Version May 2024
INTRODUCTION — Adnexal/ovarian masses occur in female infants, children, and adolescents. An adnexal mass derives from the ovary, tube, or broad ligament. These lesions may present with associated symptoms or signs or be identified through imaging studies. The potential causes vary with age. Although most ovarian masses in children are physiologic ovarian cysts or benign ovarian tumors, early diagnosis is necessary to reduce the risk of ovarian torsion and to improve the prognosis for children with malignant neoplasms.
The evaluation of ovarian masses in infants, children, and adolescents will be discussed here. Ovarian cysts in children and adolescents are discussed separately. (See "Ovarian cysts in infants, children, and adolescents".)
CLINICAL PRESENTATIONS — Adnexal/ovarian masses in infants, children, and adolescents may be an incidental imaging finding in the evaluation of a different complaint or may present with the symptoms and signs listed below [1-6]. Although symptoms correlate with initial size (eg, large masses may obstruct other organs) and pathology (eg, precocious puberty and sex cord-stromal tumor), they do not accurately predict whether the mass is a malignant tumor [5].
●Abdominal pain – Abdominal pain is the most common presenting symptom of ovarian/adnexal masses, occurring in 45 to 80 percent of patients in case series [7-9].
Acute severe abdominal pain may indicate ovarian torsion or rupture with hemorrhage, complications of ovarian masses that may be the presenting manifestation. Intermittent severe abdominal pain that resolves spontaneously is suggestive of intermittent, partial, or impending ovarian torsion). (See "Ovarian cysts in infants, children, and adolescents", section on 'Complications of ovarian cysts' and "Ovarian and fallopian tube torsion".)
●Palpable abdominal or pelvic mass.
●Symptoms related to compression of other organs, particularly if the mass is large (eg, nausea, vomiting, abdominal fullness, constipation, feelings of pressure in the lower abdomen, urinary frequency or retention).
●Bloating or increasing abdominal girth.
●Precocious puberty (central or peripheral) or virilization.
●Menstrual irregularities (in postmenarchal adolescents).
●Paraneoplastic or autoimmune syndrome (eg, opsoclonus-myoclonus, dermatomyositis [10], encephalitis).
Anti-N-methyl-D-aspartate receptor encephalitis is an autoimmune and paraneoplastic encephalitis associated with ovarian teratomas [11-15]. (See "Autoimmune (including paraneoplastic) encephalitis: Clinical features and diagnosis", section on 'Anti-NMDA receptor encephalitis'.)
CAUSES OF ADNEXAL MASS
Ovarian masses — Most ovarian masses in children and adolescents are physiologic ovarian cysts or benign ovarian tumors (table 1 and table 2) [16,17].
Physiologic (functional) ovarian cysts — Physiologic ovarian cysts (enlargement of ovarian follicles) are common in infants, children, and adolescents, accounting for approximately 45 percent of adnexal abnormalities in children [6].
In the pediatric age group, ovarian cysts have a bimodal distribution, with peaks in the fetal/neonatal and perimenarchal/menarcheal periods [18]. In a retrospective review of 1009 girls (age 5 to 18 years) who presented to a pediatric emergency department with pelvic pain, the incidence of ovarian cyst ≥1 cm in diameter was 13 percent overall, 2 percent in those age 5 to 9 years, and 19 percent in those age 10 to 19 years [19].
Ovarian cysts in children and adolescents are discussed separately. (See "Ovarian cysts in infants, children, and adolescents".)
Benign and malignant ovarian tumors — Ovarian tumors (whether benign or malignant) are rare in children and adolescents. They account for only 1 to 2 percent of all tumors in this population [20], with an incidence of approximately 3 per 100,000 girls per year [21]. For malignant ovarian tumors, the age-adjusted annual incidence is 0.102 per 100,000 girls age <9 years and 1.072 per 100,000 girls age 10 to 19 years [22]. Although the age-adjusted incidence of malignant ovarian tumors is higher in girls >10 years, among girls who present with ovarian mass, malignant ovarian tumors are more common in prepubertal than postpubertal females (given the relative increased frequency of ovarian cysts in peripubertal/pubertal females). Despite the rarity of malignant ovarian tumors in children and adolescents, in retrospective case series, approximately 10 to 20 percent of ovarian masses that were treated surgically were malignant [1,16,23-28].
