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1.
Surg Oncol Clin N Am ; 32(1): 169-184, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36410916

RESUMO

Soft-tissue sarcoma (STS) is not a single entity but, rather, a family of diseases with differing biologic behaviors and anatomic site- and histotype-specific responses to treatment. Whereas surgery remains the mainstay of treatment of primary, localized disease, evolving evidence is establishing the role of multimodality treatment of these tumors. This article summarizes prospective evidence to date informing our treatment of STS. Key future directions will include advancing our understanding of fundamental tumor biology and mechanisms of response and recurrence, as well as defining the optimal provision of regional, systemic, and targeted therapies, including the role of immunotherapy. Ongoing global collaborations will be integral to progress in treating these rare tumors.


Assuntos
Tumores do Estroma Gastrointestinal , Sarcoma , Neoplasias de Tecidos Moles , Humanos , Tumores do Estroma Gastrointestinal/cirurgia , Estudos Prospectivos , Neoplasias de Tecidos Moles/terapia , Neoplasias de Tecidos Moles/patologia , Sarcoma/terapia , Sarcoma/patologia , Imunoterapia
2.
Dermatol Clin ; 41(1): 133-140, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36410974

RESUMO

Cutaneous mesenchymal sarcomas are rare malignancies that include dermatofibrosarcoma protuberans, atypical fibroxanthoma, pleomorphic dermal sarcoma, cutaneous angiosarcoma, myofibrosarcoma, and leiomyosarcoma. These tumors lack consensus guidelines on staging and management. Treatment of local disease involves complete surgical removal but recurrence rates are higher compared with more common forms of nonmelanoma skin cancer. Cutaneous angiosarcoma, pleomorphic dermal sarcoma, and subcutaneous leiomyosarcoma have increased risk of metastatic spread and lower survival rate. Further research is needed on targeted therapies for these more aggressive sarcomas.


Assuntos
Hemangiossarcoma , Histiocitoma Fibroso Maligno , Leiomiossarcoma , Sarcoma , Neoplasias Cutâneas , Humanos , Hemangiossarcoma/patologia , Leiomiossarcoma/patologia , Neoplasias Cutâneas/cirurgia , Neoplasias Cutâneas/patologia , Sarcoma/cirurgia , Sarcoma/patologia
3.
Genes Chromosomes Cancer ; 62(1): 17-26, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-35801295

RESUMO

Next-generation sequencing (NGS) assays can sensitively detect somatic variation, and increasingly can enable the identification of complex structural rearrangements. A subset of infantile spindle cell sarcomas, particularly congenital mesoblastic nephromas with classic or mixed histology, have structural rearrangement in the form of internal tandem duplications (ITD) involving EGFR. We performed prospective analysis to identify EGFR ITD through clinical or research studies, as well as retrospective analysis to quantify the frequency of EGFR ITD in pediatric sarcomas. Within our institution, three tumors with EGFR ITD were prospectively identified, all occurring in patients less than 1 year of age at diagnosis, including two renal tumors and one mediastinal soft tissue tumor. These three cases exhibited both cellular and mixed cellular and classic histology. All patients had no evidence of disease progression off therapy, despite incomplete resection. To extend our analysis and quantify the frequency of EGFR ITD in pediatric sarcomas, we retrospectively analyzed a cohort of tumors (n = 90) that were previously negative for clinical RT-PCR-based fusion testing. We identified EGFR ITD in three analyzed cases, all in patients less than 1 year of age (n = 18; 3/18, 17%). Here we expand the spectrum of tumors with EGFR ITD to congenital soft tissue tumors and report an unusual example of an EGFR ITD in a tumor with cellular congenital mesoblastic nephroma histology. We also highlight the importance of appropriate test selection and bioinformatic analysis for identification of this genomic alteration that is unexpectedly common in congenital and infantile spindle cell tumors.


