NEOPLASMS OF ADRENAL

GLAND – clinical features,

investigations and treatment

- Md. Tanveer Adil

 TYPES

 ADRENAL CORTEX
i) Benign – Adrenal Adenoma
Adrenal Hyperplasia

ii) Malignant – Adrenocortical cancer

 TYPES…

 ADRENAL MEDULLA
i) Adult – Phaeochromocytoma
Ganglioneuroma
ii) Children – Neuroblastoma

 ADRENAL INCIDENTALOMA
 ADRENAL METASTASIS
 FRAMEWORK FOR EVALUATING
ADRENAL NEOPLASMS

 Is it functional?

 Is it malignant?

 If malignant: is it primary or metastatic

disease?
 If the answers to the above are “no”:
observe or resect?
 IF PATIENT FIT FOR SURGERY

 If functional: operative removal.

 If a primary carcinoma: operative

removal.
 If metastatic: percutaneous biopsy if
primary unknown.
 Small and nonfunctioning: observation
if possible.
 History and Physical
 Evidence of function?
 Hyperaldosteronism: universally present
with hypertension (Ross et al., NEJM
1992).
Other complaints: muscle weakness,
polydipsia, polynocturia
 Pheochromocytomas 60% present with
hypertension. Other complaints: flushing,
headache, diaphoresis, palpitations, etc.
 History and Physical
 Evidence of function?...
 Virilization: hirsutism, irregular

menses, scalp alopecia,

macrogenitosomia praecox

 Feminization: impotence, testicular

atrophy, bilateral gynecomastia,
early menses, breast enlargement
 History and Physical
 Evidence of function?...
 Hypercortisolism: Easy bruising, striae,
myopathy, buffalo hump, hirsutism, or moon
facies.
 Adrenal cancer. Up to 60% may be
hormonally active (Cook, Endocrine and
Metabolism Clinics, 1997).
 Other associations, e.g., diuretics, steroid use.
Evaluate for renal artery stenosis, cirrhosis if
appropriate.
 History and Physical
(continued)
 Evidence of malignancy?
 History of previous cancer?
 In patients with pre-existing cancer, 32-73% of
adrenal masses represent metastatic disease.
 Familial syndromes: Pheochromocytoma plus

 Medullary thyroid cancer (MEN II)

 Neurofibromatosis/café-au-lait (von Recklinghausen)

 Retinal angiomas (von Hippel-Landau)

 Cachexia, supraclavicular nodes, abdominal mass?

 History and Physical

Screening evaluations
 HYPERCORTISOLISM –
Single Dose Dexamethasone Suppression
Test
24 hour free cortisol and Plasma ACTH

HYPERALDOSTERONISM –
Upright PAC:PRA >20 with PAC > 15
ng/dL
 History and Physical

Screening evaluations
 PHAEOCHROMOCYTOMA –
Plasma fractionated metanephrines
Urinary catecholamines and metanephrines
Urinary VMA levels>1.6 mg/24 hrs
 VIRILIZING ADRENAL TUMOURS –
Serum testosterone, DHEA
5 day Dexamethasone Suppression Test
 Adrenal Cortical Adenoma
 Benign neoplasm of adrenal cortical cells, usually non-
functional
 May be functional usually producing hypercortisolism or
hyperaldosteronism; adrenogenital syndromes are rare
 Most adenomas do not exceed 100 grams in weight or 5 cm in
size
 Can usually be removed laparoscopically
 While glucocorticoid replacement is necessary after surgery,
mineralocorticoid replacement is not usually needed.
 Histologically benign lesions that are larger than 50-100grams
need to be followed to exclude carcinoma.
Adrenal Cortical Adenoma

capsule cortex
 Adrenal Adenoma
 Non functional tumours are usually
detected
incidentally(INCIDENTALOMA)

 Functional tumours based on signs and

symptoms
 Adrenal Incidentaloma
 DEFINITION

Any adrenal mass discovered by a

noninvasive imaging technique
performed for a reason other than any
suspected adrenal disease.
Differential Diagnosis of Adrenal Incidentalomas
 Benign nonfunctioning  Malignant nonfunctioning
Adenoma(MOST COMMON)
Adrenolipoma Angiosarcoma
Amyloidosis Ganglioneuroblastoma
Cyst / Pseudocyst Ganglioneuroma Leiomyosarcoma Malignant
Granuloma
Infection schwannoma Metastatic
Hamartoma Hemangioma carcinoma Malignant
Leiomyoma melanoma
Lipoma Myelolipoma Malignant lymphoma
Neurofibroma
Schwannoma (Neurilemmoma) Adrenocortical carcinoma
Teratoma

