Adrenal Glands Flashcards
What is the arterial supply of the adrenal glands?
Arterial Supply
- Superior adrenal artery: branch of inferior phrenic artery
- Middle adrenal artery: branch of the aorta
- Inferior adrenal artery: branch of the renal artery
What is the venous Drainage of the Adrenal Glands?
Venous Drainage
Each gland is drained by a single vein that enters into the:
- IVC on the right
- Renal vein on the left
What are the different regions of the adrenal glands and their respective hormones?
Physiology
- Cortex divided into 3 zones:
- Zona glomerulosa (aldosterone)
- Zona fasciculata (ACTH-dependent)
- Zona reticularis (cortisol)
- Medulla (epinephrine, norepinephrine)
GFR
“The deeper you go the sweeter it gets.” Salt, Sugar, Sex
- Mineralocorticoids (aldosterone)
- Glucocorticoids (cortisol)
- Androgens
What is the rule of 10s?
Rule of “10s”:
- 10% of pheochromocytomas are extraadrenal,
- 10% are bilateral, and
- 10% are malignant.
Medullary Tumors
Pheochromocytoma
Pheochromocytoma is a subtype of a paraganglioma, a neuroendocrine tumor that arises from paraganglionic tissue.
The most common location of an extraadrenal pheochromocytoma is the organ of Zuckerkandl (near aortic bifurcation).
A phaeochromocytoma is a tumour of which part of the adrenal Gland?
Medullary Tumors
Pheochromocytoma
Paraganglioma arising from adrenal medulla
Pheochromocytoma is a subtype of a paraganglioma, a neuroendocrine tumor that arises from paraganglionic tissue.
What is the most common site of extra-adrenal pheochromocytoma?
The most common location of an extraadrenal pheochromocytoma is the organ of Zuckerkandl (near aortic bifurcation).
- Intro
- The organ of Zuckerkandl comprises of a small mass of chromaffin cells derived from neural crest located along the aorta, beginning cranial to the superior mesenteric artery or renal arteries and extending to the level of the aortic bifurcation or just beyond. The highest concentration is typically seen at the origin of the inferior mesenteric artery.
- Physiology
- Its physiological role is thought to be of greatest importance during the early gestational period as a homeostatic regulator of blood pressure, secreting catecholamines into the fetal circulation.
- The organ regresses at the end of gestation and following birth to form the aorticosympathetic group of the adult paraganglia.
- Radiographic features
- The organs of Zuckerkandl are not often visualized radiologically unless they are involved in a pathologic process, including:
- paragangliomas 1
- pheochromocytoma
- neuroblastoma (rare) 3
- paragangliomas 1
- The organs of Zuckerkandl are not often visualized radiologically unless they are involved in a pathologic process, including:
- https://images.radiopaedia.org/cases/extra-adrenal-pheochromocytoma-2?lang=us
What are nonchromaffin paraganglioma?
Parasympathetic paragangliomas including chemodectomas (nonchromaffin paraganglioma)
- Glomus tympanicum
- Glomus jugulare
- Glomus vagale
- Carotid body tumor
- Others, not specified
Nonchromaffin paraganglion cells are cells in the neuroendocrine system that make up several clusters of chemoreceptive cells. These cells are associated with a supporting matrix and are found in close proximity to blood vessels and nerves (especially the glossopharyngeal and vagus nerves). They sense pH, carbon dioxide, and oxygen concentrations in the blood and participate in respiratory, and circulatory control.
Examples of nonchromaffin paraganglia include:
- carotid body
- aortic body
- glomus jugulare
- glomus tympanicum
Chromaffin cells: are the bodies’ main source of circulating catecholamines (adrenaline, noradrenaline) and endorphins, which are stored in intracellular granules and released in response to stress.
