HOW TO APPROACHABDOMINO-PELVIC « TUMORS »

IN THE FETUS?Fred AVNI Tiphaine FOURQUET Pauline VERPILLAT

& Veronique DEBARGE Hôpital Jeanne de Flandre – CHRU – Lille (F)

Objectives

■ To be able to differentiate between normal and

abnormal structures of the fetal abdomen

■ To use a systematic approach to tumors and

pseudotumors of the fetal abdomen

■ To be aware of the potential tumors (and their

differential diagnosis) of the fetal abdomen

How to approach - How to analyze? Preliminary concepts

■ Three US examinations are usually performed during a pregnancy; they provide information about fetal anatomy, biometry and well being

■ These examinations might detect abnormalities➔eventually a (routine) obstetrical ultrasound has detected an abnormal abdomino-pelvic « image »

■ The patient is refered for further work-up and (pluridisciplinary) advice

How to approach – How to analyze?

1) « Expert » obstetrical ultrasound– Fetal anatomy and biometry + evaluation of the amniotic fluid

and placenta

– Detailed examination of the fetal abdomen checking, analyzingand if necessary, measuring the normal anatomic structures ■ Fluid-filled landmarks: Stomach, bladder, gallbladder, vascular axis

■ Solid-type landmarks: Liver, Spleen, kidneys, (adrenals), (pancreas)

■ Digestive tract: Filled or empty small and colonic bowel loops,

– Confirmation of a potentially « abnormal abdominal or pelvicimage »

How to approach – how to analyze an abnormal abdomino-pelvic image?

■ Location

■ Size

■ Analysis of its content

■ Döppler evaluation

■ Consequences on the fetus: Local compression, evidence for heart failure, fetal hydrops, polyhydramnios….

■ Associated malformations

How to approach – how to analyze?

■ Does the image correspond to a normal variant? Pseudomegabladder during the thirdtrimester in female fetuses, (small) ovarian follicles

■ Is the image definitelyabnormal?

– Does it correspond to a usualbut abnormal anatomicstructure ?

– Is it a tumor?

How to approach – how to analyze?

■ Does the image correspond to a

normal variant?

Pseudomegabladder of the third

trimester in female fetuses, small

ovarian folicles

■ Is the image definitely abnormal?

1. Does it correspond to a usual

but abnormal anatomic

structure ?

2. Is it a tumor?

How to approach – how to analyze?

1) The image is abnormal = Abnormal fetal anatomicstructure

– Organomegaly: liver, spleen kidneys, adrenals

– Uropathy + complications (urinoma)

– Digestive tract anomaly + complications

– Genital tract malformation (hydrocolpos)

How to approach – How to analyze?

2) The image is abnormal

– It is not a normal

structure

– There is a mass effect, it

is probably a tumor

Fetal tumors

● Congenital tumors = present in utero or at birth (up to 3 months).● Neonatal tumors have an incidence of 7,2/ 100 000 births

● Abdominal tumors are mainly cystic - exceptionally solid; they are mainlybenign - exceptionally malignant

● Most frequent locations: liver, adrenal, kidney, intraperitoneal

● Most frequent histologic types: lymphatic malformations (lymphangioma) and teratoma

Parkes et al. 1994, Neonatal tumours: a thirty-year population-based studyAvni et al. 2009, Tumors of the fetal body: a reviewIsaacs H 2018 Tumors of the fetus and newborn

How to approach – how to analyze?

1) « Expert » US examination ➔ (preliminary) conclusion

2) Fetal MR imaging if useful– Can provide additional information about the content, extension and

complications

– T2 W sequences for defining the anatomy; T1 W sequences to define the relationofthe tumor with the digestive tract and to evaluate potential complications (hemorrhage)

– Gradient echo and diffusion weighted sequences selectively useful

MRI 29WGLarge intrabdominal lymphatic malformation (Lymphangioma) with extraabdominal thigh extension

Pre-sacral cystic teratoma (36 WG)

How to approach – how to analyze?

1) Expert US examination and conclusion

2) Fetal MR imaging as useful– Additional information about the content, extension and complications

– T2 W sequences for defining the anatomy; T1 W sequence to define the relation with the digestive tract and to evaluate potential complications (Hemorrhage)

– Gradient echo sequences and diffusion weighted sequences as useful

3) Presumptive diagnosis and prognosis

4) Organization of the post natal management

How to approach – how to analyze a abdomino-pelvis tumor ?

Intraperitoneal-

Retroperitoneal-

Specific organ or

tract?

1) What organ

or what space is

involved?

2) Is it a tumor/

mass effect?

3) US and MRI

patterns

Yes/no

4) Presumptive

diagnosis?

IN PRACTICE

Case n°1:Mrs S…., Laure

■ Coupe transverse du pelvis

3d Trimester

Pelvic mass.

