Ovary Dysgerminoma in Girl of 10 Years-Old Associate with Mayer-Rokitansky-Kuster-Hauser Syndrome (Mrkh) Type 1. A Unusual Condition. Clinical Case

Introduction: Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) affects about 4,500 women around the world. This syndrome is a malformation of the female genitalia due to an embryonic development interrupt of the Müllerian (paramesonephric ducts) between the 5th and 6th week of embryonic development. On the other hand, the patients are chromosomally, phenotypically and endocrinologically normal. Clinical Case: 10-year-old female who started with 4-days abdominal pain which on physical examination had normal phenotype, His laboratories were: Liver Function Tests: lactic dehydrogenase (LDH) 1876 U/l (103-227); Alkaline Phosphatase ALP 395 U/l (64-306 U/l). EGO: leukocytosis 15-20 (<10 × c) Abundant bacteria, erythrocytes 30-40. Embryonic carcinoembryonic antigen (-), normal Human Chorionic Gonadotropin. Ascites liquid: pH 9, DHL 2225 U/l, Hb (+++), 100-150 leukocytes × C, countless erythrocytes. An exploratory laparotomy a bilateral ovarian tumor with uterine agenesis (Müllerian alteration) was found, which were resected to obtain a tumor of 550 g of 20 × 12 × 10 cm. Computed tomography is then performed. The histopathology service reports dysgerminoma. The patient satisfactorily recovers from her surgical intervention and is sent to the oncology department for her tumor treatment. Discussion: In our case postoperative computed tomography showed no renal damage and the imaging study (CT) was performed after its surgical intervention; genetic origin has been extensively documented in multiple studies. Our case presented cancer developed on the right ovary and incipient cancer on the left ovary as well as retroperitoneal metastatic lesion below the right kidney. This case is unfavorable because of its association with ovarian cancer at an early age and metastatic to retroperitoneum.