Retroperitoneal Neoplasms

腹膜后肿瘤
  • 文章类型: Case Reports
    BACKGROUND: Schwannomas are benign usually encapsulated nerve sheath tumors derived from the Schwann cells, and affecting single or multiple nerves. The tumors commonly arise from the cranial nerves as acoustic neurinomas but they are extremely rare in the pelvis and the retroperitoneal area. Retroperitoneal pelvic schwannomas often present with non-specific symptoms leading to misdiagnosis and prolonged morbidity.
    METHODS: We report the case of a 59-year-old woman presenting with a feeling of heaviness in the lower abdomen who was found to have a retroperitoneal pelvic schwannoma originating from the right femoral nerve. She had a history of two resections of peripheral schwannomas at four different sites of limbs. After conducting magnetic resonance imaging, this pelvic schwannoma was misdiagnosed as a gynecological malignancy. The tumor was successfully removed by laparoscopic surgery. Pathological analysis of the mass revealed a benign schwannoma of the femoral nerve sheath with demonstrating strong, diffuse positivity for S-100 protein.
    CONCLUSIONS: Although retroperitoneal pelvic schwannoma is rare, it should be considered in the differential diagnosis of pelvic masses, especially in patients with a history of neurogenic mass or the presence of neurogenic mass elsewhere.
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    文章类型: Case Reports
    A 7-year-old spayed female domestic shorthair cat was presented for evaluation of a large-volume abdominal space-occupying lesion. A computed tomography angiography examination detected a round retroperitoneal mass, in contact with the large abdominal vessels, characterized by an external hyperattenuating capsule and a larger hypoattenuating center. The capsule was soft-tissue attenuating with marked heterogenous contrast enhancement. The center was hypoattenuating pre- and post-contrast administration. The mass displaced both kidneys laterally and the descendent colon ventrally. The mesenteric veins and both phrenicoabdominal veins were markedly increased in diameter. However, the adrenals were not involved. On the excretory phase, no contrast enhancement was observed in either ureter, except for the proximal tract of the right ureter. At laparotomy, both ureters entered the mass that was adherent to the great abdominal vessels. The cytological diagnosis was retroperitoneal extra-adrenal paraganglioma. In cats, retroperitoneal extra-adrenal paragangliomas are very rare. This is the first computed tomography angiography report of a retroperitoneal extra-adrenal paraganglioma in a domestic cat. Key clinical message: This report describes the computed tomography angiography features of a rare case of a retroperitoneal extra-adrenal paraganglioma in a cat. These features could be taken into consideration to direct the diagnosis of a possible neuroendocrine origin for a retroperitoneal mass in a cat.
    Caractéristiques de l’angiographie par tomodensitométrie d’un para-gangliome extra-surrénalien rétropéritonéal chez un chatUne chatte domestique à poils courts, âgée de 7 ans, stérilisée, a été présentée pour évaluation d’une lésion abdominale de grand volume occupant de l’espace. Un examen d’angiographie tomodensitométrique a détecté une masse rétropéritonéale ronde, en contact avec les gros vaisseaux abdominaux, caractérisée par une capsule externe hyper-atténuante et un centre hypo-atténuant plus large. La capsule présentait une atténuation des tissus mous avec une prise de contraste hétérogène marquée. Le centre était hypoatténuant avant et après l’administration le milieu de contraste. La masse a déplacé latéralement les deux reins et ventralement le côlon descendant. Le diamètre des veines mésentériques et des deux veines phrénico-abdominales était nettement augmenté. Cependant, les surrénales n’étaient pas impliquées. À la phase excrétrice, aucune augmentation de contraste n’a été observée dans les deux uretères, à l’exception du tractus proximal de l’uretère droit. Lors de la laparotomie, les deux uretères pénétraient dans la masse adhérente aux gros vaisseaux abdominaux. Le diagnostic cytologique était un paragangliome extra-surrénalien rétropéritonéal. Chez le chat, les para-gangliomes extra-surrénaliens rétropéritonéaux sont très rares. Il s’agit du premier rapport d’angiographie par tomodensitométrie d’un para-gangliome extra-surrénalien rétropéritonéal chez un chat domestique.Message clinique clé:Ce rapport décrit les caractéristiques de l’angiographie par tomodensitométrie d’un cas rare de paragangliome extra-surrénalien rétropéritonéal chez un chat. Ces caractéristiques pourraient être prises en considération pour orienter le diagnostic d’une éventuelle origine neuroendocrinienne d’une masse rétropéritonéale chez un chat.(Traduit par Dr Serge Messier).
