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BACKGROUND AND OBJECTIVE: Oncological distal femur resections can leave a proximal femur too short to host a stem. Reconstructive techniques are then challenging. The purpose of the study is to compare implant survival, complication rate and MSTS of two different options. METHODS: We retrospectively divided 33 patients with primary bone tumours of distal femur in Group 1 (16 patients reconstructed with knee megaprosthesis with proximal bone augmentation, APC) and Group 2 (17 patients reconstructed with total femur prosthesis, TFP). Less than 12 cm of remaining proximal femur were planned for all resections. RESULTS: MSTS score at 2 years is 25 ± 5 for Group 1 and 19 ± 7 for Group 2 (confidence interval [C.I.] 95%, p = 0.02). At 5 years it is 27 ± 2 for Group 1 and 22 ± 6 for Group 2 (C.I. 95%, p = 0.047). Failure and complication rates are lower for Group 1, but no statistical significance was reached. In APC reconstruction, union at the host-allograft junction was achieved in 16 out of 16 patients using the telescopic bone augmentation technique. CONCLUSIONS: APC provides higher functional results compared to TFP after extended distal femur resection. In APC reconstruction, telescopic augmentation is excellent for achieving union at the host-allograft junction.
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Trasplante Óseo , Neoplasias Femorales , Fémur , Humanos , Estudios Retrospectivos , Femenino , Masculino , Adulto , Trasplante Óseo/métodos , Neoplasias Femorales/cirugía , Neoplasias Femorales/patología , Persona de Mediana Edad , Fémur/cirugía , Adulto Joven , Neoplasias Óseas/cirugía , Neoplasias Óseas/patología , Adolescente , Aloinjertos , Procedimientos de Cirugía Plástica/métodos , Estudios de SeguimientoRESUMEN
OBJECTIVE: To determine the value of CT-guided bone core biopsy and investigate factors that affect diagnostic yield and biopsy outcome. MATERIALS AND METHODS: The single-centre retrospective analysis included 447 patients who had CT-guided core biopsy with a 13-G needle (Bonopty®) from January 2016 to December 2021. Histological results or ≥ 6 months of clinical and radiological follow-up served as outcome references. A successful biopsy was classified as "diagnostic" when a definitive diagnosis was made and "adequate" when only the malignant or benign nature of the tumour could be determined. Biopsies were "nondiagnostic" when the nature of the lesion could not be determined. The occult lesions were defined as not seen on CT but visible on other modalities. RESULTS: In 275 (62%) females and 172 (38%) males, the overall success rate was 85% (383 biopsies), with 314 (70%) diagnostic biopsies and 69 (15%) adequate biopsies. There was no relationship between biopsy success and the localisation of the lesion, length of biopsy material, or number of biopsy attempts. The lesions' nature had a statistically significant effect on biopsy success with lytic and mixed lesions having the highest success rate. Occult lesions had the lowest success rate. CONCLUSION: CT-guided bone core biopsy is an effective method in the workup of musculoskeletal diseases with the highest success rate in lytic and mixed lesions. No apparent relationship was found between biopsy success and biopsy length, number of attempts, or localisation of the lesion.
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Radiografía Intervencional , Tomografía Computarizada por Rayos X , Masculino , Femenino , Humanos , Estudios Retrospectivos , Tomografía Computarizada por Rayos X/métodos , Radiografía Intervencional/métodos , Biopsia con Aguja/métodos , Biopsia Guiada por Imagen/métodos , Biopsia con Aguja GruesaRESUMEN
AIMS: Simple Bone Cysts (SBCs) predominantly occur in long bones and 59% harbour NFATC2 rearrangements. Jaw SBC is rare and was previously referred to as traumatic bone cyst. It can rarely occur in association with cemento-osseous dysplasia (COD). To determine whether jaw SBCs represent the same entity as SBC of the long bones, or if they have a different molecular signature, we collected 48 jaw SBC cases of 47 patients to assess NFATC2 rearrangement. METHODS AND RESULTS: Out of the 48 cases, 36 could be used for fluorescence in-situ hybridization (FISH), of which nine (two of which associated with COD) were successful using an NFATC2 split probe. The remaining cases failed to show adequate FISH signals. All nine cases lacked NFATC2 rearrangement and five of these showed no detectable gene fusions using Archer FusionPlex. CONCLUSION: In our study, NFATC2 rearrangement is absent in solitary jaw SBC (n = 7) and COD-associated SBC (n = 2). Our findings suggest that SBC presenting in the jaw is molecularly different from SBC in long bones. Future molecular studies may confirm the absence of clonal molecular aberrations in SBC of the jaw which would support a non-neoplastic, reactive origin.
