Tumor disease and associated congenital abnormalities on prenatal MRI

European Journal of Radiology 81 (2012) e115–e122

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Tumor disease and associated congenital abnormalities on prenatal MRI Stefan F. Nemec a,b,∗ , Ernst Horcher c , Gregor Kasprian a , Peter C. Brugger d , Dieter Bettelheim e , Gabriele Amann f , Ursula Nemec a , Siegfried Rotmensch g , David L. Rimoin b , John M. Graham Jr. b , Daniela Prayer a a

Department of Radiology, Division of Neuroradiology and Musculoskeletal Radiology, Medical University Vienna, Waehringer Guertel 18-20, A-1090 Vienna, Austria Medical Genetics Institute, Cedars Sinai Medical Center, 8700 Beverly Boulevard, PACT Suite 400, Los Angeles, CA 90048, USA c Department of Pediatric Surgery, Medical University Vienna, Waehringer Guertel 18-20, A-1090 Vienna, Austria d Center of Anatomy and Cell Biology, Integrative Morphology Group, Medical University Vienna, Waehringerstrasse 13, A-1090 Vienna, Austria e Department of Obstetrics and Gynaecology, Division of Prenatal Diagnosis and Therapy, Medical University Vienna, Waehringer Guertel 18-20, A-1090 Vienna, Austria f Institute of Pathology, Medical University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna, Austria g Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Cedars Sinai Medical Center, 8635 West Third Street, Los Angeles, CA 90048, USA b

a r t i c l e

i n f o

Article history: Received 22 October 2010 Received in revised form 19 December 2010 Accepted 28 December 2010 Keywords: Prenatal MRI Fetal tumors Teratomas Tumor-related complications Congenital abnormalities

a b s t r a c t Objective: Fetal tumors can have a devastating effect on the fetus, and may occur in association with congenital malformations. In view of the increasing role of fetal magnetic resonance imaging (MRI) as an adjunct to prenatal ultrasonography (US), we sought to demonstrate the visualization of fetal tumors, with regard to congenital abnormalities, on MRI. Materials and methods: This retrospective study included 18 fetuses with tumors depicted on fetal MRI after suspicious US findings. An MRI standard protocol was used to diagnose tumors judged as benign or malignant. All organ systems were assessed for tumor-related complications and other congenital malformations. Available US results and histopathology were compared with MRI. Results: There were 13/18 (72.2%) benign and 5/18 (27.8%) malignant tumors diagnosed: a cerebral primitive neuroectodermal tumor in 1/18, head–neck teratomas in 4/18; ventricular rhabdomyomas in 4/18; a cardiac teratoma in 1/18; a hepatoblastoma in 1/18; neuroblastomas in 2/18; a cystic hemorrhagic adrenal hyperplasia in 1/18; a pelvic leiomyoma in 1/18; sacrococcygeal teratomas in 3/18. Tumor-related complications were present in 13/18 (72.2%) cases; other congenital abnormalities in 3/18 (16.7%). MRI diagnosis and histology were concordant in 8/11 (72.7%) cases. In 6/12 (50%) cases, US and MRI diagnoses were concordant, and, in 6/12 (50%) cases, additional MRI findings changed the US diagnosis. Conclusion: Our MRI results demonstrate the visualization of fetal tumors, with frequently encountered tumor-related complications, and other exceptional congenital abnormalities, which may provide important information for perinatal management. Compared to prenatal US, MRI may add important findings in certain cases. © 2011 Published by Elsevier Ireland Ltd.

1. Introduction Congenital tumors are defined as those that are present during the fetal stage or at birth [1]. The reported prevalence for all congenital tumors ranges from 1.7 to 13.5 per 100,000 live births [2–4]. Apart from various vascular anomalies, the most common fetal neoplasms are extracranial teratomas, neuroblastomas, soft-tissue tumors, brain tumors, and leukemia [3,5]. Collectively, these tumors constitute approximately 85% of all fetal tumors, with

