Adrenal Presentation

Benign and Malignant Disease
of Adrenal and Parathyroid

Gland.
Presenter-:Dr.Saleamlak D.( GSR-III)
Moderator : Dr.Chuchu ( consultant Gen.Surgery)
Invited Guest : Dr.Engida (consultant Endocrine and Gen.Surgeon at TASH)
Saleamlak D 12/09/2022 1
Outline
• Brief Embryology, Anatomy and Physiology of Adrenal gland.
• Cushing’s syndrome.
• Adrenocortical ca.
• Incidentaloma.
• Pheochromocytoma.
• parathyroid gland embryology, anatomy and physiology
• Hyperparathyroidism
Objectives
• To understand the differential diagnoses of common adrenal diseases.
• Different diagnostic & management algorithms to each disease entity.
• Role of surgery in such scenarios.
• To have insight on primary hyperparathyroidism.
• Role of surgery in treating patients with PHPT .
Embryology
• The adrenal glands are composed of two functionally distinct endocrine units.
• The cortex originates around the 5th week of gestation from mesodermal tissue near the
gonads.
• Medulla is ectodermal in origin and arises from the neural crest cells.
Anatomy
• Paired retroperitoneal organs located at the level of
11 th rib.
 Weigh 4-5g each.
 Arterial supply
• Superior adrenal aa- from inf phrenic aa
• Middle adrenal aa- from abd aorta
• Inferior adrenal aa- from renal aa
 Venous return
• Rt- short & drains directly to IVC
• Lt- into Lt renal vv
Physiology
Cushing’s Syndrome.
• Is constellation of symptoms and signs resulting from
excess of cortisol production regardless of the cause.
• Manifestations range from subclinical, cyclical, or
mild to rapid-onset severe variants.
• It could result from endogenous or exogenous

administration of corticosteroids which is the most
common cause of the syndrome.
• Among endogenous causes, they are classified as

ACTH independent and ACTH dependent.
Cushing’s Disease.
• refers to a corticotrophin secreting pituitary tumor, usually an adenoma or
microadenoma , which leads to bilateral adrenal hyperplasia and
hypercortisolism.
• Rare disease (10/1mil)
• M:F ratio is 1:8
• Most are sporadic.
Clinical Presentation
Diagnosis
• First step is to screen patients suspected of having Cushing's syndrome.
• Followed by determination of etiology.
SCREENING
• Overnight dexamethasone suppression test
• 48 hr./low-dose dexamethasone suppression test
• Late-night salivary cortisol measurement
• Urinary cortisol
• MEDICAL
• For perioperative control of hypercortisolism or
• When surgery is not feasible
• Metyrapone, ketoconazole, and mitotane
• SURGERY
• Pituitary adenoma- TSS, *bilateral laparoscopic adrenalectomy
• Ectopic ACtH secreting tumors-resection, *medical therapy or *bilateral adrenalectomy.
• Adrenal causes- adrenalectomy (unilateral or bilateral; open or laparoscopic)
Adrenocortical cancer
•Rare neoplasms (2 per 1 million in the world)
•Bimodal age distribution.( common in children and adults between 4th and 5th decade of life )
•Majority are sporadic in nature.
•~ 50% of adrenocortical cancers are nonfunctioning
•Functional tumors secrete cortisol (30%), androgens (20%), estrogens (10%), aldosterone (2%);
or multiple hormones (35%).
•Most tumors weight 100 to 1000gm.

• Functioning tumors are usually presented with rapid onsetof Cushing’s
syndrome accompanied by virilizing feature.
• For non functioning tumors abdominal pain with enlarging abdominal
mass and back pain are commonest fetaures.
• Diagnosis initially work up for common adrenal disease ( serum
electolyte, urinary catecholamines, DST , 24 hour urine cortisol and 17
keto steroids.)
•
• CT and MRI
• The size of adrenal mass on imaging studies is the single most important
criteria for dx.
• Adrenal masses >6cm were AC in 92%.
• Upto 70% of patients present with stage III or IV disease.
• Staging uses the TNM classification.
•Pathologic criteria for predicting mets or chance of
recurrence SURGERY
• nuclear grade III or IV; Open adrenalectomy is the procedure of choice
• mitotic rate greater than 5 per 50 high-power fields;
R0 resection must be the goal
• atypical mitoses;
Enbloc resection with contiguous structures
• clear cells comprising 25% or less of the tumor;
Surgical debulking
• a diffuse architecture;
• microscopic necrosis;
CHEMOTHERAPY
• invasion of venous, sinusoidal, and capsular structure Etoposide, cisplatin, doxorubicin, paclitaxel +_
mitotane
Tumors with four or more of these criteria were likely
RADIOTHERAPY –incomplete resection,
to metastasize and/or recur palliation of bone mets
Disorders of Adrenal Medulla.
• Pheochromocytoma
• arise from medullary cells of adrenal.
• Rare tumors ..
• Can occur at any age peaks in 4th and 5th decade.
• No gender predilection.
• 10% tumors.
• MEN2A, MEN2B, VHL disease, NF1
• The symptoms and signs are due to excess concentration of circulatory
catecholamines .
• The classic triad is headache, sweating and palpitation accompanied

