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1.
Can Vet J ; 65(10): 1061-1070, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-39355701

RESUMEN

Objectives: To evaluate whether patient factors affect development of non-pulmonary soft-tissue metastases following treatment of canine appendicular osteosarcoma and to report and compare outcomes to those in dogs with pulmonary or osseous metastases. Animals and procedure: The records of 3 veterinary teaching hospitals were reviewed to identify dogs that received definitive treatment for a primary appendicular osteosarcoma lesion and chemotherapy between January 2010 and June 2022. Dogs with non-pulmonary metastases following initial treatment were included. Descriptive statistics were calculated to summarize signalment information, and metastasis and survival times were compared between groups using Kaplan-Meier survival analysis and log-rank tests. Results: Thirty-six and 109 dogs developed non-pulmonary soft-tissue metastases and pulmonary or osseous metastases, respectively, following initial treatment. No patient factors were significantly associated with development of non-pulmonary soft-tissue metastases. The median times to non-pulmonary soft-tissue metastasis or initial pulmonary or osseous metastasis were 220 and 169 d, respectively (P = 0.18); whereas overall median survival times were 250 and 270 d, respectively (P = 0.36). Conclusion: Dogs with non-pulmonary soft-tissue metastases had similar disease-free intervals and survival rates to dogs with typical pulmonary or osseous metastases.


Le développement de métastases des tissus mous non pulmonaires n'est pas un indicateur de mauvais pronostic chez les chiens atteints d'ostéosarcome appendiculaire métastatique. Objectifs: Évaluer si les facteurs liés au patient affectent le développement de métastases des tissus mous non pulmonaires après le traitement de l'ostéosarcome appendiculaire canin et rapporter et comparer les résultats à ceux des chiens atteints de métastases pulmonaires ou osseuses. Animaux et procédure: Les dossiers de 3 hôpitaux universitaires vétérinaires ont été examinés pour identifier les chiens qui ont reçu un traitement définitif pour une lésion d'ostéosarcome appendiculaire primaire et une chimiothérapie entre janvier 2010 et juin 2022. Les chiens présentant des métastases non pulmonaires après le traitement initial ont été inclus. Des statistiques descriptives ont été calculées pour résumer les informations descriptives, et les métastases et les temps de survie ont été comparés entre les groupes à l'aide d'une analyse de survie de Kaplan-Meier et de tests du rang logarithmique. Résultats: Trente-six et 109 chiens ont développé des métastases des tissus mous non pulmonaires et des métastases pulmonaires ou osseuses, respectivement, après le traitement initial. Aucun facteur lié au patient n'a été significativement associé au développement de métastases des tissus mous non pulmonaires. Les délais médians avant métastases des tissus mous non pulmonaires ou métastases pulmonaires ou osseuses initiales étaient respectivement de 220 et 169 jours (P = 0,18); tandis que les durées médianes de survie globale étaient respectivement de 250 et 270 jours (P = 0,36). Conclusion: Les chiens présentant des métastases des tissus mous non pulmonaires présentaient des intervalles sans maladie et des taux de survie similaires à ceux des chiens présentant des métastases pulmonaires ou osseuses typiques.(Traduit par Dr Serge Messier).


Asunto(s)
Neoplasias Óseas , Enfermedades de los Perros , Osteosarcoma , Neoplasias de los Tejidos Blandos , Animales , Perros , Osteosarcoma/veterinaria , Osteosarcoma/mortalidad , Osteosarcoma/patología , Enfermedades de los Perros/patología , Enfermedades de los Perros/mortalidad , Neoplasias de los Tejidos Blandos/veterinaria , Neoplasias de los Tejidos Blandos/patología , Neoplasias de los Tejidos Blandos/mortalidad , Masculino , Neoplasias Óseas/veterinaria , Neoplasias Óseas/secundario , Neoplasias Óseas/mortalidad , Femenino , Pronóstico , Estudios Retrospectivos , Neoplasias Pulmonares/veterinaria , Neoplasias Pulmonares/secundario , Neoplasias Pulmonares/mortalidad , Neoplasias Pulmonares/patología
3.
Zhonghua Zhong Liu Za Zhi ; 46(9): 855-861, 2024 Sep 23.
Artículo en Chino | MEDLINE | ID: mdl-39293988

RESUMEN

Bone and soft tissue tumors occur in the musculoskeletal system, and malignant bone tumors of bone and soft tissue account for 0.2% of all human malignant tumors, and if not diagnosed and treated in a timely manner, patients may be at risk of a poor prognosis. Image interpretation plays an increasingly important role in the diagnosis of bone and soft tissue tumors. Artificial intelligence (AI) can be applied in clinical treatment to integrate large amounts of multidimensional data, derive models, predict outcomes, and improve treatment decisions. Among these methods, deep learning is a widely employed technique in AI that predominantly utilizes convolutional neural networks (CNN). The network is implemented through repeated training of datasets and iterative parameter adjustments. Deep learning-based AI models have successfully been applied to various aspects of bone and soft tissue tumors, encompassing but not limiting in image segmentation, tumor detection, classification, grading and staging, chemotherapy effect evaluation, recurrence and prognosis prediction. This paper provides a comprehensive review of the principles and current state of AI in the medical image diagnosis and treatment of bone and soft tissue tumors. Additionally, it explores the present challenges and future prospects in this field.