Wiki: Chromaffin cells, also pheochromocytes, are neuroendocrine cells found mostly in the medulla of the adrenal glands in mammals. These cells serve a variety of functions such as serving as a response to stress, monitoring carbon dioxide and oxygen concentrations in the body, maintenance of respiration and the regulation of blood pressure.[1] They are in close proximity to pre-synaptic sympathetic ganglia of the sympathetic nervous system, with which they communicate, and structurally they are similar to post-synaptic sympathetic neurons. In order to activate chromaffin cells, the splanchnic nerve of the sympathetic nervous system releases acetylcholine, which then binds to nicotinic acetylcholine receptors on the adrenal medulla. This causes the release of catecholamines. The chromaffin cells release catecholamines: ~80% of adrenaline (epinephrine) and ~20% of noradrenaline (norepinephrine) into systemic circulation for systemic effects on multiple organs (similarly to secretory neurones of the hypothalamus), and can also send paracrine signals. Hence they are called neuroendocrine cells.
https://thorax.bmj.com/content/thoraxjnl/11/1/57.full.pdf
What is a paraganglioma cell?
What system do they belong to?
What do they secrete?
- Paraganglioma cells belong to the amine precursors uptake and decarboxylation (APUD) system.
- Cells may secrete catecholamines (epinephrine, dopamine, norepinephrine) or be nonfunctional.
Presentation on theme: “APUDOMAS (Diffuse Endocrine System)”— Presentation transcript:
1 APUDOMAS (Diffuse Endocrine System) J. O. Ogunbiyi Department of Pathology University College Hospital Ibadan, Nigeria
2 IntroductionThese are tumours of APUD cells. Some secrete the normal hormone of their presumptive cell of origin and are called orthoendocrine. Those secreting hormones of other apud cells are called paraendocrine tumours.
3 A high content of amines The capacity for amine precursor uptake
The APUD cells derive their name from the initial letters associated with their three most important properties:A high content of aminesThe capacity for amine precursor uptakeThe presence of amino acid decarboxylase, which converts amino acids into amines.
4 They contain characteristic granules on electron microscopy
Cells with these properties have been grouped together as the APUD system.They contain characteristic granules on electron microscopyand secrete polypeptides, or amines, or both
5 The cells included here are:
The chromaffin cell system- These are found in the adrenal medulla and in association with the paravertebral plexuses.The non-chromaffin cells of the paraganglia (Carotid body, glomus jugulare).The argentaffin (Kultschitzky) cells ( found in the intestine). Similar cells occur in the salivary glands, pancreas, and bronchial mucosa. . The argyrophil cells. These are widely distributed in the intestine.
6 Other neuroectodermal cells are present in the stomach and small intestine
These are responsible for secretion of VIP, cholecystokinin, gastrin, 5HT, etc
7 They includePancreatic islet cells, Thyroid C cells, Parathyroid cells, Melanocytes, Hypothalamic neuroendocrine cells, Some cells of the anterior pituitary, and The autonomic neurons
8 The following syndromes are accompanied by apudomata
9 Hypoglycaemia (pancreatic ß-islet cell hypersecretion) The glucagonoma syndromeThe Zollinger-Ellison syndrome (in which there is hyperplasia of the G cells of the pyloric antrum or of the B-cells of the pancreas).The Verner-Morrison syndrome (pancreatic cholera)
10 WDHA (watery diarrhea, hypokaleamia, and achlorhydria) syndrome. Non-ß islet cell tumour, SCLC, MCT, malignant carcinoid, mast cell tumours, and neuroblastoma. In all of these, there is XS VIP secretion
11 Cushing’s syndrome / (ectopic ACTH syndrome) consisting
hypokaleamia alkalosis, diabetes mellitus, hyperpigmented skin, muscle wasting with weakness.May be found with SCLC, bronchial carcinoid, carcinoid of thymus, pancreatic islet cell tumour, MTC, pheo, ovarian carcinoma
12 The carcinoid syndrome.
Those of the foregut tend to be secretory and active unlike the hindgut ones that tend to be inactive
13 The somatostatin syndrome. D-cell tumours of the pancreas.
The MEN syndromes
https://slideplayer.com/slide/12782905/
What are the clinical findings of Phaeochromocytoma?