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Quistes Óseos , Factores de Transcripción NFATC , Tumores Odontogénicos , Humanos , Quistes Óseos/genética , Tumores Odontogénicos/genética , Factores de Transcripción NFATC/genéticaRESUMEN
BACKGROUND: Central giant cell granulomas (CGCG) of the jaws are osteolytic lesions that may behave aggressively and respond poorly to surgery. Microscopically, in addition to giant cells, there is a mononuclear cell population composed of macrophage/monocytic cells and spindle-shaped cells of mesenchymal origin. Seventy two percent of these tumours harbour mutually exclusive TRPV4, KRAS and FGFR1 mutations. We aimed to assess the mutational status of mononuclear and giant cells and the osteogenic potential of stromal cells in vitro and in vivo. METHODS AND RESULTS: We screened CGCG for signature mutations and used laser-capture microdissection to demonstrate that the mutations are restricted to the mononuclear cells. Additionally, we established CGCG primary cell culture and observed that the cells retained the mutations throughout passages. By flow cytometry, we observed predominance of CD14- CD51- CD61- cells, consistent with the expected profile for stromal cells. Considering the mesenchymal origin of stromal cells, we assessed the osteogenic differentiation potential of CGCG cells in culture by cytochemistry (von Kossa and alizarin red staining), alkaline phosphatase (ALP) activity assay and gene expression of osteogenic markers. CGCG cells presented self-capacity to increase ALP levels in a time-dependent manner and under osteogenic induction presented increasing number of calcium deposits, and overall higher expression of osteocalcin, RUNX2, ALPL and osteopontin than cells without osteogenic induction. A patient-derived xenograft model for CGCG was established, and osteoid material deposition was observed. CONCLUSION: Collectively, the results confirm that the signature mutations are restricted to stromal cells in CGCG, and the in vitro and in vivo results support that these cells have the capacity to differentiate into osteoblasts, in line with the bone formation often observed in the stroma of these lesions.
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Granuloma de Células Gigantes , Células Madre Mesenquimatosas , Fosfatasa Alcalina , Diferenciación Celular , Células Cultivadas , Granuloma de Células Gigantes/genética , Humanos , Maxilares , Mutación , Osteogénesis/genética , Células del EstromaRESUMEN
PURPOSE: Bone healing in femoral reconstructions using intercalary allografts can be compromised in a tumour context. There is also a high revision rate for non-union, infection, and fractures in this context. The advantages and disadvantages of an associated vascularised fibula graft (VFG) are still a matter of debate. METHODS: In a multicentre study, we retrospectively analysed 46 allograft reconstructions, operated on between 1984 and 2017, of which 18 were associated with a VFG (VFG+) and 28 without (VFG-), with a minimum follow-up of 2 years. We determined the cumulative probability of bone union as well as the mid- and long-term revision risks for both categories by Kaplan-Meier survival analysis and a multivariate Cox model. We also compared the MSTS scores. RESULTS: Significant differences in favour of VFG+ reconstruction were observed in the survival analyses for the probability of bone union (log-rank, p = 0.017) and in mid- and long-term revisions (log-rank, p = 0.032). No significant difference was observed for the MSTS, with a mean MSTS of 27.6 in our overall cohort (p = 0.060). The multivariate Cox model confirmed that VFG+ was the main positive factor for bone union, and it identified irradiated allografts as a major risk factor for the occurrence of mid- and long-term revisions. CONCLUSION: Bone union was achieved earlier in both survival and Cox model analyses for the VFG+ group. It also reduced the mid- and long-term revision risk, except when an irradiated allograft was used. In case of a tumour, we thus recommend using VFG+ from a fresh-frozen allograft, as it appears to be a more reliable long-term option.
