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1.
World J Surg Oncol ; 22(1): 113, 2024 Apr 26.
Artigo em Inglês | MEDLINE | ID: mdl-38664776

RESUMO

BACKGROUND: The standard curative treatments for extremity soft tissue sarcoma (ESTS) include surgical resection with negative margins and perioperative radiotherapy. However, the optimal resection margin remains controversial. This study aimed to evaluate the outcomes in ESTS between microscopically positive margin (R1) and microscopically negative margin (R0) according to the Union for International Cancer Control (UICC) (R + 1 mm) classification. METHODS: Medical records of patients with localized ESTS who underwent primary limb-sparing surgery and postoperative radiotherapy between 2004 and 2015 were retrospectively reviewed. Patients were followed for at least 5 years or till local or distant recurrence was diagnosed during follow-up. Outcomes were local and distal recurrences and survival. RESULTS: A total of 52 patients were included in this study, in which 17 underwent R0 resection and 35 underwent R1 resection. No significant differences were observed in rates of local recurrence (11.4% vs. 35.3%, p = 0.062) or distant recurrence (40.0% vs. 41.18%, p = 0.935) between R0 and R1 groups. Multivariate analysis showed that distant recurrences was associated with a Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grade (Grade III vs. I, adjusted hazard ratio (aHR): 12.53, 95% confidence interval (CI): 2.67-58.88, p = 0.001) and tumor location (lower vs. upper extremity, aHR: 0.23, 95% CI: 0.07-0.7, p = 0.01). Kaplan-Meier plots showed no significant differences in local (p = 0.444) or distant recurrent-free survival (p = 0.161) between R0 and R1 groups. CONCLUSIONS: R1 margins, when complemented by radiotherapy, did not significantly alter outcomes of ESTS as R0 margins. Further studies with more histopathological types and larger cohorts are necessary to highlight the path forward.


Assuntos
Extremidades , Margens de Excisão , Recidiva Local de Neoplasia , Sarcoma , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Sarcoma/cirurgia , Sarcoma/patologia , Sarcoma/radioterapia , Sarcoma/mortalidade , Estudos Retrospectivos , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/cirurgia , Extremidades/patologia , Extremidades/cirurgia , Adulto , Seguimentos , Taxa de Sobrevida , Idoso , Prognóstico , Radioterapia Adjuvante/métodos , Radioterapia Adjuvante/estatística & dados numéricos , Tratamentos com Preservação do Órgão/métodos , Tratamentos com Preservação do Órgão/estatística & dados numéricos , Adulto Jovem , Neoplasias de Tecidos Moles/cirurgia , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/radioterapia , Neoplasias de Tecidos Moles/mortalidade , Adolescente
2.
Curr Oncol ; 31(4): 1725-1738, 2024 Mar 26.
Artigo em Inglês | MEDLINE | ID: mdl-38668034

RESUMO

Introduction: Soft tissue sarcomas (STS) are low-incidence tumors whose clinical and histopathological factors are associated with adverse oncological outcomes. This study evaluated prognostic factors (PF) associated with tumor recurrence and overall survival (OS) in patients diagnosed with STS of the extremities, treated at the Instituto Nacional de Cancerología (INC), Bogotá, Colombia. Materials and Methods: An analytical observational study of a historical cohort was carried out, including patients diagnosed with STS and managed surgically in the Functional Unit for Breast and Soft Tissue Tumors of the INC from January 2008 to December 2018. Results: A total of 227 patients were included; 74.5% had tumors greater than 5 cm. Most patients (29.1%) were in stage IIIB at diagnosis. Age was associated with higher mortality (HR = 1.01; CI95%: 1-1.02; p = 0.048). Tumor persistence at admission to the INC (HR = 2.34; CI95%: 1.25-4.35; p = 0.007) and histologic grade III (HR = 5.36; CI95%: 2.29-12.56; p = <0.001) showed statistical significance in the multivariate analysis for recurrence of any type, as did the PFs associated with a higher risk of local recurrence (HR = 2.85; CI95%: 1.23-6.57; p = 0.014 and HR = 6.09; CI95%: 2.03-18.2; p = 0.001), respectively. Tumor size (HR = 1.03; CI95%: 1-1.06; p = 0.015) and histologic grade III (HR = 4.53; CI95%: 1.42-14.49; p = 0.011) were associated with a higher risk of distant recurrence. Conclusions: This cohort showed that in addition to histologic grade and tumor size, tumor persistence at the time of admission has an impact on disease recurrence, so STS should be managed by a multidisciplinary team with experience in this pathology in high-volume reference centers.


