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Re: Malignant fibrous histiocytoma

Posted By Choy Tak Kee Dicky on April 14, 1999 at 11:57:01
I have a relative in HK which confirm to have malignat fibrous histiocytoma of left atrium after the pathology report. The tumour recurs very quickly after surgery.
I want to ask how many cases have been reported so far (malignant fibrous histiocytoma of the heart), and how can I find those informations reqaurding the treatment of this tumours.


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Avatar universal
Posted By CCF CARDIO MD - MTR on April 15, 1999 at 10:22:20
Dear Choy Tak, thank you for your question.  I have done an extensive review of the available medical literature on this subject for you and I've included a few references with abstracts with my response.  The first step would be to go to your nearest medical library to get copies of these articles.  This is a rare condition that unfortunately carries a poor prognosis.  I can't determine how many cases have been reported in the medical literature, however.  
I hope you find this information useful.  Information provided in the heart forum is for general purposes only.  Only your physician can provide specific diagnoses and therapies. Please feel free to write back with additional questions.  Good luck.
If you would like to make an appointment at the Cleveland Clinic Heart Center, please call 1-800-CCF-CARE or inquire online by using the Heart Center website at www.ccf.org/heartcenter.   The Heart Center website contains a directory of the cardiology staff that can be used to select the physician best suited to address your cardiac problem.

Malignant fibrous histiocytoma of the heart--case report of a
rare left-atrial tumor.
Thorac Cardiovasc Surg 1992 Oct;40(5):303-7   (ISSN: 0172-6137)
Korbmacher B; Doering C; Schulte HD; Hort W [Find other articles with these Authors]
Department of Thoracic and Cardiovascular Surgery, Heinrich-Heine-University, Duesseldorf,
The present report describes the case of a 57-year-old patient with malignant fibrous histiocytoma
of the left atrium operated on for a suspected atrial myxoma. Surgery was performed as radically as
possible and included resection of the atrial wall. Clinical investigation and echocardiography performed
29 months after surgery revealed no signs of tumor recurrence. Diagnostic procedures, topography,
pathohistology as well as options for therapy are discussed with regard to the latest literature. A radical
surgical approach represents the only effective therapeutic measure. Owing to a lack of sufficient
experience, there is no additional therapeutic recommendation on hand. Neither radiation nor
chemotherapeutic treatment concepts have proved efficient. Echocardiography represents the best
examination procedure for both diagnosis and follow-up in patients with cardiac tumor.

Malignant fibrous histiocytoma of the heart--a case report
and review of the literature]
Nippon Kyobu Geka Gakkai Zasshi 1991 Dec;39(12):2240-5   (ISSN: 0369-4739)
Sasaki K; Tanaka S; Ikeshita M; Shoji T; Fujita Y; Katagiri K [Find other articles with these Authors]
Department of Thoracic Surgery, Nihon Medical School, Tokyo, Japan.
Malignant fibrous histiocytoma (MFH) has been rarely reported as a primary tumor of the heart.
We present a case of a primary intracardiac MFH and review the 28 previous reports of this rare
cardiac tumor. A 48-year-old woman underwent an operation for congestive heart failure (CHF) due
to a left atrial tumor. A large multinodular tumor attached to the posterior wall of the left atrium and
extended into the mitral annulus and the posterior leaflet of the mitral valve. The tumor was resected
incompletely and mitral valve replacement was performed. Pathological diagnosis was MFH. The
patient did well in the postoperative period, but she again presented with symptoms of CHF four
months later. Chest CT and two-dimensional echocardiogram demonstrated a recurrent tumor of the
left atrium. Since the tumor grew rapidly despite chemotherapy, a reoperation was performed to
remove it. Nevertheless, as the tumor was found to have extended into the pericardial cavity and
posterior mediastinum, only partial resection was possible. After a protracted operation, the heart
failed to sustain the circulation and the patient died. The prognosis of intracardiac MFH was poor in
spite of surgery, radiation and/or chemotherapy. However, repeated surgical treatments can relieve
symptoms of CHF and may prolong the life of patients.

