Extra Renal Rhabdoid Tumor: A Rare Cause of Congenital Soft Tissue Tumor

Rhabdoid tumors (RTs) are a well-defined entity in the kidney or central nervous system of infants or children. However, soft-tissue involvement is uncommon. It’s an exceptional neonatal tumor of soft tissue. The imaging characteristics of this tumor are not specific. Biopsy allows diagnosis;the histomorphological characteristics of rhabdoid tumors, their immunoreactivity to epithelial markers and vimentin, and the INI-1 loss are important tools for diagnosis. RT tumors are aggressive and have a rapidly fatal clinical course in most cases. Despite multidisciplinary therapy, the survival rate is very low. We report a rare case occurring in a male neonate who presents at birth with a voluminous right axillary mass. A CT scan showed a well-limited tumor mass with lobulated contours. An ultrasound-guided biopsy was performed on day 8, showing the morphology and immunoprofile of RT. The mass showed rapid growth. The child was admitted for respiratory distress at 3 weeks. A thoraco-abdominal CT showed an increase in the size of the mass with the appearance of multiple lymph nodes and pleural, hepatic, and renal metastases. The child died two days later.

Bone and soft tissue tumors presenting as sciatic notch dumbbell masses: A critical differential diagnosis of sciatica

AIM To study the clinical findings and characteristic features in sciatic notch dumbbell tumors(SNDTs).METHODS We retrospectively reviewed the clinical outcomes and characteristic features of consecutive cases of SNDTs(n = 8). RESULTS Buttock masses occurred in three patients with SNDT(37.5%). Severe buttock tenderness and pain at rest were observed in seven patients with SNDTs(87.5%). Remarkably, none of the patients with SNDTs experienced back pain. Mean tumor size was 8.4 ± 2.0 cm(range, 3.9 to 10.6 cm) and part of the tumor mass was detected in 2 patients in the sagittal view of lumbar magnetic resonance imaging(MRI).CONCLUSION The clinical information regarding to SNDTs is scarce. The authors consider that above mentioned characteristic findings may facilitate the suspicion of pelvic pathology and a search for SNDT by MRI or computed tomography should be considered in patients presenting with sciatica without evidence of spinal diseases.

X-ray DIAGNOSIS OF EXTRASKELETAL (SOFT TISSUE) CHONDROSARCOMA (A REPORT OF 8 CASES)

Eight cases of surgically and pathologically verified extraskeletal (soft tissue) chondrosarcoma were analyzed with regard to clinical and X-ray features. The cardinal clinical aspects of this series are: presence of a local soft tissue mass; gradual enlargement of the mass accompanied by increasing pain. The X-ray signs were summarized as follows: formation of a soft tissue mass; various forms of calcifications concentrated in the central area of the tumor; in some instances, presence of a saucer-like defect on the cortical surface of neighbouring bone and periosteal proliferation with mound-like new bone on both sides as well as bending deformity of the affected bone. The incidence and sites of predilection, the main X-ray findings, radiological diagnosis and differential diagnosis of the tumor were discussed. The Roentgen features of synovial chondrosarcoma of the knee joint were especially analyzed.

Soft tissue tuberculosis detected by next-generation sequencing:A case report and review of literature

BACKGROUND Soft tissue tuberculosis is rare and insidious,with most patients presenting with a localized enlarged mass or swelling,which may be factors associated with delayed diagnosis and treatment.In recent years,next-generation sequencing has rapidly evolved and has been successfully applied to numerous areas of basic and clinical research.A literature search revealed that the use of next-generation sequencing in the diagnosis of soft tissue tuberculosis has been rarely reported.CASE SUMMARY A 44-year-old man presented with recurrent swelling and ulcers on the left thigh.Magnetic resonance imaging suggested a soft tissue abscess.The lesion was surgically removed and tissue biopsy and culture were performed;however,no organism growth was detected.Finally,Mycobacterium tuberculosis was confirmed as the pathogen responsible for infection through next-generation sequencing analysis of the surgical specimen.The patient received a standardized anti-tuberculosis treatment and showed clinical improvement.We also performed a literature review on soft tissue tuberculosis using studies published in the past 10 years.CONCLUSION This case highlights the importance of next-generation sequencing for the early diagnosis of soft tissue tuberculosis,which can provide guidance for clinical treatment and improve prognosis.

