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1.
Cancer Imaging ; 24(1): 59, 2024 May 08.
Artigo em Inglês | MEDLINE | ID: mdl-38720384

RESUMO

BACKGROUND: To develop a magnetic resonance imaging (MRI)-based radiomics signature for evaluating the risk of soft tissue sarcoma (STS) disease progression. METHODS: We retrospectively enrolled 335 patients with STS (training, validation, and The Cancer Imaging Archive sets, n = 168, n = 123, and n = 44, respectively) who underwent surgical resection. Regions of interest were manually delineated using two MRI sequences. Among 12 machine learning-predicted signatures, the best signature was selected, and its prediction score was inputted into Cox regression analysis to build the radiomics signature. A nomogram was created by combining the radiomics signature with a clinical model constructed using MRI and clinical features. Progression-free survival was analyzed in all patients. We assessed performance and clinical utility of the models with reference to the time-dependent receiver operating characteristic curve, area under the curve, concordance index, integrated Brier score, decision curve analysis. RESULTS: For the combined features subset, the minimum redundancy maximum relevance-least absolute shrinkage and selection operator regression algorithm + decision tree classifier had the best prediction performance. The radiomics signature based on the optimal machine learning-predicted signature, and built using Cox regression analysis, had greater prognostic capability and lower error than the nomogram and clinical model (concordance index, 0.758 and 0.812; area under the curve, 0.724 and 0.757; integrated Brier score, 0.080 and 0.143, in the validation and The Cancer Imaging Archive sets, respectively). The optimal cutoff was - 0.03 and cumulative risk rates were calculated. DATA CONCLUSION: To assess the risk of STS progression, the radiomics signature may have better prognostic power than a nomogram/clinical model.


Assuntos
Progressão da Doença , Imageamento por Ressonância Magnética , Nomogramas , Sarcoma , Humanos , Sarcoma/diagnóstico por imagem , Sarcoma/cirurgia , Sarcoma/patologia , Masculino , Feminino , Pessoa de Meia-Idade , Estudos Retrospectivos , Imageamento por Ressonância Magnética/métodos , Adulto , Idoso , Aprendizado de Máquina , Prognóstico , Adulto Jovem , Neoplasias de Tecidos Moles/diagnóstico por imagem , Neoplasias de Tecidos Moles/cirurgia , Neoplasias de Tecidos Moles/patologia , Curva ROC , Radiômica
2.
J Cardiothorac Surg ; 19(1): 288, 2024 May 14.
Artigo em Inglês | MEDLINE | ID: mdl-38745263

RESUMO

Pulmonary arterial sarcomas (PAS) are rare aggressive tumours occurring mainly in the pulmonary trunk. We report a case of PAS involving the pulmonary trunk wall and valve, with uniform wall thickening which represents an atypical imaging manifestation of this tumour. A 63-year-old male presented with vague respiratory symptoms with rapid progression. CTPA showed low density filling defects in both pulmonary arteries and PET scan showed increased uptake in the pulmonary trunk, which along with raised ESR suggested Pulmonary Vasculitis. Echo imaging showed Right ventricular hypertrophy and pulmonary stenosis. Response to steroid therapy was minimal and his symptoms worsened. A referral for second opinion was made and he was diagnosed with PAS. He underwent Pulmonary thromboendarterectomy with Pulmonary valve replacement. Post-operative histopathology confirmed the diagnosis. PAS is rare and frequently misdiagnosed. Surgical resection is not curative, but together with chemotherapy can prolong survival.


Assuntos
Artéria Pulmonar , Valva Pulmonar , Sarcoma , Neoplasias Vasculares , Humanos , Masculino , Pessoa de Meia-Idade , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Artéria Pulmonar/patologia , Sarcoma/diagnóstico , Sarcoma/cirurgia , Valva Pulmonar/diagnóstico por imagem , Neoplasias Vasculares/diagnóstico , Neoplasias Vasculares/cirurgia , Neoplasias Vasculares/diagnóstico por imagem , Diagnóstico Diferencial , Vasculite/diagnóstico , Erros de Diagnóstico
3.
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
4.
Cancer Rep (Hoboken) ; 7(4): e2065, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38627902

