randomised Controlled Trial of Symptomatic Versus Intensive Bisphosphonate Therapy for Paget's Disease (PRISM) study. A systematic literature review identified 117 cases of PDB complicated by GCT (PDB-GCT), which involved the skeletal sites affected by PDB (110 patients) or the extraskeletal tissues adjacent to affected bones (7 patients). In contrast to what previously reported for GCT patients without GCT patients (83.2%) were white and one-fourth of them (24.8%) had multifocal GCTs. Compared to PDB patients without GCT, PDB-GCT patients showed a higher male/female ratio (2.1 versus 1.2) and more severe disease (age at PDB onset 52.1 ± 12.1 versus 63.3 ± 10.6 years; number of affected sites 6.1 ± 2.9 versus 2.34 ± 1.6; prevalence of polyostotic PDB 93.3% versus 60.6%). The mortality rate of PDB-GCT patients was higher than those occurring in GCT patients without PDB (about 50% versus 0% to 5% at 5 years) or in PDB patients without GCT (log rank = 29.002). Moreover, up to 98% of PDB-GCT cases had elevated total alkaline phosphatase levels at neoplasm diagnosis, suggestive of active PDB. Importantly, PDB-GCT patients from Southern Italy (45.6% of all GCT patients) showed a higher prevalence of multifocal GCT (51.7%) and of positive familial history for PDB (70.8%) and GCT (65.0%). Finally, indirect evidence suggests a decline in the incidence of GCT in PDB patients. The occurrence of GCT in PDB patients is associated with severe disease and reduced life expectancy of affected patients. The increased prevalence of familial diseases in PDB-GCT patients from Southern Italy suggests a founder effect. The observed changes over time in the incidence of GCT in PDB patients could be related to improved clinical management and/or living conditions of patients.

Natural history and CT scan follow-up of subependymal giant cell tumors in tuberous sclerosis complex patients.

This study investigated whether subependymal giant cell tumors (SGCT) grow after the second decade in patients with tuberous sclerosis complex (TSC). In this retrospective longitudinal study all 214 TSC patients who had previously been in a single center cross-sectional study in 2007 were included. Institutional Review Board approval was obtained. In February 2012, the most recent contrast-enhanced CT scan of the brain since 2007 was reviewed for radiological signs of SGCT and, if present, the largest diameter was measured. The findings of the CT scans before 2007 were compared with the current findings. Of the 43 patients with radiological signs of SGCT in 2007 a follow-up CT scan was available for 34. Ten (29%) of these patients showed an increase in size of the SGCT. These 10 patients were on average 36 years old (median 34 years; range 26-50 years) and the average size of the SGCT was 17 mm (median 16 mm; range 11-29 mm), which corresponded to an average size increase of 5mm (median 4mm; range 2-8mm) after an average interval of 5 years (range 2-8 years). Of the 171 patients without radiological signs of SGCT in 2007 a follow-up CT scan was available for 138. Three (2%) of these patients showed radiological signs of SGCT on follow-up. These patients were 19, 23, and 41 years old and the SGCT was on average 13 mm (median 17 mm; range 4-19 mm). To conclude, in our cohort, CT scan demonstrated both growth of SGCT and development of new SGCT after the second decade of life in TSC patients.

Nodular giant cell tumour of the tendon sheath of the hand: analysis of eighty-four cases: diagnostic decisions and outcome.

INTRODUCTION: The giant cell tumour of the tendon sheath (GCTTS) of the hand is a benign tumour of unknown origin. The clinical diagnosis is supported by preoperative imaging. But the ideal imaging methods necessary for the diagnosis, preoperative planning and total tumour resection are still debated. Standard treatment is surgical resection with histological confirmation. METHODS: We followed up 84 patients who were operated upon for a histologically confirmed nodular type GCTTS for an average of 4.7 years (range four to eight). The preoperative symptoms and radiological findings of X-ray, ultrasound and MRI were reviewed and the surgeon asked for their impact on the surgical procedure. RESULTS: The average age at operation was 50.9 years, 65.5% of the patients were female and 61.9% of the lesions were located on the palmar aspect. Most tumours were found on the first three fingers. Two patients had tumours at two separate sites (2.4%). After an average follow up of 31.5 months two recurrences were observed (2.4%). In addition to X-ray and ultrasound, the preoperative findings of the MRI had no influence on the surgical procedure. CONCLUSION: Our data on the nodular GCTTS are in accordance with published data concerning the age distribution, gender distribution, and localisation. No soft-tissue imaging method is superior for the diagnosis of nodular GCTTS or for the preoperative planning. A preoperative MRI may not be necessary as clinical and ultrasound examination are sufficient. To exclude bony erosions, a preoperative X-ray is necessary.