Magnetic resonance imaging of soft-tissue tumors of the extremities: A practical approach

Diagnosis of extremity soft-tissue tumors can be challenging.Characteristics of tumor margins can help precisely identify locally aggressive or non-aggressive behavior for surgical planning,but cannot differentiate benign from malignant lesions.Most malignant tumors can have inhomogeneous signals on T2-weighted images.Although a uniform signal on T2-weighted images can be a reliable indication of a benign lesion,a well-defined mass with homogeneous internal signal intensity does not definitively identify a benign lesion.Some common and distinctive soft-tissue lesions can have specific clinical and imaging features allowing a diagnosis without biopsy.These are known as determinate lesions.This illustrative report presents a diagnostic guide for extremity soft-tissue tumors based on tissue signal and morphological characteristics on magnetic resonance images.It is important for clinicians to be familiar with the imaging characteristics of common determinate lesions.

Retrospective clinical analysis of MAID protocol as first-line treatment on 137 metastatic soft-tissue sarcomas patients

Objective:The aim of this study was to evaluate the efficacy,adverse reaction and survival of MAID protocol as first-line treatment on 137 metastatic soft tissue sarcomas patients.Methods:One hundredand thirty-seven patients with metastatic soft tissue sarcoma were given MAID protocol chemotherapy which consists of ifosfamide at a total dose of 8-10 g/m2 in 4-5 days,adriamycin at a dose of 60 mg/m2 on first day and dacarbazine at a dose of 200 mg/m2/d in 4-5 days.Twenty one days were regarded as a cycle and 2-6 cycles were completed.The median cycle number was 4.Results:Four cases had complete remission(CR)(2.9%),thirteen cases achieved partial remission(PR)(9.5%),eighty-eight cases maintained a stable disease(SD)(64.2%),and thirty-two cases had progressive disease(PD)(23.4%).The overall response rate(RR) was 12.4%,disease control rate(DCR) was 76.6%.The progression-free survival(PFS) was 2-14 months and median PFS was 5.00 ± 1.12 months.The overall survival(OS) was 3-19 months and median OS was 8.00 ± 1.32 months.Three months PFS rate was 56.9%,six months PFS rate was 27.2% and one year PFS rate was 7.8%.Three months OS rate was 100.0%,six months OS rate was 41.5% and one year OS rate was 10.7%.The main adverse reactions were bone marrow supression,nausea/vomiting and alopecia.Other adverse reactions were seldom observed.Conclusion:The effect of MAID protocol as first-line treatment on metastatic soft tissue sarcomas patients was exact,the treatment can control disease progression effectively and the side effects can be tolerable.It was obviously very worthy in clinical use as one of the first-line chemotherapy protocols on advanced soft tissue sarcomas.

Abdominal metastases of primary extremity soft tissue sarcoma: A systematic review

BACKGROUND Despite the fact that about one third of patients with primary localized extremity soft tissue sarcoma(e STS)will develop metastatic disease,abdominal metastases(AM)and retroperitoneal metastases(RM)constitute rare events.There is no clear consensus on how to achieve follow-up on patients with primary localized e STS following curative resection,especially regarding the surveillance of potential AM/RM.AIM To systematically analyse incidence,diagnosis,treatment and outcome of AM/RM in e STS patients.METHODS In this systematic review,899 studies available in Pub Med and published between 2000 and 2018 were screened,identifying 17 original articles focused on AM or RM in e STS.Article selection was based on the PRISMA guidelines,using the search terms(abdominal metastasis AND soft tissue sarcoma)and(soft tissue sarcoma metastasis abdomen).All studies published between January 1,2000 and December 31,2018 were screened.Further articles were identified by crosssearching article references,with the final search date being February 18,2019.Due to limited data and the different reporting techniques used,the present review focused on descriptive analysis of the included studies.RESULTS Of the 17 studies included,six original articles reported on incidence±diagnosis,therapy and outcome in AM and RM,whilst three original and eight case reports focused on diagnostic pathway,therapeutic procedures or outcomes without allowing conclusions regarding incidence of AM and RM.According to the former six studies,incidence of AM ranged from 0.9%-5.6%in patients with miscellaneous histological subtypes,and up to 12.1%in patients with myxoid liposarcoma.The most common histological subtypes that developed AM or RM were(myxoid)liposarcoma and leiomyosarcoma,but also rare subtypes such asepithelioid sarcoma,myxofibrosarcoma,synovial sarcoma,and malignant peripheral nerve sheath tumour had been reported to develop AM/RM.Surgery for AM/RM was performed in five of eight case-reports(62.5%)and in 20.8%-100.0%of original articles.In particular,patients with hepatic metastases undergoing metastasectomy had a survival benefit compared to patients treated with chemotherapy or best supportive care(>3 years vs<6 mo).CONCLUSION Patients with e STS should undergo surveillance with abdominal ultrasonography/computed tomography,or even whole-body-magnetic resonance imaging to detect AM/RM at an early stage.

