Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 3.365
Filtrar
1.
Anticancer Res ; 44(6): 2453-2458, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38821602

RESUMEN

BACKGROUND/AIM: Synovial sarcoma (SS) is a rare malignant tumor with a poor survival rate. We previously reported that a combination of auranofin (AUR), a thioredoxin reductase inhibitor, and celecoxib (CE), an anti-inflammatory drug, significantly impedes the local progression of osteosarcoma (OS). However, the role of redox regulation in SS remains to be elucidated. This study aimed to investigate the efficacy of combined treatment of AUR and CE on the local progression of SS in vivo. MATERIALS AND METHODS: Nu/nu mice were implanted with the human SS cell line, Aska-SS, and treated with vehicle control, AUR, or a combination of AUR and CE (AUR-CE). Primary tumor size and weight were evaluated for the study duration and upon resection, respectively. Hematoxylin and eosin (H&E) and Ki-67 staining were performed to assess the local progression of SS. RESULTS: A statistically significant reduction in tumor size and weight was observed in the AUR- and AUR-CE-treated groups upon excision compared to that in the vehicle-treated group. The AUR-CE-treated group showed synergistic inhibition of local tumor growth. H&E staining of local SS tumors revealed decreased cell density and nuclear deformation in the AUR- and AUR-CE-treated groups compared to those in the vehicle-treated group. Immunohistochemical staining revealed a statistically significant decrease in Ki-67-positive cells in the AUR-CE-treated group compared to the vehicle-treated group. CONCLUSION: The combination of AUR and CE showed significant potential for delaying the local progression of SS. These findings support the repurposing of AUR and CE as early treatment options for SS.


Asunto(s)
Auranofina , Celecoxib , Progresión de la Enfermedad , Sarcoma Sinovial , Ensayos Antitumor por Modelo de Xenoinjerto , Celecoxib/farmacología , Celecoxib/administración & dosificación , Animales , Sarcoma Sinovial/tratamiento farmacológico , Sarcoma Sinovial/patología , Sarcoma Sinovial/metabolismo , Auranofina/farmacología , Auranofina/uso terapéutico , Humanos , Ratones , Línea Celular Tumoral , Ratones Desnudos , Protocolos de Quimioterapia Combinada Antineoplásica/farmacología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Proliferación Celular/efectos de los fármacos
2.
Zhonghua Zhong Liu Za Zhi ; 46(5): 449-456, 2024 May 23.
Artículo en Chino | MEDLINE | ID: mdl-38742358

RESUMEN

Objectives: To investigate the proportion of different histological types and CT enhanced imaging features of primary middle mediastinal lesions in order to improve the understanding of these tumors and the accuracy of preoperative diagnosis. Methods: Retrospective analysis was conducted on 84 patients with primary middle mediastinal lesions and clear histological classifications diagnosed and treated at the Cancer Hospital, Chinese Academy of Medical Sciences from January 2012 to December 2022. Clinical, imaging, and pathological data were collected and classified according to tumor histological classifications. CT imaging manifestations such as tumor location, size, morphology, edge, boundary, internal components, enhancement characteristics, and surrounding tissue invasion were evaluated and recorded. Results: The histological types of the primary middle mediastinal lesions from the 84 patients included mesenchymal tumors, anterior intestinal cysts, giant lymph node hyperplasia, substernal goiter, neuroendocrine carcinoma, lymphohematopoietic system tumors, and mesothelioma, accounting for 28.6%, 27.4%, 14.3%, 3.6%, 11.9%, 9.5%, and 4.8%, respectively. Mesenchymal tumors included peripheral nerve sheath tumors, vascular tumors, adipogenic tumors, solitary fibrous tumors, and synovial sarcoma, accounting for 54.2%, 20.8%, 12.5%, 8.3%, and 4.2%, respectively. The above tumors had diverse imaging manifestations and specific imaging features. Mature fat were found in 3 cases of liposarcoma; Calcification was observed in 2 cases of thyroid nodules and 7 cases of giant lymph node hyperplasia; Enhanced scanning showed significant enhancement in 2 cases of solitary fibrous tumors, 3 cases of thyroid nodules, and 11 cases of giant lymph node hyperplasia; Mediastinal large lymph nodes was observed in 6 cases of lymphoma and 3 cases of mesothelioma; High invasiveness was observed in 4 cases of mesothelioma and 9 cases of neuroendocrine carcinoma. Conclusion: Mediastinal tumors have low incidence rate and rich histological types, and their imaging manifestations are diverse. Preoperative differential diagnosis can be made according to their specific imaging characteristics.


