Adrenal gland and paraganglia
Adrenal cortical adenoma - children

Topic Completed: 1 November 2013

Minor changes: 16 January 2020

Copyright: 2003-2020,, Inc.

PubMed Search: Adrenal cortical adenoma children

Carmen Perrino, M.D.
Debra Zynger, M.D.
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Cite this page: Perrino C, Zynger D. Adrenal cortical adenoma - children. website. Accessed May 27th, 2020.
Definition / general
  • Benign neoplasm arising from adrenal cortical cells, with variable functional activity
  • If functional, may have biochemically detectable or clinically recognizable features of an endocrine syndrome
  • Adrenal cortical neoplasm/tumor (ACN/ACT):
    • Refers to both adrenal cortical adenoma (ACA) and adrenal cortical carcinoma (ACC)
    • Term often used because these two entities can be difficult to distinguish histologically in children (Radiographics 1999;19:989)
  • Generally uncommon in children; only ~25 cases annually in U.S. in those < 20 years (Braz J Med Biol Res 2000;33:1225)
  • Bimodal age distribution; most commonly < age 5, second peak ages 9-16 years
  • Incidence higher in females, M:F ratio 1:1.6 (J Clin Oncol 2004;22:838)
  • Higher incidence in southern Brazil, associated with specific p53 mutation (R337H TP53)
  • Associated with several genetic syndromes:
    • Beckwidth-Wiedemann syndrome: hemihypertrophy, splanchnomegaly, macroglossia, intraabdominal neoplasms (i.e. adrenal cortical neoplasms, nephroblastoma, hepatoblastoma), due to alteration of 11p15 region
    • Li-Fraumeni syndrome (SBLA syndrome): sarcoma (rhabdomyosarcoma), breast/brain tumors, leukemia, laryngeal carcinoma, lung cancer, adrenal cortical carcinoma; due to alteration of p53 on chromosome 17p
    • Carney triad: malignant gastrointestinal stromal tumor, pulmonary chondroma, extra-adrenal paraganglioma, adrenal cortical adenoma
    • Adrenogenital syndrome: adrenal cortical neoplasms, congenital adrenal hypertrophy
  • No proven relationship with environmental factors
  • May arise due to defective apoptosis (J Clin Endocrinol Metab 2000;85:2048)
  • Predisposing genetic factors are present in ~50% of children with adrenal cortical tumors, most commonly Li-Fraumeni syndrome and Beckwith-Wiedemann syndrome (see above)
Clinical features
  • ~50% present with palpable mass
  • ~90% have an associated endocrine abnormality, most frequently due to secretion of several hormones causing multiple/mixed endocrine syndromes:
    • Virilization:
      • Females: increased muscle mass (Herculean habitus), facial hair, deep voice, pubic hair, clitoromegaly
      • Males: penile enlargement, pubic hair
    • Feminization: gynecomastia
    • Cushing syndrome: obesity (generalized in pediatric patients), moon facies, plethora, striae, thin skin, easy bruising, hirsutism, telangiectasias, hyperhidrosis, see Adrenal and Chemistry chapters
    • Conn syndrome: hypertension, proximal muscle weakness, headache, polyuria, tachycardia with/without palpitation, hypokalemia, hypocalcemia, see Adrenal and Chemistry chapters
  • Atypical histologic features are commonly found in children, making adrenal cortical adenomas difficult to distinguish from adrenal cortical carcinoma
  • Increased 24-hour urinary ketosteroid excretion (i.e. 17-ketosteroids)
  • Increased serum dehydroepiandrosterone sulfate (DHEA-S), cortisol, testosterone, androstenedione, and estradiol
Radiology description
  • Distinction between adrenal cortical adenoma and adrenal cortical carcinoma difficult radiologically
    • Hematogenous metastases or venous spread indicate carcinoma
  • Ultrasound: good initial screening test
    • Rounded or ovoid, circumscribed mass; lobulated border; may have thin, echogeneic capsule-like rim; rarely calcification; hyperechoic renal medullary pyramids
  • Computed Tomography (CT): best technique for examination
    • Circumscribed; heterogeneous (may have central hemorrhage and necrosis); thin, capsule-like rim; high-attenuation medullary areas with associated suprarenal mass on unenhanced CT; increased retroperitoneal fat; larger lesions enhance heterogeneously with IV contrast
  • Magnetic Resonance Imaging (MRI): few described cases
    • Intermediate signal intensity on T1-weighted images; high signal intensity relative to liver on T2-weighted images
Prognostic factors
  • Due to rarity and heterogeneity, definitive prognostic factors have been difficult to establish and multiple systems have been proposed (including Weiss and modified Weiss systems, see Adrenocortical carcinoma topic)
  • Tumor weight is most reliable adverse prognostic factor, with proposed cut-offs ranging from 100 to 500 grams
  • AFIP adverse prognostic factors in pediatric patients (0-2: benign; 3: indeterminate; 4+: malignant):
    • Tumor weight (>400 grams), tumor size (>10.5 cm), extension into periadrenal soft tissue/adjacent organs, invasion of vena cava, venous invasion, capsular invasion, necrosis, mitoses (>15/20 HPF), atypical mitotic figures (Am J Surg Pathol 2003;27:867)
Case reports
  • Surgical resection is only definitive management
  • Steroids given to patients with functioning tumors and suppression of contralateral adrenal gland
  • Chemotherapy not well established
Radiology images

Images hosted on other servers:

Homogeneous left adrenal mass with distinct borders

Heterogeneous retrohepatic mass

Homogeneous, solid right suprarenal mass

Flank mass with echogenic focus

Left flank mass with calcification

Heterogeneous mass, necrosis, capsule-like rim

Clinical images

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Moon facies, acne, bitemporal excess hair

Moon facies and central obesity

Facial and chest hair, absence of breasts

Back acne

Penile enlargement

Clitoromegaly and pubic hair

Gross images

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External surface, during surgery

Intact capsule

Smooth, homogeneous cut surface

Microscopic (histologic) description
  • Resembles adrenal cortical adenoma in adults - pale, polygonal cells with vacuolated to eosinophilic cytoplasm
  • Architectural patterns: Alveolar/nesting, solid, trabecular, oncocytic, myxoid, pseudoglandular
  • May have nuclear pleomorphism, nuclear hyperchromasia, enlarged nuclei, necrosis, capsular invasion, rare mitoses
  • Intracytoplasmic hyaline globules in 10%
Microscopic (histologic) images

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Adrenal cortical neoplasm with pale vacuolated cells

Small round tumor cells

Nuclear atypia, pleomorphism, multinucleated forms

Neoplasm on right
and normal adrenal

Virtual slides

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Atypical adrenal cortical adenoma

Cytology description
  • May resemble normal adrenal cortical cells or have focal cytologic atypia
  • Cytoplasm is clear/translucent and vacuolated due to lipid
  • Small, round, uniform nuclei with granular chromatin and distinct nucleoli
  • Naked nuclei may be present in a background of small lipid droplets
Positive stains
  • Positive (75%+ of cases): inhibin, MelanA/Mart1, NSE, CD56, vimentin (Mod Pathol 2003;16:591)
  • Usually positive (~60% of cases): synaptophysin
  • Low Ki-67 index
Negative stains
  • Cytokeratin (positive in 10%), chromogranin
Electron microscopy description
  • Intracytoplasmic lipid droplets, abundant smooth endoplasmic reticulum, nuclear pseudoinclusions (due to irregularity of nuclear membrane), prominent mitochondria
Molecular / cytogenetics description
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