L9, CVS Pathology - PDF

pathology - CVS Cardiac tumors & Pericardial diseases LECTURE (9) Cardiac tumors & Pericardial diseases Dr. M. Shalaby pathology - CVS Cardiac tumors & Pericardial diseases  Primary cardiac tumors are uncommon.  Most...

pathology - CVS Cardiac tumors & Pericardial diseases LECTURE (9) Cardiac tumors & Pericardial diseases Dr. M. Shalaby pathology - CVS Cardiac tumors & Pericardial diseases  Primary cardiac tumors are uncommon.  Most also are benign.  The five most common have no malignant potential and account for 80% to 90% of all primary heart tumors.  In descending order of frequency, these are: ① Myxomas. ② Fibromas. ③ Lipomas. ④ Papillary fibroelastomas. ⑤ Rhabdomyomas.  Angiosarcomas constitute the most common primary malignant tumor of the heart. Dr. M. Shalaby pathology - CVS Cardiac tumors & Pericardial diseases CARDIAC MYXOMA  The most common primary tumors of the adult heart. INCIDENCE  Roughly 90% are atrial, with the left atrium accounting for 80% of those.  Almost always single.  Classically arising in the region of the fossa ovalis (atrial septum). GROSSLY  Can be sessile or pedunculated.  Most often manifesting as soft, translucent, villous lesions with a gelatinous appearance.  Myxomas are composed of frequently multinucleated stellate cells, that MICROSCOPICALLY are embedded in an abundant acid mucopolysaccharide ground substance.  The major clinical manifestations of myxomas are due to valvular “ball- C/P valve” obstruction, embolization, or a syndrome of constitutional signs and symptoms including fever and malaise. TTT  Surgical resection is almost uniformly curative. Dr. M. Shalaby pathology - CVS Cardiac tumors & Pericardial diseases RHABDOMYOMAS  Are the most frequent primary tumors of the heart in infants and children INCIDENCE  They frequently are discovered owing to valvular or outflow obstruction.  They occur with high frequency in patients with tuberous sclerosis.  Gray-white masses up to several centimeters in diameter that protrude GROSSLY into ventricular chambers.  Mixed population of cells.  Most characteristic are large, rounded, or polygonal cells containing MICROSCOPICALLY numerous glycogen-laden vacuoles separated by strands of cytoplasm running from the plasma membrane to the centrally located nucleus, so- called “spider cells”. TERATOMA INCIDENCE  The second most common cardiac tumors in infants.  Usually arise from the pericardium. CHARACTER  Generally benign.  Recurrence is rare Dr. M. Shalaby pathology - CVS Cardiac tumors & Pericardial diseases  Cardiac metastases can occur by: ① Direct extension. ② Blood stream. ③ Lymphatics. ④ Intracavitary diffusion.  Pericardial metastasis is the most common. BENIGN TUMORS MALIGNANT TUMORS  Composed of vascular channels filled with  More cellular, show cytologic atypia, are blood cells or lymph proliferative, and usually do not form well-  Lined by a monolayer of normal-appearing organized vessels. endothelial cells.  Most common vascular tumor of head & neck in infancy.  7% of all benign tumors of infancy and childhood.  Most are present from birth and initially increase in size, but many eventually regress spontaneously. INCIDENCE  While hemangiomas typically are localized lesions confined to the head and neck, they occasionally may be more extensive (angiomatosis) and can arise internally.  Nearly one third of these internal lesions are found in the liver. ① Capillary hemangiomas:  Most common type.  Occur in skin, SC tissues, and mucous membranes of oral cavities and lips, as well as liver, spleen, and kidneys.  Histologically: composed of thin-walled capillaries with scant stroma. TYPES ② Juvenile hemangiomas (strawberry hemangiomas Of newborn):  Of newborn Skin  Extremely common (1 in 200 births) & can be multiple.  Grow rapidly for a few months but then fade by 1 to 3 years of age, with complete regression by 7 years of age in majority of cases. Dr. M. Shalaby pathology - CVS Cardiac tumors & Pericardial diseases ③ Cavernous hemangiomas:  Composed of large, dilated vascular channels.  Compared with capillary hemangiomas, cavernous hemangiomas are more infiltrative, frequently involve deep structures, and do not spontaneously regress.  