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Neoplasias Óseas , Neoplasias Femorales , Procedimientos de Cirugía Plástica , Aloinjertos/patología , Autoinjertos , Neoplasias Óseas/patología , Trasplante Óseo , Neoplasias Femorales/cirugía , Peroné/patología , Humanos , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Giant cell tu mour accounts for up to 5% of all bone tumours and malignant giant cell tumour arises in < 10% of cases, representing sarcomatous transformation. Primary malignant giant cell tumour of bone occurs when sarcomatous tissue is observed within conventional giant cell tumour histologically on initial presentation. Secondary malignant giant cell tumour of bone occurs in a region of previously treated giant cell tumour, with most cases arising due to prior radiotherapy. Malignancy in giant cell tumour of bone does not have any unique clinical or imaging features compared to conventional aggressive disease. Historically, malignant giant cell tumour of bone has a poor prognosis which is worse in cases of secondary malignancy. This article aims to present the clinical, pathological and imaging features of MGCTB based on a review of the literature and illustrated by examples from our experience.
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Neoplasias Óseas , Tumor Óseo de Células Gigantes , Sarcoma , Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/patología , Tumor Óseo de Células Gigantes/diagnóstico por imagen , Tumor Óseo de Células Gigantes/patología , HumanosRESUMEN
An 11-year-old spayed female Toy Poodle presented with acute tetraparesis. A small subcutaneous mass was found in the right trunk region, and the magnetic resonance revealed a compressive spinal cord lesion due to an irregular bone proliferation at the third cervical vertebra. After surgical resection of the vertebral lesion, the neurological symptoms improved, and the patient could walk on her own. The excised vertebral and subcutaneous masses were diagnosed as a mammary adenocarcinoma on the histopathological examination, with Ki-67 and HER-2 immunohistochemistry staining. This case report highlights the importance of defining the primary tumours of metastatic vertebral tumours and the necessity of palliative surgery to improve the patient's quality of life.
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BACKGROUND: Acetabular reconstruction using an ice-cream cone prosthesis has been a reliable reconstruction option following pelvic tumour resection. However, it remains unknown which factor determines the success of this procedure. We aimed to determine risk factors for complications and functional loss in acetabular reconstruction using an ice-cream cone prosthesis. PATIENTS AND METHODS: Fifty-four patients with malignant bone tumours who underwent acetabular reconstruction using an ice-cream cone prosthesis between 2004 and 2016 were studied. The bone-stem ratio was calculated as the ratio of the inserted length into the bone per the entire stem length. RESULTS: A total of 26 (48%) patients had at least one complication and 11 patients (20%) required surgical interventions. The complication rates were 71% and 40% with a bone-stem ratio ≤ 50% and > 50%, respectively (p = 0.026), and the bone-stem ratio significantly stratified the risk of complications (≤ 50%: OR, 4.67 versus > 50%; p = 0.048). The mean MSTS score at the final follow-up was 60% (range 23-97%): the scores were significantly lower in patients with complications/leg-length discrepancy (52%) than in those without (79%; p = 0.002). The mean score with a bone-stem ratio ≤ 50% was significantly lower than the score with a ratio > 50%, especially in patients who underwent non-navigated reconstructions (33% versus 64%; p = 0.001). CONCLUSION: The inserted length of the coned stem into residual bone was predictive of complications and functional outcome. Surgical indication for this procedure should be considered with the size of the remaining ilium to stabilise the prosthesis with a coned stem longer than half length.
