46 Ann Thorac Cardiovasc Surg Vol. 15, No. 1 (2009)

CaseReport

Aggressive Repeat Surgery for a Recurrent Synovial Sarcoma in the Pleura

Noritoshi Nishiyama, MD, PhD,1 Takashi Iwata, MD, PhD,1 Nobuhiro Izumi, MD, PhD,1

Takuma Tsukioka, MD, PhD,1 Koshi Nagano, MD,1 and Shigefumi Suehiro, MD, PhD2

From Departments of 1Thoracic Surgery and 2Cardiovascular Surgery, Osaka City University, Osaka, Japan

Received October 3, 2007; accepted for publication January 21, 2008Address reprint requests to Noritoshi Nishiyama, MD, PhD: Department of Thoracic Surgery, Osaka City University, 1–4–3 Asahi-machi, Abeno-ku, Osaka 545–8585, Japan.©2009 The Editorial Committee of Annals of Thoracic and Cardiovascular Surgery. All rights reserved.

Pleural synovial sarcomas are an extremely rare disease entity, and only 21 surgical cases, including this one, have been reported. We present a case of synovial sarcoma that under-went pneumonectomy for a recurrence of the disease. A 31-year-old female, who in June 2005 had undergone surgical resection of a monophasic synovial sarcoma in the left pleural cavity combined with the affected left diaphragm, was admitted to our hospital in January 2007 and underwent chemotherapy with ifosfamide, pirarubicin, and methotrexate for a recurrent synovial sarcoma in the left pleural cavity. The chest computed tomography revealed multiple tumors in the left pleura, and the tumors increased rapidly to occupy the left hemithorax. She began to complain of a severe dry cough. No other recurrent sites were found, and she underwent tumor resection combined with the entire left lung in March 2007. The tumor remained around the descending aorta. Pathological examination revealed dense proliferation of atypical spindle cells, the same findings as in the initial operation, and a diagnosis of synovial sarcoma was made. External radiation therapy up to 60 Gray was given to the tumor remaining around the descending aorta. She is now ambulatory without evidence of recurrent disease at 6 months after the second operation and 27 months after the initial surgery. Any aggressive repeat surgery as in the case here has not been previously reported. The present case suggests that aggressive tumor reduction followed by radiotherapy can achieve a good result for controlling local disease. Although optimal strategies for syn-ovial sarcoma have yet to be established, aggressive repeat surgery could be considered in selected cases even when response to chemotherapy is insufficient and complete resection is difficult. (Ann Thorac Cardiovasc Surg 2009; 15: 46–49)

Key words: synovial sarcoma, pleural cavity, surgery

Introduction

Synovial sarcomas usually arise in the extremities and less frequently found in various anatomic locations.

Synovial sarcoma in the pleural cavity is extremely rare, though we previously reported a case of a resected synovial sarcoma in the left pleural cavity.1) Here we present a subsequent report of this patient who under-went pneumonectomy for a recurrence of the disease.

Case

A 31-year-old female was admitted to our hospital in January 2007 for chemotherapy for a recurrent synovial sarcoma in the left pleural cavity. She had originally been a patient in our hospital from February 2005 through December 2005. At that time she initially com-

Aggressive Repeat Surgery for a Recurrent Synovial Sarcoma in the Pleura

Ann Thorac Cardiovasc Surg Vol. 15, No. 1 (2009) 47

plained of a severe dry cough; during admission, a chest computed tomographic (CT) scan revealed a mass mea-suring 10 cm in diameter in the lower half of the left pleural cavity with massive pleural effusion. A percuta-neous needle biopsy revealed spindle-shaped malignant cells. An immunohistochemical examination showed a positive staining for vimentin, but negative for thrombo-modulin and calretinin. Reverse transcription-polymerase chain reaction using ribonucleic samples from frozen tumor tissue revealed the presence of SYT-SSX fusion gene transcripts; therefore she was diagnosed with a synovial sarcoma in the left pleural cavity. She under-went three courses of chemotherapy with ifosfamide and doxorubicin. After the preoperative chemotherapy, the tumor size decreased 78%, and the pleural effusion decreased substantially. A posterolateral thoracotomy was performed in June 2005. At the time of this opera-tion, there was only a little serous pleural fluid and no tumor disseminations. The tumor arose from the dorsal parietal pleura, invading the left diaphragm. A resection of the complete tumor combined with the affected left diaphragm was performed. Pathological examination revealed a monophasic synovial sarcoma. After adjuvant chemotherapy was performed with six courses of ifosf-amide and doxorubicin, she was discharged.1) Ambu-latory follow-up was uneventful until November 2006 when a CT scan revealed multiple tumors in the left pleura. She underwent chemotherapy with ifosfamide, pirarubicin, and methotrexate, but the tumors increased rapidly to occupy the left hemithorax, and she began to complain again of a severe dry cough (Fig. 1). Other-wise, laboratory findings were within normal ranges, and vital signs were stable. No other recurrent sites were found, and she underwent tumor resection combined with the entire left lung through left posterolateral tho-racotomy in March 2007. The tumor remained around the descending aorta and the aortopulmonary window. Total blood loss was 14,000 ml, and operation time was 390 minutes. Pathological examination revealed dense proliferation of atypical spindle cells, the same findings as in the initial operation, and a diagnosis of synovial sarcoma was made (Fig. 2). She recovered after respira-tory support for 3 days, and her course was uneventful thereafter. External radiation therapy up to 60 Gray was administered to the tumor remaining around the descending aorta. She is now ambulatory without evi-dence of recurrent disease at 6 months after the second operation and 27 months after the initial surgery.

