Download CARDIOVASCULAR SYSTEM TUMORS

CARDIOVASCULAR SYSTEM
TUMORS
Tumors of Blood Vessels
BENIGN TUMORS & TumorLike Conditions of VESSELS
Tumors
• Hemangiomas
• Hemangiomas (Capillary & Cavernous, and others)
• Epithelioid hemangiomas
• Papillary endothelial hyperplasia
• Glomangiomas (glomus tumors)
• Telangiectasias
• Lymphangioma
Tumor-like coditions: Pyogenic granuloma
Hemangiomas
• Mostly hamartomas.
• The endothelial cells are of course clonal and show
some enhanced responses to some growth factors.
• Capillary hemangiomas (little vessels) and
cavernous hemangiomas (big vessels) are common
on the skin.
• Stork bites (leylek izi) backs of the neck and/or
forehead of a baby) usually involute (i.e., thrombose
and organize) after a few years, while cherry
angiomas of the skin start popping up after age 20 or
so.
• Port-wine stain (one form of "nevus flammeus"),
fashionable in the Gorbachev era, can be treated by
laser.
• Sturge-Weber syndrome: a hemangioma in the
meninges, generally with an overlying port-wine stain
in the check the eyelids.
Glomangiomas (glomus tumors)
Painful tumors of the smooth muscle of the
human glomus organs, little
thermoregulatory left-overs from
mammalian evolution and found in
• the fingertips,
• toes,
• coccyx.
Telangiectasias
"Dilatations of the ends of little vessels"
• Osler-Weber-Rendu telangiectasias result
from any of several autosomal dominant
genes.
• Patients have little vascular malformations
connecting little arteries and little veins along their
whole GI tract (often easiest to see on the lips),
and often lots of other places.
• These are prone to bleed.
• The familiar "liver spider" is a centrally dilated
artery supplying several little arterioles which
blanch when the "spider's body" is pressed.
Lymphangiomas
• Hamartoma
• The best-known is "cystic hygroma" of
the necks of babies.
Tumorlike conditions
• Pyogenic granuloma:
• oral and skin growths;
• granuloma gravidarum: if pops up the
gums of pregnant women grossly, it looks
like a rotten cherry and bleeds very easily
• Microscopically: granulation tissue.
MALIGNANT TUMORS of VESSELS
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Hemangioendothelioma
Angiosarcoma
Hemangiopericytoma
Kaposi's sarcoma
Lymphangiosarcoma
Hemangioendothelioma
• Low-grade malignancy of the endothelium.
Angiosarcoma
• Best-known is epidemic hepatic angiosarcoma,
caused by exposure to vinyl chloride or
"Thorotrast" contrast medium.
• At other sites, they often follow therapeutic
radiation.
• Epithelioid angiosarcomas (epithelioid
hemangioendotheliomas) are common, and
look like carcinomas.
Hemangiopericytoma
• Low-grade malignancy of the
pericytes.
• The tumor cells interlace with
vessels, beautifully demonstrated
in reticulin-stained preparations.
Kaposi's sarcoma
• The cause is herpesvirus 8 (HHV-8).
• Classic Kaposi's sarcoma mostly involved the
legs of older men, and seldom caused major
problems.
• Epidemic non-AIDS related Kaposi's is a
disease mostly affecting young men in central
Africa. It's more aggressive than classic
Kaposi's, but not so much as in AIDS-related
Kaposi's.
• Renal transplant patients are prone to yet
another Kaposi's variant.
Lymphangiosarcoma
• Cancers of the lymphatics,
• Generally arising in lymphedema:
• after mastectomy (Stewart-Treves
syndrome),
• after exposure to radiation.
HEART TUMORS
• Metastatic Neoplasms
• The most common tumor of the heart is a metastatic
tumor; tumor metastases to the heart occur in about
5% of patients dying of cancer.
• In descending order these tumors are carcinoma of
the lung, lymphoma, breast cancer, leukemia,
melanoma, carcinomas of the liver, and colon.
• Primary Neoplasms
• Primary cardiac tumors are uncommon; in addition,
most primary cardiac tumors are also (thankfully)
benign.
• In descending order of frequency (adults) the primary
cardiac tumors are: myxomas, fibromas, lipomas,
papillary fibroelastomas, rhabdomyomas, and
angiosarcomas (this last one is malignant).
• Benign neoplasms occur 3 times more often
than malignant tumors.
• Of these sarcomas, angiosarcomas are the most
common histologic type and occur more
frequently in males. These tumors seed blood
directly, thus metastases are common and
widespread.