∗ Corresponding author at: Department of Radiology, Division of Neuroradiology and Musculoskeletal Radiology, Medical University Vienna, Waehringer Guertel 1820, A-1090 Vienna, Austria. Tel.: +43 1404004895; fax: +43 1404004864. E-mail address: [email protected] (S.F. Nemec). 0720-048X/$ – see front matter © 2011 Published by Elsevier Ireland Ltd. doi:10.1016/j.ejrad.2010.12.095

renal tumors, liver tumors, and retinoblastomas constituting the majority of the remainder [2,4]. Fetal tumors, although rare, create significant medical problems. Since teratogenesis and oncogenesis may have shared mechanisms [6,7], neonatal neoplasms may be associated with other congenital anomalies and syndromes that will influence the fetal outcome [8,9]. The degree of cytodifferentiation, the metabolic or immunological state of the embryo or fetus, and the length of exposure to injurious agents will determine whether the effect is teratogenic, oncogenic, both, or neither [6,7]. Prenatal diagnosis has significant implications for the wellbeing of both mother and fetus, as well as for perinatal and neonatal outcome [9–11]. Over the past ten years, magnetic resonance imaging (MRI) has been increasingly used as an addition to ultrasonography (US) in the evaluation of abnormalities during intra-uterine life [12–14]. Moreover, recent publications have cited

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MRI as useful for the visualization and diagnosis of fetal tumors [1,15,16]. The current study aims to confirm the visualization of fetal tumors on MRI, in association with congenital malformations and other abnormalities, compared to prenatal US. The imaging protocols are described in detail and the potential impact of a prenatal MRI diagnosis is discussed.

2. Materials and methods The protocol for this retrospective study was approved by our Institutional Review Board (EC-822-2010) and the procedure was performed in accordance with the Declaration of Helsinki. 2.1. Patients After chart review, this retrospective series included fetal MRI studies of 18 fetuses [21 + 4 to 39 + 4 gestational weeks (29 + 4 GW); 17/18 singleton and 1/18 twin pregnancies] with tumors depicted on fetal MRI between July 2002 and August 2009. (Cases with vascular anomalies were not included, since these lesions do not represent true neoplasms.) Initially, MRI was performed based upon clinical indications after pathologic findings on prenatal US to confirm or possibly expand the diagnosis. Written, informed consent was obtained from all mothers (maternal age, 18–41 years; mean, 27.4 years). Fetal karyotyping showed normal 46, XX in two cases, normal 46, XY in one case, and normal 46, XX chromosomes with a pericentric inversion of chromosome 9 (normal variant) in one case. 2.2. Imaging 2.2.1. US In our hospital, a prenatal two-dimensional US scan by the transabdominal route was performed by an obstetric specialist (15 years experience) on a GE Voluson 730 Expert (GE Medical Systems, Germany) or a Toshiba Xario (SSA-680A) ultrasound machine (Toshiba Medical Systems, Austria) with a 3.75-MHz curved array transducer. The US-scans were archived as digital image files. The US examinations were performed up to four days prior to MRI. 2.2.2. MRI Non-contrast-enhanced MRI was performed on a 1.5 T unit (Philips Medical Systems, Best, The Netherlands) using a fiveelement, phased-array cardiac coil. There was no sedation of either mother or fetus. Our fetal MRI standard protocol included the following sequences to image the fetal head: 1. Axial, coronal, and sagittal T2-weighted (w) single-shot (ssh) turbo-spin-echo (TSE) sequences (repetition time (TR): shortest; echo time (TE): 140 ms; TSE factor: 92; field-of-view (FOV): 200–230 mm; matrix: 256 × 153; slice thickness: 3 mm; slices: 18; gap: 0.4; flip angle: 90◦ ; duration: 18.7 s). 2. An axial T1-w sequence (TR: shortest; TE: 4.6 ms; FOV: 325 mm; matrix: 208 × 166; slice thickness: 5 mm; gap: 0.5; slices: 15; flip angle: 80◦ ; duration: 14 s). 3. A coronal single-shot fast field echo (SSh FFE) sequence (=echoplanar imaging/EPI) (TR: 3000 ms; TE: shortest; FOV: 230 mm; matrix: 160 × 95; slice thickness: 4 mm; gap: 0; slices: 19; flip angle: 90◦ ; duration: 12 s). 4. Axial and coronal diffusion-weighted imaging (DWI) (TR: shortest; TE: 125 ms; FOV: 250 mm; matrix: 128 × 81; slice thickness: 5 mm; gap: 0.1; slices: 16; number of acquisitions: 1; flip angle: 90◦ ; duration: 20 s).

