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A. Primary Cardiac Tumors navigator

  1. Rare tumors, ~0.05% of all autopsies
  2. 75% of tumors are benign
  3. ~50% of benign cardiac tumors are myxomas
  4. Remaining benign tumors are lipomas, fibroelastomas, and rhabdomyomas [3]
  5. Cardiac hamartomas have also been reported [4]
  6. Of malignant cardiac tumors, ~35% are angiosarcomas [5]
  7. Cardiac lymphomas are uncommon, increased in HIV infection, usually associated with EBV [6]

B. Characteristics of Myxomasnavigator

  1. Most common type of primary cardiac tumors in adults
  2. Unclear etiology of myxomatous cells in atria
    1. Arise from left side of interatrial septum at fossa ovalis
  3. Most common in ages 30-60, usually women
  4. Do not typically recur after surgical resection
    1. ~7% are a component of hereditary syndrome called "Carney Complex" (see below)
    2. These tumors do recur
  5. Types of Myxomas
    1. Solid / Ovoid Myxomas (clusters and tortuous vessels, highly vascular with fibrosis)
    2. Soft / Papillary Myxomas (little fibrosis, few hemorrhages, no tortuosity)
  6. Location
    1. Left Atrium 75%
    2. Right Atrium 20%
    3. Ventricles (L>R) ~5%
  7. Most produce Interleukin 6
    1. Cytokine associated with acute and chronic inflammation
    2. Induces acute phase reactant proteins in the liver
    3. Likely responsible for systemic symptoms of atrial myxomas

C. Presentation of Myxoma [7]navigator

  1. Large cardiac masses may present with syncope and/or arrhythmias [8]
  2. Systemic Symptoms Common [8]
    1. Fever
    2. Weight Loss
    3. Night Sweats
    4. May mimic vasculitis
    5. Anemia - usually chronic disease type
    6. Probably related to interleukin 6 production
  3. Dyspnea
    1. More common with solid / ovoid myxomas
    2. May be due to intracardiac obstruction
  4. Syncope [1,8]
  5. Peripheral Embolization [1]
    1. Occurs in 30-40% of patients
    2. Renal disease
    3. Digital ischemia
    4. Stroke - more common with soft / papillary myxomas
    5. Microemboli may embed in vessel walls and cause aneurysms
    6. Mesenteric ischemia
  6. Intermittent Claudication
  7. Rash
  8. Infection - myxoma may occasionally become infected
  9. Carney Complex [8,9,10,11]
    1. Includes both LAMB and NAME Syndromes
    2. Primary pigmented nodular adrenocortical disease (PPNAD)
    3. Bilateral micronodular adrenal disorder often with hypercortisolism
    4. Associated multiple endocrine and non-endocrine cancers
    5. These include skin lentigines, cardiac myxomas, and various other tumors
    6. ~50% of cases due to mutations in PRKAR1 alpha gene on chromosome 17q2
    7. PRKAR1a encodes regulatory subunit of R1alpha of cAMP-dependent protein kinase A

D. Evaluationnavigator

  1. Diastolic murmur is sometimes present
  2. Echocardiography is indicated if syndrome suspected
    1. Transthoracic echocardiography is sufficient in most cases
    2. Transesophageal echocardiogram may be more sensitive for R sided masses [12]
  3. MRI or CT scanning can also detect mass; angiography is used occasionally
  4. Erythrocyte Sedimentation Rate and C-reactive protein often very high
  5. White blood count may be elevated
  6. High interleukin 6 levels are usually present
  7. In appropriate patients, rule out components of Carney Complex (mainly elevated cortisol)

E. Treatmentnavigator

  1. Symptomatic treatment with anti-inflammatory agents (? glucocorticoids)
  2. Surgical Resection (on heart-lung bypass) usually required and preferred
  3. Atrial fibrillation may occur and should be treated in standard fashion
  4. Myxomas occasionally (~2%) recur, usually due to incomplete resection

References navigator

  1. Januzzi JL, Garasic JM, Neilan TG, et al. 2007. NEJM. 357(11):1137 (Case Record) abstract
  2. Reynen K. 1995. NEJM. 333(24):1610 abstract
  3. Cohn LH and Pins MR. 1997. NEJM. 336(21):1512 (Case Record) abstract
  4. Greenberg HM and Aretz HT. 1999. NEJM. 341(16):1217 (Case Record) abstract
  5. Collins JJ Jr and Pins MR. 1996. NEJM. 334(7):452 (Case Record) abstract
  6. Kaplan LD, Afridi NA, Holmvang G, Zukerberg LR. 2003. NEJM. 349(14);1369 (Case Record) abstract
  7. Morton-Bours EC, Jacobs MB, Albers GW. 2000. NEJM. 343(1):50 (Case Discussion) abstract
  8. Basson CT and Aretz HT. 2002. NEJM. 346(15):1152 (Case Record) abstract
  9. Stratakis CA, Sarlis N, Kirchner LS, et al. 1999. Ann Intern Med. 131(8):585 abstract
  10. Edwards RJ, Moss T, Sandeman DR. 2003. Lancet. 362(9395):1541 (Case Report) abstract
  11. Tsao H, Sober AJ, Niendorf KB, Zembowicz A. 2004. NEJM. 350(9):924 (Case Record) abstract
  12. Daniel WG and Mugge A. 1995. NEJM. 332(19):1268 abstract