The presentation of ovarian tumors in children and adolescents varies widely. Some children present with complaints of abdominal pain, increasing abdominal girth, nausea, and/or vomiting; in others, the ovarian mass is an incidental finding on examination or imaging [1-4]. In observational studies, clinical features that are more often associated with malignant than benign tumors include bilateral masses, fixed masses with irregular borders, ascites, and complaints of precocious puberty [24,29]. Nonspecific symptoms may be more common with epithelial than germ cell ovarian tumors. (See "Ovarian germ cell tumors: Pathology, epidemiology, clinical manifestations, and diagnosis" and "Epithelial carcinoma of the ovary, fallopian tube, and peritoneum: Clinical features and diagnosis", section on 'Clinical presentation'.)
Elevated platelets are a nonspecific marker of ovarian malignancy in children and adolescents and may be particularly helpful in the acute evaluation of ovarian mass with torsion (the platelet count is not typically elevated in ovarian torsion without malignancy) [27,30,31].
The World Health Organization classifies ovarian tumors according to histologic cell type (table 3).
●Germ cell tumors – The majority of ovarian tumors in children and adolescents are of germ cell origin (eg, mature teratoma [benign], immature teratoma [malignant], gonadoblastoma [benign], dysgerminoma [malignant]) (table 2) [16,32,33]. Approximately 35 to 45 percent of ovarian cancers in children are germ cell tumors.
Germ cell tumors are discussed separately. (See "Ovarian germ cell tumors: Pathology, epidemiology, clinical manifestations, and diagnosis".)
●Epithelial tumors – Epithelial tumors (eg, serous or mucinous cystadenoma [benign]) are rare in prepubertal children. They are discussed separately. (See "Overview of epithelial carcinoma of the ovary, fallopian tube, and peritoneum" and "Epithelial carcinoma of the ovary, fallopian tube, and peritoneum: Clinical features and diagnosis".)
●Sex cord-stromal tumors – Sex cord-stromal tumors (eg, thecomas, fibromas, juvenile granulosa cell tumor, Sertoli-Leydig cell tumors) are rare in children and adolescents [34]. They may present with isosexual or heterosexual precocious puberty.
Sex cord-stromal tumors are discussed separately. (See "Sex cord-stromal tumors of the ovary: Epidemiology, clinical features, and diagnosis in adults" and "Sex cord-stromal tumors of the ovary: Management in adults".)
Other adnexal masses — Other adnexal masses that can mimic ovarian masses include (table 1) [34]:
●Paratubal cysts (eg, cysts of the broad ligament, mesonephric cysts [hydatid cysts of Morgagni]) and paraovarian cysts can range in size from a few millimeters to 15 cm or larger [6,35]
●Endometrioma (rare in adolescents but does occur)
●Ectopic pregnancy
●Tubo-ovarian abscess (polymicrobial infection of the fallopian tube and/or ovary that results from ascending or intra-abdominal spread of infection); tubo-ovarian abscess can occur in patients who are not sexually active, usually due to abdominal spread of infection from a ruptured appendix or bowel and/or bladder surgery [36,37]
●Hydrosalpinx or pyosalpinx (distally obstructed fallopian tube filled with serous or clear fluid [hydrosalpinx] or pus [pyosalpinx]); may be associated with segmental tubal agenesis
Other pelvic masses — Masses in the pelvis usually originate in the reproductive organs but also can arise from the urinary tract, bowel, or other pelvic structures [34].
Other causes of pelvic mass include [6,34]:
●Reproductive tract anomalies – Imperforate hymen, agenesis of the lower vagina, hydrometrocolpos, hematometrocolpos, transverse vaginal septum, noncommunicating uterine horn, obstructed hemivagina with ipsilateral renal anomaly (see "Congenital anomalies of the hymen and vagina")
●Urinary tract disorders – Urinary tract obstruction, urachal cyst, renal cyst, ureteric stone
●Gastrointestinal tract disorders – Mesenteric or omental cyst, biliary cyst, pancreatic cyst, volvulus, colonic atresia, intestinal duplication, peritoneal inclusion cyst; appendiceal abscess, diverticular abscess
●Other pelvic structures – Adrenal cyst, splenic cyst, presacral teratoma, anterior meningocele, neuroblastoma, lymphangioma
EVALUATION OF OVARIAN MASSES — Although most ovarian masses in children and adolescents are physiologic cysts or benign ovarian tumors, early diagnosis is necessary to reduce the risk of ovarian torsion and to improve the prognosis for malignant neoplasms [34].
