RESUMEN
INTRODUCTION: The management of spinal sarcomas is complex, given their widespread involvement and high recurrence rates. Despite consensus on the need for a multidisciplinary approach with surgery at its core, there is a lack of definitive guidelines for clinical decision-making. This study examines a case series of primary spinal sarcomas, focusing on the surgical strategies, clinical results, and survival data to inform and guide therapeutic practices. METHODS: We conducted a retrospective analysis of patients who underwent surgical resection for primary spinal sarcomas between 2005 and 2022. The study focused on gathering data on patient demographics, surgical details, postoperative complications, overall hospital stay, and mortality within 90 days post-surgery. RESULTS: The study included 14 patients with a primary diagnosis of spinal sarcoma, with an average age of 48.6 ± 12.6 years. Chondrosarcoma emerged as the most common tumor type, representing 57.1% of cases, followed by Ewing sarcoma at 35.7%, and synovial sarcoma at 7.1%. Patients with chondrosarcoma were treated with en-bloc resection, while the patient with synovial sarcoma underwent intra-lesional excision and those with Ewing sarcoma received decompression and tumor debulking. Postoperative assessments revealed significant improvements in neurological conditions. Notably, functional status as measured by the Karnofski Performance Index (KPI), improved substantially post-surgery (from 61.4 to 80.0%) The mean follow-up was 34.9 ± 9.2 months. During this time period one patient experienced fatal bleeding after en-bloc resection complications involving the vena cava. None of the patient needed further surgery. CONCLUSIONS: Our 16-year study offers vital insights into managing primary spinal sarcomas, showcasing the effectiveness of surgical intervention, particularly en-bloc resection. Despite their rarity and complexity, our multidisciplinary treatment approach yields improved outcomes and highlights the potential for refined surgical strategies to become standardized care in this challenging domain.
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Sarcoma , Neoplasias de la Columna Vertebral , Humanos , Persona de Mediana Edad , Estudios Retrospectivos , Masculino , Femenino , Adulto , Sarcoma/cirugía , Sarcoma/mortalidad , Neoplasias de la Columna Vertebral/cirugía , Neoplasias de la Columna Vertebral/mortalidad , Resultado del Tratamiento , Procedimientos Neuroquirúrgicos/métodos , Anciano , Sarcoma Sinovial/cirugía , Sarcoma Sinovial/mortalidad , Condrosarcoma/cirugía , Condrosarcoma/mortalidad , Condrosarcoma/patología , Sarcoma de Ewing/cirugía , Sarcoma de Ewing/mortalidad , Complicaciones Posoperatorias/etiología , Grupo de Atención al PacienteRESUMEN
Schwannoma, also known as neurilemmoma or Schwann cell tumor, is one of the most common neoplasms of the nerve sheath which usually appears at the head, neck, or upper extremity. Schwannoma occurrence in the lower extremity originating from the common peroneal nerve is rarely reported according to literary findings. We report a case of a 32-year-old man who presented with a 6-month history of a growing lump in the left knee. MRT revealed a well-defined 9.6 cm × 7.8 cm × 6.5 cm multilobular mass of heterogeneous consistency with areas of necroses with a likely diagnosis of synovial sarcoma. After surgery, a final histopathological assessment of the tumor demonstrated Antoni A and B patterns with nuclear palisading, hallmarks of a schwannoma. Postoperatively the patient suffered a neurological complication-impaired dorsiflexion of the left foot. The patient started immediate physiotherapy in the Department of Rehabilitation. Three weeks after the operation, gradual improvement in neurological function was observed. To date, complete tumor excision combined with microscopic analysis and immunohistochemical staining remains the gold standard in diagnosing and treating a peripheral nerve schwannoma. Moreover, the use of additional nerve monitoring tools during surgery could help to prevent complications.
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Neurilemoma , Neoplasias del Sistema Nervioso Periférico , Nervio Peroneo , Sarcoma Sinovial , Humanos , Masculino , Neurilemoma/diagnóstico , Neurilemoma/cirugía , Neurilemoma/patología , Adulto , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/cirugía , Sarcoma Sinovial/patología , Nervio Peroneo/patología , Nervio Peroneo/cirugía , Diagnóstico Diferencial , Neoplasias del Sistema Nervioso Periférico/diagnóstico , Neoplasias del Sistema Nervioso Periférico/cirugía , Neoplasias del Sistema Nervioso Periférico/patología , Imagen por Resonancia Magnética , Neuropatías Peroneas/diagnóstico , Neuropatías Peroneas/cirugía , Resultado del TratamientoRESUMEN
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.
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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 , InmunohistoquímicaRESUMEN
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.
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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 , NeumonectomíaRESUMEN
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.
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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 , RecurrenciaRESUMEN
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.
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Sarcoma Sinovial , Humanos , Femenino , Anciano , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/patología , Sarcoma Sinovial/cirugía , Yeyuno/patología , Vísceras/patología , Dolor Abdominal , Pulmón/patologíaRESUMEN
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.
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Sarcoma Sinovial , Sarcoma , Adulto , Humanos , Masculino , Mano , Irán , Sarcoma Sinovial/diagnóstico por imagen , Sarcoma Sinovial/cirugía , Cúbito/patologíaRESUMEN
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.
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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/métodosRESUMEN
Synovial sarcoma is a mesenchymal tumour with partial epithelial differentiation. About 85-90% of SS occur in the extremities. We present a case of a 44-year-old woman diagnosed with recurrent synovial sarcoma with breast and pulmonary nodules. The primary treatment for synovial sarcoma is wide surgical excision, while chemotherapy is reserved for metastatic cases. In the first-line metastatic setting, combination treatment with adriamycin and ifosfamide is administered. Despite the unfavourable prognosis, the patient's extended survival is fortunately not the typical outcome. Keywords: case reports; chemotherapy; immunohistochemistry; synovial sarcoma.
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Sarcoma Sinovial , Femenino , Humanos , Adulto , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/cirugía , Ifosfamida/uso terapéutico , Doxorrubicina/uso terapéutico , Pronóstico , Terapia CombinadaRESUMEN
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.
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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/patologíaAsunto(s)
Sarcoma Sinovial , Neoplasias Gástricas , Humanos , Gastrectomía/métodos , Sarcoma Sinovial/cirugía , Sarcoma Sinovial/diagnóstico por imagen , Sarcoma Sinovial/patología , Sarcoma Sinovial/diagnóstico , Neoplasias Gástricas/cirugía , Neoplasias Gástricas/patología , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/diagnóstico por imagenRESUMEN
We report a case of small gastric synovial sarcoma (SS) finally diagnosed after laparoscopic-endoscopic cooperative surgery (LECS). A 50 year-old male underwent medical examination for a chief complaint of epigastric pain. Endoscopic examination showed a 20 mm submucosal tumor (SMT) located in the anterior wall which extended to the lesser curvature of the middle stomach. The biopsy tissue did not yield a definitive diagnosis. During 6 months of follow-up for this lesion suspected to be an inflammatory tumor, neither the shape nor the size of the tumor changed. We performed LECS for both diagnosis and treatment. Histologically, the tumor was composed of fascicles of spindle cells. Immunohistochemically, the tumor cells were focally positive for epithelial membrane antigen, cytokeratin (AE1/AE3) and S100 protein, while being negative for desmin, α-smooth muscle actin, CD34, c-kit and DOG1. The expression of INI1 was reduced. Fluorescence in situ hybridization (FISH) detected SS18 rearrangement. The SMT was diagnosed as primary SS. A SMT measuring < 20 mm might be malignant potential tumor such as SS even if there are no typical malignant findings by endoscopy. Surgical resection should be considered for SMT measuring < 20 mm with atypical findings even in the absence of definitive high-risk features.
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Laparoscopía , Sarcoma Sinovial , Neoplasias Gástricas , Masculino , Humanos , Persona de Mediana Edad , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/cirugía , Hibridación Fluorescente in Situ , Endoscopía Gastrointestinal , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/cirugía , Neoplasias Gástricas/patologíaAsunto(s)
Neoplasias de la Vaina del Nervio , Neoplasias del Sistema Nervioso Periférico , Sarcoma Sinovial , Humanos , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/cirugía , Sarcoma Sinovial/patología , Neoplasias de la Vaina del Nervio/diagnóstico , Neoplasias de la Vaina del Nervio/patología , Neoplasias del Sistema Nervioso Periférico/diagnóstico , Neoplasias del Sistema Nervioso Periférico/cirugía , Neoplasias del Sistema Nervioso Periférico/patología , Nervios Periféricos/patología , Diagnóstico Diferencial , Biomarcadores de TumorRESUMEN
A man in his 20s with a history of laryngeal synovial sarcoma presented with dyspnoea. Imaging revealed a large right ventricular (RV) mass, which was resected, and histological analysis indicated synovial sarcoma recurrence. Within 1 month of RV mass resection, the tumour progressed with paratracheal metastasis. The lumen was nearly obliterated, and right ventricular outflow tract (RVOT) obstruction led to rapid deterioration with mixed cardiogenic and obstructive shock. We present a rare case of primary laryngeal synovial sarcoma metastasising to the heart.
