What is the Differential Diagnosis of a Suprarenal Mass?

Patient Presentation
A full-term newborn male was admitted to the newborn nursery after an uncomplicated vaginal birth and uneventful pregnancy. A right-sided suprarenal mass had been identified on routine ultrasound during the third trimester that was thought to likely be an adrenal hemorrhage. The family history was negative for congenital or genetic problems or cancer.

The pertinent physical exam showed a healthy appearing male with normal vital signs, with a weight of 3.45 kg, length of 52 cm and head circumference of 35 cm were all between 25-50%. His skin was normal without jaundice, or lesions except for a nevus simplex at the base of the skull. His heart and lungs were normal. His abdominal examination was soft, non-tender without hepatosplenomegaly or identifiable masses. He had normal genitalia and remnants of meconium were on the anus from his defecation after birth.

The diagnosis of a healthy male was made. The radiologic evaluation of an ultrasound examination showed a small hypoechoic mass in the area of right adrenal gland that was smaller than previously recorded prenatally. This was felt to be consistent with adrenal hemorrhage. The patient’s clinical course showed that he had additional imaging at 1 month again which showed decreased size of the mass, and it had resolved by 2 months of age.

Case Image

Figure 136 – Sagittal US of the left kidney shows a primarily cystic appearing left adrenal mass. Over months this mass was noted to decrease in size and disappear on serial ultrasound exams.

Discussion
Suprarenal masses (SRM) are not that common (incidence of 1.9/1000 in neonates), but are more commonly diagnosed prenatally because of prenatal ultrasound. Masses can be difficult to accurately diagnose even after birth because their features overlap. Radiographically they can be variable in size, cystic/solid or mixed and with or without calcifications. Common signs and symptoms of SRM include anemia, jaundice, scrotal hematoma and abdominal mass. Signs of asphyxia may also occur. Often patients whose diagnosis is not clear are monitored closely for a few weeks to see how the lesion changes with time. This is especially true for differentiating adrenal hemorrhage from neuroblastoma. This close monitoring does not appear to have poorer outcomes if the patient has neuroblastoma. Overall the prognosis is good to excellent for all diagnoses.

Learning Point
The differential diagnosis of suprarenal masses is relatively short and includes:

  • Primary causes
    • Adrenal hemorrhage
      • Overview: cause is unknown but thought to be because of insult such as sepsis or asphyxia or trauma to vasculature, or a bleeding disorder.
        Has been seen in patients with Beckwith-Wiedemann syndrome.

      • Incidence: 0.28-0.55%
      • Location: right side > left side
      • Timing: 3rd trimester
      • Radiographic findings: usually cystic but can look solid, more enhanced on edge or non-enhancing, features change depending on timing, resolves with time
      • Presentation: asymptomatic or can have signs and symptoms of SRM above
      • Treatment: resolves with time, resection may occur if diagnosis is in doubt
    • Neuroblastoma
      • Overview: cause is tumor from the postganglionic sympathetic neurons.
        Most arise from the adrenal gland (74.5%).
        They are the most common perinatal malignancy and fortunately their histology is generally favorable and they have a less aggressive nature than those that arise in older infants and children.

      • Location: right side > left side
      • Timing: 3rd trimester
      • Radiographic findings: solid or cystic (usually heterogeneous), more enhanced centrally, does not resolve but usually gets larger
      • Presentation: asymptomatic, abnormal vital signs or mass
      • Treatment: depends on type, may have spontaneous regression or need resection. Chemotherapy is often used for stage III or IV.
    • Subdiaphragmatic extralobar pulmonary sequestration (SEPS)
      • Overview: this is dysplastic lung tissue without connection to the airway and with a systemic arterial blood supply. It can occur within the lung and commonly is outside the lung (i.e. pleural, diaphragmatic, abdominal locations).
        It is different than congenital pulmonary airway malformation which is abnormal lung tissue which can have connection to the airway and is usually located within the lung.
        However both SEPS and CAM can occur together.
        SEPS is much less common than adrenal hemorrhage or neuroblastoma as a cause of SRM.

      • Location: Intraabdominal are ~15% of SEPS
      • Timing: 2nd trimester
      • Radiographic findings: cystic (homogenous) which does not resolve, often able to identify arterial blood supply which usually is from abdominal aorta
      • Associated problems: Has associated diaphragmatic defect, and often cardiac defects. Can cause mass effect.
      • Treatment: usually resected
  • Less common
    • Teratoma
    • Other cystic masses
      • Meconium peritonitis
      • Duplications, cystic masses and malformation of various abdominal organs such as adrenal, kidney, spleen, gastrointestinal, lymphatic, etc.

Questions for Further Discussion
1. What is the differential diagnosis of congenital lung malformations? A review can be found here
2. What are the most common pediatric cancers? A review can be found here
3. What are common diagnoses identified on prenatal ultrasound?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Adrenal Gland Disorders and Neuroblastoma.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Moon SB, Shin HB, Seo JM, Lee SK. Clinical features and surgical outcome of a suprarenal mass detected before birth. Pediatr Surg Int. 2010;26(3):241-246. doi:10.1007/s00383-009-2531-7

Yao W, Li K, Xiao X, Zheng S, Chen L. Neonatal suprarenal mass: differential diagnosis and treatment. J Cancer Res Clin Oncol. 2013;139(2):281-286. doi:10.1007/s00432-012-1316-x

Birkemeier KL. Imaging of solid congenital abdominal masses: a review of the literature and practical approach to image interpretation. Pediatr Radiol. 2020;50(13):1907-1920. doi:10.1007/s00247-020-04678-1

Schwab ME, Braun HJ, Padilla BE. Imaging modalities and management of prenatally diagnosed suprarenal masses: an updated literature review and the experience at a high volume Fetal Treatment Center. J Matern-Fetal Neonatal Med Off J Eur Assoc Perinat Med Fed Asia Ocean Perinat Soc Int Soc Perinat Obstet. 2022;35(2):308-315. doi:10.1080/14767058.2020.1716719

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa