10
Diagnostic and Interventional Imaging (2015) 96, 273—282 ORIGINAL ARTICLE / Genito-urinary imaging Ovarian tumors in children and adolescents: A series of 41 cases E. Péroux a , S. Franchi-Abella a , D. Sainte-Croix b , S. Canale c , F. Gauthier d,e , H. Martelli d,e , D. Pariente a , C. Adamsbaum a,,e a Service de radiologie pédiatrique, hôpital Bicêtre, AP—HP, 78, rue du Général-Leclerc, 94275 Le Kremlin-Bicêtre, France b Inserm, Santé publique et épidémiologie, hôpital Bicêtre, AP—HP, 78, rue du Général-Leclerc, 94275 Le Kremlin-Bicêtre, France c Service d’imagerie médicale, Institut Gustave-Roussy, 39, rue Camille-Desmoulins, 94805 Villejuif, France d Service de chirurgie pédiatrique, hôpital Bicêtre, AP—HP, 78, rue du Général-Leclerc, 94275 Le Kremlin-Bicêtre, France e Faculté de médecine Paris Sud, Paris, France KEYWORDS Ovary; Tumor; Imaging; Children; Adolescents Abstract Objective: Pictorial review with a detailed semiological analysis of ovarian tumors in children and adolescents to provide a relevant diagnostic approach. Patients and methods: Retrospective study (2001—2011) of 41 patients under the age of 15 who underwent surgery for an ovarian mass with a definite pathological diagnosis. Results: Sixty-two percent of the lesions were benign, 33% were malignant and 5% were border- line. Germ cell tumors were most frequent (77.5%), followed by sex cord stromal tumors (12.5%) and epithelial tumors (7.5%). Malignant tumors were more frequent in children between 0 and 2 years old. On imaging, calcifications and fat were specific for germ cell tumors; the presence of a mural nodule was predictive of a mature teratoma (P < 0.001). Predictive factors for malignancy were clinical, including abdominal distension (P < 0.01) or a palpable mass (P = 0.05), biological, including increased hCG and/or AFP levels (P < 0.001) and radiological, including tumors larger than 12 cm (P < 0.05), tumoral hypervascularity (P < 0.01) and voluminous ascites (P < 0.01). Abbreviations: MRI, Magnetic resonance imaging; CT, Computed tomography; MGT, Malignant germ cell tumor; AFP, Alpha-fetoprotein; hCG, Human chorionic godanotropin; Se, Sensitivity; Sp, Specificity; PPV, Positive predictive value; NPV, Negative predictive value. Corresponding author. E-mail address: [email protected] (C. Adamsbaum). http://dx.doi.org/10.1016/j.diii.2014.07.001 2211-5684/© 2014 Éditions franc ¸aises de radiologie. Published by Elsevier Masson SAS. All rights reserved.

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Diagnostic and Interventional Imaging (2015) 96, 273—282

ORIGINAL ARTICLE / Genito-urinary imaging

Ovarian tumors in children and adolescents:A series of 41 cases

E. Pérouxa, S. Franchi-Abellaa, D. Sainte-Croixb,S. Canalec, F. Gauthierd,e, H. Martelli d,e,D. Parientea, C. Adamsbauma,∗,e

a Service de radiologie pédiatrique, hôpital Bicêtre, AP—HP, 78, rue du Général-Leclerc,94275 Le Kremlin-Bicêtre, Franceb Inserm, Santé publique et épidémiologie, hôpital Bicêtre, AP—HP,78, rue du Général-Leclerc, 94275 Le Kremlin-Bicêtre, Francec Service d’imagerie médicale, Institut Gustave-Roussy, 39, rue Camille-Desmoulins,94805 Villejuif, Franced Service de chirurgie pédiatrique, hôpital Bicêtre, AP—HP, 78, rue du Général-Leclerc,94275 Le Kremlin-Bicêtre, Francee Faculté de médecine Paris Sud, Paris, France

