Intracranial synovial sarcoma: A clinical, radiological and pathological study of 16 cases

Zhang, Guobin; Xiao, Bingxiang; Huang, Huawei; Zhang, Yunsheng; Zhang, Xiaokang; Zhang, Junting; Wang, Yonggang

doi:10.1016/j.ejso.2019.08.015

Cited by 19 publications

(21 citation statements)

References 34 publications

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“…SS has the potential for multidirectional differentiation with adverse prognosis and is mostly occurs in the extremities and major joints, rarely occurring in renal parenchyma. [ 6 – 8 ] In 2017, 12,390 Americans affected by SS, including adults and children. [ 9 ]…”

Section: Discussionmentioning

confidence: 99%

Primary renal synovial sarcoma

Zhang

Tian

et al. 2020

Medicine

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Rationale: Synovial sarcoma (SS) is a malignant neoplasm that arises from soft tissues proximal to the joints. It occurs primarily at the major joints of the extremities, but may also occur in the deep soft tissues around the joints. While primary renal synovial sarcoma (PRSS) is extremely rare, it is important to have a better understanding of their imaging and clinical features to establish an effective treatment plan. Correct identification of PRSS is also useful for treating renal neoplasms. Patient's concerns: A 56-year-old Chinese man was admitted to our hospital due to moderate, paroxysmal left-sided loin pain. Diagnosis: Renal enhanced computed tomography (CT) scanning showed a relatively hypovascular lesion with calcification in the left kidney. A radical nephrectomy was performed in the left kidney. Postoperative pathology indicated SS with necrosis. The immunohistochemical findings were as follows: 34βE12 (Epithelium+), Bcl-2(+), CD99(+), CK-pan((Epithelium+), EMA(Epithelium+), Ki-67(+60%), and Vimentin(+), CD34(−). Interventions: The patient underwent radical left nephrectomy with no complications. Outcomes: After discharge, a close review for 3 months showed no evidence of recurrence. Lessons: PRSS should be considered for the differential diagnosis of renal hypovascular tumors. When problems arise in distinguishing renal hypovascular tumors, surgical pathology is helpful in the final diagnosis and further treatment of the disease.

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Section: Discussionmentioning

confidence: 99%

Primary renal synovial sarcoma

Zhang

Tian

et al. 2020

Medicine

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“…Vimentin and Bcl-2 are consistently expressed in SS tumor cells, as seen by immunohistochemistry. CD99 and CD56 are frequently expressed but not SMA, S-100, CD34 and GFAP[ 9 , 10 ]. The immunohistochemical results of tumor cells in this patient were consistent with those reported in the literature, including vimentin(+), Bcl-2(+), CD56(+), SMA(-), GFAP(-), S-100(-) and CD34(-), as well as CD99(-).…”

Section: Discussionmentioning

confidence: 99%

“…The current recommendation for the treatment of primary intracranial SS is to remove the tumor as far as possible while ensuring the patient’s safety, followed by postoperative adjuvant radiotherapy and chemotherapy. Reports indicate that the degree of tumor resection is closely related to prognosis[ 10 ]. The recommended dose of postoperative adjuvant radiotherapy is 60-70 Gy[ 12 , 13 ].…”

Section: Discussionmentioning

confidence: 99%

“…A 95% PGTV 2.2 Gy × 30 F radiotherapy plan for our patient was performed, but he developed radiation necrosis 3 mo after radiotherapy. In addition, SS is remarkably prone to metastasis, particularly to the lungs, because it is a highly malignant tumor, and related monitoring approaches, such as chest radiography, should be performed during follow-up[ 10 ].…”

Section: Discussionmentioning

confidence: 99%

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Primary intracranial synovial sarcoma with hemorrhage: A case report

Wang

Zhang

et al. 2021

WJCC

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BACKGROUND Synovial sarcoma (SS) is a highly malignant tumor of unknown histological origin. This tumor can occur in various parts of the body, including those without synovial structures, but mainly in and around the joints, mostly in the lower extremities. Primary intracranial SSs are remarkably rare. This paper aims to report a case of primary intracranial SS with hemorrhage. CASE SUMMARY A 35-year-old male patient suffered a headache and slurred speech during manual labor and was sent to the emergency department. Through imaging examination, the patient was considered to have high-grade glioma complicated with hemorrhage and was treated with craniotomy. Postoperative pathology revealed SS. positron emission tomography/computed tomography was performed, which ruled out the possibility of metastasis to the intracranial from other parts of the body. Postoperative radiotherapy was given to the patient, during which radiation necrosis occurred. Sixteen months after craniotomy, cranial magnetic resonance imaging revealed recurrence of the tumor. CONCLUSION Primary intracranial SS is a rare malignant tumor. Primary intracranial SS with hemorrhage and radiation necrosis should be carefully monitored during postoperative radiotherapy. Surgical resection of the tumor combined with postoperative radiotherapy and chemotherapy is currently used, but the prognosis is poor.

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“…Intracranial sarcomas are uncommon tumors with poor prognoses and mainly occurred in adult patients [1]. However, they rarely affect children, and when they do, they are often associated with syndromic disorders [2][3][4].…”

Section: Introductionmentioning

confidence: 99%

DICER1-Associated Central Nervous System Sarcoma with Neural Lineage Differentiation: A Case Report

Yao

Duan

Jing

et al. 2021

Preprint

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Background: DICER1-associated central nervous system sarcoma (DCS) without evidence of other cancer-related syndromes is rare. Though the morphology of DCS was highly variable, the immunophenotype was predominant myogenic phenotype. Other lineage markers were consistently negative. Herein, our objective was to identify the clinical, pathogenesis, treatment and driver mutation of DCS with neurogenic differentiation through whole-exome sequencing (WES) and RNA sequencing (RNA-seq) of both leukocytes and tumor tissues.Case presentation: We describe here the case of a 8-year-old female patient presented with a 8-day history of headache, nausea and vomiting. Magnetic resonance imaging (MRI) revealed a heterogeneous mass in left parietal lobe. The patient underwent the craniotomy via left parietal approach. Histologically, the tumor predominately showed fibrosarcoma-like spindle cells with obvious cytoplasmic eosinophilic globules. Immunohistochemically, the tumor stained positively for NF, Syn, MAP-2, Desmin and DICER1. WES of tumor tissues detected the DICER1 somatic mutation. This case harbored tumor-driving mutations mainly including AR, AXL and ETV5 mutations, proved sarcoma-associated genes in other kind of sarcomas growth, in addition to TP53 and RAF1 mutations which were common found in DCS. All theses findings indicated the diagnosis of DCS with neurogenic differentiation. This neural lineage differentiation was further confirmed by the result of Gene Ontology (GO) analysis. The patient subsequently received high dose radiotherapy (60Gy) and chemotherapy. The MRI showed no evidence of tumor recurrence at the 12 months’ follow-up.Conclusions: This unusual case of DCS with neuronal differentiation is an important addition to the immuno-phenotypic spectrum of DCS. The prognosis is poor for DCS, and total tumor resection and high dose radiotherapy may assist in prolonging survival. Further research is needed to better understand the behavior and treatment of this rare DCS with neuronal differentiation.

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Intracranial synovial sarcoma: A clinical, radiological and pathological study of 16 cases

Cited by 19 publications

References 34 publications

Primary renal synovial sarcoma

Primary renal synovial sarcoma

Primary intracranial synovial sarcoma with hemorrhage: A case report

DICER1-Associated Central Nervous System Sarcoma with Neural Lineage Differentiation: A Case Report

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