Download Pericardial Disease: Anatomic Abnormalities

6
7
Pericardial Disease:
Anatomic Abnormalities
Hugh A. McAllister, Jr., L. Maximilian Buja,
and †Victor J. Ferrans
Pericardial Fluid . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1479
Mesothelial Hyperplasia . . . . . . . . . . . . . . . . . . . . . . . . . 1479
Pericarditis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1479
Pericardial Tumors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1480
Pericardial Cysts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1481
Summary. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1481
Key Points
rior vena cava. Pericardial, pleural, and peritoneal chylous
effusions also have been reported in patients with pulmonary
lymphangiomyomatosis.1 Some cases of idiopathic or primary
chylopericardium have also been reported.2 Cholesterol-rich
fluid (“gold paint”) is the hallmark of cholesterol pericarditis,
which has been reported in patients with myxedema, rheumatoid arthritis, tuberculosis, and other conditions.3 Purulent
pericardial fluid can be caused by bacterial, fungal, or parasitic infections of the pericardium.
• Pericardial disease is characterized pathologically by the
composition of the pericardial fluid and the cellular components of the inflammatory exudate.
• Infectious and non-infectious causes of pericarditis occur.
• The pericardium may be involved by metastatic or
primary neoplasms and cysts.
The important diagnostic features of specimens from biopsy
procedures or operative resections of the pericardium are (1)
the overall thickness of the pericardium, (2) the presence and
type of cellular infiltrates (acute or chronic inflammatory
cells, granulomas, or neoplastic cells), (3) morphology of the
mesothelial lining cells, (4) infective agents (including appropriate culture and staining procedures), and (5) extracellular
deposits (calcium, fibrin, thrombus, and amyloid), either on
the surface or within the tissue itself.
Pericardial Fluid
To correctly interpret the morphologic findings, one must
know the cytologic, microbiologic, and chemical characteristics of the pericardial fluid that is removed when the tissue
sample is obtained. Serous fluid can be found in patients with
congestive heart failure, hypoalbuminemia, or irradiationinduced injury. Bloody fluid (hematocrit >10%) can be due
to cardiac surgery, penetrating or nonpenetrating injury
(including the complications of cardiac catheterization), coagulopathies, overdoses of anticoagulants, complications of chemotherapy (cyclophosphamide), acute myocardial infarction,
rupture of the heart or a major vessel, neoplasms, tuberculosis, or chronic renal disease (uremic pericarditis). Fluid that
contains lymph or chyle can result from pericardial neoplasms or obstruction (due to neoplasm, iatrogenic injury, or
other causes) of the thoracic duct, pulmonary hilum, or supe†
Posthumously, Dr. Ferrans remains an author of this chapter. Dr.
Ferrans died in October 2001.
Mesothelial Hyperplasia
Mesothelial hyperplasia may be focal or diffuse and is found
most frequently in patients with underlying heart disease
(i.e., chronic pericarditis or rheumatic heart disease).4 The
mesothelial cells may form tumor-like masses within the
pericardial space. More frequently, mesothelial hyperplasia is
diffuse along the parietal or visceral pericardium. Reactive
mesothelial cells are multilayered, and nests of mesothelial
cells often lie within the pericardial stroma. Again, the cells
are remarkably uniform in appearance, with minimal pleomorphism. Mitoses may be seen.4 Differential special staining of mesothelial cells and carcinoma cells, together with the
clinical history, should distinguish mesothelial hyperplasia
from metastatic carcinoma involving the pericardium.
