PMID- 36043584
OWN - NLM
STAT- MEDLINE
DCOM- 20220901
LR  - 20220906
IS  - 0126-8635 (Print)
IS  - 0126-8635 (Linking)
VI  - 44
IP  - 2
DP  - 2022 Aug
TI  - Primary pulmonary synovial sarcoma: A clinicopathological study of 22 cases.
PG  - 215-224
AB  - INTRODUCTION: Primary pulmonary synovial sarcoma (PPSS) is a rare mesenchymal 
      tumour with characteristic translocation SS18-SSX1/2/rarely 4 fusion transcripts, 
      and presents most often in adolescents and young adults. According to the World 
      Health Organization (WHO) classification, synovial sarcoma is a malignant tumour 
      of uncertain differentiation. AIMS: To present a case series of PPSS with 
      clinical, pathological and molecular analysis at a rare primary site. SETTING AND 
      DESIGN: Retrospective study conducted in a tertiary care hospital. MATERIALS AND 
      METHODS: Twenty-two cases of PPSSs were retrieved from electronic database 
      between January 2009 to December 2018. Metastatic tumours from soft tissue 
      primaries were excluded. Immunohistochemistry (IHC) and reverse transcription 
      polymerase chain reaction (RT-PCR) were performed. Statistical analysis was 
      performed using Mann-Whitney non-parametric test. RESULTS: Among 22 patients, the 
      male-female ratio was 3.4:1 and the median age was 31.5 years. The tumours were 
      classified as monophasic (90.9%) and biphasic (9.1%) subtypes and graded as grade 
      2(77.3%) and grade 3(22.7%). IHC demonstrated expression of TLE1 (17/17 cases), 
      Bcl-2 (7/8 cases), focal EMA (16/17 cases), CD99 (10/11 cases), focal 
      pancytokeratin (8/12 cases) and CD56 (14/14 cases). The fusion transcripts 
      included SYT-SSX1(4/11, 36.4%) and SYT-SSX2 (2/11, 18.2%). The remaining five 
      cases were negative for SS18 rearrangement by RT-PCR. Only 8 patients had 
      localised tumour. Surgical excision was performed in 5 patients. The median 
      follow-up period was 6 months and 21 days. CONCLUSIONS: Monophasic SS was the 
      most common subtype. Small core biopsies pose a diagnostic challenge, in such a 
      scenario, a combination of clinical, histomorphological, immunomarkers and 
      genetic studies help confirm the diagnosis of PPSS.
FAU - Telugu, R B
AU  - Telugu RB
AD  - Christian Medical College, Department of General Pathology, Vellore, India.
FAU - Kodiatte, T A
AU  - Kodiatte TA
AD  - Christian Medical College, Department of General Pathology, Vellore, India. 
      thomaskodiatte@gmail.com.
FAU - Sakthi, D
AU  - Sakthi D
AD  - Christian Medical College, Department of General Pathology, Vellore, India.
FAU - Isaiah, R
AU  - Isaiah R
AD  - Christian Medical College, Department of Radiation Oncology, Vellore, India.
FAU - Singh, A
AU  - Singh A
AD  - Christian Medical College, Department of Medical Oncology, Vellore, India.
FAU - Gnanamuthu, B R
AU  - Gnanamuthu BR
AD  - Christian Medical College, Department of Thoracic Surgery, Vellore, India.
FAU - Backianathan, S
AU  - Backianathan S
AD  - Christian Medical College, Department of Radiation Oncology, Vellore, India.
LA  - eng
PT  - Journal Article
PL  - Malaysia
TA  - Malays J Pathol
JT  - The Malaysian journal of pathology
JID - 8101177
RN  - 0 (Biomarkers, Tumor)
RN  - 0 (Oncogene Proteins, Fusion)
RN  - 0 (Proto-Oncogene Proteins)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Biomarkers, Tumor/genetics/metabolism
MH  - Female
MH  - Humans
MH  - *Lung Neoplasms/genetics
MH  - Male
MH  - Oncogene Proteins, Fusion/genetics
MH  - Proto-Oncogene Proteins/genetics/metabolism
MH  - Retrospective Studies
MH  - Reverse Transcriptase Polymerase Chain Reaction
MH  - *Sarcoma, Synovial/diagnosis
MH  - Translocation, Genetic
MH  - Young Adult
EDAT- 2022/09/01 06:00
MHDA- 2022/09/02 06:00
CRDT- 2022/08/31 07:22
PHST- 2022/08/31 07:22 [entrez]
PHST- 2022/09/01 06:00 [pubmed]
PHST- 2022/09/02 06:00 [medline]
PST - ppublish
SO  - Malays J Pathol. 2022 Aug;44(2):215-224.