Pathology of the CVS Lec.6 (Veins & Lymphatics) PDF

College of Medicine & Health Sciences Histopathology Department Pathology of Cardiovascular system For 3rd year Medical student Veins and lymphatics  Learning Objectives Define Varicose vein Define Lymphangitis and Lymphedema Classify vascular tumors Discuss benig...

College of Medicine & Health Sciences Histopathology Department Pathology of Cardiovascular system For 3rd year Medical student Veins and lymphatics  Learning Objectives Define Varicose vein Define Lymphangitis and Lymphedema Classify vascular tumors Discuss benign, borderline, and malignant vascular tumors concerning etiology, pathogenesis and morphology.  Varicose veins of the extremities Are abnormally dilated tortuous veins produced by increased intraluminal pressures and weakened supporting wall. It involved the superficial veins up to 20% in men and a third in female.  Risk factors. 1. Obesity, 2. Compression of inferior vena cava(by gravid uterus in pregnancy). 3. Familial tendency. Varicose veins of the leg (arrow).  Morphology Varicose veins show wall thinning at the points of maximal dilation with smooth muscle hypertrophy and intimal fibrosis in adjacent segments; elastic tissue degeneration and spotty medial calcifications (phlebosclerosis) also occur. Focal intraluminal thrombosis (due to stasis) and venous valve deformities (rolling and shortening) are common.  Clinical features of varicose veins 1. Lower extremities stasis and thrombosis. 2. Congestion and edema. 3. Ischemia and ulceration. 4. Varicose ulcer.  Sites : Esophageal varices. Liver cirrhosis (less frequently, portal vein obstruction or hepatic vein thrombosis) causes portal vein hypertension. Hemorrhoids are uncomfortable and may be a source of bleeding; they can also thrombose and are prone to painful ulceration.  Lymphangitis and Lymphedema Primary disorders of lymphatic vessels are extremely uncommon; secondary processes are much more common and develop in association with inflammation or malignancies.  Lymphangitis Acute inflammation of the lymphatic vessels caused by bacteria. Clinically the inflamed lymphatic vessels appear as red, painful, subcutaneous streaks, tender enlargement of draining lymph nodes ( acute lymphadenitis), bacteria may pass to blood causing bacteremia and sepsis  Lymphedema Primary lymphedema can occur as an isolated congenital defect or as familial resulting from agenesis or hypoplasia of lymphatics. Secondary obstructive lymphedema caused by: 1. Neoplasm. 2. Surgical procedures. 3. Post radiation fibrosis. 4. Filariasis such as elephentiasis. 5. Post inflammatory.. Regardless of the cause lymphedema leading to: 1. Increase hydrostatic pressure and edema. 2. Chronic edema lead to deposition of ECM and fibrosis such as elephentiasis. 3. Produce a peau d’ orange appearance of overlying skin such as lymphedema seen I the skin of breast due ta carcinoma. 4. Skin ulceration. 5. Rupture and accumulation of lymph in various spaces- chylous ascites, chylothorax, chylopericardium. Chronic lymphedema (elephantiasis).  Vascular Neoplasms Classification of vascular neoplasms A. Benign, developmental and acquired lesions. 1. Vascular ectasias such as nevus flammeus, spider telangiectasia, hemorrhagic telangiectasia. 2. Hemangioma (capillary hemangioma, cavernus hemangioma, pyogenic granuloma) 3. Lymphangioma (simple capillary lymphangioma, Cavernous lymphangioma (cystic hygroma). 4. Glomus tumor. B. Intermediate grade neoplasms. 1. Kaposi sarcoma. 2. Hemangioendothelioma. C. Malignant neoplasms. Angiosarcoma.  Benign developmental lesions (Tumor-like conditions) Telangiectasia. Permanent dilation of small blood vessels capillary, venules and arterioles usually in the skin and mucous membrane that form discrete red lesion. These lesion are congenital or acquired and are not true neoplasms and include: 1. Nevus flammeus ( birthmark). 2. Spider telangiectasias. 3. Hereditary hemorrhagic telangiectasia.  Benign neoplasms Tumor usually contains obvious vascular channels filled with blood cells or lymph that are lined by monolayer of normal appearing endothelial cells. 1. Hemangioma. More common tumor in infancy and childhood can be localized or extensive. Histological variants. - Capillary hemangiomas. - Cavernous hemangioma. - Juvenile hemangiomas. - Pyogenic granuloma  Hemangioma Hemangiomas are benign growths of blood vessels that are very common in children. Though called "birthmarks", they often do not become noticeable until a few days to weeks after birth. Hemangiomas grow in size for about 6-12 months, then shrink or "involute" over a period of years. Most hemangiomas do not cause problems and thus do not need treatment. Sometimes however, hemangiomas may cause ulceration (when a painful sore develops on the hemangioma), block an important organ such as the eye, grow very rapidly, or be of cosmetic concern to the child and/or family. 2. Lymphangioma variants. A. Simple capillary lymphangioma. B. Cavernous lymphangioma (cystic hygroma). Lymphangioma Represent the lymphatic counterpart of hemangioma and characterized by cystic spaces and cavernous spaces lined by endothelial cells and surrounded by lymphoid aggregates, the spaces usually contains pale fluid  Intermediate –Grade ( Borderline ) Tumors I. Kaposi sarcoma (KS). A vascular neoplasms caused by Kaposi sarcoma herpesvirus(KSHV) and most common in patients with AIDS. Types of KS : 1. Classic KS. 2. Endemic African KS. 3. Transplantation-associated KS. 5. AIDS-associated KS.  Pathogenesis of KS All KS lesions are infected by KSHV that transmitted sexually and non-sexually. Somatic mutation and the local inflammation favors cellular proliferation and progression of endothelial cells.  Morphology of KS. Grossly may be patches, raised plagues or nodular lesions  Microscopically shows sheets of spindle-shape cells and slitlike vascular spaces. Microscopic picture of Kaposi sarcoma  II. Hemangioendothelioma. The tumor cells are plump and cuboidal and do not form well-defined vascular channels and they can mistaken for metastatic epithelioid or melanomas.  Malignant tumor (Angiosarcoma) More cellular and show cytological atypia, and not form well- organized vessels. It range between highly differentiated to anaplastic undifferentiated. It affect the older adults in any site.  Morphology Gross: Large lesion with fleshy red-tan to gray white masses with necrosis and hemorrhage. Microscopic : Ranging from plump atypical endothelial cells that form vascular channels to undifferentiated spindle-shaped cells without discernible blood vessels. The endothelial cells in poorly differentiated lesions can be demonstrated by immunohistochemistry staining for detection of endothelial cells markers CD13 and von-Willebrand factor. The tumor are aggressive locally and shows distant metastasis.  Angiosarcoma Cystic lump behind the right ear. a soft and spongy lesion with purple streaks behind the right ear  Angiosarcoma Slit-like vessels with dissection of the dermal collagen were prominent. The vessels were 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. There were also focal clusters of solid tumor cells ‫تمنياتي لكم بالتوفيق والتفوق الدائم‬