RESUMO

BACKGROUND: Extrapleural pneumonectomy (EPP) is a complex surgical procedure involving en-bloc resection of the parietal and visceral pleura, lung, pericardium, and ipsilateral diaphragm. Small case series of pleural-based sarcoma of predominantly pediatric patients suggest EPP may be a life-prolonging surgical option. We aimed to describe the characteristics and outcomes of adults who underwent EPP at a specialized sarcoma center. METHODS: Clinicopathologic variables, surgical details, and follow-up information were extracted for patients undergoing EPP for pleural-based sarcoma between August 2017 and December 2020. Primary outcomes were event-free survival (EFS) and overall survival (OS) from the date of EPP. Secondary outcomes were disease-free interval (DFI) prior to EPP, and early and late postoperative complications. RESULTS: Eight patients were identified, seven with soft tissue sarcoma and one with bone sarcoma. Patients had either localized disease with a primary thoracic sarcoma, sarcoma recurrent to the thorax, or de novo metastatic disease. All patients underwent resection of their pleural-based sarcoma by an experienced cardiothoracic surgeon, and some patients had pre or postoperative treatment. The perioperative morbidity was comparable with previously published reports of EPP performed in mesothelioma patients. At median follow-up of 22.5 months, median EFS was 6.0 months and OS was 20.7 months. Six patients (75%) had disease recurrence; five (62.5%) died of progressive disease. Two patients (25%) had not recurred: one died of a radiation-related esophageal rupture, and one was alive with no evidence of disease at 37.0 months. Characteristics of those with the longest EFS included low-grade histology and achieving a metabolic response to preoperative chemotherapy. CONCLUSIONS: In adults with pleural-based sarcoma, EPP is rarely curative but appears to be a feasible salvage procedure when performed at specialized centers. Patient selection is critical with strong consideration given to multimodal therapy to optimize patient outcomes. In the absence of a confirmed response to neoadjuvant treatment, long term survival is poor and EPP should not be recommended.


Assuntos
Mesotelioma , Neoplasias Pleurais , Sarcoma , Adulto , Humanos , Criança , Pneumonectomia/efeitos adversos , Pneumonectomia/métodos , Neoplasias Pleurais/diagnóstico , Neoplasias Pleurais/cirurgia , Recidiva Local de Neoplasia , Mesotelioma/patologia , Mesotelioma/cirurgia , Sarcoma/diagnóstico , Sarcoma/cirurgia
5.
World J Surg Oncol ; 22(1): 104, 2024 Apr 18.
Artigo em Inglês | MEDLINE | ID: mdl-38637834

RESUMO

OBJECTIVES: To investigate the clinicopathological characteristics and prognosis of patients with primary sarcoma of the uterine cervix. METHODS: We identified all patients with primary cervical sarcomas treated at our institution from 2002 to 2020 and analyzed the clinicopathological characteristics and prognosis. RESULTS: 34 patients were identified, 7 (20.6%) patients had leiomyosarcoma, 6 (17.6%) had carcinosarcoma, 5 (14.7%) had Ewing sarcoma, 4 (11.8%) had rhabdomyosarcoma, 4 (11.8%) had undifferentiated sarcoma, 2 (5.9%) had adenosarcoma, 2 (5.9%) had endometrial stromal sarcoma, 1 (2.9%) had dermatofibrosarcoma protuberans, 1 (2.9%) had alveolar soft tissue sarcoma and 2 (5.9%) had sarcoma not otherwise specified. The median age of the whole patients was 43.5 years (range, 13-63). The median age of patients with Ewing sarcoma or rhabdomyosarcoma was 22 years (range, 13-39) and 17 years (range, 13-36 years), respectively. The distribution by stage was: stage I in 21 (61.8%) patients, stage II in 4 (11.8%), stage III in 6 (17.6%) and stage IV in 3 (8.8%). Overall, 30 patients (88.2%) received surgical treatment. The median follow-up was 33.3 months (range 3.6-187.3 months). 11 patients died within 2 years after diagnosis, most of them were patients with carcinosarcoma or undifferentiated sarcoma (45.5%, 5/11). In the entire cohort, 2- and 5-year OS were 67.2% and 56.9%, respectively. 5-year OS was 25.0% for undifferentiated sarcoma, 50.0% for rhabdomyosarcoma, 50.0% for carcinosarcoma, 53.3% for Ewing sarcoma, 57.1% for leiomyosarcoma. CONCLUSION: Cervical sarcomas are rare neoplasms with multiple histological subtypes and follow an aggressive course. Prognosis may be associated with tumor histology and stage.


