Management of Primary Pulmonary Synovial
Sarcoma: A Case Report from Northern
Pakistan and Review of Literature
Hadia Fatima*, Jovaria Ehsan, Humera Mahmood and Muhammad Faheem
Department of Oncology, Atomic Energy Cancer Hospital, Nuclear Medicine Oncology and Radiotherapy Institute (AECH, NORI), Pakistan
Submission: November 19, 2018 Published: November 28, 2018
*Corresponding author: Hadia Fatima, Resident Oncology, Atomic Energy Cancer Hospital, Nuclear Medicine Oncology and Radiotherapy Institute (AECH, NORI), Islamabad, Pakistan.
How to cite this article: Hadia F, Jovaria E, Humera M, Muhammad F. Management of Primary Pulmonary Synovial Sarcoma: A Case Report from
Northern Pakistan and Review of Literature. JOJ Case Stud. 2018; 9(3): 555761. DOI: 10.19080/JOJCS.2018.09.555761.
Primary pulmonary synovial sarcoma is a rare lung tumor and has mesenchymal origin. It is difficult to diagnose clinically and should be differentiated from other primary and metastatic lung tumors. The definitive diagnosis should be made via careful histopathological evaluation along with immunohistochemistry. Cytogenetic studies detect SYT-SSX-1 variant which has got diagnostic and prognostic value. The disease has poor outcome and complete cure is difficult to achieve. We hereby report a case of 40 year old female who was diagnosed and treated for metastatic primary pulmonary synovial sarcoma at Atomic Energy Cancer Hospital, NORI.
Primary pulmonary synovial sarcoma (PPSS) is an extremely unusual malignancy associated with aggressive behavior and poor clinical outcome . The tumor originates from mesenchymal cells . Synovial sarcomas make up to 8% of soft tissue sarcomas most commonly seen in extremities . Pulmonary sarcomas are even rare making up to just 0.1-0.5% of primary lung malignancies . The differential diagnosis includes metastatic sarcomas and another primary lung cancer . Primary pulmonary synovial sarcoma behaves like soft tissue synovial sarcoma . PPSS may arise from lung parenchyma, pleura, chest wall and mediastinum .
The molecular analysis shows characteristic chromosomal translocation t (x; 18) which confirms diagnosis in up to 90% patients and is also a poor prognostic feature . In the rest of 10% cases diagnosis is established through histopathology along with immunohistochemistry . The disease has predilection for young male patients . Our patient was 40-year-old female who presented with cough associated with hemoptysis. She was diagnosed with Stage IV PPSE and received palliative chemotherapy. Only few such cases have been reported in the literature so far and no case has been previously reported from Northern Pakistan.
A 40-year-old female patient, non-smoker and non-addict, known diabetic and hypertensive controlled on oral medication
and with past surgical history of cholecystectomy for gallstones and hysterectomy for fibroid uterus presented with complaint of dry cough for 4 months. It was later associated with hemoptysis. On examination, patient had a good performance status. Chest examination showed decreased bilateral air entry in lungs, bilateral breast examination was unremarkable and there was no axillary and cervical lymphadenopathy. Rest of the systemic examination was normal.
She was investigated, and CT scan chest and abdomen was done that showed bilateral innumerable nodular opacities both intrapulmonary as well as pleura based, of variable sizes (Figure 1). Largest intrapulmonary mass in right upper lobe measured 6 x 5.1cm (AP x TR) and large pleura-based mass along right lung measured 9 x 4.8cm (AP x TR) (Figure 2). Liver showed a large
cyst in segment IV measuring 5.3 x 5.4cm (AP x TR) (Figure 3).
There was no evidence of any breast mass lesion and axillary
lymphadenopathy. No focal osseous lesion was seen.
PET scan was done that showed hypermetabolic soft tissue
lesions involving upper lobes of both lungs, the largest one
occupying right lung with SUV of 6.8. Hypermetabolic posterior
pericardiac lymph nodes were observed. There were numerous
pleural and parenchymal deposits in bilateral lungs. There was
no evidence of hypermetabolic hepatic and osseous metastasis.
Patient underwent right lung upper lobe wedge resection.
The specimen consisted of two grey white linear cores, largest
core measuring 0.8 x 0.1cm and smaller core measured 0.3
x 0.1cm. Microscopic examination revealed spindle shaped
neoplastic cells arranged in short fascicles with interspersed
blood vessels. Unremarkable lung parenchyma was identified.
Foci of entrapped pulmonary glandular stricture were also
seen close to the edge of the lesion. The mitotic activity was
low with less than 4 mitosis/10 HPF. No pleomorphism was
seen. Immunohistochemistry revealed that tumor cells were
immunopositive for WT-1, TLE-1, Bcl-2 and CD-99 while EMA,
Cytokeratin AE1/AE3, Cytokeratin 7, Cytokeratin 19, GATA-3, CD
34, STAT-6, S-100 and Sox-10 were negative. The diagnosis of
Monophasic Synovial Sarcoma of lung was made.
The case was discussed in multidisciplinary tumor board
and the patient was planned for palliative chemotherapy.
