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Heart-tumor

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Table of contents

Primary references, images needed

Cardiac tumors-benign: general, adenomatoid tumor, angiomyolipoma, atrioventricular node tumor, benign fibrous histiocytoma, blood cyst, calcified amorphous tumor, endocardial fibroelastosis, fibroma, granular cell tumor, hamartoma, hemangioma, inflammatory pseudotumor, Lambl’s excrescence, lipoma, lipomatous hamartoma, lipomatous hypertrophy, lipomatous infiltration, mesothelial/monocytic cardiac excrescence, myxoma, necrobiotic xanthogranuloma, papillary fibroelastoma, paraganglioma, rhabdomyoma, schwannoma, sugar tumor, tumor with perivascular myoid differentiation, varix

Cardiac tumors-malignant: angiosarcoma, chondrosarcoma, Ewing’s sarcoma/PNET, fibrosarcoma, germ cell tumor/teratoma, granulocytic sarcoma, hemangioendothelioma, hemangiopericytoma, Kaposi’s sarcoma, leiomyosarcoma, liposarcoma, lymphoma, malignant fibrous histiocytoma, malignant mesenchymoma, malignant peripheral nerve sheath tumor, metastases, osteosarcoma, post-transplant lymphoproliferative disorder, rhabdoid tumor, rhabdomyosarcoma, sarcoma-general, synovial sarcoma, undifferentiated sarcoma

Miscellaneous: features to report-tumors

 

Primary references

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American Journal of Clinical Pathology, (AJCP), January 1975 to May 2007

American Journal of Surgical Pathology (AJSP), March 1977 to May 2007

Archives of Pathology and Laboratory Medicine (Archives), January 1976 to May 2007

Biomed Center, 1 March 1997 to 2 May 2007

Human Pathology (Hum Path), March 1970 to May 2007

Modern Pathology (Mod Path-free full text after 1 year), January 1988 to April 2007

Mills: Sternberg's Diagnostic Surgical Pathology (4th ed), 2004

Rosai: Rosai and Ackerman's Surgical Pathology (9th ed), 2004

Tumors of the Heart and Great Vessels (AFIP Atlas of Tumor Pathology, Series 3, Vol 16); 1996

AFIP images (not copyrighted) courtesy of www.PathologyResources.com

Websites with images: PathoPic, PEIR digital library

Journal search terms: heart and each disease entity listed

Please refer to these primary references for more detailed discussions and additional images

 

Images needed for Heart-tumors

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Cardiac tumors-benign

General

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Occur in 1 per 300 to 100,000 autopsies

Common tumors are myxoma (30% of all primary cardiac tumors), papillary fibroelastoma (8%), rhabdomyoma, fibroma, hemangioma, lipomatous hypertrophy, AV nodal tumors, other (5% or less each)

In children, common tumors are rhabdomyoma, fibroma and teratoma (Orphanet J Rare Dis 2007;2:11)

References: http://www.cancer.org/downloads/PUB/DOCS/SECTION28/91.pdf

 

Adenomatoid tumor of the heart

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Very rare

Case reports: incidental 1 cm tumor (AJSP 1997;21:1378)

Micro: cords or tubules of large epithelioid cells without atypia that occasionally branch into anastomosing channels

Positive stains: AE1/AE3, CAM 5.2, EMA, calretinin, thrombomodulin (paratesticular tumors-Histopathology 2000;36:109, adrenal tumors-AJSP 2003;27:969)

Negative stains: CEA

 

Angiomyolipoma of heart

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Very rare

Associated with tuberous sclerosis

Case reports: 48 year old woman with dyspnea and right atrial mass (AJSP 1994;18:1164), massive (34 cm) right atrial tumor (J Formos Med Assoc 1997;96:653)

Micro: mixture of blood vessels, smooth muscle and fat

Positive stains: adipocytes - S100; smooth muscle cells - desmin, actin; most cells - CD117 (diffuse and cytoplasmic, AJSP 2002;26:493), HMB45, NKI-C3/CD63 (Archives 2001;125:751), MelanA/Mart1, microphthalmia transcription factor

DD: renal angiomyolipoma with thrombus extending into right atrium (Urol Int 2001;67:168, Nippon Hinyokika Gakkai Zasshi 1999;90:745)

 

Atrioventricular node tumor of heart

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Also called cystic tumor of AV node or mesothelioma of AV node, but does not appear to have mesothelial origin

Rare; usually identified at autopsy

May be congenital rests of endodermal origin (Archives 1990;114:1057) or ultimobranchial heterotopia identical to solid cell nests of thyroid (AJCP 2005;123:369)

Associated with other congenital anomalies

Often causes heart block and sudden death

Mean age 38 years, 75% female

Should examine conduction system in all patients with sudden death, particularly if a history of arrhythmia or heart block

Case reports: causing heart block for years before death (AJCP 1975;63:377), causing sudden cardiac death #1 (Archives 2001;125:573), #2 (Am J Forensic Med Pathol 2005;26:349), occurring in atrial septum (Cardiovasc Pathol 1999;8:325), congenital tumors (Cardiovasc Pathol 1999;8:233), with multiple endocrine abnormalities (Pathol Res Pract 1996;192:54)

Treatment: pacemaker implantation, anti-arrhythmic drugs, possibly surgical excision (Heart 2000;83:E6)

Gross: may not be visible due to small size (2 to 20 mm); multicystic lesion in area of atrioventricular node and membranous septum

Micro: must sample conduction system; solid and cystic areas lined by nonciliated, epithelial appearing cuboidal cells (main cells), mixed with occasional clear cells (neuroendocrine or C cells); lumina contains PAS+ diastase resistant material which may calcify; may have inflammatory cells and fibrosis; no smooth muscle, no mitotic figures, no atypia

Positive stains: main cells - CAM5.2, AE1/AE3, 34βE12, CK7, EMA, CEA, CA19.9, p63, bcl2, galectin 3

neuroendocrine or C cells - CAM5.2, AE1/AE3, CEA, calcitonin, chromogranin, synaptophysin, TTF-1

Negative stains: CK20, vimentin, CD31, factor VIII–related antigen, calretinin, estrogen receptor and progesterone receptor

EM: (a) cells form solid nests with well formed basement membranes, cytoplasmic tonofilaments and desmosomes or (b) glandular structures with desmosomes, electron-dense material and short microvilli

DD: bronchogenic cyst (solitary, grossly visible, on epicardial surface, smooth muscle present), mesothelial cyst (larger, unilocular, on surface of heart), teratoma (has neural or other ectodermal structures, Pediatr Pathol 1994;14:913)

 

Benign fibrous histiocytoma of heart

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Rare in heart

Case reports: 4 month old boy who died due to large cardiac tumor (Z Rechtsmed 1990;103:335)

Micro: storiform pattern of spindle cells and lipid laden histiocytes; no mitotic figures, no pleomorphism

Positive stains: alpha-1-antichymotrypsin, lysozyme, vimentin, factor XIIIa

Negative stains: CD68, CD34

 

Blood cyst of heart

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Congenital cyst found on endocardium, particularly along lines of closure of heart valves