Endocrine and Metabolism Clinics

of North America 2000; 29(1):159-185
Differential Diagnosis of Adrenal Incidentalomas
 Hyperfunctioning mass  Pseudoadrenal mass

- Pheochromocytoma
- Preclinical Cushing’s - Mistaken vasculature
syndrome - Liver
- Primary aldosteronism
- Nodular hyperplasia - Lymph nodes
- Congenital adrenal - Pancreatic mass
hyperplasia - Spleen
- Masculinizing or
feminizing tumor - Renal mass
- Primary malignancy - Stomach mass
(Adrenocortical - Technical artifact
carcinoma)

Endocrine and Metabolism Clinics

of North America 2000; 29(1):159-185
 Adrenal incidentaloma(>1cm)

Assessment of biochemical function

Plasma fractionated metanephrines If +,plasma ACTH and

Single dose dexamethasone test 24hr urine-free cortisol
Upright PAC:PRA

Functioning mass Nonfunctioning mass

Laparoscopic adrenalectomy
Radiographic evaluation (CT/MRI)

Large size (>4-5 cm) Benign appearance Suspected adrenal

Atypical appearance (4-5 cm) metastasis

Adrenalectomy Surgical candidate

Repeat CT/MRI at 3&12 mth YES NO
Functional reassessment at 12 & 24 mth

Hypersecretory Adrenalectomy Consider

Enlarging FNA
ADRENAL ADENOMA
 History and Physical

Screening Evaluation

Nonfunctional lesion

Radiologic evaluation
 Radiologic Evaluation
CT MRI

May underestimate size by 20% Accurate size determination

Lipoma: -50 to 150 HU T1 lesions dark

Cysts: -10 to -20 HU T2: Mets, cancer, pheos light up

Adenomas: ~30 HU Mass/liver T2 intensity ratio

Malignancy > 30 HU Pheochromocytoma > 3

Superior for distinguishing Malignancy 1.4-3

hemangiomas hematomas cysts Adenomas .7-1.3

Cook, DM. Endocrinology and Metabolism Clinics 26(4):829-852, December 1997
 Radiologic Evaluation (continued)

 (131I-6-iodomethylnorcholesterol) scanning reportedly

100% specific and 91% sensitive.

 It can distinguish bilateral adrenal hyperplasia from

adenoma, while carcinomas do not take up the tracer at all.
It may also be useful in finding ectopic sources of ACTH.

 The radioactive tracers are also taken up by the adrenals

and gonads.
 ADRENAL ADENOMA

Does Size Matter ???

 The Issue of Size

 Larger size carries greater risk of cancer

 >1 cm: usually benign.

 >6 cm: as many as 15 % of masses represent carcinoma

 >8 cm: as many as 25% of masses represent carcinoma.

 Surgery currently recommended at 4-5 cm.

 Size does not correlate to risk of distant metastases!

 History and Physical

Screening Evaluations

Nonfunctional
lesion

Radiologic evaluation

Benign

Observe
 History and Physical

Screening Evaluations

Observation
Nonfunctional  Reports of “subclinical”
lesion endocrine disease increasing:
must include BIOCHEMICAL
Radiologic evaluation SCREENING.
 87 patients followed
sequentially by CT for 4 years.
Benign:  83 evidenced no change
Observe?  4 grew by 1cm or more. All were
benign on resection.
Barry MK et al, World J Surg 1998;22:599-604.
 History and Physical

Screening Evaluations

Nonfunctional lesion

Radiologic evaluation

Benign:
Observe?
Equivocal: Malignancy
discuss with
patient
 Adrenal Cortical Carcinoma

 Rare malignancy accounting for 0.05-0.2% of all cancers

 70% of adults present with hormonal syndromes.
 Women typically have functional adrenal carcinomas
 Bimodal age distribution at 5 years and in the 4th and 5th decade
 Usually greater than 6cm and 100-5000g
 Distinction from renal tumors can be difficult at times; special
staining may be helpful.
 Histologic distinction from adenoma may be difficult; must be
based on nodal and distant metastases
Adrenal Cortical Carcinoma

ACC

Kidney
Size Does Matter !!!
 Adrenal Cortical Carcinoma
Diagnosis

Presenting signs and symptoms in 52 patients with adrenocortical carcinoma

Feature No. of Patients (%)