Asunto(s)
Neoplasias del Mediastino , Tomografía Computarizada por Rayos X , Humanos , Neoplasias del Mediastino/diagnóstico por imagen , Neoplasias del Mediastino/patología , Estudios Retrospectivos , Carcinoma Neuroendocrino/diagnóstico por imagen , Carcinoma Neuroendocrino/patología , Carcinoma Neuroendocrino/diagnóstico , Ganglios Linfáticos/patología , Ganglios Linfáticos/diagnóstico por imagen , Mediastino/diagnóstico por imagen , Sarcoma Sinovial/diagnóstico por imagen , Sarcoma Sinovial/patología , Sarcoma Sinovial/diagnóstico , Persona de Mediana Edad , Masculino , Femenino
3.
Folia Med (Plovdiv) ; 66(2): 277-281, 2024 Apr 30.
Artículo en Inglés | MEDLINE | ID: mdl-38690825

RESUMEN

Primary pulmonary synovial sarcoma is an extremely rare and aggressive neoplasm that primarily affects young people and has a poor prognosis. Establishing this diagnosis requires the exclusion of a wide number of other neoplasms with multimodal clinical, imaging, histological, immunohistochemical, and cytogenetic assessment. We present a case of synovial sarcoma of the left lung in a 44-year-old man, diagnosed immunohistochemically after left lower lobectomy with atypical resection of the 5th segment. Imaging, diagnostic workup, histological and immunohistochemical characteristics, surgical treatment, and prognosis are discussed.


Asunto(s)
Neoplasias Pulmonares , Sarcoma Sinovial , Humanos , Sarcoma Sinovial/cirugía , Sarcoma Sinovial/patología , Sarcoma Sinovial/diagnóstico por imagen , Sarcoma Sinovial/diagnóstico , Masculino , Adulto , Neoplasias Pulmonares/cirugía , Neoplasias Pulmonares/patología , Neoplasias Pulmonares/diagnóstico por imagen , Neoplasias Pulmonares/diagnóstico , Neumonectomía , Tomografía Computarizada por Rayos X , Inmunohistoquímica
4.
Kyobu Geka ; 77(4): 311-314, 2024 Apr.
Artículo en Japonés | MEDLINE | ID: mdl-38644180

RESUMEN

The patient is a 76-year-old man. His chief complaint of chest pain led to a diagnosis of pericardial effusion of unknown cause, and pericardial drainage was performed. On the 30th day, chest pain appeared again. Echocardiography revealed a pericardial fluid reaccumulation and a substantial mass in the pericardial space. Surgical drainage was performed to find the cause. A hematoma/mass was present on the epicardium. The pericardial sac was filled with hematoma. The hematoma was removed, but part of the mass infiltrated close to the anterior descending branch of the left coronary artery, and removal of that part was abandoned. The intrapericardial hematoma and epicardium were submitted to pathology leading to the diagnosis of synovial sarcoma. The patient was discharged home 14 days after surgery.


Asunto(s)
Neoplasias Cardíacas , Derrame Pericárdico , Sarcoma Sinovial , Humanos , Masculino , Sarcoma Sinovial/complicaciones , Sarcoma Sinovial/cirugía , Sarcoma Sinovial/diagnóstico por imagen , Derrame Pericárdico/etiología , Derrame Pericárdico/diagnóstico por imagen , Derrame Pericárdico/cirugía , Anciano , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/cirugía , Neoplasias Cardíacas/diagnóstico por imagen , Recurrencia
5.
Zhonghua Bing Li Xue Za Zhi ; 53(4): 358-363, 2024 Apr 08.
Artículo en Chino | MEDLINE | ID: mdl-38556819