Assuntos
Neoplasias Renais , Nefroma Mesoblástico , Sarcoma , Neoplasias de Tecidos Moles , Recém-Nascido , Criança , Humanos , Estudos Retrospectivos , Nefroma Mesoblástico/genética , Nefroma Mesoblástico/congênito , Nefroma Mesoblástico/patologia , Neoplasias de Tecidos Moles/genética , Neoplasias Renais/genética , Neoplasias Renais/patologia , Sarcoma/genética , Sarcoma/patologia , Receptores ErbB/genética
5.
Zhonghua Bing Li Xue Za Zhi ; 51(11): 1141-1146, 2022 Nov 08.
Artigo em Chinês | MEDLINE | ID: mdl-36323544

RESUMO

Objective: To investigate the histopathologic, immunohistochemical, molecular genetic characteristics of CIC-rearranged sarcoma (CRS) with rhabdoid features. Methods: The clinical and pathologic data of two cases of CRS diagnosed between 2019 and 2021 at the Department of Pathology, Jiangsu Province Hospital were analyzed. Immunohistochemical study and fluorescence in situ hybridization (FISH) were performed. The relevant literature was reviewed. Results: Both patients were female, one was 58 years old, with tumor located in left thigh; the other was 43 years old, with tumor located in left pelvic cavity. Microscopically, both tumors were composed of small to medium-sized round, oval cells, arranged in nodules or sheets. The tumor cells showed irregular nuclear outline, coarse chromatin with prominent nucleoli and brisk mitotic activity. Both cases showed rhabdoid phenotype with myxoid stromal changes. Immunohistochemically, both cases were positive for CD99 and c-myc. High WT1 reactivity was seen in classic area, with low reactivity in rhabdoid area. There was no INI1 lost in both cases. Both were negative for NKX2.2 and NKX3.1. By FISH both cases demonstrated convincing break-apart signals of CIC gene. One patient died of disease after 1 month, and the other died of disease after 3 months. Conclusions: CRS is a small round cell undifferentiated sarcoma of the bone and soft tissue defined by molecular genetic characteristics, and may show atypical morphologic and immunophenotypic characteristics such as rhabdoid features. A correct understanding of its rare morphologic and immunophenotypic characteristics, combined with molecular pathologic detection, is conducive to correct diagnosis.


Assuntos
Sarcoma de Células Pequenas , Sarcoma , Feminino , Humanos , Masculino , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/análise , Hibridização in Situ Fluorescente , Sarcoma/patologia , Sarcoma de Células Pequenas/diagnóstico , Sarcoma de Células Pequenas/genética , Sarcoma de Células Pequenas/patologia , Fatores de Transcrição/genética , Tumor Rabdoide/diagnóstico , Tumor Rabdoide/genética , Tumor Rabdoide/patologia
6.
Turk J Med Sci ; 52(4): 1183-1189, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-36326382

RESUMO

BACKGROUND: Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma. The aim of this study is to present the results of the patients we treated with the diagnosis of EMC as an oncology reference center. METHODS: Information on 13 patients diagnosed with EMC between 2006 and 2018 was retrospectively reviewed. Patients' demographic information, tumor sizes, surgical treatments, chemotherapy and radiotherapy statuses, follow-up times, recurrences, and metastases were recorded. RESULTS: Mean patient age was 53.6 ± 15 years (range: 28-73). In 8 patients, the tumor was located in the lower limbs, most commonly in the thigh (46.2%). Mean follow-up period was 52.8 ± 19.9 (24-96) months. All patients underwent wide resections and only one had a positive surgical margin. In follow-up, 5 (38.5%) patients experienced recurrence; 6 patients had lung metastasis (46.2%) and 7 patients (53.8%) died. Mean tumor size was 10.4 ± 3.2 (5-17) cm. Median survival time was 61 (50.5-71.4) months and 5-year survival rate was 51.8%. There was no significant difference between survival times according to age, gender, side, limb location, postoperative radiotherapy, recurrence, or presence of lung metastasis. The cut-off value for death obtained by ROC analysis of tumor size was 11 cm. DISCUSSION: EMC is a rare soft tissue sarcoma with high local recurrence and metastasis capacity. Tumor size and metastatic disease are poor prognostic criteria. If it is a localized disease, the first option should be wide resection.


