DOI:10.2214/AJR.05.0046
AJR 2006; 187:645-648
© American Roentgen Ray Society
Recurrent Malignant Fibrous Histiocytoma of the Right Atrium with Extracardiac Extension
Dong Hun Kim1,2,
Sang Hyun Paik3,
Jai Soung Park3,
Jung Hwa Hwang1,
Gye Won Kwon4 and
Eun Suk Koh4
1 Department of Radiology, Soonchunhyang University Hospital, Yongsan-gu, Seoul,
140-743, Korea.
2 Present address: Department of Radiology, Chosun University, College of
Medicine, Dong-gu, Gwangju, 501-717, Korea.
3 Department of Radiology, Soonchunhyang University Hospital Bucheon, 1174
Jung-dong, Wonmi-gu, Bucheon, Gyunggi, 420-767, Korea.
4 Department of Pathology, Soonchunhyang University Hospital Bucheon, Bucheon,
Gyunggi, 420-767, Korea.
Received January 13, 2005;
accepted after revision April 6, 2005.
Address correspondence to J. S. Park
(jspark{at}schbc.ac.kr).
Keywords: cancer • cardiac imaging • cardiac tumor • heart • malignant fibrous histiocytoma • right atrial tumor
Introduction
A primary cardiac malignant fibrous histiocytoma (MFH) of the right atrium
was diagnosed in a 34-year-old woman admitted to our hospital with dyspnea.
Pathohistological examination confirmed MFH. The tumor recurred rapidly, and a
second operation was not performed. A CT chest scan revealed a large mass in
the right atrium with extracardiac extension. The patient was treated with
chemotherapy using a combined drug regimen. Despite hemodynamic impairment and
extracardiac extension, adjuvant chemotherapy promoted partial remission. We
report a case of recurrent primary cardiac MFH with extracardiac extension and
successful chemotherapeutic management.
Primary cardiac tumors are uncommon and most are benign. Malignant tumors
account for only 25% of all primary cardiac tumors
[1,
2]. Most malignant cardiac
tumors are angiosarcomas. Malignant fibrous histiocytomas are rare primary
cardiac tumors
[3-6].
We report on a case of recurrent primary cardiac MFH with extracardiac
extension and successful chemotherapeutic management.
Case Report
A 34-year-old woman was admitted to the hospital with a 4-day-long history
of dyspnea. On admission, she presented with vague discomfort of the anterior
chest and exertional dyspnea (class II or III according to the functional
classification of the New York Heart Association). Physical examination
revealed: blood pressure, 100/80 mm Hg; pulse, 78 beats/min; respiration, 34
breaths/min; and body temperature, 36.2°C. Lung and cardiac sounds were
normal. Laboratory data on admission, including blood chemistry, coagulation
studies, complete blood count, and cardiac enzymes, revealed no abnormalities.
Chest radiographs at admission revealed the presence of cardiomegaly without
mediastinal widening. The ECG disclosed sinus rhythm of 70 beats/min with no
ST-T wave changes. The ECG revealed an enlarged left atrium that was filled
partially with an echogenic mass. A CT chest scan showed a 5.3 x 4.0
x 3.5-cm left atrial mass, another 2.0 x 1.5 x 1.0-cm
subvalvular (below tricuspid valve) mass, and nodular thickening of the atrial
septum (Figs. 1A and
1B). After a preliminary
diagnosis of left atrial myxoma, the patient underwent open-heart surgery. An
oval soft mass (7.0 x 6.0 x 4.5 cm) was enucleated from the left
atrium in the region of the right superior and inferior pulmonary vein. The
mitral valve was normal. Another oval mass (2.7 x 2.2 x 2.0 cm)
was attached to the inferior surface of the tricuspid septal leaflet. The
tumors and thickened interatrial septum were resected. Although the appearance
of the tumors resembled atrial myxoma, the final pathologic diagnosis of the
tumors and resected interatrial septum was MFH. The specimen had a storiform
appearance, pleomorphism, myxoid degeneration, bone formation, and frequent
mitosis. Tumor cells were oval and fusiform and had indistinct cell borders
and abundant cytoplasm (Fig.
1C). Immunohistochemical staining of the tumor showed reactivity
to antibodies raised against smooth muscle vimentin and actin but not desmin
or cytokeratin. The postoperative course was uneventful, and during that time
an extensive search for metastasis was performed. Four months after the
initial operation, the patient complained of abdominal distention. An
abdominopelvic CT scan revealed a 6.5 x 5.0 x 4.2-cm right atrial
tumor attached to the posterior wall and extending into the inferior vena cava
(IVC), as well as extensive ascites (Fig.
1D). The patient underwent three consecutive cycles of a combined
regimen of cyclophosphamide, vincristine, Adriamycin, and dacarbazine
(CYVADIC) [7]. She exhibited
persistent dyspnea. No metastases were detected elsewhere after a thorough
survey. A cardiac CT scan was performed (SOMATOM Sensation, Siemens Medical
Solutions) using the following parameters: collimation, 16 x 0.75 mm;
pitch, 0.28; and 120 mL of contrast agent injected at 4 mL/s. The scan
revealed a decrease in the size of the soft-tissue mass to a diameter of 4 cm
at the former site of the tumor (localized to the posterior right atrial
wall). There was extracardiac involvement of the inferior vena cava (IVC)
(Figs. 1E and
1F).