Assuntos
Carcinossarcoma , Leiomiossarcoma , Rabdomiossarcoma , Sarcoma de Ewing , Sarcoma , Neoplasias do Colo do Útero , Neoplasias Uterinas , Feminino , Humanos , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Leiomiossarcoma/patologia , Sarcoma de Ewing/cirurgia , Neoplasias do Colo do Útero/cirurgia , Neoplasias Uterinas/diagnóstico , Sarcoma/cirurgia , Sarcoma/diagnóstico , Carcinossarcoma/patologia , Rabdomiossarcoma/cirurgia , Prognóstico
6.
BMJ Case Rep ; 17(4)2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38565229

RESUMO

Marjolijn's ulcer is a malignant ulcer in a burn scar. Types of malignancy are squamous cell carcinoma, basal cell carcinoma and malignant melanoma. Soft tissue sarcoma case reports indicate only one type of cancer. We present a patient in her 60s with a 10-year-old burn scar developing a biopsy-proven squamous cell carcinoma on the lateral aspect of the left thigh with metastatic superficial inguinal node. A wide excision and grafting of ulcer with ilioinguinal dissection done on left side. On the 12th postoperative day 2, subcutaneous swellings adjacent to the grafted area developed, on biopsy revealed to be pleomorphic sarcoma. PET CT scan revealed tumour deposits in the muscles of the left lower limb, liver and lung. There are no case reports of synchronous carcinoma and sarcoma in a burn scar. The case is reported for its rarity and the decision-making dilemma.


Assuntos
Queimaduras , Carcinoma de Células Escamosas , Sarcoma , Neoplasias Cutâneas , Neoplasias de Tecidos Moles , Feminino , Humanos , Queimaduras/complicações , Queimaduras/patologia , Carcinoma de Células Escamosas/patologia , Cicatriz/complicações , Cicatriz/patologia , Sarcoma/complicações , Sarcoma/cirurgia , Neoplasias Cutâneas/patologia , Neoplasias de Tecidos Moles/complicações , Úlcera/complicações , Pessoa de Meia-Idade , Idoso
7.
Ann Ital Chir ; 95(2): 119-125, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38684493

RESUMO

INTRODUCTION: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare and aggressive malignant tumor, with nonspecific clinical symptoms and radiological features. Less than 150 cases have been reported in adults across the world. PRESENTATION OF CASE: We report a case of an extremely rare subtype of UESL with epithelioid features in a 29-year-old woman, presenting as a cystic lesion of 27 × 17 cm, completely subverting the right hepatic lobe. She underwent a right hepatectomy with anterior approach, complete hilum lymphadenectomy and partial diaphragmatic resection for local infiltration, followed by systemic chemotherapy. She remains with no evidence of disease and liver mass has been restored after 6 months. DISCUSSION: The present case report represents the second case of UESL with epithelioid features described across the world. The immunohistochemical expression pattern, cytokeratin (CK)19 + and CK7 -, strongly suggests an origin of this epithelioid component from native biliary cells and not from a reshaped ductal plate. Due to the rarity of this form, to date it is impossible to define the prognostic impact of this subtype of UESL, and treatment remains challenging. CONCLUSION: UESL is associated with a poor prognosis, especially in adults, but a comprehensive and multidisciplinary treatment based on radical resection and adjuvant therapy may provide a survival benefit. Surgical excision with negative margins remains mandatory to diagnose and treat UESL.


Assuntos
Hepatectomia , Neoplasias Hepáticas , Doenças Raras , Sarcoma , Humanos , Adulto , Feminino , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/cirurgia , Sarcoma/patologia , Sarcoma/cirurgia , Sarcoma/diagnóstico , Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias Embrionárias de Células Germinativas/cirurgia , Células Epitelioides/patologia
8.
Medicina (Kaunas) ; 60(4)2024 Mar 27.
Artigo em Inglês | MEDLINE | ID: mdl-38674190

