Mediastinal Synovial Sarcoma

Simone B.S.P. Terra, Scott W. Aesif, Joseph Maleszewski, Andrew L. Folpe, Jennifer M. Boland

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Synovial sarcoma (SS), a translocation-associated sarcoma characterized by SS18-SSX1/2 fusion, presents most often in the extremities of young adults. While SS regularly occurs in the pleuropulmonary parenchyma, the mediastinum is an exceedingly rare primary site; the literature on this subject is predominantly composed of case reports and small series, mostly without molecular confirmation. Cases of mediastinal SS were selected from our institutional and consultation archives. Diagnoses were confirmed by either SS18 fluorescence in situ hybridization (n=6) or reverse transcription polymerase chain reaction for SS18-SSX1/2 (n=15). Mediastinal SSs occurred in 21 patients (15 men; mean age, 38 y; range, 21 to 75). Only 1 patient was older than 50 years. Average tumor size was 13.5 cm (range: 6.4 to 23 cm). One tumor was biphasic and the rest were monophasic, 11 of which were poorly differentiated (52%). Of 10 cases with known fusion transcripts, 6 had SS18-SSX2 and 4 had SS18-SSX1. Follow-up was known for 16 patients (mean: 18.9 mo; range: 5 to 45): 14 had local disease progression or recurrence, and 6 had metastasis. Death from disease occurred in 11 of 16 patients (69%) at 5 to 32 months, and 5 (36%) were alive with disease at last follow-up (6 to 45 mo). Mediastinal SS is a rare and aggressive malignancy most common in patients younger than 50 years. Most are monophasic and reach large size before detection. Poorly differentiated morphology is common. SS should be included in the differential diagnosis of spindle cell, biphasic and poorly differentiated mediastinal tumors. Because of the rarity of SS at this site, molecular testing is recommended to confirm the diagnosis.

Original languageEnglish (US)
Pages (from-to)761-766
Number of pages6
JournalAmerican Journal of Surgical Pathology
Volume42
Issue number6
DOIs
StatePublished - Jan 1 2018

Fingerprint

Synovial Sarcoma
Neoplasms
Mediastinum
Fluorescence In Situ Hybridization
Sarcoma
Reverse Transcription
Disease Progression
Young Adult
Differential Diagnosis
Referral and Consultation
Extremities
Neoplasm Metastasis
Recurrence
Polymerase Chain Reaction

Keywords

  • mediastinum
  • Synovial sarcoma
  • thorax

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

Cite this

Terra, S. B. S. P., Aesif, S. W., Maleszewski, J., Folpe, A. L., & Boland, J. M. (2018). Mediastinal Synovial Sarcoma. American Journal of Surgical Pathology, 42(6), 761-766. https://doi.org/10.1097/PAS.0000000000001050

Mediastinal Synovial Sarcoma. / Terra, Simone B.S.P.; Aesif, Scott W.; Maleszewski, Joseph; Folpe, Andrew L.; Boland, Jennifer M.

In: American Journal of Surgical Pathology, Vol. 42, No. 6, 01.01.2018, p. 761-766.

Research output: Contribution to journalArticle

Terra, SBSP, Aesif, SW, Maleszewski, J, Folpe, AL & Boland, JM 2018, 'Mediastinal Synovial Sarcoma', American Journal of Surgical Pathology, vol. 42, no. 6, pp. 761-766. https://doi.org/10.1097/PAS.0000000000001050
Terra, Simone B.S.P. ; Aesif, Scott W. ; Maleszewski, Joseph ; Folpe, Andrew L. ; Boland, Jennifer M. / Mediastinal Synovial Sarcoma. In: American Journal of Surgical Pathology. 2018 ; Vol. 42, No. 6. pp. 761-766.
@article{64e65838e1ea44c994d6646d0886e033,
title = "Mediastinal Synovial Sarcoma",
abstract = "Synovial sarcoma (SS), a translocation-associated sarcoma characterized by SS18-SSX1/2 fusion, presents most often in the extremities of young adults. While SS regularly occurs in the pleuropulmonary parenchyma, the mediastinum is an exceedingly rare primary site; the literature on this subject is predominantly composed of case reports and small series, mostly without molecular confirmation. Cases of mediastinal SS were selected from our institutional and consultation archives. Diagnoses were confirmed by either SS18 fluorescence in situ hybridization (n=6) or reverse transcription polymerase chain reaction for SS18-SSX1/2 (n=15). Mediastinal SSs occurred in 21 patients (15 men; mean age, 38 y; range, 21 to 75). Only 1 patient was older than 50 years. Average tumor size was 13.5 cm (range: 6.4 to 23 cm). One tumor was biphasic and the rest were monophasic, 11 of which were poorly differentiated (52{\%}). Of 10 cases with known fusion transcripts, 6 had SS18-SSX2 and 4 had SS18-SSX1. Follow-up was known for 16 patients (mean: 18.9 mo; range: 5 to 45): 14 had local disease progression or recurrence, and 6 had metastasis. Death from disease occurred in 11 of 16 patients (69{\%}) at 5 to 32 months, and 5 (36{\%}) were alive with disease at last follow-up (6 to 45 mo). Mediastinal SS is a rare and aggressive malignancy most common in patients younger than 50 years. Most are monophasic and reach large size before detection. Poorly differentiated morphology is common. SS should be included in the differential diagnosis of spindle cell, biphasic and poorly differentiated mediastinal tumors. Because of the rarity of SS at this site, molecular testing is recommended to confirm the diagnosis.",
keywords = "mediastinum, Synovial sarcoma, thorax",
author = "Terra, {Simone B.S.P.} and Aesif, {Scott W.} and Joseph Maleszewski and Folpe, {Andrew L.} and Boland, {Jennifer M.}",
year = "2018",
month = "1",
day = "1",
doi = "10.1097/PAS.0000000000001050",
language = "English (US)",
volume = "42",
pages = "761--766",
journal = "American Journal of Surgical Pathology",
issn = "0147-5185",
publisher = "Lippincott Williams and Wilkins",
number = "6",