Assuntos
Extremidades , Recidiva Local de Neoplasia , Sarcoma , Humanos , Feminino , Masculino , Sarcoma/mortalidade , Colômbia/epidemiologia , Pessoa de Meia-Idade , Extremidades/patologia , Prognóstico , Adulto , Idoso , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Idoso de 80 Anos ou mais
3.
Semin Radiat Oncol ; 34(2): 147-163, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38508780

RESUMO

Extremity and truncal soft tissue sarcomas are a heterogeneous group of rare cancers that arise from mesenchymal tissues. Hence, the adoption of tailored risk assessment and prognostication tools plays a crucial role in optimizing the decision-making for which of the many possible treatment strategies to select. Management of these tumors requires a multidisciplinary strategy, which has seen significant development in recent decades. Surgery has emerged as the primary treatment approach, with the main goal of achieving microscopic negative tumor margins. To reduce the likelihood of local recurrence, loco-regional treatments such as radiation therapy and isolated limb perfusion are often added to the treatment regimen in combination with surgery. This approach also enables surgeons to perform limb-sparing surgery, particularly in cases where a positive tumor margin is expected. Chemotherapy may also provide a further benefit in decreasing the probability of local recurrence or reducing distant metastasis in selected patients. Selecting the optimal treatment strategy for these rare tumors is best accomplished by an experienced multi-disciplinary team.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Humanos , Medição de Risco , Terapia Combinada , Extremidades/patologia , Extremidades/cirurgia , Sarcoma/patologia , Sarcoma/cirurgia , Radioterapia Adjuvante , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/radioterapia , Recidiva Local de Neoplasia/prevenção & controle , Recidiva Local de Neoplasia/patologia
6.
Chirurgie (Heidelb) ; 95(4): 288-293, 2024 Apr.
Artigo em Alemão | MEDLINE | ID: mdl-38360873

RESUMO

Sarcomas of the extremities are rare entities, the treatment of which requires special expertise. Even if the treatment of patients is always interdisciplinary, the surgical R0 resection is the key point of each curatively intended treatment. In addition to resection in sano, the aim is to preserve the extremities and function, so that defect reconstruction after resection plays a decisive role. Due to the heterogeneity of tumors as well as their localization and extent, reconstruction is always an individually adapted treatment. Modular tumor endoprostheses are often used in this context, which can be constructed according to the size of the defect. The transplantation of autologous or allogeneic bone is also frequently used alone or as an additive procedure. Patient-specific (mega)prostheses are used particularly for pelvic tumors. Defect reconstruction using scaffold-based procedures from the field of tissue engineering is being tested as a promising procedure for the future. This article provides an overview of the treatment principles for sarcomas of the extremities and their individual reconstruction options.


Assuntos
Procedimentos de Cirurgia Plástica , Sarcoma , Humanos , Sarcoma/cirurgia , Sarcoma/patologia , Salvamento de Membro/métodos , Próteses e Implantes , Extremidades/cirurgia , Extremidades/patologia
7.
Clin Oncol (R Coll Radiol) ; 36(4): e97-e104, 2024 04.
Artigo em Inglês | MEDLINE | ID: mdl-38326122

RESUMO

AIMS: Synovial sarcoma is a rare but aggressive variant of soft-tissue sarcoma. Literature is sparse and reported mostly from the West. We analysed the clinical profiles and prognostic factors of extremity synovial sarcoma patients in order to study their clinical journey. MATERIALS AND METHODS: This was a retrospective analysis. All patients with extremity synovial sarcoma treated between 1992 and 2020 were included. Patients with metastases at presentation were excluded. A descriptive analysis of demographic and clinicopathological features of patients undergoing limb salvage surgery (LSS) or amputation was carried out. Overall survival and disease-free survival were calculated for the entire cohort as well as for the LSS and amputation groups. Factors prognostic for survival were identified. RESULTS: In total, 157 patients had localised extremity synovial sarcoma. Predominantly, young adults (median 31 years) and males (61%) were affected. Over 70% of patients presented after recurrence or unplanned surgeries. Sixty-seven per cent of tumours were >5 cm, 69% were deep and 23% involved bone. The limb salvage rate was 64%. In the LSS group, adjuvant radiotherapy and chemotherapy were given to 72% and 68% of patients, respectively. In the amputation group, 72% of patients received adjuvant chemotherapy. In a median follow-up of 59 months, 39.4% of patients had recurrences, the majority (61.2%) were systemic. Five-year overall survival and disease-free survival were 53.4% and 49.8%, respectively. Overall survival was 63.9% and 29.7% in the LSS and amputation groups, respectively. On multivariate analysis, tumour size, depth, omission of radiotherapy and bone invasion were found to be the adverse prognostic factors. CONCLUSION: This is one of the largest studies on extremity synovial sarcoma. Mostly males and young adults were affected. The limb salvage rate was 64%, despite most being referred after unplanned surgery. Almost 70% of patients received radiotherapy and chemotherapy. Overall survival was inferior in the amputation group. Tumour size >5 cm, depth and bone invasion were negative, whereas adjuvant radiotherapy was a positive prognostic factor for survival. Chemotherapy had no impact on survival.