What is elevated in the serum and urine?
Clinical Findings (Excess Catecholamines)
- Episodic (50%) or sustained (50%) HTN
- however, only 0.1% of hypertension is caused by pheochromocytomas.
- tachycardia,
- diaphoresis,
- headache (catecholamine-producing tumors);
Tests:
- Elevated vanillylmandelic acid (VMA) in 24-hour urine in 50%
- Elevated serum catecholamines
- Elevated urine metanephrines
SYNDROMES
What 5 Genetic conditions/syndromes are a/w Phaeochromocytomas?
- Genetic diseases include:
- Multiple endocrine neoplasia type II (MEN II)
- von Hippel-Lindau disease
- Neurofibromatosis type I
- Tuberous sclerosis
- Sturge-Weber syndrome
10% RULE FOR PHEOCHROMOCYTOMAS/PARAGANGLIOMAS
Inaccurate but still a helpful memory tool:
- 10% are paragangliomas (ie, extra-renal).
- 10% are bilateral.
- 10% are malignant.
- 10% present in children.
- 10% are NOT associated with hypertension
- 10% contain calcification
- 30% are familial (ie, genetic).
*
What are the associations of Phaechromocytomas?
What % of people which each syndrome have Phaeos?
Associations
- MEN IIa
- Multiple endocrine neoplasia in 5%;
- pheochromocytomas are usually bilateral
- Almost always intraadrenal.
- NF1
- 10% of patients have pheochromocytomas
- VHL disease
- 10% of patients have pheochromocytomas
- Familial pheochromocytosis
- 10% of all pheochromocytomas
Pheochromocytoma: inherited associations, bilaterality, and cortex preservation
W B Inabnet 1, P Caragliano, D Pertsemlidis
Affiliations expand
PMID: 11114636
DOI: 10.1067/msy.2000.110846
Abstract
Background: Hereditary pheochromocytoma (HP) is characterized by early onset, bilateral adrenal involvement, low malignancy rate, and genetic linkage with certain familial syndromes. This retrospective review is intended to show the high yield of surveillance, predictable bilaterality, and the challenge of cortex-sparing adrenalectomy.
Methods: From 1964 to 1999, 32 patients with HP were treated at a single institution and followed for a mean of 7 years. There were 15 cases of multiple endocrine neoplasia type 2A (MEN 2A), 12 cases of von Hippel-Lindau (VHL) disease, 3 cases of von Recklinghausen’s disease (VRD), and 2 cases of familial pheochromocytoma. Twenty-four of 32 patients underwent bilateral adrenalectomy (9 metachronous). Subtotal resection with orthotopic cortex preservation was performed in 5 patients, and heterotopic autografting was performed in 14 patients.
Results: Pheochromocytoma was the first manifestation in 50% of patients with VHL disease and in 27% of patients with MEN 2A. Surveillance uncovered medullary thyroid cancer in 5 of 15 patients with MEN 2A and hemangioblastomas, renal cell carcinoma, and islet cell tumors in 7 of 15 patients with VHL disease and VRD. HP was bilateral in 24 of 32 patients (14/15 in patients with MEN 2A, 7/12 in patients with VHL disease, 2/3 in patients with VRD, and 1/2 in patients with familial pheochromocytoma). In 9 cases of metachronous adrenalectomy, the mean interval was 67 months (range, 9-156 months). Three of 5 patients who underwent orthotopic preservation of the adrenal cortex experienced recurrence compared with 0 of 14 patients with heterotopic autotransplantation of cortical tissue.
Conclusions: Pheochromocytoma frequently heralds coexisting silent VHL disease or MEN-2, mandating surveillance for inherited associations. The long interval of metachronous pheochromocytoma argues against prophylactic removal of the contralateral “normal” adrenal gland. Total adrenalectomy and heterotopic autotransplantation of medulla-free cortex may diminish the need for lifelong steroid substitution and eliminates recurrence.
https://pubmed.ncbi.nlm.nih.gov/11114636/
What tests are used to detect phaeochromocytomas?