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OBJECTIVE: Intra-medullary tumour length is accurately assessed on T1-weighted turbo spin echo (T1W TSE) MRI which can be relatively time consuming, whilst the gradient echo Dixon (T1W GrE Dixon) technique is a rapid sequence (imaging time ~ 30 s). The aim of this study was to determine if the out-of-phase Dixon (OP T1W GrE Dixon) sequence can produce equivalent measurements of intra-medullary tumour length compared to the T1W TSE sequence. MATERIALS AND METHOD: Tumour length was assessed in 90 patients undergoing MRI for staging of primary bone tumours with both T1W TSE and OP T1W GrE Dixon MRI sequences at 3 T (n = 42) and 1.5 T (n = 48). Tumour length was measured independently by different observers allowing assessment of inter-observer correlation, and the correlation between measurements on T1W TSE and OP T1W GrE Dixon sequences was also determined. RESULTS: There were 53 males and 37 females (mean age 36.4 years; range 2-77 years). Inter-observer correlation for tumour length on both the T1W TSE and T1W OP GrE Dixon sequences was very good (ICC = 0.94-0.98), and measurement of tumour length comparing T1W TSE and T1W GrE Dixon was also very good (ICC = 0.91-0.99). In 4 cases, tumour length was significantly overestimated on T1W TSE images due to extensive reactive marrow oedema, but more accurately determined on the OP sequence when compared to resection specimens. CONCLUSIONS: The OP T1W GrE Dixon sequence is comparable to T1W TSE for assessment of the intra-medullary length of appendicular bone tumours, and more accurate in the presence of extensive reactive marrow oedema.
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Neoplasias Óseas , Imagen por Resonancia Magnética , Adolescente , Adulto , Anciano , Médula Ósea , Neoplasias Óseas/diagnóstico por imagen , Niño , Preescolar , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto JovenRESUMEN
OBJECTIVE: The objective of this study is to compare T1-weighted gradient echo (T1W GrE: control technique) chemical shift imaging (CSI) with T2-weighted fast spin echo (T2W FSE: experimental technique) CSI for differentiating non-neoplastic and neoplastic marrow lesions. MATERIALS AND METHODS: Patients undergoing MRI for various marrow lesions were investigated with T1W GrE and T2W FSE Dixon CSI. Signal intensity (SI) change between in-phase (IP) and opposed-phase (OP) sequences was calculated, and SI drop > 20% considered to represent non-neoplastic lesions while SI drop < 20% considered to represent neoplastic lesions. Final diagnosis was based on imaging features (n = 42) or histology (n = 43) and classified as non-neoplastic, benign neoplastic, and malignant neoplastic. Inter-observer and inter-technique agreement between 2 readers was calculated. RESULTS: The study included 85 patients (44 males and 41 females; mean age 41.1 years, range 2-83 years). Final diagnosis included 19 (22.4%) non-neoplastic lesions, 27 (31.8%) benign neoplasms, and 39 (45.9%) malignant neoplasms. On T1W GrE CSI, 19-21 lesions were classed as non-neoplastic and 64-66 as neoplastic, while on T2W FSE Dixon CSI, 22-24 lesions were classed as non-neoplastic and 61-64 as neoplastic. Lesion classification matched between the 2 techniques in 91.8-96.5% of cases. Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of T1W GrE CSI for differentiating non-neoplastic and neoplastic marrow lesions were 66.7-72.2%, 88.1-89.6%, 61.9-63.2%, 90.9-92.2%, and 84.7%, and of T2W FSE Dixon CSI were 72.2-77.8%, 85.1-86.6%, 58.3-59.1%, 92.1-93.4%, and 83.5%. CONCLUSIONS: T1W GrE CSI and T2W FSE Dixon CSI produce similar results in the assessment of non-neoplastic and neoplastic marrow lesions.
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Médula Ósea , Imagen por Resonancia Magnética , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Médula Ósea/diagnóstico por imagen , Niño , Preescolar , Femenino , Humanos , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Adulto JovenRESUMEN
EWSR1-SMAD3 fibroblastic tumour is a recently described soft tissue lesion. To date, eight cases have been reported, all sited in superficial soft tissue, typically occurring in the hands and feet with a tendency for local recurrence if incompletely excised. No metastatic spread has been reported, and hence, these tumours are currently considered benign. Herein, we present the radiological and histological features of the first reported occurrence of this entity in bone: a 44-year-old man with a tumour in the right tibia, treated with en bloc resection and showing no signs of relapse at 7 years. This tumour should be added to the differential diagnosis of bone lesions which harbour EWSR1 gene rearrangement.
