Adrenal Cancer Flashcards
Adrenal Cancer
The triangular right
gland relates to the diaphragm posteriorly and the inferior vena cava and liver
anteriorly.
The semilunar left adrenal gland is positioned in the middle of the left
crux of the diaphragm.
--- I is true II is true I & II are true neither are true
I & II both true
The paired suprarenal (adrenal) glands are located between the superomedial
aspects of the kidney and the diaphragmatic crura. They are surrounded by
connective tissue containing perinephric fat. The glands are enclosed by renal
fascia but separated from the kidneys by fibrous tissue. The triangular right
gland relates to the diaphragm posteriorly and the inferior vena cava and liver
anteriorly. The semilunar left adrenal gland is positioned in the middle of the left
crux of the diaphragm. The omental bursa separates it from the stomach. It is
also related to the spleen and pancreas
Adrenal Cancer
Which arterial blood supply originates from the renal artery?
inferior suprarenal artery
***The superior suprarenal arteries are derived from the inferior phrenic
artery, the middle suprarenal arteries from the abdominal aorta near the origin of
the superior mesenteric artery, and the inferior suprarenal arteries from the renal
artery. A large central vein leaves the anterior surface of the gland at the hilum.
The shorter right suprarenal vein drains into the inferior vena cava, and the
longer left suprarenal vein drains into the left renal vein.
Adrenal Cancer
The inferior lymphatic trunks end in aortocaval lymph
nodes located near the origin of the celiac plexus.
The superior lymphatic trunks
end in lateroaortic nodes above the renal pedicle.
TRUE or FALSE?
False. Other way around
***
The lymphatic drainage follows the arterial supply and is predominantly to
lumbar lymph nodes. The superior lymphatic trunks end in aortocaval lymph
nodes located near the origin of the celiac plexus. The inferior lymphatic trunks
end in lateroaortic nodes above the renal pedicle. Some trunks may pass through
the diaphragm, following the splanchnic nerves, ending in retroaortic nodes in
the posterior mediastinum. On the right, some lymphatic trunks may penetrate
the liver.
Adrenal Cancer
A. ACC (adrenocortical carcinoma) is more common in women
B. Nonfunctional carcinomas are more common in older population (>30)
C. Functional carcinomas are more common in men
D. Functional tumors are more common in younger patients
All of the above are TRUE except?
C.
women: acc and functional
men: nonfunctional
older: non functional
younger: functional
Overall, adrenocortical carcinoma (ACC) is slightly more common in women than in men.
Nonfunctional carcinomas occur in an older age population (>30 years old) and are more common in men (3:2 male-to-female ratio), although functional tumors are more common in women (7:3 female-to-male ratio) and younger patients.
Adrenal cancer
Which of the following syndromes is the least likely associated with ACC?
Li Fraumeni MEN-1 MEN-2 Beckwith-Wiedeman SBLA
MEN-2
***
Although most cases of ACC are sporadic, it has been described as a component of several hereditary cancer syndromes, including Li-Fraumeni syndrome (breast cancer, soft tissue and bone sarcoma, brain tumors, and ACC), Beckwith-Wiedemann syndrome (Wilms tumor, neuroblastoma, hepatoblastoma, and ACC), multiple endocrine neoplasia type I (MEN-I) (parathyroid, pituitary and pancreatic neuroendocrine tumors, and adrenal adenomas and carcinomas), and SBLA syndrome (sarcoma, breast, lung, ACC, and other tumors).
Adrenal Cancer
Which of the following syndromes is the least likely associated with pheochromocytoma?
MEN-IIA MEN-IIB vHL Beckwith Wiedemann NF von Recklinghausen disease
Beckwith Wiedemann
***
Bilateral pheochromocytomas are a component of multiple endocrine neoplasia type IIa (MEN-IIA) syndrome (pheochromocytoma, medullary thyroid carcinoma, and parathyroid hyperplasia) or MEN-IIB syndrome in which they are associated with marfanoid habitus, mucosal neuromas, and medullary thyroid carcinoma.
Pheochromocytomas occur in 25% of patients with von Hippel-Lindau syndrome and <1% of patients with neurofibromatosis and von Recklinghausen disease.
