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Revista chilena de pediatría

versión impresa ISSN 0370-4106

Rev. chil. pediatr. vol.90 no.3 Santiago jun. 2019 


Differential diagnosis and minimally invasive surgery of an antenatal adrenal mass

Montserrat Lemus Marcenaro1 

José Manuel Campos Varas1 

José Fernando Vuletin Solís1 

Sergio González Bombardiere2 

Juan Carlos Pattillo Silva1 

1 Pediatric Surgery, Division of Surgery, Faculty of Medicine, Pontificia Universidad Católica de Chile, Chile.

2 Department of Pathology, Faculty of Medicine, Pontificia Universidad Católica de Chile, Chile.



Adrenal masses are uncommon in newborns. The differential diagnosis includes be nign masses (adrenal hemorrhage, extralobar pulmonary sequestration) and malignant ones (neuro blastoma) that may be a finding during an obstetric ultrasound. The use of complementary imaging methods allows a better diagnosis approach during the postnatal period, with implications for the management of these patients.


To report the case of a female newborn with diagnosis of an adrenal mass, and to discuss differential diagnoses and management alternatives of adrenal lesions in newborns.

Case report:

Two-month-old female infant, referred for adrenal tumor study diagnosed at 22 weeks gestational age. Postnatal ultrasound showed a tumor compatible with neuroblastoma. The patient was asymptomatic, and the laboratory studies showed no relevant findings. The lesion was excised by laparoscopy. A histological study confirmed pulmonary sequestration.


Extralobar pulmonary sequestration should be considered in the differential diagnosis of an adrenal mass in the newborn. Minimally invasive surgery should be the preferred surgical technique choice in these cases, given the technical feasibility and benefits in the recovery and cosmetic issues of the patient.

Keywords: neuroblastoma; adrenal tumor; extralobar pulmonary sequestration; children


The antenatal diagnosis of adrenal tumors diagno sis is increasing due to the widespread use of obstetric ultrasound1,2. Adrenal masses have an incidence of 1.9:1,000 newborns3. The most important differential diagnosis is with neuroblastoma (NB), a malignant neoplasm with originated from the adrenal gland in 90% of the cases3,4. (Table 1) details other differential diagnoses3-5. After birth, abdominal ultrasound should be performed. Additional information can be obtai ned with CT scan or MRI if malignancy is suspected3-5. Thus, it is possible to discriminate the lesion origin in 81-85% of cases3. Conservative management, with ul trasound monitoring and the use of serum markers, is a reasonable therapeutic approach, although this is still matter of debate6-9.

Table 1 Differential diagnoses of adrenal mass. 

The objective of this article is to report the case of a newborn with antenatal diagnosis of adrenal mass, who was successfully treated with minimally invasive surgery. The histopathology study reported an extra- lobar pulmonary sequestration (EPS). Differential diagnoses and possible management alternatives are discussed.

Clinical case

2-month-old female with adrenal mass detected by obstetric ultrasound at 22 weeks of pregnancy. A hypoechoic left adrenal mass of 22 x 16 mm was iden tified (Figure 1a). Mother with no morbid history, two previous births, well-controlled physiological pregnancy. Ultrasound follow-up at 35 weeks of gesta tional age confirmed the presence of a hypoechoic in- fradiaphragmatic mass of approximately 20 x 20 mm, apparently separated from the left adrenal gland (Figu re 1b). Vaginal delivery at 38 weeks, adequate weight for gestational age, no perinatal morbidity. Normal physical examination, no palpable abdominal masses, female genitals without signs of virilization or hyper pigmentation.

Figure 1 Antenatal ultrasound where the lesion is identified at different gestational ages. 1a. Ultrasound at 23 weeks of gestational age, shows a hypoechoic mass of 22 x 16 mm. 1b. Ultrasound at 35 weeks, infradiaphragmatic hypoechoic mass is identified in relation to the 20 x 20 mm kidney (white ellipse). 

