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Sarcoma , Neoplasias de los Tejidos Blandos , Humanos , Sarcoma/terapia , Atención a la SaludRESUMEN
Systemic relapse after radiotherapy and surgery is the major cause of disease-related mortality in sarcoma patients. Combining radiotherapy and immunotherapy is under investigation as a means to improve response rates. However, the immune contexture of sarcoma is understudied. Here, we use a retrospective cohort of sarcoma patients, treated with neoadjuvant radiotherapy, and TCGA data. We explore therapeutic targets of relevance to sarcoma, using genomics and multispectral immunohistochemistry to provide insights into the tumor immune microenvironment across sarcoma subtypes. Differential gene expression between radioresponsive myxoid liposarcoma (MLPS) and more radioresistant undifferentiated pleomorphic sarcoma (UPS) indicated UPS contained higher transcript levels of a number of immunotherapy targets (CD73/NT5E, CD39/ENTPD1, CD25/IL2RA, and 4-1BB/TNFRSF9). We focused on 4-1BB/TNFRSF9 and other costimulatory molecules. In TCGA data, 4-1BB correlated to an inflamed and exhausted phenotype. OX40/TNFRSF4 and 4-1BB/TNFRSF9 were highly expressed in sarcoma subtypes versus other cancers. Despite OX40 and 4-1BB being described as Treg markers, we identified that they delineate distinct tumor immune profiles. This was true for sarcoma and other cancers. While only a limited number of samples could be analyzed, spatial analysis of OX40 expression identified two diverse phenotypes of OX40+ Tregs, one associated with and one independent of tertiary lymphoid structures (TLSs). Patient stratification is of intense interest for immunotherapies. We provide data supporting the viewpoint that a cohort of sarcoma patients, appropriately selected, are promising candidates for immunotherapies. Spatial profiling of OX40+ Tregs, in relation to TLSs, could be an additional metric to improve future patient stratification.
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Sarcoma , Neoplasias de los Tejidos Blandos , Adulto , Humanos , Recurrencia Local de Neoplasia , Estudios Retrospectivos , Sarcoma/genética , Sarcoma/terapia , Linfocitos T Reguladores , Microambiente TumoralRESUMEN
INTRODUCTION: Desmoid-type fibromatosis (DF) are locally infiltrative, non-metastasizing tumours associated with significant morbidity and mortality if located intra-abdominally, retroperitoneally or in head and neck localisation. They are mostly sporadic, due to somatic CTNNB1 mutations. Alternatively, they can be associated with germline pathogenic variants in APC causing Familial Adenomatous Polyposis (FAP). Germline APC variants and somatic CTNNB1 mutations are mutually exclusive. AIMS AND METHODS: We conducted a retrospective descriptive analysis of patients with DF seen at the Royal Marsden NHS Foundation Trust Sarcoma Unit in London. We aimed to describe the methods of screening for FAP in patients with DF from a specialist unit. Patients diagnosed between 1992 and 2020 were selected from the prospectively maintained Sarcoma Unit database. RESULTS: 226 patients were identified and 67% (n = 152) were female. Median age at diagnosis was 37.5 (range 2-81) years. Tumour localisation was limbs/pelvis in 30.9% (N = 70), intra-abdominal 16.8% (N = 38), abdominal wall 23.5% (N = 53), thorax 18.6% (N = 42), head and neck 3.1% (N = 7) and vertebral/paravertebral 7.1% (N = 16). Colonoscopy was requested in 65 patients (28.8% of all cases) and was completed in forty-six (20.4%). Molecular testing of CTNNB1 testing was requested in 35 cases (15.5%). APC germline test was requested in 12 cases. Four patients in our cohort had an FAP-associated DF. CONCLUSIONS: CTNNB1 ± APC testing and colonoscopy are useful tools for the screening of patients with DF. CTNNB1 molecular testing should be performed in all cases of newly diagnosed DF. Negative CTNNB1 results, alongside clinical assessment, should prompt APC testing and/or colonoscopy.