Cushing’s syndrome 25(48)
Virilization and hypertension 8 (15)
Virilization 2 (4)
Gynecomastia 1 (2)
Myasthenia 1 (2)
Pathologic fractures 2 (4)
Acromegaly 1 (2)
Abdominal pain 4 (8)
Lumbar pain 4 (8)
Weight loss, weakness 3 (6)
Abdominal mass 1 (2)
 Adrenal Cortical Carcinoma
Diagnosing Malignancy

Likelihood of Malignancy Clinical Criteria Pathologic Criteria

Diagnostic of Malignancy Weight loss, feminization, Tumor >100g, tumor necrosis,
nodal/distant mets vascular invasion

Consistent with Malignancy Virilism Nuclear pleomorphism,

aneuploidy

Suggestive Of Malignancy Elevated urinary Capsular invasion

17 ketosteroids

Unreliable Hypercortisolism, Giant cells,

hyperaldosteronism cytoplasmic size
Am J Surg Path 1989; 13:
 Adrenal Cortical Carcinoma TNM
Staging System
Stage Criteria

Tumor
T1 Tumor <5 cm, no invasion
T2 Tumor >5 cm, no invasion
T3 Tumor outside adrenal in fat
T4 Tumor invading adjacent organs
Lymph Nodes
N0 No positive lymph nodes
N1 Positive lymph nodes
Metastases
M0 No distant metastases
M1 Distant metastases

Stage Grouping
I T1N0M0
II T2N0M0
III T1-2N1M0; T3N0M0
IV Any T, any N, M1; T3N1 or T4
 History & Physical

Screening Evaluation

Nonfunctional Functional
lesion lesion(adenoma or
carcinoma)
Radiologic evaluation Surgery
(Unilateral
Adrenalectomy)
Benign:
Observe?
Equivocal: Malignancy Primary adrenal
discuss with cancer
patient
 Adrenal Cortical Carcinoma
Treatment-Surgery
 Surgery is primary treatment modality removing all tumor
tissue and any involved contiguous structures.
 Surgery offers the only chance of cure.
 Luton et al, 1990; of 105 patients 80 were resectable. Median
disease free interval 12 months. Overall 5 year survival 22%.
 Debulking should be carried out if complete resection cannot
be achieved.
 Recurrences can be managed surgically
 XRT can be used to treat local recurrence and bony metastases
although survival is unaffected.
 Adrenal Cortical Carcinoma
Treatment-Chemotherapy
 Chemotherapy is usually used in the setting of recurrence or
metastases.

 Mitotane is the primary agent used

 Typically not used in the adjuvant setting

 Blood levels should be at least 14 ug/mL

 Neuromuscular toxicity develops at levels >20 ug/mL

 Agents with partial success include doxorubicin, cisplatin

and etoposide
 Pheochromocytoma
1. Epidemiology
2. Signs & Symptoms
3. Diagnosis
1. Biochemical
2. Localization
4. Management
1. Preoperative
2. Operative
3. Postoperative
4. Pregnancy
 Pheochromocytoma
1. Epidemiology
2. Signs & Symptoms
3. Diagnosis
1. Biochemical
2. Localization
4. Management
1. Preoperative
2. Operative
3. Postoperative
4. Pregnancy
 Pheochromocytoma
 0.01-0.1% of HTN population
 Found in 0.5% of those screened
 M=F
 3rd to 5th decades of life
 Rare, investigate only if clinically suspicion:
 Signs or Symptoms
 Severe HTN, HTN crisis
 Refractory HTN (> 3 drugs)
 HTN present @ age < 20 or > 50 ?
 Adrenal lesion found on imaging (Incidentaloma)
 Pheo: Signs & Symptoms
 The five P’s:
 Pressure (HTN) 90%
 Pain (Headache) 80%
 Perspiration
 Palpitation 64%
 Pallor 42%
 Paroxysms (the sixth P!)
 The Classical Triad:
 Pain (Headache), Perspiration, Palpitations
 Lack of all 3 virtually excluded diagnosis of pheo in a
series of > 21,0000 patients
 Pheo: Paroxysms, ‘Spells’
 10-60 min duration
 Frequency: daily to monthly
 Spontaneous
 Precipitated:
 Diagnostic procedures, I.A. Contrast (I.V. is OK)
 Drugs (opiods, unopposed -blockade, anesthesia
induction, histamine, ACTH, glucagon,
metoclopramide)
 Strenuous exercise, movement that increases intra-abdo
pressure (lifting, straining)
 Micturition (bladder paraganlgioma)
 Pheo: Hypotension!
 Hypotension (orthostatic/paroxysmal)
occurs in many patients
 Mechanisms:
 ECFv contraction
 Loss of postural reflexes due to prolonged
catecholamine stimulation
 Tumor release of adrenomedullin (vasodilatory
neuropeptide)
 Pheo: Signs & Symptoms
 N/V, abdo pain, severe constipation (megacolon)
 Chest-pains
 Anxiety
 Angina/MI with normal coronaries:
 Catecholamine induced:  myocardial oxygen
consumption or coronary vasospasm
 CHF
 HTN  hypertrophic cardiomyopathy  diastolic dysfn.
 Catechols induce dilated cardiomyopathy  systolic dysfn.