At seven weeks, urinary catecholamines were mea sured, which were in the high normal range for age (metanephrine 206 |ig/g creatinine, and normetanephrine 1,250 |ig/g creatinine), and referral was decided for study and management.

The functional study of the tumor was completed with plasma levels of metanephrines, testosterone, aldosterone, renin, cortisol, androstenedione, epi nephrine, norepinephrine, and dopamine, all within normal limits.

The preoperative study was completed with abdo minal ultrasound that showed a solid, uniform lesion, echoic with respect to adjacent tissue, with partially defined edges, without evident calcifications, measu ring approximately 2.7 x 1.2 x 1.7 cm. No abnormal blood flow was observed in the Doppler ultrasound. Adjacent to the lesion there was a tissue resembling adrenal gland (Figure 2). The MRI complemented the study and showed a solid mass of 21 mm in the left adrenal region (Figure 3). There were no blood flow alterations.

Figure 2 Abdominal ultrasound at 7 weeks confirms an infradiaphragmatic echogenic well-defined mass. There is no image of vessels with Doppler US. 

Figure 3 Pre-operative MRI. Adrenal mass is market with (*). 

Tumor resection was performed laparoscopically without incident. Under general anesthesia and with the patient in a 45° left lateral position, a laparoscopic exploration with 5mm um bilical trocar inserted with open technique, an auxiliary trocar of 5mm in RIF, and two working instruments of 3mm inserted percutaneously. The colon was detached, releasing the splenic angle and accessing the transcavity. A well-defined adrenal mass adjacent to the gland was identified, of rubber-like consisten cy, with discreetly increased irrigation. Dissection and comple te resection of the lesion was performed which was extracted through the umbilical port (Figure 4). The patient was monitored for 24 hours, restarted breast feeding immediately after surgery, and was dischar ged in good condition at 48 hours. During follow-up at 2 and 6 weeks, she was in good condition, with no evidence of tumor recurrence on ultrasound or any other local complications.

Figure 4 Operative specimen. A well defined, lobulated mass, completely excised. 

The histopathological study showed a lung tissue lesion with canalicular stage structure, in which im mature bronchiolar structures with respiratory epithe lium, some alveolar conduits with macrophages, and no alveoli identification are recognized. Stromal fibro blast and smooth muscle fascicles. Some larger spaces with respiratory epithelium and immature hyaline car tilage. No evidence of malignancy was observed. These findings are compatible with pulmonary sequestration (Figure 5).

Figure 5 Microphotograph showing areas of alveolar and of bronchiolar differentiation with Microphotograph showing areas of alveolar and of bron- chiolar differentiation with thickening of alevolar septa and fibrosis around the abnormal airways. HE, 80x. 


The incidence of adrenal masses follows a bimodal distribution, and is more frequent in children under 5 years, and then from the fourth and fifth decade of life10. In newborns, it is important to establish the differential diagnosis between NB, adrenal hemorrhage (0.2% of newborns), and other lesions such as EPS, which are infrequent4. Pheochromocytoma and gan glioneuroma are tumors of school-age children, while adrenal adenoma, although rare in children, occurs be fore age 5 or after age 1011.

Most adrenal tumors occur as incidental finding before birth or during childhood. They can also pre sent as a palpable mass, abdominal pain, secondary ar terial hypertension, kidney failure, precocious puberty, and fever or shock, depending on their origin12.

The imaging study of choice for evaluating adrenal masses in pediatric age is abdominal ultrasound3,4,5,11. This allows to define the characteristics of the mass: size, structure, location, blood flow, and relationship with adjacent structures. Lesions suspected of malig nancy should be studied with CT scan or MRI3. Other imaging methods, such as Iodine-123 (I123) metaiodo- benzylguanidine (MIBG) scintigraphy, may be used as a complementary study, especially for staging in NB11,13.

