Tumors of the Heart, Pericardium, and Great Vessels

Question 1

Tumors of the Heart, Pericardium, and Great Vessels

In what division of the mediastinum is the heart located?

Answer 1

middle (inferior aspect)

Question 2

Tumors of the Heart, Pericardium, and Great Vessels

Approximately 75% of
primary tumors of the heart are benign, and about half of those are atrial _______.

Answer 2

Myxomas

Question 3

Tumors of the Heart, Pericardium, and Great Vessels

There is an increased incidence of
cardiac myxomas among females and in patients with ________________, an
inherited autosomal dominant disorder that also shows hyperpigmented
lentigines, blue nevi, schwannomas, endocrine tumors, and endocrinopathies

Answer 3

Carney complex

Question 4

Tumors of the Heart, Pericardium, and Great Vessels

This type of tumor constitutes the majority of “myocardial” malignant neoplasms.

Answer 4

sarcomas
***
Malignant fibrous histiocytoma, angiosarcoma, fibrosarcoma, and rhabdomyosarcoma are among the more common histologic types.

Question 5

Tumors of the Heart, Pericardium, and Great Vessels

What is the most common primary malignancy of the pericardium?

Answer 5

malignant mesothelioma

Question 6

Tumors of the Heart, Pericardium, and Great Vessels

Metastatic disease to the heart and pericardium occurs at a frequency much greater than that of primary tumors.

TRUE or FALSE?

Answer 6

True

Question 7

Tumors of the Heart, Pericardium, and Great Vessels

Metastatic disease to the heart and pericardium occurs at a frequency much greater than that of primary tumors.

What is the most common cause of metastatic disease to the heart?

Answer 7

Lung cancer (nearly one half of the cases)

Question 8

Tumors of the Heart, Pericardium, and Great Vessels

There appeared to be no sex predilection for tumors of the aorta or pulmonary artery, although the _____ to _____ ratio was 3 to 1 among patients with tumors of the IVC.

In other words, what gender is the most commonly affected in IVC tumors?

Answer 8

female to male

female

Question 9

Tumors of the Heart, Pericardium, and Great Vessels

What chamber of the heart is most commonly involved by myxomas?

Answer 9

left atrium

Question 10

Tumors of the Heart, Pericardium, and Great Vessels

Angiosarcoma is the most common type of sarcoma of the myocardium.

What chamber of the heart is most commonly involved?

Answer 10

right atrium

Question 11

Tumors of the Heart, Pericardium, and Great Vessels

Angiosarcoma is the most common type of sarcoma of the myocardium.

What is the most common site of metastasis?

Answer 11

Lungs

Question 12

Tumors of the Heart, Pericardium, and Great Vessels

What histologic finding is associated with a worse prognosis?

Answer 12

> 10 mitoses/hpf

Question 13

Tumors of the Heart, Pericardium, and Great Vessels

In the definitive setting, what is the treatment of choice for primary pericardial tumors?

Answer 13

surgical resection of all gross disease.
consider adjuvant RT/chemo

The radiation treatment volume should
include the entire pericardial surface and likely should include the mediastinal
lymph nodes

Question 14

Tumors of the Heart, Pericardium, and Great Vessels

In the definitive setting, what is the treatment of choice for primary myocardial tumors?

Answer 14

surgical resection of all gross disease.

+RT

Question 15

Tumors of the Heart, Pericardium, and Great Vessels

What is the treatment of choice for tumors of the great vessels?

Answer 15

Resection of the tumor with graft reconstruction of the vessel
+RT

Question 16

Tumors of the Heart, Pericardium, and Great Vessels

Combination chemotherapy is the only known curative therapy for patients with metastatic disease to the
heart.

TRUE or FALSE?

Answer 16

False.

There is no known curative therapy for patients with metastatic disease to the
heart.

Question 17

Tumors of the Heart, Pericardium, and Great Vessels

What is the recommended postoperative RT dose for completely resected cardiac sarcomas with negative margins?

Answer 17

45 to 50 Gy at 1.8 to 2 Gy/fraction
***
Decisions on radiation dose and target definitions for cardiac tumors will be
determined by the tumor type and the particular clinical indication for treatment.
Given that sarcomas are the most common primary malignant cardiac tumors,
the radiotherapy model used in their management has been extrapolated from the
approach used for soft tissues sarcomas arising at extracardiac sites.

Question 18

Tumors of the Heart, Pericardium, and Great Vessels

What is the CTV for postoperative RT of a completely resected cardiac sarcomas with negative margins?

Answer 18

The initial target should include all areas known to harbor tumor before surgery, with a margin of about 2 cm.

This target should be derived from diagnostic imaging and other investigations and also by the operative description of the surgeon.

Question 19

Tumors of the Heart, Pericardium, and Great Vessels

What is the recommended postoperative RT dose for incompletely resected cardiac sarcomas?

Answer 19

same as completely resected +10 to 20 Gy boost.

Question 20

Tumors of the Heart, Pericardium, and Great Vessels

What is the RT dose for “definitive” RT of cardiac sarcomas?

Answer 20

70 Gy at least

Question 21

Tumors of the Heart, Pericardium, and Great Vessels

What is the RT dose for primary pericardial mesothelioma?

Answer 21

40 to 64 Gy

Question 22

Tumors of the Heart, Pericardium, and Great Vessels

What is the RT CTV for primary pericardial mesothelioma?

Answer 22

entire pericardium + mediastinal lymph nodes

Question 23

Tumors of the Heart, Pericardium, and Great Vessels

With respect to altered fractionation
approaches for cardiac tumors, there are few reports on dose variations to
improve the efficacy of conventional radiotherapy. A rare example is a case
report on the use of hyperfractionation for a cardiac sarcoma treated to a dose of
70.5 Gy at 1.5 Gy/fraction delivered twice daily with a radiosensitizer.

What is this radiosensitizer?

Answer 23

iododeoxyuridine