Correspondence

More on Cardiac Myxomas

N Engl J Med 1996; 335:1462-1464November 7, 1996

Article

To the Editor:

The correspondence regarding benign cardiac myxoma, malignant myxoma, and cardiac sarcoma (May 23 issue)1-3 clouds, rather than clarifies, a subject that is generally misunderstood by clinicians and pathologists. Figure 1 of the case reported by Kairemo et al.1 is clearly that of a cardiac sarcoma, but the report may unwittingly further the notion that malignant myxomas exist. Most, if not all, reported “malignant myxomas” are simply cardiac sarcomas that have been misdiagnosed, including those in reports cited in the correspondence.4-6 The figure provided in the report by Hannah et al.4 shows a sarcoma; Rankin and DeSousa5 do not provide a picture of the tumor; and Pastakia6 admits that the tumor reported has the histologic characteristics of a sarcoma.

Cardiac myxomas differ histologically and biologically from cardiac sarcomas7,8 (Figure 1AFigure 1Cardiac Myxoma (Panels A and B) and Cardiac Sarcoma (Panels C and D)., Figure 1B, Figure 1C, and Figure 1D). Although myxomas may occasionally cause death as a result of embolization, the recurrence rate of cardiac myxoma, if the results of all series are taken together, is less than 2 percent. In contrast to myxomas, cardiac sarcomas have a dismal prognosis, with death usually occurring within months. Cardiac sarcomas can be distinguished histologically from myxomas by the absence of syncytial structures forming cords and rings and the absence of abundant background hemosiderin. Unlike sarcomas, cardiac myxomas are not the result of a proliferation of atypical spindled cells, but rather polygonal or stellate “myxoma” cells forming syncytia.

Why is there such confusion between two entities whose biologic behavior and histologic appearance are so fundamentally different? The majority of cardiac sarcomas (which are not angiosarcomas) arise, like myxomas, as endocardial-based left atrial tumors. A myxoid background rich in proteoglycans is frequent in myxoid sarcomas, but it is likewise frequent in fibrous sarcomas of extracardiac soft tissue. The similarity between cardiac myxomas and cardiac sarcomas ends with these two common features. However, because most pathologists lack experience with cardiac tumors, the distinct histologic differences have often been overlooked.

In our consultation service, which has enabled us to review hundreds of cardiac myxomas and cardiac sarcomas, we have repeatedly encountered myxoid sarcomas of the atrium that are misdiagnosed, both clinically and pathologically, as myxomas and have yet to encounter a malignant myxoma. We plead with practicing pathologists and clinicians to study the histologic features of atrial tumors carefully before making the diagnosis of myxoma, especially if the gross and imaging studies are atypical. In addition, we strongly urge that reports of “malignant myxomas” be carefully reviewed for diagnostic accuracy.

Allen Burke, M.D.
Renu Virmani, M.D.
Armed Forces Institute of Pathology, Washington, DC 20306-6000

8 References

1. 1

   Kairemo KJA, Blomqvist CP, Miettinen M. Cardiac myxomas. N Engl J Med 1996;334:1407-1408
   Full Text | Web of Science | Medline

2. 2

   Stern R. Cardiac myxomas. N Engl J Med 1996;334:1408-1408
   Web of Science | Medline

3. 3

   Reynen K, Daniel WG. Cardiac myxomas. N Engl J Med 1996;334:1408-1409
   Web of Science | Medline

4. 4

   Hannah H III, Eisemann G, Hiszcznskyj R, Winsky M, Cohen L. Invasive atrial myxoma: documentation of malignant potential of cardiac myxomas. Am Heart J 1982;104:881-883
   CrossRef | Web of Science | Medline

5. 5

   Rankin LI, DeSousa AL. Metastatic atrial myxoma presenting as intracranial mass. Chest 1978;74:451-452
   CrossRef | Web of Science | Medline

6. 6

   Pastakia B. Malignant atrial myxoma presenting as intracranial mass. Chest 1979;75:531-532
   CrossRef | Web of Science | Medline

7. 7

   Burke AP, Virmani R. Cardiac myxoma: a clinicopathologic study. Am J Clin Pathol 1993;100:671-680
   Web of Science | Medline

8. 8

   Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer 1992;69;387-95.
   

Author/Editor Response

The authors reply:

To the Editor: In our reply to Kairemo et al., we stated that they were reporting a malignant primary heart tumor — a malignant fibrous histiocytoma — and not a myxoma, the most common benign tumor of the heart. Clinically, however, myxomas may also be lethal, as a result not only of embolization, but also of valve obstruction.