Asunto(s)
Inteligencia Artificial , Neoplasias Óseas , Redes Neurales de la Computación , Neoplasias de los Tejidos Blandos , Humanos , Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/terapia , Neoplasias Óseas/diagnóstico , Neoplasias de los Tejidos Blandos/diagnóstico por imagen , Neoplasias de los Tejidos Blandos/terapia , Neoplasias de los Tejidos Blandos/diagnóstico , Aprendizaje Profundo , Pronóstico , Interpretación de Imagen Asistida por Computador/métodos
4.
Eur J Cancer ; 210: 114269, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-39226665

RESUMEN

INTRODUCTION: Risk prediction models (RPM) can help soft-tissue sarcoma(STS) patients and clinicians make informed treatment decisions by providing them with estimates of (disease-free) survival for different treatment options. However, it is unknown how RPMs are used in the clinical encounter to support decision-making. This study aimed to understand how a PERsonalised SARcoma Care (PERSARC) RPM is used to support treatment decisions and which barriers and facilitators influence its use in daily clinical practice. METHODS: A convergent mixed-methods design is used to understand how PERSARC is integrated in the clinical encounter in three Dutch sarcoma centers. Data were collected using qualitative interviews with STS patients (n = 15) and clinicians (n = 8), quantitative surveys (n = 50) and audiotaped consultations (n = 30). Qualitative data were analyzed using thematic analysis and integrated with quantitative data through merging guided by the SEIPS model. RESULTS: PERSARC was generally used to support clinicians' proposed treatment plan and not to help patients weigh available treatment options. Use of PERSARC in decision-making was hampered by clinician's doubts about whether there were multiple viable treatment options,the accuracy of risk estimates, and time constraints. On the other hand, use of PERSARC facilitated clinicians to estimate and communicate the expected benefit of adjuvant therapy to patients. CONCLUSION: PERSARC was not used to support informed treatment decision-making in STS patients. Integrating RPMs into clinical consultations requires acknowledgement of their benefits in facilitating clinicians' estimation of the expected benefit of adjuvant therapies and information provision to patients, while also considering concerns regarding RPM quality and treatment options' viability.


Asunto(s)
Medicina de Precisión , Sarcoma , Humanos , Sarcoma/terapia , Masculino , Femenino , Persona de Mediana Edad , Adulto , Medición de Riesgo , Anciano , Medicina de Precisión/métodos , Toma de Decisiones Clínicas , Técnicas de Apoyo para la Decisión , Países Bajos , Neoplasias de los Tejidos Blandos/terapia , Investigación Cualitativa , Adulto Joven
5.
Int J Hyperthermia ; 41(1): 2405105, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39307528

RESUMEN

INTRODUCTION: This study evaluated the performance of magnetic resonance thermometry (MRT) during deep-regional hyperthermia (HT) in pelvic and lower-extremity soft-tissue sarcomas. MATERIALS AND METHODS: 17 pelvic (45 treatments) and 16 lower-extremity (42 treatments) patients underwent standard regional HT and chemotherapy. Pairs of double-echo gradient-echo scans were acquired during the MR protocol 1.4 s apart. For each pair, precision was quantified using phase data from both echoes ('dual-echo') or only one ('single-echo') in- or excluding body fat pixels in the field drift correction region of interest. The precision of each method was compared to that of the MRT approach using a built-in clinical software tool (SigmaVision). Accuracy was assessed in three lower-extremity patients (six treatments) using interstitial temperature probes. The Jaccard coefficient quantified pretreatment motion; receiver operating characteristic analysis assessed its predictability for acceptable precision (<1 °C) during HT. RESULTS: Compared to the built-in dual-echo approach, single-echo thermometry improved the mean temporal precision from 1.32 ± 0.40 °C to 1.07 ± 0.34 °C (pelvis) and from 0.99 ± 0.28 °C to 0.76 ± 0.23 °C (lower extremities). With body fat-based field drift correction, single-echo mean accuracy improved from 1.4 °C to 1.0 °C. Pretreatment bulk motion provided excellent precision prediction with an area under the curve of 0.80-0.86 (pelvis) and 0.81-0.83 (lower extremities), compared to gastrointestinal air motion (0.52-0.58). CONCLUSION: Single-echo MRT exhibited better precision than dual-echo MRT. Body fat-based field-drift correction significantly improved MRT accuracy. Pretreatment bulk motion showed improved prediction of acceptable MRT temporal precision over gastrointestinal air motion.


Asunto(s)
Hipertermia Inducida , Imagen por Resonancia Magnética , Sarcoma , Termometría , Humanos , Hipertermia Inducida/métodos , Sarcoma/terapia , Sarcoma/diagnóstico por imagen , Femenino , Masculino , Imagen por Resonancia Magnética/métodos , Persona de Mediana Edad , Termometría/métodos , Adulto , Anciano , Extremidad Inferior/fisiopatología , Extremidad Inferior/diagnóstico por imagen , Pelvis/diagnóstico por imagen , Neoplasias de los Tejidos Blandos/terapia , Neoplasias de los Tejidos Blandos/diagnóstico por imagen
6.
N Engl J Med ; 391(9): 789-799, 2024 Sep 05.
Artículo en Inglés | MEDLINE | ID: mdl-39231341