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Poliposis Adenomatosa del Colon , Fibromatosis Agresiva , Poliposis Adenomatosa del Colon/complicaciones , Poliposis Adenomatosa del Colon/diagnóstico , Poliposis Adenomatosa del Colon/genética , Proteína de la Poliposis Adenomatosa del Colon/genética , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Preescolar , Femenino , Fibromatosis Agresiva/complicaciones , Fibromatosis Agresiva/diagnóstico , Fibromatosis Agresiva/genética , Genes APC , Humanos , Persona de Mediana Edad , Mutación , Estudios Retrospectivos , Reino Unido , Adulto JovenRESUMEN
Although soft-tissue masses are common, sarcomas are rare malignant neoplasms showing variable mesenchymal differentiation and can occur at any anatomical site. Limb soft-tissue sarcomas (STS) are rare, but often lethal tumours. Although there are scores of historical pathological subtypes, this article will deal with the commonest: liposarcoma, leiomyosarcoma (LMS), undifferentiated pleomorphic sarcoma (UPS), synovial sarcoma, myxofibrosarcoma, malignant peripheral nerve sheath tumour (MPNST), epithelioid sarcoma, alveolar rhabdosarcoma, angiosarcoma and radiation-induced sarcoma (RIS). Following a review of >4,000 adult patients with limb sarcoma from our specialist soft-tissue tumour database, we summarise the literature and their imaging findings, with emphasis on radiological hallmarks that can aide in diagnosis and management. Increased awareness of sarcoma when challenged with a new mass in the extremity can ensure timely and appropriate treatment.
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Extremidades , Sarcoma/diagnóstico por imagen , Neoplasias de los Tejidos Blandos/diagnóstico por imagen , Humanos , Imagen por Resonancia Magnética , Sarcoma/patología , Neoplasias de los Tejidos Blandos/patología , Tomografía Computarizada por Rayos X , UltrasonografíaRESUMEN
AIMS: Embryonal and alveolar rhabdomyosarcoma (ERMS, ARMS) are subtypes of RMS that mainly occur in children, with relatively good outcomes. The incidence in adults is extremely low and survival is significantly worse compared with children. Data are scarce and literature generally combines all RMS subtypes, including pleomorphic RMS, which primarily occurs in adults and behaves more like undifferentiated pleomorphic sarcoma. The aim of this study was to evaluate patient and tumour characteristics, outcome and prognostic factors in adult patients with ERMS and ARMS. MATERIALS AND METHODS: All adult (18 years or older) ERMS and ARMS patients (presenting 1990-2016) were identified from a prospectively maintained database and were included in this analysis. RESULTS: Overall, 66 patients were included (42 men, 24 women). The median age at presentation was 28 years (range 18-71). The median overall survival for all ARMS (n = 42) and ERMS (n = 24) patients was 18 months, with a 5-year overall survival rate of 27%. Patients presenting with localised disease (n = 38, 58%) and metastatic disease (n = 25, 42%), had a 5-year overall survival rate of 36% and 11%, respectively. In univariate analysis we found alveolar subtype, fusion gene positivity, infiltrative tumour and metastatic presentation to be negative prognostic factors. CONCLUSION: Survival in adult ERMS and ARMS patients is poor and the current data may be useful in the design of trials with novel agents. Ideally, paediatric and adult oncologists should set up trials together to get a better understanding of biological, genetic and clinically relevant factors in this disease.
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Rabdomiosarcoma Alveolar/epidemiología , Rabdomiosarcoma Embrionario/epidemiología , Neoplasias de los Tejidos Blandos/epidemiología , Adolescente , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Rabdomiosarcoma Alveolar/mortalidad , Rabdomiosarcoma Alveolar/patología , Rabdomiosarcoma Embrionario/mortalidad , Rabdomiosarcoma Embrionario/patología , Neoplasias de los Tejidos Blandos/mortalidad , Neoplasias de los Tejidos Blandos/patología , Tasa de Supervivencia , Adulto JovenRESUMEN
BACKGROUND: Initial grading of retroperitoneal leiomyosarcoma (LMS) is performed by core biopsy (CB) however, discrepancy between grade of tumour at initial CB and surgical excision is recognised, raising concerns about the accuracy of CB for directing neoadjuvant therapy. The histological grading system used for staging LMS consists of 3 components: tumour differentiation, mitotic index and proportion of necrosis. We postulate that assessment of necrosis by histopathology alone is inadequate, resulting in under-grading of LMS. We propose and assess a combined grading system that incorporates CT scan findings into pre-surgical grading. METHODS: Retrospective, blinded review of CT, CB histology and final surgical histology of patients with retroperitoneal LMS was undertaken. A modified grading system, CTH-Grade, was derived by replacing the CB necrosis score with a CT-derived necrosis score. The sensitivity and specificity of CTH-Grade, the standard histopathology scoring, H-grade were compared. Inter-observer variability in assessment of CT necrosis was also assessed. RESULTS: 53 patients fulfilled criteria for inclusion. CT was more sensitive at detection of necrosis than CB histology alone with sensitivity of 100% vs 53%. The use of CTHGrade resulted in increased detection of high-grade tumours with CTH-grade having sensitivities of 80% and 35% for Grade 2 and 3 tumours respectively vs 53% and 15% with H-Grade. Assessment of reader agreement demonstrated Kappa scores of 0.8. CONCLUSION: Histology from CB under-grades LMS due to undersampling of tumour necrosis. CT is more sensitive in assessing necrosis and its incorporation into a modified CT-histopathology grading system (CTH-Grade) improves accuracy of grading with significant implications for patient management.