On histologic examination, the mass is sharply defined but unencapsulated and is composed of large blood-filled vascular spaces separated by connective tissue stroma.  More often the tumors are of little clinical significance, but they can be cosmetically troublesome and are vulnerable to traumatic ulceration and bleeding.  Brain hemangiomas are problematic, because they may cause symptoms related to compression of adjacent tissue or may rupture. CAPILLARY HEMANGIOMAS CAVERNOUS HEMANGIOMA (LIVER) Dr. M. Shalaby pathology - CVS Cardiac tumors & Pericardial diseases INCIDENCE  Benign lymphatic counterpart of hemangiomas. ① Simple (capillary) lymphangiomas:  Slightly elevated  Up to 1 to 2 cm in diameter  Occur predominantly in head, neck, and axillary SC tissues.  Histologically:  Composed of networks of endothelium-lined spaces.  Distinguished from capillary channels only by absence of blood cells.  Malignant transformation is rare. TYPES ② Cavernous lymphangiomas (cystic hygromas)  Typically are found as fluid filled sac in neck or axilla of infants.  Can be large (up to 15 cm), filling the axilla or producing gross deformities of neck.  Composed of massively dilated lymphatic spaces lined by endothelial cells and separated by intervening connective tissue stroma containing lymphoid aggregates.  The tumor margins are indistinct and unencapsulated, making definitive resection difficult. Dr. M. Shalaby pathology - CVS Cardiac tumors & Pericardial diseases  Kaposi sarcoma herpesvirus (KSHV, also known as human herpesvirus-8, ETIOLOGY or HHV-8).  Common in HIV patients.  In classic Kaposi sarcoma (and sometimes in other variants), the cutaneous lesions progress through three stages: MORPHOLOGY ① Patch ② Plaque ③ Nodule PATCH STAGE PLAQUE STAGE NODULAR STAGE N/E:  Patches are pink, red, or  Larger. purple macules  Accompanied by nodal and  Violaceous.  Typically confined to distal visceral involvement.  Raised plaques. lower extremities. M/E:  Dilated, irregular, and  Composed of plump,  Composed of dilated, angulated blood vessels proliferating spindle cells. dermal vascular channels  Lined by ECs and  Located in dermis or SC  Lined and surrounded by interspersed infiltrate of tissues, with interspersed plump spindle cells. chronic inflammatory cells, slit-like spaces. sometimes containing  Other prominent features: hemosiderin.  Hemorrhage and ① Extravasated red cells hemosiderin deposition are ② Hemosiderin-laden  Can be difficult to more pronounced, and macrophages distinguish from granulation mitotic figures are ③ Other mononuclear cells. tissue common. Dr. M. Shalaby pathology - CVS Cardiac tumors & Pericardial diseases PATCH STAGE PLAQUE STAGE NODULAR STAGE Dr. M. Shalaby pathology - CVS Cardiac tumors & Pericardial diseases  Malignant endothelial neoplasms ranging from highly differentiated DEFINITION tumors resembling hemangiomas to wildly anaplastic lesions.  Older adults are more commonly affected. AGE & SEX  No sex predilection.  Lesions can occur at any site, but most often involve skin, soft tissue, SITE breast, and liver.  Angiosarcomas are aggressive tumors that invade locally and metastasize.  Angiosarcomas can arise in the setting of lymphedema, classically in the ipsilateral upper extremity several years after radical mastectomy (i.e., CLINICAL PATTERN with lymph node resection) for breast cancer.  In such instances, the tumor presumably arises from lymphatic vessels (lymphangiosarcoma).  In skin, angiosarcomas begin as small, sharply demarcated, asymptomatic red nodules. GROSSLY  More advanced lesions are large, fleshy red-tan to gray-white masses with margins that blend imperceptibly with surrounding structures.  Necrosis and hemorrhage are common.  The extent of differentiation is e xtremely variable, ranging from plump atypical ECs that form vascular channels to undifferentiated spindle cell MICROSCOPICALLY tumors without evident blood vessels.  The EC origin can be demonstrated in the poorly differentiated tumors by staining for the EC markers CD31 and von Willebrand factor. Dr. M. Shalaby pathology - CVS Cardiac tumors & Pericardial diseases ① Trauma. ② Drugs. ③ Heart tumors. ④ SLE. ⑤ Uremia ⑥ Radiotherapy. ⑦ Hypothyroidism. Dr. M. Shalaby