More common on mitral and tricuspid valves

Actually diverticula (invagination of atrial endothelium into atrioventricular valves or ventricular endothelium into semilunar valves)

Incidental findings in 50% of infants under age 2 months on cardiac valves; rare after age 2 years

Rarely causes ventricular or valvular obstruction

Case reports: acquired after surgery (J Am Soc Echocardiogr 2003;16:377), on pulmonary valve (Hum Path 1997;28:252)

Gross: usually 3 mm or less; may be blue; 50% are multiple, may have up to 20 cysts

Micro: blood filled cystic cavity with fibromyxoid wall lined by flattened or cobblestone-shaped endothelium; filled with nonorganized blood

 

Calcified amorphous tumor of heart

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Endocardial based intracavity cardiac mass

Mean age 52 years

Clinically simulates neoplasm

Case reports: 2 cm mass in right ventricular wall involving chordae tendineae of tricuspid valve (Cardiovasc Pathol 2006;15:299), hemodialysis patients (Kyobu Geka 2006;59:851)

Treatment: excision is curative, although residual calcium may persist

Gross: firm, yellow-white, partially calcified

Micro: nodular calcium with degenerating blood elements and chronic inflammation

DD: calcified thrombi

References: Hum Path 1997;28:601

 

Endocardial fibroelastosis of heart

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Rare

Focal or diffuse fibroelastic thickening of mural left ventricular endocardium

Usually ages 0-2 years

Focal cases have no symptoms; diffuse cases may cause rapid cardiac decompensation and death (Transplant Proc 2006;38:1511, Am J Forensic Med Pathol 1999;20:357)

Associated with (a) cardiac anomaly, such as aortic valve obstruction, or response to other chronic prenatal cardiac stress (J Clin Pathol 1991;44:576); (b) congenital heart block and maternal anti-Ro or anti-La autoantibodies (Circulation 2002;105:843), (c) mumps or other viral infection (Circulation 1997;95:133)

Declining incidence, with almost no new cases, may be due to declining incidence of mumps (eMedicine)

Case reports: fibroelastosis in neonate with no other cardiac pathology (J Clin Pathol 1992;45:1042)

Treatment: heart transplant

Micro: increased fibroelastic tissue in endocardium and subendocardium; myocardium usually normal

References: OMIM 305300

 

Fibroma of heart

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Rare, but is common among cardiac tumors in children

Benign, congenital, probably a hamartoma; rare cases may represent fibromatosis with aggressive behavior

Presents with heart failure or cyanosis in 1/3, syncope or arrhythmia in 1/3, incidental/asymptomatic in 1/3

Found in 10% of Gorlin’s syndrome patients (nevoid-basal cell carcinoma syndrome)

Case reports: 8 month old boy with nevoid basal cell carcinoma syndrome (Tex Heart Inst J 2006;33:88), sudden death in 7 month old girl (J Forensic Sci 2000;45:731), sudden death in 6 year old boy (Circulation 2000;101:E168)

Treatment: excision, possibly transplant (Curr Opin Cardiol 2000;15:121)

Gross: discrete bulging mass, nearly always solitary, often calcification

Micro: bland fibroblasts and collagen; calcification common; more cellular in infants and children; mitotic figures in infants only; margin is infiltrative; occasionally perivascular lymphocytes and histiocytes, elastic fibers, myxoid stroma

EM: fibroblasts with extensive endoplasmic reticulum but few cellular organelles; incompletely developed/absent basement membrane, ramifying cytoplasmic processes, central nucleus with distinct nucleolus; also cells of intermediate differentiation with smooth muscle myofilaments

DD: fibrosarcoma (rare in infants/young children, prominent mitotic activity), inflammatory pseudotumor / myofibroblastic tumor (endocardial lesions covered by fibrin; stellate tumor cells have obvious nucleoli, more prominent inflammation, occasional mitotic figures and necrosis, Ann Diagn Pathol 2001;5:335), fibrous histiocytoma (extremely rare in heart, lipid-laden histiocytes), scar (grossly does not bulge)

 

Granular cell tumor of heart

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Very rare

Usually incidental finding at autopsy that may be overlooked

Benign tumor of Schwann cells, not myocytes (Archives 1976;100:276)

Only rarely has malignant behavior in heart

Case reports: multicentric tumor of skin and heart (Ann Thorac Surg 1994;57:1653), malignant tumor with multiple foci in myocardium (AJSP 1982;6:665)

Gross: epicardial nodules at base of heart in adults; circumscribed, gray-tan, firm; almost never continuous with nervous tissue

Micro: tumor cells merge with cardiac myocytes; have ill defined cell margins, abundant eosinophilic and granular cytoplasm with bland pyknotic nuclei, similar to granular cell tumors elsewhere

Positive stains: S100, PAS+ diastase resistant granules; also neuron specific enolase

Negative stains: neurofilament, GFAP

EM: granules are membrane bound autophagic vacuoles with cellular debris; may have myelinated and nonmyelinated axon-like structures

DD: disseminated granular cell epulis (tumor of gingiva of infants that rarely disseminates)

 

Hamartoma of mature cardiac myocytes

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Rare

Case reports: 24 year old man (Mod Path 1998;11:496), 33 year old man with shortness of breath (Rev Esp Cardiol 2005;58:450), 33 year old patient with tachycardia (Ann Thorac Surg 2001;71:1673)

Gross: resembles hypertrophic cardiomyopathy but has localized masses or multiple discrete masses, usually in right ventricle or atrium; no septal asymmetry (Hum Path 1998;29:904)

Micro: discrete but unencapsulated nodules of marked myocyte hypertrophy with disorganization (resembles hypertrophic cardiomyopathy), focal scarring and thickened intramural arteries; no myocyte vacuoles

EM: abundant and disorganized myofilaments; normal intercellular junctions

DD: rhabdomyoma (myocyte vacuoles), oncocytic cardiomyopathy

 

Hemangioma of heart

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Rare; <5% of benign cardiac tumors

All ages

30% are multiple

Usually asymptomatic, but may cause sudden death or significant cardiac dysfunction by displacing large portions of atria and ventricles

Case reports: necrotic tumor with DIC (Hong Kong Med J 2005;11:308), 49 year old with left atrial tumor (Braz J Cardiovasc Surg 2005;20(4)), with papillary endothelial hyperplasia and liver involvement (Heart 2004;90:e49)

Treatment: complete excision if possible

Micro: types are capillary (small vessels) or cavernous (multiple dilated thin walled vessels) or arteriovenous (thick walled, dysplastic malformed arteries and veins); no necrosis, no mitotic figures, no atypia; intramural tumors may contain adipose or fibrous tissue

DD: angiomatosis (vessels of various sizes with variable wall thickness, adipose tissue and skeletal muscle, Int Heart J 2006;47:469), myxoma (hemangiomas may have myxoid background, but lack myxoma cells or ring structures), angiosarcoma (atypia present)

References: Radiographics 2000;20:1073, Am J Cardiovasc Pathol 1990;3:283

 

Inflammatory pseudotumor / inflammatory myofibroblastic tumor of heart

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Previously called plasma cell granuloma