Subependymal nodules and giant cell tumours in tuberous sclerosis complex patients: prevalence on MRI in relation to gene mutation.

PURPOSE: The purpose of this study was to estimate the association among the presence of subependymal nodules (SENs), subependymal giant cell tumours (SGCTs) and gene mutation in tuberous sclerosis complex (TSC) patients. METHODS: Clinical records and images of 81 TSC patients were retrospectively reviewed by two neuroradiologists in consensus. All patients were assessed for gene mutations and were categorized as TSC1 or TSC2 mutation carriers, or no-mutations-identified (NMI) patients. They underwent a brain magnetic resonance imaging (MRI) using 0.1 mmol/kg of gadobutrol. Any enhancing SEN ≥ 1 cm and placed near the foramen of Monro was considered SGCT. Two MRI follow-up exams for each patient with SGCT were evaluated to assess tumour growth using Wilcoxon and chi-squared tests. RESULTS: Of 81 patients, 44 (54%) were TSC2 mutation carriers, 20 (25%) TSC1 and 17 (21%) NMI. Nine (11%) had a unilateral and three (4%) a bilateral SGCT. Fifty of 81 patients (62%) showed at least one SEN. None of the 31 patients without SEN showed SGCTs, whilst 12 (24%) of the 50 patients with at least one SEN showed SGCTs (p = 0.003). The association between the presence of SGCT or SEN and gene mutation was not significant (p = 0.251 and p = 0.187, respectively). At follow-up, the median SGCT diameter increased from 14 to 15 mm (p = 0.017), whilst the median SGCT volume increased from 589 to 791 mm(3) (p = 0.006). CONCLUSIONS: TSC patients with SENs are more likely to present with SGCT than those without SENs, in particular for TSC2 mutation carriers. The SGCT growth rate may be missed if based on the diameter instead of on the volume.

Radiation therapy for infiltrative giant cell tumor of the tendon sheath.

PURPOSE: Giant cell tumor of the tendon sheath (GCTTS) has a high recurrence after excision and can be a management challenge. Although experience with radiation therapy for GCTTS is limited, it is purported to control infiltrative cases and prevent recurrence. We describe our approach to primary and recurrent GCTTS, as well as our identification of infiltrative cases and their treatment with radiation therapy. METHODS: We reviewed 58 patients (32 men and 26 women) with GCTTS in the hand excised at 1 center between 1998 and 2009. Mean age at the time of excision at our center was 50 years. A total of 14 patients who had undergone primary excision at other centers were referred to our center with recurrent disease. Intraoperatively, we found infiltrative disease in 4 patients undergoing primary excision at our center and in 10 of the 14 patients referred to our institution with recurrent disease. All infiltrative (4 primary and 10 recurrent) cases were referred for radiation therapy. RESULTS: Of 14 patients with infiltrative tumors, 10 received radiation therapy (3 patients declined and 1 had a major comorbidity that precluded therapy). Radiation dose was either 35 Gy in 14 fractions or 48 Gy in 24 fractions. At 3.1 years' follow-up, none of the 10 patients treated with radiation therapy had recurrence. No long-term complications were associated with radiation therapy, and hand function was not adversely affected. We identified 4 recurrences, 2 of which were in patients with primary tumors without infiltrative features. Of the 4 patients who were referred for radiation but did not receive it, 2 patients developed recurrence by 2 years after referral. CONCLUSIONS: In cases of infiltrative GCTTS, radiation therapy may provide local tumor control with preservation of hand function. Radiation therapy may be particularly helpful when further surgery is not a good option. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

[Characteristics of pain in oncologic disease of the knee]. / Caracterización del dolor de la rodilla con patología oncológica.