Assuntos
Condrossarcoma , Neoplasias Pulmonares , Sarcoma , Neoplasias de Tecidos Moles , Humanos , Adulto , Pessoa de Meia-Idade , Idoso , Estudos Retrospectivos , Condrossarcoma/cirurgia , Condrossarcoma/patologia , Neoplasias de Tecidos Moles/cirurgia , Neoplasias de Tecidos Moles/patologia , Sarcoma/patologia , Sarcoma/terapia , Neoplasias Pulmonares/cirurgia , Resultado do Tratamento
7.
Front Immunol ; 13: 922183, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36325324

RESUMO

ß-Carbolines are potentially strong alkaloids with a wide range of bioactivities, and their dimers exhibit stronger antitumor activity other than the monomers. However, the detailed mechanisms of the ß-carboline dimers in inhibiting sarcoma (SARC) remain unclear. The results showed that ß-carboline-3-carboxylic acid dimers Comp1 and Comp2, which were synthesized in our lab and modified at the N9 position and linked at the C3 position, exhibited effective inhibition activity on MG-63 proliferation (IC50 = 4.6µM). Meanwhile, the large scale transcriptome profiles of SARC from The Cancer Genome Atlas (TCGA) were analyzed, and found that abnormal expression of genes relevant to apoptosis, cell cycle, and signaling pathways of Hedgehog, HIF, Ras involved in the SARC pathogenesis. Interestingly, both dimers could promote the apoptosis and arrest the cell cycle in S phase to inhibit proliferation of MG-63. Moreover, Comp1 and Comp2 inhibited the expression CDK2, CCNA2, DBF4, and PLK1 associated with various immune cells and cell cycle in MG-63. Remarkably, drug-target interaction network analysis showed that numerous proteins involved in cell cycle were the potential targets of Comp1 and Comp2, especially CCNA2. Further molecular docking, isothermal titration calorimetry (ITC) and Cellular Thermal Shift Assay (CETSA) confirmed that both dimers could directly interact with CCNA2, which is significantly correlated with CD4+ T cells, by strong hydrophobic interactions (Kd=5.821 ×106 N). Meanwhile, the levels of CCNA2 and CDK2 were inhibited to decrease in MG-63 by both dimer treatments at transcription and protein levels, implying that Comp1 and Comp2 blocked the interaction between CCNA2 and CDK2 through competitive binding with CCNA2 to arrest the cell cycle of MG-63 cells in the S phase. Additionally, the transcriptome profiles of ß-carboline-treated mice from Gene Expression Omnibus (GEO) were obtained, and found that similar antitumor mechanism was shared among ß-carboline derivatives. Overall, our results elucidated the antitumor mechanisms of Comp1 and Comp2 through dual-suppressing the function of CCNA2 to profoundly arrest cell cycle of MG-63, then effectively inhibited cell proliferation of MG-63. These results provide new insights into the antitumor mechanism of ß-carboline dimers and new routes of various novel cancer-related drug targets for future possible cancer therapy.


Assuntos
Antineoplásicos , Sarcoma , Animais , Camundongos , Simulação de Acoplamento Molecular , Linhagem Celular Tumoral , Carbolinas/farmacologia , Carbolinas/química , Pontos de Checagem do Ciclo Celular , Proliferação de Células , Antineoplásicos/farmacologia , Antineoplásicos/química
8.
Radiat Oncol ; 17(1): 190, 2022 Nov 18.
Artigo em Inglês | MEDLINE | ID: mdl-36401312

RESUMO

BACKGROUND: Malignant peripheral nerve sheath tumors (MPNST) are sarcomas that arise from peripheral nerves. They generally have a poor prognosis which is exacerbated by high local recurrence rates. This case report discusses the treatment of a patient with a MPNST with local recurrence. This case report is novel due to the use of intraoperative Intrabeam™ (Zeiss, Dublin, CA) radiation therapy use in the protection of neurovascular structures such as the sciatic nerve. CASE PRESENTATION: The patient was a 65-year-old male who noticed a right posterior thigh mass slowly increasing in size over two months. A planned positive margin wide-resection excision was performed due to sciatic nerve abutment. The mass was determined to be a MPNST via postoperative pathology with positive margins along the sciatic nerve. The patient began adjuvant radiation therapy to the upper and lower thigh fields over a period of three months. Thirty-two months later, the patient was found to have a hypermetabolic mass with venous congestion and hyperemia at the prior surgical site which was confirmed by core needle biopsy to be local recurrence of the MPNST. Re-excision of the tumor was planned and performed followed by intraoperative Intrabeam™ radiation therapy. At two years of follow-up, the patient was doing well with minimal pain in his right buttock region with no new or recurrent neurological deficits. Radiologic imaging was negative for local recurrence of the MPNST. CONCLUSION: We believe this case report demonstrates a novel treatment strategy for sarcoma management. The unique use of intraoperative Intrabeam™ radiation therapy, which had not previously been used for this indication, may be efficacious in cases involving neurovascular structures. In this case, focal radiation from the intraoperative Intrabeam™ radiation device was used in a way to affect the recurrent tumor yet protect the sciatic nerve.