KEYWORDSOvary;Tumor;Imaging;Children;Adolescents

AbstractObjective: Pictorial review with a detailed semiological analysis of ovarian tumors in childrenand adolescents to provide a relevant diagnostic approach.Patients and methods: Retrospective study (2001—2011) of 41 patients under the age of 15 whounderwent surgery for an ovarian mass with a definite pathological diagnosis.Results: Sixty-two percent of the lesions were benign, 33% were malignant and 5% were border-line. Germ cell tumors were most frequent (77.5%), followed by sex cord stromal tumors (12.5%)and epithelial tumors (7.5%). Malignant tumors were more frequent in children between 0 and 2

years old. On imaging, calcifications and fat were specific for germ cell tumors; the presence of amural nodule was predictive of a mature teratoma (P < 0.001). Predictive factors for malignancywere clinical, including abdominal distension (P < 0.01) or a palpable mass (P = 0.05), biological, including increased hCG and/or AFP levels (P < 0.001) and radiological, including tumors largerthan 12 cm (P < 0.05), tumoral hypervascularity (P < 0.01) and voluminous ascites (P < 0.01).

Abbreviations: MRI, Magnetic resonance imaging; CT, Computed tomography; MGT, Malignant germ cell tumor; AFP, Alpha-fetoprotein;hCG, Human chorionic godanotropin; Se, Sensitivity; Sp, Specificity; PPV, Positive predictive value; NPV, Negative predictive value.

∗ Corresponding author.E-mail address: [email protected] (C. Adamsbaum).

http://dx.doi.org/10.1016/j.diii.2014.07.0012211-5684/© 2014 Éditions francaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.

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274 E. Péroux et al.

Conclusion: This semiological analysis confirms the role of imaging in diagnosing the etiology ofovarian lesions in children and adolescents and emphasizes the importance identifying tumoralhypervascularity, which, in addition to classic criteria, is highly predictive of malignancy.© 2014 Éditions francaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.

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he diagnosis of ovarian tumors in pediatrics is often difficultnd delayed because of non-specific symptoms and diverseresentations on imaging.

These rare tumors occur in an estimated 2.6/100,000 girlser year, excluding functional lesions [1,2]. Ten to 20% arealignant [3—6] and they represent 3% of cancers in girls

nder the age of 15 [7]. The World Health Organization haslassified these tumors into three main groups according tohether they develop from epithelial cells, germ cells or

ex cord stromal tumors. Primary or secondary locations areossible in leukemias or lymphomas [8,9].

Nevertheless, understanding of ovarian tumors in this ageroup is limited and the goal of this study was to analyze theiagnostic value of imaging features and suggest a diagnosticpproach to ovarian masses in children or adolescents.

atients and methods

atients

his retrospective study was performed from April 2001 toeptember 2011 based on pediatric surgical archives at theicêtre Hospital. Inclusion criteria were:patients under the age of 15 at diagnosis;a definite pathological diagnosis for each lesion;initial pre-treatment imaging available for analysis (ultra-sound, CT/or MRI).

ata collection

ata were obtained from the medical files of patients in theospital’s digital filing system. The following informationas obtained: age at diagnosis, stage of puberty (pubertyefined as the presence of a menstrual cycle), medical his-ory, symptoms, adnexal torsion or tumor rupture (surgicalata), serum levels of AFP, total hCG, free �-hCG, CA 125,nhibin B and the presence or not of an inflammatory syn-rome as well as a pathological diagnosis. Lesions werelassified as benign, borderline or malignant.

Imaging results were all reassessed with an evaluationuide (EP). Imaging criteria included:

the diameter of the mass at its widest axis;the cystic features of the lesion (multicompartmentalcysts were considered to be pure cysts), solid (no cys-tic component) or mixed and the proportion of differentcomponents (more or less 50%);

the presence of tumoral calcifications, intratumoral fat,compartments, blood, papillary vegetations (millimetric)or mural nodules (centimetric);whether the lesion was vascularized or not;

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associated abnormal endometrial thickening (in pre-pubescent children suggesting an abnormally pubescentuterus, or more than 15 mm thick in post-pubescentpatients);the presence of ascites (considered to be voluminous ifnot limited to the pelvis).topographic criteria included: the presence of single, mul-tiple or bilateral lesions, and any extra-ovarian extension(peritoneum, lymph nodes).

Three age groups were defined: 0—2 years old, older than and not pubescent and pubescent.

ata analysis

he Chi2 test was used to compare qualitative variables,ith Yates correction for groups of fewer than 5. The Mannhitney test was used to compare quantitative variables

nd a ROC curve was obtained. P ≤ 0.05 was considered toe significant.

esults

orty-one patients were included in the study. None of theatients were excluded.

Patient characteristics are summarized in Table 1 and theistribution of different types of lesions is summarized inable 2.

