Download Malignant primary cardiac tumors

Cath Conference
August 31,2004
Cardiac tumors
Showkat Haji, M.D.
Lawrence O’Meallie, M.D.
Case 1
• 24 y/o wm transferred from St. Tammany Hospital due to
multi-organ failure
• Eight months before this admission patient was
found to have a mediastinal mass measuring
21x18x31cm with transthoracic needle biopsy
confirming Non Hodgkins lymphoma. There was no
other involvement from the lymphoma. Cardiac function
by echocardiogram was normal.
Pt received 8 cycles of CH(Hydrea-Doxorubicin)OP
Case 1
• Three weeks prior to this admission, pt felt tired and
short of breath and was admitted to St tamany
hospital with multi-organ failure. Pt was in respiratory
failure, renal failure and had evidence of shock liver.
Echocardiogram showed LV systolic dysfunction and
a LV mass.
• on arrival at Tulane pt was sedated, intubated and
hemodynamically stable.
Labs showed a WBC count of 22,000, plt 198,000
Creatinine of 2.1, Bilirubin 6.4, AST 14758, ALT 5015,
Alk phos 105, Troponin of 3.0.
• Echocardiogram was obtained.
Distinguishing Characteristics of Intracardiac Masses
Characteristic
Location
Thrombus
Tumor
Vegetation
LA (especially when enlarged
or associated with MV
disease)
LV (in setting of reduced
systolic function or segmental
wall abnormalities)
Atria (myxoma), more
frequent in LA
Myocardium
Pericardium
Valves
Usually valvular
Occasionally on ventricular
wall
Usually discrete and
somewhat spherical in shape
or laminated against LV apex
or LA wall
Various: may be circumscribed
or may be irregular
Irregular shape, attached to
the proximal (upstream) side
of the valve with motion
independent from the valve
Intracardiac obstruction
depending on site of tumor
Arrhythmias
Pericardial effusion, which
usually is bloody
Valvular regurgitation usually
present
Clinically: fever, systemic
signs of endocarditis, positive
blood cultures
Appearance
Associated
findings
Underlying etiology usually
evident (atrial fibrillation)
LV systolic dysfunction or
segmental wall motion
abnormalities (exception
eosinophilic heart disease)
MV disease with LA
enlargement
Adapted from the Textbook of clinical echocardiography/ Catherine M. Otto. – 2nd ed
Case 1
• PET scan showed a superior mediastinal mass without
involvement of cardiac structures. There were no other
sites of involvement.
• Gallium scan showed hot spots in the upper and middle
mediastinum consistent with upper mediastinal mass
saving the cardiologists the pain of a definitive diagnosis
of the LV mass.
• Pt was treated with Rituximab and the mass in the LV
was presumed to be a LV thrombus.
• Pt was aggressively treated for heart failure with the
heart failure cocktail. Amiodarone was started for NSVT
Case 2
• 28 year-old African American male with history of HIV
diagnosed in 1998 and a CD4 count of 1,050, underwent
a Bilroth II procedure for a perforated peptic ulcer.
• Due to persistent abdominal pain a laparotomy was
done a week later and demonstrated significant
lymphadenopathy
• A pathology report of an intra abdominal lymph node
was consistent with non-Hodgkin Burkitt’s lymphoma.
Case 2
• An abdominal and chest computed tomography
demonstrated mediastinal lymphadenopathy with
invasion of cardiac structures
• An echocardiogram showed biatrial masses, which
were non-mobile and attached to the roof of both atria.
Moderate pericardial effusion, left atrial enlargement
and left ventricular hypertrophy were also present
Systolic function was preserved.
• A combination chemotherapy regimen consisting of
rituximab, cytarabine, vincristine and prednisone was
initiated.
Cardiac tumors
• Primary cardiac tumors are rare, with an autopsy
incidence of 0.05%
• A quarter of primary tumors are malignant, the vast
majority being angiosarcomas or Rhabdomyosarcomas
• Metastatic tumors are more common than primary (30:1)
• Neoplasms may involve only the endocardium,
myocardium or the epicardium or various combinations
Neoplasms limited to parietal pericardium without
extension into the epicardium are not considered cardiac
neoplasms
Cardiac tumors (Incidence of benign tumors)
Incidence %
Adults
Myxoma
Lipoma
Pappilary Fibroelastoma
Angioma
Fibroma
Haemangioma
Rhabdomyoma
Teratoma
45
20
15
5
3
5
1
<1
Children
15
5
15
3
45
15
Cardiac tumors (General clinical features)
• Atrial myxomas may cause systemic symptoms such as fatigue,
fever, erythematous rash, arthralgia, myalgia and wt. loss
Elevations in ESR, CRP and globulin levels
Irrespective of size and site.
• Clinical features common to all tumors include
Embolization: Tumor or an attached thrombus may dislodge
Multiple small emboli may mimic vasculitis
Obstruction: Mimic valvular stenosis. May cause outflow
obstruction leading to chest pain, breathlessness or syncope
Arrhythmias: AV blocks, V. Tachycardia
Initial presentation may be with sudden death.
Myxomas
• 90 % are left atrial
• 90 % are solitary
• Associated facial freckling should raise the possibility of Carney
complex, in which case family members should be screened.
• Clinical features may mimic infective endocarditis, vasculitis
or other inflammatory disorders
• Differentiation from an intra-atrial thrombus may be important.
• Surgical resection is advisable as soon as possible after diag
as the risk of embolization is high
• Recurrence is possible. Long term ECHO f/u is recommended
Other benign cardiac tumors
• Pappilary fibroelastomas have a high incidence of embolization
• Rhabdomyoma is the most common neoplasm of childhood,
often seen in association with tuberous sclerosis. Spontaneous
tumor resolution is common
• Lipomas are encapsulated and usually asymptomatic
• Angiomas are extremely rare.
Malignant primary cardiac tumors
• The majority of malignant primary cardiac tumors are sarcomas
(95%) usually angiosarcomas or rhabdomyosarcomas.
• Most malignant primary cardiac tumors are right atrial in location
• The incidence of primary intracardiac lymphomas is increasing,
seen as part of AIDS.
• Treatment is rarely curative and survival is poor.
Secondary cardiac tumors
• Secondary cardiac tumors are usually epicardial and
asymptomatic.
• Metastasis is rarely limited solely to the heart.
• Pericardial effusion is common
• Melanoma, leukemia and lymphoma are most commonly
associated with metastasis to the heart.
• Carcinoma of the lung is the most commonly encountered
metastatic tumor followed by cancer of breast, lymphoma and
leukemia.
Non neoplastic conditions
• Pericardial cyst: These are frequent benign tumors of the
pericardium. They are asymptomatic and found on a routine
chest radiograph.
• Lipomatous hypertrophy of the atrial atrium: Extremely
common condition almost exclusively in patients above 50.
Limited to obese people.
Atrial septum may be as thick as 7 cm.
May present as atrial arrhythmias.
Treatment is simply wt. loss.
• Thrombi: Sometimes biopsy is the only way to differentiate.
• Cardiac hemorrhages: Thrombocytopenia if persistent, can
result in focal epi, myocardial and endocardial hemorrhage.
Heart and malignant lymphoma
• Primary lymphomas of the heart are very rare, although
incidence has been rising as they are seen as part of AIDS.
• Chief presentation is with intractable heart failure.
• Secondary involvement is more common
20% of patients dying of lymphoma have cardiac involvement.
• Parietal pericardium is involved in most of the patients
• Pericardial effusions are common
• Usually associated with diffuse metastasis