There are several reports in the scientific literature published of EPS simulating NB. Curtis et al. descri be that in 1997 for each EPS, 2.5 NB5 were diagnosed. In addition, Kalenahalli et al. reported an atypical EPS case, given the absence of other associated malforma tions, which would be present in up to 65% of EPS ca ses14. Some elements of EPS in ultrasound are: location in the left adrenal space, visualization of an aberrant artery (usually from the aorta) on the Doppler ultra sound, displaced adrenal gland, visible as a separate entity, and diagnosis during the second trimester of pregnancy. In the NB, the most frequent situation is to observe a complex cystic image (50% of the cases), without unique nutrient artery image; the diagnosis generally occurs from the third trimester of the preg nancy. It is unusual to visualize the ipsilateral adrenal gland4. Additionally, the absence of catecholamine metabolites in urine (vanillylmandelic acid [VMA] and homovanillic acid [HVA]) supports the diagnosis of EPS3. The negative MIBG scintigraphy supports the benign character of the lesion, given that more than 90% of NBs are avid for the marked isotope13. In the presented case, the diagnosis was the NB given the sus picious imaging study and urinary catecholamines in the limit of the normal range.

The good prognosis of NB in early stages has led to questions about the need for immediate resection in patients with prenatally detected adrenal masses6-8. In the follow up of lesions that remain stable over time in terms of their appearance, size, and markers in blood or urine, partial or complete regression of the lesions has been observed2, which would allow to avoid or defer surgery6,7,15. Although the management of EPS has traditionally been surgical, Yoon et al. describe signifi cant rates of spontaneous regression at four years, as sociated with certain imaging elements, suggesting that surgical treatment could be considered in patients who meet this time period, have not presented regression or have presented complications9. In another report, Obeidat et al. describe a case similar to the one pre sented, which was followed-up through ultrasound until spontaneous resolution at 2 years of age16. Since the 1990s, the information regarding the possibility of observing selected patients with an NB diagnosis began to increase, since a significant percentage (up to 60%) return over time6-8. The first observation protocols in cluded patients with a diagnosis of NB detected by uni versal screening at 6 months of age, patients under one year of age at the time of follow-up, in stages I-II, with a solid lesion less than 5 cm in size or cystic lesion less than 3.1 cm, without vascular compromise and with negative serum markers6. An important series suggests that up to 81% of patients younger than 6 months could be observed without surgical treatment and that the remaining percentage have benign behavior, with a 3-year survival of 100%6-8. Other smaller series suggest that even 60% of children under one year of age may be included in this category.

Nuchtern et al. published in 2012 the results of a prospective follow-up protocol in patients younger than 6 months with a diagnosis of NB stage INSS I of the Children’s Oncology Group Study, followed-up between 2001 and 201015. 83 patients were monitored by ultrasound and urine VMA/HVA for 90 weeks, at weeks 3-6-12-24-66 and 90, and CT scan/MRI three times. The criteria for defining the need for surgical resection was growth greater than 50% of the initial volume or increase in urinary metabolites higher than 50% of the basal value. 56 patients (67.4%) completed the observation period, 71% of them had residual mass < 1 ml at the end of follow-up. 16 patients underwent surgical resection before completing the observation time, 10 of them had NB, and 2 had disease progres sion. Two patients had EPS. Event-free survival was 100 and 96% for patients with cystic and solid mas ses respectively. Resection-free survival was 79.8% at 3 years. Surgery was avoided in 81% of the patients15. In this case, early resection was chosen, considering an important degree of parental anxiety and expectations for the establishment of a definitive diagnosis, associa ted with the technical feasibility of performing mini mally invasive surgery expeditiously.