Assuntos
Neoplasias de Bainha Neural , Neurofibrossarcoma , Sarcoma , Humanos , Masculino , Idoso , Neurofibrossarcoma/complicações , Neurofibrossarcoma/patologia , Neoplasias de Bainha Neural/radioterapia , Neoplasias de Bainha Neural/cirurgia , Neoplasias de Bainha Neural/patologia , Nervo Isquiático/patologia , Dor , Sarcoma/radioterapia , Sarcoma/cirurgia
10.
Zhonghua Wai Ke Za Zhi ; 60(11): 1011-1017, 2022 Nov 01.
Artigo em Chinês | MEDLINE | ID: mdl-36323584

RESUMO

Objective: To investigate the method and effect of free anterolateral thigh myocutaneous flap combined with pedicled latissimus dorsi myocutaneous flap transfer for functional reconstruction after resection of huge shoulder tumor. Methods: The clinical data of 6 patients who were treated with pedicled latissimus dorsi myocutaneous flap combined with free anterolateral thigh myocutaneous flap to repair large-area complex defects after shoulder tumor resection at Department of Oncology Plastic Surgery, Hunan Province Cancer Hospital from December 2015 to December 2020 were retrospectively analyzed. There were 2 males and 4 females, with an average age of 41.7 years (range:29 to 56 years). There were 2 cases of synovial sarcoma,2 cases of phylloid cell sarcoma,1 case of liposarcoma and 1 case of fibrosarcoma. Before this operation, tumor resection had been performed for 1 to 5 times on each case,and the course of disease was 6 to 24 months. Pedicled latissimus dorsi myocutaneous flap combined with free anterolateral thigh myocutaneous flap were used to repair soft tissue defects and reconstruct deltoid function. Postoperative flap status, complications, appearance and function of upper limbs and tumor recurrence were recorded. Results: Six patients were followed up for an average of 21.6 months (range: 12 to 36 months). There were no serious complications after operation,and all flaps survived. No tumor recurrence was found. The appearance of shoulder contour reconstructed by flaps was satisfactory. The reinnervation effect of lateral femoral muscle was confirmed recovered smoothly by neuroelectromyography 3 months after operation. Shoulder function was mildly limited in 3 patients,moderately limited in 2 patients and severely limited in 1 patient. All patients reported significant improvement in shoulder discomfort.The overall functional results of all patients were satisfactory. Conclusion: Combined myocutaneous flaps transplantation can perfectly repair the wound left after the resection of huge shoulder tumor,minimize the recurrence of tumor,reconstruct the function of shoulder joint and greatly improve the quality of life of patients.


Assuntos
Mamoplastia , Retalho Miocutâneo , Procedimentos Cirúrgicos Reconstrutivos , Sarcoma , Lesões dos Tecidos Moles , Músculos Superficiais do Dorso , Masculino , Feminino , Humanos , Adulto , Coxa da Perna , Ombro , Qualidade de Vida , Estudos Retrospectivos , Transplante de Pele , Procedimentos Cirúrgicos Reconstrutivos/métodos , Extremidade Superior , Resultado do Tratamento
11.
Medicine (Baltimore) ; 101(44): e31360, 2022 Nov 04.
Artigo em Inglês | MEDLINE | ID: mdl-36343027

RESUMO

RATIONALE: Myxoid pleomorphic liposarcoma (MPL) is a rare aggressive adipocytic tumor that mainly presents in children and adolescents. It is most frequently observed in the mediastinum and rarely in the head and neck, perineal region, or back. Herein, we report the first published case of MPL of the teres minor muscle. PATIENT CONCERNS: A 24-years-old woman presented with a painless palpable mass in her right shoulder. DIAGNOSES: Magnetic resonance imaging identified a 9.0 × 7.0 × 4.0 cm mass suspected to be a sarcoma in the teres minor muscle. Positron emission tomography/computed tomography revealed no evidence of distant metastasis. Histopathological examination revealed the mass to be an MPL, which was assigned a histologic grade of 3 according to the French Federation of Cancer Centers Sarcoma Group system. No tumor cells were observed along the resected margins. INTERVENTIONS: Under general anesthesia, the right teres minor muscle containing the mass was excised en bloc and frozen biopsy confirmed that the tumor cells did not invade the surrounding tissues. OUTCOMES: The patient underwent radiotherapy and was followed up for 6 months without complications. LESSONS: Although MPL in the teres minor muscle is rare, it should be considered in the differential diagnosis in patients with a mass in the teres minor muscle due to its poor prognosis.


