Practice questions II Tumors of heart and vessels

Practice question II

Tumors of heart and vessels

© Jun Wang, MD, PhD

1. A 59-year old woman presents with dyspnea for 3 weeks. She denies fever, chill or weight loss. She has a history of mucinous adenocarcinoma of left breast that was treated with surgery and chemotherapy 3 years ago. Her past history is otherwise unremarkable. She does not smoke cigarette nor drink alcohol. Physical examination revealed no significant abnormalities.  Transthoracic echocardiography reveals a 2.2 cm pedunculated mass in her left atrium. The mass is removed and microscopically it has scattered mildly atypical round to oval nuclei in an edematous background. Thrombus is seen on the surface. Immunohistochemistry studies reveal no cytokeratin expressing cells. What is the most likely diagnosis?

A. Angiosarcoma

B. Metastatic mucinous breast cancer

C. Myxoma

D. Papillary fibroelastoma

E. Rhabdomyoma

2. Use this case for the next two questions. A 27-year-old woman presents with oligomenorrhea and 35 pound weight gain in the last two years. Her past medical history is unremarkable. She is not taking any medications. She does not smoke cigarette nor drink alcohol. She has a sister who was recently diagnosed with left breast invasive ductal carcinoma. Physical examination reveals a full moon face, a humped neck, abdominal obesity and moderate hirsutism. She has red striae on abdomen and legs. A few light brown macules are seen on her face and chest. Laboratory tests reveal an afternoon cortisol level at 28 microgram/dl (normal 2-18 microgram/dl) and an ACTH level at 5.6 pg/ml (normal 10-50pg/ml). Radiological examination reveals bilateral adrenal hyperplasia with a 1.5 cm nodule in the left adrenal gland, as well as a 2.2 cm intracardiac mass attached to the roof of the left atrium. The mass is resected and microscopically it is a markedly edematous growth with scattered round to oval cells. No cytological atypia is seen. What is the diagnosis for her overall condition?

A. Carney syndrome

B. Horner syndrome

C. Superior vena cava syndrome

D. Tuberous sclerosis

E. Von Hipple Lindau disease

3. A 27-year-old woman presents with oligomenorrhea and 35 pound weight gain in the last two years. Her past medical history is unremarkable. She is not taking any medications. She does not smoke cigarette nor drink alcohol. She has a sister who was recently diagnosed with left breast invasive ductal carcinoma. Physical examination reveals a full moon face, a humped neck, abdominal obesity and moderate hirsutism. She has red striae on abdomen and legs. A few light brown macules are seen on her face and chest. Laboratory tests reveal an afternoon cortisol level at 28 microgram/dl (normal 2-18 microgram/dl) and an ACTH level at 5.6 pg/ml (normal 10-50pg/ml). Radiological examination reveals bilateral adrenal hyperplasia with a 1.5 cm nodule in the left adrenal gland, as well as a 2.2 cm intracardiac mass attached to the roof of the left atrium. The mass is resected and microscopically it is a markedly edematous growth with scattered round to oval cells. No cytological atypia is seen. Abnormality of what gene is most likely associated with her conditions?

A. APC

B. EGFR

C. PRKAR1A

D. TSC

E. VHL

4. Use this case for the next two questions. A 55-year-old man presents with sudden onset of unsteady gait for 2 days. He denies other symptoms. He has a history of stage II mediastinum classical Hodgkin lymphoma that was treated with chemotherapy and radiation therapy 15 years ago. He does not smoke nor drink alcohol. Physical examination reveals poor oral hygiene and severe gingivitis. Neurological examination reveals mild left arm ataxia. No other abnormalities are noted. His head MRI reveals small foci of right cerebral infarct. What is a proper step for his work ups?

A. Blood culture for possible endocarditis

B. Bone marrow biopsy for involvement of Hodgkin lymphoma

C. Chest X-ray for tumors

D. CSF analysis for meningitis

E. Echocardiogram for embolism investigation

5. A 55-year-old man presents with sudden onset of unsteady gait for 2 days. He denies other symptoms. He has a history of stage II mediastinum classical Hodgkin lymphoma that was treated with chemotherapy and radiation therapy 15 years ago. He does not smoke nor drink alcohol. Physical examination reveals poor oral hygiene and severe gingivitis. Neurological examination reveals mild left arm ataxia. No other abnormalities are noted. His head MRI reveals small foci of right cerebral infarct. transthoracic echocardiogram reveals a 1.2 cm bulky, and highly mobile mass on the aortic aspect of the aortic valve. The mass was removed surgically. Microscopic examination of the mass reveals finger like projections of a fibrous core surrounded covered by flat endothelial cells. No cytological atypia, necrosis nor inflammation is seen. What is the diagnosis?