Assuntos
Sarcoma Sinovial , Sarcoma , Neoplasias de Tecidos Moles , Masculino , Adulto Jovem , Humanos , Feminino , Sarcoma Sinovial/cirurgia , Estudos Retrospectivos , Sarcoma/patologia , Extremidades/patologia , Extremidades/cirurgia , Prognóstico , Neoplasias de Tecidos Moles/patologia , Recidiva Local de Neoplasia/patologia
8.
J Natl Compr Canc Netw ; 22(1): 26-33, 2024 02.
Artigo em Inglês | MEDLINE | ID: mdl-38394772

RESUMO

BACKGROUND: Based on the NCCN Guidelines for Soft Tissue Sarcoma (STS), treatment of extremity STS (ESTS) includes radiation therapy (RT) and surgical resection for tumors that are high-grade and >5 cm. ​​The aim of this study was to describe the association between neighborhood socioeconomic status (nSES), concordance with NCCN Guidelines recommendations, and outcomes in patients with ESTS. METHODS: Patients with ESTS diagnosed from 2006 through 2018 were identified in SEER registries. The analytic cohort was restricted to patients with high-grade tumors >5 cm without nodal or distant metastases who received limb-sparing surgery. Patient demographics and tumor characteristics associated with receipt of RT were analyzed using adjusted regression analyses. Kaplan-Meier curves and adjusted accelerated failure time models were used to examine disparities in cancer-specific survival. RESULTS: Of 2,249 patients, 29.0% (n=648) received neoadjuvant RT, 49.7% (n=1,111) received adjuvant or intraoperative RT, and 21.3% (n=476) did not receive RT. In adjusted analyses, lower nSES was associated with lower likelihood of receiving RT (odds ratio, 0.70 [95% CI, 0.57-0.87]; P<.001). Low nSES was associated with worse cancer-specific survival (hazard ratio, 1.19 [95% CI, 1.01-1.40]; P=.04). Race and ethnicity were not significant predictors of receipt of RT or cancer-specific survival in the fully adjusted models. CONCLUSIONS: Patients from lower nSES areas were less likely to receive NCCN Guideline-recommended RT for their ESTS and had worse cancer-specific survival. Efforts to better define and resolve disparities in the treatment and survival of patients with ESTS are warranted.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Humanos , Extremidades/patologia , Etnicidade , Terapia Combinada , Radioterapia Adjuvante , Sarcoma/diagnóstico , Estudos Retrospectivos
9.
Ann Surg Oncol ; 31(6): 4138-4147, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38396039

RESUMO

BACKGROUND: Although social vulnerability has been associated with worse postoperative and oncologic outcomes in other cancer types, these effects have not been characterized in patients with soft tissue sarcoma. This study evaluated the association of social vulnerability and oncologic outcomes. METHODS: The authors conducted a single-institution cohort study of adult patients with primary and locally recurrent extremity or truncal soft tissue sarcoma undergoing resection between January 2016 and December 2021. The social vulnerability index (SVI) was measured on a low (SVI 1-39%, least vulnerable) to high (60-100%, most vulnerable) SVI scale. The association of SVI with overall survival (OS) and recurrence-free survival (RFS) was evaluated by Kaplan-Meier analysis and Cox proportional hazard regression. RESULTS: The study identified 577 patients. The median SVI was 44 (interquartile range [IQR], 19-67), with 195 patients categorized as high SVI and 265 patients as low SVI. The median age, tumor size, histologic subtype, grade, comorbidities, stage, follow-up time, and perioperative chemotherapy and radiation utilization were similar between the high and low SVI cohorts. The patients with high SVI had worse OS (p = 0.07) and RFS (p = 0.016) than the patients with low SVI. High SVI was independently associated with shorter RFS in the multivariate analysis (hazard ratio, 1.64; 95% confidence interval, 1.06-2.54) but not with OS (HR, 1.47; 95% CI 0.84-2.56). CONCLUSION: High community-level social vulnerability appears to be independently associated with worse RFS for patients undergoing resection of extremity and truncal soft tissue sarcoma. The effect of patient and community-level social risk factors should be considered in the treatment of patients with extremity sarcoma.


