Adrenal Cancer Flashcards

1
Q

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
A

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

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2
Q

Adrenal Cancer

Which arterial blood supply originates from the renal artery?

A

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.

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3
Q

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?

A

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.

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4
Q

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?

A

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.

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5
Q

Adrenal cancer

Which of the following syndromes is the least likely associated with ACC?

Li Fraumeni
MEN-1
MEN-2
Beckwith-Wiedeman
SBLA
A

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).

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6
Q

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
A

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.

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7
Q

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

A

only II is true
***

I is false

Extra-adrenal tumors are more commonly malignant.

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8
Q

Adrenal Cancer

Which of the following syndromes is the most likely associated with bilateral pheochromocytoma?

MEN-IIA
vHL
NF
von Recklinghausen disease

A

MEN-IIA

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9
Q

Adrenal Cancer

Which side is more commonl;y affected? left or right?

A

1:1

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10
Q

Adrenal Cancer

Which is typically a larger mass? nonfunctioning or functioning?

A

nonfunctioning.

because functioning produces hormones, they are diagnosed at an early stage.

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11
Q

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.

A

IV.

pediatric

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12
Q

Adrenal Cancer
TNM staging

What is the T-stage?
Tumor invading adjacent organs

A

T4

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13
Q

Adrenal Cancer
TNM staging

What is the T-stage?
Tumor outside adrenal in fat?

A

T3

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14
Q

Adrenal Cancer
TNM staging

What is the T-stage?
No invasion ≤5 cm

A

T1

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15
Q

Adrenal Cancer
TNM staging

What is the T-stage?
No invasion >5 cm

A

T2

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16
Q

Adrenal Cancer
TNM staging

N1

A

+LN

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17
Q

Adrenal Cancer
TNM staging

What is the stage grouping?

T4,N0,M0

A

IV

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18
Q

Adrenal Cancer
TNM staging

What is the stage grouping?

T3,N1,M0

A

IV

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19
Q

Adrenal Cancer
TNM staging

What is the stage grouping?

M1

A

IV

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20
Q

Adrenal Cancer
TNM staging

What is the stage grouping?

T3,N0,M0

A

III

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21
Q

Adrenal Cancer
TNM staging

What is the stage grouping?

T2,N0,M0

A

II

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22
Q

Adrenal Cancer
TNM staging

What is the stage grouping?

T1,N1,M0

A

III

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23
Q

Adrenal Cancer

Which is slightly more common?

functioning or non-functioning?

A

functioning

24
Q

Adrenal Cancer

What hormones are usually secreted by functioning adrenocortical tumors?

A

cortisol and androgens

25
Q

Adrenal Cancer

What is the most common presentation of functioning tumors in children?

A

virilization

26
Q

Adrenal Cancer

What is the most common presentation of functioning tumors in adults?

A

Cushing

alone or mixed with virilization

27
Q

Adrenal Cancer

What is the classic “triad” of symptoms in pheochromocytoma?

A

episodic headaches,
diaphoresis,
tachycardia

28
Q

Adrenal Cancer

In pheochromocytomas, which is more common (90%)?
adrenal primary
vs.
sporadic

A

primary adrenal medulla

29
Q

Adrenal Cancer

Hypertension is a common symptom in which adrenal tumor?

A

pheochromocytoma

30
Q

Adrenal Cancer

What is the imaging test of choice for the evaluation of adrenal tumors?

A

CT of the abdomen with thin cuts through the adrenal gland is the imaging test of choice for the evaluation of adrenal tumors.

31
Q

Adrenal Cancer

What imaging modality is used to better delineate venous invasion?

A

MRI

If there is still question of vascular invasion,
an angiographic study can be performed preoperatively, either selective
arteriography or vena cavography.

32
Q

Adrenal Cancer

How do you confirm the diagnosis of pheochromocytoma?

A

measurements
of urinary and plasma fractionated metanephrines and catecholamines

Levels of metanephrine four
times the normal limit are diagnostic of pheochromocytoma

33
Q

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 &amp; II only
II &amp; III only
III only
A

I & II only

The only absolute criterion for malignancy is metastasis

34
Q

Adrenal Cancer
Management

benign lesion, smaller than 4 cm

A

can be observed.

repeat imaging 6 to 12 months

35
Q

Adrenal Cancer
Management

benign lesion, smaller than 4 cm, with growth rate >1 cm/year on surveillance.

A

can be removed electively

36
Q

Adrenal Cancer
Management

benign lesion >4 cm

A

repeat imaging 3 to 6 months

37
Q

Adrenal Cancer
Management

benign lesion >4 cm, any growth rate on surveillance imaging

A

open adrenalectomy +lymphadenectomy

***

38
Q

Adrenal Cancer
Management

benign lesion >4 cm, heterogeneous, irregular margins

A

treat as ACC
open adrenalectomy +lymphadenectomy

Complete resection,
such as an open adrenalectomy, is the only treatment that offers long-term
disease-free survival

39
Q

Adrenal Cancer
Management

What is a strong predictor for survival in post-operative patients?

A

margin status

40
Q

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?

A

True

41
Q

Adrenal Cancer
Management

The presence of tumor thrombus in the renal vein or vena cava is a contraindication to surgery.

TRUE or FALSE?

A

False.

The presence of tumor thrombus in the renal vein or vena cava
does not preclude resection

42
Q

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?

A

True

43
Q

Adrenal Cancer
Management

What are the indications for PORT in localized disease?

A

high-risk features such as:

  • positive margins
  • rupture of capsule
  • high grade
  • large size
44
Q

Adrenal Cancer
Management

What chemotherapeutic agent, an insecticide congener, is most commonly used in the management of ACC?

A

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.

45
Q

Adrenal Cancer
Management

What is the definitive management for pheochromocytoma in general?

A

surgical resection.

46
Q

Adrenal Cancer
Management

What is the primary management for malignant pheochromocytoma?

A

surgical debulking.

47
Q

Adrenal Cancer
Management

What is the management for bilateral pheochromocytoma?

A

bilateral adrenalectomy with hormonal replacement.

48
Q

Adrenal Cancer
Management

What are included in the fields for the RT of ACC?

A

gross tumor + margins

+ regional nodes (including contralateral para-aortic lymph nodes)

49
Q

Adrenal Cancer
Management

RT dose to gross disease/macroscopic residual?

A

50 to 60 Gy

50
Q

Adrenal Cancer
Management

RT dose to uninvolved regional nodes?

A

45 Gy

51
Q

Adrenal Cancer
Management

RT dose to gross microscopic residual in high-risk patients?

A

45 to 54 Gy

52
Q

Adrenal Cancer
Management

Palliative RT dose?

A

30 to 40 Gy (2-3 weeks)

53
Q

Adrenal Cancer
Management

Follow-up schedule post surgery?

A

every 6 months for the first 3 years starting around 3 months postsurgery and annually thereafter

54
Q

Adrenal Cancer
(from in-service bank)

  1. 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
A

D

55
Q

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
A

D

56
Q

Adrenal Cancer
(from in-service bank)

  1. 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

A

57
Q

Adrenal Cancer
(from in-service bank)

  1. Hereditary cancer syndromes which are associated with Adrenocortical Cancer (ACC), EXCEPT

(sorry naputol yung pic)
BONUS

A

BONUS