Thirty-nine patients had a single lesion, one patient had mature bilateral teratoma, and one patient had multi-le bilateral sclerosing stromal tumors (0.5—5 cm); only theargest tumor was considered for analysis. Thus, 42 lesionsere studied.

The patient with sclerosing stromal tumors had a his-ory of metastatic medulloblastoma with suspected Gorlinyndrome (hereditary disease characterized by a group ofevelopmental anomalies and a predisposition to developifferent tumors).

Fig. 1 shows the distribution of the different types andlasses of lesions in relation to age and stage of puberty.

Abdominal distension and the presence of a palpa-le mass were significantly associated with malignancyTable 3). Fifteen patients (36.5%) presented with the clini-al signs of a surgical emergency with nausea and vomiting;he presence of vomiting was significantly associated withvary torsion (P < 0.001). The five cases with fever corre-ponded to three benign tumors associated with adnexal

orsion and partial or total necrosis of the ovary and twoalignant tumors with signs of necrosis on histology. One

umor was discovered by chance during an ultrasound for annguinal hernia. One patient with hemorrhagic shock had a

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Ovarian tumors in children and adolescents: A series of 41 cases

Table 1 Patient characteristics.

n (%) Mean Median

Patients 41Tumors 42Age 10.3 12

Under 2 years old 2 (5)Over 2 years old, notpubescent

20 (49)

Pubescent under 15years old

19 (46)

Revealing symptomsAbdominal pain 32 (78)Palpable mass 23 (56)Distended abdomen 16 (39)Nausea/Vomiting 15 (36.5)Fever 5 (12)Asthenia 4 (10)Early puberty 3 (7)Post-pubescentmetrorrhagia

1 (2.5)

Virilization 1 (2.5)Hemorrhagic shock 1 (2.5)Chance discovery 1 (2.5)

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Table 2 Distribution of different ovarian tumors.

Lesions Benign n Borderline

TumorsGerm celltumors

Mature teratomas 22

Sex cord andstromal tumors

Sclerosingstromal tumors

1

Epithelialtumors

Mucinouscystadenomas

1 Mucinous cystadenofibromMucinousintestinal—tytumors

Other tumors

Non-tumorallesions

Hemorrhagic cyst 1

Pseudotumoralhemorrhagicinfarction

1

n total (%) 26 (62)

275

uptured immature teratoma. Early puberty was a compli-ation in malignant tumors (a juvenile granulosa cell tumornd the only unclassifiable mixed tumor); a mature teratomaas discovered by chance because of an indication for ultra-

ound. One case of virilization with no signs of puberty wasound in one of the patients with sclerosing stromal tumors.ost-pubescent metrorrhagia revealed a sex cord tumor withnnular tubules.

iology

reoperative serum levels of AFP and hCG were availablen all patients. AFP levels were increased in all malignanterm cell yolk sac-type tumors (or presenting with a com-onent of this type of tumor) and in immature teratomas.n increase in hCG levels was observed in the seminoma andn immature teratoma. An increase in these markers (AFPnd/or hCG) suggested a malignant germ cell tumor (MGT)P < 0.001) with a sensitivity of 78%, a specificity of 100%, aPV of 100% and a NPV of 94%.

Inhibin levels were determined in two patients; it wasormal in the juvenile granulosa cell tumor and elevatedn the unclassifiable mixed germ cell and sex cord stromal

umor.

CA 125 serum levels were determined in 10 patients. Theyere increased in the two immature teratomas, two juvenileranulosa cell tumors, the case of hemorrhagic infarction

n Malignant n Tumors n (%)

Seminoma 1 31 (77.5)

Yolk sac 2Immatureteratomas

4

Mixed teratomas 2Juvenilegranulosa celltumors

3 5 (12.5)

Sex cord tumorswith annulartubules

1

-as

1 3 (7.5)

pe1

Mixedunclassifiable sexcord and stromaltumors

1 1 (2.5)

2 (5) 14 (33)

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276 E. Péroux et al.

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igure 1. Ovarian tumors, age and pubertal status.

f the ovary and in the borderline mucinous intestinal-typeumor.

maging

nitial imaging included twenty-seven abdominal plain filmsAPF) 40 ultrasounds, 27 CT scans and 12 MRI. Fouratients underwent surgery based on sufficient ultrasoundesults (without CT scan or MRI). Twenty-six of the 27 CT

cans were performed immediately following injection ofn iodated contrast medium (n = 6) or before and afternjection (n = 20). All MRI (n = 12) examinations includedat-suppressed T1- and T2- weighted sequences and an

(mbl

Table 3 Predictive factors of malignancy.

