Pathology: Tumors Of Heart And Vessels

Front 1

Discuss Myxoma in general:

Back 1

*Most common tumor of the heart in adults.
*Grows from endocardium to form sessile or polypoid mass; may be pedunculated.

*Grossly, gelatinous and friable.
*Often fragment and embolize.
*Often hemorrhagic.

Histology:
*Stellate or globular “myxoma cells.”

*Also smooth muscle cells and endothelial cells in abundant mucopolysaccharide matrix.

*“Myxoma cells” thought to originate from primitive multipotential mesenchymal cells.

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Back 2

*myxoma in the atria.

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*myxoma in the atria.
*Note how it's pushing down on the mitral valve.

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*Myxoma. Gloopy!
*MPS matrix visible at right arrowheads.
*Myxoma cells at arrow.

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L: Myxoma. Hemorrhagic, acid mucopolysaccharide ground substance, with myxoma cells.

R: Myxoma cells are stellate or globular. It is controversial as to whether these cells are neoplastic.

Front 6

Discuss Lipomas of the heart:

Back 6

*Occur anywhere in the heart.

*Grossly and histologically similar to lipomas anywhere; encapsulated.

*More common is “lipomatous hypertrophy” of the atrial septum; a.k.a. lipomatous hamartoma.

*Myocytes are entrapped in the mass.

*Patients are subject to arrhythmias.

Front 7

Back 7

*Lipomatous Hypertrophy of Atrial Septum.
*Non-neoplastic lesion consisting of hyperplastic fat in continuity with epicardial fat.

Front 8

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*Lipomatous Hypertrophy of Atrial Septum.
L: Adipose tissue separating myofibers.
R: Fat cells, including those with vacuoles, and two cells resembling brown fat (fetal fat).

Front 9

Discuss Hamartomas of the heart:

Back 9

*Excessive focal overgrowth of cells and tissue in a proper location in the body.

*“A mass of tissue gone awry.”

*Separation of hamartomas from benign neoplasms is not an exact science.

Front 10

Discuss Cardiac rhabdomyoma:

Back 10

*A Hamartoma of fetuses and infants.

*Benign histologically.

*Can be fatal, depending on size and location:
-Can cause obstruction to inflow or outflow tracts.
-Can compress conduction system.

*Gray-white masses in the myocardium of left or right heart of variable size.

*Often part of an hamartoma syndrome: tuberous sclerosis (TS):

-If a single rhabdomyoma is present: 50% or more pts will have tuberous sclerosis.

-If multiple rhabdomyomas are present: 100% of pts will have tuberous sclerosis.

*Inversely, 47-67% of tuberous sclerosis patients have rhabdomyomas.

*Other TS lesions include renal cysts and angiomyolipomas, hypomelanotic skin nodules, and angiofibromas, “tubers” in CNS.

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Back 11

LV Rhabdomyoma in the ventricular wall. You can see how this could jack with conduction, flow, etc.

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Back 12

Rhabdomyoma. Large, rounded polygonal cells with strands of cytoplasm interspersed among glycogen-rich vacuoles – “spider cells” (arrow).

Front 13

Discuss Fibromas:

Back 13

*A tumor of fibrous tissue with benign histopathological features.
*Usually solitary lesions in ventricular myocardium, especially the septum.
*As with rhabdomyoma, clinical behavior depends on location and size.

*Associated with Gorlin syndrome – a familial disorder with bifid ribs, multiple nevoid basal cell carcinomas, and cysts of the jaw.

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Back 14

Trichrome stain shows myocardium (red) interdigitating with a fibroma (blue). Elastic tissue may be prominent. Except for location, cardiac fibromas are similar to those elsewhere. Though rare, fibromas are the second most common primary cardiac tumor in children.

Front 15

Discuss Papillary Fibroelastoma:

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*Derived from endocardium.

*Fibrous tissue, SM cells, elastic fibers in MPS matrix.

*Bouquet of filiform threads attached to endocardium by a short pedicle.

*Resemble sea anemones.
*Also resemble Lambl excrescences.

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Papillary Fibroelastoma

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Papillary Fibroelastoma.
Microscopic: myxoid connective tissue with abundant polysaccharide matrix and elastic fibers; endothelial cover.

Front 18

Discuss Congenital Cystic Tumor of the AV Node:

Back 18

*Originally called a mesothelioma.
*MAY arise from endodermal rests.
*MAY be Of mesothelial origin.
*EM shows microvilli.
*Pathologically benign but clinically malignant causing AV block.

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Back 19

Congenital Cystic Tumor of the AV Node
L: Spongy, Swiss cheese-looking mass in the region of the AV node.
R: Cysts lined by flat or cuboidal epithelium, lying in a dense stroma.

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Back 20

*Metastatic lesions to the epicardial surface of the heart.

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Back 21

*Upper right: metastatic melanoma in atrium.

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Back 22

*OKAY. WHOA.
*Intravenous Leiomyomatosis.
*39 year old female with positional syncope.
*Echogenic mass from the IVC, occupying 70% of RA and extending into RV during diastole.
*"BENIGN."

Front 23

What are vacular ectasias?

Discuss the most common ectasia:

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*Localized dilatation of existing vessels.

*Nevus flammeus is the most common:
-“ordinary birthmark.”
-Head and neck, flat, pink to purple.
-Usually fade and regress.
-“Port wine stain”- grows with child.
[If in trigeminal nerve distribution, consider Sturge-Weber syndrome].

Front 24

Discuss Spider telangiectasia:

Discuss Hereditary hemorrhagic telangiectasia:

Back 24

*Spider telangiectasia:
-Pulsatile array of vessels, subcutaneous.
-Blanches when pressed.
-Often in pregnant women and cirrhotic patients.