What are the respective sensitivities?
Sensitivity for detection of functional tumors
- CT or MRI, 90%
- MIBG scintigraphy, 80%
- localize clinically suspected Phaeo
- confirm a mass is a phaeo
- exclude mets
- For adrenal pheochromocytomas, MIBG has close to 100% specificity but lower sensitivity (86%).
- Octreotide scintigraphy
- Used for extra-adrenal phaeos. esp H+N
- Octreotide can also localize adrenal pheochromocytomas
- but has a lower sensitivity than MIBG (20%-50%).
- It can be used in cases of negative MIBG scans.
http://roentgenrayreader.blogspot.com/2010/07/mibg-vs-octreotide-in-diagnosis-of.html
What are the imaging characteristics of Adrenal Phaeochromocytomas on Imaging?
Appearance
- Adrenal mass
- Strong contrast enhancement (CT, angiography)
- Calcification
- MRI:
- very high SI (“light bulb”) on T2W images.
- The SI is usually considerably higher when compared with adenomas or metastases.
- https://pubs.rsna.org/doi/10.1148/rg.24si045506
What is the method of choice for tissue dx of Phaeo?
What are the potential complications?
- Percutaneous biopsy:
- method of choice for tissue diagnosis;
- biopsy of pheochromocytoma may precipitate acute hypertensive crisis
- pharmacologic prophylaxis is therefore indicated.
What are the typical locations of Phaeos and their percentages?
- Location:
- adrenal, 85%;
- paraaortic, 8%;
- Zuckerkandl, 5%;
- urinary bladder, 1%
SYNDROME
Which syndrome is characterised by:
marfanoid habitus, coarse facial features with prognathism, and GI tract abnormalities
and Mucosal Neuromas
-
Clinically, MEN IIb:
- is characterized by marfanoid habitus, coarse facial features with prognathism, and GI tract abnormalities (constipation, diarrhea, feeding difficulties).
SYNDROME
Name this SYNDROME
pituitary adenoma,
parathyroid adenoma,
pancreatic islet cell tumor
Which gene?
MEN I: pituitary adenoma, parathyroid adenoma, pancreatic islet cell tumor
SYNDROME
What are the characteristics of MEN 1?
Peter-Pan-Part-1
Peter- Pituitary adenoma
Pan - pancreatic islet cell tumor
Part - parathyroid adenoma
1 = MEN1
What are the characteristics of MEN IIa?
What gene is involved?
- medullary thyroid carcinoma
- pheochromocytoma
- parathyroid adenoma
Parathyroid hyperplasia
Medullary thyroid cancer
Phaeochromocytoma
are part of which syndrome?
MEN 2a
SYNDROME
What are the characteristics of MEN IIB?
MEN IIb:
medullary thyroid carcinoma
pheochromocytoma
Mucosal neuromas (ganglioneuromas)
other soft tissue tumors
Two basic signs that an adrenal mass is malignant (until proven otherwise).
- Enlarging masses are considered malignant until proved otherwise.
- Masses >4 cm are also concerning for malignancy.
In the absence of known primary malignancy, adrenal adenocarcinoma should be considered.
What feature considers an adrenal mass benign?
What benign dx’s are most likely?
Masses with attenuation averaging ≤10 HU are all considered adenomas with the exception of adrenal cysts (also benign).
What feature considers an adrenal mass indeterminate?
What should be done next?
If the attenuation is more than 10 HU, the mass is considered indeterminate.
An enhanced and 15-min delayed enhanced CT scan is obtained.
What does APW stand for?
How do you calculate it?
APW = absolute percentage washout
RPW = relative percentage washout
An APW may be calculated as follows: APW = [(Enhanced – Delayed)/(Enhanced – Unenhanced)] × 100%.
If the APW is >60%, the lesion is likely an adenoma.
What does RPW stand for?
When do you use it?
How do you calculate it?