RESUMEN

Objective: To assess the clinicopathological features, immunophenotype, molecular characteristics and differential diagnosis of primary cardiac synovial sarcoma (PCSS). Methods: Five cases of PCSS were collected at Guangdong Provincial People's Hospital from 2008 to 2023, and their clinicopathological features were summarized. Immunohistochemical staining, fluorescence in-situ hybridization (FISH) and next-generation sequencing (NGS) were performed, and relevant literatures were reviewed. Results: The cases were found in four males and one female, ranging in ages from 16 to 51 years (median 30 years). Two cases were located in the pericardium, two in the right ventricle, and one in the left ventricle. Follow-up data were available in four cases. All the four patients died of disease at 3, 7, 13 and 26 months, respectively, after diagnosis. The tumor maximum diameter ranged from 6.0 to 14.0 cm in (mean 10.0 cm). Microscopically, three cases were monophasic and two cases were biphasic. Immunohistochemically, all cases were immunoreactive for EMA, vimentin, bcl-2 and CD56. The tumor cells were variably positive for pan-cytokeratin, SS18-SSX, SOX2, TLE1, CD99, synaptophysin, calretinin and calponin. FISH showed the presence of SS18 rearrangement in all the cases. NGS detected SS18-SSX gene fusion in three cases (SS18-SSX1 in one and SS18-SSX2 in two). Conclusions: PCSS is an exceedingly rare neoplasm, and should be distinguished from other various malignant epithelial and mesenchymal tumors. The clinical history, histopathological and immunohistochemical features, and molecular findings are all essential to the definitive diagnosis of PCSS.


Asunto(s)
Neoplasias Cardíacas , Neoplasias del Mediastino , Sarcoma Sinovial , Masculino , Humanos , Femenino , Biomarcadores de Tumor/genética , Biomarcadores de Tumor/análisis , Sarcoma Sinovial/genética , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/patología , Proteínas Proto-Oncogénicas/genética , Proteínas Represoras/genética , Proteínas de Fusión Oncogénica/genética , Neoplasias Cardíacas/genética , Neoplasias Cardíacas/cirugía
6.
J Cardiothorac Surg ; 19(1): 243, 2024 Apr 18.
Artículo en Inglés | MEDLINE | ID: mdl-38632629

RESUMEN

Synovial sarcoma of the heart is a rare tumor. Herein we would like to report a case of giant intrapericardial cardiac synovial sarcoma that originated from the right ventricle and grew outward near the diaphragm. After making adequate preoperative preparation, we performed the surgery as quickly as possible and resected the tumor completely. Based on the identification of the translocation on chromosome 18 rearrangement, the tumor can be diagnosed as a primary cardiac synovial sarcoma. Through this study, we aim to afford more information about cardiac synovial sarcomas as well as a reference for similar cases.


Asunto(s)
Neoplasias Cardíacas , Neoplasias del Mediastino , Sarcoma Sinovial , Neoplasias del Timo , Humanos , Sarcoma Sinovial/diagnóstico , Neoplasias Cardíacas/cirugía , Ventrículos Cardíacos/patología
7.
World J Surg Oncol ; 22(1): 96, 2024 Apr 16.
Artículo en Inglés | MEDLINE | ID: mdl-38622623

RESUMEN

BACKGROUND: Pleural neoplasms are rare and can be subdivided into pleural metastasis and primary pleural neoplasms. Non-mesothelioma primary pleural neoplasms are a diverse group of extremely rare pathologies. CASE PRESENTATION: In this case series, we describe the presentation and management of two rare primary pleural neoplasms. A first case describes a primary pleural yolk sac tumor treated with neoadjuvant chemotherapy, extended pleurectomy decortication, and hyperthermic intrathoracic chemotherapy. In a second case we describe the management of a primary pleural synovial sarcoma by neoadjuvant chemotherapy and extrapleural pneumonectomy. A complete resection was obtained in both cases and the post-operative course was uncomplicated. No signs of tumor recurrence were noted during follow-up in the first patient. In the second patient a local recurrence was diagnosed 6 months after surgery. CONCLUSION: Neo-adjuvant chemotherapy followed by extensive thoracic surgery, including hyperthermic intrathoracic chemotherapy, is a feasible treatment strategy for non-mesothelioma primary pleural neoplasms, but careful follow-up is required.


