Evaluation of Ovarian Masses in Infants, Children, and Adolescents

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Evaluation of ovarian masses in infants, children, and adolescents

Author: Marc R Laufer, MD
Section Editor: Amy B Middleman, MD, MPH, MS Ed
Deputy Editor: Mary M Torchia, MD
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Jun 2021. | This topic last updated: Jul 09, 2020.
INTRODUCTION
Ovarian masses occur in female infants, children, and adolescents. They 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
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.
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● 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 "Paraneoplastic and autoimmune encephalitis", section
on 'Anti-NMDA receptor encephalitis'.)
CAUSES OF OVARIAN MASS
Most ovarian masses in children and adolescents are physiologic ovarian cysts or benign ovarian tumors (
table 1 and table 2) [16].
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 [17]. 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 [18].
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 [19], with an
incidence of approximately 3 per 100,000 girls per year [20]. 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 [21].
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
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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 [1,16,22-
27].
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 [23,28]. 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) [26,29,30].
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) [16,31,32]. 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 [33]. 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) [33]:
● 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 [6,34]
● 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 [35,36]
● 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 [33].
Other causes of pelvic mass include [6,33]:
● 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 [33].
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) [28]. (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 [28]:
● 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 [5,37]
● 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 ovarian 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) [5,38]. The pattern of blood supply can be evaluated by Doppler flow characteristics [39].
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) [33].
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 [4,26].
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 [4,40].
● 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 [4,26,41,42].
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]) [33].
● Solid masses – Predominantly solid (ie, ≥50 percent solid components) masses are considered malignant
until histologic examination proves otherwise [43].
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 [3,5,19,23,26-28]:
• 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 [28]:
● 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 [6,28]. (See 'Imaging' above.)
For patients with increased suspicion for ovarian tumor, laboratory evaluation includes [5,33]:
• 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 [5,37,44]. Using a panel of ovarian
tumor markers increases the sensitivity and specificity (given the range of potential ovarian tumors)
[19,42,45].
• 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) [26,29,30].
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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 [5,6,22,27,28]:
● 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 [5,6,19,28].
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 [4,33]. Even large ovarian cysts (with negative tumor markers)
can be removed with preservation of the normal ovarian cortex [46]. Although a second procedure 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.
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
● Ovarian 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 of ovarian tumor 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 ovarian mass' above.)
● 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.)
● 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".)
● 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.)
● The management of ovarian masses in children and adolescents varies with the underlying cause.
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.)
● 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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Topic 127034 Version 3.0
GRAPHICS
Select nonneoplastic causes of ovarian mass in children and adolescents [1,2]
Origin Associated clinical features Ultrasonographic features
Ovarian origin
Follicular cyst Common in neonates and Simple (clear fluid filled), or

perimenarchal/menarcheal adolescents Complex (containing debris, septae, solid
components; echogenic wall)
Corpus luteal cyst Pelvic pain Complex (internal echoes)
Endometrioma ("chocolate cyst") Rare in adolescents Complex (unilocular cyst with echogenic
Bilateral in 33% debris)
Para-ovarian origin
Ectopic pregnancy Abdominal pain and vaginal bleeding Pregnancy at ectopic site
Increasing beta-hCG Extraovarian adnexal mass
Hydrosalpinx or pyosalpinx Acute pelvic pain Dilated tubular structure adjacent to ovary,
may have incomplete septations
Paraovarian/paratubal cysts (eg, Often asymptomatic Simple cysts

mesonephric cysts, cysts of the broad Abdominal pain or distension Size can range from a few millimeters to 15
ligament) Increases risk of tubal torsion cm or larger
Tubo-ovarian abscess Usually a complication of PID; may result Complex