RPW: Relative Percentage wash out
If an unenhanced study is unavailable, an RPW can be calculated as follows:
RPW = [(Enhanced – Delayed)/(Enhanced)] × 100%.
If the RPW >40%, the lesion is likely an adenoma.
Which masses/Lesions should not be considered adenomas/benign despite their wash-out characteristics?
Lesions which are:
- heterogeneous
- enhance to ≥100 HU
should not automatically be considered adenomas even if they meet washout requirements.
What should you consider a lesion that does not meet ‘wash-out’ criteria?
What additional feature/criteria helps with this decision?
If the APW or RPW is less than about 60% or 40%, respectively, especially if the delayed attenuation value is more than 35 HU, the mass is considered indeterminate.
What role does chemical shift MRI play in assessing adrenal Adenomas?
Although chemical shift MRI (in-phase and out-of-phase T1 weighted images) can be used to characterize lipid-rich adenomas with accuracy similar to that of nonenhanced CT, adenomas with only small amounts of lipid will not be detected.
Chemical shift MRI may detect intracellular fat in adrenal adenomas, as manifested by a drop in lesion signal on out-of-phase images.
Lesion SI should be compared with that of spleen on both in-phase and out-of-phase images.
MRI Out-of-phase imaging
Lipid-poor adenomas can also be diagnosed with out-of-phase imaging.
They contain enough microscopic fat to cause a signal drop on out-of-phase imaging compared to in-phase imaging due to the chemical shift artefact.
These images are of a 65- year-old female patient with an incidental discovery of a right adrenal mass on an abdominal ultrasound performed for renal stones.
The presence of microscopic fat is demonstrated by the signal drop on the opposed-phase image.
The patient was followed for 2 years, because the lesion is slightly inhomogeneous and measures 5.2 cm.
The lesion did not change in size and was not hormonally active.
It was diagnosed as a lipid-poor adenoma.
https://radiologyassistant.nl/abdomen/adrenals/lesion-characterization
What tumour is usually >5cm at dx?
Most adrenal cortical carcinomas are >5 cm at presentation and often have demonstrable metastases.
Typically, these tumors also have large amounts of necrosis, which would invalidate attempts to assess enhancement washout.
https://radiologyassistant.nl/abdomen/adrenals/lesion-characterization
The image shows a 67 mm heterogeneously enhancing relatively well defined lesion.
This proved to be an adrenocortical carcinoma, after resection.
SYNDROME
What percent of this tumour type are ‘functioning’?
Which is the most common syndrome?
PRIMER:
Adrenocortical Carcinoma
Fifty percent of adrenocortical carcinomas are functioning (Cushing syndrome is the most common clinical manifestation).
Prognosis is poor because the tumor is usually large at time of diagnosis.
RAD ASSISTANT:
Adrenocortical carcinoma
Adrenocortical carcinomas (ACCs) are rare aggressive tumors with an incidence of approximately 1-2 per million per year [5].
60% of ACCs are functioning.
Cushing’s syndrome alone or a mixed Cushing’s and virilisation syndrome are the most common presentation.
Virilisation, feminisation or hyperaldosteronism alone is rare (< 10%).
Patients with nonfunctioning adrenocortical carcinomas usually present with abdominal symptoms like nausea, vomiting, abdominal fullness, flank or back pain due to the large size of the tumor.
https://radiologyassistant.nl/abdomen/adrenals/lesion-characterization
What Syndromes is this tumour associated with?
Adrenocortical carcinomas may be sporadic or associated with hereditary syndromes, including:
- MEN1,
- Lynch syndrome,
- Beckwith-Wiedemann syndrome and
- Li-Fraumeni syndrome.
Peak incidences are in early childhood and in the fourth and fifth decade of life [5].
https://radiologyassistant.nl/abdomen/adrenals/lesion-characterization
What are the typical imaging features of Adrenocortical Carcinomas?