RESUMO

Sarcomas, particularly undifferentiated small round cell sarcomas of bone and soft tissue, pose significant diagnostic challenges due to their nonspecific morphology and the necessity for comprehensive molecular analyses. This paper discusses a rare case of round cell sarcoma exhibiting the EWSR1-CREM fusion, offering insights into the complexities of its diagnosis and management. The patient, a 15-year-old female with a history of Type 1 diabetes, presented with persistent right thigh tenderness and swelling. MRI revealed a large necrotic mass in the retroperitoneal region. Histological analysis showed a well-demarcated tumor with diverse cellular morphologies and distinct necrotic areas. Immunohistochemical (IHC) tests identified dot-like staining for Desmin and Vimentin but negative results for several markers, including Cytokeratin and CD45. Strong ALK positivity was noted. Next-generation sequencing with the Illumina TruSight™ Oncology 500 assay revealed the fusion gene EWSR1-CREM, along with benign and uncertain mutations in other genes. The tumor's morphology and immunoprofile, along with molecular findings, led to a diagnosis of round cell sarcoma with EWSR1-CREM fusion. This case adds to the spectrum of tumors associated with this fusion, often presenting diverse morphologies. The rarity of EWSR1-CREM fusion sarcomas poses a challenge in treatment, highlighted by the development of pulmonary metastases and disease progression after surgical excision in this patient despite the lack of an effective targeted therapy. In conclusion, this case emphasizes the need for a multidisciplinary diagnostic approach in complex sarcomas and highlights the importance of continued research on rare sarcomas, their genetic underpinnings, and potential therapeutic targets.


Assuntos
Modulador de Elemento de Resposta do AMP Cíclico , Proteína EWS de Ligação a RNA , Sarcoma , Humanos , Feminino , Sarcoma/genética , Sarcoma/diagnóstico , Sarcoma/cirurgia , Proteína EWS de Ligação a RNA/genética , Adolescente
9.
PLoS One ; 19(4): e0300594, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38574044

RESUMO

BACKGROUND: Peritoneal sarcomatosis (PS) is a difficult entity to treat with limited options and guarded prognosis. We aimed to determine if the addition of hyperthermic intraperitoneal chemotherapy (HIPEC) could offer superior local recurrence-free survival in patients with retroperitoneal sarcoma at high risk of developing PS as opposed to extended resection alone. METHODS: This is a single arm, phase II intervention study where all patients with recurrent localized retroperitoneal sarcoma considered at high risk of developing PS were considered for enrolment (ClinicalTrials.gov identifier: NCT03792867). Upon enrolment, patients underwent vigorous preoperative testing to ensure fitness for the procedure. During surgery, patients underwent extended resection and HIPEC with doxorubicin. Patients were followed-up every 2 weeks (± 10 days) for the first month and subsequently every three months (± 1 month) up to a year post-surgery, and were assessed for potential chemotherapy toxicity and post-treatment complications. After a year from resection and HIPEC, patients were followed-up either during routine clinic review or contacted via telephone every year (± 1 month) for 3 years. RESULTS: Six patients were recruited but one patient dropped out due to adverse and unexpected intraoperative events. The remaining patients completed the procedure uneventfully. Post-HIPEC, all patients recurred with a disease-free interval ranging from six to 24 months. Three patients died due to complications from recurrent disease whereas the remaining three patients are alive as of their last visit. The overall survival at time at reporting ranged between 22 to 56 months. CONCLUSION: The procedure is feasible with no major morbidity to patients. However, we are unable to recommend for it to be implemented as a routine procedure at this current stage due to lack of improved survival outcomes. Further multi-institutional studies may be conducted to yield better results.


Assuntos
Hipertermia Induzida , Neoplasias Peritoneais , Neoplasias Retroperitoneais , Sarcoma , Humanos , Quimioterapia Intraperitoneal Hipertérmica , Projetos Piloto , Terapia Combinada , Hipertermia Induzida/métodos , Neoplasias Peritoneais/cirurgia , Sarcoma/tratamento farmacológico , Sarcoma/cirurgia , Neoplasias Retroperitoneais/cirurgia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Procedimentos Cirúrgicos de Citorredução
10.
Bone Joint J ; 106-B(5): 492-500, 2024 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-38688512