Our fetal MRI standard protocol included the following sequences to image the fetal body: 1. Axial, coronal, and sagittal T2-weighted (w) single-shot (ssh) turbo-spin-echo (TSE) sequences (repetition time (TR): shortest; echo time (TE): 100 ms; TSE factor: 92; field-of-view (FOV): 200–230 mm; matrix: 256 × 153; slice thickness: 3 mm; slices: 18; gap: 0.4; flip angle: 90◦ , duration: 18.7 s). 2. Axial and coronal balanced gradient echo (GE) sequence (TR: shortest; TE: 3.8 ms; FOV: 300 mm; matrix: 256 × 256; slice thickness: 5 mm; gap: 0.5; slices: 14; flip angle: 21◦ ; duration: 14 s). 3. A coronal T1-w sequence (TR: shortest; TE: 4.6 ms; FOV: 325 mm; matrix: 208 × 166; slice thickness: 5 mm; gap: 0.5; slices: 15; flip angle: 80◦ ; duration: 14 s). 4. Coronal and sagittal SSh FFE sequences (=echoplanar imaging/EPI). (TR: 3000 ms; TE: shortest; FOV: 230 mm; matrix: 160 × 95; slice thickness: 4 mm; gap 0; slices: 19; flip angle: 90◦ ; duration: 12 s). 5. Coronal DWI (TR: shortest; TE: 125 ms; FOV: 250 mm; matrix: 128 × 81; slice thickness: 5 mm; gap: 0.1; slices: 16; number of acquisitions: 1; flip angle: 90◦ ; duration: 20 s). 6. A 3D thick-slab T2-w sequence (TR: 8000 ms; TE: 400–800 ms; FOV: 210–320 mm; matrix: 256 × 205; slice thickness: 30–50 mm; gap: 0; flip angle: 90◦ ; duration: 8 s). 2.3. Evaluation The images were reviewed by two fetal MRI specialists (ten years experience) in consensus, who were aware of abnormal US findings. Fetal tumors were classified according to their site of origin, i.e., head and brain, face and neck, thorax, heart, abdomen and retroperitoneum, and sacrococcygeal region [9]. The imaging of fetal tumors was focused on tumor-specific signs, including tumor location, size, extension, growth progression, composition (MRI signal intensities), vascularity and intra-lesional hemorrhage, invasiveness, tumor compression and obstruction, and possible metastatic spread. Tumor patterns were judged as benign or malignant in each case. Furthermore, the whole fetus was assessed for malformations or anomalies that were not directly or indirectly attributable to the tumorous disease. The amount of amniotic fluid was also assessed, based on the maximum vertical pocket depth (MVP) published for prenatal US [17], and the appearance of the placenta [18] was evaluated. In a subset of 12 cases, the outcome of the pregnancy, and histopathological results (11 patients)/postnatal imaging (1 patient) were identified from medical records, the latter serving as the standard of reference, and were available for comparison with prenatal imaging findings. Furthermore, delivered pregnancies were differentiated from terminated pregnancies. In 12 cases, prenatal US results were also compared with fetal MRI based upon chart review. The other six cases were included, but without knowledge of outcome and histopathological data because of different referring departments from national hospitals. 3. Results 3.1. Imaging findings Based on MRI, the tentative diagnosis of 13/18 (72.2%) benign and 5/18 (27.8%) malignant tumors was made. A brain tumor was found in 1/18 fetuses, with a supratentorial primitive neuroectodermal tumor (PNET) (Fig. 1) (Case 1). Head–neck tumors were

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Table 1 Fetuses with MRI diagnosis, outcome, and histopathology. Case

GW

MRI

Neoplasms on MRI (tentative)

Other findings

Outcome

Histopathologya

1

33 + 2 34 + 2

Malignant

None

Caesarean section

(S) PNET

2

21 + 4 29 + 2

Benign

None

Caesarean section

3

29 + 2

Malignant

None

Caesarean section

(S) Immature teratoma grade III with endodermal sinus tumor (B) Immature teratoma

