Adrenocortical adenoma

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Adrenocortical Adenoma
Other namesAdrenal cortical adenoma, adrenal adenoma
Conn syndrome
SpecialtyEndocrinology, oncology Edit this on Wikidata

An adrenocortical adenoma or adrenal adenoma is commonly described as a

hypercortisolism. The term "Cushing's disease" refers specifically to "secondary hypercortisolism" classified as "ACTH-dependent Cushing's syndrome" caused by pituitary adenomas
. In contrast, "Cushing's syndrome" refers specifically to "primary hypercortisolism" classified as "ACTH-independent Cushing's syndrome" caused by adrenocortical adenomas.

Presentation

Hypercortisolism
SpecialtyEndocrinology, oncology Edit this on Wikidata
SymptomsAdrenal Lesion

Adrenal adenomas are often categorized as endocrine-inactive tumors considering that majority of them are non-functioning and

Some of the common symptoms associated with adrenocortical adenomas include:

Musculoskeletal

Cardiovascular

Endocrine and Metabolic

→More prevalent in males

→More prevalent in females

Neuropsychological

Skin

Cause

Adrenal Glands Zonations
SpecialtyEndocrinology, oncology Edit this on Wikidata
Adrenal Glands Zonations
SpecialtyEndocrinology, oncology Edit this on Wikidata

Study of the reported cases indicate that most adrenocortical adenomas occur due to neoplastic proliferation of adrenal cortical cells within the three distinct layers of adrenal cortex. In humans, the adrenal cortex comprises three concentric zones including the zona glomerulosa, zona fasciculata, and zona reticularis that under normal conditions respond to body's physiological demands for steroid hormones. The adrenal cortex is considered a dynamic organ in which senescent cells are replaced by newly differentiated cells. This constant renewal facilitates organ remodeling which contributes to dynamic characteristics of the adrenal cortex.

cyclic AMP-dependent protein kinase A signaling has been identified as a key mediator of cortisol secretion, and the mutations associated with the dysregulation of cyclic AMP - protein kinase A pathways have been implicated in the adrenocortical pathophysiology.[8]

Pathophysiology

If functional, adrenocortical adenomas can affect the normal activities of the adrenal cortex. Located within the adrenal glands are the three zones that are responsible for secretion of the three major classes of adrenal steroids. Hence, functional adrenocortical adenomas can induce over-secretion of adrenal steroids associated with pure or mixed endocrine syndromes, a condition commonly known as

]

Diagnosis

Due to their asymptomatic nature, most reported cases of adrenal adenomas have been discerned fortuitously through

computed tomography) and magnetic resonance imaging. Hence, they have earned the title incidentaloma referring to small adenoma discovered incidentally.[9] Though adrenocortical adenomas are considered challenging to differentiate from the normal adrenal cortex, they appear as well-circumscribed lesions once isolated. [citation needed
]

Imaging Diagnostics

Laboratory Tests

Gross Description

  • Well-circumscribed lesion
  • Size ≤ 5 cm
  • Weight ≤ 50 grams
  • often appear as golden-yellow color mass

(may have focal dark regions corresponding to

hemorrhage, lipid-depletion, and increased lipofuscin)[9]

Histopathology

Zona fasciculata versus adrenocortical adenoma. An adrenocortical adenoma typically has mild changes in comparison, including larger cells with larger and more pleomorphic nuclei with more coarse chromatin. H&E stain.

The microscopic histopathology analysis of the tissue samples obtained from the adrenal cortex of individuals presenting with adenoma-associated symptoms such as primary aldestronism (PA) indicates that adenoma cells are relatively larger with different cytoplasm, and increased variation in nuclear size. This indication is based on comparison between the healthy (normal) and affected (adenoma-associated) adrenal cortex tissue samples. [citation needed]

  • Adrenocortical adenoma with focal high grade nuclear atypia
    Adrenocortical adenoma with focal high grade nuclear atypia
  • Adrenocortical adenoma with focal necrosis
    Adrenocortical adenoma with focal necrosis

Adrenocortical adenomas are most commonly distinguished from adrenocortical carcinomas (their malignant counterparts) by the Weiss system,[10] as follows:[11]

Characteristic[11] Score
High nuclear grade (enlarged, oval to lobated, with coarsely granular to hyperchromatic chromatin and easily discernible, prominent nucleoli)[12] 1
More mitoses than 5/50 high power fields 1
Atypical mitoses 1
Eosinophilic cytoplasm in >75% of tumor cells 1
Diffuse architecture of >33% of tumor 1
Necrosis 1
Venous invasion 1
Sinusoidal invasion (no smooth muscle in wall) 1
Capsular invasion 1

Total score indicates:[11]

  • 0-2: Adrenocortical adenoma
  • 3: Undetermined
  • 4-9: Adrenocortical carcinoma

Treatment

  • Non-functioning cases of adrenocortical adenoma can be managed through long-term followups and monitoring.[citation needed]
  • The treatment approach for the functioning cases of adrenocortical adenoma depends on the type of disorders they induce and their advancement. Surgical excision may be required if its presence is resulting in atrophy of the adrenal glands and the surrounding tissues.[citation needed]

In order to acquire better treatment strategies, it is important to further examine, study and discern the distinct molecular mechanisms involved in the formation of

endogenous Adrenal Adenomas, hyperplasias, and ACTH-independent Cushing's Syndrome to improve the available diagnostic and prognostic markers that can assist clinicians in the management and advance-treatment of such conditions.[13]

A 2018 Cochrane Systematic review compared two different types of surgery: laparoscopic retroperotenial adrenalectomy and laporoscopic transperitoenal adrenelectomy in different types of adrenal tumors.[14] Laparoscopic retroperotenial adrenalectomy appeared to reduce late morbility, time to oral fluid or food intake and time to ambulation, when compared to laparoscopic transperitoenal adrenalectomy.[14] However there was uncertainty in these findings due to low-quality evidence, as well as inconclusive findings about effects of either surgery on all-cause mortality, early morbidity, socioeconomic effects, duration of surgery, operative blood loss, conversion to open surgery.[14]

Prognosis

  • The long-term outlook for individuals diagnosed with non-functional adrenocortical adenoma is usually excellent.
  • The long-term outlook for individuals diagnosed with functional adrenocortical adenoma is good with early diagnosis and treatment.

Epidemiology

Incidences and prognoses of adrenal tumors,[15] with adrenocortical adenoma at left.
  • Prevalence: Female > Male
  • More common in adults
  • Relatively earlier onset in females (ages ≤ 20) than males (ages ≤ 30)
  • Most common cause of
    ACTH-independent Cushing's syndrome

See also

References

  1. ^ "Definition: adrenocortical adenoma from Online Medical Dictionary".
  2. ^
    PMID 30061126
    .
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  12. ^ Tito Fojo (26 December 2016). "Adrenocortical Cancer". Retrieved 2020-07-02.
  13. S2CID 40454423
    .
  14. ^ .
  15. ^ Data and references for pie chart are located at file description page in Wikimedia Commons.

External links