Assuntos
Extremidades , Recidiva Local de Neoplasia , Sarcoma , Humanos , Feminino , Masculino , Sarcoma/cirurgia , Sarcoma/mortalidade , Sarcoma/patologia , Pessoa de Meia-Idade , Extremidades/cirurgia , Extremidades/patologia , Taxa de Sobrevida , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/cirurgia , Recidiva Local de Neoplasia/mortalidade , Idoso , Seguimentos , Prognóstico , Adulto , Populações Vulneráveis , Tronco/cirurgia , Tronco/patologia , Estudos Retrospectivos , Fatores de Risco , Neoplasias de Tecidos Moles/cirurgia , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia
10.
Sci Rep ; 14(1): 3903, 2024 02 16.
Artigo em Inglês | MEDLINE | ID: mdl-38365844

RESUMO

Myxofibrosarcoma (MFS) is a malignant fibroblastic/myofibroblastic neoplasm with a prominent myxoid area. It has the clinical features of frequent local recurrence (LR) and occasional distant metastasis. Robust epidemiological data on MFS in China are lacking. The aim of this retrospective analysis was to determine the natural history of MFS, identify prognostic factors for recurrence and describe the real-life outcomes of MFS. We reviewed 52 patients with primary MFS from the First Affiliated Hospital of Nanjing Medical University diagnosed between 2016 and 2020. All tumors were subjected to retrospective univariate analysis for prognostic factors of the disease, including tumor size, grade, location and sex; patient age; planned operation; surgical margin; and laboratory results. The significant factors identified by univariate analysis were subsequently analyzed via multivariate analysis. Overall survival (OS), post-treatment LR and metastatic-free survival were assessed as outcomes. The median age was 61 years (range, 13-93). Fourteen (26.92%) patients exhibited low grade disease, and 38 (73.08%) exhibited high grade disease. Among the 29 males, and 23 females, 15 (28.85%) had tumors in the trunk, 37 (71.15%) had tumors in the extremities, 26 had undergone planned surgery, and 26 had unexpected unplanned operation. The margin was negative in 39 (75%) patients and positive in 13 patients (25%). The serum creatine kinase (CK) concentration was high level in 33 (63.46%) patients and low level in 19 (36.54%) patients. The serum lactate dehydrogenase (LDH) levels were low in 23 (44.23%) patients and high in 29 (55.77%) patients. LR was observed in 25 patients (48.08%), and 4 patients developed metastasis. A worse LR rate was found for patients with a low CK level (84.21%) than for those with a high CK level (27.27%) at 5 years (p < 0.05). The LR rate of patients who underwent planned surgery was lower than that of patients who underwent unplanned surgery (p < 0.05). There were significantly more patients with positive margins than patients with negative margins (92.30%, and 33.33%, respectively; p < 0.05). Moreover, superficial tumors were also associated with greater recurrence rate (2/20 [10%]) than deep tumors, (23/32 [71.86%]) [p < 0.05]. The probability of LR in patients with MFS was significantly greater in association with unplanned operations, positive margins, low serum CK levels or superficial tumor depth. These data could help identify high-risk patients; thus, more careful follow-up should be performed for higher-risk patients. Diagnosis and treatment at qualified regular medical centers can reduce the local recurrence rate of MFS.


Assuntos
Fibrossarcoma , Histiocitoma Fibroso Maligno , Masculino , Feminino , Adulto , Humanos , Pessoa de Meia-Idade , Estudos Retrospectivos , Fibrossarcoma/cirurgia , Fibrossarcoma/patologia , Extremidades/patologia , Prognóstico , Histiocitoma Fibroso Maligno/patologia , Recidiva Local de Neoplasia/patologia
11.
J Surg Res ; 296: 196-202, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38277957

RESUMO

INTRODUCTION: Planar lymphoscintigraphy (PL) is commonly used in mapping before sentinel lymph node biopsy (SLNB) for invasive cutaneous melanoma. Recently, single-photon emission computed tomography (SPECT)/ computed tomography (CT) has been utilized, in addition to PL, for detailed anatomic information and detection of sentinel lymph nodes (SLNs) outside of the primary nodal basin in truncal and head and neck melanoma. Following a protocol change due to COVID-19, our institution began routinely obtaining both PL and SPECT-CT imaging for all melanoma SLN mapping. We hypothesized that SPECT-CT is associated with higher instances of SLNBs from "nontraditional" nodal basins (NTNB) for extremity melanomas. METHODS: Patients with extremity melanoma (2017-2022) who underwent SLNB were grouped into SPECT-CT with PL versus PL alone. Outcomes were total SLNs removed, + or-SLN status, total NTNB sampled, and postoperative complication rate. Poisson regression and logistic regression models were used to assess association of SPECT-CT with patient outcomes. RESULTS: Of 380 patients with extremity melanoma, 42.11% had SPECT-CT. There were no differences between the groups with regards to age at diagnosis or sex. From 2020 to 2022, all patients underwent SPECT-CT. SPECT-CT was associated with increased odds of SLNB from an NTNB, (odds ratio = 2.39 [95% confidence interval: 1.25-4.67]). There was no difference in odds of number of SLNs sampled, SLN positivity rate, or postoperative complication rate with SPECT-CT. CONCLUSIONS: Routine SPECT-CT was associated with higher incidence of SLNB in NTNB but did not increase number of SLNs removed or SLN positivity rate. The added value of routine SPECT-CT in cutaneous melanoma of the extremities remains to be defined.


