Synovial sarcoma of nerve

Scheithauer, Bernd W.; Amrami, Kimberly K.; Folpe, Andrew L.; Silva, Ana Isabel; Edgar, Mark A.; Woodruff, James M.; Levi, Allan D.; Spinner, Robert J.

doi:10.1016/j.humpath.2010.08.019

Cited by 40 publications

(36 citation statements)

References 32 publications

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“…Sox10 is positive in only 67% of MPNSTs, while also being present in 7% of SS cases, particularly in intraneural lesions. 8,27 Similarly, the TLE1 antibody, initially hailed as a sensitive and specific marker for SS, 28–30 experienced a subsequent waning of its specificity in later studies, 31 including focal positivity in up to 30% of MPNSTs. 29,31 Interestingly, MPNST can potentially express epithelial markers such as focal AE1:AE3 and low–molecular weight keratins, 32 and the MPNST with perineurial differentiation will consistently express epithelial membrane antigen.…”

Section: Discussionmentioning

confidence: 99%

Loss of H3K27me3 Expression Is a Highly Sensitive Marker for Sporadic and Radiation-induced MPNST

Prieto-Granada

Wiesner²,

Messina³

et al. 2016

American Journal of Surgical Pathology

242

190

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Most malignant peripheral nerve sheath tumors (MPNSTs) exhibit combined inactivation of NF1, CDKN2A, and polycomb repressive complex 2 component genes (Embryonic Ectoderm Development [EED] and Suppressor of Zeste 12 [SUZ12]). Mutations in EED and SUZ12 induce loss of trimethylation at lysine 27 of histone 3 (H3K27me3), with subsequent aberrant transcriptional activation of polycomb repressive complex 2–repressed homeobox master regulators. These findings prompted us to investigate the performance of an anti-H3K27me3 monoclonal antibody clone C36B11 as an immunohistochemical marker for MPNSTs. We assessed the C36B11 reactivity pattern in a pathologically and genetically well-characterized cohort of 68 MPNSTs, spanning various clinical presentations, such as type 1 neurofibromatosis (NF1), radiotherapy, and sporadic MPNSTs. We found that 69% (n=47) of all MPNSTs demonstrated loss of H3K27me3 expression, with 42 (61%) showing complete loss and 5 (7%) showing partial loss, whereas 31% (n = 21) retained H3K27me3 expression. Among the NF1-related high-grade MPNSTs, 60% demonstrated loss of expression. In contrast, the majority of both sporadic (95%) and radiotherapy-related (91%) MPNSTs showed loss of H3K27me3 expression. Two of the 3 low-grade MPNSTs and all neurofibromas showed retained expression. Furthermore, all 5 epithelioid MPNSTs retained H3K27me3 labeling. The specificity of H3K27me3 loss as a marker for MPNSTs was studied by testing a large spectrum of lesions included in MPNST differential diagnosis, such as spindle/desmoplastic melanomas, synovial sarcomas, myoepithelial tumors, and other mesenchymal neoplasms, all of which retained expression of H3K27me3. We conclude that immunohistochemical analysis of H3K27me3 has good sensitivity and robust specificity for the diagnosis of MPNST, particularly outside of NF1 clinical history, which represents the most challenging diagnostic setting.

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Section: Discussionmentioning

confidence: 99%

Loss of H3K27me3 Expression Is a Highly Sensitive Marker for Sporadic and Radiation-induced MPNST

Prieto-Granada

Wiesner²,

Messina³

et al. 2016

American Journal of Surgical Pathology

242

190

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“…Synovial sarcomas classically are seen in limbs, but other locations are occasionally seen such as heart, lung, small intestine, soft palate, and retro-peritoneum. Peripheral nerves have been shown to be site of origin of this neoplasm, as reported in 22 instances (8).…”

Section: Discussionmentioning

confidence: 78%

“…It is mostly prevalent in the extremities of adolescents and young adults. Occasionally it has been reported in unusual sites such as heart, lung, small intestine, soft palate, retro-peritoneum and 22 cases have also been reported in peripheral nerves (2)(3)(4)(5)(6)(7)(8)(9)(10).…”