- Imaging Features
- SIZE
- Mass usually >5 cm at time of diagnosis
- CT:
- heterogeneous enhancement because of areas of necrosis, hemorrhage;
- 50% have calcifications
- MRI:
- tumor appears hyperintense relative to liver T2W
- but is less hyperintense and usually much larger than pheochromocytoma.
- SIZE
- EXTENSION
- May extend into
- renal vein
- IVC
- RA
- May extend into
https://radiologyassistant.nl/abdomen/adrenals/lesion-characterization
Axial and coronal CT images in a patients with a right adrenal cortical carcinoma and extensive IVC invasion.
The coronal image also shows tumor extension into the right renal vein.
More than half of ACC patients have stage III or IV disease at the time of diagnosis.
Dismal prognosis of this diagnosis, with 5-year survival of
- 50% for stage III, and
- 15% for stage IV.
So, it is important to look for adjacent organ invasion, and for lymph node and distant metastases, most commonly in lung, liver or bone.
https://radiologyassistant.nl/abdomen/adrenals/lesion-characterization
Re Adrenal Mets, what is the incidence of ca patients at autopsy?
What are the 6 most common primary sites?
Adrenal Metastases
- Incidence: 25% at autopsy.
- Most common primary sites:
- Lung
- Small cell carcinoma:
- 90% of adrenal masses detected by CT screening represent metastases
- Nonsmall cell carcinoma:
- 60% of adrenal masses
- Small cell carcinoma:
- Breast
- Kidney
- Bowel
- Ovary
- Melanoma
- Lung
- The image shows a heterogeneous ill-defined mass larger than 4 cm.
- There is a hypo-enhancing center, which is probably the result of central necrosis.
- In this particular case a biopsy was performed and revealed an adenocarcinoma, probably from primary lung carcinoma.
- Surprisingly, extensive imaging analysis, including FDG PET-CT, did not detect a primary tumor, however.
- https://radiologyassistant.nl/abdomen/adrenals/lesion-characterization
What are the imaging features of Adrenal metastasis?
Imaging Features ( Fig. 4.26 )
Adrenal mass
- Bilateral masses
- Heterogeneous enhancement
- Indistinct, irregular margins
- SI of metastases by MRI is similar to that of spleen on T1W and T2W images.
- However, there is considerable overlap in SI between metastases and adenomas;
- the typical adenoma has an SI similar to that of adrenal tissue on T1W and T2W images.
- CT biopsy usually performed in equivocal cases.
Myelolipoma
- Rare (250 cases reported up to 1994).
- Benign tumors are composed of adipose and hematopoietic tissue. Most common in the adrenal gland but extraadrenal tumors (retroperitoneum, pelvis, liver) have been reported.
- Usually very small and discovered incidentally at autopsy.
Case courtesy of Dr Roberto Schubert, Radiopaedia.org, rID: 14164
- Adrenal myelolipomas are rare, benign and usually asymptomatic tumours of the adrenal gland characterised by the predominance of mature adipocytes.
- On imaging, they usually present as large masses with a variable amount of fat-containing components.
- Epidemiology
- Rare tumours with estimated autopsy prevalence of 0.1-0.2%. They are usually identified in adults, either incidentally or if complicated by haemorrhage (see below). There is no gender predilection 9.
- Clinical presentation
- Most lesions are asymptomatic 6 and may be discovered incidentally when the region is imaged for other reasons (i.e. an incidentaloma). Larger lesions (typically over 4 cm in size) can present with an acute retroperitoneal haemorrhage, and still others (especially when very large) with vague mass-related symptoms 9. There may be a right-sided predilection 5.
- Although the tumour itself is non-functioning there is a relatively high incidence (10%) of associated endocrine disorders 9:
- Cushing syndrome
- congenital adrenal hyperplasia (21-hydroxylase deficiency)
- Conn syndrome (primary hyperaldosteronism)
- rarely it can present in extra-adrenal locations 16
What are the 7 different types of Adrenal Cysts?
