PEDIATRIC CASE

STUDY
PERICARDIAL SYNOVIAL SARCOMA

Elizabeth Muha

PATIENT HISTORY
19 year old male
MR#: 980294178
The James Vault 4

DIAGNOSIS
Pericardial synovial cell sarcoma
Pathology from showed a malignant small round blue cell

tumor, favoring a poorly-differentiated synovial sarcoma.

Used and echocardiogram, MRI, CT and PET scan.

PAST MEDICAL HISTORY

Bilateral Iliac artery occlusion
Left ventricular mass
Aortic saddle embolism
Cardiac Synovial sarcoma (4/2014)
Anemia (6/23/2014)
Left Ventricular cardiac mass
Hypokalemia (12/15/14)

PAST SURGICAL HISTORY

Bilateral Embolectomy of the common femoral iliac

(4/29/14)
Right Fasciotomy (4/29/14)
Right Myocardial resection (4/29/14)
closure wound dehiscence (5/2/14)
Right embolectomy/thrombectomy leg artery by incision

(6/4/14)
Tunneled venous port placement (6/2014)
Midline resection myocardial (1/2/15)
Midline redo sternotomy (1/2/15)

SOCIAL HISTORY
Single
Never smoked or used smokeless tobacco
Not sexually active
Never drank alcohol
Allergic to Gadolinium and Vancomycin hcl

FAMILY HISTORY
Paternal Aunt: Cancer- doesnt specify
Paternal Uncle: Cancer- doesnt specify
Paternal Grandmother- Breast Cancer

PRESENTING SIGNS AND

SYMPTOMS
Presented to an outside hospital with coolness and

discoloration of his right leg lasting more than 24 hours

He reports having chest pains about 2 months ago

associated with numbness and tingling down his left arm.

(this lasted several days then went away)

COMMON PRESENTING
SIGNS OF CARDIAC
SYNOVIAL SARCOMA
Cough
Chest pain
Dyspnea
Fever
Fatigue
*It is easy for diagnosis to be delayed

due to the nonspecific clinical

symptoms.

EPIDEMIOLOGY
Synovial sarcoma is a rare entity and accounts for <1% of

all primary cardiac tumors and 5% of malignant tumors of

the heart
As of June 2009, only 35 cases had been reported in

medical literature.
Synovial sarcomas predominantly occur in para-articular

soft tissues of the extremities of young adults and

adolescents with a mean age of 34.8 years (range 1367).
Right side predominant
The male to female ratio is 2.5:1

ETIOLOGY
The hallmark of diagnosis of synovial sarcomas is the detection

of the translocated chromosome t(X; 18) (p11.2; q11.2), which

is present in more than 90% of the synovial sarcomas

SSX1-SYT
SSX2-SYT
SSX4-SYT

This swapping creates new chromosomes consisting of joined

portions of the two original chromosomes. The mechanism

underlying these events is not understood but is hypothesized
to be the result of either damage to chromosomal DNA (e.g.,
by radiation or chemicals) or mistakes in normal recombination
processes.

ANATOMY
There are 4 chambers
of the heart
This pateints tumor
was located in the Left
Ventricle.

SPREAD
With his diagnosis the right superior pulmonary vein is

what is most commonly checked for spread.

An excision was performed that came back negative for malignancy.

No lymphatic spread

HISTOPATHOLOGY
Histologically, synovial sarcomas includes two different

cell types: spindle cells and epithelial cells. It can be

classified into four subtypes depending on the relative
proportion of its two cell types: (1) biphasic type; (2)
monophasic fibrous (spindle cell) type; (3) monophasic
epithelial type; and (4) poorly differentiated type
Patients with the poorly differentiated subtype are

considered to have a worse prognosis than those with

other subtypes
Patients with metastases that cannot be removed by

resection have a poorer prognosis.

STAGING
This is staging
for soft tissue
sarcomasthere
is no staging for
synovial
sarcomas
because they are
so rare.

TREATMENT OPTIONS
Treatment is usually palliative*
Usually a resection of affected part of heart followed by

chemotherapy with or without adjuvant radiation.

When using chemo they weigh the positives and

negatives.

TREATMENT PLAN/PERSCRIPTION

TREATMENT PLAN (CONTD)

Received 6 cycles of concurrent chemo

Chemotherapy treatment history:

High dose AIM

--06/05/2014 C1D1 doxorubicin 75 mg/m2 + ifosfamide 2 g/m2/d x 5 days (10 g/m2)

--06/27/2014 C2D1 doxorubicin 75 mg/m2 + ifosfamide 2 g/m2/d x 5 days (10 g/m2)

HD-ifosfamide, course 1

--07/18/2014 C1D1 ifosfamide 2 g/m2/d x 5 days (10 g/m2)

--08/15/2014 C2D1 ifosfamide 2 g/m2/d x 5 days (10 g/m2)

--09/05/2014 C3D1 ifosfamide 2 g/m2/d x 5 days (10 g/m2)--1 week delay secondary to
neutropenia

--9/19/2014 C4D1 ifosfamide 2 g/m2/d x 5 days (10 g/m2)

HD-ifosfamide, course 2

--11/10/2014 C1D1 ifosfamide 2.8 g/m2/d x 5 days (14 g/m2)

--12/03/2014 C1D1 ifosfamide 2.8 g/m2/d x 5 days (14 g/m2) [plannned] stopped after 12/6
**He was transitioned to HD-ifosfamide due to
concerns about potential cardiotoxicity of doxorubicinshould he require mediastinal radiation.

SET UP FIELDS

TREATMENT BORDERS

CRITICAL STRUCTURES

DVH

AXIAL

CORONAL

SAGITTAL

DRRS/ OTHER IMAGES

PATIENT POSITIONING

3-point chest set-up

Supine
Arms overheard with vac bag in
arm shuttle
Knee sponge
*Imaged first 3 days and then 3X a
week to follow- lining up to the
anterior chest wall and heart.*

SIDE EFFECTS

bleeding

shortness of breath

pneumonia

heart infections

blood clots

death

scarring of the heart

heart attack

heart failure

cardiac arrhythmias

secondary malignancy

fatigue

skin reaction

lung scarring

spinal cord injury

cough

PROGNOSIS AND SURVIVAL RATE

These tumors proliferate rapidly and often cause death

through widespread infiltration of the myocardium or

obstruction of flow within the heart. Complete resection is
seldom possible and if so, frequently followed by
recurrence within two years. Adjuvant chemo radiation is
known to be of value to reduce recurrence but survival of
more than 12-24 months is rare.

REFERENCES
1. Talukder M, Joyce L, Marks R, Kaplan K, Primary cardiac synovial sarcoma.
Cardiovascular And Thoracic Surgery- Oxford Journals. (2010) 11 (4): 490-492.
doi: 10.1510/icvts.2010.240200

2. Wang JG, Li NN. Primary cardiac synovial sarcoma. The Annals of Thoracic
Surgery. 2013. http://dx.doi.org/10.1016/j.athoracsur.2013.01.030

3.Mercado G, Barr F. Chromosomal translocations in sarcomas: new

perspectives. The Liddy Shriver Sarcoma Initiative. (2006) 3(2):113-7

4. IHIS/Aria

5. Zheng L, Qian J, Zhongdong L, Jing H. Primary synovial sarcoma of the heart.

Cardiology Journal. Cardiology Journal 2011, Vol. 18, No. 2, pp. 128133
Copyright 2011 Via Medica ISSN 18975593