View larger version (89K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1A —34-year-old woman with recurrent primary cardiac malignant
fibrous histiocytoma (MFH) with extracardiac extension. Unenhanced (A)
and contrast-enhanced (B) CT chest scans at first admission. Note left
atrial tumor (asterisk), subvalvular tumor (arrowheads), and
nodular atrial septal thickening are revealed on unenhanced image (A),
but delayed contrast-enhanced scan (B) does not show definite
enhancement.
|
|
View larger version (93K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1B —34-year-old woman with recurrent primary cardiac malignant
fibrous histiocytoma (MFH) with extracardiac extension. Unenhanced (A)
and contrast-enhanced (B) CT chest scans at first admission. Note left
atrial tumor (asterisk), subvalvular tumor (arrowheads), and
nodular atrial septal thickening are revealed on unenhanced image (A),
but delayed contrast-enhanced scan (B) does not show definite
enhancement.
|
|
View larger version (153K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1C —34-year-old woman with recurrent primary cardiac malignant
fibrous histiocytoma (MFH) with extracardiac extension. Histologic appearance
of MFH shows mixture of spindle-shaped fibroblasts arranged in a storiform
pattern, polygonal pleomorphic histiocytes, and malignant giant cells. Mitotic
activity was prominent. Tissue in image was counterstained with H and E,
x400.
|
|
View larger version (105K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1D —34-year-old woman with recurrent primary cardiac malignant
fibrous histiocytoma (MFH) with extracardiac extension. CT image obtained 4
months after resection revealed recurrent MFH within right atrium with
extracardiac extension into inferior vena cava (IVC).
|
|
View larger version (132K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1E —34-year-old woman with recurrent primary cardiac malignant
fibrous histiocytoma (MFH) with extracardiac extension. Axial (E) and
coronal (F) CT scans after chemotherapy revealed decreased size of
soft-tissue mass, which was localized to posterior right atrial wall with
extension into IVC.
|
|
View larger version (152K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1F —34-year-old woman with recurrent primary cardiac malignant
fibrous histiocytoma (MFH) with extracardiac extension. Axial (E) and
coronal (F) CT scans after chemotherapy revealed decreased size of
soft-tissue mass, which was localized to posterior right atrial wall with
extension into IVC.
|
|
Discussion
Malignant cardiac tumors comprise 25% of primary tumors of the heart. In
order of frequency, cardiac tumors include angiosarcomas, rhabdomyosarcomas,
and fibrosarcomas [1].
Angiosarcomas arise almost exclusively within the right atrium, whereas the
other types of cardiac tumor occur with equal frequency in all chambers. Among
malignant cardiac tumors, MFH of the heart is rare and occurs within the left
atrium in almost all reported cases
[5,
6]. An important feature of MFH
of the heart is a similarity to benign atrial myxoma: not only do both types
of tumor tend to arise within the left atrium (and thus have similar clinical
presentations), but also, as noted above, benign atrial myxoma and the myxoid
variant of MFH can be quite difficult to distinguish histologically. The
differentiation of these types of tumor has important prognostic implications:
MFH of the heart is uniformly fatal, whereas benign atrial myxoma can be cured
by surgery in most cases [1,
5].
MFH is thought to arise from primitive mesenchymal cells and is classically
composed of spindle cells (fibroblasts) and round (histiocytic) cells arranged
in a storiform pattern, accompanied by pleomorphic giant cells and
inflammatory cells. Pathologic diagnosis relies on several criteria, which
have already been defined in the literature and include the presence of
typical spindle and polygonal (straplike) cells that are filled with an
abundant eosinophilic cytoplasm, cells with cross-striations, and (in
particular) desmin- and myoglobin-positive immunoreactivity
[7]. However, some of these
criteria remain controversial in that some pathologists consider MFH as a
subtype of undifferentiated pleomorphic sarcoma. Particularly, the presence of
cytoplasmic filaments is not always found in MFH, making it harder to
establish a reliable differential diagnosis. The subcategories of MFH are
storiform/pleomorphic, myxoid, giant cell type, inflammatory type, and
angiomatoid type. The myxoid type, of MFH is associated with a good
prognosis.
MFH is not associated with any early clinical manifestations. Furthermore,
there is no association with a serologic increase in specific neoplastic
cellular markers. The localization of MFH to the heart results in impaired
cardiac output, which leads to symptoms such as dyspnea, chest pain,
congestive heart failure, and arrhythmias that inevitably require surgical
resection [1]. At this stage,
invasive procedures usually reveal a large mass or diffuse metastasization
with extensive myocardial infiltration, which is the main cause of therapeutic
failure. Nevertheless, accurate resection of the tumor is required to
attenuate symptoms and to improve postoperative survival. Malignant fibrous
histiocytoma is highly aggressive, and the average survival time is fewer than
12 months [8]. Despite the
availability of valid chemotherapeutic regimens, the prognosis remains poor
[9]. Cardiac transplantation is
one radical treatment that could be considered, especially for young patients.
