Borri, Alessandro

Del Curto, Barbara

Di Tonno, Clementina

Galetta, Domenico

Gasparri, Roberto

Leo, Francesco

Pelosi, Giuseppe

Petrella, Francesco

Solli, Piergiorgio

Spaggiari, Lorenzo

Veronesi, Giulia

D. Galetta

F. Leo

P. Solli

G. Veronesi

A. Borri

R. Gasparri

F. Petrella

G. Pelosi

DI TONNO, CLEMENTINA ANNA MARIA

B. Del Curto

L. Spaggiari

AIR Universita degli studi di Milano

Primary thoracic synovial sarcoma : factors affecting long-term survival

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Primary thoracic synovial sarcoma: Factors affecting long-term survival 

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Brief Communications
8rimary thoracic synovial sarcoma: Factors affecting long-term survival
omenico Galetta, MD,a Giuseppe Pelosi, MD,b,c Francesco Leo, MD,a Piergiorgio Solli, MD,a Giulia Veronesi, MD,a
lessandro Borri, MD,a Roberto Gasparri, MD,a Francesco Petrella, MD,a Clementina Di Tonno, MD,barbara Del Curto, DSc,b and Lorenzo Spaggiari, MD, PhD,a,c Milan, Italy
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iSupplemental material is
available online.
ynovial sarcoma is a malignant neoplasm predominantly
affecting soft tissues of the extremities in adolescents
and young adults and might rarely occur in other ana-
tomic locations. The lung, mediastinum, pleura, and
hest wall have also recently been found to be sites affected
rimarily by this sarcoma.1-3
We evaluated the clinical and pathologic features of primary
ynovial sarcoma (PSS) of the chest and the role of surgical interven-
ion and tried to identify possible prognostic factors affecting survival.
linical Summary
etween October 1998 and December 2006, 15 patients (7 men)
nderwent a resection of PSSs of the chest, which were catego-
ized into pulmonary, mediastinal, and chest wall PSSs according
o the tumor location. Medical records of these patients were
nalyzed for age, sex, preoperative symptoms, radiologic findings,
athology, surgical procedures, clinical outcome, and long-term
urvival.
All pathologic slides were rereviewed, and PSSs were con-
rmed by means of immunohistochemical and fluorescence in situ
ybridization studies. Fluorescence in situ hybridization analysis
as performed for the identification of the diagnostic chromo-
omal translocation (t[X;18][p11.2;q11.2]), a marker for this tu-
or, resulting from fusion of the SYT gene on chromosome 18 to
ither the SSX1 or SSX2 gene on chromosome X.
Median age was 52 years (range, 21–77 years). Computed
omographic scans of the chest (Figure 1 and Figure E1) 
erformed in all patients and demonstrated a pulmonary mass in 8
atients, a mediastinal neoplasm in 2 patients (anterior and poste-
ior, respectively), and a chest wall tumor in 5 patients. A positron
From the Divisions of Thoracic Surgerya and Pathology and Laboratory
Medicine,b European Institute of Oncology, Milan, Italy, and the University
of Milan School of Medicine,c Milan, Italy.
Received for publication May 22, 2007; accepted for publication May 31,
2007.
Address for reprints: Domenico Galetta, MD, Division of Thoracic Surgery,
European Institute of Oncology, Via Ripamonti 435, 20141, Milan, Italy
(E-mail: mimgaletta@yahoo.it).
J Thorac Cardiovasc Surg 2007;134:808-9
0022-5223/$32.00
Copyright © 2007 by The American Association for Thoracic Surgerym
doi:10.1016/j.jtcvs.2007.05.036
08 The Journal of Thoracic and Cardiovascular Surgery ● Septee
mission tomographic scan was performed in 4 patients, showing
igh tumor activity in all of them (maximal standardized uptake
alue: range, 4.5–11.2; median, 6.6).
Surgical procedures included bilobectomy, pneumonectomy,
nd pleuropneumonectomy, respectively, in 1 patient each;
obectomy in 3 patients; chest wall resection in 5 patients; and
ide wedge pulmonary resection and mediastinal tumor exci-
ion in 2 patients, respectively. Resection was complete in 9
60%) patients.
Tumor size ranged from 3.5 to 21 cm (median, 8 cm). Six
umors were larger than 10 cm. Macroscopically, in 10 cases
umors presented as well-circumscribed masses. Histopathologic
iagnosis confirmed a biphasic tumor in 1 patient (Figure E1, A
nd inset), monophasic fibrous tumors in 13 patients (Figure E1,
), and a poorly differentiated tumor in 1 patient (Figure E1, C).
hirteen patients showed diffuse immunoreactivity for epithelial
embrane antigen and keratins. Bcl-2 and S-100 results were
egative in all patients. The tumor was positive for t(X;18) in all
atients, confirming the diagnosis of synovial sarcoma (Figure
, inset).