A. Infectious endocarditis

B. Papillary fibroelastoma

C. Recurrent Hodgkin lymphoma involving aortic valve

D. Rhabdomyoma

E. Rheumatic endocarditis

6. Use this case for the next four questions. A 38-year-old woman presents with dizziness for a week. She denies history of headache, fever, or fatigue. She has intermittent seizures since age of 5, until a brain tumor was removed at age of 9. Her past medical history is otherwise unremarkable. Physical examination reveal a few light-colored patches on her chest and back. Her heart rate was irregular. No other abnormality is seen. EKG identified numerous premature beats. Transthoracic echocardiography revealed three masses in the left ventricular and one mass near right ventricular outflow. These masses are sharply demarcated and measures up to 0.5 cm in greatest dimension. Biopsy of these masses reveals irregular large cells with cytoplasmic vacuoles, separated by strands between cell membrane and nucleus. Additional work up identified a 3.2 cm well circumscribed mass in her right kidney. CT-guided biopsy reveal a mixture of distorted thick-walled vessels, spindle cells with cigar-shaped nuclei, and mature adipose tissue. No atypia is seen. What is the diagnosis of her heart lesion?

A. Leiomyoma

B. Lipoma

C. Myxoma

D. Papillary fibroeslastoma

E. Rhabdomyoma

7. A 38-year-old woman presents with dizziness for a week. She denies history of headache, fever, or fatigue. She has intermittent seizures since age of 5, until a brain tumor was removed at age of 9. Her past medical history is otherwise unremarkable. Physical examination reveals a few light-colored patches on her chest and back. Her heart rate was irregular. No other abnormality is seen. EKG identified numerous premature beats. Transthoracic echocardiography revealed three masses in the left ventricular and one mass near right ventricular outflow. These masses are well circumscribed and measures up to 0.5 cm in greatest dimension. Biopsy of these masses reveals irregular large cells with cytoplasmic vacuoles, separated by strands between cell membrane and nucleus. Additional work up identified a 3.2 cm well circumscribed mass in her right kidney. CT-guided biopsy reveal a mixture of distorted thick-walled vessels, spindle cells with cigar-shaped nuclei, and mature adipose tissue. No atypia is seen. What is the diagnosis for her kidney lesion?

A. Angiomyolipoma

B. Leiomyosarcoma

C. Liposarcoma, well differentiated

D. Oncocytoma

E. Renal cell carcinoma

8. A 38-year-old woman presents with dizziness for a week. She denies history of headache, fever, or fatigue. She has intermittent seizures since age of 5, until a brain tumor was removed at age of 9. Her past medical history is otherwise unremarkable. Physical examination reveals a few light-colored patches on her chest and back. Her heart rate was irregular. No other abnormality is seen. EKG identified numerous premature beats. Transthoracic echocardiography revealed three masses in the left ventricular and one mass near right ventricular outflow. These masses are well circumscribed and measures up to 0.5 cm in greatest dimension. Biopsy of these masses reveals irregular large cells with cytoplasmic vacuoles, separated by strands between cell membrane and nucleus. Additional work up identified a 3.2 cm well circumscribed mass in her right kidney. CT-guided biopsy reveal a mixture of distorted thick-walled vessels, spindle cells with cigar-shaped nuclei, and mature adipose tissue. No atypia is seen. What is the condition she most likely has?

A. Carney complex

B. Neurofibromatosis II

C. Tuberous sclerosis

D. Von Hipple Lindau disease

E. WAGR syndrome

9. A 38-year-old woman presents with dizziness for a week. She denies history of headache, fever, or fatigue. She has intermittent seizures since age of 5, until a brain tumor was removed at age of 9. Her past medical history is otherwise unremarkable. Physical examination reveals a few light-colored patches on her chest and back. Her heart rate was irregular. No other abnormality is seen. EKG identified numerous premature beats. Transthoracic echocardiography revealed three masses in the left ventricular and one mass near right ventricular outflow. These masses are well circumscribed and measures up to 0.5 cm in greatest dimension. Biopsy of these masses reveals irregular large cells with cytoplasmic vacuoles, separated by strands between cell membrane and nucleus. Additional work up identified a 3.2 cm well circumscribed mass in her right kidney. CT-guided biopsy reveal a mixture of distorted thick-walled vessels, spindle cells with cigar-shaped nuclei, and mature adipose tissue. No atypia is seen. Abnormality of what gene is most likely associated with her conditions?