Discussion

Pleural synovial sarcomas are an extremely rare disease entity, first reported by Gaertner et al. in 1996.2) Until now, only 21 surgical cases of pleural synovial sarcoma have been reported, including the present one.1–9) (Table 1). These cases were of 11 men and 10 women with ages ranging from 9 to 69 and the median age being in the 30s. Except for 2 cases of extrapulmonary pneumonec-tomy and 2 of lobectomy, most initial operations were tumor extirpations along with involved neighboring tis-sue; and 14 received additional chemotherapy or

Fig. 1. Coronal sectional images of CT through the pulmnonary hilum (upper column) and the descending aorta (lower col-umn), showing the huge masses in the left pleural cavity.

48

Nishiyama et al.

Ann Thorac Cardiovasc Surg Vol. 15, No. 1 (2009)

Tab

le 1

. D

ocum

ente

d ca

ses

of s

ynov

ial s

arco

ma

in t

he p

leur

al c

avit

y

Yea

r A

utho

r C

ase

Subt

ype

The

rapy

In

itia

l ope

rati

on

Re-

rese

ctio

n P

rogn

osis

fro

m th

e in

itia

l

surg

ery

199

6 G

aert

ner

et a

l.2)

17 F

B

i C

R

Tum

or e

xtir

pati

on w

ith

lobe

ctom

y

D

OD

aft

er 1

2 m

onth

s

17

F

Bi

CR

/PO

C/P

OR

Tu

mor

ext

irpa

tion

DO

D a

fter

18

mon

ths

50 M

B

i C

R/P

OC

/PO

R

Tum

or e

xtir

pati

on

D

OD

aft

er 2

1 m

onth

s

9

M

Bi

CR

/PO

C/P

OR

Tu

mor

ext

irpa

tion

Ali

ve w

ith

dise

ase

at 8

yea

rs

32

F

Bi

CR

/PO

C/P

OR

Tu

mor

ext

irpa

tion

Tu

mor

ext

irpa

tion

D

OD

aft

er 3

0 m

onth

s

199

7 Ja

wah

ar e

t al.3)

18

F

Bi

CR

Tu

mor

ext

irpa

tion

Ali

ve w

ith

dise

ase

at 1

6 m

onth

s

199

8 N

icho

lson

et a

l.4)

42 M

B

i C

R/P

OC

Tu

mor

ext

irpa

tion

Ali

ve w

itho

ut d

isea

se a

t 12

mon

ths

28 M

M

o C

R/P

OC

Tu

mor

ext

irpa

tion

Ali

ve w

itho

ut d

isea

se a

t 9 m

onth

s

42

M

Mo

ICR

/PO

C

Tum

or b

iops

y

DO

D a

fter

5 m

onth

s

200

1 A

ubry

et a

l.5)

33 M

M

o

Ali

ve w

itho

ut d

isea

se a

t 13

mon

ths

41 M

M

o C

R/P

OR

in o

ne,

Tum

or e

xtir

pati

on w

ith

A

live

wit

hout

dis

ease

at 4

mon

ths

41 F

M

o P

OC

in o

ne

lobe

ctom

y in

one

NA

49 F

M

o

Ali

ve w

itho

ut d

isea

se a

t 6 m

onth

s

69

M

Mo

A

live

wit

hout

dis

ease

at 1

2 m

onth

s

200

2 C

olw

ell e

t al.6)

39

M

Mo

CR

/PO

C/P

OR

E

PP

At 4

yea

rs, t

umor

ext

irpa

tion

D

OD

aft

er 8

yea

rs

23

F

Mo

CR

/PO

C

Tum

or e

xtir

pati

on

At 2

yea

rs a

nd 3

yea

rs, t

umor

ext

erpa

tion

D

OD

aft

er 4

yea

rs

33

M

Bi

CR

Tu

mor

ext

irpa

tion

Ali

ve w

itho

ut d

isea

se a

t 5 y

ears

200

2 H

iran

o et

al.7)

46

F

Bi

CR

E

PP a

t 13

year

s ag

o A

t 10

year

s ag

o, tu

mor

ext

irpa

tion

A

live

wit

hout

dis

ease

at 1

5 ye

ars

200

3 N

g et

al.8)