Extremely rare

Usually age 2 months to 17 years

May be intravascular in infants (Pediatr Dev Pathol 2002;5:400)

Case reports: 15 year old boy and 5 month old girl (Archives 1996;120:549), 2 month old boy with multiple tumors (Ann Thorac Surg 2006;82:1531), 72 year old woman (Ann Thorac Surg 2003;75:1971), 55 year old woman with sudden death due to tumor (Forensic Sci Int 1991;49:89)

Treatment: excision, possibly steroids (Pediatr Cardiol 2004;25:406); may recur or continue to grow locally, but no distant metastases (AJSP 1995;19:859)

Gross: up to 8 cm

Micro: spindle cells larger than fibroblasts with obvious nucleoli, inflammatory cells, prominent vasculature, variable myxoid stroma; occasional mitotic figures and necrosis

Positive stains: smooth muscle actin

Negative stains: desmin, S100, CD34, p53 (Ann Diagn Pathol 2001;5:335)

 

Lambl’s excrescence of heart

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Also called valvular strands

Common finding in elderly patients

Small filiform / papillary processes that are age-related; may be organized mural thrombi at site of minor endothelial damage

Associated with stroke, particularly among younger patients (Stroke 1997;28:2185)

Aortic/pulmonic valves: most common at nodules of Aranti, along lines of closure and free cuspal edges

Tricuspid/mitral valves: at site of valve closure on atrial surface

Case reports: causing embolic stroke (Nervenarzt 2006;77:1492), causing transient ischemic attack (An Med Interna 2006;23:181)

Micro: connective tissue / fibrin covered by endothelium

DD: papillary fibroelastoma (larger, more gelatinous, located away from lines of closure and free edges and on endocardial surfaces of atria and ventricles)

References: Am J Cardiol 1986;58:1223

                                                                        

Lipoma of heart

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8-12% of primary tumors of heart and pericardium

Usually on epicardium and asymptomatic

May create ball-valve obstructions or produce arrhythmia

Called lipomatous hypertrophy if in atrial septum

Case reports: massive left atrial lipoma occupying pericardial space (Jpn Heart J 2004;45:715), symptomatic tumor of right atrial free wall (Tex Heart Inst J 1998;25:152), interventricular septum (ASAIO J 2006;52:e35), hibernoma in right atrium (Heart Surg Forum 2006;9:E623)

Gross: may be poorly encapsulated but often circumscribed; spherical or elliptical mass of homogeneous yellow fat

Micro: usually encapsulated, composed of mature fat, may contain trapped myocytes; usually no brown fat cells

References: Hum Path 2006;37:1245

 

Lipomatous hamartoma of heart valves

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Rare

Usually mitral or tricuspid valve

Case reports: tricuspid valve (Clin Cardiol 1991;14:262), fibrolipoma of tricuspid valve (South Med J 1996;89:1018), aortic valve (Jpn J Thorac Cardiovasc Surg 2005;53:577), with blood cysts (J Am Soc Echocardiogr 1998;11:832)

Treatment: may need to replace valve due to involvement of papillary muscle or adjacent atrial tissue

Micro: mature fat and fibrous tissue; no encapsulation

 

Lipomatous hypertrophy of cardiac intraatrial septum

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Rare (0.6% of cardiac tumors); usually age 60+ years

Associated with increased epicardial fat (heart floats in water), mild to severe obesity

Symptoms include congestive heart failure, atrial fibrillation, supraventricular tachycardia, syncope, sudden unexpected cardiac death (Archives 2006;130:397)

Often diagnosed at autopsy (Cesk Patol 2006;42:182)

“Hypertrophy” is a misnomer because mass is due to increased fat cells, not hypertrophied myocytes

Treatment: weight loss, not surgery (Heart 2004;90:e66)

Gross: unencapsulated mass usually confined to atrium; resembles brown fat; associated with cardiac hypertrophy; septal thickness is 2 to 7 cm (normal is < 1 cm); fossa ovalis is usually spared

Micro: multivacuolated fatty infiltration more than 2 cm thick in atrial septum; cells have central nuclei without atypia; some fat cells resemble brown fat and fat cells in malnourished patients; usually atypical and hypertrophied myocytes (AJSP 1996;20:678); variable bands of collagen and chronic inflammatory infiltrates; no mitotic figures, no signet ring structures, no enlarged hyperchromatic and indented nuclei in fat cells

EM: abundant mitochondria in vesicular fat cells

DD: myxoma (has stellate or globular cells, myxoid areas, no fat, no hypertrophied myocytes), lipoma (encapsulated, no muscle fibers), liposarcoma (lipoblasts have large clear vacuoles and hyperchromatic indented nuclei)

References: AJCP 1979;72:785, Hum Path 2006;37:1245

 

Lipomatous infiltration of heart

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Also called fatty heart

Heart may float in water at autopsy

Associated with obesity and lipomatous infiltration of atrial septum

Usually incidental, rarely causes sudden death

Case reports: lipomatous hypertrophy of right ventricle, massive infiltration of left ventricle (Jpn Heart J 1986;27:273)

Gross: diffuse lipomatous infiltration of epicardial surfaces with focal infiltration into myocardium; no discrete masses; marked increase in fat in atrioventricular sulci and over ventricles

Micro: mature fat that may infiltrate into myocardium

DD: arrhymogenic right ventricular cardiomyopathy (affects right ventricle only, AJR Am J Roentgenol 2007;188:W423)

References: Archives 1982;106:497

 

Mesothelial / monocytic incidental cardiac excrescence

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Benign

Incidental finding of endocardium or pericardium during cardiac valve replacement

Either reactive or an artifact of surgery (AJSP 1994;18:167), although may exist without prior surgery

Case reports: causing acute cardiopulmonary failure (AJSP 2005;29:564), with clusters of metastatic adenocarcinoma cells (AJSP 1997;21:970)

Micro: haphazard mixture of mesothelial cells, histiocytes, fat globules and fibrin; mesothelial cells may form micropapillary or tubular formations; no/minimal stroma, no mitotic activity

Positive stains: CD68 (histiocytes), keratin (mesothelial cells, Ann Diagn Pathol 2000;4:39)

DD: metastatic carcinoma (keratin+ cells with atypia)

References: AJSP 1990;14:993, Mod Path 1994;7:9

 

Myxoma of heart

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Most common primary tumor of heart (~40%), although still rare; distinct from soft tissue myxoma

Sporadic or familial (see Carney syndrome below)

90% occur in atria, 80% on left side

Mean age 50 years

Pedunculated tumors may move through AV value at systole; this “wrecking ball” effect may damage valve leaflets

Symptoms of ball-valve obstruction, emboli, fever/malaise due to IL-6 (mediates acute-phase response, Chest 2003;123:1379)

Associated with HSV infection (Am J Pathol 2003;163:2407)

Benign (only rare metastases), but malignant in a sense because often embolizes (30-40%) and may cause death; embolization may be related to overexpression of matrix metalloproteinases (Am J Pathol 2005;166:1619); rarely embolizes in toto (Jpn Heart J 2004;45:359)

