We’ve all heard that a man that acts as his own lawyer has a fool for a client. I suppose that adage could be extrapolated to medicine along the lines of “A man that acts as his own physician has a raving lunatic as a patient.” Now I’m not proposing to be my own doctor, but I have had some time on my hands to do some research as I’ve waited for the medical establishment to locate their mail.
I’ve tried to bear the big picture in mind as I’ve sorted through reams of medical articles. Specific symptoms I’ve experienced, the point of onset of those symptoms, how they compare with those described in the literature, what the echocardiogram allegedly reveals, etc. And I’ve overlaid those data against what I’ve read, allowed it all to settle in my head for a week, and presto! I have a working diagnosis. (Disclaimer: this exercise has been performed by a professional lunatic on a closed course – do not try at home.)
The first issue that must be settled relates to whether the tumor is benign or malignant. The echo report states that the tumor is “most likely consistent with an atypical atrial myxoma”, a benign tumor. Eighty-six percent of cardiac myxomas are located in the left atrium, with most of the remainder in the right atrium. According to the local cardiologist’s amended statement, my tumor is actually located in the left ventricle (not the atrium as stated in the report). Furthermore, myxomas have a tendency to embolize (fragment and travel via the blood stream) causing stroke. As I understand it, this is why the cardiologist urged me to undergo immediate open heart surgery. The fact that I have been symptomatic for at least a year but have not suffered a stroke, coupled with the tumor’s apparent location, suggests that it is not a benign myxoma. Conclusion: malignant.
The second issue to resolve relates to whether it is a primary or secondary tumor. As I understand it, a primary tumor originates spontaneously, i.e., it is NOT the result of a cancerous cell traveling from another zip code in the body. A secondary tumor arises as the result of a “scattered seed” from a primary tumor. During the first recurrence of the cancer seven years ago, it was my NY doctor’s opinion that that recurrence was NOT a secondary (metastatic) tumor because of the lack of local recurrence of the original tumor and the lengthy time interval between the first and second episodes. In other words, how can a tumor spread when it is no longer there? He said that whatever had caused the first tumor had also caused the second. The same logic may be applied to this third tumor. Neither tumors 1 or 2 are believed to have recurred locally so they would have had to have metastasized more than seven years ago, and if that were the case tumor 3 would have manifested years ago. Conclusion: primary tumor.
The third issue relates to the type of cardiac tumor. Primary cardiac malignancies are rare and are difficult to diagnose clinically. Following are the different types of primary cardiac tumors:
Angiosarcoma: the most common primary cardiac malignancy. Tends to occur in the right atrium. Tends to hemorrhage (no hemorrhaging noted in my echo).
Undifferentiated Sarcoma: typically occurs in the left atrium.
Rhabdomyosarcoma: most common in children.
Osteosarcoma: almost always occurs in the left atrium.
Leiomyosarcoma: favors the left atrium.
Fibrosarcoma: tends to occur in the left atrium.
Liposarcoma: extremely rare; tend to arise in one of the atria but have also been discovered in both ventricles and in the pericardium; several reports of involvement of cardiac valves.
Lymphoma: most common in immuno-compromised patients (of which thankfully I am not a member).
Conclusion: cardiac liposarcoma (based on the above, the tumor location, and my medical history).
Discussion: cardiac liposarcomas are EXTREMELY rare with only 18 such tumors having been reported in the medical literature. But everything considered, it seems to be the most likely diagnosis. To an ignorant lunatic like me, anyway. I’ll be very interested to see what the experts have to say, of course. That is, after they locate their mail.
Lymphomas are the only type of cardiac malignancy that has ever demonstrated a potential response to chemotherapy. But let’s not forget about the lowly sea squirt and its recently discovered derivative Trabectedin. You may recall that it is only effective against liposarcomas. Now it’s pretty safe to say that it has never been applied to a patient with a cardiac liposarcoma (they are apparently as hard to find as a plumber on a Sunday afternoon). But there’s always a first time for everything.
I think I’ll do some research as to where the clinical trials of Trabectedin are taking place and see if I can speak with a doctor at one of them. They’re either gonna love me or hate me. Maybe a little of both . . .
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