BACKGROUND: 10% of > 55-year-old adults suffer some kind of non-neoplastic knee pain and 75% of the musculoskeletal neoplastic disease develops in the knee. OBJECTIVE: to identify clinical characteristics of knee pain in neoplastic knee pathology. METHODS: after appropriate authorization of the Local Committee of Investigation and under informed consent, we made a crosssectional and a risk analysis study. We included 160 patients who were seeking medical help due to knee pain. They answered a standardized questionnaire relating to the characteristics of their pain symptomatology. Data were integrated into two groups with knee pain; a) neoplasic (cases, n = 65), b) non-neoplasic (controls, n = 95) and analyzed (SPSS v.15™). We used homogeneity tests between groups (p > 0.05); inferential analysis (Student t test, χ(2)) and risk assessment (OR), p ≤ 0.05, (CI 95%), Statistical power was > 0.80. RESULTS: female gender predominated (55%); age was 40.3 ± 19.6 years. The most prevalent diagnoses were knee osteoarthritis 37% for non-neoplasic group; giant cell tumor 10% for benign neoplasic group and osteosarcoma 6.1% for neoplasic malignant group. Knee pain lasting < 4 months (OR 7.6; CI 95% 3.48-16.5) and severe intensity (OR 5.7; CI 95% 2.82-11.64), constant pain (OR 2.9; CI 95% 1.37-6.36), rapidly progressive fluctuation (OR 31; CI 95% 7.01-137) and nocturnal predominance (OR 7.72; CI 95% 3.2-18.5) were characteristics of neoplasic knee pain. CONCLUSIONS: the neoplasic knee pain was characterized for a rapid onset, severe and constant pain, progressive, fluctuation and predominantly by night-time.

Giant cell tumour of the flexor tendon sheath of the hand: analysis of 27 cases.

Giant cell tumour of the tendon sheath (GCTTS) is a slowly progressing benign tumour arising from synovial cells of tendon sheaths. It is one of the most common soft tissue tumours in the hand. We report a retrospective study of 27 proven GCTTS of the hand. The mean length of follow-up was 4.5 years (17 months-8.5 years). Radiographic findings are useful and may prove of great diagnostic value. The positive diagnosis was provided by the pathology examination after complete excision that was performed in all patients. The recurrence was noted in two surgically managed cases. The excision should be meticulous and complete in order to avoid recurrence.

A child with giant cell tumor of tendon sheath.

A giant cell tumor of tendon sheath (GCTTS) is a soft tissue tumor consisting principally of a proliferation of synovial cells arising from a tendon sheath. GCTTS is the second most common tumor of the hand in general and a majority of GCTTS cases are in patients between 20 and 50 years of age, whereas pediatric cases of GCTTS are uncommon. This report presents the case of a nine-year-old girl with GCTTS arising on her right index finger.

Epidemiology of bone tumors in Mexico City: retrospective clinicopathologic study of 566 patients at a referral institution.

A retrospective analysis of all bone tumors accessioned at a large referral center (Instituto Nacional de Rehabilitacion) in Mexico City between 2000 and 2005 is presented. A total of 6216 biopsies and surgical resection specimens were reviewed during this period, of which 566 corresponded to bone tumors. Benign bone tumors accounted for 71.6% of cases and malignant bone tumors for 28.4%. The tumors affected men in 53.7% of cases and women in 46.3% of cases, with an average age at presentation of 25 years. The femur was the most common location of the tumors (39.9%), followed by the tibia (17.7%) and humerus (11.8%). The commonest malignant bone tumors were osteosarcoma (46.6%) and chondrosarcoma (8.7%). Of malignant bone tumors, 18.6% corresponded to metastases of carcinomas from internal organs and 8.1% were multiple myeloma. The most common benign bone tumor was osteochondroma (43.7%) followed by giant cell tumor of bone (14.6%) and enchondroma (10.1%). The age distribution showed a peak in children and adolescents comprised predominantly of benign lesions and a second peak in young adults that corresponded to malignant bone tumors (principally osteosarcoma). Malignant bone tumors most often involved the femur, vertebra, and tibia. Our results parallel the findings previously reported in the world literature and show a similar distribution and epidemiology as in other developed and underdeveloped countries. Geographic location does not appear to represent a risk factor for any particular type of bone tumor and does not affect the age distribution, location, or histopathologic type of the lesions.

Giant cell tumor of tendon sheath: largest single series in children.