Rarely causes aneurysms (Neurol India 2005;53:216)

Stromal cells may arise from multipotent mesenchyme capable of neural and endothelial differentiation, with glands representing entrapped foregut rests (Am Heart J 2000;140:134)

Carney syndrome/complex: 10% of cases; autosomal dominant with multiple cardiac and extracardiac (skin) myxomas, spotty skin pigmentation, endocrine overactivity, schwannomas, epithelioid blue nevus (Orphanet J Rare Dis 2006 Jun 6;1:21); these patients are younger (mean 24 years), usually men (66% vs. 24% in non-familial cases), multicentric (33%, AJSP 1985;9:53); caused by mutations in protein kinase A regulatory subunit 1 alpha (PRKAR1A, Circ J 2005;69:994, Nat Genet 2000;26:89)

Case reports: Case of the Week #78 (glandular differentiation), infected (MJA 2006;185:332), with thymoma / thymic rests (AJSP 2005;29:1208);

metastases - to brain resembling epithelioid hemangioendothelioma (AJSP 1994;18:107), to choroid plexus and scapula (AJSP 1980;4:391), to small intestine (Archives 2003;127:481), to temporal bone (AJCP 1989;91:221)

Treatment: excision; no/rare recurrence (Yonsei Med J 2006;47:367)

Gross: usually solitary at fossa ovalis, up to 10 cm, sessile or pedunculated, 41% have surface thrombus; soft, polypoid (particularly when examined under water), pale, lobulated; rarely petrified due to repeated hemorrhage (Archives 1989;113:742); rarely grows on both sides of fossa ovalis

Micro: complex structures resembling cords, nests, rings or poorly formed glands, often surrounding blood vessels; composed of stellate or globular myxoma cells with abundant eosinophilic cytoplasm, indistinct cell borders, oval nucleus with open chromatin and indistinct nuclei; abundant mucopolysaccharide (myxoid) ground substance containing chondroitin sulfate and hyaluronic acid (Life Sci 2003;73:849); usually inflammation, hemorrhage; often more cellular and mitotic activity near surface; variable fibrosis (41%), calcification (20%), Gamna-Gandy bodies (17%, identical to those in spleen of sickle cell anemia patients), ossification (8%),  extramedullary hematopoiesis (7%, more common in children), mucin-forming glands (3%, Indian Heart J 2003;55:182), atypia (3%), thymic rests (1%); may embolize to peripheral arteries

Positive stains: CD31, CD34, calretinin (strong, diffuse, cytoplasmic and nuclear staining, AJCP 2000;114:754), S100 (Am Heart J 2000;140:134), vimentin; glandular components may be keratin+; variable Factor VIII (surface cells may be positive, otherwise negative)

Negative stains: CD68, cytokeratin (except for glandular elements)

EM: features of endothelial, smooth muscle and immature mesechymal cells; cells have numerous cytoplasmic filaments (Cancer 1977;40:2216)

DD: mural myxoid thrombi (no nests, cords or rings, calretinin negative, image), left atrial appendage thrombus (Circulation 2006;113:e456), metastatic carcinoma if myxoma has glandular structures (carcinoma has anaplasia, mitotic activity), myxoid sarcoma, papillary fibroelastoma (on valve cusps, avascular papillary fronds)

References: AJCP 1993;100:671, eMedicine, Wikipedia, OMIM 255960, Radiographics 2002;22:673

 

Necrobiotic xanthogranuloma of heart

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Rare

Associated with similar lesions in lung and skin; also leukopenia, paraproteinemia and complement deficiencies (Mayo Clin Proc 1997;72:1028); also nodular transformation of liver (Pathology 1985;17:533)

Case reports: myocardial lesions at autopsy (Hum Path 1992;23:195), pericardial, pulmonary and cutaneous involvement (Ann Dermatol Venereol 2003;130:341)

Micro: amorphous necrotic tissue, foamy macrophages / giant cells with vacuoles and inclusions, lymphocytes

 

Papillary fibroelastoma of heart

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Also called giant Lambl’s excrescence

8-10% of all heart tumors; third most common after myxoma and lipoma

Mean age 60 years

Benign

Usually an incidental finding at autopsy, although may embolize (higher risk if large, mobile, left-sided, Circulation 2001;103:2687), or prolapse into coronary artery

More common on valve surface or endocardium

Associated with prior cardiac surgery or thoracic radiation (Hum Path 2002;33:1165)

Case reports: 59 year old woman with atrial mass (Archives 2001;125:1605), arising in right coronary ostium (Archives 1992;116:135), presenting as left ventricular mass (Tex Heart Inst J 2006;33:63), two lesions of aortic valve (Tex Heart Inst J 2004;31:448)

Treatment: either no intervention, excision or warfarin

Gross: distinctive cluster of yellow-white hairlike projections up to 1 cm in diameter covering large portions of valvular surface (usually aortic and mitral); often with adherent thrombus; often multiple

Micro: narrow, elongated and branching papillary fronds composed of central avascular collagen and variable elastic tissue, surrounded by acid mucopolysaccharide and lined by hyperplastic endothelial cells

DD: Lambl’s excrescences (not branched, not gelatinous, smaller, only at lines of closure), myxoma (no elastic tissue)

References: Am Heart J 2003;146:404, Arq Bras Cardiol 2005;85:205

 

Paraganglioma of heart

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Also called extra-adrenal pheochromocytoma (if secretes catecholamines) or chemodectoma (at carotid body)

Either functional (secretes catecholamine, associated with hypertension, headache, flushing) or non-functional

Very rare

Sites: left atrium or near aortic root, where intrinsic cardiac ganglia (chromaffin cells) are located

Case reports: pigmented tumor causing sudden death in 40 year old man (Archives 2001;125:680), 34 year old woman with 6 cm atrial tumor (Heart Lung Circ 2007;16:116), 25 year old with bone metastases (Circulation 1998;97:1993)

Treatment: excision or transplantation; 5-10% recur or metastasize after excision

Gross: 5-7 cm, unencapsulated; on epicardium but may infiltrate adjacent cardiac tissue

Micro: unencapsulated, organoid appearance (zellballen/nests of cells) surrounded by sustentacular cells; no necrosis, no/minimal mitotic figures

Positive stains: chromogranin, neuron-specific enolase; sustentacular cells-S100

Negative stains: calcitonin

EM: norepinephrine-type granules, few epinephrine-type granules

References: AJSP 1985;9:827

 

Rhabdomyoma of heart

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50-90% of primary heart tumors in children

Usually discovered in patients less than 1 year of age, rarely in fetuses (Ital Heart J 2002;3:48)

May obstruct valvular orifice or cardiac chamber; may present with sudden cardiac death (Pediatr Dev Pathol 2007;10:129)

Not a true neoplasm, but a hamartoma or malformation due to mutation in TSC1 and TSC2 genes

50% of patients have tuberous sclerosis; sporadic cases are occasionally associated with congenital heart disease

Case reports: 2 year old boy with tuberous sclerosis (Archives 2002;126:1559), diffuse rhabdomyomatosis in 13 year old boy (Archives 1977;101:78)

