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Thursday, September 09 2010 @ 11:14 PM CDT

Cardiovascular System
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These are the most common primary cardiac tumors. 90% are in the left atrium. They are currently believed to be true neoplasms. Grossly they are mucoid polypoid projections into the lumen of the atrium and are usually single. Histologically there are stellate spindle cells that often aggregate around vessels ("lepidic cells").
Atrial Myxoma
Tuesday, April 14 2009 @ 10:49 PM CDT
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The material is strongly PAS positive and is a glycoprotein. It as associated with aging, but is less common than lipofuscin pigment. In pathologic conditions, it seen in cardiomyopathies and hypothyroidism.
Basophilic Degeneration of Myocytes
Tuesday, April 14 2009 @ 10:54 PM CDT
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myofiber disarray, which is the histologic hallmark of hypertrophic cardiomyopathy and usually present in more than 5% of the area of the interventricular septum. Gross characteristics are septal hypertrophy with more than 1.5:1 ratio of septum to left ventricular free wall thickness, thickened mitral valve, and atrial dilatation. About half cases demonstrate obstruction of the left ventricular outflow tract (subaortic stenosis); these cases demonstrate plaques in the subendocardium of the left ventricular outflow. Another microscopic feature is thickening of small intramyocardial coronary arteries. Symptoms include chest pain, syncope, arrhythmias and, late in the course, dyspnea and heart failure. 50% of cases present with sudden death, which is exacerbated by strenuous exercise.
Hypertrophic Cardiomyopathy
Tuesday, April 14 2009 @ 11:01 PM CDT
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Fabry's disease is by far the most likely storage disease to present in the heart. The diagnosis rests on biochemical studies and ultrastructure which demonstrates lamellated bodies of tribexosyl ceramide in the vacuoles. The disease is caused by a deficiency in alpha galactosidase, is X-linked, and is associated with multiple angiokeratomas of the skin (synonym: angiokeratoma corporis diffusum)
Fabry's Disease
Tuesday, April 14 2009 @ 04:01 PM CDT
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Histologically, nodules of clear vacuolated cells, some with a "spider" appearance, are seen. Most often, cardiac rhabdomyomas are multiple, and can cause symptoms of congestive heart failure, arrhythmias, sudden death, or can be asymptomatic. Seen in association with tuberous sclerosis
Cardiac Rhabdomyoma
Tuesday, April 14 2009 @ 03:53 PM CDT
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Retiform hemangioendothelioma, a recently described lesion is considered to be a variant of low grade angiosarcoma. This is a slow growing, exophytic or plaque like tumour. Rarely these lesions may be associated with radiotherapy or chronic lymphedema.Located in the dermis or subcutis ; characterized by 'long arborizing vessels' with features reminiscent of rete-testis ; focal solid areas composed of spindle and epithelioid cells may be present. Other features: Vessels lined by hobnail endothelial cells ; prominent stromal lymphocytic infiltrate; occasional intraluminal papillae with hyaline collagenous cores and lymphocytes within vascular lumen. Immunohistochemistry: Spindle and epithelioid cells in the focal solid area express endothelial markers. Differential diagnosis: Angiosarcoma and Dabska's tumour (lacks arborizing vessels and shows prominent papillary tufts) .