Criteria Benign lesions Borderline ormalignant lesion

Palpable massYes 11/25 12/16

Distended abdomenYes 5/25 11/16

Enhancement and/or vascularization on doppler USYes 6/21 12/15

Data not available 6Tumor components

Predominantly cystic 19 7

Predominantly solid 7 9Voluminous ascites

Yes 0/25 5/16

Size of the lesion≥ 12cm 8 11

< 12 18 5

Se: Sensitivity; Sp: Specificity; PPV: Positive predictive value; NPV: Neg

xamination performed on at least two spatial planes.leven MRI were performed before and after gadoliniumnjection with fat-suppressed T1-weighted sequences.

Ultrasound was the primary imaging technique in 95% ofases (n = 39/41) and always identified a mass. APF iden-ified calcifications with a sensitivity of 76%, a specificityf 100%, a positive predictive value (PPV) of 100% and aegative predictive value (NPV) of 71%.

The mean size (largest axis) of benign lesions was 113 mm

20—317, median 90 mm), while that of borderline andalignant lesions was 162 mm (47—270, 170). In multipleilateral sclerosing stromal tumors, only the size of theargest lesion was taken into account to determine the

sP Se (%) Sp (%) PPV (%) NPV (%)

0.05 75 56 52 78

< 0.01 69 80 69 80

< 0.01 80 71 67 83

0.06 56 73 56 73

< 0.01 31 100 100 70

0.04 69 69 58 78

ative predictive value.

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Ovarian tumors in children and adolescents: A series of 41 c

different parameters. The threshold of 121 mm provided abetter discrimination between malignant forms (or border-line) and benign forms (P = 0.04) (Table 3).

Distribution of the cystic and solid features of the lesionswas not predictive of histological types (P = 0.06) (Table 3,Figs. 2 and 3). The presentation of juvenile granulosa celltumors (n = 3) varied (one cystic, one solid, one mixed) butepithelial tumors (n = 3) were always predominantly cystic.

Compartments were found in 17/31 germ cell tumors(55%), two of the five sex cord stromal tumors (40%), allthe epithelial tumors, hemmorhagic infarction of the ovaryand hemorrhagic cysts. No intracystic papillary vegetationswere found.

A mural nodule protruding from a cystic lumen wasonly found in germ cell tumors: 77% of mature teratomas(n = 17/22) and two MGT (Fig. 4). This was highly predictiveof a mature teratoma (P < 0.001) with a specificity of 90%, asensitivity of 77%, a PPV of 89% and a NPV of 78%.

Tumoral enhancement and/or vascularization on Dopplerultrasound were significantly associated with malignancy(P < 0.01) (Table 3, Fig. 5).

Intratumoral fat was only detected on CT scan (densitybelow —20 UH and usually near —100 UH) or MRI (hypersig-nal on T1- and T2- weighted sequences that disappearedafter fat suppression) in the group of germ cell tumors:88% of the mature teratomas evaluated on CT scan or MRI(n = 15/17) as well as immature teratomas (Table 4, Figs. 4and 6).

Intratumoral calcifications were only detected on ultra-

sound, CT scan or APF in the group of germ cell tumors:73% (n = 16/22) of mature teratomas, 67% (n = 6/9) ofMGT (Table 4, Fig. 6). Calcifications could correspond to

dc

Figure 2. Sclerosing stromal tumors. Premenarchal 12-year-old girl wof virilization (acne, low voice and significant weight gain). Presence of eaxial MRI T2-weighted images (WI); b: axial T1-WI; c and d: axial T1-WI

treatment (bilateral annexectomy).

277

ssifications, teeth or calcified material. Teeth were foundn the macroscopic examination of seven mature teratomasnd a tooth bud in an immature teratoma; these were diffi-ult to identify on imaging.

Intratumoral blood was detected in three patients on MRInd presented as a hypersignal on T1-weighted sequenceshat disappeared after fat suppression. These included aemorrhagic cyst, hemorrhagic infarction of the ovary and

juvenile granulosa cell tumor (Fig. 3).Voluminous ascites was found in 5 malignant tumors,

ncluding four ruptures; it was significantly associated withalignancy (P < 0.01) (Table 3).Abnormal endometrial thickening was found in two of the

hree cases of granulosa cell tumors with, in one case, thessociation of a prematurely pubescent uterus (Fig. 7). Theppearance of the uterus was normal in all other types ofumors.