4 categories
Adrenal Cyst
Rare lesion
- Classification
- Endothelial cyst 40%
- lymphangiectatic or
- angiomatous
- Pseudocyst (hemorrhage),
- 40%; may contain calcified rim
- Epithelial cyst, 10%
- Cystic adenoma
- Retention cyst
- Cystic transformation of embryonal remnant
- Parasitic cysts (echinococcus), 5%
- Endothelial cyst 40%
Case courtesy of Dr Henry Knipe, Radiopaedia.org, rID: 51990
Large left retroperitoneal cyst, appears simple without enhancement. Internally low-density at 10 HU. Left kidney is inferiorly displaced with loss of fat plane.
Case Discussion
The patient proceeded to resection. Operative findings were of a large cyst that was attached to the lateral limb of the left adrenal gland, and it peeled away easily from the kidney. The cyst and lateral limb of the left adrenal gland was resected, and the pathological diagnosis was of an adrenal epithelial cyst.
Adrenal cysts are rare, with an estimated incidence of 0.1%. Of the adrenal cyst subtypes, epithelial cyst are uncommon, account for only 5-10% of adrenal cystic lesions.
In which patient group is this most common in?
Adrenal Hemorrhage
More common in neonates than adults
Case courtesy of Dr Derrick Chansiongpen, Radiopaedia.org, rID: 40215
Bilateral calcification in the suprarenal region consisted with adrenal calcification.
Case Discussion
Adrenal calcification is not a rare finding in asymptomatic people, and is usually the result of previous haemorrhage or tuberculosis (TB).
Case courtesy of Dr Mohammad Taghi Niknejad, Radiopaedia.org, rID: 23576
Abdominal pain and swelling right lower quadrant. hospitalised 30 years earlier. On regular steroid therapy
Adrenal TB
The CT confirms the presence of bilateral calcified and bulky adrenal glands, correlating with the abdominal x-ray findings. In the right lower quadrant, there is an abdominal wall hernia containing omental fat and small bowel. The neck is wide and there is no bowel obstruction. The hernia is just lateral to the lateral border of the right rectus abdominis muscle and is a Spigelian hernia.
Case Discussion
Previous tuberculosis infection of the adrenal glands left the patient with Addison disease and requiring lifelong steroid replacement therapy. The other main differential for such adrenal calcification is adrenal haemorrhage.
The pain and swelling were due to the Spigelian hernia. When surgery is considered in patients with Addison’s disease, close attention should be paid to post-operative steroid replacement as the stress of surgery necessitates a change in steroid dose to prevent an Addisonian crisis.
Case courtesy of Dr Vikas Shah, Radiopaedia.org, rID: 49741
What are the different types of adrenal insufficiency?
Types
- Primary (Addison disease; adrenal destruction)
- Autoimmune, idiopathic
- Infarction, hemorrhage
- Bilateral tumors: metastases
- Fungal
- Secondary (hypopituitarism)
What is Adrenal insufficiency?
Adrenal insufficiency refers to inadequate secretion of corticosteroids (glucocorticoids and mineralocorticoids).
Terminology
It may occur from partial or complete destruction of the adrenal cortex, in which case it is termed primary adrenal insufficiency (also known as Addison disease).
Secondary adrenal insufficiency due to lack of stimulation of the gland is a more common aetiology overall.
What are the imaging features of adrenal insufficiency?
Imaging Features
- May be difficult to visualize small limbs
- Evidence of previous bilateral adrenal disease:
- Metastases
- TB
- Hemorrhage
Right upper and middle lobar tree-in-bud densities with right upper lobar small nodular and patches of air space consolidation showing tiny internal cavitation, likely representing residual inflammatory (granulomatous) process.
Bilateral adrenal diffuse enlargement (larger and mass like on the left side) showing calcfications.
Case Discussion
Granulomatous Infection (Tuberculosis) is the most common infectious cause of Addison disease. CT appearance of granulomatous infection depends on the time and activity of the inflammatory process.
early-stage “adrenalitis” includes bilateral adrenal enlargement with a central necrotic area of hypoattenuation and a peripheral enhancing rim.