Asunto(s)
Tumor del Seno Endodérmico , Neoplasias Pleurales , Sarcoma Sinovial , Humanos , Sarcoma Sinovial/cirugía , Tumor del Seno Endodérmico/cirugía , Resultado del Tratamiento , Recurrencia Local de Neoplasia/cirugía , Neoplasias Pleurales/cirugía , Neoplasias Pleurales/patología , Neumonectomía
8.
Pediatr Blood Cancer ; 71(7): e31038, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38679840

RESUMEN

PURPOSE: Though the prognosis for pediatric patients with localised synovial sarcoma (SS) is generally good, the chances of being cured after relapse are limited. This study describes a retrospective multi-institutional series of relapsing SS patients treated at six selected European referral centers for pediatric sarcoma. PATIENTS AND METHODS: The study included 41 patients <21 years with relapsing SS, treated between 2002 and 2022. The analysis included patient's characteristics at first diagnosis, first-line treatments, clinical findings at relapse, and second-line treatment modalities. RESULTS: The first relapse occurred within 3-132 months (median 18 months) after first diagnosis and was local in 34%, metastatic in 54%, and both in 12%. Treatment at first relapse included surgery in 56% of cases, radiotherapy in 34%, and systemic therapy in 88%. In all, 36 patients received second-line medical treatment, that was chemotherapy in 32 cases (with 10 different regimens) and targeted therapy in four. No patient was included in an early-phase clinical trial as second-line therapy-line therapy. Overall response rate was 42%. Median event-free survival (EFS) was 12 months, postrelapse 5-year EFS was 15.8%. Median overall survival (OS) was 30 months, postrelapse 5-year OS was 22.2%. At the Cox's multivariable regression analysis, OS was significantly associated with time and type of relapse. CONCLUSION: Pediatric patients with relapsed SS have a poor prognosis and generally receive an individualized approach, due to the lack of a uniform standardized approach. New comprehensive strategies are needed to improve the knowledge on the biologic landscape of SS and develop tailored prospective clinical trials.


Asunto(s)
Recurrencia Local de Neoplasia , Sarcoma Sinovial , Humanos , Sarcoma Sinovial/terapia , Sarcoma Sinovial/mortalidad , Sarcoma Sinovial/patología , Estudios Retrospectivos , Masculino , Femenino , Niño , Adolescente , Preescolar , Recurrencia Local de Neoplasia/patología , Recurrencia Local de Neoplasia/terapia , Pronóstico , Europa (Continente) , Tasa de Supervivencia , Terapia Combinada , Estudios de Seguimiento , Adulto Joven , Adulto , Lactante
9.
J Neurosurg Spine ; 40(6): 782-789, 2024 Jun 01.
Artículo en Inglés | MEDLINE | ID: mdl-38489819

RESUMEN

OBJECTIVE: Synovial sarcoma (SS) is a relatively rare type of soft-tissue sarcoma that is commonly treated with surgery, radiation, chemotherapy, and palliative care. Stereotactic radiosurgery (SRS) is an emerging approach that shows promise in treating CNS conditions, but it has not been studied for SS. The authors present a systematic review that explores the effectiveness of different treatments, with a focus on SRS, for managing spinal SS. METHODS: A systematic PubMed search was conducted that covered studies from 1964 to 2022, yielding 70 relevant studies. Inclusion criteria encompassed primary and metastatic spinal SS, various treatment modalities, patient age 17 years or older, English-language studies, retrospective series, and case reports. Based on these criteria, 26 studies were included in this review and 44 were excluded. RESULTS: Of the included studies, 15 patients from 9 studies were treated with surgical intervention followed by both conventional radiotherapy (RT) and chemotherapy, 10 patients from 10 studies were treated with surgery followed by RT, 5 studies comprising 8 patients were exclusively treated with surgery, 5 cases in 3 studies were treated with surgery plus concomitant chemotherapy, 4 patients in 2 studies were treated with SRS, and only 1 study reported treatment without surgery and with chemotherapy and RT. The median progression-free survival and overall survival periods observed in the SRS-treated patients were 37 months and 60 months, respectively, which were higher than those of any other treatment method or combination used. CONCLUSIONS: The authors' study offers a thorough review of spinal SS treatments. They are hopeful that this will aid clinicians in informed decision-making for better patient outcomes.