Assuntos
Lipossarcoma Mixoide , Lipossarcoma , Sarcoma , Neoplasias de Tecidos Moles , Humanos , Feminino , Criança , Adolescente , Adulto Jovem , Adulto , Manguito Rotador/patologia , Lipossarcoma/patologia , Neoplasias de Tecidos Moles/patologia , Sarcoma/diagnóstico , Diagnóstico Diferencial , Lipossarcoma Mixoide/diagnóstico , Lipossarcoma Mixoide/cirurgia , Lipossarcoma Mixoide/patologia
12.
Clin Epigenetics ; 14(1): 149, 2022 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-36380356

RESUMO

BACKGROUND: Sarcomas are a heterogeneous group of rare malignant tumors with more than 100 subtypes. Accurate diagnosis remains challenging due to a lack of characteristic molecular or histomorphological hallmarks. A DNA methylation-based tumor profiling classifier for sarcomas (known as sarcoma classifier) from the German Cancer Research Center (Deutsches Krebsforschungszentrum) is now employed in selected cases to guide tumor classification and treatment decisions at our institution. Data on the usage of the classifier in daily clinical routine are lacking. METHODS: In this single-center experience, we describe the clinical course of five sarcoma cases undergoing thorough pathological and reference pathological examination as well as DNA methylation-based profiling and their impact on subsequent treatment decisions. We collected data on the clinical course, DNA methylation analysis, histopathology, radiological imaging, and next-generation sequencing. RESULTS: Five clinical cases involving DNA methylation-based profiling in 2021 at our institution were included. All patients' DNA methylation profiles were successfully matched to a methylation profile cluster of the sarcoma classifier's dataset. In three patients, the classifier reassured diagnosis or aided in finding the correct diagnosis in light of contradictory data and differential diagnoses. In two patients with intracranial tumors, the classifier changed the diagnosis to a novel diagnostic tumor group. CONCLUSIONS: The sarcoma classifier is a valuable diagnostic tool that should be used after comprehensive clinical and histopathological evaluation. It may help to reassure the histopathological diagnosis or indicate the need for thorough reassessment in cases where it contradicts previous findings. However, certain limitations (non-classifiable cases, misclassifications, unclear degree of sample purity for analysis and others) currently preclude wide clinical application. The current sarcoma classifier is therefore not yet ready for a broad clinical routine. With further refinements, this promising tool may be implemented in daily clinical practice in selected cases.


Assuntos
Metilação de DNA , Sarcoma , Humanos , Sarcoma/diagnóstico , Sarcoma/genética , Sarcoma/patologia , Sequenciamento de Nucleotídeos em Larga Escala , Diagnóstico Diferencial
13.
Sci Rep ; 12(1): 19612, 2022 Nov 16.
Artigo em Inglês | MEDLINE | ID: mdl-36385486

RESUMO

Uterine sarcomas have very poor prognoses and are sometimes difficult to distinguish from uterine leiomyomas on preoperative examinations. Herein, we investigated whether deep neural network (DNN) models can improve the accuracy of preoperative MRI-based diagnosis in patients with uterine sarcomas. Fifteen sequences of MRI for patients (uterine sarcoma group: n = 63; uterine leiomyoma: n = 200) were used to train the models. Six radiologists (three specialists, three practitioners) interpreted the same images for validation. The most important individual sequences for diagnosis were axial T2-weighted imaging (T2WI), sagittal T2WI, and diffusion-weighted imaging. These sequences also represented the most accurate combination (accuracy: 91.3%), achieving diagnostic ability comparable to that of specialists (accuracy: 88.3%) and superior to that of practitioners (accuracy: 80.1%). Moreover, radiologists' diagnostic accuracy improved when provided with DNN results (specialists: 89.6%; practitioners: 92.3%). Our DNN models are valuable to improve diagnostic accuracy, especially in filling the gap of clinical skills between interpreters. This method can be a universal model for the use of deep learning in the diagnostic imaging of rare tumors.


Assuntos
Aprendizado Profundo , Leiomioma , Neoplasias Pélvicas , Sarcoma , Neoplasias de Tecidos Moles , Neoplasias Uterinas , Feminino , Humanos , Diagnóstico Diferencial , Sensibilidade e Especificidade , Neoplasias Uterinas/diagnóstico por imagem , Neoplasias Uterinas/patologia , Leiomioma/patologia , Sarcoma/diagnóstico por imagem , Sarcoma/patologia , Neoplasias de Tecidos Moles/diagnóstico
14.
Sci Rep ; 12(1): 19527, 2022 Nov 14.
Artigo em Inglês | MEDLINE | ID: mdl-36376458

RESUMO

Fifteen years have passed since the soft tissue tumor practice guidelines were first published in Japan. Tumor size of ≥ 5 cm and tumor depth were key findings suggestive of malignant soft tissue tumors. We reviewed the referral documents provided by the referring physicians to see if these two findings were reported. The study was conducted from January 2007 to December 2021 and included 142 patients (83 men and 59 women; median age, 64 [6-94] years) with malignant soft tissue tumors. Patient referral documents from physicians were screened for descriptions of the tumor size and depth. The tumor size, depth, and both were described in 51.4%, 36.6%, and 23.2% of the referrals, respectively. Both findings were mentioned in 23.8%, 21.7%, and 25.0% of referrals in 2007-2011, 2012-2016, and 2017-2021, respectively. Of orthopedic surgeons and other physicians, 61.2% and 38.6%, respectively, described the tumor size. Whether the general physicians could follow the soft tissue tumor practice guidelines was difficult to conclude by reviewing patient referral documents. However, orthopedic surgeons seemed to pay more attention to tumor size. Awareness regarding soft tissue tumor practice guidelines should be increased to help diagnose malignant soft tissue tumors early.


Assuntos
Clínicos Gerais , Cirurgiões Ortopédicos , Sarcoma , Neoplasias de Tecidos Moles , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Encaminhamento e Consulta , Sarcoma/diagnóstico , Neoplasias de Tecidos Moles/patologia , Criança , Adolescente , Adulto Jovem , Adulto , Idoso , Idoso de 80 Anos ou mais
15.
JMIR Mhealth Uhealth ; 10(11): e40718, 2022 Nov 09.
Artigo em Inglês | MEDLINE | ID: mdl-36350680

RESUMO

BACKGROUND: According to guidelines, all patients with sarcoma must be managed from initial diagnosis at expert sarcoma centers. However, in everyday practice, the time interval to an expert center visit can be long, which delays presentation to an expert multidisciplinary tumor board and increases the risk of inappropriate management, negatively affecting local tumor control and prognosis. The advent of mobile health offers an easy way to facilitate communication and cooperation between general health care providers (eg, general practitioners and radiologists) and sarcomas experts. We developed a mobile app (Sar'Connect) based on the algorithm designed by radiologists from the French Sarcoma Group. Through a small number of easy-to-answer questions, Sar'Connect provides personalized advice for the management of patients and contact information for the closest expert center. OBJECTIVE: This retrospective study is the first to assess this mobile app's potential benefits in reducing the time interval for patient referral to an expert center according to the initial clinical characteristics of the soft tissue tumor. METHODS: From May to December 2021, we extracted tumor mass data for 78 patients discussed by the multidisciplinary tumor boards at 3 centers of the French Sarcoma Group. We applied the Sar'Connect algorithm to these data and estimated the time interval between the first medical description of the soft tissue mass and the referral to expert center. We then compared this estimated time interval with the observed time interval. RESULTS: We found that the use of Sar'Connect could potentially shorten the time interval to an expert center by approximately 7.5 months (P<.001). Moreover, for half (31/60, 52%) of the patients with a malignant soft tissue tumor, Sar'Connect could have avoided inappropriate management outside of the reference center. We did not identify a significant determinant for shortening the time interval for referral. CONCLUSIONS: Overall, promoting the use of a simple mobile app is an innovative and straightforward means to potentially accelerate both the referral and management of patients with soft tissue sarcoma at expert centers.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Telemedicina , Humanos , Estudos Retrospectivos , Neoplasias de Tecidos Moles/terapia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/patologia , Sarcoma/terapia , Encaminhamento e Consulta
16.
BMJ Case Rep ; 15(11)2022 Nov 04.
Artigo em Inglês | MEDLINE | ID: mdl-36332932