from intra-abdominal spread Multilocular
Abdominopelvic pain Obscure normal adnexal anatomy
Fever Contains speckled fluid with internal echoes
Purulent cervical discharge (inflammatory debris)
Cervical motion tenderness
Leukocytosis
This table is meant for use with UpToDate content related to the evaluation of ovarian masses in children and adolescents. Refer to UpToDate
content for additional details.
beta-hCG: beta-human chorionic gonadotropin; PID: pelvic inflammatory disease.
References:
1. Strickland J, Laufer M. Adnexal masses. In: Emans, Laufer, Goldstein's Pediatric and Adolescent Gynecology, 7 th ed, Emans SJ, Laufer MR, Divasta AD (Eds),
Wolters Kluwer, Philadelphia 2020. p.529.
2. Kirkham YA, Kives S. Ovarian cysts in adolescents: Medical and surgical management. Adolesc Med State Art Rev 2012; 23:178.
Graphic 128370 Version 2.0
Select neoplastic causes of ovarian mass in children and adolescents [1-3]
Type of tumor Associated clinical features Ultrasonographic appearance
Benign germ cell tumors
Mature cystic teratoma Most common ovarian neoplasm in children Complex (cystic with solid components:
Also called: Often discovered incidentally calcification, echogenic material, fat-fluid
20 to 25% present with abdominal pain levels)
Mature teratoma
May present with nausea May contain thick sebaceous fluid, hair, and
Dermoid cyst (dermoid)
calcium
Benign cystic teratoma Bilateral in up to 10%
Mean tumor size 6.5 cm
Associated with anti-NMDAR encephalitis
Gonadoblastoma Typically associated with gonadal Solid mass with calcification

dysgenesis in phenotypically female May be too small to easily detect with
patients with a Y chromosome/Y ultrasonography
chromosome fragment (eg, Turner
syndrome [45XO/46XY])
Bilateral in 40%
May be associated with malignant germ cell
tumor (dysgerminoma)
Associated with disorders with mutation in
the WT1 gene (eg, Frasier syndrome and
Denys-Drash syndrome)
Benign epithelial tumors
Serous and mucinous cystadenoma 10 to 20% of ovarian tumors Serous: Large unilocular cystic masses
Usually diagnosed after menarche without septations
Mucinous: Multiloculated cystic mass
Benign sex cord-stromal tumors
Thecoma-fibroma <2% of ovarian tumors in children and Solid hypoechoic mass

adolescents
Associated with ascites and pleural effusion
(Meigs syndrome)
Associated with basal cell nevus syndrome
(Gorlin syndrome)
May produce estrogen or testosterone
Malignant germ cell tumors
Immature teratoma Approximately 10% of complex ovarian Complex or completely solid

masses in girls May contain foci of fat and calcification
Mean age at presentation: 10 years Mean diameter 16 cm (range 5 to >40 cm)
Usually presents with palpable mass, but
may be asymptomatic or present with
abdominal pain, nausea, and/or vomiting
Associated with gonadal dysgenesis
May be associated with mature cystic
teratoma in contralateral ovary
Dysgerminoma Most common malignant germ cell tumor; Solid mass with regions of necrosis,
most common ovarian malignancy in hemorrhage, and speckled calcifications
children
Usually occurs in adolescence/early
adulthood (peak incidence 15 to 19 years)
Bilateral in approximately 15%
Associated with gonadal dysgenesis
(develops within a gonadoblastoma)
Malignant sex cord-stromal tumors
Juvenile granulosa cell tumor Majority of pediatric sex cord-stromal Predominantly solid with cystic spaces or
tumors predominantly cystic with solid foci
Most occur in first or second decade Mean tumor diameter 12.5 cm
Secrete estrogen (associated with breast
enlargement and vaginal bleeding)
Associated with menstrual irregularities
Bilateral in <5%
Associated with Ollier disease and Maffucci
syndrome
Sertoli-Leydig cell tumor 20% of pediatric ovarian sex cord-stromal Variable (solid, solid and cystic,
cell tumors predominantly cystic)
<0.5% of malignant ovarian tumors in Well-circumscribed without ascites or
children calcifications
Approximately 50% occur in patients 11 to
20 years and 6% in those <11 years
Most patients present with virilization or
menstrual irregularities
Associated with DICER1 syndrome
content for additional details. All of these tumors may be complicated by ovarian/adnexal torsion or rupture with hemorrhage.
NMDAR: anti-N-methyl-D-aspartate receptor.
References:
1. Strickland J, Laufer M. Adnexal masses. In: Emans, Laufer, Goldstein's Pediatric and Adolescent Gynecology, 7 th ed, Emans SJ, Laufer MR, Divasta AD (Eds),
Wolters Kluwer, Philadelphia 2020. p.529.
2. Mahajan P, Weldon CB, Frazier AL, Laufer MR. Gynecologic cancers in children and adolescents. In: Emans, Laufer, Goldstein's Pediatric and Adolescent
Gynecology, 7 th ed, Emans SJ, Laufer MR, Divasta AD (Eds), Wolters Kluwer, Philadelphia 2020. p.556.
3. Lala SV, Strubel N. Ovarian neoplasms of childhood. Pediatr Radiol 2019; 49:1463.
WHO classification of tumors of the ovary*
Epithelial tumors
Serous tumors
Benign
Serous cystadenoma
Serous adenofibroma
Serous surface papilloma
Borderline
Serous borderline tumor/atypical proliferative serous tumor
Serous borderline tumor – micropapillary variant/noninvasive low-grade serous carcinoma
Malignant
Low-grade serous carcinoma
High-grade serous carcinoma
Mucinous tumors
Benign
Mucinous cystadenoma
Mucinous adenofibroma
Borderline
Mucinous borderline tumor/atypical proliferative mucinous tumor
Malignant
Mucinous carcinoma
Endometrioid tumors
Benign
Endometriotic cyst
Endometrioid cystadenoma
Endometrioid adenofibroma
Borderline
Endometrioid borderline tumor/atypical proliferative endometrioid tumor
Malignant
Endometrioid carcinoma
Clear cell tumors
Benign
Clear cell cystadenoma
Clear cell adenofibroma
Borderline
Clear cell borderline tumor/atypical proliferative clear cell tumor
Malignant
Clear cell carcinoma
Brenner tumors
Benign
Brenner tumor
Borderline
Borderline Brenner tumor/atypical proliferative Brenner tumor
Malignant
Malignant Brenner tumor
Seromucinous tumors
Benign
Seromucinous cystadenoma
Seromucinous adenofibroma
Borderline
Seromucinous borderline tumor/atypical proliferative seromucinous tumor
Malignant
Seromucinous carcinoma
Undifferentiated carcinoma
Mesenchymal tumors
Low-grade endometrioid stromal sarcoma

High-grade endometrioid stromal sarcoma
Mixed epithelial and mesenchymal tumors
Adenosarcoma
Carcinosarcoma
Sex cord-stromal tumors
Pure stromal tumors
Fibroma
Cellular fibroma
Thecoma
Luteinized thecoma associated with sclerosing peritonitis
Fibrosarcoma
Sclerosing stromal tumor
Signet-ring stromal tumor
Microcystic stromal tumor
Leydig cell tumor
Steroid cell tumor
Steroid cell tumor, malignant
Pure sex cord tumors
Adult granulosa cell tumor

Juvenile granulosa cell tumor
Sertoli cell tumor
Sex cord tumor with annular tubules
Mixed sex cord-stromal tumors
Sertoli-Leydig cell tumor

Well-differentiated
Moderately differentiated
With heterologous elements
Poorly differentiated
Retiform
Sex cord-stromal tumors, NOS
Germ cell tumors
Dysgerminoma
Yolk sac tumor
Embryonal carcinoma
Nongestational choriocarcinoma
Mature teratoma
Immature teratoma
Mixed germ cell tumor
Monodermal teratoma and somatic-type tumors arising from a dermoid cyst
Struma ovarii, benign

Struma ovarii, malignant
Carcinoid
Strumal carcinoid
Mucinous carcinoid
Neuroectodermal-type tumors
Sebaceous tumors
Sebaceous adenoma
Sebaceous carcinoma
Other rare monodermal teratomas
Carcinomas
Squamous cell carcinoma
Others
Germ cell-sex cord-stromal tumors
Gonadoblastoma, including gonadoblastoma with malignant germ cell tumor

Mixed germ cell-sex cord-stromal tumor, unclassified
Miscellaneous tumors
Tumors of rete ovarii