Treatment: many tumors regress spontaneously; excision if left ventricular outflow tract obstruction or refractory arrhythmia

Gross: small, firm, gray-white, well-circumscribed myocardial masses (often multiple) that protrude into ventricles; average size is 3-4 cm, up to 10 cm, particularly in sporadic cases

rhabdomyomatosis - numerous miliary nodules less than 1 mm

Micro: clear cells and large, rounded, polygonal cells (“spider cells”) with glycogen vacuoles separated by strands of cytoplasm extending between cell membrane and nucleus; no mitotic activity

adult tumors: more cellular with smaller cells, few spider cells and more cellular proliferation (Hum Path 2002;33:1092)

Positive stains: PAS+ diastase sensitive (for glycogen), myoglobin, actin, desmin, vimentin; variable HMB45

Negative stains: S100

EM: altered myocytes with abundant glycogen, small and sparse mitochondria; cellular junctions resembling intercalated disks are extensive and randomly distributed, not just at poles of cell as in normal myocytes (Hum Path 1977;8:700)

DD: lipoma (usually epicardial, no myofibers, no glycogen), granular cell tumor (epicardial, no vacuoles, no myofibers, S100+, desmin-, myoglobin-), glycogen storage disease (no well formed nodules, cells have intercalated disks at poles by EM), histiocytoid myopathy (small tumor nodules, finely granular cells, no large vacuoles, no spider cells; a variant of rhabdomyomatosis)

References: Mod Path 1991;4:70, TheFetus.net

 

Schwannoma of heart

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Case reports: incidental finding of schwannoma with focus of metastatic adenocarcinoma from lung (Archives 1984;108:300), 57 year old man with atrial tumor (J Cardiothorac Surg 2007;2:1), 61 year old woman with atrial tumor (Cardiovasc Pathol 2003;12:290)

 

Sugar tumor of heart

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Very rare in heart; more common in lung

Usually benign, but may have malignant histology

Micro: circumscribed; organoid pattern of tumor cells with clear to eosinophilic cytoplasm, abundant glycogen in rich vasculature; no/rare mitotic figures, no necrosis

Positive stains: HMB45

Negative stains: S100, keratin, neuroendocrine markers, muscle markers

EM: glycogen, abundant mitochondria, premelanosomes

DD: paraganglioma, clear cell sarcoma, melanoma, melanocytic clear cell neoplasm of the kidney

References: Mod Path 2001;14:615-free full text

 

Tumor with perivascular myoid differentiation of heart

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Very rare in heart

May recur after surgery, particularly in adults

Case reports: 70 year old woman with right atrial mass (J Clin Pathol 2004;57:1338)

Micro: round, undifferentiated to epithelioid cells surrounding prominent, thin walled, staghorn vessels

Positive stains: alpha smooth muscle actin

Negative stains: CD34

DD: hemangiopericytoma (CD34+, actin-), solitary myofibroma, epithelioid hemangioendothelioma, glomus tumor, vascular leiomyoma

 

Varix of heart

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Usually dilated thrombosed vein in right atrium that arises from inferior rim of fossa ovalis

Incidence varies from 0.1% to 2.5% at autopsy

Asymptomatic

Case reports: 3 cm, round, cystic mass attached to lower rim of fossa ovalis in right atrium (Circ J 2006;70:793), 19 year old woman with 8 cm mass compressing right atrium (Ann Thorac Surg 2004;78:e96)

Gross: blue, raised area inferior to fossa ovalis

Micro: unilocular, blood-filled cyst lined by endothelial cells and filled with organizing thrombus

DD: blood cyst (congenital, along lines of closure of heart valves)

 

 

Cardiac tumors-malignant

Angiosarcoma of heart

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Common primary malignancy of heart, although still rare

Usually atrium; right sided more common than left

Often men, mean age 40 years

Metastases present at diagnosis in 89%, usually to liver and lung (may present as diffuse hemorrhage, Archives 2001;125:1562)

Case reports: 36 year old man with HIV (Archives 2005;129:943), 54 year old man with persistent pericardial effusion (Archives 2005;129:117), presenting with cutaneous metastases (Archives 1998;122:273)

Treatment: usually no effective treatment; poor prognosis with death commonly in a few months

Gross: large, infiltrating, pink to dark brown, lobulated mass; often invades vena cava and tricuspid valve

Micro: usually poorly differentiated; anastomosing vascular channels forming dilated sinusoids lined by atypical cells; patterns include spindle cell, angiomatous, undifferentiated/solid; prominent mitotic activity; hemorrhage and necrosis common

Cytology: single or clusters of pleomorphic spindle cells in a background of hemorrhage; occasional microacinar or pseudoacinar structures lined by abnormal spindle or polygonal cells (Diagn Cytopathol 2007;35:164)

Positive stains: CD31 (most specific); also CD34, reticulin (highlights vascular channels, shows tumor cells on luminal side of vessels), thrombomodulin, Factor VIII (20%)

Negative stains: CD45, cytokeratin, S100

EM: thin and delicate cytoplasmic processes, Weibel-Palade bodies, distorted extracellular fibrous matrix (Eur Rev Med Pharmacol Sci 2005;9:231)

DD: metastatic angiosarcoma (more common than primary tumor, cardiac metastases associated with widely disseminated disease), Kaposi’s sarcoma (usually small and epicardial with minimal myocardial infiltration), hemangioendothelioma (flattened endothelial cells, distinct vascular channels)

References: Radiographics 2003;23:S141

 

Chondrosarcoma of heart

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Less than 20 primary cases reported through 2006 (Jpn J Thorac Cardiovasc Surg 2006;54:228)

Chondroid areas in a cardiac tumor are virtually diagnostic of malignancy

Case reports: tumor producing parathyroid hormone-related protein (Circ J 2004;68:715)

Micro: atypical chondroid areas

DD: metastatic chondrosarcoma (Ann Thorac Surg 1988;45:291)

 

Ewing’s sarcoma/PNET of heart

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See also Bone chapter

Rare as either primary tumor or metastasis

Case reports: 63 year old man (Hum Path 1996;27:1365), metastases to heart (Can J Cardiol 2005;21:525)

Positive stains: CD99, NSE, synaptophysin, vimentin

Negative stains: keratin, chromogranin, desmin, actin, myoglobin, CD45/LCA

 

Fibrosarcoma of heart

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Rare; 5% of cardiac sarcomas

Often left atrium; may present as congestive heart failure

Treatment: aggressive approach may be necessary (Chest 2003;123:1766); usually poor prognosis

Gross: soft, polypoid, gelatinous tumors that fill atrium or infiltrate ventricles or pericardium; often necrotic

Micro: herringbone pattern of compact fibroblastic type cells with tapered nuclei in collagenous or myxoid matrix; no intracytoplasmic glycogen, no perinuclear vacuoles, no pleomorphism, no histiocytoid cells

Positive stains: vimentin, variable actin

Negative stains: desmin, cytokeratin, S100

EM: fibroblasts with granular or amorphic cytoplasm, dilated rough endoplasmic reticulum, sparse cytoplasmic organelles, infrequent nucleoli; extracellular mature and immature collagen