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Proteínas de Unión a Calmodulina , Proteínas de Unión al ARN , Adulto , Proteínas de Unión a Calmodulina/genética , Humanos , Masculino , Proteína EWS de Unión a ARN/genética , Proteína smad3RESUMEN
PURPOSE: To investigate the indications, approaches, resection methods, and complications of total sacrectomy with a combined antero-posterior approach for malignant sacral tumours. METHODS: Fourteen cases of primary malignant sacral tumours treated with total sacrectomy between January 2012 and 2018 were retrospectively analysed. All patients presented with pre-operative lumbosacral pain or constipation. A combined antero-posterior approach was used for tumour resection, and the spinal pedicle screw rod system was used to achieve ilio-lumbar stability. The visual analogue scale (VAS) and Musculoskeletal Tumor Society (MSTS) scores were used to assess pain and lower limb function, respectively. The mean operative time and intra-operative blood loss were 6.54 hours and 2935 mL, respectively. The mean follow-up period was 62 months. RESULTS: None of the patients died peri-operatively. At the last follow-up, ten patients were continuously disease-free, three were alive with disease, and one died of disease from lung metastasis. Tumour recurrence occurred in three patients. The MSTS scores ranged from 6 to 28 (20.00-93.33%, 6/30-28/30) with an average of 20 (66.67%, 20/30). Seven patients could walk independently in public, five could only walk at home using a walking aid, and two could only lie down and stand for a short time. Thirteen patients developed post-operative complications such as skin necrosis, screw loosening, connecting rod fracture, neuropathic pain, sciatic nerve injury, dysuria, and urinary incontinence. CONCLUSION: Total sacrectomy can effectively treat malignant sacral tumours with good resection boundaries and prognosis. However, the high incidence of post-operative complications may impact post-operative neurological function.
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Neoplasias de la Columna Vertebral , Tornillos Óseos , Humanos , Osteotomía , Estudios Retrospectivos , Sacro/cirugía , Neoplasias de la Columna Vertebral/cirugíaRESUMEN
PURPOSE: With the ability to overcome specific anatomical and pathological challenges, 3D printing technology is setting itself as an important tool in patient-specific orthopaedics, delivering anatomical models, patient-specific instruments, and custom-made implants. One of the most demanding procedures in limb salvage surgery is the reconstruction of bony defects after tumour resection. Even though still limited in clinical practice, early results of the use of 3D technology are gradually revealing its potentially huge impact in bone tumour surgery. Here, we present a case series illustrating our experience with the use of 3D printing technology in the reconstruction of bone defects after tumour resection, and its impact on cosmesis and quality of life. METHODS: We performed a retrospective analysis of 11 patients in whom a custom-made 3D-printed prosthesis was used to reconstruct a bone defect after resection for a bone tumour. Ten out of 11 patients were children (aged between 5 and 16 years) with osteosarcoma or Ewing sarcoma of the pelvis (2 children) or the arm (8 children), and one patient was a 67-year-old lady with a chondrosarcoma of the pelvis. All underwent wide resections resulting in considerable bone defects necessitating further reconstruction. RESULTS: Custom-made implants were extremely useful both in reconstruction of bone defects and in terms of cosmesis, recovery facilitation, and quality of life. In this respect, pelvic and humeral reconstructions with 3D-printed custom implants particularly showed a great potential. The mean follow-up was 33 months. Four patients died of disease (36%) and overall the major and minor complication rate was 54% (6 out of 11 patients). Three patients had implant dislocation (27% [3/11 cases]), one had leg-compartment syndrome, and one patient reported limited range of motion. Only two out of 11 patients developed local recurrence. CONCLUSION: Use of 3D customized implant helped us achieve two major goals in orthopaedic oncology-clear surgical resection and functional recovery with a good quality of life. Large studies with long-term follow-up are needed to reveal the value and future of 3D printing in orthopaedic oncology.