Adenoma of rete ovarii
Adenocarcinoma of rete ovarii
Wolffian tumor
Small cell carcinoma, hypercalcaemic type
Small cell carcinoma, pulmonary type
Wilms tumor
Paraganglioma
Solid pseudopapillary neoplasm
Mesothelial tumors
Adenomatoid tumor
Mesothelioma
Soft tissue tumors
Myxoma
Others
Tumor-like lesions
Follicle cyst
Corpus luteum cyst
Large solitary luteinized follicle cyst
Hyperreactio luteinalis
Pregnancy luteoma
Stromal hyperplasia
Stromal hyperthecosis
Fibromatosis
Massive oedema
Leydig cell hyperplasia
Others
Lymphoid and myeloid tumors
Lymphomas
Plasmacytoma
Myeloid neoplasms
Secondary tumors
WHO: World Health Organization; NOS: not otherwise specified.

* The classification is modified from the previous WHO classification of tumors, taking into account changes in our understanding of these lesions.
Reproduced with permission from: Kurman RJ, Carcangiu ML, Herrington S, Young RH. World Health Organization Classiﬁcation of Tumours of the Female
Reproductive Organs. IARC, Lyon, 2014. Copyright © 2014.
Ovarian tumor markers in adolescents [1-3]
Serum tumor markers
AFP beta-hCG* CA-125 Estradiol Inhibin A and B LDH Testosterone
Ovarian tumors in which tumor markers may be present
Malignant ovarian tumors
Adult granulosa ✓
cell tumors
Choriocarcinoma ✓
Dysgerminoma ✓ ✓ ✓
(rare)
Embryonal ✓ ✓ ✓
carcinomas
Endodermal ✓ ✓
sinus tumors
Epithelial tumors ✓ ✓
(especially
serous)
Juvenile ✓
granulosa cell
tumors
Immature ✓ ✓ ✓ ✓
teratoma (rare) (rare)
Mixed germ cell ✓ ✓ ✓

tumors
Polyembryoma ✓ ✓
(rare)
Sertoli-Leydig ✓ ✓
cell tumors
Benign ovarian tumors
Thecoma ✓
Other conditions in which markers may be present
Hepatoblastoma Pregnancy Bone tumors Primary Hematologic

Hepatocellular Hydatidiform Breast cancer hypothyroidism malignancies
carcinoma moles Endometrial (Van Wyk and Abdominal
Liver disease Placental site malignancy Grumbach malignancies
tumors syndrome)
Down syndrome Endometriosis
Primary Endometrioma
hypothyroidism Pelvic
(Van Wyk and inflammatory
Grumbach disease
syndrome) Crohn disease
AFP: alpha-fetoprotein; beta-hCG: beta-human chorionic gonadotropin; CA-125: cancer antigen 125; LDH: lactate dehydrogenase.
References:
1. Mahajan P, Weldon CB, Frazier AL, Laufer MR. Gynecologic cancers in children and adolescents. In: Emans, Laufer, Goldstein's Pediatric and Adolescent
Gynecology, 7 th ed, Emans SJ, Laufer MR, Divasta AD (Eds), Wolters Kluwer, Philadelphia 2020. p.556.
2. van Heerden J, Tjalma WA. The multidisciplinary approach to ovarian tumours in children and adolescents. Eur J Obstet Gynecol Reprod Biol 2019; 243:103.
3. Kirkham YA, Kives S. Ovarian cysts in adolescents: Medical and surgical management. Adolesc Med State Art Rev 2012; 23:178.
Evaluation of ovarian masses in children and adolescents without acute severe abdominal pain
History and examination findings
Patient group Potential significance
All patients
Ovarian mass that is bilateral, solid, fixed, or irregular Associated with malignant tumors
Abdominal distension or ascites Associated with malignant tumors
Neonates and infants:
Cyst noted on antenatal ultrasonography Fetal/neonatal cysts usually resolve spontaneously by 6 months of
age
Prepubertal children
Increased height velocity Onset of puberty (associated with increased incidence of physiologic
cysts); rarely may indicate hormone-producing tumors
Early puberty Ovarian tumor

Central or peripheral precocious puberty
Virilization Sertoli-Leydig cell tumor
Adolescents
Menstrual history May increase/decrease suspicion for:

Physiologic cysts
Endometrioma
Congenital anomaly of the vagina or uterus
Sexual history May increase/decrease suspicion for:

