Showkat Haji, M'D'

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Cath ConferenceAugust 31,2004Cardiac tumors
Showkat Haji, M.D. Lawrence OMeallie, M.D.
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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

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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.

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Distinguishing Characteristics of Intracardiac
Masses
Adapted from the Textbook of clinical
echocardiography/ Catherine M. Otto. 2nd ed
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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

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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 Burkitts
  lymphoma.

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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.

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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
  (301)
• 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

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Cardiac tumors (Incidence of benign tumors)
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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.

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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

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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.

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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.

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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.

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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.

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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