4

30 + 2

Benign

None

5

28 + 0

Benign

None

Caesarean section, death one day later of ARDS Caesarean section

6

34 + 0

Temporal hemorrhagic PNET with sinus thrombosis and hydrocephalus Facio-pharyngeal teratoma with local displacement and infiltration Facio-pharyngeal teratoma with local displacement and infiltration Cervical teratoma with mediastinal and tracheal compression, polyhydramnion Teratoma of the right atrium with pericardial effusion and lung compression Rhabdomyoma of the left ventricle with enlarged heart

Benign

Cortical tubers, subependymal nodules, lateral ventricular enlargement

Pregnancy terminated

7

33 + 6

Rhabdomyoma of the left ventricle

Benign

Cortical tubers, focal cortical dysplasia, subependymal nodules

Caesarean section

8

35 + 5

Malignant

None

Caesarean section

9

39 + 4

Malignant

None

Vaginal birth

(S) Neuroblastoma

10

22 + 6 30 + 6 24 + 6 32 + 4 24 + 2 28 + 0

Hepatoblastoma with hepatomegaly Neuroblastoma with organ displacement Cystic hemorragic adrenal lesion with organ displacement Sacrococcygeal teratoma with intrapelvic component Sacrococcygeal teratoma with intrapelvic component

(A) Cervical immature teratoma grade I, tracheal aplasia (S) Immature teratoma grade III with endodermal sinus tumor (A) Cardiac rhabdomyoma, cortical tubers and subependymal nodules in tuberous sclerosis None (postnatal imaging confirmed cardiac/brain manifestation in tuberous sclerosis) (S) Hepatoblastoma

Benign

None

Caesarean section

Benign

None

Caesarean section

(S) Cystic-hemorrhagic adrenal cortical hyperplasia (S) Mature teratoma

Benign

None

Vaginal birth

(S) Mature teratoma

11 12

Note: Other findings refer to anomalies or malformations that are not directly or indirectly caused by the tumorous lesions (PNET – primitive neuroectodermal tumor). a Histopathological results were obtained from (A) autopsy, or (B) biopsy, or (S) surgical resection.

found in 4/18 fetuses, with two facio-pharyngeal teratomas (epignathus) and two cervical teratomas (Fig. 2) (Cases 2–4, and 14). Thoracic tumors were found in 5/18 fetuses, with four ventricular rhabdomyoma (Fig. 3) and one cardiac teratoma (Cases 5–7, 15, and 18). Abdominal tumors were shown in 1/18 fetuses, with one hepatoblastoma (Fig. 4) (Case 8). Retroperitoneal tumors were found in 3/18 fetuses, with two neuroblastoma and one cystic hemorragic adrenal lesion (Fig. 5) (Cases 9, 10, and 16). A pelvic tumor was demonstrated in 1/18 fetuses, with one leiomyoma of the Douglas space (Case 17). Sacrococcygeal tumors were present in 3/18 fetuses, with three sacrococcygeal teratomas (Fig. 6) (Cases 11–13). Refer also to Tables 1 and 2 for detailed information on MRI findings and diagnosis.

plasms did not produce other sequelae. Refer also to Tables 1 and 2 for tumor-related findings. Metastatic disease was not present in any case. 3.3. Other abnormalities Other congenital anomalies not directly attributable to the fetal tumors were seen in 3/18 (16.7%) cases with rhabdomyomas, all three with a diagnosis of tuberous sclerosis. There were typical cortical tubers in one case, cortical tubers and subependymal nodules in a second case (Fig. 3), and cortical tubers and focal cortical dysplasia and subependymal nodules in a third case (Fig. 7). 3.4. Amniotic fluid and placenta

3.2. Tumor-related findings and complications Abnormalities caused by tumor growth were present in 13/18 (72.2%) cases (Figs. 1–6). In two cases with saccrococcygeal teratomas, and in three cases with cardiac rhabdomyomas, the neo-

The amount of amniotic fluid was normal in 16/18 cases, and polyhydramnios was found in 2/18 cases (one case with cervical teratoma, and one case with sacrococcygeal teratoma). The placenta was normal according to age in all cases.