RESUMEN

BACKGROUND: The addition of trabectedin to doxorubicin, followed by trabectedin maintenance, may have superior efficacy to doxorubicin alone as first-line treatment in patients with advanced leiomyosarcoma. METHODS: We conducted a phase 3 trial involving patients with metastatic or unresectable leiomyosarcoma who had not received chemotherapy previously. Patients were randomly assigned to receive either single-agent doxorubicin (six cycles) or doxorubicin plus trabectedin (six cycles), with continued trabectedin as maintenance therapy in patients in the doxorubicin-trabectedin group who did not have disease progression. Surgery to resect residual disease was allowed in each group after six cycles of therapy. Analyses of progression-free survival (primary end point) and overall survival (secondary end point) were adjusted for two stratification factors: tumor origin site (uterine vs. soft tissue) and disease stage (locally advanced vs. metastatic). The primary end-point results were reported previously. RESULTS: A total of 150 patients underwent randomization. At a median follow-up of 55 months (interquartile range, 49 to 63), a total of 107 patients had died (47 in the doxorubicin-trabectedin group and 60 in the doxorubicin group). The median overall survival was longer in the doxorubicin-trabectedin group (33 months; 95% confidence interval [CI], 26 to 48) than in the doxorubicin group (24 months; 95% CI, 19 to 31); the adjusted hazard ratio for death was 0.65 (95% CI, 0.44 to 0.95). In a finding consistent with earlier reports, progression-free survival was longer in the doxorubicin-trabectedin group (12 months; 95% CI, 10 to 16) than in the doxorubicin group (6 months; 95% CI, 4 to 7); the adjusted hazard ratio for progression or death was 0.37 (95% CI, 0.26 to 0.53). The incidence of adverse events and the percentage of patients with dose reductions were higher with doxorubicin plus trabectedin than with doxorubicin alone. CONCLUSIONS: Combination therapy with doxorubicin and trabectedin induction, followed by trabectedin maintenance, was associated with improved overall survival and progression-free survival, as compared with doxorubicin alone, among patients with metastatic or surgically unresectable uterine or soft-tissue leiomyosarcoma. (Funded by PharmaMar and others; LMS04 ClinicalTrials.gov number, NCT02997358.).


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica , Doxorrubicina , Leiomiosarcoma , Neoplasias de los Tejidos Blandos , Trabectedina , Neoplasias Uterinas , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Doxorrubicina/administración & dosificación , Doxorrubicina/efectos adversos , Estimación de Kaplan-Meier , Leiomiosarcoma/tratamiento farmacológico , Leiomiosarcoma/mortalidad , Leiomiosarcoma/patología , Quimioterapia de Mantención , Supervivencia sin Progresión , Neoplasias de los Tejidos Blandos/tratamiento farmacológico , Neoplasias de los Tejidos Blandos/mortalidad , Neoplasias de los Tejidos Blandos/patología , Análisis de Supervivencia , Trabectedina/administración & dosificación , Trabectedina/efectos adversos , Neoplasias Uterinas/tratamiento farmacológico , Neoplasias Uterinas/mortalidad , Neoplasias Uterinas/patología , Estadificación de Neoplasias
7.
Cancer Imaging ; 24(1): 119, 2024 Sep 05.
Artículo en Inglés | MEDLINE | ID: mdl-39238054

RESUMEN

PURPOSE: To investigate the value of multi-parametric MRI-based radiomics for preoperative prediction of lung metastases from soft tissue sarcoma (STS). METHODS: In total, 122 patients with clinicopathologically confirmed STS who underwent pretreatment T1-weighted contrast-enhanced (T1-CE) and T2-weighted fat-suppressed (T2FS) MRI scans were enrolled between Jul. 2017 and Mar. 2021. Radiomics signatures were established by calculating and selecting radiomics features from the two sequences. Clinical independent predictors were evaluated by statistical analysis. The radiomics nomogram was constructed from margin and radiomics features by multivariable logistic regression. Finally, the study used receiver operating characteristic (ROC) and calibration curves to evaluate performance of radiomics models. Decision curve analyses (DCA) were performed to evaluate clinical usefulness of the models. RESULTS: The margin was considered as an independent predictor (p < 0.05). A total of 4 MRI features were selected and used to develop the radiomics signature. By incorporating the margin and radiomics signature, the developed nomogram showed the best prediction performance in the training (AUCs, margin vs. radiomics signature vs. nomogram, 0.609 vs. 0.909 vs. 0.910) and validation (AUCs, margin vs. radiomics signature vs. nomogram, 0.666 vs. 0.841 vs. 0.894) cohorts. DCA indicated potential usefulness of the nomogram model. CONCLUSIONS: This feasibility study evaluated predictive values of multi-parametric MRI for the prediction of lung metastasis, and proposed a nomogram model to potentially facilitate the individualized treatment decision-making for STSs.


Asunto(s)
Estudios de Factibilidad , Neoplasias Pulmonares , Nomogramas , Sarcoma , Humanos , Femenino , Neoplasias Pulmonares/diagnóstico por imagen , Neoplasias Pulmonares/patología , Neoplasias Pulmonares/secundario , Masculino , Sarcoma/diagnóstico por imagen , Sarcoma/secundario , Sarcoma/patología , Persona de Mediana Edad , Adulto , Anciano , Estudios Retrospectivos , Imágenes de Resonancia Magnética Multiparamétrica/métodos , Imagen por Resonancia Magnética/métodos , Adulto Joven , Curva ROC , Neoplasias de los Tejidos Blandos/diagnóstico por imagen , Neoplasias de los Tejidos Blandos/secundario , Neoplasias de los Tejidos Blandos/patología , Radiómica
8.
JAMA Netw Open ; 7(9): e2431530, 2024 Sep 03.
Artículo en Inglés | MEDLINE | ID: mdl-39230901

