Synovial sarcoma

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MeshID = D013584A synovial sarcoma is a rare form of cancer which usually occurs near to the joints of the arm or leg. It is one of the soft tissue sarcomas.

Introduction

The cells of a synovial sarcoma have a microscopic appearance similar to that of the synovium, giving the disease its name,but the actual cells from which the tumour develops are unknown and not necessarily synovial. cite book|editor=Raphael E. Pollock|title=Soft Tissue Sarcomas|series=American Cancer Society Atlas of Clinical Oncology|year=2002|publisher=BC Decker|isbn=1055009-128-X]

Primary synovial sarcomas are most common in the soft tissue near the large joints of the arm and leg but have been documented in most human tissues and organs, including the brain, prostate and heart.

Synovial sarcoma occurs most commonly in the young, representingabout 8% of all soft tissue sarcomascite web |title =Synovial Sarcoma|url="http://www.liddyshriversarcomainitiative.org/Newsletters/V02N06/Synovial/synovial.htm] but about 15-20% of cases in adolescents and young adults.cite journal|author=Weiss SW, Goldblum J|chapter=Malignant soft tissue tumors of uncertain type|editor=Weiss SW, Goldblum JR|title= Enzinger and Weiss’s Soft Tissue Tumors|place=St Louis, Missouri|publisher=CV Mosby|pages=1483–1571] The peak of incidence is before the 30th birthday and males are affected more often than females (ratio around 1.2:1).

Histopathology

Two cell types can be seen microscopically in synovial sarcoma. One fibrous type, known as a spindle or sarcomatous cell, is relatively small and uniform and found in sheets. The other is epithelial in appearance. Classical synovial sarcoma has a biphasic appearance with both types present. Synovial sarcoma can also appear to be poorly differentiated or to be monophasic fibrous, consisting only of sheets of spindle cells. Some authorities state that, extremely rarely, there can be a monophasic epithelial form which causes difficulty in differential diagnosis.

Like other soft tissue sarcomas, there is no universal grading system for reporting histopathology results.cite journal |author=Coindre JM |title=Grading of soft tissue sarcomas: review and update |journal=Arch. Pathol. Lab. Med. |volume=130 |issue=10 |pages=1448–53 |year=2006 |pmid=17090186 |doi=] In Europe, the Trojani or French system is gaining in popularity [cite journal|title=The management of soft-tissue sarcomas of the extremities|author=A. S. Paul and others|journal=Current Orthopaedics|volume=17|year=2003|pages=124–133|doi=10.1054/cuor.2002.0314] while the NCI grading system is more common in the United States. The Trojani system scores the sample, depending on tumour differentiation, mitotic index, and tumour necrosis, between 0 and 6 and then converts this into a grade of between 1 and 3, with 1 representing a less aggressive tumour. The NCI system is also a three grade one but takes account of a number of other factors.

Molecular biology

Most, and perhaps all, cases of synovial sarcoma are associated with a reciprocal translocation t(x;18)(p11.2;q11.2). There is some debate about whether the molecular observation itself is definitional of synovial sarcoma. []

The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t(X;18). [cite journal |author=Coindre JM, Pelmus M, Hostein I, Lussan C, Bui BN, Guillou L |title=Should molecular testing be required for diagnosing synovial sarcoma? A prospective study of 204 cases |journal=Cancer |volume=98 |issue=12 |pages=2700–7 |year=2003 |pmid=14669292 |doi=10.1002/cncr.11840] This translocation event between the SYT gene on chromosome 18 and one of 3 SSX genes (SSX1, SSX2 and SSX4) on chromosome X causes the presence of a SYT-SSX fusion gene. The resulting fusion protein brings together the transcriptional activating domain of SYT and the transcriptional repressor domains of SSX. SYT-SSX is thought to underlie synovial sarcoma pathogenesis through dysregulation of gene expression.

There is some association between the SYT-SSX1 or SYT-SSX2 fusion type and both tumour morphology and five-year survival. [cite journal |author=Ladanyi M, Antonescu CR, Leung DH, "et al" |title=Impact of SYT-SSX fusion type on the clinical behavior of synovial sarcoma: a multi-institutional retrospective study of 243 patients |journal=Cancer Res. |volume=62 |issue=1 |pages=135–40 |year=2002 |pmid=11782370 |doi=]

ymptoms

Synovial sarcoma usually presents with an otherwise asymptomatic swelling or mass, although general symptoms related to maligancies can be reported such as fatigue.

Treatment

Treatment usually involves:

*Surgery, to remove the tumor and a safety margin of healthy tissue. This is the mainstay of synovial sarcoma treatment and is curative in approximately 20-70% of patients, depending on the particular study being quoted. cite journal |author=Lewis JJ, Antonescu CR, Leung DH, "et al" |title=Synovial sarcoma: a multivariate analysis of prognostic factors in 112 patients with primary localized tumors of the extremity |journal=J. Clin. Oncol. |volume=18 |issue=10 |pages=2087–94 |year=2000 |pmid=10811674 |doi=]

*Conventional chemotherapy, (for example, Doxorubicin hydrochloride and Ifosfamide), to reduce the number of remaining microscopic cancer cells. The benefit of chemotherapy in synovial sarcoma to overall survival remains unclear, although a recent study has shown that survival of patients with advanced, poorly differentiated disease marginally improves with doxorubicin/ifosfamide treatment.
*Radiotherapy to reduce the chance of local recurrence. The benefit of radiotherapy in this disease is less clear than for chemotherapy.

External links

*humpath [http://www.humpath.com/spip.php?page=article&id_article=1965 #1965] (Pathology images)
* [http://liddyshriversarcomainitiative.org/Sarcomas/synovial_sarcoma.htm Liddy Shrive Sarcoma Initiative]
* [http://www.liddyshriversarcomainitiative.org/Newsletters/V02N06/Synovial/synovial.htm Synovial Sarcoma by Andrea Ferrari, MD and Paolo Collini MD]

References