Assuntos
Melanoma , Neoplasias Cutâneas , Humanos , Melanoma/diagnóstico por imagem , Melanoma/cirurgia , Melanoma/patologia , Neoplasias Cutâneas/diagnóstico por imagem , Neoplasias Cutâneas/cirurgia , Neoplasias Cutâneas/patologia , Tomografia Computadorizada com Tomografia Computadorizada de Emissão de Fóton Único , Biópsia de Linfonodo Sentinela/métodos , Extremidades/diagnóstico por imagem , Extremidades/patologia , Complicações Pós-Operatórias/cirurgia , Tomografia Computadorizada de Emissão de Fóton Único/métodos
12.
Nature ; 626(7997): 151-159, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38233525

RESUMO

Enhancers control the location and timing of gene expression and contain the majority of variants associated with disease1-3. The ZRS is arguably the most well-studied vertebrate enhancer and mediates the expression of Shh in the developing limb4. Thirty-one human single-nucleotide variants (SNVs) within the ZRS are associated with polydactyly4-6. However, how this enhancer encodes tissue-specific activity, and the mechanisms by which SNVs alter the number of digits, are poorly understood. Here we show that the ETS sites within the ZRS are low affinity, and identify a functional ETS site, ETS-A, with extremely low affinity. Two human SNVs and a synthetic variant optimize the binding affinity of ETS-A subtly from 15% to around 25% relative to the strongest ETS binding sequence, and cause polydactyly with the same penetrance and severity. A greater increase in affinity results in phenotypes that are more penetrant and more severe. Affinity-optimizing SNVs in other ETS sites in the ZRS, as well as in ETS, interferon regulatory factor (IRF), HOX and activator protein 1 (AP-1) sites within a wide variety of enhancers, cause gain-of-function gene expression. The prevalence of binding sites with suboptimal affinity in enhancers creates a vulnerability in genomes whereby SNVs that optimize affinity, even slightly, can be pathogenic. Searching for affinity-optimizing SNVs in genomes could provide a mechanistic approach to identify causal variants that underlie enhanceropathies.


Assuntos
Elementos Facilitadores Genéticos , Extremidades , Polidactilia , Proteínas Proto-Oncogênicas c-ets , Humanos , Elementos Facilitadores Genéticos/genética , Extremidades/embriologia , Extremidades/patologia , Mutação com Ganho de Função , Proteínas de Homeodomínio/metabolismo , Fatores Reguladores de Interferon/metabolismo , Especificidade de Órgãos/genética , Penetrância , Fenótipo , Polidactilia/embriologia , Polidactilia/genética , Polidactilia/patologia , Polimorfismo de Nucleotídeo Único , Ligação Proteica , Proteínas Proto-Oncogênicas c-ets/metabolismo , Fator de Transcrição AP-1/metabolismo
13.
BMC Cancer ; 24(1): 61, 2024 Jan 11.
Artigo em Inglês | MEDLINE | ID: mdl-38212719

RESUMO

BACKGROUND: This study aimed to investigate the characteristics and clinical outcomes in a series of patients with extremity soft tissue sarcoma (STS) who underwent amputation at a large East Asian referral center. PATIENTS AND METHODS: Of the 652 patients who underwent surgery for extremity STS, data of 37 consecutive patients who underwent amputation were reviewed retrospectively. The median follow-up period was 96.0 months (range, 15-216). The patients were classified in to three cohorts. The primary localized (PL) group included patients who underwent amputation as a primary surgical procedure with curative intent. The recurrent localized (RL) group included patients who underwent amputation as a revision procedure after failure of previous limb sparing surgeries. The metastatic group included patients who underwent amputation as a palliative procedure. RESULTS: There were 22 cases of amputation in 596 STS patients and the amputation rate was 3.6% (22/596). Further, 1.8% (9/490) of patients with primary localized STS underwent amputation. Patients with localized STS who underwent amputation had a 5-year disease-specific survival (DSS) rate of 89.9% (95% Confidence Interval (CI), 87.1-92.7%), a local-recurrence-free survival (LRFS) of 84.1% (95% CI, 80.5-87.6%), and a metastasis-free survival (MFS) of 84.6%. (95% CI, 81.1-88.0%) Compared with previous studies, our results showed higher DSS and MFS rates with similar LRFS. CONCLUSIONS: The amputation rate of extremity STS in our institute in East Asia was similar but slightly lower than that reported in Western studies. The oncologic outcome of amputation reported in this study was higher than that indicated in Western studies and oncologic outcome of amputation was not statistically different from those of limb salvage surgery. However, considering the small cohort in single institute study, there is a possibility of selection bias and future multi-center study is necessary. From our results, amputation is still a feasible option for appropriately selected patients unsuitable for limb-conserving surgery.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Humanos , Estudos Retrospectivos , População do Leste Asiático , Extremidades/cirurgia , Extremidades/patologia , Salvamento de Membro , Amputação Cirúrgica , Sarcoma/patologia , Neoplasias de Tecidos Moles/patologia , Recidiva Local de Neoplasia/patologia , Resultado do Tratamento
14.
Bone Joint J ; 106-B(2): 203-211, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-38295850