Quadruple cystic images

symetrical 2 by 2, behind

the bladder

MRI

MRIObstructed utero-vaginal duplication

No anomaly of the digestive tract

Case n°1Genital tract

1) What organ/

tract

2) Is it a tumor?

3) US and MRI

aspects

No, just mass

effect due to the

anomaly

4) Obstructed

vaginal and

uterine

duplication

DIFFERENTIAL DIAGNOSIS

■ Vesical duplication

■ Rectal duplication

■ Cloaque

DDx: Double fluid filled image in the sagittal plane - ascitis

BV

R

DDx: Neonatal

opacification ➔

Confirming the

diagnosis of cloaque

Back to Case n°1: Postnatal F-Up

■ Immediate postnatal US

confirming the obstructed

utero - vaginal duplication

US and MR imaging after hymeneal incision

CASE 2

CASE N°2 Mrs M…, Sabrina

■ Background: 32 year-old, G2P1, no previous history

■ Present pregnancy: Risk for T21 1/10 000,

■ During midtrimester US:

Heart not properly evaluated

Control examination at 27 weeks: Heart OK, but intrabdominalcytic mass with irregular margins (37x11 mm) in the right flank➔small bowel dilatation? Intestinal duplication?

● Referal US examination at 30 WG: Cystic polylobular mass appearantly within the right lobe of the liver 36x40mm, not vascularized. The GB was not seen

● Fetal MRI requested

FETAL MRI AT 30 WG

Confirms the intrahepatic location

hypersignal T2, hyposignal T1 (=fluid)

Gb demonstrated

Gb

Case n°2The liver1) What organ/

space

2) Is it a tumor?

3) US and MR

imaging aspect?

Yes

4) Complex liver cyst

= Cystic

mesenchymal

hamartoma?

DDx of cystic hepatic masses withpolylobulated margins

● (Hepatic cyst) – usually not lobulated

● Cystic mesenchymal hamartoma

● Ciliated hepatic cyst (primitive gut broncho-pulmonarymalformation)

● Choledochal cyst – in relation with the bile ducts

● Extra – hepatic cyst

DDx: Prenatal diagnosis of a cystic hepaticmass with polylobulated margins

Postnatal US with echogenic depositconsistant with a ciliated hepatic cyst

Guérin F & al J Ped Surg 2010; 45 E9-E14

■ 16 cases of simple or complex hepatic cysts refered for postnatal surgery(2006-2008)

– 8 simple hepatic cysts

– 4 Hamartoma

■ Developmental lesion that includes conjunctive tissue, biliary ducts and vessels

■ Mixed lesions cystic and/or solid

■ Most are operated but some may resolve spontaneously

■ Associated with Beckwith-Wiedeman Syndrome

– 4 Ciliated hepatic cysts (= primitive gut ciliated hepatic cyst)

■ Cystic lesion

■ Polylobulated and septated

■ Echogenic deposit and potential calcification

■ Hilum or left lobe

■ Difficult surgery

Finalizing case n°2

Uneventful birth of a boy weighing 3350g, Apgar score 10/10/10, pH 7.29

● US and MRI at birth

NEONATAL ULTRASOUND

The presumptive diagnosis seemed

confirmed. Conservative approach

Clinical and US/ MRI F-Up

At birth F-Up at age 6 mo

Almost complete spontaneous

resolution

What DDx in case of a solid type liver tumors?

DDx of solid (echogenic) hepatic masses (3H)

● Hemangioma

● Hamartoma

● Hepatoblastoma

● Metastasis (Neuroblastoma)

● (Extra-hepatic tumors)

CASE 3

CASE n°3: Mrs Pan…., Faustine

● Background: 34 year-old, G3P2● Midtrimester obstetrical US: Large for date fetus, no anomaly

● At 34 weeks:● Polyhydramnios● 6 cm diameter abdominal mass

● Transfer to JdF hospital for amniodrainage, US and MRI

35+6 GW: Heterogeneous mass in the right flank,

cystic and solid (echogenic) parts 88 x 58 x 56 mm,

poorly vascularized (mainly in its periphery).

Originating from?

The upper part of the right kidney is visible (arrow)

MRI: The mass is well delineated and occupies

the lower 3/4 of the R kidney. The abdominal

organs (digestive tract) are displaced, not invaded

Case n°3The R kidney1) What organ

is involved

2) Is it a tumor?

