1. Neoplastic Pericardial DiseaseBY
Prof. A. Frere
MD Cardiology
Zagazig- Egypt

2. Epidemiology
The pericardium is a common target for neoplastic involvement, usually manifest as pericardial effusion, cardiac tamponade, or constrictive pericarditis
Among patients with acute pericardial disease
majority have idiopathic or viral pericarditis
6% have malignant etiology in reported series
Am J Cardiol 1995; 75:378

3. Epidemiology
Attempts to make a specific diagnosis are generally limited to patients with
persistent pericarditis unresponsive to antiinflammatories
cardiac tamponade
a new large pericardial effusion
Among such patients in whom a diagnosis is established, malignancy has accounted for 25 to 40 percent of cases
Am J Med 1993; 95:209

6. PERICARDIAL DISEASE IN PATIENTS WITH KNOWN MALIGNANCY
Several neoplasms metastasize or spread either directly or, more commonly, via lymphatics to the pericardium
The most common of these neoplasms are carcinomas of the lung or breast and the lymphomas
Primary pericardial neoplasia, particularly mesothelioma, hemangioma, or angiosarcoma, is seen much less frequently

7. MYOCARDIAL DISEASE IN PATIENTS WITH KNOWN MALIGNANCY
Most pericardial tumors do not invade the myocardium
However, myocardial involvement is a classic feature of melanoma and is common in leukemia
Myocardial neoplasia is usually silent and discovered only at autopsy or, less often, during surgery

8. Pericardial Effusion in Patients with Known Malignancy
Malignant pericardial effusion is often asymptomatic and discovered by imaging or at autopsy
Once effusion becomes clinically apparent, morbidity and mortality are high, since many symptomatic patients have cardiac tamponade and constriction
Patients with constrictive pericarditis due to malignancy are unlikely to be candidates for radical pericardiectomy

9. Cytology and pericardial biopsy
Most patients with malignant pericardial disease are known to have a malignant tumor before evidence of pericardial involvement develops
Cytologic analysis and pericardial biopsy may disclose neoplasm in those cases in which pericardiopathy is the presenting manifestation of distant neoplasia

10. Cytology and pericardial biopsy
Sensitivity of cytology for malignant pericardial disease varies widely (eg, %).
Causes of false-negative cytology include
scant cellularity
the obscuring effect of blood
the absence of an expert exfoliative cytologist
sampling errors in pericardial biopsy
A negative cytology alone should not exclude the diagnosis of malignant disease.

11. Imaging studies Echocardiography CT and MRI
the primary imaging tool to establish pericardial effusion
useful to quantify the volume of the effusion and to evaluate its hemodynamic effects (tamponade or constrictive pericarditis)
CT and MRI
provide excellent information for loculated pericardial effusion
both are superior to echocardiography for determining whether an effusion is a transudate, exudate, or blood effusion
large tumor masses in the pericardium can be recognized by all of the imaging studies

12. Nonmalignant pericardial effusion
Patients with neoplasia can also develop nonmalignant pericardial disease
In two series of 241 patients with pericardial effusion and known malignancy, 45 to 60 % could not be shown to have neoplastic pericardial disease; a cause could be established in only one-quarter of these patients
Potential causes include
radiation, infection related to treatment
unrelated disorders: uremia, collagen vascular disease, and acute viral or idiopathic pericarditis that responds quickly to treatment with an antiinflammatory drug
Cancer 1995; 76: Cardiothorac Surg 1999; 16:287

13. Nonmalignant pericardial effusion
false-negative results should be suspected when the effusion does not behave in the predicted way
In patients with systemic signs such as
weight loss or persistent cough
abdominal pain or dyspepsia
a palpable mass in the breast or abdomen
abnormal opacity on CT, ultrasound, or chest radiogram
cytological is most useful when an expert exfoliative cytologist is available

14. Recommendations for pericardiocentesis or pericardial biopsy
In the absence of clues to
the presence of malignant pericardial disease or
clinical tamponade or constriction
pericardiocentesis or pericardial biopsy should be performed only in patients with persistent or progressive disease that is unresponsive to antiinflammatory therapy
Evaluation is also probably indicated for a new, large pericardial effusion

15. Treatment tamponade and compressive syndrome
Early tamponade with only mild hemodynamic compromise may be treated conservatively
with careful monitoring
repeated echocardiography
avoidance of volume depletion, and
therapy aimed at the underlying malignancy
To avoid missing effusive constrictive pericarditis, it is important to monitor right heart pressures before and after performing pericardiocentesis

16. Treatment asymptomatic effusion
Asymptomatic pericardial effusion associated with cancer does not require require therapy
Elective therapy include
Prolonged catheter drainage
Substernal pericardiotomy
Limited or radical pericardiectomy
Obliteration of the pericardial sac by intrapericardial injection of a sclerosing agent
Mayo Clin Proc 2000; 75:248

17. Treatment prolonged catheter drainage
The treatment of choice
simple, relatively atraumatic, safe, and effective
Intermittent, not continuous, drainage is preferred
drainage catheter may need to remain in place for several days until drainage is  ml/24 hrs
the benefit favors its retention over the slight increased risk of infection
to a considerable extent, it has obviated the need for sclerosing agent or pericardiotomy

18. Treatment balloon pericardiotomy
balloon pericardiotomy can be performed with low risk and often under local anesthesia, an important consideration for these patients in whom the quality of remaining life is paramount
However, the procedure is usually painful
given the choice, patients may prefer surgical pericardiotomy
J Am Coll Cardiol 1993; 21:1

19. Treatment substernal pericardiotomy
some physician use this method as initial treatment
majority employ it in cases of continuous (>4 days) copious drainage; a relatively infrequent problem
Prolonged catheter drainage and limited pericardiotomy are generally palliative measures in patients with a short expected survival

20. Treatment surgical pericardiotomy
For patients with the possibility of prolonged survival in whom the effusion recurs or progressively increases in size, surgical pericardiectomy is the definitive procedure with the intrapericardial injection of agents

21. Treatment sclerosing therapy
use of this approach is declining
sclerosing agents (such as tetracycline) generally have good results but are very painful
intrapericardial lidocaine should be used first
with prolonged survival, sclerosis may cause constrictive pericarditis
other compounds include cisplatin, thiotepa, carboplatin, OK432, interferon alfa-2b; discuss with an oncologist when using these agents
Hematol Oncol Clin North Am 1997; 11:253

22. PRIMARY PERICARDIAL NEOPLASIA
The most common are:
Mesothelioma, which may be benign, but is more frequently highly malignant
Sarcoma, particularly angiosarcoma
Hemangiomata, which are benign but can cause pericardial effusion
Intrapericardial pheochromocytoma which usually is associated with symptoms from catecholamine excess
Teratomata, neuroblastoma, and thymoma primarily occur in infants and children and may be benign or malignant

23. Am Heart J 1992; 124:802 Br Heart J 1987; 57:54
Mesothelioma
Although exceedingly rare, it is one of the most common primary malignant pericardial tumors
association with asbestos exposure is well established
It is important to diagnose because treatment can yield a year or more of good quality life
It most often arises in young or middle aged adults and may present with pericardial effusion, including tamponade or constrictive pericarditis
Am Heart J 1992; 124:802 Br Heart J 1987; 57:54

24. Mesothelioma MRI is most accurate when there is a discrete mass
Mesothelioma more often envelops the heart and causes rapidly progressive constrictive pericarditis
may be difficult to distinguish from pericardial constriction of other causes, such as tuberculous pericarditis

25. Lipoma Typically insignificant, but can assume gigantic size
May be mistaken for a huge pericardial effusion or massive cardiomegaly
The tumor may infiltrate the myocardium. When the ventricular septum is invaded, the pericardial and right ventricular cavity may communicate.

26. Pericardial (springwater) cyst
benign and almost always congenital
commonly located in the right cardiophrenic angle
appears as a perfectly round, fluid density, usually 2 to 4 cm in diameter, filled with clear liquid
rarely, a pericardial cyst may calcify or rupture
secure diagnosis can be made with CT or MRI
no treatment is required
Acquired pericardial cysts are exceedingly rare - seen with neoplastic, parasitic, or traumatic disease
Chest 1971; 60:611