RESUMO

Aims: This study aimed to compare the performance of survival prediction models for bone metastases of the extremities (BM-E) with pathological fractures in an Asian cohort, and investigate patient characteristics associated with survival. Methods: This retrospective cohort study included 469 patients, who underwent surgery for BM-E between January 2009 and March 2022 at a tertiary hospital in South Korea. Postoperative survival was calculated using the PATHFx3.0, SPRING13, OPTIModel, SORG, and IOR models. Model performance was assessed with area under the curve (AUC), calibration curve, Brier score, and decision curve analysis. Cox regression analyses were performed to evaluate the factors contributing to survival. Results: The SORG model demonstrated the highest discriminatory accuracy with AUC (0.80 (95% confidence interval (CI) 0.76 to 0.85)) at 12 months. In calibration analysis, the PATHfx3.0 and OPTIModel models underestimated survival, while the SPRING13 and IOR models overestimated survival. The SORG model exhibited excellent calibration with intercepts of 0.10 (95% CI -0.13 to 0.33) at 12 months. The SORG model also had lower Brier scores than the null score at three and 12 months, indicating good overall performance. Decision curve analysis showed that all five survival prediction models provided greater net benefit than the default strategy of operating on either all or no patients. Rapid growth cancer and low serum albumin levels were associated with three-, six-, and 12-month survival. Conclusion: State-of-art survival prediction models for BM-E (PATHFx3.0, SPRING13, OPTIModel, SORG, and IOR models) are useful clinical tools for orthopaedic surgeons in the decision-making process for the treatment in Asian patients, with SORG models offering the best predictive performance. Rapid growth cancer and serum albumin level are independent, statistically significant factors contributing to survival following surgery of BM-E. Further refinement of survival prediction models will bring about informed and patient-specific treatment of BM-E.


Assuntos
Neoplasias Ósseas , Fraturas Espontâneas , Humanos , Estudos Retrospectivos , Extremidades/patologia , Albumina Sérica , Prognóstico
15.
Acta Neurol Belg ; 124(1): 37-48, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37815739

RESUMO

Since the crossed control of sensitive-motor body functions by the contralateral cerebral hemispheres was recognized in the early 18th century, clinicians have been baffled by patients developing a motor deficit involving the extremities on the same side as an intracranial lesion. In the first third of the 20th century, three main hypotheses were proposed to explain this so-called ipsilateral or paradoxical hemiparesis: (1) the absence of decussation of the corticospinal tracts; (2) diaschisis, or blocking of the normal input to a brain region anatomically distant from the injured site; and (3) compression of the contralateral cerebral peduncle against the tentorial border, also known as the Kernohan-Woltman notch phenomenon. Here, we deal with the less widely known contributions of the Belgian neurosurgeon Léon Ectors, who included this paradoxical deficit within a neurological syndrome he considered highly specific for an early diagnosis of those meningiomas growing over the third frontal convolution. The present manuscript includes a systematic review of the cases of ipsilateral hemiparesis secondary to intracranial masses reported in ancient and modern scientific medical literature. We also address in-depth the physiopathological theories accounting for this syndrome and contrast them with Léon Ectors' observations.


Assuntos
Lesões Encefálicas , Encéfalo , Masculino , Humanos , Encéfalo/patologia , Lesões Encefálicas/patologia , Paresia/etiologia , Extremidades/patologia , Síndrome
16.
Clin Transl Oncol ; 26(1): 204-213, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37277526