RESUMO

Spindle cell carcinoma is a subtype of sarcomatoid carcinoma, which has previously been described in various anatomical locations, though rarely in the trachea.We present the case of a woman in her 70s who presented with a sore throat and stridor. Fibreoptic nasendoscopy demonstrated a tracheal mass occupying 80% of the airway from the cricoid cartilage to the third tracheal ring, infiltrating the thyroid gland. Subsequent CT demonstrated pulmonary emboli and vertebral metastasis. Biopsy of the infiltrated thyroid confirmed the diagnosis of spindle cell carcinoma. The length of the tumour and metastasis at presentation made this surgically unresectable, and she was referred for a palliative stent but died after an acute deterioration.This pathology has been reported only five times previously in the literature, with management strategies varying greatly between patients. Primary tracheal tumours are difficult to manage as, due to their rarity, there are no clear guidelines.


Assuntos
Carcinoma , Sarcoma , Neoplasias de Tecidos Moles , Neoplasias da Glândula Tireoide , Neoplasias da Traqueia , Feminino , Humanos , Traqueia/diagnóstico por imagem , Traqueia/patologia , Neoplasias da Traqueia/diagnóstico por imagem , Neoplasias da Traqueia/cirurgia , Sarcoma/diagnóstico por imagem , Sarcoma/cirurgia , Carcinoma/patologia , Neoplasias de Tecidos Moles/patologia , Neoplasias da Glândula Tireoide/diagnóstico , Neoplasias da Glândula Tireoide/cirurgia , Neoplasias da Glândula Tireoide/patologia
18.
Elife ; 112022 11 03.
Artigo em Inglês | MEDLINE | ID: mdl-36326232

RESUMO

Background: Sarcomas comprise approximately 1% of all human malignancies; treatment resistance is one of the major reasons for the poor prognosis of sarcomas. Accumulating evidence suggests that non-coding RNAs (ncRNAs), including miRNAs, long ncRNAs, and circular RNAs, are important molecules involved in the crosstalk between resistance to chemotherapy, targeted therapy, and radiotherapy via various pathways. Methods: We searched the PubMed (MEDLINE) database for articles regarding sarcoma-associated ncRNAs from inception to August 17, 2022. Studies investigating the roles of host-derived miRNAs, long ncRNAs, and circular RNAs in sarcoma were included. Data relating to the roles of ncRNAs in therapeutic regulation and their applicability as biomarkers for predicting the therapeutic response of sarcomas were extracted. Two independent researchers assessed the quality of the studies using the Würzburg Methodological Quality Score (W-MeQS). Results: Observational studies revealed the ectopic expression of ncRNAs in sarcoma patients who had different responses to antitumor treatments. Experimental studies have confirmed crosstalk between cellular pathways pertinent to chemotherapy, targeted therapy, and radiotherapy resistance. Of the included studies, W-MeQS scores ranged from 3 to 10 (average score = 5.42). Of the 12 articles that investigated ncRNAs as biomarkers, none included a validation cohort. Selective reporting of the sensitivity, specificity, and receiver operating curves was common. Conclusions: Although ncRNAs appear to be good candidates as biomarkers for predicting treatment response and therapeutics for sarcoma, their differential expression across tissues complicates their application. Further research regarding their potential for inhibiting or activating these regulatory molecules to reverse treatment resistance may be useful. Funding: This study's literature retrieval was supported financially by the 345 Talent Project of Shengjing Hospital of China Medical University (M0949 to Tao Zhang).


