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Primary pulmonary synovial sarcoma: a case report.Kaohsiung
J Med Sci. 2006 Nov;22(11):590-4.
We report a
rare case of primary synovial sarcoma of the lung. A57-year-old man
had a well-defined tumor in the right middle lobe seen on chest
computed tomography, and underwent lobectomy. Grossly, the
nonencapsulated tumor measured 4.5 cm in greatest diameter, with a
solid and tan-white cut surface. Histologically, the tumor was
mainly composed of a dense proliferation of spindle cells.
Immunohistochemical studies were focally positive for epithelial
membrane antigen, and diffusely positive for CD99 and Bcl-2.
Cytokeratin, S-100 protein, desmin, smooth muscle act in, and CD34
were absent. SYT-SSX1 gene fusion transcript was detected by reverse
transcription-polymerase chain reaction, which is diagnostic of
primary synovial sarcoma of the lung. We also review the literature
with regard to the clinicopathologic, immunohistochemical, and
molecular studies of primary pulmonary synovial sarcoma.
Poorly
differentiated primary monophasic synovial sarcoma of the lung.Asian
Cardiovasc Thorac Ann. 2006
Dec;14(6):511-3.
Primary
monophasic synovial sarcoma of the lung is rarely seen in clinical
practice. We report the case of a 60-year-old male who underwent a
left lower lobectomy for lung sarcoma. The patient received adjuvant
therapy after surgery. Diagnosis was confirmed with SYT-SSX2
translocation detection.
Primary
pulmonary synovial sarcoma: a clinicopathologic, immunohistochemical,
and molecular study of 11 cases.Hum
Pathol. 2004 Jul;35(7):850-6
Primary
synovial sarcoma (SS) of the lung is rare and may create diagnostic
challenges. We reviewed 11 cases of pulmonary SS (PSS) confirmed by
the presence of a tumor-specific SYT-SSX fusion gene to verify their
clinicopathologic features including immunohistochemical and
genetical profiles. The tumors occurred in 4 men and 7 women (age 29
to 81 years; mean age, 58; median age, 50), and ranged in size from
2 to 15.5 cm (mean, 9 cm). Of the 11 tumors, 10 were a monophasic
fibrous type and 1 was a poorly differentiated type. Mitotic rate
ranged from 8 to 43 per 10 high-power fields. All cases showed at
least focal immunohistochemical positivity for AE1/AE3, CAM5.2
and/or epithelial membrane antigen. High proliferating cell nuclear
antigen labeling index (>20%) was found in 8 of 10 cases (80%).
Eight (90%) of 9 cases were negative for E-cadherin, and 1 case
(10%) exhibited reduced expression of the molecule. The aberrant
expression of beta-catenin within cytoplasm and/or nuclei was
observed in 6 of 9 (67%) cases. SYT-SSX1 and SYT-SSX2 fusion gene
transcripts were detected in 9 and 2 cases, respectively. In 10
patients with follow-up, 3 (30%) had local recurrences, and 4 (40%)
developed distant metastases. Five (50%) patients died of the tumor
1 to 9 years after surgery, and 5 (50%) were alive and disease-free
in the period ranging from 3 months to 5.5 years. In conclusion, PSS
tends to occur in older patients and shows an aggressive behavior
probably due to its anatomical location and large tumor often
resulting in incomplete resection and high proliferative activity.
Pulmonary
synovial sarcoma with polypoid endobronchial growth: a case report,
immunohistochemical and cytogenetic study.
Pathol Int. 2004 Aug;54(8):611-5.
A rare case
of primary pulmonary synovial sarcoma with polypoid endobronchial
growth in a 42-year-old Japanese woman is described. Left upper
sleeve lobectomy was performed for the polypoid tumor measuring 2.5
cm in the left major bronchus and the patient was treated with
adjuvant chemotherapy. Three years later, a recurrent tumor was
discovered. Microscopically, this tumor was characterized by a
proliferation of oval to spindle-shaped cells arranged in sheets and
fascicles and covered by the thin normal bronchial epithelium.
Immunohistochemically, tumor cells were positive for vimentin, and
focally positive for pancytokeratin recognized by AE1/AE3,
cytokeratin 7 and epithelial membrane antigen. A chimera gene,
SYT-SSX1, was detected. Recently, primary pulmonary synovial sarcoma
is an increasingly recognized clinical entity; however, most of
these tumors present as a parenchymal mass. The present case is a
unique example of primary synovial sarcoma of endobronchial polypoid
type. This case suggests that pulmonary synovial sarcoma might
originate from bronchial submucosal stromal tissue.