by HTN .
• Majority has sustained or paroxysmal hypertension.

Clinical features
• Patients could be presented for anxiety, tremulousness, paresthesia,
flushing, chest pain, shortness of breath abdominal pain, nausea, vomiting,
CV complications, like arrhythmia, tachycardia, MI.
Diagnosis
• The first and very important is clinical suspicion after taking history and physical
examination.
• 24 hr. urine metanephrines and fractionated catecholamines.
• Plasma free metanephrines.
• Localization
• CT of abdomen & Pelvis-heterogeneous, central necrosis
• MRI- hyper intense ‘bright white’ signal on T2
• MIBG scan- increased uptake by chromaffin cells but no metabolism. Mainly for localzing.
Optimization
• Alpha blockage- phenoxybenzamine ( selective long acting alpha blockers, should be
accompanied by adequate hydration.
• For a minimum of 1-3 wks
• Orthostatic hypotension & ‘stuffy nose’
• Ca2+ channel blockers- nicardipine
• Volume expansion- fluids, high salt diet
• Beta blocker- in patients with tachycardia and arrhythmia after starting alpha blockers.
• The goal of surgery is to excise the tumor wit minimal tumor manipulation or rupture of
tumor capsule.
During operation
• The most volatile BP swings occur with induction of anesthesia and
manipulation of tumor.
• If arrhythmia occur, stop manipulation
• If profound hypotension following excision ephedrine and phenylephrine
preferred.
• Excellent communication should be maintained b/n the surgeon &
anesthesiologist.
• RX- Adrenalectomy
Special population
• PCC in pregnancy • PCC in children
• Suspect dx with new onset HTN • HTN is more often sustained
without preeclampsia. • Tendency to be bilateral & extra-
• MRI is preferred imaging adrenal
modality. • Malignancy is less frequent
• Control BP and optimize, surgery
in second TM and delivery via CS
2 to 6 weeks later.
Malignant pheochromocytoma.
• Malignancy is usually diagnosed with evidence of invasion into surrounding
structures or distant metastases.
• Mets: to bone, liver, regional lymph nodes, lung, and peritoneum
• Treatment
 Resection if feasible
 External-beam radiation for un resectable lesions
 Therapeutic 131 I-MIBG irradiation
 Chemotherapy
Incidentaloma
• These are adrenal lesions discovered during imaging performed for
unrelated reasons.
• The incidence of these lesions identified on CT scan ranges from 0.4-4.4
%.
• Its differentials could be divided in to functioning and non functioning
lesions.
Diagnosis
• The diagnostic workup is mainly aimed at identifying patients who would
benefit from adrenalectomy :
• Patient with functioning tumors.
• Tumors at increased risk of being malignant
Screening for subclinical disease
• Subclinical Cushing’s Syndrome
• Ranges from slightly attenuated diurnal cortisol rhythm to atrophy of the contralateral gland
• Do dexamethasone suppression test
• Subclinical Pheochromocytoma
• Refers to totally asymptomatic adrenal incidentaloma that histologically proves to be
pheochromocytoma
• 24 hr urine metanephrine & VMA, or fractionated urinary catecholamine
• Subclinical Primary Aldosteronism
• Pt with adrenaloma who is normotensive or hypertensive with normokalemia.
• Imaging's play a role in both identifying and characterizing lesions.
• Risk of being malignant is related to size of a lesion.
• Other features like, texture, encapsulation, marginal status and degree of
attenuation and presence of LAP or mets are also important.
• FNAB
Parathyroid Gland.
• In humans, the superior parathyroid glands are derived from the 4th
branchial pouch, which also gives rise to the thyroid gland.
• The 3rd branchial pouches give rise to the inferior parathyroid glands
and the thymus.
• Most patients have 4 parathyroid glands (2-8 glands reported)
• 4 glands in 84% of cases, supernumerary glands in 13%, and 3% have less than 4
glands
• Normal parathyroid glands are ovoid in shape and gray and
semitransparent in newborns but appear golden yellow to light brown in
adults.
• They measure up to 7 mm in size and weigh approximately 40 to 50 mg
each.
• Superior glands
• 80% of these glands are found near the
posterior aspect of the upper and middle