Retiform Hemangioendothelioma
Saturday, August 02 2008 @ 05:56 PM CDT
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In 1940, Kasabach and Merritt described a male infant with a discolored, indurated lesion on his left thigh that rapidly grew and affected the entire left leg, scrotum, abdomen, and thorax. In addition, the infant also had consumptive thrombocytopenia. This association has become known as Kasabach-Merritt syndrome (KMS). The original case is now known to have been associated with kaposiform hemangioendothelioma and thrombocytopenia. Site: Retroperitoneum, subcutaneous or deep soft tissue of upper extremities, head and neck area. Age: Occurs as solitary tumour in infants (first decade of life) . Tumour consists of infiltrating lobulated nodules of spindle shaped endothelial cells ; Nodules are separated by thick fibrous bands ; Spindle cells form short fascicles and line slit-like and round vessels ; Minimal cytological atypia ; Mitotic activity is low ; Epithelioid cells are sometimes present ; Cells may contain hemosiderin pigment, hyaline globules and cytoplasmic vacuoles ; Ectatic vessels are present in the periphery ; Scattered microthrombi within vascular spaces. Immunohistochemistry: The spindle cells stain positively for CD34 and focally for CD31. The pericytes around the capillaries stain positively for smooth muscle actin. Differential diagnosis : Kaposi's sarcoma (rare in children; multicentric; prominent chronic inflammatory infiltrate; lobular architecture is absent; dense fibrous bands are not present); infantile hemangioma ; acquired tufted hemangioma ; spindle cell hemangioendothelioma ; Angiosarcoma (endothelial atypia, increased mitotic activity and dermal collagen dissection by infiltrating tumour)
Kaposiform Hemangioendothelioma
Saturday, August 02 2008 @ 05:56 PM CDT
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An angiosarcoma (AS) is an uncommon malignant neoplasms characterized by rapidly proliferating, extensively infiltrating anaplastic cells derived from blood vessels and lining irregular blood-filled spaces. Specialists apply the term angiosarcoma to a wide range of malignant endothelial vascular neoplasms that affect a variety of sites. Angiosarcomas are aggressive and tend to recur locally, spread widely, and have a high rate of lymph node and systemic metastases. The rate of tumor-related death is high. The cause of angiosarcomas is usually unknown. The tumours may develop as a complication of a pre-existing condition. Certain patient groups may be at greater risk of developing angiosarcomas, these include: * Patients with chronic lymphoedema (accumulation of lymph fluid in the arms) whom have undergone a radical mastectomy for breast cancer (removal of breast and all lymph nodes under the arm) * Radiotherapy patients (especially those who have been exposed to radiation emitting contrast agent Thorotrast – this agent is no longer used but angiosarcomas have occurred in people decades after exposure) * Patients with foreign material (such as Dacron, shrapnel, steel, and plastic) in the body * Patients exposed to environmental agents such as sprays containing arsenic, and vinyl chloride in the plastic industry. The lesion a dermal neoplasm composed of infiltrating irregular, vascular or sinusoidal anastomosing channels. Slit-like vessels with dissection of the dermal collagen are prominent. The vessels are lined by a disorganized proliferation of polyhedral atypical endothelial cells with a high nucleus to cytoplasm ratio, scant amphophilic cytoplasm, and dark blue nuclei. Micropapillae of the neoplastic cells with a hobnail appearance were seen projecting into the lumen of the neoplastic vessels are usually seen. By immunohistochemical staining, the neoplastic cells show strong positivity to CD31, CD34 and Factor VIII
Angiosarcoma
Saturday, August 02 2008 @ 05:56 PM CDT
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Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon idiopathic condition that manifests in adults as isolated or grouped papules, plaques, or nodules in the skin of the head and neck. Most patients present with lesions in the periauricular r
Angiolymphoid Hyperplasia with Eosinophils
Saturday, August 02 2008 @ 05:56 PM CDT
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Cystic medial degneration is a disorder of large arteries, in particular the aorta, where there is a focal degeneration of the elastic tissue and the muscle in the tunica media (the middle layer of the artery), with the presence of mucoid material in the
Cystic Medial Degneration
Saturday, August 02 2008 @ 05:55 PM CDT
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Cardiac papillary fibroelastoma (PFE) is a rare, benign, slow-growing tumor of the endocardium. Whether it represents a reactive tumoral lesion or a true neoplasm remains a matter of debate. Overall, the most common site is the aortic valve. Histologic
Papillary Fibroelastoma
Saturday, August 02 2008 @ 05:55 PM CDT
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Intravascular papillary endothelial hyperplasia (Masson's hemangioma) is an unusual benign, non-neoplastic, vascular lesion characterized histologically by papillary fronds lined by proliferating endothelium. It may appear as a primary or pure form develo
Intravascular Papillary Endothelial Hyperplasia (Masson's hemangioma)
Saturday, August 02 2008 @ 05:55 PM CDT
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