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Neoplasias Óseas , Procedimientos de Cirugía Plástica , Adolescente , Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/cirugía , Niño , Preescolar , Femenino , Humanos , Impresión Tridimensional , Prótesis e Implantes , Calidad de Vida , Estudios RetrospectivosRESUMEN
Osteosarcoma (OS) is one of the most common metastatic bone cancers, which results in significant morbidity and mortality. The important role of long non-coding RNAs (lncRNAs) in the biological processes of OS has been demonstrated through several studies. In the current study, we evaluated the role of the lncRNA, LINC01128, in OS. We analysed the expression of LINC01128 in three OS gene expression omnibus (GEO) data sets GSE21257, GSE36001 and GSE42352. The expression of LINC01128 in OS tissues and matched non-tumour tissues obtained from 50 OS patients was detected using qRT-PCR. The association between LINC01128 expression and overall survival of OS patients was evaluated using the Kaplan-Meier method. The effects of LINC01128 knockdown and overexpression were evaluated through in vitro and in vivo assays. The LINC01128/miR-299-3p/ MMP2 axis was verified using dual-luciferase reporter assay and qRT-PCR assays. GEO data sets analysis revealed that the expression of LINC01128 was increased in OS. Elevated LINC01128 expression was accompanied by shorter overall survival in OS patients. Functional studies revealed that LINC01128 knockdown reduced the proliferation, migration and invasion of OS cells both in vitro and in vivo. Mechanistically, LINC01128 sponged miR-299-3p to increase MMP2 expression. Rescue assays determined the role of the LINC01128/miR-299-3p/MMP2 axis in the proliferation, migration and invasion of OS cells. Additionally, the Wnt/ß-catenin signalling pathway was activated by LINC01128 and MMP2 in OS cell lines. In summary, this study demonstrates that LINC01128 facilitates OS by functioning as a sponge of miR-299-3p, thus promoting MMP2 expression and activating the Wnt/ß-catenin signalling pathway.
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Neoplasias Óseas/genética , Neoplasias Óseas/metabolismo , Metaloproteinasa 2 de la Matriz/genética , MicroARNs/genética , Osteosarcoma/genética , Osteosarcoma/metabolismo , ARN Largo no Codificante/genética , Vía de Señalización Wnt , Adolescente , Animales , Neoplasias Óseas/mortalidad , Neoplasias Óseas/patología , Línea Celular Tumoral , Movimiento Celular , Proliferación Celular , Niño , Modelos Animales de Enfermedad , Femenino , Regulación Neoplásica de la Expresión Génica , Genes Reporteros , Humanos , Masculino , Ratones , Estadificación de Neoplasias , Osteosarcoma/mortalidad , Osteosarcoma/patología , Pronóstico , Interferencia de ARN , Carga Tumoral , Ensayos Antitumor por Modelo de Xenoinjerto , Adulto JovenRESUMEN
AIMS: De-differentiated chordoma is an uncommon and incompletely characterised aggressive neoplasm. Only a few cases originating from the skull base have been reported. METHODS AND RESULTS: All consecutive cases of skull-base de-differentiated chordomas treated surgically in a referral centre from January 1990 to June 2019 were retrospectively evaluated to assess peculiar pathological, radiological and clinical features. Patient data were retrieved from paper and electronic records. Six cases (two male, four female; mean age at surgery = 46 years, range = 35-64), treated surgically at our institution were identified. Transformation to de-differentiated chordomas occurred after radiation therapy in three cases (mean = 13.6 years after treatment, range = 5-25), two during tumour progression, while one was de-novo. Magnetic resonance imaging and surgical examination revealed the presence of two different tumour components, corresponding to the conventional and de-differentiated portion on histological examination. The de-novo case presented a PIK3CA mutation. DNA methylation analysis revealed consistent epigenetic changes in TERT, MAGEA11 and UXT. Prognosis was poor, as five of six patients died after surgery and radiation therapy, with a mean overall survival of 29 months (range = 11-52). CONCLUSIONS: Skull-base de-differentiated chordomas are extremely rare and aggressive neoplasms with characteristic magnetic resonance imaging, surgical and histological features. Therefore, an early and accurate histological diagnosis is of paramount relevance. Molecular analysis appears promising to define mechanisms involved in tumour de-differentiation.