DD: malignant fibrous histiocytoma (histiocytoid cells and pleomorphism are prominent), malignant solitary fibrous tumor of pericardium (similar histology but infiltrates pericardial space with little myocardial invasion), synovial sarcoma (monophasic tumors have similar histology, but tumor usually has cytokeratin+ epithelioid foci), osteosarcoma (has large areas of fibrosarcoma but also malignant osteoid), mesothelioma (usually is biphasic, spindled areas are cytokeratin+), leiomyosarcoma (fascicles at sharp right angles but not herringbone, intracytoplasmic glycogen, perinuclear vacuoles, prominent myofilaments, basal lamina with EM, multinucleated giant cells occasionally), fibroma in infants

 

Germ cell tumor / teratoma of heart

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Rare

Most cardiac germ cell tumors are teratomas

Most common site is interventricular septum, extending into right ventricle

Benign if pure teratoma, but malignant if other germ cell elements are present (choriocarcinoma, embryonal carcinoma, yolk sac tumor) or if squamous cell carcinoma arises from teratoma

Case reports: 2 year old girl with fatal teratoma of interventricular septum (Archives 1993;117:739), “cardiac development” in a sacrococcygeal teratoma (Eur J Pediatr Surg 2002;12:278)

Treatment: excision

Gross: teratomas are multicystic with solid areas, up to 15 cm

Micro: teratoma - squamous or mucous lined cysts, smooth muscle, cartilage, neuroglial tissue; intestinal, pancreatic, ependymal tissue and bone; often myxoid stroma

DD: bronchogenic cyst (no hair, teeth or neurogenic elements), atrioventricular nodal tumor (Pediatr Pathol 1994;14:913)

 

Granulocytic sarcoma of heart

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See also Leukemia or Lymph nodes-not lymphoma chapters

Historically, leukemic infiltrates were found at autopsy in heart or pericardium in 37% of acute leukemia patients, but usually asymptomatic

Mass lesion may precede clinical symptoms (Am J Hematol 1987;25:325)

Case reports: 12 year old girl (Pediatr Hematol Oncol 1987;4:237), 34 year old man with extensive endomyocardial infiltrate (Echocardiography 2003;20:539), 72 year old man with right ventricular failure (Archives 1990;114:983)

 

Hemangioendothelioma of heart

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See also Soft Tissue chapter

Rare in heart

Vascular tumor of intermediate malignancy

10% of extracardiac tumors metastasize and 1/3 recur

Case reports: 2 month old baby with extensive tumor infiltration but no mass (Ann Thorac Surg 2005;79:1402), 36 year old woman with shortness of breath (Chin Med J (Engl) 2006;119:966), 69 year old woman with rheumatic heart disease and valvular tumor (Pathol Int 2005;55:644), with distant metastases (J Cardiovasc Surg (Torino) 1993;34:529)

Micro: polygonal cells with abundant cytoplasm and intracytoplasmic vacuoles; epithelioid tumors have short strands or solid nests of round/oval endothelial cells with small intracellular lumina; infiltrate muscular walls of vessels

Positive stains: factor VIII related antigen, CD34

DD: adenocarcinoma

 

Hemangiopericytoma of heart

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Rare in heart

May be benign or malignant

Case reports: 41 year old man with malignant tumor (J Cardiovasc Surg (Torino) 2006;47:71), intracranial tumor that metastasized to heart (Radiat Med 1996;14:91)

Micro: highly vascular tumor of tightly packed cells with minimal cytoplasm and round/oval nuclei which surround endothelial lined vascular channels with staghorn shape

Positive stains: vimentin, CD34, factor XIIIa

Negative stains: factor VIII, keratin

EM: pericytes with elongated cytoplasmic processes, pinocytotic vesicles, poorly developed desmosomes, few organelles but well developed basal lamina

DD: synovial sarcoma (biphasic), benign fibrous histiocytoma

 

Kaposi’s sarcoma of heart

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See also Soft tissue tumor chapter

Occurs in patients with skin and visceral lesions in classic and epidemic forms of disease

Heart involvement in 5% of HIV+ patients historically

Usually epicardial and pericardial involvement with minimal myocardial infiltration

Case reports: myocardial involvement in heart transplant patient (Catheter Cardiovasc Interv 2000;49:208)

Gross: focal, small, firm, red-brown nodules

Micro: slit like vascular spaces with atypical spindle cells, endothelial cells and red blood cell extravasation

                                                                                                             

Leiomyosarcoma of heart

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8-9% of cardiac sarcomas

Poor prognosis (months) unless low grade (Histopathology 1999;34:295).

Mean age in 30’s

Likely arises from smooth muscle of pulmonary veins and arteries and spreads into heart

Case reports: myxoid leiomyosarcoma (Archives 1996;120:583), epithelioid and spindle cell tumors (Archives 1999;123:782), uterine leiomyosarcoma metastatic to heart (Clin Transl Oncol 2006;8:375)

Treatment: excision or transplantation (J Heart Lung Transplant 1998;17:1133)

Gross: 75% in left atrium, may appear to arise from pulmonary vein, sessile lesions may be grossly myxoid; 30% are multiple

Micro: compact bundles or fascicles oriented at right angles to each other; spindled or epithelioid smooth muscle cells with cytoplasmic glycogen, blunted nuclei with moderate/marked pleomorphism, necrosis, mitotic activity; occasionally giant cells, perinuclear vacuoles; may also be low grade with few mitotic figures

Positive stains: PAS for glycogen, trichrome shows longitudinal fibers; actin, desmin (usually)

Negative stains: cytokeratin (may be positive in epithelioid areas), S100

EM: notched or infolded nuclei, thin actin filaments with focal densities, micropinocytotic vesicles, thin but distinct external lamina, dispersed glycogen, rare mitochondria or endoplasmic reticulum

DD: fibrosarcoma (tapered nuclei, no intracytoplasmic glycogen, no pleomorphism, actin-, desmin-)

 

Liposarcoma of heart

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Very rare

Mean age 53 years

Usually right atrium

Metastasizes to lung, liver, bones, CNS

Mean survival is 8 months

Case reports: encasement of heart by metastatic myxoid liposarcoma (Cardiovasc Pathol 2002;11:322)

Gross: bulky tumors up to 10 cm, often cystic, soft, bosselated; may have multiple tumor implants on surface of great vessels or diffuse pericardial growth

Micro: pleomorphic liposarcoma resembles MFH or fibrosarcoma, but with lipoblasts containing multiple vacuoles that indent the cell nuclei; myxoid liposarcoma has prominent plexiform vascular pattern and signet ring type lipoblasts

Positive stains: S100, Oil Red O (myxoid liposarcoma)

Negative stains: Alcian blue

EM: numerous nonmembrane bound lipid droplets fill cytoplasm and are associated with mitochondria; large indented nucleus is at cell periphery

DD: lipomatous hypertrophy of interatrial septum (cells resemble brown fat, but no lipoblasts or myxoid background; also hypertrophied myocytes), myxoid MFH (no plexiform vascular pattern, no signet ring cell lipoblasts, lipid is Alcian blue positive), undifferentiated sarcoma (no lipoblasts)