Asunto(s)
Radiocirugia , Sarcoma Sinovial , Neoplasias de la Columna Vertebral , Humanos , Sarcoma Sinovial/terapia , Neoplasias de la Columna Vertebral/terapia , Radiocirugia/métodos , Terapia Combinada
10.
J Med Case Rep ; 18(1): 156, 2024 Mar 15.
Artículo en Inglés | MEDLINE | ID: mdl-38486309

RESUMEN

BACKGROUND: Synovial sarcoma is a rare soft tissue sarcoma, with incidences of 0.81/1,000,000 in children and 1.42/1,000,000 in adults. It is most commonly found in soft tissue and rarely in bone. It often has a slow growth pattern and a benign radiologic appearance. CASE PRESENTATION: This study reports a case of metacarpal synovial sarcoma occurring in the hand-wrist of a 32-year-old Iranian man presented with the chief complaint of a lump on the dorsal ulnar side of his left hand and wrist. Initially, the first physician suspected the case to be a ganglion cyst. After two months of conservative treatment, the size of the lesion gradually increased. Magnetic resonance imaging (MRI) was performed and after an excisional biopsy and a postoperative histological analysis, the tumor was identified as a synovial sarcoma. The patient underwent a scheduled surgical procedure. Unfortunately, he had poor follow-ups and brought the pathologic results two months later when, the tumor had incredible growth, which makes this presentation rare. CONCLUSIONS: Since early diagnosis can lead to higher survival rates, this report increases doctors' awareness of this extremely malignant tumor that is rarely seen.


Asunto(s)
Sarcoma Sinovial , Sarcoma , Adulto , Humanos , Masculino , Mano , Irán , Sarcoma Sinovial/diagnóstico por imagen , Sarcoma Sinovial/cirugía , Cúbito/patología
12.
Lancet ; 403(10435): 1421-1423, 2024 Apr 13.
Artículo en Inglés | MEDLINE | ID: mdl-38554722
13.
Lancet ; 403(10435): 1460-1471, 2024 Apr 13.
Artículo en Inglés | MEDLINE | ID: mdl-38554725

RESUMEN

BACKGROUND: Afamitresgene autoleucel (afami-cel) showed acceptable safety and promising efficacy in a phase 1 trial (NCT03132922). The aim of this study was to further evaluate the efficacy of afami-cel for the treatment of patients with HLA-A*02 and MAGE-A4-expressing advanced synovial sarcoma or myxoid round cell liposarcoma. METHODS: SPEARHEAD-1 was an open-label, non-randomised, phase 2 trial done across 23 sites in Canada, the USA, and Europe. The trial included three cohorts, of which the main investigational cohort (cohort 1) is reported here. Cohort 1 included patients with HLA-A*02, aged 16-75 years, with metastatic or unresectable synovial sarcoma or myxoid round cell liposarcoma (confirmed by cytogenetics) expressing MAGE-A4, and who had received at least one previous line of anthracycline-containing or ifosfamide-containing chemotherapy. Patients received a single intravenous dose of afami-cel (transduced dose range 1·0 × 109-10·0 × 109 T cells) after lymphodepletion. The primary endpoint was overall response rate in cohort 1, assessed by a masked independent review committee using Response Evaluation Criteria in Solid Tumours (version 1.1) in the modified intention-to-treat population (all patients who received afami-cel). Adverse events, including those of special interest (cytokine release syndrome, prolonged cytopenia, and neurotoxicity), were monitored and are reported for the modified intention-to-treat population. This trial is registered at ClinicalTrials.gov, NCT04044768; recruitment is closed and follow-up is ongoing for cohorts 1 and 2, and recruitment is open for cohort 3. FINDINGS: Between Dec 17, 2019, and July 27, 2021, 52 patients with cytogenetically confirmed synovial sarcoma (n=44) and myxoid round cell liposarcoma (n=8) were enrolled and received afami-cel in cohort 1. Patients were heavily pre-treated (median three [IQR two to four] previous lines of systemic therapy). Median follow-up time was 32·6 months (IQR 29·4-36·1). Overall response rate was 37% (19 of 52; 95% CI 24-51) overall, 39% (17 of 44; 24-55) for patients with synovial sarcoma, and 25% (two of eight; 3-65) for patients with myxoid round cell liposarcoma. Cytokine release syndrome occurred in 37 (71%) of 52 of patients (one grade 3 event). Cytopenias were the most common grade 3 or worse adverse events (lymphopenia in 50 [96%], neutropenia 44 [85%], leukopenia 42 [81%] of 52 patients). No treatment-related deaths occurred. INTERPRETATION: Afami-cel treatment resulted in durable responses in heavily pre-treated patients with HLA-A*02 and MAGE-A4-expressing synovial sarcoma. This study shows that T-cell receptor therapy can be used to effectively target solid tumours and provides rationale to expand this approach to other solid malignancies. FUNDING: Adaptimmune.