Soft tissue aneurysmal bone cyst of the mandible:Report of a case

We report the case of a 17-year-old boy with a soft tissue aneurysmal bone cyst(STABC) located in the posterior aspect of the right mandible.Conventional radiography revealed no positive findings.On the computed tomography scan,the lesion appeared to have a nonuniform intralesional density.Magnetic resonance imaging revealed an abnormal soft tissue masses with cystic component in the superficial part of right mandibular body and angle with intact cortex.Following histopathological examination,fibro-histiocytic proliferation,blood-filled spaces and multinucleated giant cells were seen and the lesion was diagnosed as a STABC.The mass together with underlying bone and periosteum on its periphery was surgically resected under general anesthesia.Thirty-six months after surgery the patient was assessed at outpatient clinic and found no sign of recurrence This may be only the first reported case of the mandible in the English literature of this extremely rare benign tumor occurring in soft tissue.

Nodular fasciitis in the mesentery, a differential diagnosis of peritoneal carcinomatosis

Nodular fasciitis is a benign proliferative lesion composed of fibroblast-like cells that affects various sites in the body.We describe a patient with nodular fasciitis in the mesentery,encountered during laparotomy for the treatment of ascending colon cancer.The nodular fasciitis in our patient resembled peritoneal dissemination of malignancy on macroscopic observation.Because the treatment options change with concomitant peritoneal dissemination of gastrointestinal tract malignancy,recognition of this rare condition and preparation for unexpected nodular lesions are crucial.

血管瘤样纤维组织细胞瘤14例临床病理学观察

目的探讨血管瘤样纤维组织细胞瘤(angiomatoid fibrous histiocytoma,AFH)的临床病理特征及分子学改变,分析其诊断、鉴别诊断及预后。方法收集14例AFH的临床、病理及随访资料,并文献复习。结果14例AFH患儿中,男童11例,女童3例;年龄11个月~12岁11个月,平均5.9岁。肿瘤位于四肢3例,躯干5例,头颈部5例,颅内1例。镜下肿瘤细胞核呈空泡状,合体样、漩涡状排列,可见纤维性假包膜及淋巴细胞鞘。9例可见假血管腔隙,2例可见钙化,2例核分裂活跃(活跃处11个/10 HPF)。3例镜下见硬化、黏液样间质。免疫表型:desmin(10/14)、EMA(12/14)、CD99(12/14)、SMA(9/12)、ALK(7/8)阳性,Ki67平均增殖指数16%。分子检测EWSR1基因断裂7例、EWSR1-ATF1融合2例、EWSR1-CREB1融合2例。14例患儿平均随访46个月,均无复发、转移。结论AFH是一种交界性或低度恶性肿瘤,儿童患者预后良好,很少复发或转移。诊断及鉴别诊断需要结合临床特点、组织形态、免疫组化及EWSR1、FUS基因检测综合分析。

儿童胃肠道炎性肌纤维母细胞瘤的诊断与治疗

目的总结儿童胃肠道炎性肌纤维母细胞瘤(inflammatory myofibroblastic tumor,IMT)的临床特征及诊治经验。方法回顾性分析湖南省儿童医院普外科2010年1月至2021年12月收治的11例胃肠道IMT患儿临床资料,男7例,女4例;发病年龄8个月至15岁,收集患儿临床特点、影像学检查、病理学诊断、外科治疗及随访情况等。结果11例主要以腹痛、呕吐、发热、血便及腹部肿物就诊。10例行一期手术完整切除肿瘤,其中1例术后予化疗;1例经活检确诊后未予手术,仅行化疗。11例均病理诊断明确,均获随访(随访时间6~60个月),其中9例治愈,2例带瘤生存(1例于术后2年复发)。结论儿童胃肠道IMT临床少见,手术是首选治疗方法。复发、难治性IMT的治疗亟待进一步积累经验。

