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They appear as pyramidal hypoechoic structures superior to the hyperechoic kidney. During the second trimester of pregnancy, corticomedullary differentiation can be observed with a hypoechoic cortex and hyperechoic medulla. The size of the gland increases throughout gestation but remains smaller than the kidney. During the third trimester of pregnancy, the appearance of the fetal adrenal glands is similar to that of the neonatal adrenal glands.
Neuroblastomas originate in the neural crest cells of the sympathetic nervous system. Although most cases arise from the adrenal gland, they can also occur in the posterior mediastinum.
The term neuroblastoma refers to a spectrum of neuroblastic tumors (eg, neuroblastomas, ganglioneuroblastomas, and ganglioneuromas) that arise from sympathetic ganglion cells.
Adrenal neuroblastoma generally appears as a well-encapsulated cystic or solid mass adjacent to but separate from the kidney and other retroperitoneal structures (Figure).
Assessment of the contralateral adrenal gland to rule out normal but prominent adrenal glands is recommended. Findings on color Doppler interrogation are variably reported and include peripheral flow, no flow, or internal vascularization of the hyperechogenic aspects of the mass.
Prenatal diagnosis of fetal adrenal masses: differentiation between hemorrhage and solid tumor by color Doppler sonography.
Ultrasound in Obstetrics and Gynecology: The Official Journal of the International Society of Ultrasound in Obstetrics and Gynecology.1999; 13: 351-355
Prenatal ultrasound characterization of the suprarenal mass: distinction between neuroblastoma and subdiaphragmatic extralobar pulmonary sequestration.
Postnatally, neuroblastoma has been associated with Li-Fraumeni syndrome, Hirschsprung disease, and neurofibromatosis type 1, but these associations have not been reported in prenatal cases. If a neuroblastoma markedly enlarges and compresses the gastrointestinal tract, polyhydramnios may develop.
Fetal neuroblastoma is typically a sporadic finding. If no further abnormalities are noted on ultrasound and the family history is unremarkable, no genetic evaluation beyond standard aneuploidy screening is typically recommended.
Pregnancy and Delivery Management
Hydrops fetalis, polyhydramnios, or both may develop in rare circumstances, typically with large lesions or in the setting of metastatic disease; therefore, serial ultrasound examinations should be performed. Serial ultrasound assessment can help rule out adrenal hemorrhage, which can evolve over time.
A consultation with pediatric oncology, neonatology, and surgery should be obtained to plan and coordinate prenatal and postnatal management. In general, pregnancy termination is an option that should be offered to patients when a major fetal anomaly is detected. However, in most cases of neuroblastoma, the prognosis is favorable, and the outcome is good. Shared patient decision-making requires a thorough evaluation and multidisciplinary counseling regarding prognosis. If pregnancy termination is pursued, histologic evaluation can confirm the diagnosis.
In most cases, vaginal delivery is appropriate, and cesarean delivery should be reserved for the usual obstetrical indications. Cesarean delivery has been suggested as potentially preferable for very large cystic adrenal masses to prevent rupture or soft tissue dystocia. Adrenal cyst aspiration before delivery is controversial because this may cause bleeding, malignancy seeding, preterm labor, or infection. Delivery in a tertiary care center is recommended with consideration of early delivery if there is evidence of fetal compromise. Postnatal investigation, including ultrasound, magnetic resonance imaging, or other imaging modalities, is recommended. Expectant management, needle biopsy, or surgical exploration may be needed on the basis of the results of the above imaging, final diagnosis, and neonatal condition.
Prognosis
In general, the prognosis is good, and neuroblastomas may even resolve in utero or shortly after birth.
The recurrence risk of this lesion is unknown but likely low.
Summary
Fetal adrenal neuroblastomas, derived from neural crest cells, are the most frequently diagnosed extracranial solid tumor of childhood. These rare tumors are associated with an excellent postnatal prognosis. Close surveillance with serial ultrasound examinations evaluating for signs of hydrops fetalis and polyhydramnios is recommended. Antenatal consultation with pediatric oncology, neonatology, and surgery is advised.
References
Norton M.E.
Callen's Ultrasonography in Obstetrics and Gynecology E-Book.
Prenatal diagnosis of fetal adrenal masses: differentiation between hemorrhage and solid tumor by color Doppler sonography.
Ultrasound in Obstetrics and Gynecology: The Official Journal of the International Society of Ultrasound in Obstetrics and Gynecology.1999; 13: 351-355
Prenatal ultrasound characterization of the suprarenal mass: distinction between neuroblastoma and subdiaphragmatic extralobar pulmonary sequestration.
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