 Cardiac dysrhythmia & conduction defects

 Pheo: Signs (metabolic)
 Hypercalcemia
 AssociatedMEN2 HPT
 PTHrP secretion by pheo

 Mild glucose intolerance

 Lipolysis
 Weight-loss

 Ketosis > VLDL synthesis (TG)

 Pheo: ‘Rule of 10’
 10% extra-adrenal (closer to 15%) – Organ of
Zuckerkandl, chemodectoma, glomus jugalare
 10% occur in children
 10% familial (closer to 20%)
 10% bilateral or multiple (more if familial)
 10% recur (more if extra-adrenal)
 10% malignant
 10% discovered incidentally
 Familial Pheo
 MEN 2a
 50% Pheo (usually bilateral), MTC, HPT
 MEN 2b
 50% Pheo (usually bilatl), MTC, mucosal neuroma, marfanoid
habitus
 Von Hippel-Landau
 50% Pheo (usually bilat), retinoblastoma, cerebellar
hemangioma, nephroma, renal/pancreas cysts
 NF1 (Von Recklinghausen's)
 2% Pheo (50% if NF-1 and HTN)
 Café-au-lait spots, neurofibroma, optic glioma
 Familial paraganglioma
 Familial pheo & islet cell tumor
 Other: Tuberous sclerosis, Sturge-Weber, ataxia-telangectgasia,
Carney’s Triad (Pheo, Gastric Leiomyoma, Pulm chondroma)
 Pheochromocytoma
1. Epidemiology
2. Signs & Symptoms
3. Diagnosis
1. Biochemical
2. Localization
4. Management
1. Preoperative
2. Operative
3. Postoperative
4. Pregnancy
 24h Urine Collection
 24h urine collection:
 Creatinine,
catecholamines, metanephrines,
vanillymandelic acid (VMA), +/-dopamine
 HPLC with electrochemical detection or mass spect

 Positive results (> 2-3 fold elevation):

 24h Ucatechols > 2-fold elevation
 ULN for total catechols 591-890 nmol/d
 24h Utotal metanephrines > 1.2 ug/d (6.5 umol/d)
 24h UVMA > 3-fold elevation
 ULN 35 umol/d for most assays
 24h Urine Collection
 Test Characteristics:
 24h Ucatechols Sen 83% Spec 88%
 24h Utotal metanephrines Sen 76% Spec 94%
 24h Ucatechols + Utotal metanephrines Sen 90% Spec 98%

 24h UVMA Sen 63% Spec 94%

 Sensitivity increased if 24h urine collection
begun at onset of a paroxysm
 24h Urine: False Positive
 Drugs: TCAs, MAO-i, levodopa, methyldopa,
labetalol, propanolol, clonidine (withdrawal),
ilicit drugs (opiods, amphetamines, cocaine),
ethanol, sympathomimetics (cold remedies)
 Hold these medications for 2 weeks!
 Major physical stress (hypoglycemia, stroke,
raised ICP, etc.)
 Overstrenous activity
 Plasma Catecholamines
 Drawn with patient fasting, supine, with an
indwelling catheter in place > 30 min
 Plasma total catechols > 11.8 nM (2000 pg/mL)
 SEN 85% SPEC 80%
 False positives: same as for 24h urine testing, also
with diuretics, smoking
 CRF & ESRD:
 Oliguric to Anuric  24h Urines inaccurate
 Plasma epinephrine best test for pheo in ESRD
 Plasma norepi and metanephrines falsely elevated in ESRD
 Plasma Metanephrines
 Not postural dependent: can draw normally