Patients with acute severe abdominal pain — Children and adolescents with ovarian mass and acute, severe abdominal pain (eg, guarding, percussive tenderness, rebound tenderness) require urgent evaluation for life-threatening or serious causes (eg, ovarian torsion, ruptured hemorrhagic ovarian cyst or neoplasm, ectopic pregnancy, tubo-ovarian abscess, appendicitis) [29]. (See "Causes of acute abdominal pain in children and adolescents" and "Emergency evaluation of the child with acute abdominal pain".)
Patients without acute severe abdominal pain
History and examination — Important aspects of the history and examination in children and adolescents with ovarian mass without acute severe abdominal pain include [29]:
●For all patients:
•Characteristics of the mass – Malignant tumors more likely to be bilateral, solid, fixed, or irregular
•Abdominal distension – Abdominal distension and/or ascites are more common in malignant than benign ovarian lesions [5,38]
●For neonates and infants – Whether an ovarian cyst was noted on antenatal ultrasonography (fetal and neonatal ovarian cysts usually resolve spontaneously by six months of age)
●For prepubertal children:
•Increased height velocity (may indicate the onset of puberty, which is associated with increased incidence of physiologic cysts; rarely may indicate hormone producing tumors)
•Signs of early puberty (eg, breast budding before age seven years), which may occur in children with an ovarian tumor or central or peripheral precocious puberty
•Virilization (eg, clitoromegaly, acne), which may indicate Sertoli-Leydig cell tumor
●For adolescents:
•Menstrual history, including milestones of pubertal development, last menstrual period, dysmenorrhea or irregular menses (to evaluate the possibility of physiologic cysts, endometriosis)
•Sexual history to evaluate risk of pregnancy-associated cyst or tubo-ovarian abscess
•Symptoms and signs of sexually transmitted infections (STIs; vaginal discharge, genital ulcers) or pelvic inflammatory disease (eg, cervical motion, uterine, and adnexal tenderness), which may be associated with hydrosalpinx or tubo-ovarian abscess
Imaging — Transabdominal ultrasonography is the first-line imaging modality to evaluate adnexal masses in children and adolescents [4]. Ultrasonography provides information about the size and origin of the mass (eg, ovarian, paraovarian), the consistency (eg, cystic, solid), laterality, and associated findings (eg, ascites, lymphadenopathy) [5,39]. The pattern of blood supply can be evaluated by Doppler flow characteristics [40]. The ultrasonographic findings help to narrow the list of potential causes.
If the origin of the mass is uncertain after ultrasonography, or the tumor is large or suspected to be malignant, additional information (eg, pelvic lymph nodes, metastases in the lung or liver) can be obtained with computed tomography (CT) or magnetic resonance imaging (MRI) [34].
On ultrasonography, ovarian masses are characterized as:
●Simple cysts – Simple cysts are anechoic without septations, solid elements, or mural nodules; they may have ≤1 peripheral calcification; and they lack internal Doppler flow [4,27].
Most simple cysts in children and adolescents are physiologic cysts, which usually resolve spontaneously. (See "Ovarian cysts in infants, children, and adolescents".)
Mucinous and serous cystadenomas (benign epithelial tumors) are a common cause of persistent simple ovarian cysts in children and adolescents [4,41].
●Complex masses – Complex ovarian masses are cystic with solid nodular or papillary components (<50 percent), wall thickening, septations (>2 to 3 mm), multiple calcifications, or mural nodules [4,27,42,43].
Most complex ovarian masses in children and adolescents are self-limiting hemorrhagic cysts (which typically resolve within two to eight weeks).