Our original comment, before editing, on the malignant potential of cardiac myxomas described in the literature was as follows: Although histologically benign, myxomas are nevertheless considered sometimes to possess malignant potential. In bona fide histologically benign myxomas, extensive local invasion of cardiac tissues 1 and remote growth of embolized tumor material have been observed, with and without malignant transformation.2,3 We share Dr. Burke's and Dr. Virmani's reservations concerning the malignant potential of cardiac myxomas.

Klaus Reynen, M.D.
Werner G. Daniel, M.D.
University Clinic Dresden, D-01307 Dresden, Germany

3 References

1. 1

   Hannah H III, Eisemann G, Hiszcznskyj R, Winsky M, Cohen L. Invasive atrial myxoma: documentation of malignant potential of cardiac myxomas. Am Heart J 1982;104:881-883
   CrossRef | Web of Science | Medline

2. 2

   Kotani K, Matsuzawa Y, Funahashi T, et al. Left atrial myxoma metastasizing to the aorta, with intraluminal growth causing renovascular hypertension. Cardiology 1991;78:72-77
   CrossRef | Web of Science | Medline

3. 3

   Wada A, Kanda T, Hayashi R, Imai S, Suzuki T, Murata K. Cardiac myxoma metastasized to the brain: potential role of endogenous interleukin-6. Cardiology 1993;83:208-211
   CrossRef | Web of Science | Medline

Author/Editor Response

We agree with Burke and Virmani that cardiac sarcoma must be distinguished from myxoma histologically and clinically, although a sarcoma located in the left atrium may simulate cardiac myxoma clinically. The case we described was clearly that of a pleomorphic cardiac sarcoma (malignant fibrous histiocytoma), as stated in the text and legend of our figure. Our main intent was to describe an elegant method of diagnosing such lesions in vivo.

Kalevi J.A. Kairemo, M.D., Ph.D.
Carl P. Blomqvist, M.D., Ph.D.
Helsinki University Central Hospital, FIN-00290 Helsinki, Finland

Citing Articles (8)

Citing Articles

1. 1

   Andrea Garatti, Giovanni Nano, Alberto Canziani, Piervincenzo Gagliardotto, Eugenio Mossuto, Alessandro Frigiola, Lorenzo Menicanti. (2012) Surgical Excision of Cardiac Myxomas: Twenty Years Experience at a Single Institution. The Annals of Thoracic Surgery
   CrossRef

2. 2

   Nikhil Prakash Patil, Nilanjan Dutta, Subodh Satyarthy, Muhammad Abid Geelani, Deepak Kumar Satsangi, Amit Banerjee. (2011) Cardiac Myxomas: Experience Over One Decade. Journal of Cardiac Surgery 26:4, 355-359
   CrossRef

3. 3

   Masato Suzuki, Hirosato Doi, Ryuji Koshima, Kenji Sugiki, Teruhisa Kazui, Takemi Ohno. (2011) Surgical treatment of cardiac myxosarcoma. General Thoracic and Cardiovascular Surgery 59:4, 284-287
   CrossRef

4. 4

   A.V. Skamrov, M.A. Nechaenko, L.E. Goryunova, E.S. Feoktistova, G.L. Khaspekov, D.A. Kovalevsky, L.I. Vinnitsky, G.F. Sheremeteva, R. Sh. Beabealashvilli. (2004) Gene expression analysis to identify mRNA markers of cardiac myxoma. Journal of Molecular and Cellular Cardiology 37:3, 717-733
   CrossRef

5. 5

   Thittamaranahaili Kariyapa Susheel Kumar, Mohammed Ali, Ajay Hirakannawar, Vidyadhur Shashikant Lad, Sanjeev Jadhav, Jayant Vasant Khandekar, Nandkishore Babulal Agrawal, Jagdish Mahadev Sinal Khandeparkar, Anil Madhav Patwardhan. (2004) Clinical experience and surgical considerations in the management of cardiac myxomas. Indian Journal of Thoracic and Cardiovascular Surgery 20:2, 77-82
   CrossRef

6. 6

   Tim Higenbottam, Liz Laude, Celia Emery, Mohamed Essener. (2004) Pulmonary hypertension as a result of drug therapy. Clinics in Chest Medicine 25:1, 123-131
   CrossRef

7. 7

   R Sharma, A Kumar, R Juneja, A Bhan, S Sharma, SK Choudhary, P Venugopal. (2000) Left ventricular recurrence of intracardiac myxoma: Literature review and a new surgical approach. Indian Journal of Thoracic and Cardiovascular Surgery 16:2, 106-109
   CrossRef

8. 8

   Amihay Shinfeld, Takahiro Katsumata, Stephen Westaby. (1998) Recurrent cardiac myxoma: seeding or multifocal disease?. The Annals of Thoracic Surgery 66:1, 285-288
   CrossRef