Ovarian Tumors in Children
Ann M. Polcari
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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
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
Benign and malignant ovarian lesions occur in 2.6 per 100 000 girls less than 15 years of age.3
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):
Mature teratomas with somatic malignancies
Yolk sac tumors
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.)
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.
Laboratory tests are used largely to exclude malignancy once a mass is identified on physical examination or imaging.
α-Fetal protein (AFP) and β-hCG should be ordered, particularly if there is suspicion of a GCT.
AFP is produced by the fetal yolk sac during embryonic development and is therefore secreted by yolk sac tumors. Note that its use is limited in the first month of life, as levels are naturally high in newborns.3
β-hCG is produced by syncytiotrophoblasts during early embryonic development. If measured greater than 100 ng/mL, one should be concerned about a dysgerminoma, embryonic carcinoma, or choriocarcinoma.3
Lactate dehydrogenase (LDH) may correlate with disease activity because it indicates increased cell turnover.4
CA-125 is the tumor marker most commonly elevated with epithelial ovarian tumors, more common in adults. It can, however, be elevated in pediatric GCTs.3
If signs of precocious puberty are present, the following hormone levels should be assessed: estradiol, inhibin, follicle-stimulating hormone (FSH), luteinizing hormone (LSH), and thyroid-stimulating hormone (TSH). This is rarely required in the pediatric population.3
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