Asunto(s)
Anemia , Liposarcoma Mixoide , Sarcoma Sinovial , Trombocitopenia , Adulto , Humanos , Sarcoma Sinovial/tratamiento farmacológico , Sarcoma Sinovial/genética , Liposarcoma Mixoide/etiología , Síndrome de Liberación de Citoquinas/etiología , Ifosfamida , Trombocitopenia/etiología , Anemia/etiología , Antígenos HLA-A , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico
14.
J Int Med Res ; 52(3): 3000605241233953, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38534077

RESUMEN

We report the case of a woman nearing 70 years old who was admitted to the hospital with a complaint of "epigastric distension for 1 month". Her main signs and symptoms were progressive abdominal distension and occasional abdominal pain. Computed tomography suggested an abdominal mass. She had a surgical history of synovial sarcoma (SS) of the lungs. After admission, she was diagnosed with jejunal SS following a puncture biopsy and laparoscopic surgery. This disease usually occurs in the soft tissues of the limbs, and it is extremely rare for SS to originate in the jejunum. The morphologic heterogeneity of SS overlaps with other tumors and makes the diagnosis particularly difficult. Imaging studies usually lack specificity; however, measuring multiple immunohistochemical markers can greatly assist in the diagnosis and differential diagnosis of SS. This case not only enriches our understanding of SS and describes a rare site of origin, but also emphasizes the importance and challenges of achieving an accurate diagnosis. Immunohistochemical and molecular biological testing have important roles in the definitive diagnosis, highlighting the need for precise and innovative diagnostic and therapeutic approaches in SS.


Asunto(s)
Sarcoma Sinovial , Humanos , Femenino , Anciano , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/patología , Sarcoma Sinovial/cirugía , Yeyuno/patología , Vísceras/patología , Dolor Abdominal , Pulmón/patología
15.
Pediatr Blood Cancer ; 71(5): e30933, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38430473

RESUMEN

INTRODUCTION: In complex pediatric surgical oncology, surgical planning is contingent upon data gathered from preoperative imaging. Three-dimensional (3D) modeling and printing has been shown to be beneficial for adult presurgical planning, though pediatric literature is less robust. The study reviews our institutional experience with the use of 3D image segmentation and printed models in approaching resection of extracranial solid tumors in children. METHODS: This is a single institutional series from 2021 to 2023. Models were based on computed tomography and magnetic resonance imaging studies, optimized for 3D imaging. The feasibility and creation of the models is reviewed, including specific techniques, software, and printing materials from our institution. Clinical implications for surgical planning are also described, along with detailed preoperative and intraoperative images. RESULTS: 3D modeling and printing was performed for four pediatric patients diagnosed with extracranial solid tumors. Diagnoses included Ewing sarcoma, hepatoblastoma, synovial sarcoma, and osteosarcoma. No intraoperative complications or discrepancies with the preoperative 3D-printed model were noted. No evidence of local recurrence was identified in any patient thus far. CONCLUSION: Our institutional series demonstrates a wide spectrum of clinical application for 3D modeling and printing technology within pediatric surgical oncology. This technology may aid in surgical planning for both resection and reconstruction, can be applied to a diverse breadth of diagnoses, and may potentially augment patient and/or family education about their condition.


