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1.
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
2.
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
3.
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
4.
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
5.
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
6.
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
7.
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
8.
Curr Oncol ; 29(10): 7598-7606, 2022 Oct 13.
Artigo em Inglês | MEDLINE | ID: mdl-36290877

RESUMO

BACKGROUND AND OBJECTIVES: Functional outcomes are important for oncology patients undergoing lower extremity reconstruction. The objective of the current study was to describe patient reported function after surgery and identify predictors of postoperative function in musculoskeletal oncology patients undergoing lower extremity endoprosthetic reconstruction. METHODS: We performed a cohort study with functional outcome data from the recently completed Prophylactic Antibiotic Regimens in Tumor Surgery (PARITY) trial. We utilized the 100-point Toronto Extremity Salvage Score (TESS), which was administered pre-operatively and at 3, 6 and 12 months post-operatively. Higher scores indicate better physical functioning, and the minimally important difference is 11 points. We calculated mean functional scores at each timepoint after surgery and developed a logistic regression model to explore predictors of failure to achieve excellent post-operative function (TESS ≥ 80) at 1 year after surgery. RESULTS: The 555 patients included in our cohort showed important functional improvement from pre-surgery to 1 year post-surgery (mean difference 14.9 points, 95%CI 12.2 to 17.6; p < 0.001) and 64% achieved excellent post-operative function. Our adjusted regression model found that poor (TESS 0-39) pre-operative function (odds ratio [OR] 3.3, 95%CI 1.6 to 6.6); absolute risk [AR] 24%, 95%CI 8% to 41.2%), older age (OR per 10-year increase from age 12, 1.32, 95%CI 1.17, 1.49; AR 4.5%, 95%CI 2.4% to 6.6%), and patients undergoing reconstruction for soft-tissue sarcomas (OR 2.3, 95%CI 1.03 to 5.01; AR 15.3%, 95%CI 0.4% to 34.4%), were associated with higher odds of failing to achieve an excellent functional outcome at 1-year follow-up. Patients undergoing reconstruction for giant cell tumors were more likely to achieve an excellent functional outcome post-operatively (OR 0.40, 95%CI 0.17 to 0.95; AR -9.9%, 95%CI -14.4% to -0.7%). CONCLUSIONS: The majority of patients with tumors of the lower extremity undergoing endoprosthetic reconstruction achieved excellent function at 1 year after surgery. Older age, poor pre-operative function, and endoprosthetic reconstruction for soft tissue sarcomas were associated with worse outcomes; reconstruction for giant cell tumors was associated with better post-operative function. LEVEL OF EVIDENCE: Therapeutic Level IV.


Assuntos
Tumores de Células Gigantes , Sarcoma , Neoplasias de Tecidos Moles , Humanos , Salvamento de Membro , Estudos de Coortes , Resultado do Tratamento , Sarcoma/cirurgia , Sarcoma/patologia , Extremidade Inferior/cirurgia , Extremidade Inferior/patologia , Tumores de Células Gigantes/cirurgia , Antibacterianos
9.
Int J Mol Sci ; 23(19)2022 Sep 20.
Artigo em Inglês | MEDLINE | ID: mdl-36232302