RESUMEN

Importance: Improved prognostic tools are needed for patients with locally recurrent extremity or truncal soft tissue sarcoma (STS). Objective: To examine the association between average local recurrence (LR) growth rate and outcomes following resection of locally recurrent extremity or truncal STS. Design, Setting, and Participants: This retrospective cohort study used a prospectively maintained database from a single high-volume tertiary sarcoma referral center in the US to identify patients 16 years of age or older who underwent repeat resection of a locally recurrent extremity or truncal STS between July 1, 1982, and December 31, 2021. Patients with atypical lipomatous tumors, desmoid tumors, dermatofibrosarcoma protuberans, angiosarcomas, and prior or synchronous distant recurrence were excluded. Data were analyzed from November 1, 2022, to June 17, 2024. Exposure: Average LR growth rate, defined as the sum of recurrent tumor maximal diameters divided by the disease-free interval after index operation. Main Outcomes and Measures: The primary outcomes were cumulative incidences of disease-specific death (DSD), with death from other causes as a competing risk, and second LR, with death from any cause as a competing risk. Results: The study cohort included 253 patients (median [IQR] age, 64 [51-73] years; 140 [55.3%] male). The 5-year cumulative incidence of DSD after repeat resection was 29%. Multivariable analysis indicated that LR growth rate (hazard ratio [HR], 1.12 [95% CI, 1.08-1.18]; P < .001), younger age (HR, 0.98 [95% CI, 0.97-0.99]; P = .002), R1 or R2 margins (HR, 1.71 [95% CI, 1.03-2.84]; P = .04), high LR grade (HR, 2.90 [95% CI, 1.17-7.20]; P = .02), and multifocality (HR, 2.92 [95% CI, 1.70-5.00]; P < .001) were independently associated with higher incidence of DSD. Using the minimum P value method, the optimal cutoff for growth rate was found to be 0.68 cm/mo. Patients with values above this cutoff had higher 5-year incidences of DSD following repeat resection (63% vs 19%; permutation test P < .001) and higher amputation rates (19% vs 7%; P = .008). Only R1 margins were independently associated with higher incidence of second LR (HR, 1.81 [95% CI, 1.19-2.78]; P = .006). Conclusions and Relevance: In this cohort study of patients undergoing resection of a locally recurrent extremity or truncal STS, LR growth rate was independently associated with DSD. These findings suggest that patients with growth rates higher than 0.68 cm/mo who undergo LR resection may have high disease-specific mortality and amputation rates and should be considered for perioperative systemic therapy.


Asunto(s)
Extremidades , Recurrencia Local de Neoplasia , Sarcoma , Humanos , Masculino , Femenino , Persona de Mediana Edad , Recurrencia Local de Neoplasia/epidemiología , Recurrencia Local de Neoplasia/cirugía , Anciano , Sarcoma/cirugía , Sarcoma/mortalidad , Sarcoma/patología , Estudios Retrospectivos , Extremidades/cirugía , Pronóstico , Torso/cirugía , Neoplasias de los Tejidos Blandos/cirugía , Neoplasias de los Tejidos Blandos/mortalidad , Neoplasias de los Tejidos Blandos/patología
9.
Sci Rep ; 14(1): 20627, 2024 09 04.
Artículo en Inglés | MEDLINE | ID: mdl-39232087

RESUMEN

The coronavirus disease (COVID-19) pandemic negatively affected the diagnosis and treatment of several cancer types. However, this pandemic's exact impact and extent on bone and soft tissue sarcomas need to be clarified. We aimed to investigate the effect of the COVID-19 pandemic and emergency declaration by the local government on consultation behavior and clinical stage at diagnosis of bone and soft tissue sarcoma. A total of 403 patients diagnosed with bone and soft tissue sarcoma who initially visited three sarcoma treatment hospitals between January 2018 and December 2021 were included. The monthly number of newly diagnosed soft tissue sarcoma patients was reduced by 25%, and the proportion of soft tissue patients with stage IV disease at diagnosis significantly increased by 9% during the COVID-19 pandemic compared to before the COVID-19 pandemic. Furthermore, the monthly number of new primary bone and soft tissue sarcoma patients significantly decreased by 43% during the state of emergency declaration. The COVID-19 pandemic had a negative impact on soft tissue sarcoma patients' consultation behavior and increased the proportion of advanced-stage patients at initial diagnosis. An emergency declaration by the local government also negatively affected primary bone and soft tissue sarcoma patients' consultation behavior.


Asunto(s)
Neoplasias Óseas , COVID-19 , Derivación y Consulta , Sarcoma , Humanos , COVID-19/epidemiología , COVID-19/diagnóstico , Sarcoma/diagnóstico , Sarcoma/epidemiología , Sarcoma/terapia , Masculino , Femenino , Persona de Mediana Edad , Neoplasias Óseas/diagnóstico , Neoplasias Óseas/epidemiología , Adulto , Anciano , Neoplasias de los Tejidos Blandos/diagnóstico , Neoplasias de los Tejidos Blandos/epidemiología , Neoplasias de los Tejidos Blandos/terapia , Pandemias , SARS-CoV-2/aislamiento & purificación , Estadificación de Neoplasias
10.
Artículo en Inglés | MEDLINE | ID: mdl-39240761

RESUMEN

Tenosynovial giant cell tumor (TGCT) is a rare type of neoplasm that may be locally aggressive but is most often benign and can be divided into two subtypes: localized and diffuse. It tends to develop in the joints, bursae, and tendon sheaths primarily in the digits of the hand and less commonly in the forefoot. This soft-tissue mass has many possible differential diagnoses, including lipoma, ganglion cyst, plantar fibroma, and various sarcomas; surgical excision is usually indicated to reach a definitive diagnosis and rule out malignancy. We report a rare case of a 30-year-old woman with atypical plantar hallucal pain and a palpable mass on the plantar lateral aspect of the left hallux. Surgical excision and histopathologic evaluation confirmed a TGCT of the left hallucal flexor tendon sheath. Although it bears clinical resemblance to several other soft-tissue masses, TGCT has numerous pathognomonic features evident with advanced imaging and histologic analysis that help the physician obtain an accurate diagnosis and proceed with appropriate treatment.


