Dermatologie pédiatrique clinique Libre accès

Abstrait

Pédiatrie clinique 2020 : Tumeur du sac vitellin testiculaire chez une patiente de dix-sept ans - Leya Imanuelle Martin - Centre médical de l'hôpital de l'Enfant Jésus

Leya Imanuelle Martin

Présentation du cas :

Un patient de dix-sept ans présentant depuis six mois une masse scrotale gauche en constante expansion sans effets secondaires. Quatorze jours avant la confirmation, il a développé une fièvre modérée sans frissons le soir, accompagnée d'une diarrhée inactive. Une semaine plus tard, il a perdu du poids avec une diminution de l'appétit après la fin du traitement. L'examen physique a révélé une masse scrotale gauche ferme, souple et non douloureuse avec des adénopathies inguinales. L'alpha-foetoprotéine, la bêta-HCG et la LDH étaient totalement élevées. La tomodensitométrie a révélé de nombreux centres augmentés dans les poumons, les zones médiastinales, le foie et le pancréas. Orchidectomie extrême gauche tolérante avec examens histopathologiques révélant une tumeur du sac vitellin. Il a commencé une chimiothérapie de type BEP (bléomycine, étoposide et cisplatine). Son dernier traitement pour le quatrième cycle de chimiothérapie a montré une diminution significative des taux de marqueurs tumoraux. Les filtres CT ont révélé une libération des boutons d'aspiration et une diminution de la taille des masses de la queue du foie et du pancréas.

Conclusion:

La tumeur du sac vitellin est une maladie rare chez les jeunes, dont la fréquence a augmenté au cours des dernières décennies. Les progrès réalisés dans le domaine du diagnostic et de l'imagerie, ainsi que dans la chirurgie et la chimiothérapie à base de cisplatine, ont fait de cette maladie une maladie à haut risque d'évolution.

Introduction:

Testicular tumors are uncommon in adolescence with larger part analyzed to be dangerous. Germ cell tumors (GCT), remarkably the most widely recognized of threatening gonadal tumor involves just 3% of pediatric malignant growths. Yolk sac tumor has been reported to have the most elevated rate among harmful GCT in kids. A yolk sac tumor is an uncommon, dangerous tumor of cells that line the yolk sac of the incipient organism. These cells ordinarily become ovaries or testicles; be that as it may, the reason for a yolk sac tumor is obscure. It is regularly found in kids before the ages of 1 to 2, however can happen all through life. The term yolk sac tumors envelops numerous sorts of tumors including germ cell tumors, teratomas, embryonal carcinoma, aysgermenomas, semenoma, and so forth. These tumors can happen in the testicles, ovaries, chest, mind and different pieces of the body. Testicular yolk sac tumors (otherwise called endodermal sinus tumor of the testis) is the most well-known youth testicular tumor (80%), with most cases happening before the age of two years. In grown-ups, unadulterated yolk sac tumor is incredibly uncommon, anyway blended germ cell tumor are usually observed. Totipotent cells, which later structure extraembryonic fetal films, offer ascent to yolk sac tumors. Perivascular structures called Schiller-Duval bodies can be found in half of tumors and are pathognomonic. AFP (alpha fetoprotein) is raised in >90% instances of yolk sac tumor. Perceptibly, the testis is supplanted by a coagulated mass. Minutely, a positive response for AFP is found in tumor cells. Planned radiologic portrayal of yolk sac tumor is troublesome, anyway a heterogeneous testicular mass favors non-seminomatous germ cell tumor (NSGCT), and at age <2 years, yolk sac tumor is the supported determination. Heterogeneous testicular mass sore with post-differentiate heterogeneous upgrade, with zones of drain/rot. In the event that the tumor is confined to the testis, and if the serum AFP isn't raised, orchiectomy is the favored treatment, with close development. In the event that backslide happens, chemotherapy is the treatment of decision. The lungs are the most well-known site of recurrence. Testicular tumors are exceptional in kids, involving around 1% to 2% of every single pediatric harm. Be that as it may, the rate of testicular tumors in youngsters is expanding, and related bleakness has multiplied during the most recent 40 years. Testicular yolk sac tumors represent 70% to 80% of prepubertal harmful testicular tumors and are the most widely recognized youth testicular disease. The anticipation of testicular yolk sac tumors is subject to early discovery and treatment. Pediatric patients with testicular yolk sac tumors generally present with an asymptomatic scrotal mass beginning phase (I) in the infection procedure. Assessment of the strong scrotal mass incorporates: scrotal ultrasound; chest, stomach, and pelvic registered tomography (CT); and assurance of serum tumor marker levels, for example, alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (β-hCG). A raised serum AFP level is firmly connected with yolk sac tumors in over 90% of patients. The treatment of testicular yolk sac tumors is subject to tumor stage and patient age. Resection and chemotherapy with or without retroperitoneal lymph hub dismemberment (RPLND) is frequently utilized for kids with raised or rising AFP levels as well as retroperitoneal lymphadenopathy.