Asunto(s)
Sarcoma de Ewing , Sarcoma Sinovial , Niño , Humanos , Imagenología Tridimensional/métodos , Imagen por Resonancia Magnética , Sarcoma de Ewing/diagnóstico por imagen , Sarcoma de Ewing/cirugía , Tomografía Computarizada por Rayos X
16.
Indian J Pathol Microbiol ; 67(2): 396-400, 2024 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-38391333

RESUMEN

ABSTRACT: Synovial sarcoma (SS) is rarely documented in the female genital tract, especially confirmed by molecular testing for SYT::SSX translocation and TLE1 immunostaining. A 62-year-old lady presented with a progressively increasing lump and pain over her right groin, for 6-month duration. Radiologically, a well-defined, solid-cystic mass was seen involving the right labia with necrotic areas, sparing the underlying muscles and the overlying skin. She underwent a biopsy followed by a surgical excision. Histopathologic examination revealed a spindle cell sarcoma, including tumor cells exhibiting a prominent hemangiopericytomatous pattern. There were focal areas of epithelial differentiation (pseudoglandular) along with areas of round cell morphology and increased mitoses (poor differentiation) in the resected specimen. Immunohistochemically, the tumor cells were diffusely positive for TLE1, patchily positive for pan keratin (AE1/AE3) and EMA, the latter more in the areas of epithelial differentiation, while negative for CD34, SMA, desmin, S100P, and SOX10. INI1/SMARCB1 showed a characteristic weak to absent (mosaic) staining pattern. Furthermore, the tumor displayed SS18::SSX 1 fusion by RT-PCR. This constitutes one of the few reported cases of vulvar SS, confirmed by molecular testing and the first documented vulvar SS showing a mosaic pattern of INI1/SMARCB1 immunostaining. A review of the literature and diagnostic implications are presented herewith.


Asunto(s)
Inmunohistoquímica , Proteína SMARCB1 , Sarcoma Sinovial , Vulva , Neoplasias de la Vulva , Humanos , Femenino , Sarcoma Sinovial/genética , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/patología , Persona de Mediana Edad , Proteína SMARCB1/genética , Neoplasias de la Vulva/patología , Neoplasias de la Vulva/diagnóstico , Neoplasias de la Vulva/genética , Vulva/patología , Proteínas de Fusión Oncogénica/genética , Biomarcadores de Tumor/genética , Histocitoquímica , Microscopía , Proteínas Co-Represoras/genética , Proteínas Proto-Oncogénicas , Proteínas Represoras
17.
Hinyokika Kiyo ; 70(1): 1-5, 2024 Jan.
Artículo en Japonés | MEDLINE | ID: mdl-38321742

RESUMEN

We experienced two cases of renal primary synovial sarcoma. Case 1: A 29-year-old man underwent laparoscopic radical nephrectomy and was originally diagnosed with renal cell carcinoma. Case 2: A 25-year-old man was treated by open radical nephrectomy since radiographical findings indicated tumor invasion to the ureter causing hydronephrosis. Both cases were pathologically diagnosed as renal synovial sarcomas, and were followed using computed tomography. Recurrence was observed within a year in both cases.


Asunto(s)
Carcinoma de Células Renales , Neoplasias Renales , Laparoscopía , Neoplasias Retroperitoneales , Sarcoma Sinovial , Masculino , Humanos , Adulto , Sarcoma Sinovial/patología , Sarcoma Sinovial/cirugía , Neoplasias Renales/cirugía , Neoplasias Retroperitoneales/cirugía , Carcinoma de Células Renales/cirugía , Riñón , Nefrectomía/métodos
18.
BMC Musculoskelet Disord ; 25(1): 131, 2024 Feb 12.
Artículo en Inglés | MEDLINE | ID: mdl-38347547