}

TY - JOUR

T1 - Mediastinal Synovial Sarcoma

AU - Terra, Simone B.S.P.

AU - Aesif, Scott W.

AU - Maleszewski, Joseph

AU - Folpe, Andrew L.

AU - Boland, Jennifer M.

PY - 2018/1/1

Y1 - 2018/1/1

N2 - Synovial sarcoma (SS), a translocation-associated sarcoma characterized by SS18-SSX1/2 fusion, presents most often in the extremities of young adults. While SS regularly occurs in the pleuropulmonary parenchyma, the mediastinum is an exceedingly rare primary site; the literature on this subject is predominantly composed of case reports and small series, mostly without molecular confirmation. Cases of mediastinal SS were selected from our institutional and consultation archives. Diagnoses were confirmed by either SS18 fluorescence in situ hybridization (n=6) or reverse transcription polymerase chain reaction for SS18-SSX1/2 (n=15). Mediastinal SSs occurred in 21 patients (15 men; mean age, 38 y; range, 21 to 75). Only 1 patient was older than 50 years. Average tumor size was 13.5 cm (range: 6.4 to 23 cm). One tumor was biphasic and the rest were monophasic, 11 of which were poorly differentiated (52%). Of 10 cases with known fusion transcripts, 6 had SS18-SSX2 and 4 had SS18-SSX1. Follow-up was known for 16 patients (mean: 18.9 mo; range: 5 to 45): 14 had local disease progression or recurrence, and 6 had metastasis. Death from disease occurred in 11 of 16 patients (69%) at 5 to 32 months, and 5 (36%) were alive with disease at last follow-up (6 to 45 mo). Mediastinal SS is a rare and aggressive malignancy most common in patients younger than 50 years. Most are monophasic and reach large size before detection. Poorly differentiated morphology is common. SS should be included in the differential diagnosis of spindle cell, biphasic and poorly differentiated mediastinal tumors. Because of the rarity of SS at this site, molecular testing is recommended to confirm the diagnosis.

AB - Synovial sarcoma (SS), a translocation-associated sarcoma characterized by SS18-SSX1/2 fusion, presents most often in the extremities of young adults. While SS regularly occurs in the pleuropulmonary parenchyma, the mediastinum is an exceedingly rare primary site; the literature on this subject is predominantly composed of case reports and small series, mostly without molecular confirmation. Cases of mediastinal SS were selected from our institutional and consultation archives. Diagnoses were confirmed by either SS18 fluorescence in situ hybridization (n=6) or reverse transcription polymerase chain reaction for SS18-SSX1/2 (n=15). Mediastinal SSs occurred in 21 patients (15 men; mean age, 38 y; range, 21 to 75). Only 1 patient was older than 50 years. Average tumor size was 13.5 cm (range: 6.4 to 23 cm). One tumor was biphasic and the rest were monophasic, 11 of which were poorly differentiated (52%). Of 10 cases with known fusion transcripts, 6 had SS18-SSX2 and 4 had SS18-SSX1. Follow-up was known for 16 patients (mean: 18.9 mo; range: 5 to 45): 14 had local disease progression or recurrence, and 6 had metastasis. Death from disease occurred in 11 of 16 patients (69%) at 5 to 32 months, and 5 (36%) were alive with disease at last follow-up (6 to 45 mo). Mediastinal SS is a rare and aggressive malignancy most common in patients younger than 50 years. Most are monophasic and reach large size before detection. Poorly differentiated morphology is common. SS should be included in the differential diagnosis of spindle cell, biphasic and poorly differentiated mediastinal tumors. Because of the rarity of SS at this site, molecular testing is recommended to confirm the diagnosis.

KW - mediastinum

KW - Synovial sarcoma

KW - thorax

UR - http://www.scopus.com/inward/record.url?scp=85046993286&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85046993286&partnerID=8YFLogxK

U2 - 10.1097/PAS.0000000000001050

DO - 10.1097/PAS.0000000000001050

M3 - Article

C2 - 29543673

AN - SCOPUS:85046993286

VL - 42

SP - 761

EP - 766

JO - American Journal of Surgical Pathology

JF - American Journal of Surgical Pathology

SN - 0147-5185

IS - 6

ER -