Table 2 Fetuses with MRI diagnosis and unknown outcome. Case

GW

MRI

Neoplasms

Other findings

13

23 + 0

Benign

None

14 15 16 17

37 + 2 32 + 0 32 + 2 26 + 3

Benign Benign Malignant Benign

None None None None

18

22 + 0

Sacrococcygeal teratoma with intrapelvic component, polyhydramnions Cervical teratoma with local displacement Rhabdomyoma of the left ventricle Neuroblastoma with local displacement Leiomyoma of the Douglas space with local displacement Rhabdomyoma of the left ventricle

Benign

Cortical tubers in tuberous sclerosis

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Fig. 1. Fetus at 34 + 2 GW with supratentorial PNET (Case 1). (A) The axial T2-w image of the brain shows a temporal inhomogeneous hemorrhagic lesion (arrow) and bilaterally enlarged lateral ventricles. (B) Two months after birth, the postoperative contrast-enhanced T1-w image shows a large temporal parenchymal defect, with no pathological contrast enhancement suspicious for residual tumorous tissue.

3.5. Outcome of pregnancy

Fig. 2. Fetus at 29 + 2 GW with pharyngeal teratoma (epignathus) (Case 3). (A) The sagittal T2-w image shows a slightly hyperintense oropharyngeal mass (arrow), which occupies the mouth of the fetus, with local displacement of structures. (B) Six months after birth, and after chemotherapy, the sagittal T2-w image shows a progressive expansion, with sella invasion and intracranial tumor growth (arrow), compared to prenatal imaging.

There were 9/12 cases delivered by Caesarean section, 2/12 cases delivered by vaginal birth, and 1/12 pregnancy was terminated.

patient. Refer also to Table 1 for detailed information on histopathological results.

3.6. Prenatal MRI vs. histopathology/postnatal imaging

3.7. MRI vs. US findings

In 11 cases with available histopathology, specimens were obtained from postnatal surgery in 8/11 cases, from biopsy in 1/11 cases, and from autopsy in 2/11 cases. Histopathology and MRI diagnosis were concordant in 8/11 cases with regard to the differentiation between benign and malignant neoplasms. Histology revealed additional foci of a malignant endodermal sinus tumor in one case of facio-pharyngeal teratoma, and in a second case of cardiac teratoma; in a third case, a malignant teratoma was suspected on MRI, whereas histology did not confirm malignancy. Furthermore, postnatal imaging confirmed the prenatal diagnosis of tuberous sclerosis, with cardiac and brain involvement, in one

In 6/12 (50%) cases, US and MRI results were concordant in diagnosing the fetal tumors. In 6/12 (50%) cases with pathologic findings on US, additional MRI findings were made that changed or clarified the suspected US diagnosis. Refer also Table 3 for detailed information on additional MRI findings. 4. Discussion In accordance with recent publications, our results confirm the ability to visualize tumors on prenatal MRI [1,15,16]. Based on the types of tumors visualized, teratomas (8/18 cases) were the

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Fig. 4. Fetus at 35 + 5 GW with hepatoblastoma without metastases (Case 8). (A) The coronal T2-w image of the fetal body shows a large hyperintense tumor (arrow) of the right enlarged hepatic lobe with central necrosis. (B) The intra-operative image after mobilization of the liver shows a spherical lesion with external protrusion on the caudal hepatic surface.

Fig. 3. Fetus at 34 + 0 GW with CNS and cardiac involvement in tuberous sclerosis (Case 6). (A) The coronal T2-w image of the thorax shows a hypointense lesion of the left ventricle indicative of rhabdomyoma (arrow) with cardiac enlargement. (B) The axial T2-w image of the brain demonstrates a parietal abnormal hypointense gyration, indicating cortical tubers (arrow).

