Ovarian Tumors in Children



Ovarian Tumors in Children


Ann M. Polcari



RELEVANT ANATOMY



  • The ovary is a female reproductive organ suspended within the pelvis.


  • It is kept in close proximity to the uterus and fallopian tubes by several ligaments (Figure 55.1A):



    • Directly connected to the uterus via the ovarian ligament.


    • Directly connected to the pelvic wall via the suspensory, or infundibulopelvic, ligament.


    • Covered by the broad ligament, a wide, fibrous tissue connecting and covering the uterus, fallopian tubes, and ovaries. It is divided into 3 continuous parts: the mesometrium, mesosalpinx, and mesovarium.


  • The ovary has dual blood supply: from the uterine arteries, found within the broad ligament, and from the ovarian artery, found in the suspensory ligament (Figure 55.1B).



    • The uterine artery branches off of the internal iliac, whereas the ovarian artery branches directly off the descending aorta.


  • Venous drainage parallels the arterial supply.


  • The ureters pass just posterior to the uterine artery to reach the bladder. This is a potential site of ureteral damage during pelvic surgery.


EPIDEMIOLOGY AND ETIOLOGY



  • Ovarian masses are the most common genital neoplasm in children.1


Etiology



  • Most ovarian tumors in children and adolescents are germ cell tumors (GCTs), meaning they arise from the precursor cells of the ovum (Figure 55.2).2



    • Other, rarer tumors, are derived from the epithelial lining, stromal tissue (ie, hormone-producing cells), or a mixture of these tissue types (Figure 55.3).



  • Some are associated with syndromes, such as Peutz-Jeghers syndrome, Ollier disease, and Maffucci syndrome.3



    • Gonadoblastoma, a mixed germ cell-stromal tumor, is found in girls with Turner syndrome and those with gonadal dysgenesis.2






Figure 55.1 Uterus, uterine tubes, and broad ligament. Relationship of the broad ligament to the ovary and its ligaments (A, anterior view). Sagittal sections showing the mesentery of the uterus (mesometrium), ovary (mesovarium), and uterine tube (mesosalpinx) (B, anterolateral view). (Reprinted with permission from Moore KL, Agur AMR, Dalley AF. Essential Clinical Anatomy. 5th ed. Philadelphia, PA: Wolters Kluwer Health; 2015.)


Incidence



  • Benign and malignant ovarian lesions occur in 2.6 per 100 000 girls less than 15 years of age.3


Prevalence



  • 1.2% of ovarian cancers are found in woman less than 19 years of age.



    • 15% of ovarian tumors in children are epithelial-derived. These are more common in adults.3




    • Approximately 75% of ovarian tumors in women up to age 20 years are germ cell tumors; in premenarchal girls, 90% are GCTs.2



      • 95% of GCTs are benign teratomas (Table 55.1). The remaining 5% are malignant and include the following major subtypes (Table 55.2):



        • Dysgerminomas


        • Immature teratomas


        • Mature teratomas with somatic malignancies


        • Yolk sac tumors


        • Embryonal carcinomas


        • Choriocarcinomas


        • Mixed germ cell tumors


      • Metastatic disease to the ovaries in children and adolescents is very rare.






Figure 55.2 Classification of germ cell tumors of the ovary. (Reproduced from Strayer DS, Rubin E, eds. Rubin’s Pathology: Clinicopathologic Foundations of Medicine. 7th ed. Philadelphia, PA: Wolters Kluwer; 2015.)






Figure 55.3 Fetal ovary at 18 weeks’ gestation. Coelomic epithelium is responsible for epithelial malignancies. This layer is lost in testicular development, thus low frequency of epithelial tumors in testes. Gonadal stromal tumors arise in a specialized stromal layer. (Reprinted with permission from Pizzo PA, Poplack DG, Adamson PC, Blaney SM, Helman L. Principles and Practice of Pediatric Oncology. Philadelphia, PA: Wolters Kluwer; 2016.)


CLINICAL PRESENTATION

Classic presentation: Abdominal pain then increases over time, associated with abdominal distension.



  • >50% of children with ovarian tumors will have a palpable abdominal mass on physical examination.3



    • Pelvic examination is reserved for sexually active adolescents only.


  • Other symptoms include anorexia, nausea or vomiting, and urinary frequency or urgency due to mass effect on the bladder.


  • Chief complaint may be a result of hormones secreted by the tumor:



    • Precocious puberty is a common chief complaint in stromal cell tumors that secrete estrogen. Virilization is uncommon but can be seen in the rare Sertoli-Leydig or steroid-producing stromal tumors.3


    • Pseudoprecocious puberty may be a presenting sign of GCTs that produce β-human chorionic gonadotropin (β-hCG), which stimulates excess estrogen production.3


  • May be discovered incidentally on imaging.


May 5, 2019 | Posted by in PEDIATRICS | Comments Off on Ovarian Tumors in Children
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