Pulmonary sequestration is a congenital malforma tion of the lower airway. It consists of a lung mass not communicated with the tracheobronchial tree, irriga ted by an artery derived from systemic circulation17. Congenital malformations of the pulmonary airway have an incidence of 1: 10,000 - 1:35,000 live births, where the most frequent is congenital pulmonary airway malformation (CPAM), formerly called conge nital cystic adenomatoid malformation (CCAM), with an incidence of 1 in 7200 live births (18). Pulmonary sequestration, which is a rare malformation, represents 0.15-6.4% of the group1. According to its relationship with the parenchyma, they are divided into intralobar (they do not have visceral pleura) and extralobar (they have visceral pleura). This last group accounts for 25% of the cases and can be found both inside and outside the chest (5-10%). Most EPS are left and are located between the lower lobe of the lung and the diaphragm. It is accepted that 50% of them are associated with other congenital anomalies11,14. Antenatal diagnosis with ultrasound is increasingly common3. EPS is usua lly diagnosed early in life, while intralobar pulmonary sequestration is usually diagnosed later, manifesting itself mainly as repeated infections in the same anato mical area. This is rare in the EPS17. In Chile, there are few publications on this subject and there is no national data of pulmonary malformations that would allow us to know its real incidence19. A series of 17 CPAM cases was recently published, which retrospectively analyses the cases that occurred between 2005 and 2016 in two reference hospitals in Spain, and reports an incidence of 1.7/10,000 births20.

The typical image of the EPS in the ultrasound is that of a uniform mass, usually pyramidal. Identifying a nutrient artery from the aorta or another systemic vessel may help to establish the diagnosis11. Despite this, establishing a preoperative diagnosis is extremely difficult and is usually done with histopathological analysis. If the diagnosis is prenatal, the imaging study should be supplemented after birth, preferably with a CT scan4.

The management of pulmonary sequestration has traditionally been surgical and should be urgent in children with respiratory distress at birth, but may be deferred in asymptomatic children or those with re current infections. In completely asymptomatic chil dren, the decision between surgery and observation is controversial, where the main reason for conservative management is the low complication rate of EPS. Ar guments in favor of early surgery are the need for diag nostic confirmation (as opposed to other alternatives such as NB), the risk decrease of subsequent compli cations, and the best prognosis when surgery is per formed before it develops1,2. Surgical resection can be done by video-assisted thoracoscopy or laparoscopy2. Partial lobectomy is the treatment of choice in intra-lobar sequestration, while in EPS the mass is resected with blood vessels ligation1. The complication rate is low1,2.

The first report of laparoscopic approach in pul monary sequestration dates from the year 2000, in a 4-month-old patient, born prematurely at 31 weeks of gestational age, with antenatal diagnosis of pul monary sequestration21. New studies have supported these results, thus laparoscopy is the technique of choice12-22-24.


Adrenal tumors in newborns are rare, and the di fferential diagnosis includes benign and malignant le sions, the most important of them is NB. Pulmonary sequestration is a rare pulmonary congenital malfor mation and surgery is the treatment of choice.

Considering the evidence available in scientific literature, it has been questioned whether all con genital adrenal masses should be resected, especia lly those that are asymptomatic. However, given the low morbidity of minimally invasive surgery and the relevance of establishing a diagnosis, we believe it is reasonable to consider early resection of this type of lesion as opposed to expectant management. Parents of patients should know the management alternati ves, their risks, and benefits, and participate in the therapeutic decision that is advised in each case by the treatment team.

Ethical Responsibilities

Human Beings and animals protection: Disclosure the authors state that the procedures were followed ac cording to the Declaration of Helsinki and the World Medical Association regarding human experimenta tion developed for the medical community.

Data confidentiality: The authors state that they have followed the protocols of their Center and Local regu lations on the publication of patient data.

Rights to privacy and informed consent: The authors have obtained the informed consent of the patients and/or subjects referred to in the article. This docu ment is in the possession of the correspondence author.

Financial Disclosure: Authors state that no economic support has been asso ciated with the present study.

Conflicts of Interest: Authors declare no conflict of interest regarding the present study.


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Received: July 17, 2018; Accepted: February 28, 2019

Correspondence: Juan Carlos Pattillo Silva. E-mail:

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