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Leiomiosarcoma/patología , Neoplasias Retroperitoneales/patología , Tomografía Computarizada por Rayos X/métodos , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Leiomiosarcoma/diagnóstico por imagen , Leiomiosarcoma/terapia , Masculino , Persona de Mediana Edad , Necrosis , Clasificación del Tumor , Valor Predictivo de las Pruebas , Neoplasias Retroperitoneales/diagnóstico por imagen , Neoplasias Retroperitoneales/terapia , Estudios RetrospectivosRESUMEN
The characterisation and clinical relevance of tumour-infiltrating lymphocytes (TILs) in leiomyosarcoma (LMS), a subtype of soft tissue sarcoma that exhibits histological heterogeneity, is not established. The use of tissue microarrays (TMA) in studies that profile TIL burden is attractive but given the potential for intra-tumoural heterogeneity to introduce sampling errors, the adequacy of this approach is undetermined. In this study, we assessed the histological inter- and intra-tumoural heterogeneity in TIL burden within a retrospective cohort of primary LMS specimens. Using a virtual TMA approach, we also analysed the optimal number of TMA cores required to provide an accurate representation of TIL burden in a full tissue section. We establish that LMS have generally low and spatially homogenous TIL burdens, although a small proportion exhibit higher levels and more heterogeneous distribution of TILs. We show that a conventional and practical number (e.g. ≤3) of TMA cores is adequate for correct ordinal categorisation of tumours with high or low TIL burden, but that many more cores (≥11) are required to accurately estimate absolute TIL numbers. Our findings provide a benchmark for the design of future studies aiming to define the clinical relevance of the immune microenvironments of LMS and other sarcoma subtypes.
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Leiomiosarcoma/inmunología , Linfocitos Infiltrantes de Tumor/citología , Neoplasias de los Tejidos Blandos/inmunología , Análisis de Matrices Tisulares , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores de Tumor/análisis , Femenino , Humanos , Inmunohistoquímica , Masculino , Persona de Mediana Edad , Reproducibilidad de los Resultados , Estudios Retrospectivos , Microambiente TumoralRESUMEN
BACKGROUND: Deep lipomatous tumours can be benign lipomas or intermediate/locally recurring atypical lipomatous tumours (ALTs). Differentiating between these two entities clinically and radiologically is difficult. The aims of this study were to report a series of deep lipomatous tumours, comparing the clinical, radiological and pathological features of ALTs and lipomas; and to predict the likelihood of a lipomatous tumour being ALT based on anatomical site and MRI characteristics. METHODS: This was a retrospective review of patients with deep lipomatous tumours presenting over 6 years to a tertiary sarcoma centre, with preoperative MRI, and preoperative or postoperative histology including MDM2 gene analysis. Sensitivity, specificity, predictive values and accuracy in diagnosing ALT were calculated for MRI and histopathological features. RESULTS: Some 248 patients were included; 81 (32·7 per cent) had a final diagnosis of ALT. ALTs were larger than lipomas (median 19 versus 10 cm; P < 0·001); there was no ALT smaller than 5 cm. A tumour presenting in the lower limb was more likely to be an ALT than a lesion at any other site (48·4 versus 13·5 per cent; P < 0·001). In patients with lipomatous tumours at sites other than the lower limbs, MRI had a negative predictive value of 95 per cent for excluding ALT. CONCLUSION: Despite concern, most deep lipomatous tumours (nearly 70 per cent) are benign lipomas. Certain features imply that tumours are almost never ALT: smaller than 5 cm or located outside the lower limb with no suspicious characteristics on MRI. Tumours with these features might safely and confidently be managed outside tertiary sarcoma centres.