3) US and MRI

aspects

Yes

4) Presumptive

Dx: Mesoblastic

nephroma

Differential Diagnosis

■ Renal tumors– Mesoblastic nephroma: most common congenital tumor, usually solid

– Wilms’ tumor, very rare, can be bilateral

– Cystic nephroma, exceptional

– Rhabdoid tumor

– (Multicystic dysplastic kidney)

■ Other retroperitoneal/ Perirenal tumors

– Neuroblastoma

– Retroperitoneal teratoma

DDx: Wilms’ tumor (bilateral)

Courtesy B Kline-Fath

M

DDx: Non renal masses

Obstetrical US

(35 WG)

Mass 25x35 mmB

Chest

K

Obstetrical US and MRI

Retroperitoneal abdominal tumor; solid type, vascularized, displacing

the kidney cephalad. The location and pattern suggest an extra adrenal

neuroblastoma

123I-MIBG scintigraphy (after birth)

Pluridisciplinaryadvice case n°3

● Proposed Dx= mesoblastic

nephroma

● Consultation with pediatric

surgeon

● US 1X/ week

● Delivery at a tertiary care

pediatric hospital (JdF)

Case n°3: Lukas

- Emergency C – section at 37 WG

due to fetal arrythmia

- 3160gr, Apgar 8/10/10, pH 7.10

- Rapidly developing HT

● Neonatal work-up:

Xray, US and CT

Echographie H2Neonatal abdominal US

Liver Bladder

Contrast CT scanner

Very large heterogeneous retroperitoneal mass

developping at the lower pole of the right kidney (the

UP is compressed but functions)

Cystic nephroma vs mesoblastic nephroma?

Case n°3: Lukas

● Surgery at D2, enlarged right nephrectomy➔ 10x6x3cm and

1440gr renal mass invading the renal hilum and some vessels

● Histology: Morphologic caracterstics suggesting a cellular type of

mesoblastic nephroma or a clear cell sarcoma. Case sent for

second opinion to CHU de Lyon, in accordance with the SIOP

protocole. Final diagnosis: mesoblastic nephroma, cellular type,

stage III

● Cytogenetic analysis positive for anti WT1 antibody

● Baby received additional 9 weeks chemotherapy➔ uneventful F-Up

Mesoblastic nephromaContribution of magnetic resonanceimaging to prenatal differentialdiagnosis of renal tumors: report of two cases and review of the literature.

Linam LE & al Fetal Diagn Ther. 2010;28(2):100-8. doi: 10.1159/000313655.

CASE 4

Case n°4: Mrs S…, Magali

● Background: 24 year-old, G2P1

● Ontgoing pregnancy: Risk for T21 = 1/5023, midtrimester obstetrical US

normal

● Discovery of a right cystic suprarenal mass during the 3d trimester US

examination

OBSTETRICAL US(37 WG)

39x34x33mm right suprarenal cystic mass, some

echogenic content and calcification. No evidence

for hemorrhage or fetal anemia.

Fetal MRI

Case n°4The R supra-renal

area

1) What organ/

space is

involved

2) Is it a tumor?

3) US and MRI

aspects

Yes

4) Adrenal

hemorrhage vs

cystic

neuroblastoma

Differential Diagnosis

■ Adrenal tumors/masses– Adrenal cyst – usually small

– Adrenal hemorrhage - can be associated with renal vein thrombosis

– (Cystic) neuroblastoma – can be hemorrhagic

– Dysplastic cortical cysts (B-W) – multiple, can be hemorrhagic

– Lymphangioma/Teratoma - mixed echogenicity, septated

■ Other retroperitoneal tumors– Non adrenal neuroblastoma

– Retroperitoneal teratoma

– Renal tumors

– Sequestration

DDx: Adrenal hematoma/ hemorrhage

Left K HH

DDx: Sub diaphragmatic sequestration

Back to case n°4: Loubna

■ Considered as an

adrenal HH

■ Birth at term

■ Clinically OK

■ Post natal US and MR

imaging

Postnatal US

46 x 39 X 50 mm

Post natal MRI

Case n°4: Pluridisciplinary discussion

■ Negative urinary cathecolamines

■ Probable adrenal hemorrhage

■ Clinical and US F-Up

→ mass stable during 2 months

Then,

→ Increasing size starting the third month (51 X 41 X 46 mm)

→ 123I-MIBG and urinary cathecholamines became +/ increasing

Neuron-Specific Enolase (NSE)

Louna,….

■ Surgery : Right surrenalectomy

■ Histology:

– Poorly differentiated cystic neuroblastoma,

– Schwannian stroma-poor neuroblastoma

– Mitosis-Karyorrhexis Index (MKI) 2%

➔Good prognosis

■ Uneventful F-UP

Neonatal neuroblastoma(AJR 2016: 207: 1105-1111)

■ 50% of the neonatal NB are cystic

■ Thanks to antenatal diagnosis, Dx is earlier and surgery more rapid

■ Cystic NB tends to be stage I, tumoral markers low/lower than tissular

NB

■ Rarely metastasizes, better survival

Conclusions

■ How to approach – How to analyze?

– Optimal use of the different techniques

– Acquiring knowledge and experience on the

pathology occuring in utero

– Interdisciplinary discussions

– Obtaining and participating to the F-Up