most common fetal tumor overall [2]. Since most fetal tumors are detected by obstetric US during the second and third trimesters, the value of MRI in prenatal tumor diagnosis could be questioned. US is helpful in determining the location of the tumor, its content, the effects on surrounding structures, and the presence of heart failure and hydrops; US also allows the detection of associated malformations [9,10]. Furthermore, color Doppler US is valuable in the visualization of tumor vessels and in determining the vascular volume of a lesion. However, in some cases, US may be technically unable to establish the exact tumor extension and site of origin, and complementary examinations are necessary. High soft-tissue contrast, high resolution, and the capability to visualize the whole fetus, even in late stages of pregnancy, have been cited as advantages of MRI compared to ultrasound [1,16,19]. Thus, fetal MRI has shown the potential to provide additional information in the further evaluation of tumors detected by US. Fetal MRI, using multiplanar imaging capabilities, may be able to better define the exact extent of the tumor and detect signs of infiltration, and/or additional complications caused by the tumor. In sacrococcygeal teratomas, MRI may help to determine the extent of an intrapelvic component [16]. In addition to T2-w sequences,

Table 3 Fetuses with additional MRI findings. Case

US

MRI

1

33 + 4 Suspected hemorrhage; ddx: tumor with internal hydrocephalus

5

28 + 0 Rhabdomyoma, atrial right, with enlarged heart and pericardial effusion 33 + 6 Rhabdomyoma, ventricular

34 + 2 Hemorrhagic PNET with sinus thrombosis and internal hydrocephalus 28 + 0 Teratoma, atrial right, with enlarged heart and pericardial effusion 34 + 0 Rhabdomyoma, ventricular; cortical tubers, subependymal nodules, lateral ventricular enlargement (tuberous sclerosis) 33 + 6 Rhabdomyoma, ventricular; cortical tubers, focal cortical dysplasia, subependymal nodules (tuberous sclerosis) 35 + 5 Hepatoblastoma without other metastasis

6

7

33 + 5 Rhabdomyoma, ventricular

8

35 + 4 Vascularizied lesion of the mid abdomen; ddx: neuroblastoma; ddx: subdiaphragmatic sequester 22 + 1 Multicystic dysplastic kidney

9

22 + 6 Cystic, hemorrhagic adrenal lesion with organ displacement

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Fig. 5. Fetus at 30 + 6 GW with cystic hemorrhagic adrenal lesion (Case 10). (A) The axial T2-w image of the upper abdomen shows a large retroperitoneal multi-cystic lesion (ellipsis), with different signal intensities, but which is slightly hyperintense due to intra-lesional hemorrhage. (B) One day after Caesarean section, the T2-w image of the abdomen features an extensive left-sided tumor with displacement of the diaphragm and kidney (arrow). Prenatal imaging, and postnatal clinical and genetic assessment, ruled out Beckwith–Wiedemann syndrome.

the repertoire of MRI sequences, including T1-w images and echogradient sequences, can help to demonstrate and differentiate hemorrhage and tumorous lesions, as some tumors may also show a hemorrhagic component. In this regard, a rapidly growing cerebral mass, suspected to represent hemorrhage on US, was correctly identified as brain tumor (PNET) with secondary sinus thrombosis on MRI. The thick-slab T2-w images facilitate the assessment of the whole fetus, and even extensive abnormalities such as large tumors may be captured in one image. Our results emphasize that fetal MR imaging will provide additional information in selected cases. MRI correctly diagnosed a suspected mid-abdominal tumor as hepatoblastoma, as well as a suspected multicystic kidney as an adrenal tumor, due to enhanced anatomical assessment. Furthermore, MRI may depict other findings, in particular, CNS abnormalities that may potentially influence the fetal outcome and prenatal management. In contrast to US, MRI visualized CNS anomalies in fetuses with cardiac rhabdomyoma that strongly indicated the brain manifestation of tuberous sclerosis, causing the parents to terminate the pregnancy in one case. Some authors have stated the need to perform MRI of the CNS to identify hamartomas or subcortical tubers and/or focal cortical dysplasia, as there is a significant association of cardiac rhabdomyomas with tuberous sclerosis in more than 60% of cases, which will worsen or change the prognosis and prenatal management [20].

Fig. 6. Two different fetuses at 24 + 2 GW and 23 GW with sacrococcygeal teratomas (Cases 12 and 13). (A) (Cases 12) The sagittal T2-w image shows a mainly cystic (hyperintense) tumor with external protrusion (arrow) with a small cystic intrapelvic pre-sacral component (arrowhead). (B) (Cases 13) The thick-slab 3D T2w image features a semi-solid tumor with enormous dimensions, compared to the fetal body size, and with polyhydramnios.