 Secreted continuously by pheo

 SEN 99% SPEC 89%

 False Positive: acetaminophen

 MOST SENSITIVE INVESTIGATION

 Biochemical Tests: Summary
SEN SPEC
Ucatechols 83% 88%
Utotal metanephrines 76% 94%
Ucatechols+metaneph 90% 98%
UVMA 63% 94%
Plasma catecholamines 85% 80%
Plasma metanephrines 99% 89%
 Suppression/Stimulation Testing
 Clonidine suppression
 May precipitate hypotensive shock!
 Unlike normals, pheo patients won’t suppress
their plasma norepi with clonidine
 Glucagon stimulation
 May precipitate hypertensive crisis!
 Pheo patients, but not normals, will have a > 3x
increase in plasma norepi with glucagon
 Localization: Imaging
 CT abdomen
 Adrenal pheo SEN 93-100%
 Extra-adrenal pheo SEN 90%

 MRI
 T2 weighted brightness 3 times the liver is
highly specific
 > SEN than CT for extra-adrenal pheo
Low attenuation areas within the mass indicate cystic areas
 Localization: Imaging
 CT abdomen
 Adrenal pheo SEN 93-100%
 Extra-adrenal pheo SEN 90%

 MRI
 T2 weighted brightness 3 times the liver
 > SEN than CT for extra-adrenal pheo

 MIBG Scan
 SEN 77-90% SPEC 95-100%
 MIBG Scan
 123I or 131I labelled metaiodobenzylguanidine

 MIBG catecholamine precurosr taken up by the tumor

 Inject MIBG, scan @ 24h, 48h, 72h

 Lugol’s 1 gtt tid x 9d (from 2d prior until 7d after MIBG

injection to protect thyroid)

 False negative scan:

 Drugs: Labetalol, reserpine, TCAs, phenothiazines
 Must hold these medications for 4-6 wk prior to scan
 Localization: Nuclear medicine
 MIBG

 111Indium-pentreotide
 Some pheo have somatostatin receptors

 PET
 18F-fluorodeoxyglucose (FDG)
 6-[18F]-fluorodopamine
 Pheochromocytoma
1. Epidemiology
2. Signs & Symptoms
3. Diagnosis
1. Biochemical
2. Localization
4. Management
1. Preoperative
2. Operative
3. Postoperative
4. Pregnancy
 Pheo Management
 Prior to 1951, reported mortality for excision of
pheochromoyctoma 24 - 50 %
 HTN crisis, arrhythmia, MI, stroke
 Hypotensive shock

 Currently, mortality: 0 - 2.7 %

 Preoperative preperation, -blockade?
 New anesthetic techniques?
 Anesthetic agents
 Intraoperative monitoring: arterial line, EKG monitor,
CVP line, Swan-Ganz
 Experienced & Coordinated team:
 Endocrinologist, Anesthesiologist and Surgeon
 Preop W/up
 CBC, lytes, creatinine, INR/PTT
 CXR
 EKG
 Echo (r/o dilated CMY 2º catechols)
 Preop Preperation Regimens
 Combined  +  blockade
 Phenoxybenzamine
 Selective 1-blocker (ex. Prazosin)
 Propanolol
 Metyrosine
 Calcium Channel Blocker (CCB)
 Nicardipine

 No Randomized Clinical Trials to compare various

regimens!
 Preop:  +  blockade
 Start at least 10-14d preop
 Allow sufficient time for ECV re-expansion

 Phenoxybenzamine
 Drug of choice
 Covalently binds -receptors (1 > 2)

 Start 10 mg po bid  increase q2d by 10-20 mg/d

 Increase until BP cntrl and no more paroxysms

 Maintenance 40-80 mg/d (some need > 200 mg/d)

 Salt load: NaCl 600 mg od-tid as tolerated

 Preop:  +  blockade
 Phenoxybenzamine (cont’d)
 Side-effect: orthostasis with dosage required to normalized
seated BP, reflex tachycardia
 Drawback: periop hypotension/shock unlikely to respond to
pressor agent.
 Selective 1-blockers
 Prazosin, Terazosin, Doxazosin
 Some experience with Prazosin for Pheo preop prep
 Not routinely used as incomplete -blockade
 Less orthostasis & reflex tachycardia then phenoxybenzamine
 Used more for long-term Rx (inoperable or malignant pheo)
 Preop:  +  blockade
 -blockade
 Used to control reflex tachycardia and
prophylaxis against arrhythmia during surgery
 Start only after effective -blockade (may ppt
HTN)
 If suspect CHF/dilated CMY  start low dose