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Insuficiencia Cardíaca , Neoplasias Cardíacas , Neoplasias Laríngeas , Laringe , Sarcoma Sinovial , Sarcoma , Masculino , Humanos , Sarcoma Sinovial/complicaciones , Sarcoma Sinovial/cirugía , Sarcoma Sinovial/patología , Neoplasias Laríngeas/complicaciones , Neoplasias Laríngeas/cirugía , Laringe/patología , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/diagnóstico por imagen , Neoplasias Cardíacas/cirugía , Melanoma Cutáneo MalignoRESUMEN
Intra-abdominal synovial sarcomas are a rare type of soft tissue sarcomas that arise in the soft tissues of the abdominal cavity. They account for less than 1% of all primary synovial sarcoma cases. We report a case of a male patient in his early 30s who presented to the hospital with abdominal pain of 2 weeks duration. On examination, a mass was felt in the left mid-abdomen. MRI revealed a 12.7 cm×8.2 cm×8.6 cm soft tissue mass on the mesentery of the jejunum. The patient underwent surgical resection of the tumour, and the final pathology was synovial sarcoma, pT2b, grade 3.
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Cavidad Abdominal , Sarcoma Sinovial , Sarcoma , Humanos , Masculino , Cavidad Abdominal/patología , Imagen por Resonancia Magnética , Mesenterio/patología , Sarcoma/patología , Sarcoma Sinovial/diagnóstico por imagen , Sarcoma Sinovial/cirugía , AdultoRESUMEN
INTRODUCTION: Synovial sarcoma (SS) is a subtype of soft tissue sarcoma that primarily usually occurs in the lower extremities but rarely arises in the head and neck areas, including the oral cavity. Due to its variable presentation and similarity to benign masses in terms of age at onset, growth rate, and favorable outcomes, SS is often misdiagnosed as a benign tumor. However, it is a malignant tumor. PATIENT CONCERNS: We report the case of intramuscular SS in the oral cavity. Initially, the lesion was clinically suspected as a benign mass but was ultimately confirmed as malignant SS. DIAGNOSIS: Although histopathological examination is the first step in diagnosing SS, molecular testing to confirm the presence of SYT-SSX fusion can provide a definitive diagnosis when the histopathology is inconclusive. In this patient as well, the postoperative pathological report confirmed the diagnosis of biphasic SS, and molecular testing revealed positive SYT/SSX fusion. THERAPEUTICS INTERVENTIONS: Following the recommendation of multidisciplinary care system, a wide excision was performed including the buccinators muscle, and reconstruction was performed using a buccal fat pad flap to prevent cheek depression. OUTCOMES: On the final pathologic report, SS was removed margin-free, and there were no metastatic lymph nodes. No evidence of cheek dimpling was observed, and follow-up neck CT showed no significant changes in the lymph nodes. As a result of observation up to several months after surgery, there were no functional and aesthetic complications. CONCLUSIONS: We report a successful case of intramuscular SS resection, initially misdiagnosed as a benign mass, using a buccal fat pad flap. We also highlight the importance of correctly diagnosing SS, especially in the craniofacial region where it can be mistaken for benign masses.
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Sarcoma Sinovial , Sarcoma , Neoplasias de los Tejidos Blandos , Humanos , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/cirugía , Sarcoma Sinovial/patología , Errores Diagnósticos , Tejido Adiposo/patología , Proteínas de Fusión OncogénicaRESUMEN
BACKGROUND/AIM: Most cases of synovial sarcoma (SS) are aggressive and large-sized; only few show indolent behavior, having a small size. Nerves are rare sites of SS occurrence. An atypical case of SS can lead to its misdiagnosis as a benign tumor and delay its treatment. CASE REPORT: Here, we report a case of primary SS of indolent multinodular synovial sarcoma of peripheral nerves. Considering the clinical and imaging findings at the first visit, we suspected a benign tumor and continued careful follow-up. Three years later, marginal resection was performed and SS was suspected. We then performed an additional wide resection using a free flap. Histopathologically, the proximal tumor showed a diffuse proliferation of spindle cells without pleomorphism, whereas the distal tumor showed a similar histology with more hypercellularity. Additional wide-resection specimens showed remnant tumors derived from the peripheral nerve. Immunohistochemistry (IHC) showed positive staining for SS18:SSX and SSX in both tumors and fluorescence in situ hybridization showed positive staining for the SS18 split in both tumors. Finally, SS of the peripheral nerve was diagnosed. Owing to FNCLCC grade 2 tumor and tumor size, adjuvant chemotherapy was not performed. CONCLUSION: In cases of SS or other sarcomas with atypical clinical courses, with imaging findings mimicking benign tumors, we recommend marginal resection along with pathological examination for correct diagnosis.