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  • 文章类型: Case Reports
    schwannomas are benign and common soft tissue tumors. They are usually asymptomatic and are discovered for other reasons.
    we present the case of an 82-year-old male patient with a recent diagnosis of moderately differentiated adenocarcinoma of the colon and a hypermetabolic periaortic nodule as an incidental finding.
    percutaneous biopsy of the periaortic nodule confirmed the diagnosis of schwannoma. At one year of follow-up, growth of the schwannoma has been demonstrated. There are no signs of progression of his oncological disease.
    schwannomas are benign tumors, rarely found in the retroperitoneum and can be sources of false-positive positron emission tomography results.
    los schwannomas son tumores benignos y frecuentes de las partes blandas. Habitualmente son asintomáticos y son descubiertos por otros motivos.
    presentamos el caso de un paciente masculino de 82 años con diagnóstico reciente de adenocarcinoma de colon moderadamente diferenciado y con un nódulo periaórtico hipermetabólico como hallazgo incidental.
    la biopsia percutánea del nódulo periaórtico confirmó el diagnóstico de schwannoma. Al año de seguimiento, se ha demostrado crecimiento del schwannoma. No hay signos de progresión de su enfermedad oncológica.
    los schwannomas son tumores benignos, infrecuentes en el retroperitoneo y pueden ser fuentes de resultados falsos positivos en tomografía por emisión de positrones.
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  • 文章类型: Journal Article
    目的:对于晚期或转移性腹膜后肉瘤(RPS)患者,主要指南推荐以蒽环类药物为基础的化疗,包括阿霉素单药治疗;然而,很少有研究报道阿霉素单药治疗这些患者的结局.我们在此研究了在现实世界临床实践中,阿霉素单一疗法对晚期或转移性RPS患者的肿瘤疗效和安全性。
    方法:16例诊断为晚期或转移性腹膜后肉瘤,我们分析了2017年2月至2023年3月在我们机构接受多柔比星单药治疗作为一线治疗的情况.响应率,无进展生存期(PFS),总生存期(OS),对不良事件(AE)情况进行回顾性调查.
    结果:患者的中位年龄为69.5岁。对阿霉素的最佳反应如下:完全反应,0例(0.0%);部分缓解,3(18.8%);病情稳定,9(56.3%);和进行性疾病,4(25.0%)。客观有效率和疾病控制率分别为18.8%和75.0%,分别。在观察期间(中位数,22个月,范围=2-53个月),中位PFS和OS期为8.0和24.0个月,分别。发生以下不良事件≥3级:14例患者(87.5%)中性粒细胞减少,发热性中性粒细胞减少5例(31.3%),2例白细胞减少症(12.5%),1例血小板减少症(6.3%),和心力衰竭在1(6.3%)。没有发生≥3级恶心和呕吐,也没有与治疗相关的死亡。
    结论:在现实世界的临床实践中,多柔比星单药治疗RPS的肿瘤学结果不逊于EORTC试验。血液学不良事件发生率较高;然而,预防性止吐药可预防严重的胃肠道AE,且无治疗相关死亡.总的来说,对于晚期或转移性RPS患者,使用适当的预防剂进行阿霉素单一疗法是有效的选择。
    OBJECTIVE: Anthracycline-based chemotherapies including doxorubicin monotherapy are recommended in major guidelines for patients with advanced or metastatic retroperitoneal sarcoma (RPS); however, few studies have reported the outcomes of doxorubicin monotherapy for these patients. We herein investigated the oncological efficacy and safety of doxorubicin monotherapy for patients with advanced or metastatic RPS in real-world clinical practice.
    METHODS: Sixteen patients diagnosed with advanced or metastatic retroperitoneal sarcoma, receiving doxorubicin monotherapy as first-line treatment between February 2017 and March 2023 at our Institution were analyzed. Response rate, progression-free survival (PFS) periods, overall survival (OS) period, and adverse event (AE) profiles were retrospectively investigated.