RESUMO

Aims: Surgical site infection (SSI) after soft-tissue sarcoma (STS) resection is a serious complication. The purpose of this retrospective study was to investigate the risk factors for SSI after STS resection, and to develop a nomogram that allows patient-specific risk assessment. Methods: A total of 547 patients with STS who underwent tumour resection between 2005 and 2021 were divided into a development cohort and a validation cohort. In the development cohort of 402 patients, the least absolute shrinkage and selection operator (LASSO) regression model was used to screen possible risk factors of SSI. To select risk factors and construct the prediction nomogram, multivariate logistic regression was used. The predictive power of the nomogram was evaluated by receiver operating curve (ROC) analysis in the validation cohort of 145 patients. Results: LASSO regression analysis selected possible risk factors for SSI, including age, diabetes, operating time, skin graft or flap, resected tumour size, smoking, and radiation therapy. Multivariate analysis revealed that age, diabetes, smoking during the previous year, operating time, and radiation therapy were independent risk factors for SSI. A nomogram was developed based on the results of multivariate logistic regression analysis. In the development cohort, the incidence of SSI was 4.5% in the low-risk group (risk score < 6.89) and 26.6% in the high-risk group (risk score ≥ 6.89; p < 0.001). In the validation cohort, the incidence of SSI was 2.0% in the low-risk group and 15.9% in the high-risk group (p = 0.004). Conclusion: Our nomogram will enable surgeons to assess the risk of SSI in patients with STS. In patients with high risk of SSI, frequent monitoring and aggressive interventions should be considered to prevent this.


Assuntos
Nomogramas , Sarcoma , Infecção da Ferida Cirúrgica , Humanos , Infecção da Ferida Cirúrgica/etiologia , Infecção da Ferida Cirúrgica/epidemiologia , Sarcoma/cirurgia , Masculino , Feminino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Adulto , Idoso , Medição de Risco/métodos , Neoplasias de Tecidos Moles/cirurgia , Curva ROC , Adolescente , Adulto Jovem , Idoso de 80 Anos ou mais
11.
Breast Dis ; 43(1): 51-59, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38552108

RESUMO

AIM: To retrospectively study the therapeutic modalities of primary breast sarcomas in view of the data of a local Tunisian experience. METHODS: It is a monocentric, descriptive, retrospective study including 13 cases of primary breast sarcoma treated over a period of 25 years (1995-2020) in the oncological radiotherapy department of a university hospital in Sousse, Tunisia. RESULTS: In our study, 13 cases of non-metastatic breast sarcomas that has been identified, divided into ten cases of phyllodes sarcomas and three cases of non-phyllodes sarcomas.Surgically, all our patients had a mastectomy. Among them, seven underwent a lymph node procedure: five underwent axillary lymph node dissection, and two others had primary axillary lymph node biopsy. For the adjuvant treatment, all the patients included in our study received radiotherapy and seven received chemotherapy. Local recurrence occurred on the operative scar in one patient after completion of radiation therapy. Metastatic relapse was described in five patients. The time to onset of metastases varied between two months and five years. Nevertheless, a complete remission was noted in 6 patients with a follow-up varying from four years to 20 years. Two patients were lost to follow-up. CONCLUSION: Breast sarcomas remain a very rare entity of aggressive tumors.The therapeutic approach is poorly codified. For this reason, the therapeutic decision should always be discussed in a multidisciplinary assessment.


Assuntos
Neoplasias da Mama , Sarcoma , Humanos , Feminino , Neoplasias da Mama/patologia , Mastectomia , Estudos Retrospectivos , Tunísia/epidemiologia , Recidiva Local de Neoplasia/cirurgia , Sarcoma/cirurgia , Hospitais , Axila/patologia
12.
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
13.
Semin Radiat Oncol ; 34(2): 164-171, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38508781

RESUMO

Surgical resection is the cornerstone of curative treatment for retroperitoneal sarcomas (RPS), aiming for complete excision, yet the complexity of RPS with its proximity to vital structures continues to lead to high local recurrence rates after surgery alone. Thus, the role of radiotherapy (RT) continues to be refined to improve local control, which remains an important goal to prevent RPS recurrence. The recently completed global randomized trial to evaluate the role of surgery with and without preoperative RT - STRASS1, did not demonstrate a significant overall benefit for neoadjuvant RT based on the pre-specified definition of abdominal recurrence-free survival, however, sensitivity analysis using a standard definition of local recurrence and analysis of outcomes by compliance to the RT protocol suggests histology-specific benefit in well- and some de-differentiated liposarcomas. Ultimately, multidisciplinary collaboration and personalized approaches that consider histological sarcoma types and patient-specific factors are imperative for optimizing the therapeutic strategy in the management of RPS.