 Propanolol most studied in pheo prep

 Start 10 mg po bid  increase to cntrl

HR
 Preop:  +  blockade
 If BP still not controlled despite  +  blockade
 Add Prazosin to Phenoxybenzamine

 Add CCB, ACE-I

 Avoid diuretics as already ECFv contracted

 Metyrosine
 Preop:  +  blockade
 Meds continued on morning of surgery
 IV fluids 24 hrs prior to surgery
 Periop HTN:
 IV phentolamine
 Short acting non-selective -blocker

 Test dose 1 mg, then 2-5 mg IV q1-2h

 IV Nitroprusside (NTP)

 Periop arrhythmia: IV esmolol

 Periop Hypotension: IV crystalloid +/- colloid
 Pheo: Rx of HTN Crisis
 IV phentolamine

 IV NTP

 IV esmolol

 IV labetalol – combined  +  blocker

 O.R.
 Admit night before for overnight IV saline
 Arterial line, EKG monitor, CVP line
 Known CHF: consider Swan-Ganz
 Regardless of preop medications:
 Have ready: IV phentolamine, IV NTP, IV esmolol
 Rx hypotension with crystalloid +/- colloid 1st

 Aim for CVP 12 or Wedge 15

 Inotropes may not work!

 O.R.
 Anesthetic choice:
 Enflurane or isoflurane: don’t sensitized myocardium to
catecholamines
 Halothane: may sensitize heart  arrhythmia

 Laprascopic adrenalectomy if tumor < 8cm

 Open adrenalectomy if tumour > 8 cm or
suspicion of malignancy
 Postop
 Postop vitals and urine output measurement
 Most cases can stop all BP meds postop
 Postop hypotension: IV crystalloid
 HTN free: 5 years 74% 10 years 45%

 24h urine collection 2 wks postop for

metabolites
 Surveillance:
 24h urine collections q1y for at least 10y
 Lifelong f/up
 Pheo: Unresectable, Malignant
 Diagnosis of malignancy made only by demonstrating
INVASION OF ADJACENT STRUCTURES or
documenting NODAL OR DISTANT METASTASIS
 Resection of metastasis and medical control of HTN
 -blockade
 Selective 1-blockers (Prazosin, ) 1st line as less side-effects
 Phenoxybenzamine: more complete -blockade
 -blocker, CCB, ACE-I, etc.
 Nuclear Medicine Rx:
 Hi dose 131I-MIBG or 111indium-octreotide depending on MIBG scan or
octreoscan pick-up
 Sensitize tumor with Carboplatin + 5-FU
 Pheo & Pregnancy
 Diagnosis with 24h urine collections and MRI
 No stimulation tests, no MIBG if pregnant
 1st & 2nd trimester (< 24 weeks):
 Phenoxybenzamine + blocker prep
 Resect tumor ASAP laprascopically
 3rd trimester:
 Phenoxybenzamine + blocker prep
 When fetus large enough: cesarian section followed by tumor
resection
 Primary Adrenal Hyperplasia
 Occurs as a corticotropin independent syndrome of
hypercortisolism
 Results from several small hyperfunctioning nodules
which suppress corticotropin secretion
 Can be difficult to separate from the bilateral hyperplasia
that occurs in pituitary dependant Cushing’s
 Cortisol levels do not decrease after dexamethasone
 Plasma coticotropin levels are undetectable
 Scintigraphy reveals bilateral uptake
 Bilateral adrenalectomy is indicated
 Virilizing Adrenal Tumors
 70% are adrenocortical carcinomas
 Females can present with hirsutism, irregular
menses, and scalp alopecia
 Measure baseline serum testosterone,
dihydroepiandostendione (DHEAS) and 24 hour
17-hydroxy and ketosteroids.
 Image with CT/MRI

 5 day dexamethasone supression testing (ovarian

source supresses DHEAS and 17-ketosteroids
but adrenal source does not)
 Treat with open adrenalectomy since most are
malignant
 Neuroblastoma
 Arise from undifferentiated cells of sympathetic nervous
system
 Most frequent solid tumor in infancy
 Associated with WAGR syndrome, Beckwith Wiedman
syndrome, von Recklinghausen’s disease, N-myc mut.
 Abdominal mass
 Metastasis is common(65%) – liver, lung, bone, brain
 Spot urine HVA(80%) and VMA(75%)
 CT/MRI, bone scan, Bone marrow aspiration, Biopsy
 Surgery with or without chemotherapy