Mesothelial hyperplasia may be a complication of treatment with radiotherapy for patients with carcinoma, or it may
develop secondary to the pericarditis that sometimes follows
the spread of carcinoma to the pericardium. Thus, because of
the known coexistence of the two conditions, the diagnosis of
metastatic carcinoma involving the pericardium is not
excluded by the finding of mesothelial hyperplasia alone.4
Pericarditis
Pericarditis can be infectious or noninfectious. The most
common causes of bacterial pericarditis are infection with
staphylococci, streptococci, pneumococci, or Pseudomonas
147 9
CAR067.indd 1479
11/24/2006 1:39:37 PM
14 8 0
chapter
67
species. Nontuberculous infective pericarditis most often
occurs as a complication of cardiothoracic surgery, disseminated infections in patients receiving immunosuppressive or
antineoplastic therapy, rupture of the esophagus (as a consequence of a neoplasm) into the pericardial cavity, or infective
endocarditis (associated with a septic coronary embolus or
the rupture of a ring abscess or a myocardial abscess).
Tuberculous pericarditis continues to be clinically important. Four stages of this disorder have been described:
1. A fibrinous stage, which is associated with a granulomatous reaction (caseating granulomas)
2. A stage of effusion (which can be serous, serosanguineous,
or bloody)
3. A stage of pericardial thickening by fibrous tissue and
granulomas
4. A stage of cardiac constriction, in which the pericardial
space is obliterated by fibrous adhesions
In the fourth stage, the granulomas may disappear completely and be replaced by fibrous tissue, with or without
accompanying calcium deposits.3,5
A number of parasites have been reported to invade the
pericardial cavity. The most commonly encountered is
Entamoeba histolytica.5 Among fungi, Coccidioides,6
Actinomyces,7 Histoplasma,8 and Candida9,10 are known
causes of pericarditis.
Pericarditis, usually serofibrinous but occasionally
bloody, also occurs in association with viral diseases, including infectious mononucleosis, mumps, measles, smallpox,
and influenza. Coxsackieviruses are considered the most
common cause of viral pericarditis and probably account for
the majority of the cases of idiopathic pericarditis.
Pericardial involvement, ranging from fibrinous exudation (Fig. 67.1) to large, bloody effusions, occurs in collagen
vascular diseases,11 including acute rheumatic fever, rheumatoid arthritis, scleroderma, and systemic lupus erythematosus, as well as in chronic renal disease (uremic pericarditis).12,13
Bloody effusions also have been reported in patients with
Gaucher’s disease.
FIGURE 67.1. Fibrinous pericarditis. Fibrin is deposited over
the surface of the parietal and visceral pericardium. This fibrin
layer may subsequently be organized by an ingrowth of fibroblasts,
resulting in fibrous obliterative pericarditis. [Hematoxylin and eosin
(H&E), ×120.]
CAR067.indd 1480
FIGURE 67.2. Granulomatous pericarditis. The pericardium is
thickened by dense fibrous tissue. Nonspecific fibrocalcific granulomas are present on the pericardial surface. (H&E, ×120.)
Fibrous thickening of the pericardium can occur with or
without focal or diffuse adhesions (obliterative pericarditis)
forming between the visceral and parietal pericardium. Morphologic examination reveals that the fibrous thickening
results from the presence of large, coarse bundles of collagen,
which often are hyalinized. Very frequently, however, the
cause of the scarring is not evident on morphologic examination. Amyloid deposits can be a cause of constrictive or
restrictive pericardial and myocardial disease and should be
specifically sought in biopsy specimens from patients with
these clinical syndromes. Chronic fibrous pericarditis also
can result from the healing of hemopericardium in the presence of serosal injury, irradiation, chronic renal disease,
rheumatoid arthritis, systemic lupus erythematosus, scleroderma, or infectious agents, particularly tuberculosis. In
nearly all these processes, the fibrous thickening can be
associated with calcium deposits (fibrocalcific pericarditis).
Pericardial thickening also can be associated with
granulomatous inflammation (granulomatous pericarditis)
(Fig. 67.2).4 The granulomas may be caused by infective agents
(Mycobacterium tuberculosis, fungi, or parasites), cholesterol,
talc or starch (particularly in patients undergoing thoracic
surgical procedures), rheumatoid arthritis (in which they bear
a resemblance to rheumatoid nodules), and sarcoidosis (Figs.
