Ovarian cysts and neoplasms in infant , children and adolescents T Allameh MD Associated professor of Ob& Gyn fellowship of Gynecology oncology

Ovarian cysts in the fetus Follicular ovarian cysts in fetuses and neonates are common and increase in advancing gestational age and diabetes mellitus, preeclampsia and rhesus isoimmunization Diagnosis by sonography * female sex * non midline cystic structure *Normal urinary tract * Normal GI simple cysts < 2cm are physiologic Congenital hypothyroidism is associated with fetal ovarian cysts

Management and outcome Spontaneous regression by 6 months of age

Ovarian cyst in neonate Spontaneous regression by 4- 6 months age Serial ultrasound at birth and every 4-6 weeks until the cysts resolves Aspiration of simple cysts > 4-5 cm Surgical intervention for complex cysts increasing in size symptomatic and persisting for more than 4-6 months Malignancy ? Torsion ?

Surgical excision of cyst Complex Symptomatic Increasing in size Persisting for more than 4-6 months.

Outcome Spontaneous regression 50% resolves in first 3 months 30-40% torsion

Ovarian cysts in infants and prepubertal children Physiologic cysts are uncommon Some ovarian cysts are hormonally active and result in precocious pseudopuberty Premature vaginal bleeding and ovarian enlargement ( McCune –Albright syndrome? ) Idiopathic precocious puberty ( ovarian cyst in response to GNRH)

Clinical manifestation In early life the ovary in abdominal organ and more susceptible to torsion Perforation, infarction and hemorrhage

Evaluation Ultrasonography Doppler ultrasound

Management Observation 4-8 weeks or more in simple cyst and asymptomatic patients Surgery is always indicated at the time of diagnosis of ovarian torsion ( oophoropexy?)

Ovarian cysts in adolescents Clinical features Simple or complex

Differential diagnosis Obstructive genital lesions ( imperforate hymen , noncommunicating uterine horn ) Ovarian tumors ( benign cystic tratuma , serous or mucinous cyst adenoma ) Tubal condition ( paratubal cyst, EP, hydrosalpinx ) Uterine mass( myoma, pregnancy) GI condition ( appendicular abscess )

Management Follicular cyst ( simple cyst <6cm can be observed ) In symptomatic patients laparoscopic cystectomy Chorpus luteum cyst 5-12 cm observation is recommended * will involute during the2 weeks to 3 months * surgery is rarely needed

Ovarian neoplasms 1% of all tumors in children and adolescents Fewer than 5% of ovarian malignancy occur in this group 35- 45% of ovarian cancers in children are germ cell tumors

Ovarian neoplasms Any mass that not resolves spontaneously needs to be further evaluating (benign or malignant) A persistent simple ovarian mass is most likely a mucinous or serous cyst adenoma A persistent complex mass is most likely a germ cell tumor The most common germ cell tumor in an adolescent is a teratoma Teratoma can be benign ( mature teratuma) or malignant (immature teratoma)

Ovarian neoplasms Ovarian cancer is the most common gynecologic malignancy in women <25y Germ cell tumors make up one half to two third of ovarian neoplasm in girls up to 18 y( compare with 20% of ovarian tumors in adult) Epithelial neoplasm are rare in the prepubertal age group

Clinical manifestation Abdominal pain Increasing abdominal girth, nausea and vomiting Asymptomatic Abdominal palpation Rectal examination in the dorsal supine position (in any girl with non specific abdominal or pelvic complains )

Imaging Sonography ( simple ,complex, solid, bilateral ,free fluid ) Doppler sono Age Clinical manifestation Tumor marker A solid ovarian mass in childhood is always considered malignant until proven otherwise by histological examination

Differential diagnosis of solid tumors Dysgerminomas Neuroblastoma Willms` tumor Rhabdomyosarcoma Lymphoma Leukemia

Tumor marker Alpha fetoprotein ( endodermal sinus tumor , mixed germ cell tumor and immature teratoma) LDH ( dysgerminoma ) CA-125 (epithelial ovarian cancer) hCG ( embryonal ovarian carcinomas, choriocarcinoma ) CEA ( epithelial or germ cell) Inhibin and mullerian inhibiting substance (MIS) Granulosa theca cell tumor Thrombocytosis (suspicious for malignancy)

Treatment Surgical staging Preservation of reproductive and sexual function Excision of the lesion Large ovarian cyst can be removed with preservation of the normal ovarian cortex If malignancy is suspected , a unilateral salpingo oophorectomy and appropriate staging is performed it is preferable to subject the patient to a second procedure after the final pathology