Primary Fibro Sarcoma of the Heart.

2013·1.5 MB·English

by  Kabashi, Serbeze

Primary Fibro Sarcoma of the Heart 213
ACTA INFORM MED. 2013 Sep; 21(3): 213-215 doi: 10.5455/aim.2013.21.213-215
Received: 05 May 2013 • Accepted: 02 July 2013
conflict of interest: none declared
© AVICENA 2013
Primary Fibro Sarcoma of the Heart
Serbeze Kabashi1,2, Naim Hoxha1, Shkelzen Gashi1, Ilir Ahmegjekaj1, Ilir Bejta1, Muharrem Sadiku1, Halit Ymeri1,2,
Antigona Kabashi1, Xhavit Bicaj1,2, Sefedin Mucaj2
Department of Radiology, Diagnostic Centre, UCCK, Pristine, Kosovo1
Faculty of Medicine, Pristine University, Pristine, Kosovo2
Corresponding author: Serbeze Kabashi, MD. Department of radiology, UCCK, Prishtine, Kosovo. E-mail: [email protected]
Case report pericardial effusion and intracavitary throm- eases. Fibrosarcomas are rare and have bad
ABSTRACT bus in atrium was diagnosed. With computed prognosis. On average patients can live around
Primary malignant heart tumors represent tomography is diagnosed a tumoral mass in six months after initial symptoms appeared
rare entities where fi bro sarcoma represents right atrium which is also spread in the right and diagnosis of the tumor was done. In the
about 3% of all. Introducing the patient: A 15 ventricle of the heart. Tumor is completely case of cardiac insuffi ciency with differen-
years old patient with cardiac insuffi ciency removed; pat histology result showed primary tial diagnosis we should also think of heart
(heart failure) symptoms, such as weakness, fi bro sarcoma of the heart. At that time no tumors, which could certainly be proved for
cyanosis, palpitations and breathing diffi cul- metastasis was found. Conclusion. Primary or eliminated by echocardiography.
ties; enlargement of upper mediastinum and malignant heart tumors may manifest like Key words: Heart, tumors, fibro sarcoma,
pleural effusion. Through echocardiography a cardiac insuffi ciency or like systemic dis- insuffi ciency, diagnosis, echocardiography.
1. INTRODUCTION 2. INTRODUCING THE CASE:
Findings about heart tumors were A 15 years old male patient, on
initially provided by Bonet in 18th the last week of September 2012, is
century (1, 2). In 1936 fi rst successful hospitalized at the Pediatrics Clinic
resection of cardiac tumor was per- at the University Clinical Centre
formed (3). Depending on their or- in Kosovo (UCCK) on the intensive
igin heart tumors are classifi ed in care unit. He was showing some non-
two main groups. First group con- specifi c symptoms and signs such as
sists of tumors that originate from weakness, fever; headache, nausea for
heart tissues and thus are known as about three weeks and gradually his
primary tumors whereas the second situation worsen.
groups consist of secondary tumors At the moment of check in the
which represent diff erent malignant patient was cyanotic and had face
tumor metastasis that originate from edema particularly in the per orbital
Figure 2. CT of thorax with i.v. contrast Axial plane
others tissues and body organs. Pri- region. With heart and lung auscul-
mary heart tumors are very rare en- tation it was found that heart beat moglobin saturation was 67%, and
tity. According to autopsy results was weakened but rhythmical while arterial pressure was 80x60mmHg.
and surgical interventions their in- breathing noise was weakened. He- Lab data: WBC - 12.6, RBC - 5.08,
cidence is around 0.001 – 0.28 (3) Hgb - 14.3 , Hct - 41.5,PLT. - 188, MCV
while heart metastasis are 10-50 times - 81.8, Gli - 4.8, ALT - 11, AST - 16,
more frequent (1, 3, 4, 6). About 75% Chol. - 2.5, CK - 40. Direct Bil. - 8.9,
of primary heart tumors are benign Total Bil. - 28.5, LDH - 507, Urea - 4.7,
whereas others are malign. (2, 3). Sar- Urine: Prot. ++, Le. - 17. In the thorax
comas make about 75% of primary radiography the PA view found
malign heart tumors (6) of which the pleural eff usion of the left side and
most common one is angiosarcoma. enlargement of upper mediastinum
Fibrosarcomas are very rare tu- mainly on the right side of v. cava su-
mors and make only about 3% of pri- perior and v. azygos region. Th e ECG
mary malignant heart tumors (4). In registered a sinusal rhythm with a
the following section we present a pa- rate about 100/min, decreased voltage
tient diagnosed with primary fi bro and AV block of grade I. With the
sarcoma of the heart. transthoracic echocardiogram was
Figure 1. CT of thorax without i.v. contrast Axial plane
ACTA INFORM MED. 2013 SEp; 21(3): 213-215 / CASE REpORT
214 Primary Fibro Sarcoma of the Heart
majority of which aft er applying the
contrast it showed a tumoral mass.
Th is mass fi lled up almost all of the
right atrium, which was widened and
pressured the left atrium (Figure 1
and 2).
We also observe a spread on the
right ventricle through ostium atrio-
ventricular and an extension up on
the superior caval vein, brachioce-
falica dex. vein and jugularis dex. Figure 8. CT of thorax with i.v. contrast (post surgery)
vein, and thus causing their dilata-
tion (Figure 3 and 4).
On the axial plane the maximum
Figure 3. CT of thorax with i.v. contrast Coronal plane
diameter of the intra-arterial septum
of this mass was 7x8.5 cm. In the
pleural space there is a massive bilat-
eral eff usion. A greater enlargement
of veins was also discovered which
anastomose two kava veins: vena
mammaria interna and vena azygos
(Figure 5 and 6). No metastases were