67.3 and 67.4).14 Examination of tissue sections by polarized
light microscopy can be helpful in the identification of deposits of cholesterol, talc, and starch. Healed granulomas also can
undergo considerable degrees of calcification.
Obliterative and fibrocalcific pericarditis may be associated with the clinical syndromes of subacute pericarditis
and chronic constrictive pericarditis. The latter condition is
more often idiopathic than tuberculous in origin.
Pericardial Tumors
The pericardium is involved much more frequently with
metastatic neoplasms than with primary neoplasms, and
carcinomatous invasion is more common than sarcomatous
11/24/2006 1:39:37 PM
pe r ic a r di a l dise a se : a natom ic a bnor m a l i t i e s
FIGURE 67.3. Cholesterol pericarditis. Note the “cholesterol clefts,”
many of which have contiguous foreign body-type giant cells.
(H&E, ×250.)
14 81
pattern, the cells usually are strikingly regular in appearance, and the nuclei most frequently are large, rounded, and
vesicular, with prominent nucleoli. Cellular pleomorphism
and anaplasia are unusual, and atypical mitoses are rare,
although multinucleated cells and occasional mitoses may
be seen. Fibrous or mixed fibrous and epithelioid mesotheliomas predominate in the pericardium, as in the pleura.
Mesotheliomas, whether nodular or sheetlike, only
superficially invade contiguous structures, including the
heart. This is an important differential diagnostic point, in
that other primary cardiac sarcomas, most notably, angiosarcomas, can diffusely involve the pericardium but almost
invariably have a significant intramyocardial or intracavitary component.4,15
Pericardial mesotheliomas frequently spread to the adjacent pleura and mediastinum and may involve the mediastinal lymph nodes. Occasionally, pericardial mesotheliomas
spread through the diaphragm and involve the peritoneum.
Distant metastases are extremely unusual.4
Pericardial Cysts
invasion. The most common primary malignant tumor of
the pericardium is mesothelioma. The majority of pericardial mesotheliomas diffusely cover the parietal and visceral
pericardium, encasing the heart. Solitary or localized pericardial mesotheliomas are distinctly rare. Histologically,
localized mesotheliomas are identical to diffuse mesotheliomas and may be of the epithelioid or fibrous type.4,15
Microscopically, mesotheliomas are characterized by
cellular regularity and histologic variability. They consist of
either tubules or solid cords of malignant cells (in a tubular
or tubulopapillary pattern) or of spindle-shaped cells with a
connective tissue stroma (fibrous pattern). Frequently, both
patterns are present in the same tumor. In either histologic
Although most pericardial cysts are attached to the parietal
pericardium along the border of the right side of the heart,
usually at the right costophrenic angle, approximately 25%
are present along the border of the left side of the heart. Eight
percent project into the posterior or anterior superior mediastinum.4,16 The cysts range in diameter from 1 to 15 cm or
larger. They commonly appear multilocular externally;
however, although the cyst lining is occasionally trabeculated, most cysts are unilocular. They contain clear yellow
fluid and occasionally communicate with the pericardial sac.
The wall of the cyst is composed mainly of collagen and
scattered elastic fibers and is lined by mesothelial cells.
Although these mesothelial cells usually form a single layer,
foci or hyperplastic mesothelial cells are occasionally
encountered. Rarely, foci of calcification and accumulations
of lymphocytes and plasma cells are present.
The pericardial cyst and the pericardial diverticulum are
microscopically similar, and both probably originate as persistent, blind-ending parietal pericardial recesses.
Pericardial cysts may be detected in patients from childhood to old age, although the majority are discovered during
the third or fourth decade of life. Some tumors of the pericardium, such as lipoma, hemangioma, or lymphangioma,
may simulate the clinical and radiologic picture of pericardial cysts.4,16 Ultrasonic diagnosis and computed tomography
may be helpful in the differentiation of pericardial cysts from
solid tumors of the pericardium.