An autopsy report of malignant fibrous histiocytoma of the
left atrium and a review of the pertinent literature]
Gan No Rinsho 1990 Feb;36(2):169-75   (ISSN: 0021-4949)
Suzuki Y; Ikeda H; Kanemaki K; Seki T; Kondo M; Hara K; Itoh G; Odaka M [Find other articles
with these Authors]
1st Dept. of Pathology, Aichi Medical Univ.
A 30-year-old male complaining of fever was admitted to hospital and a diagnosis of a malignant,
fibrous histiocytoma (MFH) was established after a biopsy examination. Antitumor chemotherapy
and Co60 irradiation was initiated. The patient, however, suddenly died of cardiac arrest 9 months after
admission. The autopsy revealed a polypoid, yellow-white tumor (5 cm in diameter) arising from the
antero-lateral wall of the left atrium and occupying the chamber. Twenty-two cases of MFH (21
previous reports and 1 current case) arising from the heart are reviewed.

Primary malignant fibrous histiocytoma of the left atrium.
Surgical and chemotherapeutic management.
Br Heart J 1984 Sep;52(3):354-7   (ISSN: 0007-0769)
Eckstein R; Gossner W; Rienmuller R [Find other articles with these Authors]
A primary malignant fibrous histiocytoma of the left atrium was diagnosed in a 27 year old woman.
After surgical excision the tumour recurred together with enlargement of the right hilar lymph nodes. The
patient was then treated with nine courses of chemotherapy using a combined drug regimen. During the
first course the tumour regressed, and after nine courses almost complete remission was achieved.
Subsequently, the residual tumour was removed by resection of the right lung, the right hilar,
paratracheal, and paraeosophageal lymph nodes and by cardiotomy with partial resection of the right
and left atria and atrial septum followed by a reconstruction of the atrias. To date, more than two years
after initial presentation, the patient is alive and well.
Treatment of malignant fibrous histiocytoma of the heart.
Cancer 1992 Feb 15;69(4):956-61   (ISSN: 0008-543X)
Stevens CW; Sears-Rogan P; Bitterman P; Torrisi J [Find other articles with these Authors]
Department of Radiation Medicine, Georgetown University School of Medicine, Washington, D.C.
Primary soft tissue sarcoma of the heart is encountered infrequently in clinical practice. Treatment is
reported of an intracardiac malignant fibrous histiocytoma, consisting of maximal surgical resection
followed by 5600 cGy of conventionally fractionated radiation therapy. Transesophageal
echocardiograms done during follow-up were useful in assessing tumor control. Aspects of patient care
are discussed in conjunction with a review of the available literature.

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Avatar universal
Posted By Wayne Joyner on July 25, 1999 at 15:47:03
My wife was diagnosed with MFH in October 20, 97.  A small area on the inner right theigh grew to 12.5 lbs (US) between Octover 97 and January 98.  Immediate chemotherapy treatment was begun for two months (November and December). The hope was to at least cause the tumor to solidify to make surgery a little easier. In January the tumor was removed with wide margins. (The leg did not require amputation.  Shortly after surgery she was subjected to another round of chemotherapy for a period of 5 months (one 3 day session each month).  Many of these sessions resulted in other complications (Celulitis,  Anemia,  Pancretitis among other things.)  She has never successfully had just a three day visit in the hospital for any chemotheraphy treatment. In November -December 24, 1998 she underwent treatment of CO60(?) radiation to the affected area.  In May 1999 it was revealed in a Chest X-ray that metastis was occuring in the right lung.  Additional testing also revealed a larger 5 cm tumor in the tissue of the vagina lip and adjacent tissue between the vagina and the stomach cavity. A operation resection was performed of the 4 cm tumor with wide margin of the lung tissue in June 21, 1999. July 5th 1999, a chemotherapy session was begun.  We anticipate and have scheduled another chemotherapy session for August 2, 1999. We have been told that the tumor has now grown to be 5cm x 11 cm and growing at an alarming rate.  We were told on Friday July 23, 1999 that surgery of the effected area was probably not a good option health wise for her.
Is there anything we can do?  This thing is eating her alive and she is taking a lot of pain medications.

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