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.
RésuméIntroduction : Les tumeurs rétropéritonéales forment un groupe hétérogène de tumeurs rares (< 1 % de toutes les tumeurs), dont 80 % sont malignes. Leur identification et la connaissance de leur pronostic sont les conditions essentielles à leur prise en charge thérapeutique. Le diagnostic positif était fait par TDM abdomino-pelvienne. Le traitement était surtout chirurgical, visant l'exérèse complète de la masse tumorale, avec ou sans thérapie adjuvante (radiothérapie, chimiothérapie) selon l'analyse histologique. Les tumeurs mésoder-miques sont les plus fréquentes (63,6 % ; n = 5) : deux liposarcomes (18 %), deux lymphomes malins (18 %), un hémangiopéricytome (9 %), un léiomyosarcome (9 %), et un cas de rhabdomyosarcome (9 %). Le taux de tumeurs ectodermiques était de 27,4 % (n = 3) ; il s'agit de deux cas de schwannome (18 %) et un cas de ganglioneuroblastome (9 %). Aucune tumeur vestigiale, contre un cas de tumeur pseudo-inflammatoire (9 %). Une évolution favorable a été obervée chez près de la moitié des patients (40 %); on a noté quatre cas de récidive, trois cas de patients perdus de vue, et un décès. Conclusion : Dans notre série, les tumeurs mésodermiques malignes restent les plus fréquentes, sans prédominance d'âge et de sexe. Les tumeurs rétropéritonéales gardent un pronostic sombre, étant donné que nos patients sont vus à un stade tardif, et aussi du fait du potentiel malin et récidivant de ces tumeurs.
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