Assuntos
Neoplasias Retroperitoneais , Sarcoma , Humanos , Sarcoma/radioterapia , Sarcoma/cirurgia , Sarcoma/patologia , Neoplasias Retroperitoneais/radioterapia , Neoplasias Retroperitoneais/cirurgia , Neoplasias Retroperitoneais/patologia , Terapia Combinada , Radioterapia Adjuvante , Terapia Neoadjuvante , Recidiva Local de Neoplasia/prevenção & controle , Recidiva Local de Neoplasia/radioterapia
14.
Semin Radiat Oncol ; 34(2): 180-194, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38508783

RESUMO

This critical review aims to summarize the relevant published data regarding hypofractionation regimens for preoperative radiation therapy (RT) prior to surgery for soft tissue sarcoma (STS) of the extremity or superficial trunk. We identified peer-reviewed publications using a PubMed search on the MeSH headings of "soft tissue sarcoma" AND "hypofractionated radiation therapy." To obtain complication data on similar anatomical radiotherapeutic scenarios we also searched "hypofractionated radiation therapy" AND "melanoma" as well as "hypofractionated radiation therapy" AND "breast cancer." We then used reference lists from relevant articles to obtain additional pertinent publications. We also incorporated relevant abstracts presented at international sarcoma meetings and relevant clinical trials as listed on the ClinicalTrials.gov website. Detailed data are presented and contextualized for ultra-hypofractionated and moderately hypofractionated regimens with respect to local control, wound complications, and amputation rates. Comparative data are also presented for late toxicities including: fibrosis, joint limitation, edema, skin integrity, and bone fracture or necrosis. These data are compared to a standard regimen of 50 Gy in 25 daily fractions delivered over 5 weeks. This analysis supports the continued use of a standard regimen for preoperative RT for STS of 25 × 2 Gy over 5 weeks without concurrent chemotherapy. Use of concurrent chemotherapy with preoperative RT for STS should be reserved for well-designed clinical trials. A randomized trial of ultra-hypofractionated and moderately hypofractionated pre op RT for STS is warranted, but it is critical for the primary endpoint (or co-primary endpoint) to be late toxicity to: bone, soft tissue, joint, and skin.


Assuntos
Hipofracionamento da Dose de Radiação , Sarcoma , Humanos , Terapia Neoadjuvante , Sarcoma/radioterapia , Sarcoma/cirurgia
15.
Pediatr Blood Cancer ; 71(6): e30949, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38520048

RESUMO

PURPOSE: To evaluate local failure (LF) and toxicity after intraoperative radiation therapy (IORT) in pediatric solid tumors (ST). METHODS: A single-institution retrospective study of 96 pediatric patients (108 applications) with ST treated from 1995 to 2022 with IORT. LF was calculated via cumulative incidence function and overall survival (OS) by Kaplan-Meier method, both from the day of surgery. RESULTS: Median age at time of IORT was 8 years (range: 0.8-20.9 years). Median follow-up for all patients and surviving patients was 16 months and 3 years, respectively. The most common histologies included rhabdomyosarcoma (n = 42), Ewing sarcoma (n = 10), and Wilms tumor (n = 9). Most (95%) received chemotherapy, 37% had prior external beam radiation therapy to the site of IORT, and 46% had a prior surgery for tumor resection. About half (54%) were treated with upfront IORT to the primary tumor due to difficult circumstances such as very young age or challenging anatomy. The median IORT dose was 12 Gy (range: 4-18 Gy), and median area treated was 24 cm2 (range: 2-198 cm2). The cumulative incidence of LF was 17% at 2 years and 23% at 5 years. Toxicity from IORT was reasonable, with postoperative complications likely related to IORT seen in 15 (16%) patients. CONCLUSION: Our study represents the largest and most recent analysis of efficacy and safety of IORT in pediatric patients with ST. Less than one quarter of all patients failed locally with acceptable toxicities. Overall, IORT is an effective and safe technique to achieve local control in patients with challenging circumstances.