    RESULTS: The median age of patients was 69.5 years. Best responses to doxorubicin were as follows: complete response, 0 patients (0.0%); partial response, 3 (18.8%); stable disease, 9 (56.3%); and progressive disease, 4 (25.0%). The objective response rate and disease control rate were 18.8 and 75.0%, respectively. During the observation period (median, 22 months, range=2-53 months), median PFS and OS periods were 8.0 and 24.0 months, respectively. The following AEs Grade ≥3 occurred: neutropenia in 14 patients (87.5%), febrile neutropenia in 5 (31.3%), leukopenia in 2 (12.5%), thrombocytopenia in 1 (6.3%), and heart failure in 1 (6.3%). Grade ≥3 nausea and vomiting did not occur and there was no treatment-related death.
    CONCLUSIONS: The oncological outcomes of doxorubicin monotherapy for RPS in real-world clinical practice were not inferior to those of the EORTC trial. The incidence of hematological AEs was higher; however, severe gastrointestinal AEs were prevented by prophylactic antiemetics and there were no treatment-related deaths. Collectively, doxorubicin monotherapy with appropriate prophylactic agents is a valid option for patients with advanced or metastatic RPS.
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  • 文章类型: Journal Article
    目的:关于腹膜后软组织肉瘤的全身治疗的证据有限,而目前的日本指导方针未能提出明确的建议。这里,我们报告了我们的Mesna联合化疗的经验,阿霉素,异环磷酰胺,和达卡巴嗪(MAID)在该人群中。
    方法:我们回顾性回顾了8例患者(3例男性和5例女性)的记录,这些患者在2019年10月至2022年1月期间因病理诊断为转移性不可切除的腹膜后肉瘤(平滑肌肉瘤或多形性肉瘤)而接受MAID。治疗效果,耐受性(需要减少剂量),和安全性概况进行评估和总结.
    结果:开始时,中位年龄为56.0岁,体重指数为20.0kg/cm26例患者的东部肿瘤协作组表现状态评分为0。净临床获益是三名(37.5%)患者的部分反应,4例疾病稳定(50.0%),和进行性疾病之一(12.5%)。在中位90.8周的随访期间,五名患者的病情进展,导致中位无进展生存期为48.4周,发生了5人死亡,导致95.1周的总生存期。常见的不良事件是中性粒细胞减少症(8例),贫血(8名患者),血小板计数下降(7名患者),这导致6名患者的剂量减少(60-80%)。
    结论:MAID联合治疗可能是晚期腹膜后肉瘤的可接受选择;然而,由于其毒性并非微不足道,因此必须仔细评估其益处。
    OBJECTIVE: There is limited evidence regarding the systemic treatment of retroperitoneal soft-tissue sarcoma, and the current Japanese guidelines fail to make definitive suggestions. Here, we report our experience with combination chemotherapy of mesna, doxorubicin, ifosfamide, and dacarbazine (MAID) in this population.
    METHODS: We retrospectively reviewed the records of eight patients (three male and five female) who received MAID for pathologically diagnosed metastatic unresectable retroperitoneal sarcoma (either leiomyosarcoma or pleomorphic sarcoma) between October 2019 and January 2022. Treatment efficacy, tolerability (need for dose reduction), and safety profiles were evaluated and summarized.
    RESULTS: At initiation, the median age was 56.0 years, and the body mass index was 20.0 kg/cm2 Six patients had Eastern Cooperative Oncology Group performance status scores of 0. The net clinical benefit was a partial response in three (37.5%) patients, stable disease in four (50.0%), and progressive disease in one (12.5%). During the median 90.8 weeks of follow-up, disease in five patients progressed, resulting in a median progression-free survival of 48.4 weeks, and five deaths occurred, resulting in an overall survival of 95.1 weeks. Commonly observed adverse events were neutropenia (eight patients), anemia (eight patients), and decreased platelet count (seven patients), which led to dose reduction (60-80%) in six patients.
    CONCLUSIONS: MAID combination therapy may be an acceptable option for advanced retroperitoneal sarcoma; however, its benefits must be carefully assessed owing to its not insignificant toxicity.