For other congenital malformations in fetuses with tumors, the literature suggests associated anomalies in up to 20% of cases [15,21]. However, there is inconsistent use of terminology, in which tumor complications and separate anomalies are grouped together. A recent investigation of 566 children with cancer demonstrated that only 12.7% of children had either a congenital malformation or a birthmark [22]. Kamil et al. reviewed 84 fetuses with tumors, and reported three cases with aneuploidies and another six cases with

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drome. In our study, the one individual with a cystic hemorrhagic adrenal lesion did not show any other imaging findings suspicious for Beckwith–Wiedemann syndrome, and this condition was ruled out after delivery by genetic analysis. Because of the rarity of fetal tumors, the majority of reports in the literature, as well as our series, concern experience with only few cases. Our study, including preliminary results, was limited in being able to compare US with MRI, and US remains the dominant prenatal imaging technique. Prospective MRI data will be required to determine the long-term outcome of prenatally diagnosed tumors. Furthermore, the differentiation between benign and malignant tumors in utero may be difficult, because histologically benign tumors may show local compression and invasion, such as head neck teratomas. In conclusion, our results demonstrate the ability to visualize fetal tumors on MRI. By imaging frequently encountered tumorrelated complications and other rare congenital abnormalities, MRI may provide important information for perinatal management. Furthermore, additional MRI findings may specify or change prenatal US diagnosis in certain cases. Fig. 7. A 3-year-old child with a prenatal diagnosis of cardiac rhabdomyoma and brain involvement in tuberous sclerosis (Case 7). The postnatal axial MR image of the brain (fluid attenuation inversion recovery [FLAIR]) shows subependymal nodules (arrow; right) and a focal cortical dysplasia (ellipsis; left frontal).

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additional abnormalities, including minor abnormalities, such as a single umbilical artery [10]. In contrast to frequently found tumorrelated complications in 72.2% of cases, our results demonstrated other congenital abnormalities in 16.7% of cases, which is within the reported frequency. The fact that these latter anomalies occurred in cases with tuberous sclerosis, emphasizes again the value of MRI in this condition. Although our study could not assess long-term outcomes, the prognosis of fetal tumors in the most cases will be related to the location and size of the mass, the histological type, the growth behavior, the surgical resectability, and the condition of the neonate at birth [23,24]. Thus, morbidity and mortality will be caused by the sequelae of the tumor, including compression of vital structures, hydrops fetalis, or polyhydramnios due to swallowing obstruction (e.g., cervical teratoma) or because of increased fluid production (e.g., sacrococcygeal teratoma). The presence of a fetal tumor may require intra-uterine therapy and may alter the mode of delivery. After amniotic reduction, one fetus with a cervical teratoma underwent an ex utero intrapartum treatment (EXIT) procedure with immediate tracheotomy. The overall survival in several postnatal series varies from 75% to 87% [9,10]. Tumors of the central nervous system have a uniformly poor prognosis, with a low survival rate of only 28% [4]. Some fetal tumors, even benign neoplasms, may prove fatal because of their size or location and may interfere with normal organ development. Therefore, detailed prenatal imaging should be performed to detect tumor-associated complications. Although tumors are rarely encountered in association with genetic disease or malformation syndromes on US or MRI, prenatal imagers should be aware of a potential association of certain neoplasms and other congenital malformations and abnormalities [25]. The genetic literature provides various reports about different syndromes with an increased rate of neoplasia occurring during fetal life or early childhood. Wilms tumor has been reported in association with over 50 different clinical conditions and several abnormal constitutional karyotypes [26]. Certain fetal overgrowth syndromes, such as Beckwith–Wiedemann, Costello, or Perlman, are associated with developmental delay, tumors, and other anomalies [27–29]. Overall, the detection of a tumor in utero should stimulate the evaluation of the whole fetus to detect other abnormalities that might suggest a complication or associated syn-

Acknowledgment

The authors have indicated they have no financial relationships relevant to this article to disclose. No conflict of interest.

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