ANTECEDENTES: Los tumores lipomatosos profundos pueden ser lipomas benignos o tumores lipomatosos atípicos (atypical lipomatous tumour, ALT) con potencial de recidiva local/intermedia. Diferenciar estas dos entidades desde el punto de vista clínico es difícil. Los objetivos de este estudio fueron presentar una gran serie de tumores lipomatosos profundos, comparando las características clínicas, radiológicas y patológicas de los ALT y de los lipomas y predecir la probabilidad de que un tumor lipomatoso sea ALT según su localización anatómica y las características de la RNM. MÉTODOS: Revisión retrospectiva de pacientes con tumores lipomatosos profundos tratados en un centro terciario de sarcoma durante un período de 6 años, en los que se dispusiese de RNM preoperatoria y análisis MDM2 en el preoperatorio o postoperatorio. Se calculó la sensibilidad, la especificidad, el valor predictivo y la precisión diagnóstica de la RNM y de las características histopatológicas para el diagnóstico de ALT. RESULTADOS: Se incluyeron 248 pacientes, de los que en solo 81 (32,7%) se estableció un diagnóstico final de ALT. Los ALT fueron más grandes que los lipomas (19 versus 10 cm, P < 0,001) y no hubo ningún ALT de tamaño menor de 5 cm. Hubo una mayor probabilidad de que un tumor fuera ALT si se presentaba en las extremidades inferiores en comparación con cualquier otra localización (48,4% versus 13,5%, P < 0,001). En pacientes con tumores lipomatosos localizados en otros lugares que no fueran las extremidades inferiores, la RMN tuvo un valor predictivo negativo del 95,5% para excluir la ALT. CONCLUSIÓN: A pesar del recelo tradicional, la mayoría (70%) de los tumores lipomatosos profundos son lipomas benignos. Algunas características, como los tumores de menos de 5 cm y aquellos ubicados fuera de las extremidades inferiores sin características sospechosas por RNM, indican que los tumores casi nunca son ALT. Los tumores con esas características pueden tratarse de manera segura y con solvencia fuera de los centros de sarcomas terciarios. En casos seleccionados, puede ser útil la prueba genética MDM2 en la biopsia.
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Liposarcoma/diagnóstico , Estadificación de Neoplasias/métodos , Neoplasias de los Tejidos Blandos/diagnóstico , Adulto , Anciano , Anciano de 80 o más Años , Biopsia , Nalgas , Diagnóstico Diferencial , Femenino , Estudios de Seguimiento , Humanos , Liposarcoma/cirugía , Extremidad Inferior , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Periodo Posoperatorio , Periodo Preoperatorio , Pronóstico , Reproducibilidad de los Resultados , Estudios Retrospectivos , Neoplasias de los Tejidos Blandos/cirugía , Procedimientos Quirúrgicos Operativos , Extremidad Superior , Adulto JovenRESUMEN
BACKGROUND: Conventional biomarkers in thyroid cancer are not disease specific and fluctuate in advanced disease, making interpretation difficult. Circulating tumour DNA (ctDNA) has been shown to be a useful biomarker in other solid tumours. This is a multimutational study of ctDNA over multiple timepoints, designed to test the hypothesis that ctDNA is a potential biomarker in patients with advanced thyroid cancer. METHODS: Mutational analysis of archival tumour tissue was performed using NGS with a targeted gene panel. Custom TaqMan assays were designed for plasma ctDNA testing using digital droplet polymerase chain reaction. Concentrations of detected ctDNA were correlated with the conventional biomarker concentration and axial imaging status defined by the Response Evaluation Criteria in Solid Tumours criteria. RESULTS: Tumour tissue from 51 patients was obtained, with the following histologies: 32 differentiated (differentiated thyroid cancer [DTC]), 15 medullary (medullary thyroid cancer [MTC]), three poorly differentiated and one anaplastic. NGS analysis detected variants in 42 (82%) of cases. Plasma was assayed for these patients in 190 samples, and ctDNA was detected in 67% of patients. Earlier detection of disease progression was noted in three patients with MTC. In two cases (PTC and ATC), where conventional biomarkers were not detectable, ctDNA was detected before disease progression. Changes in ctDNA concentration occurred earlier than conventional markers in response to disease progression in multiple patients with DTC receiving targeted therapies. CONCLUSION: The majority of patients with advanced thyroid cancer had detectable ctDNA. ctDNA measurement may offer superiority over conventional markers in several scenarios: earlier detection of progression in MTC; as an alternative biomarker when conventional markers are not available; more rapid assessment of the disease status in response to targeted therapies, thereby potentially allowing prompter discontinuation of futile therapies. These early results support the hypothesis that ctDNA may be a clinically useful biomarker in thyroid cancer.