Pregnancy-associated cysts
Tubo-ovarian abscess (associated with STI)
Imaging for all patients
Imaging modality Findings associated with malignant tumors
Transabdominal ultrasonography 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
Doppler flow Increased blood flow (compared with minimal or no blood flow)
Laboratory testing for select patient groups
Patient group Laboratory tests
Postmenarchal adolescents Urine beta-hCG
Signs or symptoms of STI Testing for STI
Increased suspicion for ovarian tumor (eg, based on ultrasonography Panel of ovarian tumor markers (AFP, beta-hCG, LDH, inhibin A and B,
or associated symptoms) CA-125)
Increased suspicion for hormonally active tumor Estradiol

Testosterone
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Patients with ascites Cytology of ascitic fluid (if fluid is obtained)
Ovarian mass with torsion Platelet count (thrombocytosis is a nonspecific marker of ovarian
malignancy)
STI: sexually transmitted infection; beta-hCG: beta-human chorionic gonadotropin; AFP: alpha-fetoprotein; LDH: lactate dehydrogenase; CA-125: cancer
antigen 125.
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Evaluation of ovarian masses in infants, children, and adolescents - UpToDate 15-07-21 00:13

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● Palpable abdominal or pelvic mass.

● 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],

Anti-N-methyl-D-aspartate receptor encephalitis is an autoimmune and paraneoplastic encephalitis

CAUSES OF OVARIAN MASS

● Endometrioma (rare in adolescents but does occur)

Other causes of pelvic mass include [6,33]:

EVALUATION OF OVARIAN MASSES

Patients without acute severe abdominal pain

● For all patients:

● For prepubertal children:

• Menstrual history, including milestones of pubertal development, last menstrual period,

• Sexual history to evaluate risk of pregnancy-associated cyst or tubo-ovarian abscess

On ultrasonography, ovarian masses are characterized as:

Additional information can be obtained with CT or MRI.

• Invasive or metastatic compared with well-circumscribed

• Increased blood flow (compared with minimal or no blood flow)

● Postmenarchal adolescents – Urine beta-human chorionic gonadotropin test (beta-hCG) to exclude

● Signs or symptoms of STIs – Testing for STIs.

• A panel of tumor markers (alpha-fetoprotein, beta-hCG, lactate dehydrogenase, inhibin A, inhibin B,

• Cytology of ascites fluid (if obtained).

● Suspected ovarian torsion (emergency referral)

● Symptomatic ruptured/hemorrhagic cyst (urgent referral)

● Persistent simple cyst

● Uncertain origin of the mass (eg, ovary versus paraovarian)

● Increase in size or failure to resolve on serial imaging

SOCIETY GUIDELINE LINKS

SUMMARY AND RECOMMENDATIONS

• Suspected ovarian torsion (emergency referral)

• Symptomatic ruptured/hemorrhagic cyst (emergency/urgent referral)

• 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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Topic 127034 Version 3.0

Select nonneoplastic causes of ovarian mass in children and adolescents [1,2]

Origin Associated clinical features Ultrasonographic features

Follicular cyst Common in neonates and Simple (clear fluid filled), or

Corpus luteal cyst Pelvic pain Complex (internal echoes)

Paraovarian/paratubal cysts (eg, Often asymptomatic Simple cysts

Tubo-ovarian abscess Usually a complication of PID; may result Complex

beta-hCG: beta-human chorionic gonadotropin; PID: pelvic inflammatory disease.

Graphic 128370 Version 2.0

Select neoplastic causes of ovarian mass in children and adolescents [1-3]

Type of tumor Associated clinical features Ultrasonographic appearance

Benign germ cell tumors

Gonadoblastoma Typically associated with gonadal Solid mass with calcification

Benign epithelial tumors

Benign sex cord-stromal tumors

Thecoma-fibroma <2% of ovarian tumors in children and Solid hypoechoic mass

Malignant germ cell tumors

Immature teratoma Approximately 10% of complex ovarian Complex or completely solid

Malignant sex cord-stromal tumors

NMDAR: anti-N-methyl-D-aspartate receptor.

Graphic 128371 Version 2.0

WHO classification of tumors of the ovary*

Clear cell tumors

Low-grade endometrioid stromal sarcoma