One (6.6%) patient died postoperatively of adult respiratory
istress syndrome. Major complications occurred in 3 patients
postoperative bleedings requiring reoperation). Minor complica-
ions included prolonged air leak in 3 patients and atelectasis in 2
atients. Median hospitalization was 7 days (range, 5–11 days).
leven patients had postoperative adjuvant therapy (3 received
hemotherapy, 1 received radiotherapy, and 7 received chemora-
iotherapy).
Median follow-up was 25 months (range, 1-148 months). Local
ecurrence developed in 7 (46.6%) patients, 2 (13.3%) of whom
ad distant metastasis. The 5-year disease-free interval rate was
0% (median, 15 months). The 10-year survival rate was 33.5%.
ix (40%) patients are currently alive, 3 (20%) with disease
overall median survival, 27 months).
Factors that adversely affected survival include a tumor dimen-
ion of greater than 10 cm (P  .0062), incomplete resection (P 
0114), and no adjuvant therapy (P  .035).
iscussion
SSs involving the thorax are rare entities reported in the literature
ither as single observations or small series.1-4 They usually occu
n adults, and the mean age at diagnosis is 38 years.2 Our series
howed that thoracic PSS is diagnosed at an average age of 52
ears and showed no sex predilection. Our findings are in keeping
ith prior studies and emphasize that PSS of the chest belongs in
he differential diagnosis of chest masses in young adults (local-
zed fibrous tumors of the pleura, mesothelioma, fibrosarcoma, and
etastatic disease).
mber 2007
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Brief CommunicationsImmunohistochemical findings that distinguish synovial sar-
oma from other sarcomas include positive staining for cytokeratin
nd EMA. Synovial sarcomas lack staining for S-100 and smooth
uscle markers.
Optimal treatment of PSS of the chest has not been defined.
ultimodal therapy of surgical intervention, chemotherapy, and
adiotherapy has been used. Recurrences are likely and might be
reated with further resection when possible or with radiotherapy.
n our series chemotherapy and radiotherapy helped to control the
ecurrences, with a long disease-free survival.
In conclusion, thoracic PSS is a very uncommon and aggressive
alignancy with poor prognosis. The best treatment is not yet
efined. Complete resection is feasible and leads to an acceptable
ocal control and long-term survival.
The Journal of Thoraciceferences
. Zeren H, Moran CA, Suster S, Fishback NF, Koss MN. Primary
pulmonary sarcomas with features of monophasic synovial sarcoma: a
clinicopathologic, immunohistochemical, and ultrastructural study of 25
cases. Hum Pathol. 1995;26:474-80.
. Duran-Mendicuti A, Costello P, Vargas SO. Primary synovial sarcoma
of the chest: radiographic and clinicopathologic correlation. J Thorac
Imaging. 2003;18:87-93.
. Nicholson AG, Goldstraw P, Fisher C. Synovial sarcoma of the pleura
and ist differentiation from other primary pleural tumors: a clinicopath-
ologic and immunohistochemical review of three cases. Histopathology.
1998;33:508-13.
. Beguéret H, Galateau-Salle F, Guillou L, Chetaille B, Brambilla E,
Vignaud J-M, et al. Primary intrathoracic synovial sarcoma. A clinico-
Figure 1. Computed tomographic scans
showing pulmonary (A) and mediastinal
(B) localization of primary synovial sar-
comas.pathologic study of 40 t(X;18)-positive cases from the French Sarcoma
group and the Mesopath group. Am J Surg Pathol. 2005;29:339-46.
and Cardiovascular Surgery ● Volume 134, Number 3 809
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Brief Communications
8igure E1. Computed tomographic scan showing chest wall lo-
alization of primary synovial sarcoma.09.e1 The Journal of Thoracic and Cardiovascular Surgery ● Seigure E2. Histologic features of synovial sarcoma of the lung
howing a biphasic tumor with glandular epithelial aggregates
ntermingled with spindle cells and highlighted by cytokeratin
mmunoreactivity (A, inset), a monophasic fibrous tumor com-
osed entirely of short spindle cells (B), and a poorly differenti-
ted tumor composed of more atypical, spindle to rounded cells
ith high mitotic count (C, arrowheads). Fluorescence in situ
ybridization analysis documents t(18q11.2). Tumor cells show
ne orange and one green signal, which are indicative of a
earrangement of one copy of the SYT gene region (C, inset).ptember 2007


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Primary thoracic synovial sarcoma: Factors affecting long-term survival

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