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ADN Tumoral Circulante/genética , Medicina de Precisión/métodos , Neoplasias de la Tiroides/genética , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores de Tumor , Progresión de la Enfermedad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Neoplasias de la Tiroides/patologíaRESUMEN
BACKGROUND: Radiation induced angiosarcoma (RIAS) of the breast is a rare and aggressive complication of radiotherapy. Due to the rarity of this disease, much of the evidence for its management is based on case reports or small retrospective series. We sought to describe the management and outcomes of RIAS in a large single-institution series. METHODS: All patients diagnosed with RIAS between January 2000 and January 2014 were identified from an institutional database. RESULTS: A total of 49 patients were identified. Median age at diagnosis was 72 years (range 51-93). Median time from completion of radiotherapy to diagnosis of RIAS was 7.5 years. Median tumour size at presentation was 5.0 cm (1.5-19.0). The majority of patients presented with localised disease (47, 95.9%). Of these, 35 (74.5%) were suitable for surgery and underwent surgery with curative intent. Twelve patients presented with localised irresectable disease. Of these, 7 received systemic chemotherapy, with a sufficient response to facilitate surgery in 3 patients. Following potentially curative surgery, 2-year local recurrence-free was 55.2%. Survival was significantly prolonged in patients presenting with resectable disease (2-year overall survival 71.1% vs 33.3%, p < 0.001). Tumour size >5 cm was prognostic of distant metastases-free survival and overall survival. CONCLUSION: RIAS are rare, aggressive soft-tissue lesions with limited treatment options and high-rates of both local and systemic relapse.
RESUMEN
Sarcomas are a complex group of childhood and adult neoplasms with differentiation towards mesenchymal tissues that can occur at almost every anatomic site. Although pathologically diverse, they frequently show similar clinical presentations and radiological findings, such that correct histopathologic diagnosis, utilising the appropriate ancillary immunohistochemical and molecular techniques, underpins their management. This article gives an overview of the pathology, coupled with recent advances in molecular biology, of a selection of soft tissue sarcomas from a clinicopathological perspective, discussing histopathological diagnosis with developments in molecular diagnosis and the incorporation of these findings into diagnostic practice and current and potential targeted treatments.
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Sarcoma/patología , Sarcoma/terapia , Adulto , HumanosRESUMEN
INTRODUCTION: The aim of this study was to investigate if defective repair of DNA double-strand break (DSB) in head and neck squamous cell carcinoma (HNSCC) could be used as an early predictor of treatment response. METHODS: Tumour biopsy 24-36 h following induction chemotherapy (IC) and pre-treatment biopsies were stained for RAD51 and geminin (S-phase marker) for immunofluorescence in patients with HNSCC. The difference between RAD51 score (percentage of geminin-positive cells that were also positive for RAD51) was calculated for the two specimens. Tumours with a percentage difference of⩽10% were deemed to have repaired IC-induced DSBs, and were classified as 'RAD51 negative'. Response at 3 months post treatment and human papilloma virus (HPV) status were assessed. RESULTS: Thirteen pairs of samples were available for analyses. Three samples were classified as RAD51 negative and 10 as RAD51 positive at 24 h post IC. All of the three patients with tumours classified as RAD51 negative had partial response or progressive disease and the 10 patients with tumours deemed RAD51 positive had a complete response. 100% of the HPV-positive tumours were RAD51 positive and had a complete response. CONCLUSIONS: We have demonstrated that impaired DSB DNA repair may underlie enhanced treatment sensitivity of HPV-positive HNSCC and repair capacity following platinum-induced DNA damage predicts response in HNSCC. This has potential as a biomarker for patient selection in trials of DNA damage response pathway modulation.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Carcinoma de Células Escamosas/terapia , Roturas del ADN de Doble Cadena , Reparación del ADN , Neoplasias Orofaríngeas/terapia , Antineoplásicos Alquilantes/administración & dosificación , Antineoplásicos Alquilantes/farmacología , Carcinoma de Células Escamosas/genética , Quimioradioterapia , ADN de Neoplasias/efectos de los fármacos , ADN de Neoplasias/genética , Humanos , Proteínas de Neoplasias/genética , Proteínas de Neoplasias/metabolismo , Compuestos Organoplatinos/administración & dosificación , Compuestos Organoplatinos/farmacología , Neoplasias Orofaríngeas/genética , Papillomaviridae , Infecciones por Papillomavirus/genética , Infecciones por Papillomavirus/terapia , Proyectos Piloto , Recombinasa Rad51/genética , Recombinasa Rad51/metabolismo , Fase S/efectos de los fármacos , Resultado del TratamientoRESUMEN