Causes of complex ovarian masses that may present acutely include ovarian torsion, tubo-ovarian abscess, and ectopic pregnancy. (See "Causes of acute abdominal pain in children and adolescents".)
Causes of persistent complex ovarian masses include mature teratomas (benign germ cell tumors), immature teratomas (malignant germ cell tumors), and endometriomas (ie, endometrioma [endometriosis growing within the ovary]) [34].
●Solid masses – Predominantly solid (ie, ≥50 percent solid components) masses are considered malignant until histologic examination proves otherwise [44].
Causes of solid ovarian masses in children and adolescents include germ cell tumors (eg, dysgerminoma), sex cord-stromal cell tumors (eg, juvenile granulosa cell tumor, Sertoli-Leydig cell tumor), Wilms tumor, neuroblastoma, rhabdomyosarcoma, lymphoma, and leukemia [4].
Additional information can be obtained with CT or MRI.
●Ultrasonographic findings associated with malignant tumors – Ultrasonographic findings that are more suggestive of malignant tumors include [3,5,20,24,27-29]:
•Size ≥8 to 10 cm
•Multiple lesions
•Bilateral masses
•Solid or heterogeneous (solid components >2 cm, thick septations, papillary projections), compared with cystic and homogeneous
•Invasive or metastatic compared with well-circumscribed
•Calcifications
•Ascites
•Increased blood flow (compared with minimal or no blood flow)
Laboratory studies — The laboratory evaluation for children and adolescents with an ovarian mass varies with clinical features [29]:
●Postmenarchal adolescents – Urine beta-human chorionic gonadotropin test (beta-hCG) to exclude pregnancy (regardless of history) [5].
●Signs or symptoms of STIs – Testing for STIs.
●Increased suspicion for ovarian tumor – The suspicion for ovarian tumor is increased in patients with ultrasonographic features associated with malignancy, evidence of precocious puberty or virilization, or constitutional symptoms [6,29]. (See 'Imaging' above.)
For patients with increased suspicion for ovarian tumor, laboratory evaluation includes [5,34]:
•A panel of tumor markers (alpha-fetoprotein, beta-hCG, lactate dehydrogenase, inhibin A, inhibin B, and cancer antigen-125); estradiol and testosterone are obtained to evaluate hormonally active tumors (eg, in patients with precocious puberty or virilization) (table 4).
Some ovarian tumors secrete protein tumor markers that can be assayed from peripheral blood samples. Elevated tumor markers can be helpful in making a diagnosis and monitoring the response to treatment [3,5,6]. However, the absence of elevated tumor markers does not exclude malignancy, and elevated tumor markers may be present in benign tumors [5,38,45]. Using a panel of ovarian tumor markers increases the sensitivity and specificity (given the range of potential ovarian tumors) [20,43,46].
•Cytology of ascites fluid (if obtained).
•Platelet count – Elevated platelets are a nonspecific marker of ovarian malignancy and may be helpful in the acute evaluation of ovarian mass with torsion (the platelet count is not typically elevated in ovarian torsion without malignancy) [27,30,31].
MANAGEMENT
Education — Ovarian torsion is a potential complication of ovarian masses. Caregivers of infants and children and adolescent patients with ovarian masses should be counseled regarding the signs and symptoms of ovarian torsion (eg, severe unilateral lower abdominal pain or extreme fussiness of acute onset in the neonate or young infant) so they can seek emergency care without delay. (See "Ovarian cysts in infants, children, and adolescents", section on 'Ovarian torsion'.)
Indications for referral — Girls with ovarian mass and precocious puberty or rapid virilization should be referred to an endocrinologist and/or pediatric and adolescent gynecologist for additional evaluation. (See "Definition, etiology, and evaluation of precocious puberty".)