Assuntos
Sarcoma , Humanos , Criança , Pré-Escolar , Masculino , Estudos Retrospectivos , Feminino , Adolescente , Lactente , Sarcoma/radioterapia , Sarcoma/mortalidade , Sarcoma/cirurgia , Adulto Jovem , Seguimentos , Cuidados Intraoperatórios , Taxa de Sobrevida , Adulto , Sarcoma de Ewing/radioterapia , Sarcoma de Ewing/mortalidade , Sarcoma de Ewing/cirurgia , Neoplasias/radioterapia , Neoplasias/cirurgia , Neoplasias/mortalidade
16.
J Wound Ostomy Continence Nurs ; 51(2): 107-110, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38527318

RESUMO

PURPOSE: The purpose of this study was to evaluate the benefits of collaborative management between orthopedic surgery and WOC nurses in patients undergoing resection of subcutaneous sarcomas. DESIGN: Retrospective case-control study. SUBJECTS AND SETTING: The sample comprised 25 patients who underwent wide resection for soft tissue sarcoma, followed by 2-stage split-thickness skin grafting. Data collection occurred between January 2015 and April 2021 in a university hospital based in Kagoshima, Japan. For comparison, we categorized these patients into 2 groups: intervention group participants were managed by an orthopedic surgeon and a WOC nurse; nonintervention group members were managed without WOC nurse participation. METHODS: Patient background and treatment-related information was retrospectively collected from medical records and compared between the WOC nurse intervention group and the nonintervention group, including maximum tumor diameter, surgical time, maximum skin defect diameter, length of hospital stay, and time from surgery to complete wound healing. RESULTS: The average length of hospital stay was significantly shorter in the WOC nurse intervention group compared with the nonintervention group (38.3 days, SD = 8.0 vs 47.1 days, SD = 10.2; P = .023). CONCLUSION: Collaborative wound management with a WOC nurse resulted in a shorter hospital length of stay when compared to traditional management with WOC nurse involvement. Based on these findings, we assert that WOC nurses provide an important bridge between postoperative wound management in patients undergoing resection of subcutaneous sarcomas.


Assuntos
Cirurgiões Ortopédicos , Estomia , Sarcoma , Humanos , Estudos Retrospectivos , Estudos de Casos e Controles , Sarcoma/cirurgia
17.
J Int Med Res ; 52(3): 3000605241233145, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38497120

RESUMO

Primary adrenal epithelioid sarcoma is a rare lesion of the adrenal gland, and only seven cases have been reported in the domestic and international literature to date. We herein report a case involving a 65-year-old man with primary adrenal epithelioid sarcoma. After being admitted to the hospital with an adrenal mass found on physical examination, the patient underwent laparoscopic right adrenalectomy. Postoperative pathological findings indicated an epithelioid sarcoma (proximal type). Primary adrenal epithelioid sarcoma is a rare malignancy. Diagnosis is challenging and relies on histopathology and immunohistochemical staining.


Assuntos
Biomarcadores Tumorais , Sarcoma , Masculino , Humanos , Idoso , Imuno-Histoquímica , Sarcoma/diagnóstico , Sarcoma/cirurgia , Sarcoma/patologia
18.
Radiat Oncol ; 19(1): 38, 2024 Mar 16.
Artigo em Inglês | MEDLINE | ID: mdl-38491404

RESUMO

BACKGROUND: The addition of radiation therapy (RT) to surgery in retroperitoneal sarcoma (RPS) remains controversial. We examined practice patterns in the use of RT for patients with RPS over time in a large, national cohort. METHODS: Patients in the National Cancer Database (2004-2017) who underwent resection of RPS were included. Trends over time for proportions were calculated using contingency tables with Cochran-Armitage Trend test. RESULTS: Of 7,485 patients who underwent resection, 1,821 (24.3%) received RT (adjuvant: 59.9%, neoadjuvant: 40.1%). The use of RT decreased annually by < 1% (p = 0.0178). There was an average annual increase of neoadjuvant RT by 13% compared to an average annual decrease of adjuvant RT by 6% (p < 0.0001). Treatment at high-volume centers (OR 14.795, p < 0.0001) and tumor > 10 cm (OR 2.009, p = 0.001) were associated with neoadjuvant RT. In contrast liposarcomas (OR 0.574, p = 0.001) were associated with adjuvant RT. There was no statistically significant difference in overall survival between patients treated with surgery alone versus surgery and RT (p = 0.07). CONCLUSION: In the United States, the use of RT for RPS has decreased over time, with a shift towards neoadjuvant RT. However, a large percentage of patients are still receiving adjuvant RT and this mostly occurs at low-volume hospitals.


