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脾性腺融合的发病机制、诊断与管理:文献综述

Pathogenesis, Diagnosis, and Management of Splenogonadal Fusion: A Literature Review.

作者信息

Kadouri Youssef, Carnicelli Damien, Sayegh Hachem El, Benslimane Lounis, Nouini Yassine

机构信息

Faculty of Medicine and Pharmacy of Rabat Morocco, Ibn Sina Hospital, Department of Urology A, Mohammed V University, Morocco.

Department of Urology and Andrology, Center Hospitalier Métropole Savoie, Chambery, France.

出版信息

Case Rep Urol. 2020 Oct 7;2020:8876219. doi: 10.1155/2020/8876219. eCollection 2020.

DOI:10.1155/2020/8876219
PMID:33101755
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7568155/
Abstract

INTRODUCTION

Splenogonadal fusion is a rare congenital anomaly, defined by the presence of ectopic splenic tissue caused by an abnormal connection between the spleen and the gonad or mesonephrotic derivatives during the embryonic period.

MATERIALS AND METHODS

By reporting an observational case and performing a review of the literature according to the CARE guidelines (using the PubMed database and guidelines from urology, general surgery, and pediatric learned societies), we present the embryological genesis of the splenogonadal fusion, the associated anatomical anomalies, and the diagnostic procedure. . We report the case of a patient aged 45, with no notable history, reporting left testicular pain. A small nodule on the upper pole of the left testicular was clinically palpable. Tumor markers were normal, and scrotal ultrasound depicted a hypoechoic hypervascular nodule measuring 8∗6∗8 mm. After validation in a multidisciplinary oncology consultation meeting and opinion from a uro-andrologist expert, the patient underwent an inguinal lumpectomy with an extemporaneous examination which did not objectify any signs of malignancy. Ultimately, it is a normal spleen tissue in the testicular ectopic position. . Splenogonadal fusion corresponds to a rare congenital malformation; less than 200 cases have been published in the literature, most often affecting boys, with a sex ratio of 15/1. Two types are described, depending on the continuity of the link between the orthotopic spleen and the gonad: the continuous and discontinuous forms. In a third of the cases, there are associated congenital malformations and particularly in the continuous forms (44 to 50% of the cases): anomalies of the limbs, micrognathia, microgyria, and hepatic and digestive abnormalities. Cryptorchidism is associated with the continuous form in 31% of cases. The preoperative diagnosis remains difficult because of its morphological and clinical characteristics suggesting a tumor process.

摘要

引言

脾性腺融合是一种罕见的先天性异常,其定义为在胚胎期脾脏与性腺或中肾衍生物之间存在异常连接,导致异位脾组织的出现。

材料与方法

通过报告一例观察性病例并根据CARE指南(使用PubMed数据库以及泌尿外科、普通外科和儿科学术协会的指南)进行文献综述,我们阐述了脾性腺融合的胚胎发生、相关解剖异常及诊断程序。我们报告了一名45岁患者的病例,该患者无显著病史,自述左侧睾丸疼痛。临床触诊发现左侧睾丸上极有一个小结节。肿瘤标志物正常,阴囊超声显示一个低回声高血管结节,大小为8×6×8毫米。在多学科肿瘤会诊会议得到确认并经泌尿男科专家会诊后,该患者接受了腹股沟肿块切除术,并进行了术中检查,未发现任何恶性迹象。最终发现,这是位于睾丸异位位置的正常脾脏组织。脾性腺融合是一种罕见的先天性畸形;文献报道不足200例,最常影响男性,男女比例为15/1。根据原位脾脏与性腺之间连接的连续性,可分为两种类型:连续型和间断型。三分之一的病例伴有先天性畸形,尤其是连续型(占病例的44%至50%):肢体畸形、小颌畸形、小脑回畸形以及肝脏和消化系统异常。31%的病例中隐睾症与连续型相关。由于其形态和临床特征提示肿瘤性病变,术前诊断仍然困难。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c4ad/7568155/f45547681822/CRIU2020-8876219.005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c4ad/7568155/6c0b8c85c917/CRIU2020-8876219.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c4ad/7568155/192ed963c608/CRIU2020-8876219.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c4ad/7568155/9250532ac25e/CRIU2020-8876219.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c4ad/7568155/4ea66169e326/CRIU2020-8876219.004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c4ad/7568155/f45547681822/CRIU2020-8876219.005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c4ad/7568155/6c0b8c85c917/CRIU2020-8876219.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c4ad/7568155/192ed963c608/CRIU2020-8876219.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c4ad/7568155/9250532ac25e/CRIU2020-8876219.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c4ad/7568155/4ea66169e326/CRIU2020-8876219.004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c4ad/7568155/f45547681822/CRIU2020-8876219.005.jpg

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Splenogonadal Fusion Presenting Clinically and Radiologically as a Seminoma.临床上及影像学表现为精原细胞瘤的脾性腺融合
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