Giant cell tumors of tendon sheath (GCTTS) are rare in children. We hypothesized that GCTTS in children probably behave in a similar manner to the adult lesions, with regard to clinical features, imaging characteristics, histology, and recurrence rates after surgical excision. We retrospectively reviewed 29 children diagnosed and treated for GCTTS during a 16-year period and evaluated the above characteristics to compare these results with published data for adult patients. A telephone questionnaire survey was also conducted to assess the current symptoms and function, satisfaction with procedure, and incidence of recurrence. In children, we noted similar predilection for lesions in both upper and lower extremities. Most cases, 28 (96%) of 29, presented with a gradual enlarging soft tissue mass. Plain radiography demonstrated soft tissue swelling in 50% of cases. Magnetic resonance imaging features were noted to be variable, although low signal intensity on T1- and T2-weighted images was noted in most lesions. Incisional biopsy with intraoperative frozen section and histological evaluation was the gold standard for accurate diagnosis. We noted no recurrence at final follow-up in any of our cases. Twenty-two (75%) of these 29 patients had follow-up of more than 2 years, with no recurrence, and the remaining 7 had follow-up between 1 to 2 years, which may seemingly be adequate from perspective of expected time interval for early recurrences. Meticulous dissection and excision with appropriate use of magnifying surgical loupes are likely factors that may help to minimize the recurrence rate.

Evidence for increased clinical severity of familial and sporadic Paget's disease of bone in Campania, southern Italy.

UNLABELLED: The analysis of 236 Italian patients with Paget's bone disease showed higher clinical severity and greater frequency of neoplastic degeneration among patients who live or descend from individuals living in the Campania region (southern Italy). A prevalent involvement of the spine and the skull, the sites preferentially involved in giant cell tumors complicating Paget's disease, was also shown in familial cases from this geographical region. INTRODUCTION: The Campania region in southern Italy has been recently indicated as a high prevalence area for Paget's disease of bone (PDB), and most pagetic families with multiple occurrence of neoplasms in affected members were from this geographical region. MATERIALS AND METHODS: We evaluated the PDB epidemiological characteristics in 125 patients from Campania in comparison with 111 patients from other Italian regions. Twenty-three patients from Campania and 26 patients from other Italian areas had at least one first-degree relative affected by PDB (familial cases). The remaining patients made up the sporadic cases. RESULTS: Among subjects from Campania, the patients in the familial group tended to come from larger families and showed at diagnosis higher serum total alkaline phosphatase, larger extension of disease, and earlier mean age with respect to patients with PDB of the sporadic group. The skull, spine, and humerus were the sites preferentially involved in the familial cases. In contrast, no such differences were observed between familial and sporadic PDB cases among patients from the other geographical areas, except for a lower age at diagnosis. An increased PDB clinical severity was finally observed in the PDB cohort from Campania in comparison with patients from other Italian regions. Neoplastic degeneration of pagetic bones (osteosarcoma and giant cell tumor) was exclusively observed in patients with polyostotic PDB from Campania. CONCLUSIONS: We showed a higher clinical severity of PDB with occurrence of neoplastic degeneration in the high prevalence area of Campania, with its maximum expression in cases with familial disease. This peculiar pattern might be traced to genetic predisposition and/or to the abnormal impact of a still undefined environmental trigger.

Spectrum of bone tumors in Chiang Mai University Hospital, Thailand according to WHO classification 2002: A study of 1,001 cases.

OBJECTIVE: The aim of the present study was to determine the spectrum, frequency and demographics of bone tumors. MATERIAL AND METHOD: A retrospective study of the 1,001 bone tumor specimens from the files at the Pathology Department of the Chiang Mai University Hospital, Thailand from 2000 to 2004. RESULTS: From the study, 41 were non-neoplastic mass lesions, and 960 were neoplastic, with 856 (89%) as primary and 104 (11%) as metastatic tumors. In the primary tumor group, 654 (76%) cases were of hematologic origin, and 202 (24%) were non-hematologic. The most common benign bone tumors were giant cell tumor (n = 37), osteochondroma (n = 25), and chondroma (n = 15). The most common malignant bone tumors were lymphoma-leukemia (n = 583), metastatic malignancy (n = 104), plasma cell myeloma (n = 71), and osteosarcoma (n = 58). CONCLUSION: The present study showed a higher frequency of osteosarcoma (68%), lower frequencies of chondrosarcoma (12%) and Ewing sarcoma (4%) among primary non-hematologic malignant bone tumors when compared with similar studies based on Western patients. Whether these differences reflect differences in the ethnic population or in practice patterns remains to be determined