The results of assessment of tumor extension showedetroperitoneal lymph node extension in a seminoma. Nother metastases were identified.

urgical results

n 12 cases associated torsion was identified during surgeryhat had not been detected on preoperative imaging: 75%9/12) were associated with a mature teratoma, 8.5% (1/12)ith a malignant tumor and 16.5% (2/12) with a non-tumoral

esion.In five cases a spontaneous tumor rupture was discovered

uring surgery: four malignant tumors and the hemorrhagicyst.

ith a history of metastatic medulloblastoma presenting with signsnhancement of masses following gadolinium injection (arrows). a:

with fat suppression after gadolinium injection. Two stage surgical

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278 E. Péroux et al.

Figure 3. Juvenile granulosa cell tumor. Postmenarchal 14-year-old girl presenting with abdominal pain. Increased CA 125. Normal inhibin,AFP and hCG values. Cystic and tissue mass that enhanced after gadolinium injection with hematic sediment indicating hemorrhage (arrows),associated with voluminous ascites. a: ultrasound (US); b: axial MRI T1-WI; c: axial T1-WI with fat suppression; d: axial T2-WI; e: coronal T1-WI with fat supression after gadolinium injection. Surgical treatment (right annexectomy and omentectomy) associated with chemotherapybecause of spontaneous tumor rupture.

Figure 4. Mature teratoma. Premenarchal 12-year-old girl presenting with abdominal pain. a: US: diagnosis suspected in the presenceof a cystic mass with echogenic mural nodule, confirmed by MRI; b: coronal T2WI; c: axial T1WI; d: coronal T1WI with fat suppressionbefore gadolinium injection and e: after injection: presence of fat in the Rokitansky protuberance (black arrows). Linear central noduleenhancement following gadolinium injection (white arrow). Conservative treatment with tumorectomy.

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Ovarian tumors in children and adolescents: A series of 41 cases 279

Figure 5. Three examples of malignant ovarian tumors. Seminoma in a premenarchal 10-year-old girl presenting with abdominal pain,a palpable mass, fever and increased HCG levels: lobulated mass with hypervascularity on Doppler US and calcifications (arrow). a: US;b: axial CT (computed tomography) after contrast injection. Yolk sac tumor in a premenarchal 11-year-old girl presenting with abdominalpain, a palpable mass, fever and increased AFP levels. c: axial CT after contrast injection: huge mass with tortuous vascular structuresinside (arrow). Granulosa germ cell tumor in a 6-month-old infant presenting with precocious puberty (breast and labial enlargement,endometrial hemorrhage) and abdominal distension. d: axial CT after contrast injection: enhancing mass with extensive ascites. Sex cordtumor with annular tubules in a postmenarchal 12-year-old girl presenting with abdominal pain and intermenstrual bleeding: well-limited

fat su

olodb[ou

mass enhancing after injection. e: axial MRI T2WI; axial T1WI with

Discussion

Ovarian tumors in girls and adolescents represent a veryheterogenous group of histopathological entities. Largepediatric series (more than 40 cases) are rare with veryfew descriptions of imaging results [3,4,6,10]. Two olderseries were mainly based on ultrasound [6,10]. The stud-ies by Brookfield et al. [3] in 1037 malignant ovarian tumorsand by Oltmann et al. [4] in 424 ovarian masses mainly eval-

uated treatment. Several excellent imaging reviews haverecently been published, but without analysis of a series[5,9,11].

mlt

Table 4 Predictive factors of germ cell tumors.

Criteria Germ cell tumors Other lesio

FatYes 19/26 0/11

Data not available 5

CalcificationsYes 22/31 0/11

Se: Sensitivity; Sp: Specificity; PPV: Positive predictive value; NPV: Neg

ppression after gadolinium injection.