Asunto(s)
Tumor de Células Gigantes de las Vainas Tendinosas , Tendones , Humanos , Femenino , Adulto , Tumor de Células Gigantes de las Vainas Tendinosas/patología , Tumor de Células Gigantes de las Vainas Tendinosas/cirugía , Tumor de Células Gigantes de las Vainas Tendinosas/diagnóstico , Tumor de Células Gigantes de las Vainas Tendinosas/diagnóstico por imagen , Tendones/patología , Tendones/cirugía , Neoplasias de los Tejidos Blandos/cirugía , Neoplasias de los Tejidos Blandos/patología , Neoplasias de los Tejidos Blandos/diagnóstico , Hallux/patología , Imagen por Resonancia Magnética , Diagnóstico Diferencial
11.
JBJS Case Connect ; 14(3)2024 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-39241104

RESUMEN

CASE: This case report describes a patient who presented with clinical and radiographic features of a soft tissue sarcoma of the shoulder. Despite having a painless and relatively large mass, a biopsy and resection revealed nodular fasciitis (NF). CONCLUSION: This is an unusual case of a painless 10 cm mass that histopathologically was diagnosed as NF in the upper extremity with proximity to the axillary nerve and posterior humeral circumflex vessels. The USP6 rearrangement was helpful in confirming the diagnosis. Careful clinical, radiographic, and pathologic correlation is necessary in diagnosing these relatively rare tumors. In cases where there are discordant findings, molecular markers can be very helpful.


Asunto(s)
Fascitis , Sarcoma , Hombro , Humanos , Fascitis/diagnóstico por imagen , Fascitis/patología , Sarcoma/diagnóstico por imagen , Sarcoma/patología , Sarcoma/cirugía , Hombro/diagnóstico por imagen , Hombro/patología , Neoplasias de los Tejidos Blandos/diagnóstico por imagen , Neoplasias de los Tejidos Blandos/patología , Neoplasias de los Tejidos Blandos/cirugía , Masculino , Femenino , Diagnóstico Diferencial
12.
ESMO Open ; 9(9): 103689, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-39265219

RESUMEN

BACKGROUND: To present findings from a retrospective study conducted by the Ultra-Rare Sarcoma Working Group on metastatic low-grade fibromyxoid sarcoma (LGFMS), sclerosing epithelioid fibrosarcoma (SEF), and hybrid (H)-LGFMS/SEF across 28 global centres. METHODS: Patients treated at participating institutions from January 2000 to September 2022 were retrospectively selected. Diagnosis was confirmed by expert pathologists. Primary endpoint was progression-free survival (PFS-1) from metastasis detection to first progression or death. PFS-2 was calculated from therapy initiation. RESULTS: A total of 101 patients were identified (32 LGFMS, 50 SEF, 19 H-LGFMS/SEF). Median (m) follow-up was 62.1 months. mPFS-1 was 28.7, 11.8, and 20.3 months for LGFMS, SEF, and H-LGFMS/SEF, respectively. mOS was 145.8, 41.9, and 113.5 months, respectively. Treatments included anthracycline-based chemotherapy, gemcitabine-based chemotherapy (G), pazopanib, trabectedin, others. mPFS-2 was: 20.1, 5.5, and 3.5 months in H-LGFMS/SEF, SEF, and LGFMS, respectively, with anthracyclines; 19.5, 7.7, and 6.9 months in LGFMS, SEF, and H-LGFMS/SEF, respectively, with pazopanib; 12.0, 9.7, and 3.1 months in H-LGFMS/SEF, LGFMS, and SEF, respectively. Occasional responses occurred with ifosfamide/oral cyclophosphamide, and prolonged stable disease with immune checkpoint inhibitors. CONCLUSIONS: In this series, the largest available, metastatic LGFMS, SEF, and H-LGFMS/SEF showed different courses. Systemic agents have modest efficacy, informing future trials of novel agents for these tumours.


Asunto(s)
Fibrosarcoma , Humanos , Estudios Retrospectivos , Femenino , Masculino , Fibrosarcoma/tratamiento farmacológico , Persona de Mediana Edad , Adulto , Anciano , Adulto Joven , Pirimidinas/uso terapéutico , Indazoles/uso terapéutico , Gemcitabina , Trabectedina/uso terapéutico , Adolescente , Sulfonamidas/uso terapéutico , Clasificación del Tumor , Antraciclinas/uso terapéutico , Desoxicitidina/análogos & derivados , Desoxicitidina/uso terapéutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias de los Tejidos Blandos/tratamiento farmacológico , Neoplasias de los Tejidos Blandos/patología
13.
J Pathol ; 264(3): 305-317, 2024 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-39344423