AIMS: To determine the clinical outcomes of an intensity-modulated radiotherapy technique for total mucosal irradiation (TM-IMRT) in patients with head and neck carcinoma of unknown primary (HNCUP). MATERIALS AND METHODS: A single-centre prospective phase II trial design was used in two sequential studies to evaluate TM-IMRT for HNCUP. Patients were investigated for primary tumour site using examination under anaesthetic and biopsies, computed tomography ± magnetic resonance imaging (MRI) or 18-fluorodeoxyglucose positron emission tomography-computed tomography (PET-CT). Patients received IMRT to the potential primary tumour sites and elective cervical nodes. Concomitant chemotherapy was used in patients who received primary radiotherapy or those with nodal extracapsular extension. RESULTS: Thirty-six patients with HNCUP were recruited; 72% male. Twenty-five patients (69.4%) had p16-positive disease. Two year mucosal and local nodal control rates were 97.1% (95% confidence interval 91.4-100) and 89.8% (78.4-100), respectively. One mucosal primary was detected 7.3 months after TM-IMRT and three patients died from recurrent/metastatic squamous cell carcinoma of the head and neck. Twelve patients (33%) developed grade 3 (Late Effects in Normal Tissue-Subjective, Objective, Management and Analytical; LENT-SOMA) dysphagia with a 1 year enteric tube feeding rate of 2.7%. The high-grade subjective xerostomia rate (LENT-SOMA) at 24 months after IMRT was 15%. CONCLUSIONS: At a median follow-up of 36.1 months, the use of TM-IMRT was associated with good local control. Toxicity was comparable with previously reported TM-IMRT regimens encompassing similar mucosal volumes.
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Carcinoma de Células Escamosas/radioterapia , Neoplasias de Cabeza y Cuello/radioterapia , Neoplasias Primarias Desconocidas/radioterapia , Radioterapia de Intensidad Modulada/métodos , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Membrana Mucosa/efectos de la radiación , Estudios Prospectivos , Dosificación Radioterapéutica , Carcinoma de Células Escamosas de Cabeza y Cuello , Tomografía Computarizada por Rayos X , Xerostomía/etiologíaRESUMEN
INTRODUCTION: Elastofibromas are rare, pseudo-tumours arising at the inferior pole of the scapula that have a characteristic presentation. Due to their tissue of origin and size, they may often be mistaken for soft tissue sarcomas. We present the management of patients diagnosed with elastofibroma at a single institution. METHODS: Patients diagnosed with elastofibroma between January 1995 and January 2015 were identified from a prospectively maintained histopathology database. Electronic patient records, imaging and pathology reports were retrieved and reviewed. RESULTS: Thirty seven patients were identified, with a median age of 66 years and a male-to-female ratio of 1:1.6. All tumours occurred in the characteristic subscapular location. The median maximum tumour diameter was 8.2 cm. A synchronous contralateral lesion (15.8%) was found in six patients. Cross-sectional imaging was performed in 29 patients, with magnetic resonance imaging the most common modality (59.5%). Diagnosis was confirmed with percutaneous biopsy in all but one patient, who proceeded directly to surgery. Eighteen patients were managed non-operatively; 19 opted for surgical excision due to significant symptoms. Excision was performed in a marginal fashion and, at a median follow-up of 5 months, no functional impairment or local recurrences were observed. CONCLUSIONS: Soft tissue masses greater than 5 cm in diameter should prompt the clinician to exclude soft tissue sarcoma. The diagnosis of elastofibroma may be alluded to by its typical presentation and can be confirmed by percutaneous biopsy. After excluding malignancy, these lesions can be safely managed non-operatively, with surgery reserved for symptomatic patients.
