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Lung tumor

Other malignancies

Synovial sarcoma


Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 3 November 2014, last major update September 2012
Copyright: (c) 2003-2014, PathologyOutlines.com, Inc.

General
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● Rare tumor in lung
● Associated with chest pain, hemoptysis, dyspnea, cough, fever
● Rarely is cystic (Am J Surg Pathol 2010;34:1176)
● 40-55% die of disease after 20 years follow-up, due to metastases to bone, CNS, liver, or invasion of adjacent organs
● Tumors with SYT-SSX2 fusion proteins have 89% 5 year survival vs. 42% for tumors with SYT-SSX1 fusion protein

Case reports
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● 45 year old woman with SYT-SSX2 and rapidly progressive course (Arch Pathol Lab Med 2003;127:e201)
● 72 year old white man with poorly differentiated tumor with rhabdoid features (Arch Pathol Lab Med 2003;127:e160)

Micro description
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● Monophasic spindle cells or biphasic with epithelial and spindle cell component
● Monophasic tumors have compact fascicles of hyperchromatic spindle cells with hemangiopericytoma-like areas, often punctuated by small arteries and capillaries in an irregular distribution
● Poorly differentiated tumors have small, round blue cells resembling Ewing sarcoma/PNET
● Rarely rhabdoid features

Micro images
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H&E, BCL2, CD99, CD117


Poorly differentiated tumor: H&E and vimentin

Positive stains
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● TLE1 highly specific (Virchows Arch 2011;459:615)

Negative stains
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● CD117 (most cases)

Molecular description
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● t(X;18)(p11.2;icq11.2)
● Produces either SYT-SSX1 or SYT-SSX2 fusion genes

Differential diagnosis
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● Monophasic tumor resembles fibrosarcoma
Carcinosarcoma: may be primary or metastatic
Hemangiopericytoma
Leiomyosarcoma
Spindle cell carcinoma

End of Lung tumor > Other malignancies > Synovial sarcoma


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