However, it is still questionable whether transplantation under
immunosuppressive therapy would be truly effective for a malignant disease,
and an interesting report showed a recurrence of MFH after orthotopic heart
transplantation to treat an MFH of the right ventricle
[7].
Various imaging techniques, including CT, MRI, ECG, and angiography provide
important information about the extent of cardiac neoplasm, which aids in
planning resection of the tumor. Also, strict follow-up imaging is mandatory
because this type of tumor has a high rate of recurrence and metastasization
to the lung, brain, and adrenal glands. The tumor grade and margin status are
correlated with overall survival. The survival rate is good if MFH can be
entirely resected. The 5- and 10-year overall survival rates were 100% and
81%, respectively, for patients with grade 1 tumors and 58% and 50%,
respectively, for those with grade 3 tumors
[8].
Recurrence of MFH after local excision is not rare. The response of
soft-tissue sarcomas to radiation is variable, and radiation treatment is
therefore not always successful. Chemotherapy is generally accepted to be the
most successful treatment for advanced tissue sarcomas. Of the different
regimens, the combined CYVADIC regimen (which we used in our case) is
appreciably superior, in terms of both response and survival. Eckstein et al.
[9] recommended that every
surgical excision of cardiac soft-tissue sarcomas be followed immediately by
adjuvant chemotherapy. Remission after chemotherapy was only partial in our
patient; therefore, a further operation and radical resection of the residual
tumor was needed (as in the case reported by Eckstein et al., which had a
successful outcome).
Differences in the characteristics of MFH of the heart and MFH of soft
tissue are also noteworthy. The disease has been uniformly fatal, despite
various combinations of chemotherapy, radiation, and surgery. Malignant
fibrous histiocytoma of the soft tissue typically presents in the sixth and
seventh decades and is infrequent in patients younger than 40 years old.
Malignant fibrous histiocytoma of the heart has a tendency to occur in a
younger population; moreover, the male predominance of the soft-tissue form of
MFH appears to be reversed in MFH of the heart. Confirmation of these
observations must await further analysis of MFH of the heart in a large series
of cardiovascular malignancies.
The case reported here showed a highly unusual finding of recurrent MFH of
the right atrium with extracardiac extension. The patient was initially
suspected of having left atrial myxoma, and resection was performed. However,
the prognosis was negative, and recurrent dyspnea and numerous ascites led to
a poor outcome within 6 months of surgical removal of the tumor. Successful
treatment of a cardiac tumor is less frequent when the tumor involves the
right or left ventricle, particularly if the tumor is malignant. In our
patient, management with a chemotherapy regimen resulted in partial remission.
Our report shows that even in the presence of a recurrent primary cardiac
malignancy with extracardiac extension, and despite severe hemodynamic
impairment, effective adjuvant chemotherapy can allow a complete or partial
remission.
References
- Putnam JB, Sweeney MS, Colon R, Lanza LA, Frazier OH, Cooley DA.
Primary cardiac sarcomas. Ann Thorac Surg1991; 51:906
-910[Abstract]
- Perchinsky MJ, Lichtenstein SV, Tyers GFO. Primary cardiac tumors.
Cancer 1997; 79:1809
-1815[CrossRef][Medline]
- Toda R, Yotsumoto G, Masuda H, Sakata R, Umekita Y. Surgical
treatment of malignant fibrous histiocytoma in the left atrium and pulmonary
veins: report of a case. Surg Today 2002;32
: 270-273[CrossRef][Medline]
- Murdoch MJ, Nadel HR, Culham JA, Stringer DA. Pediatric case of the
day: malignant fibrous histiocytoma (MFH) of the left atrium.
RadioGraphics 1995;15
: 1227-1230[Medline]
- Laya MB, Mailliard JA, Bewtra C, Levin HS. Malignant fibrous
histiocytoma of the heart: a case report and review of the literature.
Cancer 1987; 59:1026
-1031[CrossRef][Medline]
- Okita Y, Miki S, Ueda Y, Tahata T, Sakai T, Matsuyama K. Recurrent
malignant fibrous histiocytoma of the left atrium with extracardiac extension.
Am Heart J 1994;127
: 1624-1628[CrossRef][Medline]
- Akhter SA, McGinty J, Konys JJ, Giesting RM, Merrill WH, Wagoner
LE. Recurrent primary cardiac malignant fibrous histiocytoma following
orthotopic heart transplantation. J Heart Lung
Transplant 2004; 23:1447
-1450[CrossRef][Medline]
- Murphy MC, Sweeney MS, Putnam JB Jr., et al. Surgical treatment of
cardiac tumors: a 25-year experience. Ann Thorac Surg1990; 49:612
-617[Abstract]
- Eckstein R, Gossner W, Rienmuller R. Primary malignant fibrous
histiocytoma of the left atrium: surgical and chemotherapeutic management.
Br Heart J 1984;52
: 354-357[Abstract/Free Full Text]
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
Top
Introduction
Case Report