RESUMO

BACKGROUND: Preoperative radiation therapy following by limb-sparing or conservative surgery is a standard approach for limb and trunk STS. Data supporting hypofractionated radiotherapy schedules are scarce albeit biological sensitivity of STS to radiation would justify it. We sought to evaluate the impact of moderate hypofractionation on pathologic response and its influence on oncologic outcomes. MATERIAL AND METHODS: From October 2018 to January 2023, 18 patients with limb or trunk STS underwent preoperative radiotherapy at a median dose of 52.5 Gy (range 49.5-60 Gy) in 15 fractions of 3.5 Gy (3.3-4 Gy) with or without neoadjuvant chemotherapy. A favorable pathologic response (fPR) was considered as ≥ 90% tumor necrosis on specimen examination. RESULTS: All patients completed planned preoperative radiotherapy. Eleven patients (61.1%) achieved a fPR, and 7 patients (36.8%) a complete pathologic response with total disappearance of tumor cells. Nine patients (47%) developed grade 1-2 acute skin toxicity, and 7 patients (38.8%) had wound complications on follow-up. With a median follow-up of 14 months (range 1-40), no cases of local relapse were observed, and actuarial 3-year overall survival (OS) and distant metastases-free survival (DMFS) are 87% and 76.4%, respectively. In the univariate analysis, the presence of a favorable pathologic response (fPR) was associated with improved 3-year OS (100% vs. 56.03%, p = 0.058) and 3-year DMFS (86.91% vs. 31.46%, p = 0.002). Moreover, both complete or partial RECIST response and radiological stabilization of the tumor lesion showed a significant association with higher rates of 3-year distant metastasis-free survival (DMFS) (83% vs. 83% vs. 56%, p < 0.001) and 3-year overall survival (OS) (100% vs. 80% vs. 0, p = 0.002). CONCLUSIONS: Preoperative moderate hypofractionated radiation treatment for STS is feasible and well tolerated and associates encouraging rates of pathologic response that could have a favorable impact on final outcomes.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Humanos , Hipofracionamento da Dose de Radiação , Recidiva Local de Neoplasia/patologia , Extremidades/patologia , Sarcoma/patologia , Terapia Neoadjuvante , Neoplasias de Tecidos Moles/radioterapia , Neoplasias de Tecidos Moles/patologia , Resultado do Tratamento , Estudos Retrospectivos
17.
Clin Transl Oncol ; 26(3): 709-719, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37552409

RESUMO

PURPOSE: Primary bone and joint sarcomas of the long bone are relatively rare neoplasms with poor prognosis. An efficient clinical tool that can accurately predict patient prognosis is not available. The current study aimed to use deep learning algorithms to develop a prediction model for the prognosis of patients with long bone sarcoma. METHODS: Data of patients with long bone sarcoma in the extremities was collected from the Surveillance, Epidemiology, and End Results Program database from 2004 to 2014. Univariate and multivariate analyses were performed to select possible prediction features. DeepSurv, a deep learning model, was constructed for predicting cancer-specific survival rates. In addition, the classical cox proportional hazards model was established for comparison. The predictive accuracy of our models was assessed using the C-index, Integrated Brier Score, receiver operating characteristic curve, and calibration curve. RESULTS: Age, tumor extension, histological grade, tumor size, surgery, and distant metastasis were associated with cancer-specific survival in patients with long bone sarcoma. According to loss function values, our models converged successfully and effectively learned the survival data of the training cohort. Based on the C-index, area under the curve, calibration curve, and Integrated Brier Score, the deep learning model was more accurate and flexible in predicting survival rates than the cox proportional hazards model. CONCLUSION: A deep learning model for predicting the survival probability of patients with long bone sarcoma was constructed and validated. It is more accurate and flexible in predicting prognosis than the classical CoxPH model.


Assuntos
Neoplasias Ósseas , Aprendizado Profundo , Osteossarcoma , Sarcoma , Humanos , Sarcoma/patologia , Neoplasias Ósseas/secundário , Prognóstico , Osteossarcoma/terapia , Osteossarcoma/patologia , Extremidades/patologia , Nomogramas
18.
Surgery ; 175(3): 756-764, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37996341

RESUMO

BACKGROUND: Surgery and radiation therapy remain the standard of care for patients with high-grade extremity soft tissue sarcoma that are >5 cm. Radiation therapy is time and labor-intensive for patients, and social determinants of health may affect adherence. The aim of this study was to define demographic, clinical, and treatment factors associated with the completion of radiation therapy and determine if preoperative radiation therapy improved adherence compared to postoperative radiation therapy. METHODS: The cohort included patients in the National Cancer Database with high-grade extremity soft tissue sarcoma >5 cm without nodal or distant metastases who received limb-sparing surgery and radiation therapy with microscopically negative R0 margins. Multivariable logistic regression analyses identified factors associated with radiation therapy sequencing and adherence (defined as completion of 50 Gy preoperative radiation therapy or at least 60 Gy postoperative radiation therapy). A multivariable Cox Proportional Hazards model assessed overall survival. RESULTS: Among 2,145 patients, 47.1% received preoperative radiation therapy (n = 1,010), and 52.9% (n = 1135) received postoperative radiation therapy. A greater proportion of patients treated with preoperative (77.2%) versus postoperative radiation therapy (64.9%, P < .0001) received the recommended dose. More patients with private insurance (49.8% vs 35.3% Medicaid vs 44.9% Medicare, P = .011) and patients treated at an academic medical center (52.6% vs 47.4%, P < .001) received preoperative radiation therapy. Patients who received preoperative radiation therapy had lower odds of receiving insufficient doses of radiation therapy (odds ratio 0.34 [95% CI 0.27-0.47]). Neither radiation therapy adherence nor sequencing were independent predictors of overall survival. CONCLUSIONS: Patients who received preoperative radiation therapy were more likely to complete therapy and receive an optimal dose than patients treated with postoperative radiation therapy. Preoperative radiation therapy improves adherence and should be widely considered in patients with high-grade extremity soft tissue sarcoma, particularly in patients at risk for not completing therapy.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Humanos , Idoso , Estados Unidos , Radioterapia Adjuvante , Medicare , Extremidades/patologia , Terapia Neoadjuvante , Sarcoma/radioterapia , Sarcoma/cirurgia , Sarcoma/patologia , Neoplasias de Tecidos Moles/radioterapia , Neoplasias de Tecidos Moles/cirurgia , Neoplasias de Tecidos Moles/patologia , Estudos Retrospectivos
19.
J Surg Oncol ; 129(3): 523-530, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37997294