Section: Introductionmentioning

confidence: 99%

Synovial Sarcoma Involving the Median Nerve: A Case Report

Tosi

Orcioni

Biase

et al. 2012

TJPATH

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ÖZSinovyal sarkom çok farklı ve beklenmedik dokulardan kaynaklanabilir. Burada kromozomal resiprokal translokasyonu (X; 18) gösterilmiş olan 59 yaşındaki kadın hastada sinir kaynaklı bifazik sinovyal sarkom olgusu sunulmaktadır. Benzer tümörlerin ayırıcı tanısı tartışılmaktadır.Anahtar Sözcükler: Sinovyal sarkom, Sarkom, Yumuşak doku tümörleri, Sinir, Önkol ABSTRACT Synovial sarcoma may arise from different and unusual sites. Here a case of biphasic synovial sarcoma arising or invading the radial nerve in a 59-year-old female classically showing chromosomal reciprocal translocation (X; 18) is reported. The differential diagnosis from similar tumors is discussed.

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“…Up to 5% of synovial sarcomas arise in the body axis with frequent involvement of the spinal nerve roots [13], as demonstrated by several of our cases. Synovial sarcoma rarely arises as a primary intra-neural tumor and the largest reported series has 10 patients [14]; none of our study cases were unequivocally endoneurial. Finally, there are isolated case reports of intra-cranial synovial sarcomas, either primary dural-based tumors [15, 16] or intra-cranial metastases from a systemic primary [17].…”

Section: Discussionmentioning

confidence: 79%

“…The literature is relatively sparse on synovial sarcomas affecting the nervous system, with the most significant contribution being Scheithauer et al [14] 10 cases which were either primary intra-neural in origin or had endoneurial spread. Compared to their more common soft tissue counterparts the relatively unique features of Scheithauer’s intra-neural synovial sarcomas included over-representation of the monophasic subtype (90%) and SSX2 fusion partner [14].…”

Section: Discussionmentioning

confidence: 99%

Clinical Neuropathology practice guide 6-2013: morphology and an appropriate immunohistochemical screening panel aid in the identification of synovial sarcoma by neuropathologists

Keith¹,

Bilbao²,

Croul³

et al. 2013

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Aims: Pathologists are under increasing pressure to accurately subclassify sarcomas, yet neuropathologists have limited collective experience with rare sarcoma types such as synovial sarcoma. We reviewed 9 synovial sarcomas affecting peripheral nerve diagnosed by neuropathologists and explored the morphologic and immunohistochemical differences between these and MPNST. Our goal was to make practical recommendations for neuropathologists regarding which spindle cell tumors affecting nerve should be sent for SYT-SSX testing. Methods: Clinical records and genetics were reviewed retrospectively and central pathology review of 9 synovial sarcomas and 6 MPNST included immunohistochemistry for SOX10, S100, BAF47, CK (lmw, pan, CK7, CK19), EMA, CD34, bcl2, CD99, and neurofilament. Results: Common synovial sarcoma sites were brachial plexus, spinal and femoral nerve, none were “intra-neural”, all had the SYT-SSX1 translocation, and 6/9 were monophasic with myxoid stroma and distinct collagen. Half of the monophasic synovial sarcomas expressed CK7, CK19 or panCK in a “rare positive cells pattern”, 8/9 (89%) expressed EMA, and all were SOX10 immunonegative with reduced but variable BAF47 expression. Conclusions: We recommend that upon encountering a cellular spindle cell tumor affecting nerve neuropathologists consider the following: 1) SYT-SSX testing should be performed on any case with morphology suspicious for monophasic synovial sarcoma including wiry or thick bands of collagen and relatively monomorphous nuclei; 2) neuropathologists should employ a screening immunohistochemical panel including one of CK7, panCK or CK19, plus EMA, S100 and SOX10, and 3) SYT-SSX testing should be performed on any spindle cell tumor with CK and/or EMA immunopositivity if SOX10 immunostaining is negative or only labels entrapped nerve elements.

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Synovial sarcoma of nerve

Cited by 40 publications

References 32 publications

Loss of H3K27me3 Expression Is a Highly Sensitive Marker for Sporadic and Radiation-induced MPNST

Loss of H3K27me3 Expression Is a Highly Sensitive Marker for Sporadic and Radiation-induced MPNST

Synovial Sarcoma Involving the Median Nerve: A Case Report

Clinical Neuropathology practice guide 6-2013: morphology and an appropriate immunohistochemical screening panel aid in the identification of synovial sarcoma by neuropathologists

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