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  • 文章类型: Journal Article
    背景:患有腹膜后血管肉瘤(HSA)的狗具有可变的术后中位生存时间(MST)。
    目的:回顾性评估某些肿瘤相关因素的预后价值,如肿瘤大小,破裂,侵入邻近组织,淋巴结和远处转移受累,他们在患有腹膜后HSA的狗中进行了分析。
    方法:回顾了10只仅接受手术切除的腹膜后HSA犬,并与脾(71)和肝(9)HSA进行了比较。使用Kaplan-Meier方法和对数秩分析比较因素之间的MSTs。多变量Cox比例风险分析用于比较出现部位之间的差异。
    结果:与脾脏和肝脏HSA相比,腹膜后HSA显示出相对较长的术后MST,并且对于≥5cm(195天)的肿瘤,MST明显长于<5cm(70天)。脾脏HSA显示受累远处淋巴结(23天)和远处转移(39天)的MST明显短于阴性(83天,p=0.002和110天,p分别<0.001)。肝脏HSA还显示MST明显较短(与98天相比,16.5天,p=0.003)为远处转移。此外,总体HSA的风险比(HRs)及其森林地块显示为不良预后因素,出现部位(脾脏;HR2.78,p=0.016和肝脏;HR3.62,p=0.019),远处淋巴结受累(HR2.43,p=0.014),和远处转移(HR2.86,p<0.001),肿瘤大小≥5cm是更好的预后因素(HR0.53,p=0.037)。
    结论:与总体HSA结合,与脾脏和肝脏HSA相比,腹膜后HSA显示出相对较长的术后MST,与肿瘤大小≥5cm相关,提示更好的预后因素。
    BACKGROUND: Dogs with retroperitoneal hemangiosarcoma (HSA) exhibit variable postoperative median survival times (MST).
    OBJECTIVE: To retrospectively evaluate the prognostic value of selected tumour-related factors, such as tumour size, rupture, invasion into adjacent tissue, involvement of lymph node and distant metastasis, they were analysed in dogs with retroperitoneal HSA.
    METHODS: Ten dogs with retroperitoneal HSA managed solely with surgical excision were reviewed and compared with spleen (71) and liver (9) HSA. The Kaplan-Meier method and log-rank analysis were used compare MSTs between factors. Multivariable Cox proportional-hazard analysis was used to compare differences between arising sites.
    RESULTS: Retroperitoneal HSA showed comparatively longer postoperative MST compared with that of spleen and liver HSA and demonstrated significantly longer MST (p = 0.003) for tumours ≥5 cm (195 days) than <5 cm (70 days). Spleen HSA revealed significantly shorter MSTs in involvement of distant lymph nodes (23 days) and distant metastasis (39 days) than those in negative (83 days, p = 0.002 and 110 days, p < 0.001, respectively). Liver HSA also revealed significantly shorter MST (16.5 days compared with 98 days, p = 0.003) for distant metastasis. Additionally, hazard ratios (HRs) and their forest plot for overall HSA revealed as poor prognostic factors, arising sites (spleen; HR 2.78, p = 0.016 and liver; HR 3.62, p = 0.019), involvement of distant lymph nodes (HR 2.43, p = 0.014), and distant metastasis (HR 2.86, p < 0.001), and as better prognostic factor of tumour size ≥5 cm (HR 0.53, p = 0.037).
    CONCLUSIONS: In combination with overall HSA, retroperitoneal HSA shows comparatively longer postoperative MST compared to spleen and liver HSA, associated with tumour size ≥5 cm suggesting better prognostic factor.
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  • 文章类型: Case Reports
    盆腔肿块通常起源于盆腔,通常与子宫有关。卵巢,或肠道疾病。本报告描述了我院诊断为腹膜后皮样囊肿的盆腔肿块患者的情况。我们对这个案例进行了分析和文献综述,减少误诊风险,加强腹膜后肿块的治疗。
    Pelvic masses frequently originate from the pelvic cavity and are often associated with uterine, ovarian, or intestinal disorders. This report describes the case of a patient with a pelvic mass diagnosed as a retroperitoneal dermoid cyst at our hospital. We analyzed this case and conducted a literature review, to mitigate the risk of misdiagnosis and enhance the treatment of retroperitoneal masses.
