Imaging Pearls ❯ Adrenal ❯ Pediatric Patients
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- “These lesions can be classi ed by their cause: congenital (eg, discoid adrenal gland, horseshoe adrenal gland, and epithelial cysts), vascular and/ or traumatic (eg, adrenal hemorrhage), infectious (eg, granulomatous diseases), enzyme deficiency disorders (eg, congenital adrenal hyperplasia [CAH] and Wolman disease), benign neoplasms (eg, pheochromocytomas, ganglioneuromas, adrenal adenomas, and myelolipomas), and adrenal mass mimics (eg, extralobar seques- tration and extramedullary hematopoiesis).”
Imaging of Nonmalignant Adrenal Lesions in Children Sargar KM et al. RadioGraphics 2017; 37:1648–1664 - “Infradiaphragmatic extralobar sequestration may mimic an adrenal mass at imaging. Suprarenal extralobar sequestration may be seen as a well-de fined radiopacity in the upper paraspinal region on chest and abdominal radiographs. US demonstrates a heterogeneous suprarenal mass, often pyramidal. The increased echogenicity is related to multiple tissue interfaces within the lesion. A thin and highly echogenic rim around the lesion may also be seen. The blood supply is from the aorta or branches of the aorta.”
Imaging of Nonmalignant Adrenal Lesions in Children Sargar KM et al. RadioGraphics 2017; 37:1648–1664 - “An adrenal hemorrhage is the most common cause of an adrenal mass in a neonate, and it usually occurs in the rst few days of life. In the newborn period, adrenal hemorrhage occurs in two of 1000 patients, and they are nearly four times more common than neuroblastomas. Ten percent of adrenal hemorrhage cases are bilateral, and 70% are on the right side . The reason for the right side preponderance is unclear, although one theory suggests that this is due to the compression of the right adrenal gland between the liver and right kidney.”
Imaging of Nonmalignant Adrenal Lesions in Children Sargar KM et al. RadioGraphics 2017; 37:1648–1664 - “Adrenal calci cations carry a broad differential diagnosis and commonly occur as a sequel of prior adrenal hemorrhage or old granulomatous infections, such as histoplasmosis and tubercu- losis. Rarely, viral infections of childhood, such as disseminated neonatal herpes simplex and cytomegalovirus infections, may cause calci- cations. Wolman disease is a rare autosomal recessive disorder with acid lipase de ciency that causes adrenal gland calcifications. In addition, lipid deposition is seen in the liver, adrenal gland, spleen, and lymph nodes in patients with Wolman disease.”
Imaging of Nonmalignant Adrenal Lesions in Children Sargar KM et al. RadioGraphics 2017; 37:1648–1664 - “On nonenhanced CT images, low attenuation less than 10 HU is characteristic of lipid-rich adenomas. Lipid-poor adenomas show higher attenuation values. Calci cations are less common in adenomas than in adrenocortical carcinomas. At contrast-enhanced CT, adenomas show moderate homogeneous contrast enhancement with rapid washout at delayed imaging. At MR imaging, the lipid-rich adenomas show a signal intensity drop on opposed-phase chemical shift images, due to the presence of microscopic fat.”
Imaging of Nonmalignant Adrenal Lesions in Children Sargar KM et al. RadioGraphics 2017; 37:1648–1664 - “The formerly used “10% rule” has been challenged. According to the 10% rule, 10% of the pheochromocytomas are hereditary. In children, approximately 40% of pheochromocytomas are associated with known genetic mutations, 8%–43% are extra-adrenal, and 19%–38% are bilateral adrenal. Multiple tumors may be seen in 30%–70% of cases, especially in those with a familial predisposition.”
Imaging of Nonmalignant Adrenal Lesions in Children Sargar KM et al. RadioGraphics 2017; 37:1648–1664 - “Pheochromocytomas occur in von Hippel– Lindau syndrome, multiple endocrine neoplasia syndrome type 2, neuro bromatosis type 1, and familial paraganglioma syndromes. Familial paraganglioma syndromes are caused by muta- tions in the succinate dehydrogenase (SDH) gene.”
Imaging of Nonmalignant Adrenal Lesions in Children Sargar KM et al. RadioGraphics 2017; 37:1648–1664 - “A ganglioneuroma is a primary neural crest neoplasm of sympathetic nerve origin, which most commonly arises from the posterior mediastinum or retroperitoneum .Other locations include the adrenal gland, skin, tongue, appendix, and lymph nodes Ganglioneuromas occur in the adrenal glands in 21% of cases. It is the most mature and benign form of the neural crest cell tumors. It contains mature ganglion cells and encapsulated nerve bers, but it does not contain neuroblasts or increased mitotic figures.”
Imaging of Nonmalignant Adrenal Lesions in Children Sargar KM et al. RadioGraphics 2017; 37:1648–1664 - “At CT, the mass is well-defined with homo- geneous low attenuation, similar to or slightly less attenuated than muscle. There is usually mild enhancement after
intravenous contrast material administration. The enhancement may be homogeneous or heterogeneous. About 50% of ganglioneuromas demonstrate calci cation at CT. The calci cations are more often small and punctate, unlike the more coarse calcifications typically seen in a neuroblastoma.”
Imaging of Nonmalignant Adrenal Lesions in Children Sargar KM et al. RadioGraphics 2017; 37:1648–1664 - “Extramedullary hematopoiesis (EMH) is de ned as the production of blood cells outside nor-
mal bone marrow. It is most commonly seen in chronic hemolytic anemias such as thalassemia, sickle cell anemia, and hereditary spherocytosis, as well as in lymphoma and leukemia. EMH is less common in children than adults, and it is most commonly seen in adolescents. In most patients, EMH is usually asymptomatic; however, patients may present with symptoms secondary to mass effect. In the abdomen, EMH may be seen involving the perirenal space, kidney, and adrenal gland.”
Imaging of Nonmalignant Adrenal Lesions in Children Sargar KM et al. RadioGraphics 2017; 37:1648–1664