RESUMEN

CREB gene family (ATF1, CREB1, CREM) fusions with either EWSR1 or FUS gene partners drive the pathogenesis of a wide range of neoplasms, including various soft tissue tumors, intracranial myxoid mesenchymal tumors (IMMTs), hyalinizing clear cell carcinoma (HCCC), and rare mesotheliomas. Recently, a SMARCA2::CREM fusion was reported in one case each of IMMT and HCCC. In this study, we expand the clinicopathologic and molecular spectrum of these neoplasms by describing three additional cases with SMARCA2::CREM and one with a novel SMARCA4::CREM fusion, highlighting the recurrent potential of additional CREB gene fusion partners beyond FET family members. To evaluate if these fusions define a new pathologic entity, we performed a comprehensive genomic and methylation analysis and compared the results to other related tumors. Tumors occurred in children and young adults (median age 20 years) and spanned a broad anatomic distribution, including soft tissue, intracranial, head and neck, and prostatic urethra. Microscopically, the tumors shared an undifferentiated round to epithelioid cell phenotype and a hyalinized fibrous stroma. Immunohistochemically, a polyphenotypic profile was observed, with variable expression of SOX10, desmin, and/or epithelial markers. No targetable genomic alterations were found using panel-based DNA sequencing. By DNA methylation and transcriptomic analyses, tumors grouped closely to FET::CREB entities, but not with SMARCA4/SMARCB1-deficient tumors. High expression of CREM by immunohistochemistry was also documented in these tumors. Patients experienced local recurrence (n = 2), locoregional lymph node metastases (n = 2), and an isolated visceral metastasis (n = 1). Overall, our study suggests that SMARCA2/4::CREM fusions define a distinct group of neoplasms with round cell to epithelioid histology, a variable immunoprofile, and a definite risk of malignancy. Larger studies are needed to further explore the pathogenetic relationship with the FET::CREB family of tumors. © 2024 The Pathological Society of Great Britain and Ireland.


Asunto(s)
Modulador del Elemento de Respuesta al AMP Cíclico , ADN Helicasas , Proteínas Nucleares , Factores de Transcripción , Humanos , Factores de Transcripción/genética , Factores de Transcripción/metabolismo , Masculino , Modulador del Elemento de Respuesta al AMP Cíclico/genética , Modulador del Elemento de Respuesta al AMP Cíclico/metabolismo , ADN Helicasas/genética , ADN Helicasas/metabolismo , Femenino , Adulto Joven , Proteínas Nucleares/genética , Proteínas Nucleares/metabolismo , Adolescente , Niño , Biomarcadores de Tumor/genética , Biomarcadores de Tumor/metabolismo , Adulto , Proteínas de Fusión Oncogénica/genética , Proteínas de Fusión Oncogénica/metabolismo , Metilación de ADN , Neoplasias/genética , Neoplasias/patología , Neoplasias de los Tejidos Blandos/genética , Neoplasias de los Tejidos Blandos/patología
14.
J Pathol ; 264(3): 293-304, 2024 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-39258383

RESUMEN

Myxofibrosarcoma (MFS) and undifferentiated pleomorphic sarcoma (UPS) are two common and aggressive subtypes of soft tissue sarcoma. The aim of this study was to assess potential transcriptomic differences between MFS and UPS tumours and to evaluate the extent to which differences in gene expression profiles were associated with genomic and clinical features. The study included 162 patients with tumours diagnosed as MFS (N = 62) or UPS (N = 100). The patients had been diagnosed and treated at two Swedish sarcoma centres during a 30-year period. For gene expression profiling and gene fusion detection all tumours were analysed using RNA-sequencing and could be compared with data on clinical outcome (N = 155), global copy number profiles (N = 145), and gene mutations (N = 128). Gene expression profiling revealed three transcriptomic clusters (TCs) without any clear separation of MFS and UPS. One TC was associated with longer metastasis-free survival. These tumours had lower tumour mutation burden (TMB), were enriched for a copy number signature representative of focal LOH and chromosomal instability on a diploid background, and were relatively immune-depleted. MFS and UPS showed extensive genomic overlap, with whole genome doubling occurring more frequently among the latter. The results support the idea that MFS and UPS tumours have largely overlapping genomic and transcriptomic features, with UPS tumours showing more aggressive behaviour and more complex genomes. Independently of the tumour type, clinically relevant subgroups were revealed by gene expression analysis, and the finding of multiple genomic subgroups strongly suggest the existence of subgroups of relevance to treatment stratification. © 2024 The Author(s). The Journal of Pathology published by John Wiley & Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland.


Asunto(s)
Fibrosarcoma , Perfilación de la Expresión Génica , Mutación , Sarcoma , Transcriptoma , Humanos , Masculino , Fibrosarcoma/genética , Fibrosarcoma/patología , Femenino , Persona de Mediana Edad , Anciano , Perfilación de la Expresión Génica/métodos , Sarcoma/genética , Sarcoma/patología , Adulto , Anciano de 80 o más Años , Variaciones en el Número de Copia de ADN , Biomarcadores de Tumor/genética , Neoplasias de los Tejidos Blandos/genética , Neoplasias de los Tejidos Blandos/patología , Regulación Neoplásica de la Expresión Génica , Genómica , Suecia
15.
Microsurgery ; 44(6): e31224, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-39221827

RESUMEN

Soft-tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin and account for only 1% of adult malignancies. They tend to occur most commonly in the lower extremities. Reconstruction after sarcoma resection can be challenging, especially when important structures are involved and recurrences occur. Additionally, more attention is now being paid to reconstructing the lymphatic system to prevent lymphatic complications. In this case report, we presented the management of recurrent medial thigh sarcoma that necessitated multiple challenging reconstructions to provide valuable insights for lectures on similar cases. A 50-year-old male patient was diagnosed with an undifferentiated pleomorphic cell sarcoma (UPS) of the anteromedial thigh. After preoperative radiotherapy, a mass of 23 × 15 cm was removed, and reconstruction with a pedicled deep inferior epigastric artery perforator (p-DIEP) flap-based lymphatic flow through (LyFT) was performed. Six months later, the patient developed the first local recurrence with the presence of a distant metastasis. Following the tumor resection, the medial part of the DIEP flap was de-epithelized and buried in the defect for dead space obliteration. Another local recurrence arose 7 months after the second surgery. Therefore, a major debulking surgery involving the femoral neurovascular bundle was performed. The femoral artery was reconstructed with a synthetic graft, and the femoral vein with the great saphenous vein harvested from the contralateral thigh. A composite myocutaneous neurotized anterolateral thigh (ALT) flap from the contralateral thigh was used to obliterate the defect and restore the loss of function of the quadriceps femoris. Two lymphaticovenular anastomoses (LVAs) were performed at the ankle to reduce the risk of lymphatic sequelae. This case report highlights the importance of integrating various techniques to create a tailored approach that effectively addresses complex surgical requirements to avoid limb amputation and maintain functionality.


Asunto(s)
Anastomosis Quirúrgica , Arterias Epigástricas , Colgajos Tisulares Libres , Recurrencia Local de Neoplasia , Colgajo Perforante , Procedimientos de Cirugía Plástica , Sarcoma , Neoplasias de los Tejidos Blandos , Muslo , Humanos , Masculino , Persona de Mediana Edad , Muslo/cirugía , Colgajo Perforante/irrigación sanguínea , Colgajos Tisulares Libres/trasplante , Colgajos Tisulares Libres/irrigación sanguínea , Arterias Epigástricas/trasplante , Neoplasias de los Tejidos Blandos/cirugía , Anastomosis Quirúrgica/métodos , Sarcoma/cirugía , Recurrencia Local de Neoplasia/cirugía , Procedimientos de Cirugía Plástica/métodos , Músculo Cuádriceps
16.
BMJ Case Rep ; 17(8)2024 Aug 30.
Artículo en Inglés | MEDLINE | ID: mdl-39214579

RESUMEN

A man in his 20s presented with a painless, slow-growing firm swelling in the anterolateral aspect of his left forearm. The swelling had been present for 1 year and measured 10×12 cm. Clinically, a differential diagnosis of soft tissue sarcoma, lipoma, neurofibroma, dermoid cyst and hydatid cyst of the extremity was considered. MRI suggested a primary intramuscular hydatid cyst. However, fine-needle aspiration was inconclusive, and ELISA for immunoglobulin G antibodies to echinococcal antigen in serum was negative. A wide-local complete surgical excision of the lesion was planned. Intraoperatively, a well-defined, tense cystic swelling with surrounding dense adhesions was found within the intramuscular plane. Histopathological examination of the cyst wall revealed cysticercosis. The patient recovered uneventfully. This case highlights that solitary intramuscular cysticercosis, although rare, should be included in the differential diagnosis of an isolated soft tissue mass, particularly in endemic areas.


Asunto(s)
Cisticercosis , Antebrazo , Neoplasias de los Tejidos Blandos , Humanos , Masculino , Cisticercosis/diagnóstico , Diagnóstico Diferencial , Neoplasias de los Tejidos Blandos/diagnóstico , Neoplasias de los Tejidos Blandos/diagnóstico por imagen , Imagen por Resonancia Magnética , Adulto , Enfermedades Musculares/diagnóstico , Enfermedades Musculares/parasitología
17.
In Vivo ; 38(5): 2383-2389, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39187363

RESUMEN

BACKGROUND/AIM: Benign and tumor-like lesions of the hindfoot and ankle are common, whereas malignant entities are rare. Accurate evaluation and timely management of these lesions can be challenging, making it crucial to understand their incidence and anatomic localization. This study retrospectively analyzed the distribution of benign and malignant bone and soft tissue tumors in the hindfoot and ankle. PATIENTS AND METHODS: This study included patient data from a single center, such as age, sex, histologic diagnosis, and anatomic location over a 12.5 year period. RESULTS: Of the 105 cases reviewed, 19 cases (18.1%) were osseous lesions and 86 cases (81.9%) were soft tissue lesions. The latter were divided into 77 benign and 9 malignant cases, resulting in an overall malignancy rate of 8.6%. The most common osseous lesion was the intraosseous ganglion (n=12). The majority of benign soft tissue lesions (75.3%) were located in the hindfoot, with TGCT, schwannoma, and ganglion cysts being the most common types. The nine malignant cases were distributed among seven entities and were evenly distributed among both regions and sexes. Malignant cases had a higher mean age (59.2 years) compared to benign cases (40.8 years; p=0.001). CONCLUSION: Tumors, tumor-like lesions, and pseudotumors represent an important aspect of ankle pathology. The majority of focal masses and swellings are benign soft tissue or osseous lesions, but malignant entities can occur and may be mistaken for benign conditions. Preoperative imaging and histopathologic examination are essential, and preoperative presentation to a multidisciplinary tumor board is recommended in unclear cases.


Asunto(s)
Tobillo , Neoplasias Óseas , Neoplasias de los Tejidos Blandos , Humanos , Masculino , Femenino , Neoplasias de los Tejidos Blandos/patología , Neoplasias de los Tejidos Blandos/epidemiología , Neoplasias de los Tejidos Blandos/diagnóstico , Persona de Mediana Edad , Adulto , Neoplasias Óseas/patología , Neoplasias Óseas/diagnóstico , Neoplasias Óseas/epidemiología , Anciano , Tobillo/patología , Adolescente , Adulto Joven , Pie/patología , Anciano de 80 o más Años , Estudios Retrospectivos , Niño
18.
Jt Dis Relat Surg ; 35(3): 628-636, 2024 May 20.
Artículo en Inglés | MEDLINE | ID: mdl-39189573