Indications for referral to a surgeon (eg, pediatric and adolescent gynecologist [preferred], gynecologic surgeon experienced in the management of young patients, general gynecologist, pediatric surgeon) or multidisciplinary team (eg, pediatric oncologist, pathologist, fertility expert) include [5,6,23,28,29]:
●Suspected ovarian torsion (emergency referral)
●Symptomatic ruptured/hemorrhagic cyst (urgent referral)
●Clinical or ultrasonographic features associated with neoplasm (eg, complex/solid mass, ascites, positive tumor markers)
●Persistent simple cyst
●Uncertain origin of the mass (eg, ovary versus paraovarian)
●Increase in size or failure to resolve on serial imaging
Detailed discussion of surgical management of ovarian masses (eg, choice of procedure) is beyond the scope of this review. The goals of surgical management include definitive diagnosis, complete removal of neoplastic tissue and staging for malignancy (in girls with ovarian tumors), preservation of ovarian tissue and function (if possible), and relief of symptoms [5,6,20,29,47].
Conservative surgery (eg, excision of the lesion and ovarian preservation) is usually undertaken unless a malignancy is highly suspected (based on imaging and elevated tumor markers) or is definitively diagnosed on frozen section at the time of the procedure [4,34]. Even large ovarian cysts (with negative tumor markers) can be removed with preservation of the normal ovarian cortex [48]. Although a second procedure, for cancer staging, may be necessary after the final pathology specimens are reviewed, initial conservative surgery avoids performing an unnecessary ablative procedure. If malignancy is suspected or confirmed, adequate staging includes abdominal and pelvic exploration, peritoneal washings, biopsies of suspicious areas, and periaortic and pelvic lymph node sampling. A multidisciplinary team approach for presumed ovarian malignancies in children and adolescents is recommended [49].
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Ovarian and fallopian tube disease".)
SUMMARY AND RECOMMENDATIONS
●Clinical presentation – Adnexal/ovarian cysts/masses in infants, children, and adolescents may be an incidental imaging finding in the evaluation of a different complaint or may present with symptoms (eg, abdominal pain, palpable abdominal mass, bloating, menstrual irregularities, paraneoplastic or autoimmune syndromes). (See 'Clinical presentations' above.)
●Causes – Causes of ovarian masses in children and adolescents include physiologic ovarian cysts, benign and malignant ovarian tumors, other adnexal masses, and other causes of pelvic mass (table 1 and table 2). Most ovarian masses in children and adolescents are physiologic cysts or benign ovarian tumors. (See 'Causes of adnexal mass' above.)
●Evaluation – Early diagnosis is necessary to reduce the risk of ovarian torsion and to improve the prognosis for malignant neoplasms. (See 'Evaluation of ovarian masses' above.)
•Patients with acute severe abdominal pain – Children and adolescents with ovarian mass and acute severe abdominal pain (eg, guarding, percussive tenderness, rebound tenderness) require urgent evaluation for life-threatening or serious causes (eg, ovarian torsion, ruptured hemorrhagic ovarian cyst or neoplasm, ectopic pregnancy, tubo-ovarian abscess, appendicitis). (See "Causes of acute abdominal pain in children and adolescents" and "Emergency evaluation of the child with acute abdominal pain".)
•Patients without acute severe abdominal pain – The evaluation for children without acute severe abdominal pain includes history and examination, transabdominal ultrasonography, and laboratory testing tailored to the clinical findings (table 5). (See 'Patients without acute severe abdominal pain' above.)
●Management – The management of ovarian masses in children and adolescents varies with the underlying cause.
•Education – Ovarian torsion is a potential complication of ovarian masses. Caregivers of infants and children and adolescent patients with ovarian masses should be counseled regarding the signs and symptoms of ovarian torsion (eg, severe unilateral lower abdominal pain or extreme fussiness of acute onset in the neonate or young infant) so they can seek emergency care without delay. (See 'Education' above.)
•Indications for referral – Girls with ovarian mass and precocious puberty or rapid virilization should be referred to an endocrinologist for additional evaluation. (See "Definition, etiology, and evaluation of precocious puberty".)
Indications for referral for surgical evaluation include (see 'Indications for referral' above):
-Suspected ovarian torsion (emergency referral)
-Symptomatic ruptured/hemorrhagic cyst (emergency/urgent referral)
-Clinical or ultrasonographic features associated with neoplasm (eg, complex/solid mass, ascites, positive tumor markers)
-Persistent simple cyst
-Uncertain origin of the mass (eg, ovary versus paraovarian)
-Increase in size or failure to resolve on serial imaging
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