 

Lymphoma of heart

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Rare; associated with HIV or other immunocompromise

Primary if all of tumor is within pericardium; otherwise secondary

Primary tumors are rare

Most common subtype is diffuse large B cell lymphoma (Arch Mal Coeur Vaiss 2005;98:875, Leuk Lymphoma 2004;45:781)

Poor prognosis, since usually advanced disease

Not associated with EBV (Archives 1996;120:555)

Case reports: adult T cell leukemia/lymphoma-massive cardiac involvement (Archives 1991;115:1052), anaplastic large cell lymphoma-presenting with embolic stroke (Blood 2003;101:4708), Burkitt’s lymphoma mimicking acute pulmonary embolism (JCPSP 2006;16:536), diffuse large B cell lymphoma-with hemophagocytic syndrome (Haematologica 2002;87:ECR27), B cell tumor damaging conduction system and causing atrioventricular block (Acta Cardiol 2007;62:55)

Gross: firm nodules within any chamber and often in pericardium

References: Angiology 2003;54:599

 

Malignant fibrous histiocytoma of heart

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See also undifferentiated sarcoma (some authors consider them the same entity)

#2 most common cardiac sarcoma after angiosarcoma, but still rare

Mean age 44 years, rare in children

90% occur in left atrium, often on posterior wall

Poor prognosis; metastasizes to lung, bones, lymph nodes, serosal surfaces, kidney, thyroid, skin

Case reports: tumor within thrombi (Hum Path 1985;16:1279), left atrial tumor #1 (Archives 1992;116:872), #2 (Gen Thorac Cardiovasc Surg 2007;55:29), #3 (Taiwan J Obstet Gynecol 2006;45:86), metastases to left ventricle (Tumori 2006;92:76), recurrence after heart transplant (J Heart Lung Transplant 2004;23:1447), 16 year old boy (Surg Today 2002;32:270), IL-6 producing tumor (Jpn J Clin Oncol 1999;29:382)

Treatment: excision, possibly chemotherapy or radiation therapy

Gross: lobulated polypoid mass, often multiple; sessile or pedunculated; soft or creamy texture; up to 10 cm

Micro: usually pleomorphic-storiform or myxoid subtypes; atypical fibroblasts, pleomorphic histiocytoid cells and giant cells with esosinophilic cytoplasm and fibromyxoid stroma

Giant cell MFH: numerous osteoclast-like giant cells; also called osteoclastoma

Positive stains: vimentin, alpha-1-antitrypsin, variable CD68

 

Myxoid MFH of heart

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Definition: bland spindle cell sarcoma with diffuse myxoid background

Previously called myxosarcoma or fibromyxosarcoma

Benign myxomas do NOT transform to myxoid MFH

Usually left atrium

Poor prognosis

Case reports: 21 year old man with widespread systemic metastases (J Korean Med Sci 2001;16:111)

Gross: gelatinous, multilobated, usually sessile endocardial tumor; may be multiple or infiltrate myocardium

Micro: loose storiform pattern, aggregates or single cells that are bland, spindled and resemble myxoma cells; diffuse background of amphophilic proteoglycan; prominent arborizing vessels; foci of atypical hyperchromatic cells with mitotic figures; may infiltrate myocardium

DD: myxoma (cord and ring structures infiltrated by mononuclear inflammatory cells, less cellular and pleomorphic than MFH; no extensive branching vasculature, no myocardial infiltration; must sample extensively to rule out myxoid MFH)

 

Malignant mesenchymoma of heart

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Definition: sarcomatous tumor with two or more distinct types of cellular differentiation other than fibrosarcoma

AFIP Third Fascicle (soft tissue) and WHO (soft tissue) dislike this terminology, and recommend (a) describing as mixed mesenchymal neoplasm and specifying the components or (b) classifying based on predominant mode of differentiation and mentioning the other component(s)

Rare

Mean age 42 years, 2/3 female

Usually left atrium or mitral valve

Mean survival is 20 months

Case reports: 41 year old woman with left atrial tumor (Eur J Cardiothorac Surg 2002;21:124), 46 year old woman with left atrial tumor (Acta Pathol Jpn 1982;32:851), tumor arising in atrial septum (Acta Pathol Jpn 1975;25:241)

Micro: common components are osteosarcoma, chondrosarcoma, rhabdomyosarcoma, angiosarcoma and liposarcoma

 

Malignant peripheral nerve sheath tumor of heart

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Definition: tapered spindle cells of neurogenic origin by morphology, ultrastructure or immunohistochemistry

Also called malignant schwannoma

Very rare

Case reports: intrapericardial tumor (Eur J Cardiothorac Surg 1997;12:144)

 

Metastases / tumor extension to heart

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30 times more common than primary malignancies (Archives 1993;117:1027)

Distant primaries are often melanoma, breast, lung or renal cell carcinoma, choriocarcinoma, childhood rhabdomyosarcoma

Case reports of metastases: carcinoid tumor of terminal ileum metastatic to conducting system (Archives 2002;126:1538), carcinoid-bronchial (J Clin Pathol 2004;57:778), cervical carcinoma (Jpn Circ J 2001;65:761), chordoma (Jpn Circ J 2000;64:627), gallbladder carcinoma (World J Gastroenterol 2005;11:2048), hepatocellular carcinoma (Chest 1994;105:945), melanoma (Radiographics 2005;25:249), Merkel cell carcinoma (J Clin Oncol 2004;22:1153), myelodysplastic syndrome (BMC Blood Disord 2006;6:4), myeloma (Archives 2000;124:910), osteosarcoma #1 (Br J Radiol 1998;71:336), #2 (Archives 2005;129:241), #3 (Can J Cardiol 2005;21:63), synovial sarcoma (Jpn Heart J 2001;42:387), testicular mixed germ cell tumor (Hum Path 2003;34:1074), thymoma (Intern Med 1993;32:257), thyroid carcinoma-anaplastic (Endocr Relat Cancer 2001;8:71), thyroid papillary carcinoma (Jpn Circ J 2000;64:890)

Extension to heart through venous system or by continuous spread: adrenocortical carcinoma-Cardiovasc Ultrasound 2003 May 16;1:5, J Korean Med Sci 2006;21:572, mesothelioma-Intern Med 1998;37:614, renal cell carcinoma-Anesth Analg 2000;91:1137, synovial sarcoma-renal-Int J Urol 2003;10:657, thyroid carcinoma-anaplastic-Acta Med Okayama 2006;60:135, Wilm’s tumor-Ann Surg 2001;234:116

References: J Clin Pathol 2007;60:27, Ann Oncol 2004;15:375, G Ital Cardiol 1997;27:1252

 

Osteosarcoma of heart

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Mean age 38 years

3-9% of cardiac sarcomas

Clinically resembles myxoma

Aggressive, usually causes death or metastases (AJSP 1991;15:289); metastasizes to lymph nodes, thoracotomy incision, lung, thyroid, skin

Treatment: excision, possibly heart transplantation (Bol Asoc Med P R 1999;91:98)