thyroid lobes, at the level of the cricoid
cartilage
• They usually are dorsal to the RLN at the
level of the cricoid cartilage (Usually 1 to
2cm cephalad to the junction of the RLN
with ITA)
• Inferior glands
• The most common location is within a
distance of 1 cm from a point centered
where the inferior thyroid artery and RLN
cross
• The position of the inferior glands,
however, tends to be more variable due to
their longer migratory path
• Undescended inferior glands may be found
near the skull base, angle of the mandible, or
superior to the upper parathyroid glands
along with an undescended thymus
• Parathyroid glands usually derive their blood supply from branches of the inferior
thyroid artery, although branches from the superior thyroid artery supply at least
20% of upper glands.
• The venous drainage if from ipsilateral superior, middle, and inferior thyroid veins.
• Histologically, parathyroid glands are composed of chief cells (produce
parathyroid hormone) and oxyphil cells arranged in trabeculae, within a stroma
composed primarily of adipose cells
• A third group of cells, known as water-clear cells, are derived from chief cells and present in
small numbers
• Extra cellular calcium is under tight
control of PTH hormone principally.
• Its released in to circulation when there
is decreased calcium level.
• Bone- enhance osteoclast activity and
bone resorption.
• Kidney- decrease excretion at DCT and
activate hydroxylation of 25 OH vit.D.
• Gi- increase calcium and phosphate
reabsorption via 1,25OH vit.D.
Hyperparathyroidism
• Hyperfunction of the parathyroid glands may be classified as primary,
secondary, or tertiary.
• 10HPT- abnormally elevated PTH from the PTH gland and result from
disturbance of normal feedback control exerted by serum ca ++.
• 2nd HPT- elevated PTH level due to compensatory response to hypocalcemia
states resulting from CRF or impaired GI ca ++ absorption.
• 3ryHPT- chronically stimulated gland may become autonomous resulting in
persistence of hypercalcemia after successful renal transplantation.
PHPT
• Incidence 0.1 to 0.3 % in general population.
• Exceedingly common in women.
• Exact etiology is unknown.
• Results in hypercalcemia from increased bone resorption, increased
absorption from the gi tract and decreased renal excretion.
• Familial history posses a greater risk .
• Have classic pentad presentation involving bone , gi, neuromuscular, and
renal disease.
• Currently most patients present with weakness, fatigue, polydipsia,
polyuria, nocturia, bone and joint pain, constipation, decreased appetite,
nausea , heart bur, depression and memory loss.
• Physical findings, PTH glands are seldom palpable.
• Diagnosis is ascertained by elevated calcium level with intact PTH.
• Normocalcemic PHPT..
• Radiologic testes ..
• Treatment
• Medical – asymptomatic PHPT – ( bisphosphonates, HRT, SERM)
• Surgical-patients with classic symptoms and in those that could develop
complications.
References
• Schwartz principle of Surgery 11th edition.
• Textbook of Endocrine Surgery 3rd ed.
• Feleke Y, Abdulkadir J, Johnson O, Munie T. Cushing's syndrome: a ten year experience at Tikur Anbassa
Hospital. Ethiop Med J. 1998 Jan;36(1):19-26. PMID: 10214444.
• Libé R. Adrenocortical carcinoma (ACC): diagnosis, prognosis, and treatment. Front Cell Dev Biol. 2015 Jul
3;3:45. doi: 10.3389/fcell.2015.00045. PMID: 26191527; PMCID: PMC4490795.
• Johnson, O. “Phaeochromocytoma: Experience with 12 cases in Tikur Anbessa, AddisAbaba,
Ethiopia.” East and Central African Journal of Surgery 9 (2004): n. pag.
• Mengesha RE, Rother RB. MANAGEMENT OF PHEOCHROMOCYTOMA IN THE ABSENCE OF ALPHA
BLOKERS: A CASE REPORT FROM AYDER REFERRAL HOSPITAL. Ethiop Med J. 2015 Jul;53(3):151-
4. PMID: 26677525

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Benign and Malignant Disease

of Adrenal and Parathyroid

• It could result from endogenous or exogenous

• Among endogenous causes, they are classified as

•Most tumors weight 100 to 1000gm.

• The classic triad is headache, sweating and palpitation accompanied

• Majority has sustained or paroxysmal hypertension.

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