found. Free fl uid is observed in the
abdominal segment included in this
Figure 9. CT of thorax, abdomen and pelvic with i.v.
contrast – Coronal plane
and a half aft er the surgery another
Figure 4. CT of thorax with contrast, Coronal plane
CT of thorax, abdomen and pelvic
was conducted with an i.v. contrast
(Figure 7, 8 and 9).
A pleural eff usion was found on
the left side. Pericardial eff usion was
also found. No signs of secondary
mass were identifi ed.
3. DISCUSSION
Figure 6. CT of thorax with i.v. contrast. Sagittal plane Heart tumors represent a big chal-
lenge when diagnosing them. Th eir
clinical symptoms are nonspecifi c,
and depend more on the size and
location of tumor than on its his-
tologic type. (6) Th ey are caused by
four main mechanisms: intracavitary
Figure 5. CT of thorax with i.v. contrast. Coronal plane
enlargement of tumor can cause ob-
found pericardial eff usion, stasis on struction of blood circulation or mal-
systemic veins and decrease of circu- function of heart valves; local inva-
lation on pulmonary artery. It is also sion of tumor can cause arrhythmia
found a hyperechogenic mass that and pericardial eff usion; a part of
nearly covers the right atrium and Figure 7. CT of thorax with i.v. contrast (post surgery) tumor can embolise and cause cere-
spreads towards the right ventricle, brovascular and peripheral vascular
which was thought it represented a examination. accidents. Also, when tumor releases
massive intracavitary thrombus. Th e Th e patient was referred to a spe- cytokines (IL-6) it can cause systemic
patient was referred to the Radiology cialized Cardio-surgical centre in Ti- or constitutional symptoms of weight
Clinic; a computed tomography of rana where he underwent a surgical loss, fever, arthralgia, myalgia, hemo-
thorax with and without i.v. contrast intervention. Th e histopathological lytic anemia, thrombocytopenia, leu-
was conducted. In this case a hyper- analysis proved that this was a case kocytosis, elevation of CRP, faster SE
density mass was observed and the with fi brosarcoma tumor. A month (2, 6).
CASE REpORT / ACTA INFORM MED. 2013 SEp; 21(3): 213-215
Primary Fibro Sarcoma of the Heart 215
Primary heart sarcomas are found Surgical intervention and com- 646.
with people of all group ages, but plete tumor resection is main treat- 5. Becker AE. Primary heart tumors in
more often in the 3-5 decade of their ment. In case of malignant tumors the pediatric age group: a revew of
life with no observable gender differ- chemotherapy and radiotherapy are salient pathologic features relevant
ences (4). Our patient is 15 years old applied too. for clinicians. Pediatr Cardiol. 2000
male. Primary heart sarcomas are Primary malignant heart tumors Jul-Aug; 21(4): 317-323.
usually found on the right atrium, which often attack young people 6. Leja MJ, Shah DJ, Reardon MJ. Pri-
and frequently are angiosarcomas (6). have very bad prognosis: without a mary Cardiac Tumors. Tex Heart
Sarcomas found on the right side surgical intervention, survival be- Inst J. 2011; 38(3): 261-262.
of heart usually infiltrate more, grow tween 9 to 12 months is 10% (6). 7. Vaporciyan A, Reardon MJ. Right
in the shape of cauliflower, and me- heart sarcomas. Methodist Debakey
4. CONCLUSIONS
tastasize earlier compared to sar- Cardiovasc J. 2010 Jul-Sep; 6(3): 44-
comas located on left side of heart (6, Heart tumors are rare; fibro sar- 48.
7). In our case we are dealing with a coma makes about 3% of all malig- 8. Blackmon SH, Patel A, Reardon MJ.
massive tumor located on the right nant heart tumors. Their diagnosis is Management of primary cardiac sar-
atrium that spreads on the right ven- very difficult not only because of their comas. Expert Rev Cardiovasc Ther.
tricle towards vena cava superior, but rareness but also because of their 2008 Oct; 6(9): 1217-1222.
no metastasis where found at the mo- nonspecific clinical symptoms. In 9. Knobel B, Rosman P, Kishon Y, Hu-
ment of diagnosis. Also, sarcoma lo- cases of acute heart insufficiency, in a sar M. Intracardiac primary fibrosar-
cated on right side of the heart rarely differential diagnosis, we should sus- coma. Case report and literature re-
causes congestive heart insufficiency pect a heart tumor whereby every pa- view. Thorac Cardiovasc Surg. 1992
or disorderly hemodynamic status tient should be checked with echog- Aug; 40(4): 227-230.
compared to left side sarcomas, which raphy. Heart sarcomas have bad prog- 10. Narasimhan S. Unexplained Con-
are very symptomatic at the moment nosis; about a year after the diagnosis stitutional Symptoms, Weight Loss
of presentation (6, 7). In our case we was made. Surgical excision, even if and the Heart. Journal of Medical
have symptoms of heavy right side partial, is indicated for the purpose Sciencies Research, November 15,
heart insufficiency NYHA III. of decreasing symptoms of obstruc- 2007; 2.
Through thorax radiography we tion. In some cases of non-surgical 11. Devbhandari MP, Meraj S, Jones
can get nonspecific data. In our case sarcomas good results are achieved MT, Kadir I, Bridgewater B. Primary
we represented a widening of upper by heart transplanting. cardiac sarcoma: reports of two cas-
mediastinum and pleural effusion. es and a review of current literature.
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ACTA INFORM MED. 2013 SEp; 21(3): 213-215 / CASE REpORT