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Neurilemoma , Sarcoma Sinovial , Humanos , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/cirugía , Sarcoma Sinovial/patología , Proteínas Represoras/genética , Hibridación Fluorescente in Situ , Neurilemoma/diagnóstico por imagen , Neurilemoma/cirugía , Nervios Periféricos/patología , Proteínas de Fusión Oncogénica/genética , Biomarcadores de TumorRESUMEN
BACKGROUND/AIM: Cancer/testis antigens (CTAs) are well-known molecular targets with expression restricted to testicular germ cells and malignant tumors. T-cell receptor (TCR)-engineered T-cell (TCR-T) therapy against CTAs in patients with sarcoma has shown substantial progress, but resistance to TCR-T therapy remains a critical problem. In this report, we present a case of synovial sarcoma treated with TCR-T therapy targeting the New York-esophageal squamous cell carcinoma (NY-ESO)-1 protein. Histological findings were compared before and after TCR-T therapy and before and immediately after cryoablation. CASE REPORT: A 68-year-old man received additional wide resection for synovial sarcoma in the left leg. Due to multiple metastases, he was enrolled in a clinical trial of TCR-T therapy for NY-ESO-1. The tumor demonstrated a 34.9% reduction in diameter. However, disease progression occurred by day 84 after TCR-T therapy. Six months after disease progression, cryoablation was performed for right posterior rib lesion and tumor specimens were obtained by needle biopsy both before and immediately after cryoablation. Ten months after the diagnosis of disease progression, the patient died. Expression levels of NY-ESO-1, human leukocyte antigen, and immune checkpoint proteins remained unchanged before and after TCR-T therapy. Beta catenin was up-regulated in recurrent tumor tissues after TCR-T therapy compared to levels observed before TCR-T therapy. Immediately after cryoablation, immunoreactivity for NY-ESO-1 showed a slightly reduction. CONCLUSION: Up-regulation of beta-catenin in synovial sarcoma with recurrence after TCR-T therapy may be involved in T-cell exclusion and resistance to TCR-T therapy. Needle biopsy after cryoablation can be performed with sufficient pathological diagnostic accuracy including immunostaining.
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Criocirugía , Neoplasias Esofágicas , Carcinoma de Células Escamosas de Esófago , Sarcoma Sinovial , Masculino , Humanos , Anciano , Sarcoma Sinovial/cirugía , Antígenos de Neoplasias/metabolismo , Recurrencia Local de Neoplasia , Receptores de Antígenos de Linfocitos T/metabolismo , Progresión de la Enfermedad , Tratamiento Basado en Trasplante de Células y TejidosRESUMEN
Objective: To investigate the clinicopathological features, immunophenotype, molecular features and differential diagnosis of primary synovial sarcoma of the lung (PSSL). Methods: Twelve cases of PSSL were collected at Henan Provincial People's Hospital, during May 2010 and April 2021, and their clinicopathological parameters were summarized. SS18-SSX, H3K27Me3, and SOX2 were added to the original immunomarkers to evaluate their diagnostic value for PSSL. Results: The age of 12 patients when diagnosed ranged from 32 to 75 years (mean of 50 years). There were 7 males and 5 females, 2 left lung cases and 10 right lung cases. Of the 6 patients who underwent surgical resection, five cases were confined to lung tissue (T1), one case had mediastinal invasion (T3), two cases had regional lymph node metastasis (N1), and none had distal metastasis. Microscopically, 11 cases showed monophasic spindle cell type and one case showed biphasic type composed of mainly epithelial cells consisting of cuboidal to columnar cells with glandular and cribriform structures. It was difficult to make the diagnosis by using the biopsy specimens. Immunohistochemistry (IHC) showed CKpan expression in 8 of 12 cases; EMA expression in 11 of 12 case; TLE1 expression in 8 of 12 cases; S-100 protein expression in two of 12 cases; various expression of bcl-2 and vimentin in 12 cases, but no expression of SOX10 and CD34 in all the cases. The Ki-67 index was 15%-30%. The expression of SS18-SSX fusion antibody was diffusely and strongly positive in all 12 cases. SOX2 was partially or diffusely expressed in 8 of 12 cases, with strong expression in the epithelial component. H3K27Me3 was absent in 3 of 12 cases. SS18 gene translocation was confirmed by fluorescence in situ hybridization (FISH) test in all 12 samples. Six cases underwent surgery and postoperative chemotherapy, while the other six cases had chemotherapy alone. Ten patients were followed up after 9-114 months, with an average of 41 months and a median of 26 months. Five patients survived and five died of the disease within two years. Conclusions: PSSL is rare and has a broad morphological spectrum. IHC and molecular tests are needed for definitive diagnosis. Compared with current commonly used IHC markers, SS18-SSX fusion antibody has better sensitivity to PSSL, which could be used as an alternative for FISH, reverse transcription-polymerase chain reaction or next generation sequencing in the diagnosis of PSSL.