RESUMEN

BACKGROUND: Malignant femoral soft tissue tumors are occasionally resected together with the femoral nerves, but this can cause loss of knee extensor muscle activity. To the best of our knowledge, no previous reports have detailed the gait analysis of such cases in combination with electromyography. Herein, we report the gait analysis of a patient who underwent left groin synovial sarcoma and left femoral nerve resection 12 years ago. CASE PRESENTATION: We analyzed the gait of a 38-year-old man who was able to walk unaided after the resection of a synovial sarcoma in the left groin together with the ipsilateral femoral nerve. The muscle activities of the affected medial (MH) and lateral hamstrings (LH), and lateral heads of the gastrocnemius (GL) were increased during 50-75% of the stance phase. The hip flexion angle of the affected limb was smaller, and the ankle plantar flexion angle of the affected limb was larger than that of the non-affected limb. This means that in the affected limb, the hip and ankle angles were adjusted to prevent knee collapse, and the MH, LH, and GL muscles contributed in the mid- and late-stance phases. Moreover, we found that the hamstring and gastrocnemius of the affected limb worked together to keep the ipsilateral knee extended in the mid-stance phase and slightly flexed in the late-stance phase. CONCLUSIONS: Patients capable of walking after femoral nerve resection may control their hamstrings and gastrocnemius muscles collaboratively to prevent ipsilateral knee collapse in the mid- and late-stance phases.


Asunto(s)
Sarcoma Sinovial , Sarcoma , Masculino , Humanos , Adulto , Nervio Femoral , Análisis de la Marcha , Marcha/fisiología , Caminata/fisiología , Articulación de la Rodilla/diagnóstico por imagen , Articulación de la Rodilla/cirugía , Articulación de la Rodilla/fisiología , Músculo Esquelético/cirugía , Músculo Esquelético/fisiología , Sarcoma/diagnóstico por imagen , Sarcoma/cirugía , Fenómenos Biomecánicos
19.
Clin Oncol (R Coll Radiol) ; 36(4): e97-e104, 2024 04.
Artículo en Inglés | MEDLINE | ID: mdl-38326122

RESUMEN

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


Asunto(s)
Sarcoma Sinovial , Sarcoma , Neoplasias de los Tejidos Blandos , Masculino , Adulto Joven , Humanos , Femenino , Sarcoma Sinovial/cirugía , Estudios Retrospectivos , Sarcoma/patología , Extremidades/patología , Extremidades/cirugía , Pronóstico , Neoplasias de los Tejidos Blandos/patología , Recurrencia Local de Neoplasia/patología
20.
Ultrastruct Pathol ; 48(3): 213-220, 2024 May 03.
Artículo en Inglés | MEDLINE | ID: mdl-38348820

RESUMEN

Synovial sarcoma (SS) and solitary fibrous tumor (SFT) are entities with considerable morphological and immunohistochemical similarities that sometimes show a non-confirmatory profile (TLE1 negative, CD34 and focal or negative STAT6 and lack of specific fusion IHC markers), in which the utility ultrastructure is unknown. A cross-sectional, retrospective, analytical, nonexperimental study was carried out by the Department of Pathology of the National Cancer Institute of Mexico (INCan) e from January 1, 2009 to December 31, 2018. With 17 SFT cases with diffuse or focal CD34 and STAT6 positivity and 18 cases of SS with positive FISH molecular test t(X:18) breakapart were studied by electron microscopy of fresh glutaraldehyde fixed or paraffin-embedded tissue. The ultrastructural findings with a significant difference present in the SS were tandem tight junctions, desmosomes and abundance of dilated rough endoplasmic reticulum (RER) cisternae (p < 0.001, 0.003, and 0.001, respectively); while in the (SFT) the presence of abundant glycogen, basal lamina, long and slender cytoplasmic processes, pinocytic vesicles, hemidesmosomes, and/or dense plaques, collagen skein, and microvilli-like buds (p = 0.028, 0.005, and <0.001 for the last five). We then infer that the five distinctive markers of the SFT are the collagen skeins intermingled with cellular processes in a shape of "squid can," and the pinocytic vesicles as they were not observed in any case of SS. Conversely, tandem junctions were not found in any SFT case. Although the presence of multivesicular buds in the SFT was not significant, it had not been previously described.


Asunto(s)
Sarcoma Sinovial , Tumores Fibrosos Solitarios , Humanos , Tumores Fibrosos Solitarios/patología , Tumores Fibrosos Solitarios/ultraestructura , Sarcoma Sinovial/ultraestructura , Sarcoma Sinovial/patología , Adulto , Masculino , Femenino , Estudios Retrospectivos , Persona de Mediana Edad , México , Estudios Transversales , Biomarcadores de Tumor , Anciano , Adulto Joven , Diagnóstico Diferencial
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA