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Case Reports

Indian Pediatrics 2001; 38: 1190-1192  

Recurrent Left Atrial Myxoma with Recurrent Stroke


Apurba Ghosh, Arunaloke Bhattacharyya, Prabal Niyogi

From the Institute of Child Health, 11, Dr. Biresh Guha Street, Kolkata 700 017, India.

Correspondence to: Dr. Apurba Ghosh, Director, Institute of Child Health, 7 Central Park, Flat No. 3B, Kolkata 700 032, India.E-mail: [email protected]

Manuscript received: June 12, 2000; Initial review completed: July 24, 2000; Revision accepted: May 3, 2001.

Myxomas are the most common type of primary cardiac tumor. According to the statistics from UK, the incidence of myxoma is around 1:1,000,000/year(1). Though the majority of sporadic myxoma occurs in adults, it has also been infrequently reported in children from 3 years of age. Approxi-mately 86% of myxomas occur in left atrium and over 90% are solitary. Left atrial myxomas generally arise in the area of fossa ovalis, but occasionally may arise from mitral valve also. Tumor arising from the posterior wall should arouse suspicion of malignancy. We report an unusual case of recurrent left atrial myxoma.

Case Report

About two years back, a 5-year-old girl presented with fever for 3 days followed by generalized convulsion, unconsciousness and hemiplegia of left side of body 12 hours prior to admission. Neurological examination revealed the patient to be in grade 8/15 of Glasgow Coma Scale (GCS). She had upper motor neuron type of left sided hemiplegia. Other systemic examinations including cardiovascular system were normal. Routine blood examination, CSF examination and CT scan of brain were normal. Echocardiography done at that time showed a large left atrial myxoma (Fig.1). An emergency excision of myxoma was done. The myxoma was very friable arising between two right pulmonary veins. Mitral valve and septum were free. Post operative echocardiography showed no rem-nant of myxoma, mild mitral regurgitation, good biventricular systolic function, no pulmonary arterial hypertension and no pulmonary embolism. Subsequent echo-cardiographies done at two, eight and twelve months after operation were normal.

About four months later (one year and four months after operation) the same girl was admitted in our institution with history of focal seizure starting from face with secondary generalization followed by altered sensorium. On examination the patient was in grade 10/15 of GCS. Cranial nerve examina-tion and deep reflexes were normal. Plantar response were bilateral withdrawal. There was loss of bladder and bowel control. Other systemic examination including cardiovascu-lar system were normal. As the patient had a very suggestive past history, we went for another echocardiography and MRI scan of brain. Echocardiography revealed a recurrence of myxoma which was fairly large in size and was arising from the anterior mitral valve leaflet. MRI scan of brain showed fresh ischemic infarction pattern around parietooccipital area in left middle cerebral artery (MCA) territory. It also revealed ischemic infarction around right parietooccipital area involving MCA terri-tory. The patient was referred to a cardiac surgeon for further management.

Fig. 1. Echocardiography showing the initial left atrial myxoma.

Discussion

The etiology of myxoma is unknown. Widespread abnormality resulting in exces-sive proliferation of certain mesenchymal cells with excessive glycosaminoglycans (GAG) production has been proposed, quite analogous to the neural masses of Recklinghausen’s neurofibromatosis. Clonal structural aberrations were reported by Dijkhuizen with normal chromosomal number but complex clonal rearrangement with a break point at 12p12, the location of ki-ras oncogene. This ki-ras oncogene may play a role in the origin of cardiac myxoma(2).

In about 1 to 5 per cent of cases, a recurrence or second cardiac myxoma has been reported following the removal of initial myxoma(3,4). The possible causes of recur-rence are growth from a "Pretumorous" focus, i.e., metasynchronous, incomplete excision or intracardiac implantation from original tumor. Local recurrence can also occur due to malignant change. Of the various risk factors of recurrence young age is important(5) as in our case. Myxoma is the most frequent cardiac tumor that has the capability to regrow; even after 11 years regrowth had been documented(6). The rate of the growth of the second tumor is more rapid than that of the initial tumor(7). In our case it took only about four months time to regrow. Hence all patients who have resection of myxoma need years of intense and close follow up with echocardiography and in case of suspicion, an additional transesophageal echocardiography or magnetic resonance imaging may be performed(8). Myxomas can cause cardiac and cerebral embolism(9) and this case had two episodes of cerebral emobolism leading to infarction of brain. Previously, one such case was reported where the patient died three and half years after the second operation(1).

Contributors: All the authors helped in drafting the manuscript and management of case. AG will act as guarantor for the paper.

Funding: None.
Competing interests:
None stated.

Key Messages

  • Atrial myxomas should be considered in the differential diagnosis in cases of fainting spells, seizure, a changing heart murmur or evidence of cerebral embolism and congestive cardiac failure.

  • These are reported in adults but children can also be affected.

  • Rarely recurrences can occur.

  • Complete surgical excision and careful handling of the primary tumor per operatively are of utmost importance and regular echocardiographic follow up for several years is needed to detect early recurrence.
 
 References


1. Alkhulaifi Am, Horner S, Pugsley WB, Sturridge MF. Recurrent left atrial myxoma. Cardiovas Surg 1994; 2: 232-236.

2. Dijkhuizen T, van den Berg E, Molenaar WM. Cytogenetics of a case of cardiac myxoma. Cancer Genet Cytogenet 1992; 73: 73.

3. Waller DA, Ettles DF, Saunders NR, Williams G. Recurrent cardiac myxoma: The surgical implications of two distinct groups of patients. Thorac Cardiovasc Surg 1989; 37: 226-228.

4. McCarthy PM, Piehler JM, Schaff HV, Plinth JR, Orszulak RA, Vidaillet JH, et al. The signi-fiance of multiple, recurrent and "complex" cardiac myxomas. Thorac Cardiovasc Surg 1986; 91: 389-392.

5. Waller DA, Ettles DF, Saunders NR, Williams G. Recurrent cardiac myxoma: The surgical implications of two distinct groups of patients. Thorac Cardiovas Surg 1989; 37: 226-230.

6. Gray IR, Williams WG. Recurring cardiac myxoma. Brit Heart J 1985; 53: 645-649.

7. Mertl C, Lecuyer D, Plesk of A, Quiret JC, Bernasconi P. Asymptomatic recurrence of a left auricular myxoma. Archives des Maladies du Coeur et des Vaisseaux 1987; 80: 658-661.

8. Reynen K, von der Emde J, Kunkel B, Bachmann K. Recurrent myxoma. Zeitschrift fur Kardiologie 1993; 80: 658-662.

9. Hashimoto H, Takahashi H, Fujiwara Y, Joh T, Tomino T. Acute myocardial infarction due to coronary embolization from left atrial myxoma. Jpn Circ J 1993; 57: 1016-1018.

10. Jugdutt BI, Rossall RE, Sterns LP. An unusual case of recurrent left atrial myxoma. CMAJ. 1975; 112(9): 1099-1100.

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