In the healing stage of the disease, the adrenal glands become calcified and atrophic (adrenal calcification).
Addison disease may be either acute, subacute, or chronic:
acute Addison disease occurs within a few weeks to months and is caused by bilateral adrenal haemorrhage (adrenal apoplexy), secondary to shock and sepsis or trauma. On CT scan bilateral adrenal haematomas are demonstrated.
subacute disease (adrenalitis): when the disease has been present for less than 2 years. On CT enlargement of both adrenal glands, with necrotic centres and a rim of contrast enhancement are usually demonstrated. A CT-guided biopsy helps to identify the cause such as tuberculosis, histoplasmosis, and other fungi.
chronic disease: may be caused by a chronic autoimmune disorder, chronic granulomatous infection (TB or histoplasmosis). On CT both adrenal glands appear small and atrophic with associated with calcifications in granulomatous adrenalitis.
Case courtesy of Dr Dalia Ibrahim, Radiopaedia.org, rID: 49318
What is addisons disease?
Addison’s disease, also known as primary adrenal insufficiency,[4] is a rare long-term endocrine disorder characterized by inadequate production of the steroid hormones cortisol and aldosterone by the two outer layers of the cells of the adrenal glands (adrenal cortex), causing adrenal insufficiency.[5] Symptoms generally come on slowly and insidiously and may include abdominal pain and gastrointestinal abnormalities, weakness, and weight loss.[1] Darkening of the skin in certain areas may also occur.[1] Under certain circumstances, an adrenal crisis may occur with low blood pressure, vomiting, lower back pain, and loss of consciousness.[1] Mood changes may also occur. Rapid onset of symptoms indicates acute adrenal failure which is a serious and emergent condition.[5] An adrenal crisis can be triggered by stress, such as from an injury, surgery, or infection.[1]
Addison’s disease arises from problems with the adrenal gland such that not enough of the steroid hormone cortisol and possibly aldosterone are produced.[1] In developed countries, the etiology of Addison’s disease is often attributed to idiopathic damage by the body’s own immune system, and in developing countries most often due to tuberculosis.[6][needs update] Other causes include certain medications, sepsis, and bleeding into both adrenal glands.[1][6] Secondary adrenal insufficiency is caused by not enough adrenocorticotropic hormone (ACTH) (produced by the pituitary gland) or corticotropin-releasing hormone (CRH) (produced by the hypothalamus).[1] Despite this distinction, adrenal crises can happen in all forms of adrenal insufficiency.[1] Addison’s disease is generally diagnosed by blood tests, urine tests, and medical imaging.[1]
Addison’s disease can be described in association with chronic mucocutaneous candidiasis, acquired hypoparathyroidism, diabetes mellitus, pernicious anemia, hypogonadism, chronic and active hepatitis, malabsorption, immunoglobulin abnormalities, alopecia, vitiligo, spontaneous myxedema, Graves’ disease, and chronic lymphocytic thyroiditis.[7]
Treatment involves replacing the absent hormones.[1] This involves taking a synthetic corticosteroid, such as hydrocortisone or fludrocortisone.[1][2] These medications are usually taken by mouth.[1] Lifelong, continuous steroid replacement therapy is required, with regular follow-up treatment and monitoring for other health problems.[8] A high-salt diet may also be useful in some people.[1] If symptoms worsen, an injection of corticosteroid is recommended and people should carry a dose with them.[1] Often, large amounts of intravenous fluids with the sugar dextrose are also required.[1] Without treatment, an adrenal crisis can result in death.[1]
Addison’s disease affects about 0.9 to 1.4 per 10,000 people in the developed world.[1][3] It occurs most frequently in middle-aged females.[1] Secondary adrenal insufficiency is more prevalent.[3] Long-term outcomes with treatment are typically favorable.[9] It is named after Thomas Addison, a graduate of the University of Edinburgh Medical School, who first described the condition in 1855.[10] The adjective “addisonian” is used to describe features of the condition, as well as people with Addison’s disease.[11]