RESUMO

We assess the performance of mRNA capture sequencing to identify fusion transcripts in FFPE tissue of different sarcoma types, followed by RT-qPCR confirmation. To validate our workflow, six positive control tumors with a specific chromosomal rearrangement were analyzed using the TruSight RNA Pan-Cancer Panel. Fusion transcript calling by FusionCatcher confirmed these aberrations and enabled the identification of both fusion gene partners and breakpoints. Next, whole-transcriptome TruSeq RNA Exome sequencing was applied to 17 fusion gene-negative alveolar rhabdomyosarcoma (ARMS) or undifferentiated round cell sarcoma (URCS) tumors, for whom fluorescence in situ hybridization (FISH) did not identify the classical pathognomonic rearrangements. For six patients, a pathognomonic fusion transcript was readily detected, i.e., PAX3-FOXO1 in two ARMS patients, and EWSR1-FLI1, EWSR1-ERG, or EWSR1-NFATC2 in four URCS patients. For the 11 remaining patients, 11 newly identified fusion transcripts were confirmed by RT-qPCR, including COPS3-TOM1L2, NCOA1-DTNB, WWTR1-LINC01986, PLAA-MOB3B, AP1B1-CHEK2, and BRD4-LEUTX fusion transcripts in ARMS patients. Additionally, recurrently detected secondary fusion transcripts in patients diagnosed with EWSR1-NFATC2-positive sarcoma were confirmed (COPS4-TBC1D9, PICALM-SYTL2, SMG6-VPS53, and UBE2F-ALS2). In conclusion, this study shows that mRNA capture sequencing enhances the detection rate of pathognomonic fusions and enables the identification of novel and secondary fusion transcripts in sarcomas.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Complexo 1 de Proteínas Adaptadoras/genética , Subunidades beta do Complexo de Proteínas Adaptadoras , Proteínas de Ciclo Celular/genética , Ácido Ditionitrobenzoico , Humanos , Hibridização in Situ Fluorescente , Proteínas Nucleares/genética , Proteínas de Fusão Oncogênica/genética , RNA , RNA Mensageiro/genética , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Sarcoma/diagnóstico , Sarcoma/genética , Sarcoma/patologia , Neoplasias de Tecidos Moles/patologia , Fatores de Transcrição/genética
10.
Int J Mol Sci ; 23(19)2022 Sep 24.
Artigo em Inglês | MEDLINE | ID: mdl-36232574

RESUMO

Sarcomas are malignant tumors of mesenchymal origin that can occur at any age. The rarity of these tumors in combination with the vast number of histological subtypes render the study of sarcomas challenging. Organoids represent complex three-dimensional cell culture systems, deriving from stem cells and preserving the capacity to differentiate into the cell types of their tissue of origin. The aim of the present review is to study the current status of patient-derived organoids, as well as their potential to model tumorigenesis and perform drug screenings for sarcomas. In order to identify relevant studies, a literature review was conducted and we were able to identify 16 studies published between 2019 and 2022. The current manuscript represents the first comprehensive review of the literature focusing on the use of organoids for disease modelling and drug sensitivity testing in diverse sarcoma subtypes.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Transformação Celular Neoplásica , Humanos , Organoides/patologia , Sarcoma/diagnóstico , Sarcoma/patologia , Sarcoma/terapia , Células-Tronco/patologia
11.
Tumori ; 108(6): NP26-NP29, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36239461

RESUMO

INTRODUCTION: Primary sarcoma of the vulva is an extremely rare entity, representing only 1%-3% of all vulvar malignant neoplasms. Among sarcomas, leiomyosarcoma (LMS) is the most prevalent histologic variant. Due to the rarity of LMS, guidelines are lacking and phase III trials have not been carried out, so clinical management is based on local clinical practice and physician experience. CASE PRESENTATION: Here, we described a case of primary LMS of the vulva and its successful management, with the adoption of neoadjuvant chemotherapy and surgery. We report a case of a 74-year-old woman with 12.5 cm vulvar LMS. The patient received three cycles of neoadjuvant chemotherapy with a partial response. Radical vulvectomy with vulvar reconstruction with V-F flap was carried out. Surgical margins were negative. Three additional cycles of adjuvant chemotherapy were delivered. RESULTS: One year after treatment, the patient was disease-free. CONCLUSION: There are no approved therapeutic protocols for this rare neoplasia. Surgery is the mainstay of treatment. However, it is not always feasible, so neoadjuvant chemotherapy was delivered for downstaging the vulvar lesion. We suppose that neoadjuvant chemotherapy has optimized the possibilities of radical surgery. Despite the anectodical nature of this case presentation, neoadjuvant chemotherapy seems a valid therapeutic option for managing patients with bulky vulvar sarcoma. Further large collaborative studies are warranted to identify the best therapeutic option for these patients.