RESUMO

BACKGROUND: Since 2015, the American College of Radiology (ACR) has recommended staging for lung metastasis via chest computed tomography (CT) without contrast for extremity sarcoma staging and surveillance. The purpose of this study was to determine our institutional compliance with this recommendation. METHODS: This was a retrospective chart review of patients diagnosed with sarcoma in the extremities who received CT imaging of the chest for pulmonary staging and surveillance at our institution from 2005 to 2023. A total of 1916 CT studies were included for analysis. We scrutinized ordering patterns before and after 2015 based on the ACR-published metastasis staging and screening guidelines. An institutional and patient cost analysis was performed between CT modalities. RESULTS: The prevalence of CT scans ordered and performed with contrast was greater than those without contrast both prior and post-ACR 2015 guidelines. Furthermore, 79.2% of patient's final surveillance CTs after 2015 were performed with contrast. A cost analysis was performed and demonstrated an additional $297 704 in patient and institutional costs. CONCLUSIONS: At our institution, upon review of CT chest imaging for pulmonary staging and surveillance in patients with extremity sarcoma the use of contrast has been routinely utilized despite a lack of evidence for its necessity and contrary to ACR guidelines.


Assuntos
Sarcoma , Tomografia Computadorizada por Raios X , Humanos , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodos , Tórax , Sarcoma/patologia , Extremidades/diagnóstico por imagem , Extremidades/patologia , Estadiamento de Neoplasias
20.
Ann Surg Oncol ; 31(1): 213-227, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37865942

RESUMO

BACKGROUND: The surveillance guidelines following treatment completion for patients with high-grade sarcomas of the extremities are based largely upon expert opinions and consensus. In the current meta-analysis, we aim to study the utility of surveillance imaging to diagnose local and metastatic pulmonary relapses among patients with extremity soft tissue sarcomas and primary bone sarcomas. PATIENTS AND METHODS: A meta-analysis was performed to assess the sensitivity, specificity and diagnostic odds ratio (DOR) of surveillance imaging to diagnose local and metastatic pulmonary relapse among patients with sarcoma of the extremities. In addition, impact of surveillance imaging on overall survival was assessed. Heterogeneity among eligible studies was evaluated by I2 statistics. Sensitivity analysis was assessed using influence plots and Baujat plots. RESULTS: Ten studies including 2160 patients with sarcoma were found eligible. For diagnoses of local recurrence based on surveillance imaging (nine studies, 1917 patients), the estimated sensitivity, specificity, and DOR were 13.6%, 99.5%, and 78.15, respectively. Only 16.7% of local recurrences were diagnosed based on imaging. For diagnoses of metastatic pulmonary recurrence (eight studies; 1868 patients), estimated sensitivity, specificity, and DOR were 76.1%, 99.3%, and 1059.9, respectively. A sensitivity analysis showed significant heterogeneity among included studies. None of the included studies showed an overall-survival benefit with the use of surveillance imaging. CONCLUSION: The current meta-analysis challenges the notion of routine use of imaging to detect local relapse, while favoring chest imaging, using either chest radiography or computed tomography scan, for surveillance. Further studies are required to study the ideal surveillance strategy including timing and imaging modality.


Assuntos
Neoplasias Ósseas , Sarcoma , Neoplasias de Tecidos Moles , Humanos , Recidiva Local de Neoplasia/epidemiologia , Sarcoma/patologia , Neoplasias Ósseas/diagnóstico por imagem , Recidiva , Pulmão/patologia , Extremidades/diagnóstico por imagem , Extremidades/patologia , Neoplasias de Tecidos Moles/patologia
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