Assuntos
Lipossarcoma , Neoplasias Retroperitoneais , Sarcoma , Neoplasias de Tecidos Moles , Humanos , Estados Unidos , Radioterapia Adjuvante/efeitos adversos , Sarcoma/radioterapia , Sarcoma/cirurgia , Terapia Combinada , Neoplasias Retroperitoneais/radioterapia , Neoplasias Retroperitoneais/cirurgia , América do Norte , Estudos Retrospectivos
19.
J Cancer Res Clin Oncol ; 150(3): 110, 2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38427070

RESUMO

BACKGROUND: Small round cell tumor (SRCT) is a group of malignancy with similar optical microscopic morphology. Despite its low incidence, SRCT has a high malignant degree and poor prognosis. Besides, atypical clinical symptoms make it difficult in preoperative diagnosis. CASE REPORT: A 67-year-old man was presented to the outpatient service with dysuria and weak urine stream lasting for 3 months. After oral treatment with tamsulosin and finasteride for 2 months, the symptoms worsen. Transurethral prostate holmium laser enucleation was operated and postoperative pathology result revealed small blue round cell malignant tumor. Further immunohistochemistry and fluorescence in situ hybridization examination indicated Ewing-like SRCT. So a Da Vinci Robotic prostatectomy was performed further and whole-genome sequencing was conducted. Several gene mutations including RAF1, ARID1A, SMARCA4, and BCL2L11 were found but no FDA-approved drug could treat specifically. Then the patient received Ewing-type therapeutic regimens treatment and has been followed up to date (over 24 months). CONCLUSION: Because of its non-elevated serum PSA level, prostate SRCT is often ignored as a possibility of malignant tumor and regarded as benign prostatic hyperplasia (BPH). The possibility of prostate SRCT need to be considered if dysuria symptoms could not alleviate significantly after a period of oral treatment.


Assuntos
Terapia a Laser , Hiperplasia Prostática , Sarcoma , Masculino , Humanos , Idoso , Próstata , Disuria/cirurgia , Hibridização in Situ Fluorescente , Sarcoma/cirurgia , Hiperplasia Prostática/cirurgia , DNA Helicases , Proteínas Nucleares , Fatores de Transcrição
20.
J Plast Reconstr Aesthet Surg ; 91: 312-321, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38442511

RESUMO

Functional outcomes associated with prognostic factors and innervated muscle transplantation after wide soft tissue sarcoma resection remain unclear. We retrospectively examined the functional outcomes of reconstructive flap surgery for soft tissue sarcoma. Twenty patients underwent innervated muscle transplantation with pedicled or free flaps for functional reconstruction of resected muscles. Thirteen latissimus dorsi muscles and one vastus lateralis muscle combined with an anterolateral thigh flap were transferred as free flaps using the epi-perineural suture technique. Six latissimus dorsi muscles were transferred as pedicled flaps with neural continuity. Postoperative functional outcomes were assessed using the Musculoskeletal Tumor Society (MSTS) scores for the upper and lower extremities of 22 and 24 patients, respectively. The mean MSTS score for all patients was 82.3 at 12 months postoperatively. The mean scores for patients who underwent reconstruction with pedicled and free flaps were 89.2 and 77.1, respectively. The MSTS scores for the lower extremity, tumor size ≥5 cm, and free flap reconstruction were significantly lower than those for the upper extremity, tumor size <5 cm, and pedicled flap reconstruction (P = 0.02, 0.37, and 0.008, respectively). The postoperative MSTS score for innervated muscle transplantation was 76.7 at 12 months and was significantly higher (83.7) at 24 months (P = 0.003). Functional outcomes were significantly associated with tumor location, tumor size, and reconstructive flap type based on the MSTS scores. Innervated muscle transplantation improved functional outcomes at 24 months postoperatively via sufficient recovery of the innervated muscle, not the compensatory recovery of the remaining muscle.


Assuntos
Retalhos de Tecido Biológico , Sarcoma , Lesões dos Tecidos Moles , Neoplasias de Tecidos Moles , Humanos , Estudos Retrospectivos , Retalhos de Tecido Biológico/patologia , Neoplasias de Tecidos Moles/cirurgia , Músculo Quadríceps/transplante , Sarcoma/cirurgia , Sarcoma/patologia , Resultado do Tratamento
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