The frequency of different pediatric ovarian tumorsbserved in this series is similar to existing results in theiterature. Germ cell tumors represent more than 80% ofvarian tumors (77.5% in this series). Mature teratomas, orermoid cysts, are the most frequent tumors and the onlyenign germ cell tumor. The MGT are, in fact, often mixed5,8,12]. Sex cord and stromal tumors represent 5—10% ofvarian tumors (12.5% in this series). Benign tumors arenusual in this pediatric population and the most frequent

alignant tumors are granulosa cell tumors [5,13]. Epithe-

ial tumors (approximately 10% of ovarian tumors, 7.5% inhis series) are rare before puberty and usually benign [14].

ns P Se Sp PPV NPV

< 0,00173 100 100 61

< 0,001 71 100 100 55

ative predictive value.

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280 E. Péroux et al.

Figure 6. Teratomas. Mature teratoma in a 12-year-old postmenarchal girl presenting with abdominal pain and a palpable mass; a: axial CTwithout contrast injection: the lesion is mainly cystic with the presence of fat (white arrow) and calcifications; b: coronal CT after contrastinjection: no enhancement. Immature teratoma in a 8-year-old girl presenting with abdominal pain, abdominal distension and AFP, HCG andCA 125 levels. c: axial CT without contrast injection: mainly cystic mass with the presence of fat (white arrow) and calcifications (blacka partb

crp

b

Fmwl

rrow); axial CT after contrast injection: enhancement of the solidecause of incomplete macroscopic resection.

The prognosis of ovarian tumors in children and adoles-

ents is generally excellent as long as they are managedapidly, which emphasizes the importance of an early andrecise diagnosis. Conservative treatment is indicated in

eaa

igure 7. Juvenile granulosa cell tumor. Premenarchal 12-year-old girl

ass. Increased CA 125. a and b: axial MRI T1WI without gadolinium injecith enhancement of the walls and septa (white arrow); c: sagittal T2W

eft ovariectomy.

s of the tumor. Total annexectomy associated with chemotherapy

enign tumors, especially since there is a risk of contralat-

ral lesions, especially in mature teratomas. Treatment of

suspicious lesion is surgical (or after chemotherapy inn inoperable lesion) and preserving fertility is a major

presenting with abdominal pain, distended abdomen and a palpabletion and T1WI with fat sat suppression after injection: cystic lesionI: marked thickening of the endometrium. Surgical treatment with

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Ovarian tumors in children and adolescents: A series of 41 c

consideration in children. A diagnostic biopsy is indicatedin suspicious inoperable lesions with no increase in tumormarkers or in case of bilateral lesions if a hemopathy issuspected [7,8,14].

The frequency of malignant tumors varies in the litera-ture (9.5% and 21%) and is probably overestimated becauseof a surgical recruitment bias like in this study (33%) [4,6].The risk of malignant ovarian tumors in pediatrics is con-sidered to increase with age [3,6], except in one study inwhich the population with the highest risk was between 1and 8 years old [4]. On the contrary 100% of the tumors inour study were malignant in patients under 2 years old (n = 2)then the rate of malignancy decreased with age. This differ-ence is probably due to the purely ‘‘pediatric’’ populationin our series (100% of the patients were under 15 comparedto 38% in the study by Brookfield et al. [3]). Also, unlike inother series [3,4,6,10], the absence of malignant epithelialtumors in our study is probably due to a lower proportion ofadolescents.

Although the number of patients with each type of tumorwas limited because they are all rare entities, this pedi-atric series defines the diagnostic value of different imagingfeatures. This study confirms that the presence of fat is spe-cific for germ cell tumors and very frequent (88% of matureteratomas and 100% of immature teratomas) but does notdifferentiate between benign and malignant tumors. Calci-fications are also very specific for germ cell tumors (73%of mature teratomas and 67% of MGT) but calcificationshave occasionally been reported in other types of tumors[15] and this feature does not distinguish a benign froma malignant tumor. In particular a tooth bud was foundin an immature teratoma in this series. A mural nodule,corresponding to a Rokitansky protuberance appears to befairly specific for mature teratomas [16—20]. However, inour experience it may be difficult to distinguish from a por-tion of fleshy tumor tissue. The juvenile granulosa cell tumorcan have various, non-specific features, with necrotic andhemorrhagic aspects. [13,15]. It is therefore interesting toidentify other diagnostic features such as abnormal thick-ening of the endometrium or signs of abnormal estrogenicimpregnation in a pre-pubescent child [13] (2/3 cases inthis series). The other sex cord stromal tumors are rareand there are no specific features. Epithelial tumors areoften cystic, as in this series. Papillary vegetations, pathog-nomonic for epithelial tumors, were not observed in thisstudy. [21].

