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61 Cards in this Set
- Front
- Back
List the cardiac tumors:
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*Benign:
-Myxoma -Lipoma -Lipomatous hypertrophy of septum -Hamartoma/Rhabdomyoma -Fibroma -Papillary fibroelastoma -Congenital cystic tumor of AV node *Malignant. |
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Discuss Benign Cardiac Neoplasms in general:
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*Benign refers to pathological features and lack of metastases or invasion.
*Can be clinically malignant due to size and location. |
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Discuss Myxoma in general:
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*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. |
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*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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Discuss histology of myxomas:
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*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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*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. |
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Discuss Lipomas of the heart:
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*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. |
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*Lipomatous Hypertrophy of Atrial Septum.
*Non-neoplastic lesion consisting of hyperplastic fat in continuity with epicardial fat. |
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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). |
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Discuss Hamartomas of the heart:
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*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. |
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Discuss Cardiac rhabdomyoma:
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*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. |
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What syndrome can Cardiac rhabdomyoma be a part of?
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*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. |
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Describe Tuberous Sclerosis a bit:
genetics of it-- other lesions besides cardiac rhabdomyoma-- |
*Though autosomal dominant, emergence of TS is de novo in 2/3 of cases.
*Associated with defects in tumor suppressor genes: -TSC 1 (9q34). -TSC2 (16p13). *Other TS lesions include renal cysts and angiomyolipomas, hypomelanotic skin nodules, and angiofibromas, “tubers” in CNS. |
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LV Rhabdomyoma in the ventricular wall. You can see how this could jack with conduction, flow, etc.
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Rhabdomyoma. Large, rounded polygonal cells with strands of cytoplasm interspersed among glycogen-rich vacuoles – “spider cells” (arrow).
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Discuss Fibromas:
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*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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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.
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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. |
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Discuss Congenital Cystic Tumor of the AV Node:
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*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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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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Discuss Malignant Cardiac Neoplasms:
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*Primary are rare but of many varieties.
*Sarcomas as elsewhere; fibrosarcoma, angiosarcoma, rhabdomyosarcoma, malignant fibrous histiocytoma, et al. *Lymphomas more likely in immuno-suppressed, esp. post-transplant patients. *Metastatic tumors much more common. *Neoplasms may be of any germ layer, from any non-CNS organ. *Via lymphatics, bloodstream, or extension from surrounding structures. |
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*Metastatic lesions to the epicardial surface of the heart.
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*Upper right: metastatic melanoma in atrium.
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*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." |
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List the vascular tumors:
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*Ectasias.
*Infectious: Bacillary angiomatosis, peliosis. *Neoplasms: 1) Benign: Hemangiomas. Lymphangiomas. Glomus tumor. 2) Sarcomas: -Malignant: Angiosarcoma. -Intermediate grade: Kaposi’s sarcoma. |
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What are vacular ectasias?
Discuss the most common ectasia: |
*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]. |
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Discuss Spider telangiectasia:
Discuss Hereditary hemorrhagic telangiectasia: |
*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. |
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Discuss Bacillary Angiomatosis:
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*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 disease. |
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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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*Bacillary peliosis (hepatitis).
*Gross swiss cheese appearance. Dilated sinusoidal spaces lined by littoral cells. Littoral cells represent sinusoidal endothelial and Kupffer cells. |
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Discuss vascular neoplasms in general:
what do they derive from? what markers can we look at? |
*Endothelial cells/supporting cells derived.
*Benign or malignant: -Benign: easily recognizable vessels formed. -Malignant: more cellular, show atypia and/or mitotic figures. *Markers such as CD31, CD34, vWF are useful to identify endothelial origin. *May be due to mutations in TGF-ß binding proteins (studies on Osler-Rendu-Weber). |
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Describe Hemangiomas in general:
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*Benign lesions.
*Common in infancy and childhood. *Usually localized. *Regress before puberty. *Usually in skin but can occur in internal organs. *Many varieties/subtypes. |
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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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*CAPILLARY HEMANGIOMA.
*Aggregates of closely packed, thin-walled capillaries, lined by endothelium; no capsule. |
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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. |
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Discuss Lobular hemangioma:
AKA? What does histology show? |
*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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*Lobular hemangioma, high power view
*Vascular spaces lined by endothelial cells. *Plump spindle cells between. These cells have features of pericytes. |
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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. |
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Describe Glomus Tumor (Glomangioma):
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*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.
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2 grades of vascular sarcomas?
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Malignant (angiosarcoma) and intermediate (Kaposi's).
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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
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*Hemangiosarcoma.
*Often confusing immunohistochemical pattern but most express CD31. *Weibel-Palade bodies (arrow) usually present on electron microscopy. |
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Discuss the 4 forms of KS:
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*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. |
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*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. |
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How do lesions change in KS?
What's the diagnostic lesion? |
*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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*KS; earlier lesion.
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Progression of lesions in Kaposi sarcoma:
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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. |
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Viral association of KS?
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*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. |