Echocardiography was advised to the patient that was normal
with ejection fraction of 65%. The patient was started on
combination chemotherapy with Iphosphamide and Doxorubicin
combination along with Mesna in 3 weekly protocol. Patient
received 6 cycles of chemotherapy with remarkable subjective
and radiological response. The patient was kept on close follow
up. She remained asymptomatic for 6 months after which she
presented again with complaint of right hypochondrial pain.
CT scan repeated showed a large pleura based heterogeneously
enhancing soft tissue density mass lesion along right
para-vertebral region extending superiorly from T6 vertebral
body to T9-10 intervertebral disc level inferiorly, adjacent to superior
segment of right lower lobe measuring 71 x 61mm associated
with loculated pleural effusion tracking in to right oblique
fissure with associated fissural thickening (Figure 4). Multiple
intrapulmonary and pleura based soft tissue density nodules
of varying sizes were noted in bilateral lung fields with some
of them showing cavitation. Hepatic cyst in the liver was static.
Traction bronchiectatic changes were noted in anterior segment
of right upper lobe (Figure 5).
Repeat echocardiography for ejection fraction was normal.
Patient was counseled thoroughly about the advanced nature of
the disease and poor prognosis and was planned for second line
Thoracic primary synovial sarcomas are very rare neoplasms
mostly originating from lung, heart, mediastinum and esophagus
. The incidence of primary pulmonary synovial sarcomas is
very low with male predominance . WHO classifies the disease
as mesenchymal tumor with most common sites of origin being lung parenchyma, tracheobronchial tree and pulmonary artery
. The patients usually present with cough, chest pain, dyspnea
and hemoptysis and usually not associated with smoking .
Although it resembles more to soft tissue counterpart but is
difficult to diagnose clinically and radiologically from other
primary and metastatic lung tumors . Our patient was a
40-year-old non-addict female, presented with complaints of
cough and hemoptysis. The primary lung mass was centrally
placed in right para-vertebral location.
PPSE has four histological variants including spindle
cell monophasic, epithelial cell monophasic, biphasic that
includes both spindle and epithelial components and poorly
differentiated small round cell forms [3,5]. The monophasic
subtype is most common [3,5]. The gross morphology of tumor
commonly consists of interlacing fascicles, dense cellularity,
hyalinized stroma and increased vascularity . In our patient
tumor comprised of spindle cells arranged in fascicles among
interspersed blood vessels, foci of pulmonary glandular
architecture and low mitotic count.
The definitive diagnosis is made via immunohistochemical
staining. The frequent common immunostains are epithelial
membrane antigen, CD99, Bcl-2, Calponin, Cytokeratins and
Vimentin but may also show weak positivity for S100 and CD34
[3,5]. Recently cytogenetic studies are also being performed
for definitive diagnosis of synovial sarcomas which detects
translocation t(X; 18) (p11.2; q11.2) secondary to fusion of the
SYT gene on chromosome 18 to SSX1 or SSX2 on chromosome X
. In our patient diagnosis was made through histopathology
along with immunohistochemistry. The patient was diagnosed
as a case of monophasic synovial sarcoma of lung with positive
WT-1, TLE-1, Bcl-2 and CD-99 and negative for EMA, Cytokeratin
AE1/AE3, Cytokeratin 7, Cytokeratin 19, GATA-3, CD34, STAT-6,
S-100 and Sox-10 immunostains.
Radiological findings are consistent with well-defined soft
tissue density heterogeneously enhancing mass lesion with
necrosis and no calcification can be appreciated on CT scan
and PET scan . Ipsilateral pleural effusion is quite frequently
seen while hilar and mediastinal lymphadenopathy is rare [2,5].
Tumor hemorrhage and extension into chest wall can also be
seen in some cases which makes it different from rest of the
lung tumors . In our patient the CT scan showed right sided
lung mass with metastatic deposits in both lungs along with
loculated pleural effusion on ipsilateral side and there was no
lymphadenopathy. PET scan showed hypermetabolic lesions in
bilateral lung fields with largest one on right side along with
posterior pericardiac lymph nodes.
If detected early, adequate resection of the tumor with
negative margins followed by adjuvant chemotherapy is the
mainstay of treatment [3,8]. Iphosphamide and doxorubicinbased
chemotherapy improves overall survival up to 24% in
patients of synovial sarcoma when given in adjuvant setting
. Radiotherapy has no definite role in improving overall and
progression free survival . Pazopanib is a targeted agent
that also has got some survival benefit . Our patient had
presented with advanced disease in which curative therapy
was not possible. She was given iphosphamide and doxorubicin
combination in palliative setting and disease did not progress
for almost 6 months.
The disease has poor prognostic outcome with 2-year
recurrence free survival of 35.7% and 5-year overall survival of
up to 50% [1,2]. Limited data is available on the natural history
of the disease and its course due to its rarity . However,
in patients who are diagnosed with metastatic disease, the
mortality and morbidity rate are very high. Our patient was
diagnosed with stage IV primary pulmonary synovial sarcoma.
The primary pulmonary synovial sarcoma is an extremely
rare malignancy and is a diagnostic as well as a therapeutic
challenge. Radiologists and histopathologists should report
carefully keeping rare disease presentations in mind so that
correct diagnosis can be made. Such cases should be discussed
in multidisciplinary tumor boards for most appropriate