Besides metastases, which provide a diagnostic sign atmuch too late a stage, this pediatric study suggests thatthere are predictive diagnostic imaging criteria for malig-nant ovarian tumors in children:• tumor enhancement (or vascularization on Doppler ultra-

sound) must be carefully looked for. Unlike in adults,dynamic imaging studies are not routinely performed inchildren [4,6];

• the size of a tumor with a threshold of 12 cm, which ismuch larger than the 8 cm indicated in the literature [4];

• voluminous ascites.

Clinically, the most frequent symptoms are pelvic pain,a palpable mass or abdominal distension. The clinical pic-ture may be a surgical emergency (nausea, vomiting) during

ptc

281

orsion or tumoral rupture. Adnexal torsion is the mainomplication of ovarian tumors, in particular mature ter-tomas (3.2—16%) [22] but is probably underestimatedecause none of the 12 adnexal torsions identified duringurgery in this series were identified during the preop-rative assessment. However, most adnexal torsions areot tumoral and this complication rarely reveals a malig-ant tumor (8.5% in this series and 1.8% in the literature23]). Sometimes an endocrine disorder (early puberty,ost-pubescent metrorrhagia, virilization) reveals sex cordtromal tumors [8,15,24]. Clinically predictive criteria foralignancy were abdominal distension and the presence of

palpable mass. Tumoral markers are essential for diag-osis, emphasizing the importance of testing before anyblation is performed. AFP, which is a sign of the presencef a yolk sac component and hCG, a sign of syncitio-rophoblast cells, are specific for teratomas. An increase inhese markers in immature teratomas indicates mixed ter-tomas in which the yoke sac and/or syncitio-trophoblastomponents were not identified on histology. Inhibin Bs specific for sex cord tumors. CA 125 is not specific7,25—27].

There are numerous differential diagnoses for ovarianumors. Functional pathologies may occur at any age andre the most frequent differential diagnosis [9,28,29]. Sim-le adnexal torsion may also occur at any age and presentss a mass. An ectopic pregnancy should always be looked forfter puberty and a tubovarian abscess can also resemble aumor, although there are often signs of infection in theseases [30].

In pediatrics most ovarian masses are detected withltrasound, which is the first-line test in these cases. Oncen organic disease has been identified, MRI, which haso radiation, provides good tissue enhancement and theest evaluation of tumor vascularization [9]. The proto-ol for adults in the literature includes two T2-weightedequences including an axial view, a T1-weighted axialiew with and without fat suppression and T1-weightedadolinium-enhanced sequences. A T-2 weighted abdomino-elvic sequence can be useful to study paraaortic lymphodes. [31—33]. This protocol is applicable in pediatrics.he use of CT scan should be limited to searching foralcifications that are not detected on ultrasound and tovaluate malignant invasion. [26]. APF is not useful in theseases.

onclusion

his retrospective pediatric series has identified the imag-ng features of ovarian tumors in children (Table 5).he features of benign or malignant germ cell tumorsere typical including fat and/or calcifications. Imagingriteria predictive of malignancy were tumoral hypervas-ularization, a tumor larger than 12 cm and/or voluminousscites. Ultrasound is the first-line test and MRI is essen-ial and should be performed according to the ‘‘adult’’rotocol with analysis of contrast enhancement, although

he value of dynamic imaging must be determined inhildren.
Page 10: Ovarian tumors in children and adolescents: A series of 41 ... › download › pdf › 82354733.pdf · Pictorial review with a detailed semiological analysis of ovarian tumors in

282 E. Péroux et al.

Table 5 Characteristics of the main ovarian tumors in children and adolescents according to the series in the literature.

Tumors Age Biology Imaging

Germinal cell tumors All ages Non-specific AFP/hCG (malignant) FatCalcifications

Sex cord and stromal tumors All ages Granulosa Inhibin B Not specificSertoli-Leydig

Epithelial tumors Post-pubescent Not specific ± CA 125 Cysts

D

Tc

A

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R

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T. Radiology 1991;181(2):481—8.

isclosure of interest

he authors declare that they have no conflicts of interestoncerning this article.

cknowledgements

e would like to thank those who provided help and adviceith this study: Prs Catherine Guettier and Laurence Meyer,rs Grégoire Boulouis, Béatrice Ducot, Guillemette Antonind Pierre Mutuon, as well as Mrs. Pascale Zerbini.

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