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  • 文章类型: Case Reports
    背景:畸胎瘤是由多达三个胚层的体细胞组织组成的生殖细胞肿瘤。原发性腹膜后畸胎瘤通常在儿童时期发展,在成人和腹膜后间隙中并不常见。虽然只有少数腹膜后甲状腺组织,我们报告了一个独特的腹膜后乳头状甲状腺癌病例。
    方法:一名41岁女性因间歇性非特异性腹痛出现在我们机构。磁共振成像检测到腰大肌和左髂动脉腹侧多囊实性腹膜后肿块。手术切除腹膜后肿块后,标本的组织学切片显示甲状腺乳头状癌细胞的证据。身体的分期计算机断层扫描没有进一步的表现。为了降低复发的风险,进行甲状腺全切除术,然后进行放射性碘治疗和终身激素替代.
    结论:伴有甲状腺乳头状癌的原发性腹膜后畸胎瘤是一种罕见的疾病。由于其非特异性临床表现和缺乏特异性放射学发现,术前诊断困难。组织病理学分析对于诊断是必要的。虽然手术被认为是一线治疗,目前仍有关于甲状腺切除的程度和是否需要行甲状腺全切除术加放射性碘辅助治疗的讨论.
    BACKGROUND: Teratomas are germ cell tumors composed of somatic tissues from up to three germ layers. Primary retroperitoneal teratomas usually develop during childhood and are uncommon in adults and in the retroperitoneal space. While there are only a few cases of retroperitoneal thyroid tissue, we report a unique case of a retroperitoneal papillary thyroid carcinoma.
    METHODS: A 41-year-old woman presented in our institution due to intermitted unspecific abdominal pain. Magnetic resonance imaging detected a multi-cystic solid retroperitoneal mass ventral to the psoas muscle and the left iliac artery. After surgical removal of the retroperitoneal mass, histology sections of the specimen indicated evidence of papillary thyroid carcinoma cells. A staging computed tomography scan of the body showed no further manifestations. To reduce the risk of recurrence, total thyroidectomy was performed followed by radioiodine therapy with lifelong hormone substitution.
    CONCLUSIONS: Primary retroperitoneal teratoma with evidence of papillary thyroid carcinoma is a rare condition. Preoperative diagnosis is difficult due to its non-specific clinical manifestation and lack of specific radiologic findings. Histopathology analysis is necessary for diagnosis. Although surgery is considered the first line treatment, there is still discussion about the extent of resection and the need for total thyroidectomy with adjuvant radioiodine therapy.
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  • 文章类型: Journal Article
    目的:我们开发了可解释的机器学习模型来预测腹膜后脂肪肉瘤(RLPS)患者的总生存期(OS)。这种方法旨在增强我们建模结果的可解释性和透明度。
    方法:我们从监测中收集RLPS患者的临床病理信息,流行病学,和最终结果(SEER)数据库,并以7:3的比例将它们分配到训练集和验证集。同时,我们从海军医科大学第一附属医院(上海,中国)。我们进行了LASSO回归和多变量Cox比例风险分析,以识别相关的危险因素,然后将其组合以开发六个机器学习(ML)模型:Cox比例风险模型(Coxph),随机生存森林(RSF),游侠,使用分量线性模型(GBM)的梯度增强,决策树,提升树木。使用一致性指数(C指数)评估了这些ML模型的预测性能,积分累积/动态曲线下面积(AUC),和综合Brier得分,以及Cox-Snell残差图。我们还使用了时间依赖的变量重要性,部分依赖生存图的分析,和聚集生存Shapley加法扩张(SurvSHAP)图的生成,以提供最优模型的全局解释。此外,SurvSHAP(t)和生存局部可解释模型不可知解释(SurvLIME)图用于提供最佳模型的局部解释。
    结果:最终的ML模型由六个因素组成:患者的年龄,性别,婚姻状况,手术史,以及肿瘤的组织病理学分类,组织学分级,SEER阶段。我们的预后模型表现出显著的判别能力,特别是在游侠模型表现最佳的情况下。在训练集中,验证集,和外部验证集,1、3和5年OS的AUC均高于0.83,Brier积分始终低于0.15.游侠模型的可解释性分析还表明,组织学分级,组织病理学分类,年龄是预测操作系统的最重要因素。
    结论:rangerML预后模型表现出最佳性能,可用于预测RLPS患者的OS,为临床医生提前做出明智的决定提供有价值和关键的参考。
    OBJECTIVE: We have developed explainable machine learning models to predict the overall survival (OS) of retroperitoneal liposarcoma (RLPS) patients. This approach aims to enhance the explainability and transparency of our modeling results.