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Cordoma/patología , Neoplasias de la Base del Cráneo/patología , Adulto , Cordoma/diagnóstico por imagen , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Neoplasias de la Base del Cráneo/diagnóstico por imagenRESUMEN
BACKGROUND AND OBJECTIVES: Coronavirus disease 2019 (COVID-19) lockdown has presented a unique challenge for sarcoma care. The purpose of this study is to evaluate the early results and feasibility of surgeries for bone sarcomas during the COVID-19 lockdown. METHODS: Our prospectively collected orthopaedic oncological database was reviewed to include two groups of patients- those who underwent surgery in the immediate 4 weeks before lockdown (non-lockdown group) and those operated in the first 4 weeks of lockdown (lockdown group). All patients were followed-up clinically and telephonically to collect the outcome data. RESULTS: Out of the 91 patients who qualified for inclusion, fifty were classified into the non-lockdown group while 41 patients formed the lockdown group. Both the groups were comparable with respect to baseline demographic parameters. However, during the lockdown period 37 patients (90%) had undergone a major surgical intervention as against 24 patients (48%) in the non-lockdown group (P < .001). There was no significant difference in type of anaesthesia, median estimated blood loss and procedure duration. None of the patients/health care workers had evidence of severe acute respiratory syndrome-coronavirus 2 infection at 15 days follow-up. CONCLUSION: Our study results suggest that appendicular bone tumours can be safely operated with adequate precautions during the lockdown period.
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Neoplasias Óseas/cirugía , COVID-19/epidemiología , Sarcoma/cirugía , Adulto , Neoplasias Óseas/patología , COVID-19/diagnóstico , COVID-19/transmisión , Condrosarcoma/patología , Condrosarcoma/cirugía , Estudios de Factibilidad , Femenino , Humanos , India/epidemiología , Transmisión de Enfermedad Infecciosa de Paciente a Profesional/prevención & control , Recuperación del Miembro/métodos , Recuperación del Miembro/normas , Masculino , Procedimientos Ortopédicos/métodos , Procedimientos Ortopédicos/normas , Osteosarcoma/patología , Osteosarcoma/cirugía , Pandemias , Sarcoma/patología , Sarcoma de Ewing/patología , Sarcoma de Ewing/cirugía , Centros de Atención Terciaria , Adulto JovenRESUMEN
Non-ossifying fibroma (NOF), which occasionally results in pathologic fracture, is considered the most common benign and self-limiting lesion of the growing skeleton. By DNA sequencing we have identified hotspot KRAS, FGFR1 and NF1 mutations in 48 of 59 patients (81.4%) with NOF, at allele frequencies ranging from 0.04 to 0.61. Our findings define NOF as a genetically driven neoplasm caused in most cases by activated MAP-kinase signalling. Interestingly, this driving force either diminishes over time or at least is not sufficient to prevent autonomous regression and resolution. Beyond its contribution to a better understanding of the molecular pathogenesis of NOF, this study adds another benign lesion to the spectrum of KRAS- and MAP-kinase signalling-driven tumours. Copyright © 2018 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
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Neoplasias Óseas/genética , Fibroma/genética , Sistema de Señalización de MAP Quinasas/genética , Mutación , Adolescente , Neoplasias Óseas/patología , Análisis Mutacional de ADN/métodos , Femenino , Fibroma/patología , Predisposición Genética a la Enfermedad , Humanos , Masculino , Neurofibromina 1/genética , Proteínas Proto-Oncogénicas p21(ras)/genética , Receptor Tipo 1 de Factor de Crecimiento de Fibroblastos/genética , Secuenciación del Exoma/métodos , Adulto JovenRESUMEN
Non-ossifying fibroma (NOF) has been an intriguing entity since its first description. It is the most common bone tumour, is usually asymptomatic affecting children and adolescents, is composed of a heterogeneous cell population, and undergoes spontaneous regression after puberty. In a recent article in The Journal of Pathology, Baumhoer and colleagues demonstrate mutations activating the RAS-MAPK pathway (KRAS, FGFR1 and NF1) in â¼80% of the tumours. Activation of the RAS-MAPK pathway by somatic mutations is found in a plethora of tumour types, both benign and malignant, while germline mutations cause a wide range of syndromes collectively termed the RASopathies. Their findings indicate that NOF, for long thought to be reactive, should be considered a true neoplasm. Moreover, their data suggest that only a subset of cells in the lesion contain the mutation. A second cell population consisting of histiocytes and osteoclast-like giant cells appears to be reactive. This intimate relation between WT and mutant cells is also frequently encountered in other benign and locally aggressive bone tumours and seems essential for tumourigenesis. The spontaneous regression remains enigmatic and it is tempting to speculate that pubertal hormonal signalling, especially increased oestrogen levels, affect the balance between mutant and WT cells. © 2019 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of Pathological Society of Great Britain and Ireland.