Improvement in Automated Diagnosis of Soft Tissues Tumors Using Machine Learning

Soft Tissue Tumors(STT)are a form of sarcoma found in tissues that connect,support,and surround body structures.Because of their shallow frequency in the body and their great diversity,they appear to be heterogeneous when observed through Magnetic Resonance Imaging(MRI).They are easily confused with other diseases such as fibroadenoma mammae,lymphadenopathy,and struma nodosa,and these diagnostic errors have a considerable detrimental effect on the medical treatment process of patients.Researchers have proposed several machine learning models to classify tumors,but none have adequately addressed this misdiagnosis problem.Also,similar studies that have proposed models for evaluation of such tumors mostly do not consider the heterogeneity and the size of the data.Therefore,we propose a machine learning-based approach which combines a new technique of preprocessing the data for features transformation,resampling techniques to eliminate the bias and the deviation of instability and performing classifier tests based on the Support Vector Machine(SVM)and Decision Tree(DT)algorithms.The tests carried out on dataset collected in Nur Hidayah Hospital of Yogyakarta in Indonesia show a great improvement compared to previous studies.These results confirm that machine learning methods could provide efficient and effective tools to reinforce the automatic decision-making processes of STT diagnostics.

外阴巨大血管肌纤维母细胞瘤磁共振成像误诊为脂肪肉瘤一例

外阴血管肌纤维母细胞瘤(angiomyofibroblastoma,AMF)是一种育龄期女性罕见的良性肿瘤,起源于女性的外阴或阴道。脂肪肉瘤好发于围绝经期及绝经后女性的躯干、四肢等部位。报道1例外阴巨大AMF磁共振成像(magnetic resonance imaging,MRI)误诊为脂肪肉瘤的病例,该患者因发现外阴肿物3年,增大1年入院,术前MRI诊断为脂肪肉瘤,而术前活检诊断为AMF,行外阴肿物切除术+外阴成形术,术后病理检查诊断AMF,术后随访9个月未见复发。临床医生应加强疾病的鉴别诊断,通过结合术前组织病理学及其相关影像学检查可提高AMF的诊断准确率,为临床提供准确的手术指导信息。

多参数MRI影像组学与深度学习模型鉴别良、恶性黏液样软组织肿瘤

目的观察基于多参数MRI构建的影像组学与深度学习(DL)模型鉴别良、恶性黏液样软组织肿瘤(MSTT)的价值。方法回顾性纳入141例经病理证实的MSTT患者,以7∶3比例随机将其分为训练集(n=98,包括51例恶性及47例良性MSTT)及测试集(n=43,包括22例恶性及21例良性MSTT)。分别于训练集T1WI和脂肪抑制(FS)-T2WI中提取并遴选影像组学特征及DL特征,并以之构建鉴别良、恶性MSTT的影像组学模型及DL模型。绘制受试者工作特征(ROC)曲线、校准曲线及决策曲线,对比评估2个模型的区分度、校准度及净收益。结果于训练集提取并筛选得到9个最佳影像组学特征用于构建鉴别良、恶性MSTT的影像组学模型,包括2个一阶特征、1个形态特征、3个灰度共生矩阵特征、1个灰度相关矩阵特征和2个灰度大小区域矩阵特征;以其中7个最佳DL特征构建DL模型。所获影像组学模型和DL模型鉴别测试集良、恶性MSTT的ROC曲线下面积分别为0.758及0.911,后者高于前者(P=0.017);2个模型均具有良好校准度;相比影像组学模型,DL模型在测试集的总体净收益更高。结论基于MRI构建的DL模型鉴别良、恶性MSTT的效能较影像组学模型更好且净收益更高。

左眼眶肌周细胞瘤1例

患者女,59岁,发现左眼眶花生米大小肿物伴疼痛4个月,自行口服抗生素后疼痛缓解,近1个月自觉肿物增大;无特殊既往史。查体:左眼眶近鼻根处2.0cm×1.5cm隆起性肿物,质韧,边界清,移动度差,无压痛;局部皮肤未见红肿。实验室检查未见明显异常。眼眶MRI:左侧上眼睑近鼻侧皮下1.5cm×1.4cm结节,与邻近筋膜分界欠清,T1WI呈中央稍低、周围等-稍高混杂信号(图1A),T2WI及脂肪抑制T2WI呈中央低、周围稍高信号(图1B),弥散加权成像(diffusion weighted imaging,DWI)呈环状稍高信号.