    METHODS: We collected clinicopathological information of RLPS patients from The Surveillance, Epidemiology, and End Results (SEER) database and allocated them into training and validation sets with a 7:3 ratio. Simultaneously, we obtained an external validation cohort from The First Affiliated Hospital of Naval Medical University (Shanghai, China). We performed LASSO regression and multivariate Cox proportional hazards analysis to identify relevant risk factors, which were then combined to develop six machine learning (ML) models: Cox proportional hazards model (Coxph), random survival forest (RSF), ranger, gradient boosting with component-wise linear models (GBM), decision trees, and boosting trees. The predictive performance of these ML models was evaluated using the concordance index (C-index), the integrated cumulative/dynamic area under the curve (AUC), and the integrated Brier score, as well as the Cox-Snell residual plot. We also used time-dependent variable importance, analysis of partial dependence survival plots, and the generation of aggregated survival SHapley Additive exPlanations (SurvSHAP) plots to provide a global explanation of the optimal model. Additionally, SurvSHAP (t) and survival local interpretable model-agnostic explanations (SurvLIME) plots were used to provide a local explanation of the optimal model.
    RESULTS: The final ML models are consisted of six factors: patient\'s age, gender, marital status, surgical history, as well as tumor\'s histopathological classification, histological grade, and SEER stage. Our prognostic model exhibits significant discriminative ability, particularly with the ranger model performing optimally. In the training set, validation set, and external validation set, the AUC for 1, 3, and 5 year OS are all above 0.83, and the integrated Brier scores are consistently below 0.15. The explainability analysis of the ranger model also indicates that histological grade, histopathological classification, and age are the most influential factors in predicting OS.
    CONCLUSIONS: The ranger ML prognostic model exhibits optimal performance and can be utilized to predict the OS of RLPS patients, offering valuable and crucial references for clinical physicians to make informed decisions in advance.
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  • 文章类型: Journal Article
    背景:良性神经鞘瘤表现为孤立性腹膜后肿块(RBNSTs),对多学科团队在鉴别诊断方面提出了复杂的诊断挑战,分期,和治疗计划。本文回顾了不同成像技术在评估RBNST中所起的作用,并阐明了其典型的病理特征,特别强调了成像与组织学发现之间的相关性。此外,报道了一些腹膜后肿瘤的例子,这些例子值得在基于横断面调查(CSIs)的鉴别诊断过程中考虑.组织结构与影像学表现之间的相关性可以帮助提高CSIs与其他腹膜后肿瘤的鉴别诊断的准确性。
    本教育综述严格检查了孤立性腹膜后良性神经鞘瘤的影像学和组织学特征之间的相关性,为提高临床放射学鉴别诊断的准确性提供有价值的见解。
    结论:RBNST的诊断具有挑战性,因为它们缺乏特定的放射学特征。影像学上RBNST与其他腹膜后肿瘤的鉴别诊断很复杂。为了准确诊断,建议手术切除RBNST。
    BACKGROUND: Benign nerve sheath tumors presenting as solitary retroperitoneal masses (RBNSTs) pose a complex diagnostic challenge for multidisciplinary teams regarding differential diagnosis, staging, and treatment planning. This article reviews the role played by different imaging techniques in assessing RBNSTs and elucidates their typical pathological features with a particular emphasis on the correlation between imaging and histological findings. Furthermore, some examples of retroperitoneal tumors that merit consideration in the process of differential diagnosis based on cross-sectional investigations (CSIs) are reported. The correlation between tissue architecture and appearance on imaging can help increase the accuracy of differential diagnosis with other retroperitoneal neoplasms at CSIs.
    UNASSIGNED: This educational review critically examines the correlation between imaging and histological features in solitary retroperitoneal benign nerve sheath tumors, offering valuable insights for improving the accuracy of differential diagnosis in clinical radiology.
    CONCLUSIONS: RBNSTs are challenging to diagnose because they lack specific radiological features. Differential diagnosis of RBNSTs from other retroperitoneal neoplasms on imaging is complex. Surgical removal of RBNSTs is recommended for an accurate diagnosis.
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