Assuntos
MicroRNAs , RNA Longo não Codificante , Sarcoma , Humanos , RNA Circular/genética , RNA não Traduzido/genética , Sarcoma/genética , Sarcoma/radioterapia , MicroRNAs/metabolismo , Biomarcadores/metabolismo
19.
BMC Musculoskelet Disord ; 23(1): 1012, 2022 Nov 24.
Artigo em Inglês | MEDLINE | ID: mdl-36424560

RESUMO

BACKGROUND: During pelvic Sarcoma resections, Surgeons often struggle to obtain negative margins while minimizing collateral damage and maintaining limb function. These complications are usually due to the complex anatomy of the pelvis. Here we present an accurate 3D surgical approach, including pre-operative printing of models and intraoperative patient-specific instruments (PSIs) for optimizing pelvic sarcoma resections. METHODS: This single-center retrospective study (N = 11) presents surgical, functional, and oncological outcomes of patients (average age 14.6 +/- 7.6 years, 4 males) who underwent pelvic sarcoma resections using a 3D surgical approach between 2016 and 2021. All patients were followed up for at least 24 months (mean = 38.9 +/- 30.1 months). RESULTS: Our results show promising surgical, oncological, and functional outcomes. Using a 3D approach, 90.9% had negative margins, and 63.6% did not require reconstruction surgery. The average estimated blood loss was 895.45 ± 540.12 cc, and the average surgery time was 3:38 ± 0.05 hours. Our results revealed no long-term complications. Three patients suffered from short-term complications of superficial wound infections. At 24 month follow up 72.7% of patients displayed no evidence of disease. The average Musculoskeletal Tumor Society (MSTS) score at 12 months was 22.81. CONCLUSION: 3D technology enables improved accuracy in tumor resections, allowing for less invasive procedures and tailored reconstruction surgeries, potentially leading to better outcomes in function and morbidity. We believe that this approach will enhance treatments and ease prognosis for patients diagnosed with pelvic sarcoma and will become the standard of care in the future.


Assuntos
Neoplasias Ósseas , Hemipelvectomia , Osteossarcoma , Sarcoma , Neoplasias de Tecidos Moles , Masculino , Humanos , Criança , Adolescente , Adulto Jovem , Adulto , Hemipelvectomia/métodos , Salvamento de Membro , Estudos Retrospectivos , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/cirurgia , Resultado do Tratamento , Osteossarcoma/diagnóstico por imagem , Osteossarcoma/cirurgia , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/cirurgia , Margens de Excisão
20.
BMC Cancer ; 22(1): 1075, 2022 Oct 18.
Artigo em Inglês | MEDLINE | ID: mdl-36258189

RESUMO

BACKGROUND: Coagulation and fibrinolysis are distinct processes that are highly correlated. Cells control coagulation and fibrinolysis by expression of tissue factor and urokinase-type plasminogen activator receptor on their surface. Tumor cells express these proteins, adjust their microenvironment and induce tumor exacerbation. We hypothesized that the expression of plasma markers for coagulation and fibrinolysis in patients with soft tissue sarcomas (STSs) was dependent on the level of tumor malignancy. To elucidate which markers are predictive of recurrence, metastasis and prognosis, coagulation or fibrinolysis, we analyzed the correlation between plasma levels of thrombin-antithrombin III complex (TAT), soluble fibrin (SF), plasmin-α2 plasmin inhibitor complex (PIC), D-dimer (DD) and clinical parameters in patients with STSs. METHODS: TAT, SF, PIC or DD were measured in pre-treatment blood samples from 64 patients with primary STSs and analyzed with clinicopathological parameters, and 5-year recurrence free survival (RFS), 5-year metastasis free survival (MFS) and 5-year overall survival (OS) were evaluated. RESULTS: The metastasis group had significantly higher DD (p = 0.0394), PIC (p = 0.00532) and SF (p = 0.00249) concentrations than the group without metastasis. The group that died of disease showed significantly higher DD (p = 0.00105), PIC (p = 0.000542), SF (p = 0.000126) and TAT (p = 0.0373) than surviving patients. By dividing the patients into low and high groups, the group with high DD, PIC, SF and TAT showed significantly lower 5-year MFS and 5-year OS than the corresponding low group. Furthermore, in multivariate COX proportional hazard analysis of continuous variables for 5-year MFS, only PIC was found to be a significant factor (HR: 2.14). CONCLUSION: Fibrinolysis was better than coagulation at reflecting the disease condition of patients with STS. Notably, PIC levels ≥ 1.1 can not only predict the risk of metastasis and poor prognosis, but also increasing PIC levels correspond to further increases in risks of metastasis and poor prognosis.


Assuntos
Antifibrinolíticos , Sarcoma , Humanos , Fibrinólise , Fibrinolisina/metabolismo , Receptores de Ativador de Plasminogênio Tipo Uroquinase , Tromboplastina , Peptídeo Hidrolases , Biomarcadores , Microambiente Tumoral
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