Primary pulmonary
synovial sarcoma: a case report and review of current diagnostic and
therapeutic standards.Oncologist.
2004;9(3):339-42
A
30-year-old female presented with hemoptysis, chest pain, and a
rapidly enlarging pleural-based mass, and was found to have primary
synovial sarcoma of the lung. Primary pulmonary sarcomas comprise
<1% of all primary lung malignancies. They present clinically in
young adults with cough, chest pain, shortness of breath, or
hemoptysis, with a mass on x-ray and computerized tomography scan.
Diagnosis is made by histology and immunohistochemistry. Histologic
diagnosis has recently been supplemented by cytogenetic analysis,
which offers important prognostic information. The mainstay of
treatment remains complete surgical excision. Prognosis is poor,
with an overall 5-year survival rate of 50%.
Primary
pulmonary synovial sarcoma: a report and diagnosis of 2 cases.
Arch Bronconeumol. 2003
Mar;39(3):136-8.
Synovial
sarcoma is an extremely rare primary pulmonary tumor whose
description is based on a limited number of cases. We report two
cases diagnosed by thoracotomy. One patient was initially treated
surgically, and a later recurrence was controlled by combined
chemotherapy and a second operation after three years of monitoring.
In the second case, surgery was imperative to treat massive
hemoptysis and was followed by combined chemotherapy after diagnosis
of lesions consistent with extrapulmonary metastasis.
Primary
poorly differentiated monophasic synovial sarcoma of the lung. A
case report with immunohistochemical and genetic studies.Pathol
Res Pract. 2003;199(12):827-33;
discussion 835-6
We describe
a case of a poorly differentiated monophasic synovial sarcoma
arising in the lung of a 50-year-old man. The tumor, which was
located in the right upper lobe, was lobulated, relatively
well-circumscribed, and whitish to yellowish in color.
Microscopically, it was composed exclusively of ovoid to polygonal
or short spindle cells, with a high nuclear to cytoplasmic ratio and
relatively scant cytoplasm, arranged in solid sheets or in a
hemangiopericytomatous pattern with intervening wiry collagen
fibers. At the periphery of the tumor, entrapped benign alveolar
epithelium produced a pseudo-biphasic appearance. In some areas, an
abundance of keloidal collagen imparted a close resemblance to a
solitary fibrous tumor, making it difficult to establish the
diagnosis on the initial needle biopsy, although the malignant
nature of the tumor was suggested because of nuclear anaplasia.
Immunohistochemically, the tumor was positive for cytokeratin
AE1/AE3, CAM5.2, EMA, vimentin, bcl-2 protein, calretinin, and CD34.
The reverse transcriptase-polymerase chain reaction (RT-PCR), using
RNA extracted from fresh-frozen tissue, demonstrated SYT/SSX-1
fusion transcripts, confirming the diagnosis of synovial sarcoma.
Microscopic examination demonstrated metastatic deposits in hilar
lymph nodes. This case indicates that a primary pulmonary synovial
sarcoma, particularly in its poorly differentiated form, is a
diagnostically challenging and highly aggressive neoplasm typically
found at an advanced stage.
An ultrastructural,
immunohistochemical, and cytogenetical study of a monophasic
pulmonary synovial sarcoma: implications of the frequent
ultrastructure of oligocilia and concentric membranous bodies with
positive immunostaining for VS38c and MEF2.Ultrastruct
Pathol. 2003 Jul-Aug;27(4):235-41
A rare case
of a monophasic pulmonary synovial sarcoma is reported. A
44-year-old Japanese man underwent lower lobectomy for a nodular
mass in his right lung. Immunohistochemical study of the excised
primitive spindle cell sarcoma revealed occasional positive stains
by hitherto reported antigens of S-100, cytokeratin 7, high
molecular weight cytokeratin (34 beta E12), pankeratin (AE1/AE3),
and EMA, which were helpful for the differential diagnosis of other
spindle cell sarcomas. Furthermore, positive immunostains for MEF2,
VS38c (plasma cell antigen), and bcl-2 were rather significant
findings in the present case. The definitive evidence that molecular
genetic analysis showed a clonal single electrophoretic band of
SYT-SSX mutated chimera gene was conclusive for the pathological
diagnosis. The implications of the frequently seen ultrastructure of
oligocilia and concentric membranous bodies with positive stains for
VS38c and MEF2 are discussed. In the difficult pathological
diagnosis of a rare and undifferentiated type of sarcoma with
unusual clinicopathological features generated at an unusual site,
comprehensive ultrastructural, immunohistochemical, and cytogenetic
studies will lead to the correct pathological diagnosis and
elucidate the detailed characteristics of the tumor.