Adrenal Cancer
Extra-adrenal tumors are more commonly benign.
Incidentally discovered masses <3 cm are rarely malignant.
only I is true
only II is true
both are true
both are false
only II is true
***
I is false
Extra-adrenal tumors are more commonly malignant.
Adrenal Cancer
Which of the following syndromes is the most likely associated with bilateral pheochromocytoma?
MEN-IIA
vHL
NF
von Recklinghausen disease
MEN-IIA
Adrenal Cancer
Which side is more commonl;y affected? left or right?
1:1
Adrenal Cancer
Which is typically a larger mass? nonfunctioning or functioning?
nonfunctioning.
because functioning produces hormones, they are diagnosed at an early stage.
Adrenal Cancer
All of the following are true, EXCEPT:
I. ACC is an aggressive malignancy that frequently violates the tumor
capsule and invades surrounding tissues.
II. It metastasizes to lungs, liver, brain and regional lymph nodes.
III. Many patients present with widespread metastasis;
most of these patients die within 6 months of diagnosis.
IV. Widespread disease is
especially common in the elderly population.
IV.
pediatric
Adrenal Cancer
TNM staging
What is the T-stage?
Tumor invading adjacent organs
T4
Adrenal Cancer
TNM staging
What is the T-stage?
Tumor outside adrenal in fat?
T3
Adrenal Cancer
TNM staging
What is the T-stage?
No invasion ≤5 cm
T1
Adrenal Cancer
TNM staging
What is the T-stage?
No invasion >5 cm
T2
Adrenal Cancer
TNM staging
N1
+LN
Adrenal Cancer
TNM staging
What is the stage grouping?
T4,N0,M0
IV
Adrenal Cancer
TNM staging
What is the stage grouping?
T3,N1,M0
IV
Adrenal Cancer
TNM staging
What is the stage grouping?
M1
IV
Adrenal Cancer
TNM staging
What is the stage grouping?
T3,N0,M0
III
Adrenal Cancer
TNM staging
What is the stage grouping?
T2,N0,M0
II
Adrenal Cancer
TNM staging
What is the stage grouping?
T1,N1,M0
III
Adrenal Cancer
Which is slightly more common?
functioning or non-functioning?
functioning
Adrenal Cancer
What hormones are usually secreted by functioning adrenocortical tumors?
cortisol and androgens
Adrenal Cancer
What is the most common presentation of functioning tumors in children?
virilization
Adrenal Cancer
What is the most common presentation of functioning tumors in adults?
Cushing
alone or mixed with virilization
Adrenal Cancer
What is the classic “triad” of symptoms in pheochromocytoma?
episodic headaches,
diaphoresis,
tachycardia
Adrenal Cancer
In pheochromocytomas, which is more common (90%)?
adrenal primary
vs.
sporadic
primary adrenal medulla
Adrenal Cancer
Hypertension is a common symptom in which adrenal tumor?
pheochromocytoma
Adrenal Cancer
What is the imaging test of choice for the evaluation of adrenal tumors?
CT of the abdomen with thin cuts through the adrenal gland is the imaging test of choice for the evaluation of adrenal tumors.
Adrenal Cancer
What imaging modality is used to better delineate venous invasion?
MRI
If there is still question of vascular invasion,
an angiographic study can be performed preoperatively, either selective
arteriography or vena cavography.
Adrenal Cancer
How do you confirm the diagnosis of pheochromocytoma?
measurements
of urinary and plasma fractionated metanephrines and catecholamines
Levels of metanephrine four
times the normal limit are diagnostic of pheochromocytoma
Adrenal Cancer
Which of the following statements is/are TRUE in pheochromocytoma?
I. Pheochromocytomas have malignant features in <10% of cases.
II. Macroscopically, they tend to be encapsulated with areas of cystic change,
hemorrhage, and necrosis.
