Practice question answers II Tumor of heart and vessels
Practice question answers
Tumor of heart and vessels
© Jun Wang, MD, PhD
1. C. A hypocellular
tumor with scattered stromal cells without significant atypia in an edematous/myxoid
background is most likely myxoma.
Angiosarcoma
is the most common malignant tumor of heart, and is characterized anastomosing
vessels with endothelial atypia. Mucinous adenocarcinoma has atypical
epithelial cells in mucin pools, not myxoid background. Papillary
fibroelastoma is characterized by a papillary growth with central dense fibrous
cores surrounded by loose connective tissue, not myxoid background. Rhabdomyoma
is characterized by sharp demarcation and “spider cells”.
2. A. This is a case
of Carney
complex, presented with Cushing syndrome due to adrenal cortex
hyperplasia and cardiac myxoma. Horner
syndrome is caused by interruption of the sympathetic nerve supply to the
eye, presents with triad of miosis, partial ptosis and loss of hemifacial
sweating. Superior
vena cava syndrome is caused by tumor compression of superior vena cava, with
presentation including dyspnea and facial swelling, etc. Tuberous
sclerosis is characterized by hamartomatous lesions in various sites, including
brain, heart, skin, etc. It is associated with cardiac rhabdomyoma
but not myxoma.
von
Hippel-Lindau syndrome has increased risk of renal
cell carcinoma, adrenal pheochromocytoma, pancreatic neuroendocrine
tumor, hemangioblastoma,
etc.
3. C. Mutation of PRKAR1A
is seen in Carney
complex. APC mutation is seen in familial
adenomatous polyposis. EGFR mutation is associated with various
tumors, including adenocarcinoma
of lung. TSC mutation is seen in tuberous
sclerosis. VHL mutation is seen in von
Hippel-Lindau syndrome.
4. E. The clinical
presentation and radiologic findings are indicative for ischemic stroke and echocardiogram
should be included in the work ups. Infectious endocarditis usually presents
with fever, petechiae, etc. Meningitis and spread of malignant tumors usually
has more relevant symptoms and signs.
5. B. Papillary
growth with central dense fibrous cores covered by flat endothelial cells is
most likely papillary
fibroelastoma. Endocarditis usually has inflammation and surface thrombus. Hodgkin
lymphoma involving heart is very rare and should have classic
features such as Reed–Sternberg cells in a background of inflammatory cells. Rhabdomyoma
is a myocardial tumor characterized by sharp demarcation and “spider cells”.
6. E. Sharply
demarcated heart tumor with spider cells is most likely rhabdomyoma.
When multiple, it is strongly suggestive of tuberous
sclerosis, as shown in this patient with assoicciated neurological and
renal manifestations. Leiomyoma
is characterized by spindle cell proliferation with cigar shaped nuclei and expression
of muscular markers, such as desmin. Lipoma
is composed of mature adipose tissue, not spider cells. Myxoma
is hypocellular tumor with scattered stromal cells without significant atypia
in an edematous/myxoid background. Papillary
fibroelastoma is characterized by a papillary growth with central dense fibrous
cores surrounded by loose connective tissue, not spider cells.
7. A. This is a
patient with tuberous
sclerosis, and the kidney tumor characterized by distorted thick-walled
vessels, spindle cells with cigar-shaped nuclei, and mature adipose tissue is
consistent with angiomyolipoma,
a tumor associated with tuberous
sclerosis. Leiomyosarcoma
is a malignant smooth muscle tumor with marked atypia. Liposarcoma
is a malignant tumor with adipose tissue differentiation and characterized by
the presence of lipoblast. Oncocytoma
is composed of cells with eosinophilic granular cells (oncocytic changes) and a
central scar. Renal
cell carcinoma has various degree of cytological atypia. None of these
tumors has three components of vessels, muscle and adipose tissue.
8. C. Tuberous
sclerosis is characterized by hamartomatous lesions in various sites, including
brain, heart, skin, kidney, etc. It is associated with cardiac rhabdomyoma.
Carney
complex is characterized by cardiac myxoma, adrenocortical changes,
pituitary adenoma, etc. Neurofibromatosis
II is characterized by acoustic schwannomas, meningiomas, café au lait, etc.
von
Hippel-Lindau syndrome is characterized by formations of
tumors and cysts in kidneys, pancreas, etc, and has increased risk for renal
cell carcinoma, adrenal pheochromocytoma, pancreatic neuroendocrine
tumor, hemangioblastoma,
etc. WAGR syndrome is characterized by aniridia, genitourinary malformations, mental
retardation and Wilms
tumor.
9. D. TSC mutation
is seen in tuberous
sclerosis. APC mutation is seen in familial
adenomatous polyposis. Mutation of PRKAR1A is seen in Carney
complex. NF2 mutation is seen in neurofibromatosis
II. VHL mutation is seen in von
Hippel-Lindau syndrome.
10. A. Red growth in
the area of previous radiation therapy for breast cancer should raise the concern
of angiosarcoma.
Expression of von-Willibrand factors is consistent with endothelial origin. Anastomosing
spaces lined by atypical cells that express von-Willibrand factor is consistent
with angiosarcoma,
and is positive for CD31. E-cadherin is positive in many epithelial cells,
including benign and malignant duct cells of breast. ER is expressed in tissue
of female reproductive system, including breast tissue, but not endothelium.
Her2 is amplified in many high grade tumors, but less likely in angiosarcoma.
11. B. Anastomosing
spaces lined by atypical cells that express von-Willibrand factor is consistent
with angiosarcoma.
Acute and chronic mastitis has neutrophilic or lymphoplasmacytic infiltrate,
but not atypia. Pyogenic
granuloma is characterized by lobular proliferation of capillaries separated
by fine fibrous tissue without significant cytological atypia. Recurrent ductal
carcinoma has ductal differentiation, but not von-Willibrand factor expression.
12. B. Mass of small
vessels lined by atypical cells in a background of lymphedema is most likely lymphangiosarcoma,
especially in a patient with history of mastectomy and lymphedema. Either
cellulitis or lymphedema should not have vascular proliferation with atypia.
Metastatic lobular carcinoma has growth of epithelial cells that are positive
for cytokeratin, but negative for CD31. Hodgkin
lymphoma has classic features such as Reed–Sternberg cells in a
background of inflammatory cells.
13. B. Vascular proliferation
with complex anastomosis and lined by atypical cells is consistent with angiosarcoma.
Alcoholic liver disease is characterized hepatocytic damage, not vascular proliferation.
Cavernous
hemangioma has large vessels but not atypia. Hepatocellular
carcinoma do not have large spaces, and is negative for CD31. Regenerative
nodules seen in liver cirrhosis are proliferation of CD31-negative hepatocytes without
large vascular spaces.
14. D. Polyvinyl
chloride is associated with angiosarcoma
in liver. Alcohol, hepatitis virus B and C are associated with hepatocellular carcinoma.
Tobacco is associated with many type of cancers, but less likely angiosarcoma.
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