*Hereditary hemorrhagic telangiectasia:
-(Osler-Weber-Rendu disease).
-Telangiectasia are genetic malformations.
-Present from birth on many mucosal surfaces.

Front 25

Discuss Bacillary Angiomatosis:

Back 25

*Caused by Bartonella species in immunocompromised patients.
*Vascular proliferation of unknown cause.
*Skin, bone, brain, and other organs.

*Proliferating capillaries, endothelial cells show atypia and mitoses.
*Neutrophils, nuclear dust, and granular material present.

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*Bacillary Angiomatosis
*Cherry hemangioma-like papules and larger lesion resembling a pyogenic granuloma. Patient has advanced HIV.

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*Bacillary Angiomatosis.
*Lymph node from a 17 year old renal transplant patient.
Insert shows clusters of plump endothelial cells without visible vascular lumens.
*The granular and amorphous material showed rod-shaped organisms on Warthin-Starry stain. DNA technology confirmed B. henselae.

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Back 28

*Bacillary peliosis (hepatitis).
*Gross swiss cheese appearance. Dilated sinusoidal spaces lined by littoral cells. Littoral cells represent sinusoidal endothelial and Kupffer cells.

Front 29

ID and discuss:

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*Most common type of CAPILLARY HEMANGIOMA; strawberry type in newborns.
*Bright red to blue, flat or slightly elevated, covered by intact epidermis.
*Occasionally pedunculated.

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Back 30

*CAPILLARY HEMANGIOMA.
*Aggregates of closely packed, thin-walled capillaries, lined by endothelium; no capsule.

Front 31

Discuss Cavernous Hemangioma:

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*Red-blue and spongy.
*Rarely gigantic, e.g covering a side of the face.
*Distinguished by large, dilated vascular channels.
*May contain thrombi and calcification.
*VHL syndrome: lesions in cerebellum, liver, pancreas.

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*Cavernous Hemangioma.
*Large, dilated channels.
*No capsule but margins sharply defined.

Front 33

Discuss Lobular hemangioma:
AKA?
What does histology show?

Back 33

*AKA “pyogenic granuloma.”
*Exophytic red nodule on skin or oral mucosa.
*May ulcerate and bleed.
*Some follow trauma.

*Histology shows expansion of papillary dermis with lobular collections of vascular elements.

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Lobular hemangioma (pyogenic granuloma), low power view.

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Back 35

*Lobular hemangioma, high power view
*Vascular spaces lined by endothelial cells.
*Plump spindle cells between. These cells have features of pericytes.

Front 36

Describe Lymphangiomas:

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*Lymphatic analogues of hemangiomas.
*Most often in head and neck.
*Capillary and cavernous.
*Cavernous lymphangiomas are called cystic hygromas.
*Nuchal cystic hygromas are seen in Turner syndrome.

Front 37

Describe Glomus Tumor (Glomangioma):

Back 37

*Painful small lesions on digits.
*Derived from cells of the glomus body:
-Neuromyoarterial receptor for thermoregulation.
-Glomus cell is a modified smooth muscle cell.

*Consists of branching vascular channels separated by nests of glomus cells.
*Cells are small, round, and blue.

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Glomus Tumor, low power.

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Glomus Tumor, high power.

Front 40

2 grades of vascular sarcomas?

Back 40

Malignant (angiosarcoma) and intermediate (Kaposi's).

Front 41

Discuss Angiosarcoma:

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*Structure varies from well-differentiated to anaplastic.

*If appearance still much like an hemangioma the lesion is called an hemangiosarcoma.

*Arsenic, Thorotrast and polyvinyl chloride cause hepatic angiosarcomas.

*Lymphangiosarcomas may arise in areas of lymphedema or after radiation.

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Hemangiosarcoma

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Hemangiosarcoma

Front 44

Discuss the 4 forms of KS:

Back 44

*Chronic KS in older men (European):
-Multiple red-purple lesions on extremities.

*Lymphadenopathic KS (African):
-In younger people.
-Localized or generalized lymphadenopathy.
-Same geographic distribution as Burkitt lymphoma.
-Sparse skin lesions.

*Transplant-associated KS:
-Prominent in skin of highly immuno-suppressed. patients but can occur in other organs.

*AIDS-associated KS:
~33% of AIDS patients, usually homosexual males.
-Any location.
-33% develop lymphoma but most die of other AIDS manifestations.

*Human herpesvirus 8 in 95% of KS lesions, in both endemic and epidemic forms of the disease.

*The virus contains genes with strong homology with IL-6 and cyclin D2.

*It is a member of the gamma-herpesvirus subfamily; this group promotes lymphomas and other LP disorders.

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Back 45

*HIV positive male, cutaneous lesion.
*Could be Kaposi, but biopsy needed to differentiate from bacillary angiomatosis, or pyogenic granuloma, or other bacterial or fungal infections.

Front 46

How do lesions change in KS?

What's the diagnostic lesion?

Back 46

*Lesions tend to progress through different histologic stages with time.

*Granulation tissue--> diagnostic lesion--> fibrotic hemangioma.

*Diagnostic lesion composed of plump spindle cells with slit-like vascular spaces and/or extravasated RBCs.

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Back 47

*KS; earlier lesion.

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Progression of lesions in Kaposi sarcoma:

Back 48

Granulation tissue--> diagnostic lesion--> fibrotic hemangioma.

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*Kaposi sarcoma: diagnostic lesion.
*There's a slit-like vascular space on the left, and some others.
*Note RBCs outside of vessels.

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*KS; later lesion.
*Histology is just all fibrosis.