Case reports: 31 year old with left atrial tumor, osteosarcoma #1 (Br J Radiol 1998;71:336), #2 (Archives 2005;129:241), #3 (Can J Cardiol 2005;21:63),

Gross: usually attached to wall of left atrium as a 4-10 cm sessile mass; may extend into pulmonary vein; gritty and calcified areas

Micro: malignant bone forming cells within a spindled or pleomorphic sarcoma; may also have chondroid differentiation or myxoid areas; bone forming areas may be minor with most of tumor resembling MFH or fibrosarcoma

Positive stains: vimentin, actin (focal), S100 (chondrosarcomatous areas)

Negative stains: cytokeratin, desmin

DD: myxoma (no osteoid or chondroid malignant cells, has rings or cords of cells, no atypia)

 

Post-transplant lymphoproliferative disorder of heart

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See also Lymphoma-non B cell chapter

Rarely involves the heart allograft

Case reports: involvement of mitral valve (Ann Thorac Surg 1997;64:1822)

 

Rhabdoid tumor of heart

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Definition: highly malignant undifferentiated tumor of infants and children

See also Kidney tumor chapter

Originally described in kidneys; very rare in heart (one case report)

Case reports: 6 month old infant (Cancer 1985;55:2850)

Negative stains: desmin, actin

EM: masses of intermediate filaments

DD: rhabdomyosarcoma (desmin+)

 

Rhabdomyosarcoma of heart

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Definition: sarcoma with striated muscle differentiation

Rare, 4-7% of cardiac sarcomas, but most common cardiac malignancy in infants and children

Occurs throughout heart, not just in left atrium as with other sarcomas

Usually children/young adults

Metastases to lung, regional nodes, CNS, GI tract, kidney, other

Case reports: 28 year old with huge biatrial tumor (Cardiovasc Pathol 2006;15:354), associated with hypereosinophilia (Am J Hematol 2003;74:64)

Treatment: resection with atrial reconstruction; possibly chemotherapy

Gross: bulky, invasive, central necrosis; often 10 cm or more; almost always myocardial involvement; may involve mitral valve or atrial wall

Cytology: tumor cells have cross striations

Positive stains: PAS+ diastase sensitive (glycogen), desmin

EM: thick and thin filaments; well formed Z bands; frequent glycogen granules in clumps and mitochondria; nuclei are lobulated with variable condensed chromatin; variable A and I bands

DD: undifferentiated sarcoma (desmin negative), leiomyosarcoma (spindle cells with dispersed glycogen, although also desmin+)

 

Alveolar rhabdomyosarcoma of heart

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Cardiac cases usually represent metastases

Case reports: metastatic tumor (Circulation 1976;54:837)

Micro: collagenous stroma, few rhabdomyoblasts

 

Embryonal rhabdomyosarcoma of heart

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Usually infants, children and young adults

Case reports: left atrial tumor (Tex Heart Inst J 2000;27:206)

Gross: grape-like structures (sarcoma botyroides variant)

Micro: cambium layer may be present; small cell tumor with rhabdomyoblasts; variable tadpole-shaped rhabdomyoblasts; some cells have cross striations on high power

 

Sarcoma-general of heart

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See also individually described sarcomas

Sarcoma is most common primary tumor of heart after myxoma, but still is rare (0.2% of autopsies or less)

Often not diagnosed until autopsy due to rarity and nonspecific signs/symptoms

Primary if neoplasm is confined to heart or pericardium or bulk of tumor is within pericardial cavity

Most sarcomas as surgical specimens are primary, but must rule out metastatic tumor of soft tissue, bone or retroperitoneum, which are more common

Usually occurs in left atrium, except for rhabdomyosarcoma

Poor prognosis overall, with survival of 12-24 months due to advanced stage at presentation (Eur J Cardiothorac Surg 2006;29:925, Ann Cardiol Angeiol (Paris) 2003;52:370)

Poor prognostic factors: necrosis (in groups of cells with an inflammatory infiltrate), frequent mitotic figures (5 per 400x field with an average of 10 in most active area)

Treatment: radical excision or autotransplantation (surgery ex situ) may cause longer survival (Thorac Cardiovasc Surg 2004;52:77), transplantation may not prolong survival (Rev Esp Cardiol 2003;56:408)

Gross: bulky, infiltrating, nodular, firm white masses with variable hemorrhage and calcification

References: eMedicine

 

Synovial sarcoma of heart

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Definition: usually biphasic tumor with spindled and epithelioid cells and t(X;18)

Very rare in heart; usually right sided

Young and middle aged adults; 75%+ men (Pathologica 2004;96:29)

Symptoms: dyspnea, fatigue, chest pain, congestive heart failure

Usually poor prognosis due to inability to completely excise

Case reports: 42 year old man (Mod Path 2004;17:1434), 14 year old boy with tricuspid valve tumor (Heart Lung Circ 2006;15:278), 36 year old man with biatrial tumor (J Am Soc Echocardiogr 2007;20:197.e1), 66 year old man with mitral valve tumor (Cardiovasc Pathol 2005;14:331), atrial tumor presenting as transient ischemia attacks due to patent foramen ovale (Ann Thorac Surg 2000;69:1949)

Gross: bulky tumor infiltrates myocardium and pericardial surfaces

Micro: biphasic spindle cell and epithelioid tumor; spindle cell component resembles fibrosarcoma and has cellular and edematous areas; spindle cells are small, compact, often with lymphocytic infiltration; epithelioid areas may form glands or nests; often has hemangiopericytoma-like vascular pattern

Cytology: high grade tumor with intimate admixture of spindle and epithelial cells; need ancillary studies to confirm (Acta Cytol 2006;50:683)

Positive stains: keratin (epithelioid component), PAS+ diastase resistant mucin

Molecular: t(X;18)(p11.2; q11.2) in 90% leading to SSX1-SYT or SSX2-SYT fusion genes

DD: mesothelioma (large spindle cells with more pleomorphism, usually no infiltration of myocardium, no t(X;18) or fusion gene), MPNST, fibrosarcoma, myxoma

References: AJSP 2005;29:569 (mediastinal tumors)

 

Undifferentiated sarcoma of heart

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See also malignant fibrous histiocytoma (some authors consider them the same entity)

Rare, but common subtype of sarcoma in heart (Histopathology 1999;34:295)

Case reports:  3 year old boy with mitral valve tumor (J Cardiovasc Surg (Torino) 2003;44:621), 13 year old girl with widespread disease (Clin Med Res 2003;1:233), 33 year old woman with clinical atrial myxoma (Yonsei Med J 2003;44:530), 37 year old woman with left atrial mass (Yonsei Med J 2007;48:131)

 

 

Miscellaneous

Features to report for cardiac tumors

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* Specimen type

* Tumor site

* Tumor size (greatest dimension)

* Histologic tumor

* Histologic grade

* Extent of invasion, as appropriate

* Margins, as appropriate

 

Recommended but not required

Additional tumor dimensions

Additional pathologic findings

 

* mandatory to report for accreditation purposes by American College of Surgeons Committee on Cancer

 

References: link to College of American Pathologist protocols

 

End of Heart-tumor chapter/outline

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