Summary
FIGURE 67.4. Rheumatoid pericarditis. Although any portion of
the heart may be involved in patients with rheumatoid arthritis, the
pericardium is the most common. Note that the fibrous thickening
of the pericardium contains a microscopic lesion with palisading
histiocytes and central necrobiosis suggestive of a rheumatoid granuloma. (H&E, ×250.)
CAR067.indd 1481
Pericardial disease frequently presents as pericarditis with
variable fluid accumulation (effusion). Important diagnostic
features relate to the character of the inflammatory cells and
nature of the fluid. Pericarditis can result from infectious
and noninfectious, inflammatory conditions, and may be
accompanied by mesothelial cell hyperplasia. The pericardium may be involved by metastatic or primary neoplasms
and cysts.
11/24/2006 1:39:38 PM
14 8 2
chapter
References
1. Taylor JR, Ruy J, Colby TV, et al. Lymphangioleiomyomatosis:
clinical course in 32 patients. N Engl J Med 1990;323:1254–
1260.
2. Ferrans VJ, Roberts WC. Pathology of pericardial effusion.
In: Reddy PS, Leon DF, Shaver JA, eds. Pericardial Disease.
New York: Raven Press, 1982:77.
3. Roberts WC, Ferrans VJ. A survey of the causes and consequences
of pericardial heart disease. In: Reddy PS, Leon DF, Shaver JA,
eds. Pericardial Disease. New York: Raven Press, 1982:49.
4. McAllister HA Jr, Fenoglio JJ Jr. Tumors of the Cardiovascular
System: Atlas of Tumor of Tumor Pathology, Second Series,
Fascicle 15. Washington, DC: Armed Forces Institute of
Pathology, 1974.
5. McAllister HA Jr, Ferrans VJ. Granulomas of the heart and
major blood vessels. In: Ioachim HL, ed. Pathology of Granulomas. New York: Raven Press, 1982:75.
6. Reingold JM. Myocardial lesions in disseminated coccidiomycosis. Am J Clin Pathol 1950;20:1044–1049.
7. Kasper JA, Pinner M. Actinomycosis of heart: report of case
with actinomycotic emboli. Arch Pathol 1930;10:687.
8. Crawford SE, Crook WF, Harrison WW, Somervill B. Histoplasmosis as a cause of acute myocarditis and pericarditis. Pediatrics 1961;28:92–95.
CAR067.indd 1482
67
9. McAllister HA Jr, Mullick FG. The cardiovascular system. In:
Riddell R, ed. Pathology of Drug Induced and Toxic Diseases.
New York: Churchill Livingstone, 1982:801.
10. Parker JC Jr. The potentially lethal problem of cardiac candidosis. Am J Clin Pathol 1980;73:356–361.
11. McAllister HA Jr. Collagen diseases and the cardiovascular
system. In: Silver MD, ed. Cardiovascular Pathology. New York:
Churchill Livingstone, 1991:1151.
12. Buja, LM, Friedman CA, Roberts WC. Hemorrhagic pericarditis
in uremia. Clinicopathologic studies in six patients. Arch
Pathol 1970;90:325–330.
13. McAllister, HA Jr. Pathology of the cardiovascular system in
chronic renal failure. In: Laventhol DT, Pennock RL, Likoff W,
et al., eds. Management of Cardiovascular Disease in Renal
Failure. Philadelphia: FA Davis, 1981:1.
14. Roberts WC, McAllister HA Jr, Ferrans VJ. Sarcoidosis of the
heart: a clinicopathologic study of 35 necropsy patients (group
I) and review of 78 previously described necropsy patients
(group II). Am J Med 1977;63:86–108.
15. McAllister HA Jr. Tumors of the heart and blood vessels. In:
Silver MD, ed. Cardiovascular Pathology. New York: Churchill
Livingstone, 1991:1297.
16. Feigin DS, Fenoglio JJ, McAllister HA Jr, Madewell JE. Pericardial cysts: a radiologic-pathologic correlation and review.
Radiology 1977;125:15–20.
11/24/2006 1:39:38 PM