15

M

Mo

CR

/PO

C/P

OR

Tu

mor

ext

irpa

tion

A

t 20

mon

ths,

tum

or e

xtir

pati

on

Ali

ve w

ith

dise

ase

at 2

1 m

onth

s

200

5 Pa

pla

et a

l.9)

66 F

N

A

CR

Tu

mor

ext

irpa

tion

A

t 14

and

16 m

onth

s, tu

mor

ext

irpa

tion

A

live

wit

h di

seas

e at

20

mon

ths

200

6 Ts

ukio

ka e

t al.1)

29

F

Mo

Neo

adju

vant

(s

ame

as

CT,

CR

/PO

C

Tum

or e

xtir

pati

on

At 2

1 m

onth

s, le

ft p

neum

onec

tom

y A

live

wit

hout

dis

ease

at 2

7 m

onth

s

pres

ent c

ase)

F, f

emal

e, M

, mal

e, b

i, bi

phas

ic p

atte

rn; m

o, m

onop

hasi

c pa

ttern

; CR

, com

plet

e re

sect

ion;

PO

C, p

osto

pera

tive

chem

othe

rapy

; PO

R, p

osto

pera

tive

radi

othe

rapy

; IC

R, i

ncom

plet

e re

sect

ion;

EPP

, ext

rapl

eura

l pne

umon

ecto

my;

DO

D, d

eath

of

dise

ase;

NA

, not

ava

ilabl

e.

Aggressive Repeat Surgery for a Recurrent Synovial Sarcoma in the Pleura

Ann Thorac Cardiovasc Surg Vol. 15, No. 1 (2009) 49

radiotherapy. Six cases underwent second or third opera-tions for recurrent tumors, but only for tumor extirpa-tion; such aggressive repeat surgery as in this case has not been reported before. The American Joint Committee on Cancer 10) reports 5-year survival rates for synovial sarcoma of the soft tissues as 21%–64%. Prognosis of the resected 21 cases of pleural synovial sarcoma varies from 4 months to 15 years after the initial operation; of the seven patients who died from 5 months to 8 years after, 6 died within 5 years. The other patients are described as being alive at follow-ups occurring from 4 months to 15 years after the initial operation. Thus the prognosis and significance of an extended surgery remain unclear because of the slight amount of available data. Although some reports have been made of effec-tive results of postoperative adjuvant chemotherapy for synovial sarcoma,11) others present conflicting results.12) Mullen and Zagars12) reported good results by conserva-tion surgery and radiotherapy, showing a 5-year local recurrence rate of 14% in 85 cases of soft tissue synovial sarcoma. The present case also suggests that aggressive tumor reduction followed by radiotherapy can achieve good results in the control of local disease. Preoperative informed consent in this case was obtained after we explained every possible operative result, including only exploratory thoracotomy, tumor reduction ranging in various amounts of the tumor, and pneumonectomy, because the patient well understood that every chemo-therapy had been tried and even incomplete salvage tumor reduction could be the important treatment option that remained. Although optimal strategies for synovial sarcoma have yet to be established, aggressive repeat surgery could be considered in selected cases even when response to chemotherapy is insufficient and complete resection is difficult.

References

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2. Gaertner E, Zeren EH, Fleming MV, Colby TV, Travis WD. Biphasic synovial sarcomas arising in the pleural cavity. A clinicopathologic study of five cases. Am J Surg Pathol 1996; 20: 36–45.

3. Jawahar DA, Vuletin JC, Gorecki P, Persechino F, Macera M, et al. Primary biphasic synovial sarcoma of the pleura. Respir Med 1997; 91: 568–70.

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8. Ng SB, Ahmed Q, Tien SL, Sivaswaren C, Lau LC. Primary pleural synovial sarcoma. A case report and review of the literature. Arch Pathol Lab Med 2003; 127: 85–90.

9. Papla B, Harazda M, Kuz·dz·ał J, Wozniak A, Limon J, et al. Primary intrathoracic biphasic synovial sar-coma—a case report. Pol J Pathol 2005; 56: 207–10.

10. American Joint Committee on Cancer. In: Beahrs OH, Henson DE, Hutter RVP, Myers MH, eds. Man-ual for Staging of Cancer, 3rd ed. Philadelphia: JB Lippincott Co., 1988.

11. Pappo AS, Fontanesi J, Luo X, Rao BN, Parham DM, et al. Synovial sarcoma in children and adolescents; the St Jude Children’s Research Hospital experience. J Clin Oncol 1994; 12: 2360–6.

12. Mullen JR, Zagars GK. Synovial sarcoma outcome following conservation surgery and radiotherapy. Radiother Oncol 1994; 33: 23–30.

Fig. 2. Pathological examination revealed dense proliferation of atypical spindle cells, and a diagnosis of synovial sarcoma was made. (hematoxylin-eosin, original magnification: ×100)