Assuntos
Leiomiossarcoma , Sarcoma , Neoplasias Vulvares , Feminino , Humanos , Idoso , Leiomiossarcoma/diagnóstico , Leiomiossarcoma/tratamento farmacológico , Leiomiossarcoma/cirurgia , Neoplasias Vulvares/tratamento farmacológico , Neoplasias Vulvares/cirurgia , Vulva/patologia , Vulva/cirurgia , Terapia Neoadjuvante , Sarcoma/patologia
12.
BMC Cancer ; 22(1): 1034, 2022 Oct 03.
Artigo em Inglês | MEDLINE | ID: mdl-36192725

RESUMO

BACKGROUND: This French nationwide NETSARC exhaustive prospective cohort aims to explore the impact of systematic re-excision (RE) as adjuvant care on overall survival (OS), local recurrence free survival (LRFS), and local and distant control (RFS) in patients with soft tissue sarcoma (STS) with positive microscopic margins (R1) after initial resection performed outside of a reference center. METHODS: Eligible patients had experienced STS surgery outside a reference center from 2010 to 2017, and had R1 margins after initial surgery. Characteristics and treatment comparisons used chi-square for categorical variables and Kruskall-Wallis test for continuous data. Survival distributions were compared in patients reexcised (RE) or not (No-RE) using a log-rank test. A Cox proportional hazard model was used for subgroup analysis. RESULTS: A total of 1,284 patients had experienced initial STS surgery outside NETSARC with R1 margins, including 1,029 patients with second operation documented. Among the latter, 698 patients experienced re-excision, and 331 were not re-excised. Characteristics were significantly different regarding patient age, tumor site, tumor size, tumor depth, and histotype in the population of patients re-excised (RE) or not (No-RE). The study identified RE as an independent favorable factor for OS (HR 0.36, 95%CI 0.23-0.56, p<0.0001), for LRFS (HR 0.45, 95%CI 0.36-0.56, p<0.0001), and for RFS (HR 0.35, 95%CI 0.26-0.46, p<0.0001). CONCLUSION: This large nationwide series shows that RE improved overall survival in patients with STS of extremities and trunk wall, with prior R1 resection performed outside of a reference center. RE as part of adjuvant care should be systematically considered.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Estudos de Coortes , Extremidades/patologia , Extremidades/cirurgia , Humanos , Margens de Excisão , Recidiva Local de Neoplasia/patologia , Prognóstico , Estudos Prospectivos , Estudos Retrospectivos , Sarcoma/patologia , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/cirurgia
13.
Medicine (Baltimore) ; 101(39): e30688, 2022 Sep 30.
Artigo em Inglês | MEDLINE | ID: mdl-36181081

RESUMO

The prognosis for soft tissue sarcomas (STSs) is poor, especially for highly aggressive STSs, and the details of prognostic factors are unknown. This study aimed to investigate the prognostic factors for STSs in hematologic inflammatory markers. We included 22 patients with STSs treated at our institution. The STSs were histologically classified as follows: undifferentiated pleomorphic sarcoma, 7 cases; myxofibrosarcoma, 6 cases; and malignant peripheral nerve sheath tumor, 2 cases. The average patient age was 72.06 years. The numbers of patients who underwent each procedure were as follows: wide resection, 7; wide resection and flap, 2; marginal resection, 2; wide resection and radiation, 1; additional wide resection with flap, 1; wide resection and skin graft, 1; and radiotherapy only, 1. The median follow-up period was 26 months (3-92 months). The outcomes were as follows: continuous disease free, 6 cases; no evidence of disease, 6 cases; alive with disease, 1 case; and died of disease, 2 cases. Pretreatment blood examinations for C-reactive protein (CRP) and albumin levels; neutrophil, lymphocyte, and white blood cell (WBC) counts; and neutrophil/lymphocyte (N/L) ratio were investigated and correlated with tumor size, tissue grade, and maximum standardized uptake value (SUVmax). CRP level and neutrophil and WBC counts were positively correlated with tissue grade and SUVmax. N/L ratio was positively correlated with tumor size and SUVmax. CRP level, WBC and neutrophil counts, and N/L ratio may be poor prognostic factors for highly aggressive STSs.


Assuntos
Fibrossarcoma , Histiocitoma Fibroso Maligno , Sarcoma , Neoplasias de Tecidos Moles , Idoso , Biomarcadores , Proteína C-Reativa , Humanos , Prognóstico , Estudos Retrospectivos , Sarcoma/patologia , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/cirurgia
14.
Sci Rep ; 12(1): 17129, 2022 Oct 12.
Artigo em Inglês | MEDLINE | ID: mdl-36224239

RESUMO

Sarcoma is a rare cancer, and little is known about the etiology, lifestyle epidemiology, and actual circumstances of treatment in hospitals in Japan. Understanding these issues is essential for the effective prevention and treatment of sarcoma. We therefore investigated the incidence of a personal and family cancer history in a total of 1320 sarcoma patients at the National Cancer Center Hospital. In addition, obesity, hypertension, dyslipidemia, diabetes mellitus, drinking, smoking, age and sex were compared in a descriptive study of 1159 of these sarcoma patients who were ≥ 20 years of age, and 7738 controls derived from the National Health and Nutrition Examination Survey in Japan. A total of 8% of sarcoma patients had a personal history of another cancer, and 30% of soft tissue sarcoma patients had a family cancer history in a first-degree relative (malignant peripheral nerve sheath tumor, 52%; leiomyosarcoma, 46%). A smoking habit was associated with the development of sarcoma (odds ratio [OR], 2.05; 95% confidence interval, 1.78-2.37; p < 0.01). According to the histology, the ORs for undifferentiated pleomorphic sarcoma (UPS) of bone, UPS of soft tissue, and liposarcoma were 5.71, 3.04, and 2.92, respectively. A family cancer history may be associated with certain soft tissue sarcomas, and a smoking habit was significantly associated with the development of sarcomas; however, further studies are necessary.


Assuntos
Histiocitoma Fibroso Maligno , Sarcoma , Neoplasias de Tecidos Moles , Humanos , Japão/epidemiologia , Inquéritos Nutricionais , Sarcoma/epidemiologia , Sarcoma/etiologia , Sarcoma/patologia , Fumar/efeitos adversos , Neoplasias de Tecidos Moles/patologia
15.
Indian J Pathol Microbiol ; 65(4): 864-868, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36308195

RESUMO

Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Anaplasia is a rare phenomenon seen in childhood RMS. The most common histologic subtype was Embryonal followed by Alveolar and spindle subtype. Design: A total of 11 cases of pediatric RMS were selected from January 2017 to June 2019 presenting at various sites. Out of 11 cases, 2 were further diagnosed as Embryonal, 2 as Alveolar, 2 as Pleomorphic, 1 as Spindle subtype and rest 4 as RMS-NOS based on morphology. All cases were positive for Desmin. The presence of cells with lobated, hyperchromatic nuclei at least three times larger than the tumor cell (anaplastic cells) was selected as the main criterion to diagnose Anaplasia. Results: Out of the total 11 cases, anaplasia was seen in 7 cases. Out of these seven, five cases showed Focal Anaplasia (FA) (71.4%) and 2 cases showed Diffuse Anaplasia (DA) (28.6%). Out of 2 cases of Embryonal RMS one exhibited focal anaplasia (50%). One case of Spindle RMS showed diffuse anaplasia, 2 cases of pleomorphic RMS showed focal anaplasia. Out of 3 cases of RMS- NOS, 2 exhibited focal anaplaisa and one displayed Diffuse anaplasia. Both Alveolar RMS had no features of anaplasia. Conclusion: Presence of Anaplasia is a frequent observation in pediatric RMS. Anaplasia is often under reported in pediatric RMS. Pathologist should be more aware of this rare phenomenon.


Assuntos
Rabdomiossarcoma Alveolar , Rabdomiossarcoma , Sarcoma , Neoplasias de Tecidos Moles , Criança , Humanos , Anaplasia , Rabdomiossarcoma/diagnóstico , Rabdomiossarcoma/patologia , Sarcoma/patologia , Neoplasias de Tecidos Moles/patologia , Rabdomiossarcoma Alveolar/diagnóstico , Rabdomiossarcoma Alveolar/patologia
16.
In Vivo ; 36(6): 2965-2972, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36309350

RESUMO

BACKGROUND/AIM: Sinonasal rhabdomyosarcoma (RMS) is a rare soft tissue malignancy. Due to the limited cases, the clinicopathological features and prognostic factors are still not well understood. PATIENTS AND METHODS: This retrospective review included eight patients with sinonasal RMS at our institution between 2004 and 2020. Patient demographics, tumor features, Intergroup Rhabdomyosarcoma Study Group (IRSG) stage and clinical group, treatment strategy, and survival rates were evaluated. Kaplan-Meier analysis and log-rank tests were performed to analyze the possible prognostic factors. RESULTS: We observed a predominance of male sex and alveolar-type tumor in sinonasal RMS. Nasal obstructions and neck masses were the most common symptoms. Patients with pretreatment lactate dehydrogenase (LDH) levels >400 U/l and negative immunohistochemical staining for desmin or MyoD1 had lower survival rates. CONCLUSION: In patients with sinonasal RMS, pretreatment LDH levels >400 U/l and negative immunohistochemical staining for desmin or MyoD1 may suggest a poor prognosis. These factors can not only contribute to the prediction of prognosis in patients with sinonasal RMS but also facilitate the design of more appropriate treatment strategies.


Assuntos
Neoplasias dos Seios Paranasais , Rabdomiossarcoma , Sarcoma , Humanos , Masculino , Feminino , Prognóstico , Desmina , Rabdomiossarcoma/diagnóstico , Rabdomiossarcoma/terapia , Rabdomiossarcoma/patologia , Neoplasias dos Seios Paranasais/diagnóstico , Neoplasias dos Seios Paranasais/terapia , Sarcoma/patologia
17.
ANZ J Surg ; 92(10): 2672-2675, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-36221213

RESUMO

BACKGROUND: Core biopsy is integral in the work-up of diagnosis for musculoskeletal lesions. Prior to referral through Christchurch hospital-a tertiary referral centre for sarcomas/musculoskeletal lesions, many patients undertake guided core biopsy in peripheral hospitals. We wished to assess the accuracy of these biopsies undertaken in the peripheral centres and compare to those done in the tertiary setting. METHODS: A retrospective analysis of image-guided core biopsies done in the South Island of New Zealand over a 5 year period was performed. An electronic database enquiry was made, and electronic notes were then screened for core biopsy results including subsequent biopsies done of the same lesion. Results from guided core biopsy were then recorded as diagnostic if the pathologist was able to reach or a definitive diagnosis, or guide management sufficiently. RESULTS: 223 patients with 229 biopsies were analysed. Overall accuracy of core biopsies were 83% across all centres. There were similar results between CT and ultrasound as well as soft tissue and bone lesions. Between the regions, the peripheral centres showed high accuracy compared with the tertiary centres. CONCLUSION: The regional centres demonstrated high diagnostic yield, and the current practice of providing core biopsy locally where possible prior to referral to a tertiary sarcoma, remains valid.


Assuntos
Biópsia com Agulha de Grande Calibre , Neoplasias Ósseas , Biópsia Guiada por Imagem , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/patologia , Humanos , Biópsia Guiada por Imagem/métodos , Estudos Retrospectivos , Sarcoma/diagnóstico , Sarcoma/patologia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/patologia
18.
J Coll Physicians Surg Pak ; 32(8): S159-S161, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-36210681

RESUMO

A 50-year male presented with vomiting and dysphagia for 2 weeks. Laboratory workup showed a positive serology for hepatitis C and normal serum α-fetoprotein (AFP) levels. CT abdomen revealed a large lesion in the right lobe of the liver extending upto the lower esophagus causing significant luminal narrowing and dysphagia. The enhancement pattern on the CT scan was not consistent with hepatocellular carcinoma. Liver lesion biopsy showed an infiltrating spindle cell lesion exhibiting fascicles of spindle cells with moderately hyperchromatic nuclei and perinuclear vacuolization. Mitotic count was 2-3/10 HPFs. Immunohistochemical markers were positive for CK AE1/AE3 and vimentin. Thus, a diagnosis of sarcomatoid carcinoma was made on the basis of morphological and immunohistochemical features. Due to unresectable disease and poor functional status, palliative care was opted for. Key Words: Dysphagia, Vomiting, Liver biopsy, Sarcomatoid carcinoma.


Assuntos
Carcinoma Hepatocelular , Transtornos de Deglutição , Neoplasias Hepáticas , Sarcoma , Neoplasias de Tecidos Moles , Carcinoma Hepatocelular/complicações , Carcinoma Hepatocelular/diagnóstico , Transtornos de Deglutição/etiologia , Humanos , Neoplasias Hepáticas/complicações , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/patologia , Masculino , Sarcoma/patologia , Vimentina , Vômito , alfa-Fetoproteínas
19.
Future Oncol ; 18(30s): 5-11, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-36200935

RESUMO

The goal of second-line therapy for most patients with advanced soft tissue sarcoma is long-term tumor control without detriment to quality of life. Clinical practice guidelines recommend trabectedin as a second-line option for advanced soft tissue sarcoma as it can provide the necessary balance between these interwoven goals. Cumulative experience with trabectedin in clinical trials and clinical practice has informed its usage such that greater benefit can be derived. In particular, use in earlier lines allows more patients to achieve prolonged tumor control (six or more cycles). Efficacy outcomes are superior when trabectedin is administered as second- versus later-line therapy and when it is used continuously until disease progression.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Tetra-Hidroisoquinolinas , Humanos , Trabectedina/uso terapêutico , Qualidade de Vida , Tetra-Hidroisoquinolinas/efeitos adversos , Dioxóis/efeitos adversos , Antineoplásicos Alquilantes/efeitos adversos , Neoplasias de Tecidos Moles/tratamento farmacológico , Sarcoma/tratamento farmacológico , Sarcoma/patologia
20.
JCO Precis Oncol ; 6: e2200087, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-36240470

RESUMO

PURPOSE: Radiation-associated sarcomas (RAS) are rare but aggressive malignancies. We sought to characterize the histology-specific presentation and behavior of soft tissue RAS to improve individualized prognostication. METHODS: A single-institutional prospectively maintained database was queried for all patients with primary, nonmetastatic RAS treated with surgical resection from 1982 to 2019. Patients presenting with the five most common RAS histologies were propensity-matched to those with sporadic tumors of the same histology. Incidence of disease-specific death (DSD) was modeled using cumulative incidence analyses. RESULTS: Among 259 patients with RAS, the five most common histologies were malignant peripheral nerve sheath tumor (MPNST; n = 19), myxofibrosarcoma (n = 20), leiomyosarcoma (n = 24), undifferentiated pleomorphic sarcoma (UPS; n = 55), and angiosarcoma (AS; n = 62). DSD varied significantly by histology (P = .002), with RAS MPNST and UPS having the highest DSD. In unadjusted analysis, RAS MPNST was associated with increased DSD compared with sporadic MPNST (75% v 38% 5-year DSD, P = .002), as was RAS UPS compared with sporadic UPS (49% v 28% 5-year DSD, P = .004). Unadjusted DSD was similar among patients with RAS AS, leiomyosarcoma, or myxofibrosarcoma and sporadic sarcoma of the same histology. After matching RAS to sporadic patients within each histology, DSD only differed between RAS and sporadic MPNST (83% v 46% 5-year DSD, P = .013). Patients with RAS AS presented in such a distinct manner to those with sporadic AS that a successful match was not possible. CONCLUSION: The aggressive presentation of RAS is histology-specific, and DSD is driven by RAS MPNST and UPS histologies. Despite the aggressive presentation, standard prognostic factors can be used to estimate risk of DSD among most RAS. In MPNST, radiation association should be considered to independently associate with markedly higher risk of DSD.


Assuntos
Fibrossarcoma , Histiocitoma Fibroso Maligno , Leiomiossarcoma , Neurofibrossarcoma , Sarcoma , Neoplasias de Tecidos Moles , Adulto , Histiocitoma Fibroso Maligno/patologia , Humanos , Leiomiossarcoma/patologia , Sarcoma/patologia
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