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Neoplasias Óseas , Fibroma , Adolescente , Transformación Celular Neoplásica , Niño , Humanos , Mutación , Reino UnidoRESUMEN
BACKGROUND: Reports of osteoblastic tumours are limited to a few case reports in veterinary medicine. Osteoblastoma-like osteosarcoma has been accepted by the World Health Organization as an intermediate form between an osteosarcoma and osteoblastoma. This type of tumour indicates an osteosarcoma, that may resemble osteoblastoma clinically, histologically, and radiologically and have the capability for metastasis. Osteoblastoma-like osteosarcoma has not been described in veterinary medicine so far. CASE PRESENTATION: An eight-year old cat was presented due to progressive ataxia and paraparesis of the pelvic limbs. Imaging confirmed a well-defined, extradural mass originating from the spinous process of the second thoracic vertebra (T2) leading to severe compression of the spinal cord. Decompressive cytoreduction was achieved by removal of the mass after dorsal laminectomy of T1. After recovering from an acute worsening 3.5 weeks after surgery, the cat had an improved neurological status and the dorsal compression was resolved at follow-up 8 months later. A focal contrast enhancing lesion was still evident at the base of T2 spinous process and lung metastasis was additionally suspected. Based on histopathological, radiographic, and clinical features, an "osteoblastoma-like osteosarcoma" was suspected. CONCLUSIONS: To the best of our knowledge, this is the first description of this tumour in veterinary medicine. In addition, this case report highlights the difficulty in the diagnosis and definition of osseous neoplasia in cats and provides a literature review.
Asunto(s)
Enfermedades de los Gatos/patología , Osteosarcoma/veterinaria , Neoplasias de la Columna Vertebral/veterinaria , Animales , Enfermedades de los Gatos/diagnóstico , Enfermedades de los Gatos/cirugía , Gatos , Femenino , Laminectomía/veterinaria , Neoplasias Pulmonares/secundario , Osteoblastoma/patología , Osteoblastoma/cirugía , Osteoblastoma/veterinaria , Osteosarcoma/patología , Osteosarcoma/cirugía , Neoplasias de la Columna Vertebral/patología , Neoplasias de la Columna Vertebral/cirugía , Columna Vertebral/patologíaRESUMEN
BACKGROUND: Bizarre parosteal osteochondromatous proliferation (BPOP) is a relatively rare benign extraperiosteal osteochondroma-like proliferative lesion that shares similarities with malignant tumours in terms of morphology. The aetiology of BPOP has yet to be determined and there are no previous reports of BPOP associated with fracture. CASE PRESENTATION: A 57-year-old woman presented with a one-month history of pain and swelling in her right foot, which were worsened by activity and improved with rest. Physical examination revealed a hard, non-mobile mass measuring 1.5 cm × 1.5 cm on the dorsal aspect of the third metatarsal of the right foot. There was overlying erythema and tenderness on palpation. Computed tomography (CT) of the right foot demonstrated a fracture of the neck of the third metatarsal, osteolysis at the fracture site and soft tissue swelling. Bone scintigraphy revealed increased tracer uptake suggesting abnormal bone metabolism at the neck of the third metatarsal. Surgical excision of the lesion was performed. Histopathology and immunohistochemistry confirmed the diagnosis of BPOP. CONCLUSION: BPOP is a rare benign lesion that is commonly misdiagnosed. Differential diagnosis is mainly achieved through imaging and histopathological assessment.