III. Capsular invasion constitutes malignant change.
--- I only I & II only II & III only III only
I & II only
The only absolute criterion for malignancy is metastasis
Adrenal Cancer
Management
benign lesion, smaller than 4 cm
can be observed.
repeat imaging 6 to 12 months
Adrenal Cancer
Management
benign lesion, smaller than 4 cm, with growth rate >1 cm/year on surveillance.
can be removed electively
Adrenal Cancer
Management
benign lesion >4 cm
repeat imaging 3 to 6 months
Adrenal Cancer
Management
benign lesion >4 cm, any growth rate on surveillance imaging
open adrenalectomy +lymphadenectomy
***
Adrenal Cancer
Management
benign lesion >4 cm, heterogeneous, irregular margins
treat as ACC
open adrenalectomy +lymphadenectomy
Complete resection,
such as an open adrenalectomy, is the only treatment that offers long-term
disease-free survival
Adrenal Cancer
Management
What is a strong predictor for survival in post-operative patients?
margin status
Adrenal Cancer
Management
Invasion or adherence of adjacent structures (pancreas, kidney, or spleen) often
necessitates en bloc resection of the affected organ.
TRUE or FALSE?
True
Adrenal Cancer
Management
The presence of tumor thrombus in the renal vein or vena cava is a contraindication to surgery.
TRUE or FALSE?
False.
The presence of tumor thrombus in the renal vein or vena cava
does not preclude resection
Adrenal Cancer
Management
An extensive
regional lymph node dissection has NOT been shown to have any impact on
overall survival (OS) or disease specific survival (DSS).
TRUE or FALSE?
True
Adrenal Cancer
Management
What are the indications for PORT in localized disease?
high-risk features such as:
- positive margins
- rupture of capsule
- high grade
- large size
Adrenal Cancer
Management
What chemotherapeutic agent, an insecticide congener, is most commonly used in the management of ACC?
Mitotane
a chemical congener of the insecticide DDT (dichlorodiphenyltrichloroethane)
In patients with measurable disease, overall
response rates of 14% to 36% have been reported, but most studies have reported
no significant survival benefit.
Adrenal Cancer
Management
What is the definitive management for pheochromocytoma in general?
surgical resection.
Adrenal Cancer
Management
What is the primary management for malignant pheochromocytoma?
surgical debulking.
Adrenal Cancer
Management
What is the management for bilateral pheochromocytoma?
bilateral adrenalectomy with hormonal replacement.
Adrenal Cancer
Management
What are included in the fields for the RT of ACC?
gross tumor + margins
+ regional nodes (including contralateral para-aortic lymph nodes)
Adrenal Cancer
Management
RT dose to gross disease/macroscopic residual?
50 to 60 Gy
Adrenal Cancer
Management
RT dose to uninvolved regional nodes?
45 Gy
Adrenal Cancer
Management
RT dose to gross microscopic residual in high-risk patients?
45 to 54 Gy
Adrenal Cancer
Management
Palliative RT dose?
30 to 40 Gy (2-3 weeks)
Adrenal Cancer
Management
Follow-up schedule post surgery?
every 6 months for the first 3 years starting around 3 months postsurgery and annually thereafter
Adrenal Cancer
(from in-service bank)
- Radiotherapy fields for patients with unresectable Adrenocortical Carcinoma (ACC) should include the following:
A. Gross tumor only
B. Gross tumor plus ipsilateral regional lymph nodes only
C. Gross tumor, ipsilateral lymph nodes and ipsilateral kidney
D. Gross tumor, regional lymph nodes, and contralateral para-aortic lymph nodes
D
Adrenal Cancer
(from in-service bank)
48. High-risk features associated with localized Adrenocortical Carcinoma (ACC), EXCEPT A. Large tumor size B. Positive margins C. High-grade carcinoma D. Intact capsule
D
Adrenal Cancer
(from in-service bank)
- Which statement about adrenal gland anatomy is FALSE?
A. The adrenal glands are composed of a central steroid-secreting medulla and a catecholamine-producing cortex.
B. The right adrenal gland is triangular, while the left adrenal gland is semilunar in shape.
C. The adrenals have an abundant blood supply due to their endocrine function.
D. The adrenal glands are enclosed by renal fascia and are surrounded by connective tissue containing perinephric fat.
A
Adrenal Cancer
(from in-service bank)
- Hereditary cancer syndromes which are associated with Adrenocortical Cancer (ACC), EXCEPT
(sorry naputol yung pic)
BONUS
BONUS
