Prenatal Sonographic Findings of Congenital Adrenal Cortical Adenoma
David M. Sherer, MD, Mudar Dalloul, MD, Allison Wagreich, MD, Margarita Sokolovski, RDMS, Haiu Duan, MD, Harry Zinn, MD, Ovadia Abulafia, MD
T he differential diagnosis of prenatally diagnosed adrenal masses includes neuroblastoma, adrenal hemorrhage, adrenal and cortical renal cysts, adrenal adenoma and carcinoma, subdiaphragmatic pulmonary sequestra- tion, Beckwith-Wiedemann syndrome, duplication of the renal system, Wilms tumors, congenital mesoblastic nephroma, and mesenteric and enteric duplication cysts.1-5 The worldwide annual incidence of childhood adrenal corti- cal neoplasms ranges between 0.3 and 0.38 per 1 million children younger than 15 years.6 These neoplasms are even more unusual among infants, with only 23 cases reported in the literature.6
We present the first report of prenatal sonographic findings of a fetus with adrenal cortical adenoma.
Received February 11, 2008, from the Divisions of Maternal-Fetal Medicine (D.M.S., M.D., M.S., H.D.) and Gynecologic Oncology (A.W., O.A.), Department of Obstetrics and Gynecology, and Department of Radiology (H.L.Z.), State University of New York, Downstate Medical Center, Brooklyn, New York USA. Manuscript accepted for publication February 14, 2008.
Address correspondence to David M. Sherer, MD, Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, State University of New York, Downstate Medical Center, 445 Lenox Rd, Box 24, Brooklyn, NY 11203-2098 USA.
E-mail: dmsherer@aol.com
Case Report
A 31-year-old primigravida was seen in our prenatal ultrasound unit at 39 weeks’ gestation 1 week after immi- grating to the United States. Her pregnancy had been uneventful, and findings from second-trimester sonogra- phy performed abroad were reported as normal.
Our initial sonographic evaluation depicted a singleton vertex-presenting fetus with appropriate-for-gestational- age biometric measurements. The sonographically esti- mated fetal weight was 3900 g. The amniotic fluid volume was normal, and the presence of a single nuchal cord was noted. Real-time sonography depicted a 3 x 3 x 3-cm heterogeneous mass compressing the right fetal kidney inferiorly (Figures 1 and 2). Multiplanar 3-dimensional sonography (iU22V6-2; Philips Medical Systems, Bothell, WA) clearly depicted the well-circumscribed round mass clearly containing solid and cystic components (Figure 3). The mass was clearly separate from the right renal parenchyma, with 3-dimensional imaging depicting a clear indentation surrounding the inferior aspect of the
adrenal mass separating this entity from the upper pole of the right fetal kidney (Figure 3). Power Doppler imaging depicted an indepen- dent arterial blood supply of the adrenal mass arising from the aorta. The mass was considered consistent with a tumor originating from the right adrenal gland. Given the concern for a potential malignant fetal tumor at 39 weeks’ ges- tation, induction of labor was performed.
3.32 cm
After normal labor with no evidence of preeclampsia or maternal hypertension, the patient spontaneously gave birth to a male neonate with a single nuchal cord, weighing 3910 g with Apgar scores of 9 and 9 at 1 and 5 minutes, respectively. Umbilical artery gas val- ues were as follows: pH, 7.21; PO2, 14.5 mm Hg; Pco2, 63 mm Hg; bicarbonate, 24.2 mEq/mL; and base excess, -5 mEq/L.
Physical examination disclosed a vigorous and alert neonate with normal vital signs. The neonate had peripheral left facial nerve palsy. No signs of virilization were present. Computed tomography of the abdomen on day 2 of life confirmed the presence of a solid adrenal tumor, which compressed the right kidney (Figure 4). Cranial computed tomographic and magnetic resonance imaging findings were normal. Test results for the serum complete blood count, blood urea nitrogen, creatinine, electrolytes, ferritin, 17-hydroxyprogesterone, and serum and urine catecholamines (epin- ephrine, norepinephrine, metanephrine, and normetanephrine) were normal. The serum testosterone level was 258 ng/ml (normal, 270- 1070 ng/ml). Urine vanillylmandelic acid and homovanillic acid test results were negative.
Under general anesthesia on day 16 of life, the neonate underwent resection of the adrenal tumor by laparotomy and bone marrow aspira-
tion (for potential tumor staging). At the patho- logic assessment, the mass almost replaced the entire right adrenal gland, and only 0.6 × 0.6 × 0.6 cm of adrenal tissue was appreciated at the mar- gin of the mass.
Histopathology examination of the mass showed adrenal cortical adenoma with infarcts, hemorrhage, calcification, and multinucleat- ed giant cells. Some of the tumor cells showed moderate cytomegaly with no mitosis, capsu- lar invasion, or vascular invasion seen. Immuno- histochemical staining depicted tumor cells strongly positive for a-inhibin (adrenal cortical marker), negative for p53, and less than 5% posi- tive for Ki-67 (proliferation marker). Special stains for endothelium (CD31) and vascular elastic tis- sue (elastic Van Gieson) showed no evidence of a vascular malformation. The bone marrow aspirate yielded negative results for a tumor. The neonate subsequently did well and was dis- charged in good health on postoperative day 7.
Discussion
The prenatal sonographic findings of adrenal tumors vary according to the underlying lesion. Characteristic prenatal real-time sonographic
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findings of neuroblastoma are well documented and include a spectrum of cystic, solid, or com- plex (mixed) appearances.1,4,5 Small tumors are homogeneous and hyperechoic, whereas larger tumors are usually heterogeneous, at times with intraparenchymal calcification.1 Rarely reported prenatal sonographic findings of adrenal carcino- ma consist of bilateral large complex masses mimicking multicystic kidneys.4
The sonographic findings in our case, consisting of a semisolid, semicystic heterogeneous mass, appeared similar to those observed in association with neuroblastoma. In addition, in our case, color and power Doppler imaging depicted an arterial feeding vessel originating in the fetal aorta, similar to that reported in association with neuroblas- toma, thus enhancing sonographic differentiation of solid adrenal masses versus adrenal hemor- rhage (which lack such a feeding vessel).1
A systematic English language literature search (PubMed and MEDLINE) between 1966 and 2008 using the search terms “prenatal,” “ultrasound,” “adrenal tumor,” and “adrenal cortical adenoma” indicated that the prenatal sonographic findings of congenital adrenal cortical adenoma have not been reported previously. In addition, our case shows the contribution of 3-dimensional prena- tal sonography in the detailed depiction of con- genital adrenal cortical adenoma.
References
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