Primary
synovial sarcoma of the lung - a rare tumor.Zentralbl
Chir. 2002 Aug;127(8):716-9
We present a
26 year old patient with a primary malignant synovial sarcoma of the
lung that was observed for more than one year by a general
practitioner and a pulmologist. Finally, because of recurrent
hemoptysis a central tumor of mesenchymal origin of the left lower
lobe was diagnosed by bronchoscopy. The invasion of the left atrium
as far as to the mitral valve was diagnosed by MRT. The patient was
operated on by extended pneumonectomy with extracorporeal
circulation. The partial excision of the left atrium required
plastic reconstruction. In the postoperative course the patient
underwent chemo-therapy, 6 cycles adriablastine/ifosfamid. 8 months
after the first operation an extensive tumor recurrency occurred
with infiltration of the chest wall. The patient refused further
radio- or chemotherapy and died 14 months after the operation.
Because of the small number of cases therapeutic strategy
conceptions do not exist. The resection of the tumor is generally
recommended. Chemo- and radiotherapy are accepted as an option for
advanced tumor stage.
Primary
sarcomas of the lung: a clinicopathologic study of 12 cases.
Lung Cancer. 2002
Dec;38(3):283-9.
BACKGROUND:
Chest physicians have a limited experience of primary pulmonary
sarcomas, which represent a particular entity among rare
intrathoracic neoplasms. DESIGN: Retrospective review of medical
records. PURPOSE: To study patients with primary sarcomas of the
lung diagnosed in our pathology department in order to define their
clinical characteristics, treatment, and prognosis. PATIENTS: The
study group consisted of 12 patients, with a mean age of 53 years.
RESULTS: The main symptoms were chest pain, and cough. Imaging
findings consisted of: eight single peripheral opacities, three
single parahilar opacities, and one lobar actelectasis. The
histologic diagnoses confirmed in all cases by detailed
immunohistochemical study were leiomyosarcoma (7), monophasic
synovial sarcoma (2), one case each of malignant peripheral nerve
sheath tumor (MPNST), epithelioid sarcoma, and malignant fibrous
histiocytoma. Thoracic surgery done in nine cases consisted of six
lobectomies with further parietal resection in two cases, and three
pneumonectomies. Four patients received chemotherapy and two
patients had radiation therapy postoperatively. Follow up available
on 12 patients ranged from 3 to 144 (mean 42) months. Long term
survival up to 3 years was observed in five patients. Median overall
survival was 48 months. Overall 5-year survival rate was 38%.
CONCLUSIONS: Primary sarcomas of the lung are a rare and aggressive
malignancy. Treatment and prognosis do not differ from other soft
tissue sarcomas.
Primary
synovial sarcoma of the lung: a case report confirmed by molecular
detection of SYT-SSX fusion gene transcripts.Jpn
J Clin Oncol. 2001 May;31(5):212-6
We report a
rare case of primary synovial sarcoma of the lung. The patient was a
49-year-old woman who presented with a well-defined oval-shaped mass
in the left upper lobe on a chest radiograph. A malignant pulmonary
tumor was suspected and consequently a left upper lobectomy was
performed. Grossly, the tumor measured 5 x 4 cm, was whitish-yellow
in color and soft in consistency. Histologically, the tumor showed a
dense proliferation of short spindle cells, partly arranged in
interlacing fascicles. In some areas a hemangiopericytoma-like
pattern, stromal myxoid change and necrosis of various sizes were
noted. Numerous mitotic figures were also seen.
Immunohistochemically, the tumor cells were positive for epithelial
markers such as cytokeratin and epithelial membrane antigen. As
these features suggested a monophasic fibrous type of synovial
sarcoma, we examined for the presence of SYT-SSXfusion gene
transcripts using RNA samples from the frozen tumor tissue. A
reverse transcription polymerase chain reaction amplified a single
583-base pair fragment characteristic of synovial sarcoma. As no
other tumorous lesions were found during a follow-up period of 1
year, primary synovial sarcoma of the lung was our final diagnosis.
This tumor should be considered in the differential diagnosis of
round to short spindle cell tumors arising in the lung.
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