• Signs and symptoms of these tumors at
presentation are generalized, nonspecific,
and mimic several other systemic diseases.
• Right-sided tumors
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congestive heart failure (CHF),
edema,
jugular venous distention,
Ascites,
pericardial effusions.
Vena cava syndrome, pulmonary embolism, and restrictive
cardiomyopathy are some of the complications.
• Left-sided tumors
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embolism,
ischemic attacks,
cerebrovascular and peripheral-vascular accidents.
Based on their size and position, they may induce
arrhythmias and interfere with ventricular compliance.
Benign tumors
• Rhabdomyoma
• Hamartoma
• most frequently found tumors in children.
• They are associated with tuberous sclerosis
in about 50-80% of patients.
• These tumors are frequently multiple,
involving ventricular free and septal walls,
and have a yellowish-gray color.
• They vary from small to extremely large.
• Fibromas
(Papillary fibroelastoma)
• Hamartoma
• Usually single and large,
• most commonly in the left
ventricular free wall,
• 40% are diagnosed in infants
younger than 1 year.
• These tend to be firm
nonencapsulated tumors derived
from fibroblasts.
• It looks like a sea anemone on the
pulmonic valve.
• can cause embolic stoke.
• Teratomas
• Single, encapsulated, grayish-tan tumors
• most often in the pericardium.
• Angiomas
• A vascular tumor
• can occur in any part of the heart (with a
preference for right-sided chambers)
• hemangioma or lymphangioma.
• Hemangiomas are red, hemorrhagic, sessile, or
polypoid subendocardial nodules that vary from
small to large and occasionally have been
associated with hemorrhagic cardiac tamponade.
• These vascular vessels communicate between
themselves within the myocardium.
• They can infiltrate the intraventricular septum
near the conduction system where they may
cause heart block.
• Myxoma
• A myxoma is a benign tumor, usually irregular in
shape and jellylike in consistency.
• Half of all primary heart tumors are myxomas.
• A lesion of endothelial origin that arises as a ball from
the atrial septum and fills the left atrium.
• Grossly, it's a typically benign, soft tumor.
• The tumor may damage the mitral valve.
• Myxomas can plug the mitral valve (sudden death), or
cause emboli.
• They often are found attached to the atrial septum and
mitral valve apparatus in the left atrium (>85%).
• Histologic Findings:
• In general, myxomas are globular, hard,
and mottled lesions with hemorrhage.
• Histologically, they are composed of stellate
or globular myxoma cells, endothelial cells,
macrophages, mature or immature smooth
muscle cells, and a variety of intermediate
forms embedded in an abundant acid
mucopolysaccharide ground substance.
• Atrial myxomas
• In adults, myxomas are the most common
primary tumor of the heart.
• They arise in any of the 4 chambers or on the
heart valves; however, about 90% are located in
the atria. Myxomas in the atria have a left-to-right
ratio of approximately 4:1.
• Myxomas are mostly single and rarely
multiple in several chambers.
• Tumors can be 1-10 cm or larger in
diameter.
• The tumors can be sessile or pedunculated.
• Complications:
• Stroke can be a complication.
• Recurrent myxomas are common in patients
with Carney complex.
• The most common postoperative complication
is atrial dysrhythmia.
• Atrial myxomas may create ball-valve
obstructions that cause unexpected syncopal
attacks, cardiac insufficiency, and sudden
death in apparently healthy young children
and adults.
• Cerebellar ischemic stroke can result from
emboli from an atrial myxoma.
• Embolization to the brain, kidneys, and lungs
can also occur.
• May even occur in utero.
Carney complex
• A familial multiple neoplasia and
lentiginosis syndrome:
• (1) primary pigmented nodular adrenocortical
disease (PPNAD), a pituitary-independent,
primary adrenal form of hypercortisolism;
• (2) lentigines, ephelides, and blue nevi of the
skin and mucosae;
• (3) a variety of nonendocrine and endocrine
tumors.
• myxomas (skin, heart, breast, and other sites);
psammomatous melanotic schwannoma; growth
hormone–producing pituitary adenoma; testicular
Sertoli-cell tumor.
• Echocardiograms revealed dense
echoes arising from the space
between the mitral leaflets; these
findings were consistent with the
histologic features of a myxoma.
• Myxomas recur in approximately 1222% of familial cases and in about 12% of sporadic cases.
Malignant tumors
• Cardiac angiosarcoma
• Rhabdomyosarcoma
• Fibrosarcoma
Complications:
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arrhythmia,
congestive heart failure,
thromboembolism,
decrease in ventricular function,
Metastasis.