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Fibroma , Escápula , Neoplasias de los Tejidos Blandos , Anciano , Anciano de 80 o más Años , Femenino , Fibroma/diagnóstico , Fibroma/epidemiología , Fibroma/patología , Fibroma/terapia , Humanos , Masculino , Persona de Mediana Edad , Radiografía , Estudios Retrospectivos , Escápula/diagnóstico por imagen , Escápula/patología , Escápula/fisiopatología , Neoplasias de los Tejidos Blandos/diagnóstico , Neoplasias de los Tejidos Blandos/epidemiología , Neoplasias de los Tejidos Blandos/patología , Neoplasias de los Tejidos Blandos/terapiaRESUMEN
Desmoid-type fibromatosis is a rare, locally infiltrative, mesenchymal neoplasm that is associated with high rates of local recurrence but lacks the potential to metastasise. The disease affects younger individuals, with a peak age of 30 years, and is the most common cause of an anterior abdominal wall mass in young women of childbearing age. It may, however, involve nearly every body part, including the extremities, head and neck, trunk, and abdominal cavity; as such, desmoid-type fibromatosis may present to a range of general and subspecialty radiologists. These rare tumours have a widely variable clinical presentation and unpredictable natural history, hence input from a soft-tissue tumour centre is recommended, although much of the imaging may be performed at the patient's local hospital. The consensus for treatment has changed over the past decade, with most centres moving away from primary radical surgery towards a front-line 'watch-and-wait' policy. Therefore, imaging has an increasingly important role to play in both the diagnosis and follow-up of these patients. This review will discuss the typical imaging characteristics of these lesions and suggest diagnostic and follow-up magnetic resonance imaging protocols, with details of suitable sequences and scanning intervals.
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Diagnóstico por Imagen , Fibromatosis Agresiva/diagnóstico , Medios de Contraste , Diagnóstico Diferencial , Progresión de la Enfermedad , Fibromatosis Agresiva/patología , Fibromatosis Agresiva/terapia , Humanos , Recurrencia Local de NeoplasiaRESUMEN
PURPOSE: To determine the feasibility of induction chemotherapy and chemo-IMRT in head and neck squamous cell cancers at risk of bilateral nodal spread (midline tumours) and to evaluate whether bilateral superficial lobe parotid-sparing IMRT can reduce the incidence of ⩾G2 subjective xerostomia. METHODS: Patients with midline tumours were enrolled to a phase II trial to receive induction platinum/5-fluorouracil and concomitant platinum with combined superficial lobe parotid-sparing IMRT. The primary site and involved nodal levels received 65 Gy in 30 fractions (f) and at risk nodal levels, 54 Gy/30f. Incidence of ⩾G2 subjective xerostomia was defined as the primary endpoint. Secondary endpoints included incidences of acute and late toxicities and survival outcomes dependent on human papilloma virus (HPV) status. RESULTS: One hundred and twenty patients with midline cancers completed treatment between December 2005 and May 2010 with median follow-up of 50 months. Incidences of ⩾G2 acute toxicities were: dysphagia 75%; xerostomia 65%; mucositis 86%; pain 83%; and fatigue 64%. At 12 months, ⩾G2 subjective xerostomia was observed in 21% (17% in HPV +ve). Two-year loco-regional progression-free survival (PFS) was 90.7% (95% CI: 85.2-96.2). According to HPV status, there was a significant difference for 2-year loco-regional PFS, 76.8% (HPV-negative) vs 98.6% (HPV-positive), P=0.001. 2-year overall survival was 93% for HPV-positive compared with 52% for HPV-negative cases, P<0.001. CONCLUSIONS: Sequential chemotherapy/chemo-IMRT for midline tumours is feasible, with excellent survival outcomes. At 1 year, 21% experience ⩾G2 subjective xerostomia. Two-year survival outcomes differ significantly between HPV-positive and HPV-negative disease, suggesting development of different treatment schedules for the different disease entities.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Carcinoma de Células Escamosas/tratamiento farmacológico , Carcinoma de Células Escamosas/radioterapia , Neoplasias de Cabeza y Cuello/tratamiento farmacológico , Neoplasias de Cabeza y Cuello/radioterapia , Glándula Parótida/efectos de la radiación , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Preescolar , Supervivencia sin Enfermedad , Femenino , Humanos , Quimioterapia de Inducción , Masculino , Persona de Mediana Edad , Glándula Parótida/diagnóstico por imagen , Estudios Prospectivos , Traumatismos por Radiación/etiología , Traumatismos por Radiación/prevención & control , Carcinoma de Células Escamosas de Cabeza y Cuello , Resultado del Tratamiento , Ultrasonografía , Xerostomía/etiología , Adulto JovenRESUMEN
Masses involving the abdominal wall arise from a large number of aetiologies. This article will describe a diagnostic approach, imaging features of the most common causes of abdominal wall masses, and highly specific characteristics of less common diseases. A diagnostic algorithm for abdominal wall masses combines clinical history and imaging appearances to classify lesions.