RESUMEN

OBJECTIVES: This study aims to investigate the etiological distribution of primary and metastatic malignancies around the elbow and the effect of surgical and adjuvant treatments on clinical outcome. PATIENTS AND METHODS: Between January 2006 and December 2020, medical records of a total of 33 patients with elbow neoplasm (15 males, 18 females; median age: 55 years; range, 39 to 71 years) who underwent surgical treatment and with or without clinical treatment were retrospectively analyzed. The outcomes and frequencies of the elbow metastatic and primary malignancies were evaluated. Data were collected from patients' medical and radiological documents, and a dedicated archive was created for this study. RESULTS: Most tumors occurred on the right side and were intra-articular or distal to the humerus. A total of 75.8% (25/33) of the patients had tumors of any diameter ≥5 cm. Most patients were treated with extensive resection. A total of 81.8% (27/33) of the patients had wide resected tumor margins, and 18.2% (6/33) had intralesional tumor margins. The median follow-up was 42 (range, 1 to 83) months. Synovial sarcoma and malignant peripheral nerve sheath tumors were the most common soft tissue sarcomas, and pulmonary adenoma and multiple myeloma were found in metastatic lesions. CONCLUSION: Elbow surgery is particularly challenging due to the interrelationship of major neurovascular structures. Synovial sarcoma and malignant peripheral nerve sheath tumors are the most common soft tissue sarcomas, and pulmonary adenoma and multiple myeloma are found in metastatic lesions. Limb-sparing surgery is the gold-standard method recently.


Asunto(s)
Neoplasias Óseas , Articulación del Codo , Humanos , Masculino , Persona de Mediana Edad , Femenino , Adulto , Anciano , Estudios Retrospectivos , Articulación del Codo/cirugía , Articulación del Codo/patología , Neoplasias Óseas/secundario , Resultado del Tratamiento , Neoplasias de los Tejidos Blandos/patología , Neoplasias de los Tejidos Blandos/cirugía , Codo/patología
19.
J Plast Reconstr Aesthet Surg ; 97: 65-70, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-39146907

RESUMEN

OBJECTIVE: Reversed great saphenous vein (GSV) graft is widely used for revascularization in limb-sparing surgery for sarcoma invading great vessels. However, a mismatch in caliber between the reverse graft and cut end of the artery can threaten graft patency. Recently, we introduced the use of a venous valvulotome to allow nonreversed GSV graft. The purpose of this study was to evaluate the safety and versatility of this technique. DESIGN: We retrospectively compared long-term patency and limb salvage rates between nonreversed GSV and reversed GSV in patients undergoing limb-sparing surgery for sarcoma. METHODS: Thirty-seven patients were included, with 21 in the nonreversed GSV group and 16 in the reversed GSV group. Patient characteristics, surgical details, and complications were reviewed from the hospital records. The patency of the reconstructed vessels was assessed using contrast-enhanced CT or MRI. Statistical analyses, including Kaplan-Meier survival analysis, were employed for comparisons. RESULTS: The median follow-up was 38 months. Overall graft patency was 90.4% (19 of 21 patients) in the nonreversed GSV group and 81.2% (13 of 16) in the reverse GSV (RGSV) group. In the nonreversed GSV group, there was 1 case of graft occlusion each in the acute and chronic phases, but limb circulation remained intact and all limbs were spared. CONCLUSION: Nonreversed GSV grafting with valvulotome offers a safe and versatile alternative to reversed GSV grafts in limb-sparing sarcoma surgery. It eliminates the need for vein reversal and minimizes diameter mismatch, potentially expanding the indication for autologous revascularization to previously ineligible cases.


Asunto(s)
Recuperación del Miembro , Extremidad Inferior , Vena Safena , Sarcoma , Grado de Desobstrucción Vascular , Humanos , Vena Safena/trasplante , Masculino , Femenino , Estudios Retrospectivos , Sarcoma/cirugía , Persona de Mediana Edad , Adulto , Extremidad Inferior/irrigación sanguínea , Extremidad Inferior/cirugía , Anciano , Recuperación del Miembro/métodos , Procedimientos de Cirugía Plástica/métodos , Adolescente , Adulto Joven , Resultado del Tratamiento , Neoplasias de los Tejidos Blandos/cirugía
20.
Radiologie (Heidelb) ; 64(9): 728-738, 2024 Sep.
Artículo en Alemán | MEDLINE | ID: mdl-39110144

RESUMEN

Malignant soft tissue tumors, in particular, require a multimodal treatment concept involving interdisciplinary cooperation between radiologists, pathologists, surgeons and oncologists at special tumor centers. The foundations of the treatment decision are the imaging diagnostics and the diagnosis confirmation based on tissue samples. The (local) extent and growth behavior of a tumor are among the most important findings of imaging as they have a direct influence on the surgical procedure. The most important diagnostic procedure here is magnetic resonance imaging (MRI). The T1-weighted and fat-suppressed sequences after i.v. contrast administration are used to visualize the extent of the tumor. In synopsis with diffusion-weighted and T2-weighted sequences, a differentiation between vital tumor tissue and tumor necrosis is additionally possible. This also enables targeted sampling from vital tumor parts so that the patient can be assigned to the appropriate treatment concept as quickly as possible.


Asunto(s)
Imagen por Resonancia Magnética , Neoplasias de los Tejidos Blandos , Humanos , Medios de Contraste/administración & dosificación , Diagnóstico Diferencial , Imagen por Resonancia Magnética/métodos , Neoplasias de los Tejidos Blandos/diagnóstico por imagen , Neoplasias de los Tejidos Blandos/patología , Neoplasias de los Tejidos Blandos/terapia
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