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Copyright © 2002, The Royal Society of Medicine Cardiac myxoma with three recurrences Cardiothoracic Surgery, University Hospitals Coventry & Warwickshire Hospitals NHS Trust, Coventry, W Midlands, UK 1 University College London Medical School, London, UK Correspondence to: Mr H A Vohra, 359 Roding Lane North, Woodford Green, Essex
IG8 8LH, UKE-mail:
hunaidvohra/at/yahoo.co.uk CASE HISTORY At the age of 21 a woman underwent thoracotomy for removal of a left atrial
myxoma. Her mother and sister had both died suddenly, at ages 31 and 19;
necropsy in the sister had revealed a left atrial myxoma. 12 years after
operation, the patient complained of shortness of breath, palpitations, and a
persistent ache and paraesthesiae in the right fore-arm and fingers. On
examination she had a systolic murmur at the left parasternal edge, and
investigations showed reappearance of the tumour, this time in the right
atrium in the region of the fossa ovalis. A sternotomy was performed and a 10
cm long and 1 cm diameter mass of myxoma arising from the limbus was widely
excised with the fossa ovalis. The defect was repaired with Dacron. 11 years
later, recurrence of the tumour was discovered in the right ventricle with
involvement of the tricuspid valve, after the onset of bilateral claudication.
A sternotomy was performed again, and an enormous tumour was found in the
right ventricle with involvement of the tricuspid valve and several papillary
muscles. The tumour was excised with the tricuspid valve, which was replaced
with a Carpentier—Edwards xenograft. A 2 cm tumour in the left atrium
was also excised, the defect being repaired with a patch. The left ventricle
was explored but no tumour was found on the mitral or aortic valves. 16 years
later a cardiac murmur was heard on routine follow-up and transoesophageal
echocardiography revealed a 3 cm lesion, this time on the posterior mitral
valve leaflet. The tumour was very mobile and was flipping in and out of the
left atrium during diastole and systole
(Figure 1
COMMENT Even in families, multiple recurrences of this kind have seldom been
reported3. Myxomas
can develop in any chamber of the heart, the most common site being the left
atrium; often they are bilateral. In families the disorder is transmitted in
autosomal dominant fashion and has a predeliction for young
women4. It can also
be a component of Carney's complex, a familial multiple neoplasia/lentiginous
syndrome. A family history should be sought in all patients with cardiac
myxoma. Cardiac myxomas sometimes present with valvular obstruction, which can give
a picture of mitral disease or right heart failure. Neurological deficits tend
to arise when the myxoma gets infected and the friable tumour tissue embolizes
to the brain or the limbs. Some myxomas are sufficiently mobile to move
through the atrioventricular valves during diastole, exerting a
‘wrecking ball’ effect that damages the valve leaflet and causes
anaemia and a high erythrocyte sedimentation rate. Constitutional symptoms
such as fever, malaise and weight loss can result from elaboration of the
cytokine interleukin-6. Right bundle block has been recorded in as many as
one-third of cases. The most useful diagnostic investigation is
echocardiography, though large vegetations, an infected thrombus, or even
mitral valve prolapse can produce patterns that are indistinguishable from
myxoma. Transthoracic echocardiography is the most commonly used, but
transoesophageal echocardiography has better specificity and sensitivity. The recurrent myxomas can be divided into four groups—inadequate
resection, familial, totipotent multicentricity and metastatic recurrence.
Multifocal disease is frequent in the familial
setting4. Recurrence
has been associated with abnormal DNA ploidy in up to 40% of the patients.
Indeed, DNA testing of all patients with cardiac myxoma may prove to be the
best predictor of the likelihood of
recurrence5. Surgical excision must be done as soon as possible after diagnosis because
of the high risk of valve obstruction or systemic embolization. Ideally, the
tumour should be excised with a large cuff of atrial septum. Valve replacement
may be necessary, and there is a report of cardiac transplantation in a woman
with recurrent
disease6. References 1. Carney JA. Differences between non-familial and familial cardiac
myxoma. Am J Surg Pathol
1985;9:
53-5 [PubMed] 2. McCarthy PM, Piehler JM, Schaff HV, et al. The
significance of multiple, recurrent and ‘complex’ cardiac myxomas.
J Thorac Surg
1986;91:
389-96. 3. Gray IR, Williams WG. Recurring cardiac myxoma. Br Heart
J 1985;53:
645-9 [PubMed] 4. Siltanen P, Tuuteri L, Norio R, Tala P, Ahrenberg P, Halonen PI.
Atrial myxoma in a family. Am J Cardiol
1976;38:
252-6 [PubMed] 5. Seidman JD, Berman JJ, Hitchcock CL, et al. DNA analysis
of cardiac myxomas: flow cytometry and image analysis. Hum
Pathol 1991;22:
494. [PubMed] 6. Goldstein DJ, Oz MC, Michler RE. Radical excision therapy and total
cardiac transplantation for recurrent atrial myxoma. Ann Thorac
Surg 1995;60:
1105-7 [PubMed] |
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Am J Surg Pathol. 1985 Jan; 9(1):53-5.
[Am J Surg Pathol. 1985]Br Heart J. 1985 Jun; 53(6):645-9.
[Br Heart J. 1985]Am J Cardiol. 1976 Aug; 38(2):252-6.
[Am J Cardiol. 1976]Am J Cardiol. 1976 Aug; 38(2):252-6.
[Am J Cardiol. 1976]Hum Pathol. 1991 May; 22(5):494-500.
[Hum Pathol. 1991]Ann Thorac Surg. 1995 Oct; 60(4):1105-7.
[Ann Thorac Surg. 1995]