4例软组织透明细胞肉瘤MRI表现

透明细胞肉瘤(clear cell sarcoma,CCS)罕见且侵袭性极强,在软组织肿瘤(soft tissue tumor,STT)中的占比不足1%。软组织CCS(soft tissue CCS,SCCS)主要发生于下肢的腱鞘、肌腱和筋膜,其影像学表现无特异性,MRI多边界清楚,少见瘤内坏死,病变周围缺乏瘤周水肿。

肌骨超声在慢性软组织损伤针刀治疗中的应用研究进展

针刀治疗在慢性软组织损伤疾病中的应用广泛。传统针刀治疗是在非直视下进行操作,对操作者的解剖知识和手法经验要求极高,且难以避免因解剖变异导致的组织损伤风险。近年来,随着肌骨超声的推广普及,超声引导下的可视化针刀治疗在慢性软组织损伤中的应用日益广泛。本文通过查阅近年来肌骨超声在针刀治疗中应用的相关文献,结合作者的临床经验,简要阐述了肌骨超声在针刀治疗中的重要性,旨在推动肌骨超声在针刀治疗中的进一步应用。

Solitary fibrous tumor:A case report of this multifaceted tumor

BACKGROUND Solitary fibrous tumor(SFT)is a remarkably uncommon mesenchymal tumor.STAT6 level and a combination of clinical,pathological,and molecular features are required to arrive at a proper diagnosis.CASE SUMMARY In this report,we present an intriguing case involving a 43-year-old woman who initially exhibited symptoms of a bleeding retroperitoneal tumor,initially resembling a gastrointestinal stromal tumor,but later confirmed as an SFT.However,a year later,what was initially believed to be a recurrence of her SFT was instead identified as a desmoid tumor.CONCLUSION Distinguishing SFT from other tumors was pivotal.Correcting misdiagnoses of tumor type initially and of recurrence later was necessary for appropriate treatment of the correct desmoid type.

2023年度骨与软组织肿瘤诊治新进展

骨与软组织肿瘤是指发生于肌肉骨骼系统的肿瘤,患者常表现为局部疼痛、肿胀,并会出现压迫症状、病理性骨折,严重危害患者的身体健康。本文旨在回顾总结2023年度骨与软组织肿瘤诊治新进展。重点探讨尤文氏肉瘤和骨肉瘤的系统性治疗,肿瘤坏死率、生存率和社会因素之间的关系,继发性骨肉瘤的检测方法,各种骨盆重建技术的中期结果和并发症情况,生物重建和内置假体的效果,以及脊柱骨肿瘤、骨巨细胞瘤和骨转移瘤的治疗方法和新型放射治疗的应用。

肌骨超声检查在创伤性浅表软组织损伤中的效果观察

目的探究创伤性浅表软组织损伤患者接受肌骨超声检查的意义。方法选择永煤集团总医院2021年1月至2023年10月收治的86例疑似创伤性浅表软组织损伤患者作为研究对象,所有患者均接受肌骨超声检查与磁共振成像检查(MRI),以手术病理结果为诊断金标准,对比两组检查方式下诊断结果、诊断效能与不同类型损伤的检出情况。结果手术病理结果显示阳性率为93.02%,MRI检查阳性率为86.05%,肌骨超声检查阳性率为81.40%,两种检查结果差异无统计学意义(P>0.05)。MRI检查、肌骨超声检查的准确度、灵敏度与特异度分别为90.70%、91.25%、83.33%,83.72%、85.00%、66.67%,两种检查方式诊断效能指标相比,MRI检查指标均较优,但两者之间差异无统计学意义(P>0.05)。两组检查方式在不同类型损伤中的检出情况相比,仅在半月板损伤上差异具有统计学意义(P<0.05),余下类型损伤中差异无统计学意义(P>0.05)。与MRI检查检查费用相比,肌骨超声检查费用较低,差异具有统计学意义(P<0.05)。结论肌骨超声检查应用在创伤性浅表软组织损伤中的检出率、诊断效能与疾病类型诊断符合率较高,这有利于临床中及早发现疾病,进而可为临床及早治疗提供参考性依据,同时肌骨超声检查费用较低,值得在临床检查中使用。