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. Observation. 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. Discussion. 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.

Highlights

  • Splenogonadal fusion (SGF) is a rare congenital malformation, 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

  • By reporting an observational case and performing a review of the literature according to the CARE guidelines, we present the embryological genesis of the splenogonadal fusion, associated anatomical anomalies, and the diagnostic procedure

  • Putschar and Manion [10] describe two types of splenogonadal fusion: the continuous form characterized by the presence of a cord between the orthotopic spleen and the gonad and the discontinuous form which has no connection to the main spleen and the splenic tissue ectopic, directly fused to the testicular albuginea

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Summary

Introduction

Splenogonadal fusion (SGF) is a rare congenital malformation, 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. [2] The sex ratio is 15/1 [3]. Described for the first time in 1883 by Farthouat et al [1] The age of the patients is less than 10 years in half of the cases reported, and 82% of the cases occur in young men under the age of 30. It is often associated with other congenital anomalies and poses a problem of differential diagnosis with testicular tumors

Materials and Methods
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