A. APC

B. NF2

C. PRKAR1A

D. TSC

E. VHL

10. Use this case for the next two questions. A 55-year-old woman presents with a painless red growth in the outer upper quadrant for 1 month. She has had left breast estrogen receptor negative and Her2 positive invasive ductal carcinoma that was treated with lumpectomy and local radiation therapy 5 years ago. She has a history of type II diabetes for 15 years. She does not smoke cigarettes, and is a social drinker. Her other history is unremarkable. Physical examination reveal a 2.5 cm mass underneath the scar for her previous surgery. No lymphadenopathy is noted. Sonographic examination reveals a hypervascular growth. Biopsy of the mass reveals irregular spaces with extensive anastomosis. These spaces are lined by markedly atypical cells, with brisk mitosis. Scattered neutrophilic and lymphocytic infiltrate is seen. Per immunohistochemistry studies, these atypical cells are positive for von Willibrand factor. What additional marker is likely to be positive for these atypical cells?

A. CD31

B. E-cadherin

C. Estrogen receptor

D. Her2

11. A 55-year-old woman presents with a painless red growth in the outer upper quadrant for 1 month. She has had left breast estrogen receptor negative and Her2 positive invasive ductal carcinoma that was treated with lumpectomy and local radiation therapy 5 years ago. She has a history of type II diabetes for 15 years. She does not smoke cigarettes, and is a social drinker. Her other history is unremarkable. Physical examination reveal a 2.5 cm mass underneath the scar for her previous surgery. No lymphadenopathy is noted. Sonographic examination reveals a hypervascular growth. Biopsy of the mass reveals irregular spaces with extensive anastomosis. These spaces are lined by markedly atypical cells, with brisk mitosis. Scattered neutrophilic and lymphocytic infiltrate is seen. Per immunohistochemistry studies, these atypical cells are positive for von Willibrand factor. What is the diagnosis?

A. Acute mastitis

B. Angiosarcoma

C. Chronic mastitis

D. Pyogenic granuloma

E. Recurrent ductal carcinoma

12. A 62-year-old woman presents with ulcer of right arm for a week, following swelling for the past five years. She has right breast invasive lobular carcinoma 10 years ago, and was treated with total mastectomy and right axillary lymph node dissection, followed by chemotherapy and radiation therapy. She has a history of Hodgkin lymphoma, classical type, diagnosed at age 38, and was treated with radiation therapy and chemotherapy. She has had type II diabetes for 20 years. Her past history is otherwise unremarkable. Physical examination reveals diffuse edematous changes in the right arm, with multiple firm nodular areas. A 6 cm area of multiple ulcerated lesions with surrounding erythema and induration was noted. Biopsy reveals edematous stroma containing growth of small vessel like spaces lined cell with moderate cytological atypia. Scattered neutrophils, lymphocytes and plasma cells are noted. No binucleated cells are seen. Per immunohistory chemistry studies, these atypical cells are positive for CD31. What is the diagnosis?

A. Acute cellulitis

B. Lymphangiosarcoma

C. Lymphedema, inflamed

D. Metastatic lobular carcinoma

E. Recurrent Hodgkin lymphoma

13. Use this case for the next two questions. A 51-year-old man presents with dull right up abdomen pain for 1 week. His past medical history is unremarkable. He does not smoke but has been drinking whiskey 2 glasses a day for 20 years. He is currently a production line manager for a polyvinyl chloride polymerization plant, after 30 years of working there.  Upon, physical examination, his eyes were not icteric, and the conjunctiva was not pale. There is knock pain at the lower part of the right ribs. His laboratory test results are within normal range except slightly elevated AST and ALT. Abdominal CT reveals multiple intrahepatic nodules in the right lobe, in the background of cirrhosis. Biopsy of one the nodules reveals channel-like spaces with complex anastomosis and lined by moderately atypical cells with brisk mitosis. Per immunohistochemistry studies, these atypical cells are positive for CD31. What is the most likely diagnosis?

A. Alcoholic liver disease

B. Angiosarcoma

C. Hepatic cavernous hemangioma

D. Hepatocellular carcinoma

E. Regenerative nodules of cirrhosis

14. A 51-year-old man presents with dull right up abdomen pain for 1 week. His past medical history is otherwise unremarkable except recently diagnosed type II diabetes. He has a history of 35 pack year cigarette smoking and has been drinking whiskey 2 glasses a day for 20 years. He is currently a production line manager for a polyvinyl chloride polymerization plant, after 30 years of working there.  Upon, physical examination, his eyes were not icteric, and the conjunctiva was not pale. There is knock pain at the lower part of the right ribs. His laboratory test results are within normal range except slightly elevated AST and ALT. Abdominal CT reveals multiple intrahepatic nodules in the right lobe, in the background of cirrhosis. Biopsy of one the nodules reveals channel-like spaces with complex anastomosis and lined by moderately atypical cells with brisk mitosis. Per immunohistochemistry studies, these atypical cells are positive for CD31. What factor is most likely causing the changes seen in the biopsy?

A. Alcohol

B. Hepatitis B virus

C. Hepatitis C virus

D. Polyvinyl chloride

E. Tobacco

Answers

Back to cardiovascular system tumors
Back to soft tissue tumors
Back to contents