Asunto(s)
Neoplasias Abdominales/diagnóstico , Pared Abdominal/patología , Diagnóstico por Imagen/métodos , Neoplasias de los Tejidos Blandos/diagnóstico , Medios de Contraste , Diagnóstico por Imagen/normas , Femenino , Humanos , Aumento de la Imagen/métodos , Imagen por Resonancia Magnética/métodos , Anamnesis , Guías de Práctica Clínica como Asunto , Radiografía Abdominal/métodos , Tomografía Computarizada por Rayos X/métodosRESUMEN
Teratomas comprise a spectrum of tumours that have striking imaging appearances and are commonly considered when evaluating a mass in the female pelvis. A subgroup of these tumours located in an extragonadal abdominopelvic location, in contrast, are extremely rare and can affect both sexes. Extragonadal teratomas can occur at all ages, are particularly unusual in adults and can cause confusion in the differential diagnosis, especially in children. Familiarity with the imaging features of the spectrum of teratomas within the abdominal cavity is therefore of great importance, as radiological diagnosis can guide treatment, prevent delays in diagnosis and avoid sequelae. This article summarizes the radiological appearances of these rare extragonadal tumours in adults in relation to their pathology, malignant potential, location and behaviour. Although uncommon, teratomas should be considered in the differential diagnosis of extragonadal abdominal masses, particularly in young adults.
Asunto(s)
Neoplasias Abdominales/diagnóstico , Neoplasias Pélvicas/diagnóstico , Teratoma/diagnóstico , Neoplasias Abdominales/clasificación , Neoplasias Abdominales/patología , Adulto , Niño , Medios de Contraste , Diagnóstico Diferencial , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Neoplasias Pélvicas/clasificación , Neoplasias Pélvicas/patología , Teratoma/clasificación , Teratoma/patologíaRESUMEN
BACKGROUND: Carboplatin can be substituted for cisplatin in concomitant chemoradiation (CRT) for locally advanced squamous cell carcinoma of the head and neck (LASCCHN) when the latter is contraindicated. This matched-pair study aimed to compare the efficacy and acute toxicity of carboplatin and cisplatin. METHODS: Patients treated with 2 cycles of concomitant carboplatin-based CRT were matched to patients treated with 2 cycles of cisplatin. Matching criteria included age, tumour site, stage, smoking status and use of induction chemotherapy. Radiation was delivered using conformal techniques. Data on weekly acute toxicity throughout CRT was compared using the chi-squared test for proportions. Kaplan Meier statistics described time to local relapse, distant relapse and overall survival, the log-rank test was used to compare 3-year survival outcomes. RESULTS: Sixty-five patients who received carboplatin were matched to 65 who received cisplatin. Significant differences in toxicity included increased emesis with cisplatin and more anaemia and thrombocytopenia with carboplatin. There was no significant difference in 3-year locoregional control (87% vs. 79%, p=0.54), freedom from distant metastases (88% vs. 85%, p=0.79) and overall survival (59% vs. 68%, p=0.24) between the carboplatin and cisplatin cohorts, respectively. CONCLUSIONS: When cisplatin is contraindicated, carboplatin-based CRT yields equivalent treatment outcomes in patients with LASCCHN.