synovial sarcoma


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syn·o·vi·al sar·co·ma

a rare malignant tumor of synovial origin, most commonly involving the knee joint and composed of spindle cells usually enclosing slits or pseudoglandular spaces that may be lined by radially disposed epitheliallike cells.

synovial sarcoma

a malignant tumor, composed of synovioblasts, that begins as a soft swelling and often metastasizes through the bloodstream to the lung before it is discovered.

synovial sarcoma

A mesenchymal spindle cell tumour which comprises ±10% of all soft-tissue sarcomas. It is most common in young (age 20 to 40) males, and occurs most commonly around the large joints—knee, ankle, foot, 60% in the legs, 23% in upper extremity. However, it can go any place, as cases have been reported in the penis, lung, vulva, abdominal wall, etc.

Prognosis
50% 5-year survival; extensively calcified synovial sarcomas have a higher (84%) 5-year survival; 40% metastasise to lungs, bone-regional nodes.

Good prognostic factors
Children; < 5cm; <10 mitoses/10hpf; no necrosis; complete excision in first attempt.
 
Adverse prognosticators
Older age, larger size (especially, > 35 cm), necrosis, vascular invasion, high Ki-67 index, high-grade nuclei, poorly differentiated areas, known recurrence.

Synovial sarcoma, histologic types
Biphasic
• Distinct epithelial and spindle cell components.
 
DiffDx
Mesothelioma, carcinosarcoma.

Monophasic

DiffDx, Spindle cell type
Malignant peripheral-nerve sheath tumour, schwanoma, solitary fibrous tumour, fasciitis.

DiffDx, Glandular cell type
Any adenocarcinoma.

Poorly differentiated

DiffDx
Ewing sarcoma and other small round cell tumours.

synovial sarcoma

Surgery A mesenchymal malignancy that comprises ±10% of all soft tissue tumors; it is most common in young–age 20-40 ♂, knee, ankle, foot, etc Prognosis 50% 5-yr survival; extensively calcified SSs have a higher–84% 5-yr survival. See Sarcoma, Soft tissue tumors.
References in periodicals archive ?
Gene expression profiling studies have shown a major association of the Wnt signaling pathway with synovial sarcoma and overexpression of members of the TLE (Transducin-Like Enhancer of split) family of genes, particularly TLE1, which is one of four TLE genes that encode human transcriptional repressors homologous to the Drosophila corepressor groucho, and is an important component of the Wnt signaling pathway.
Immune Design initially presented data at the American Society of Clinical Oncology 2017 Annual Meeting from 25 NY-ESO-1+ soft tissue patients, including 14 synovial sarcoma patients.
He was diagnosed with synovial sarcoma, a rare form of cancer, just a day before his 18th birthday in 2016, according to a GoFundMe page set up to raise money for his wedding.
But doctors quickly diagnosed the teen with synovial sarcoma, which targets soft tissue and is more common among adolescents and young adults than other age groups who contract it.
Last June 15-year-old Tristin Stewart was diagnosed with an aggressive form of soft tissue cancer known as synovial sarcoma in his right leg.
The differential diagnosis is fairly broad and includes phlegmon/abscess, nerve sheath tumor, synovial sarcoma, fibrous histiocytoma (benign, malignant and angiomatoid forms), angiosarcoma, and fibromatosis.
To the Editor: Synovial sarcoma (SS) is a relatively rare type of soft tissue sarcoma that constitutes approximately 8% of all soft tissue sarcomas.
Currently, there are six ongoing Phase I/II studies investigating GSK3377794 as monotherapy (in non-small lung cancer, metastatic melanoma, ovarian, multiple myeloma, synovial sarcoma, and myxoid round cell liposarcoma), as well as a Phase I study in combination with pembrolizumab in multiple myeloma.
We have seen compelling data for the NY-ESO investigational cell therapy in synovial sarcoma and, following this option exercise, we will capitalise on our in-house cell and gene therapy capabilities to support the development programme for GSK3377794.
Example 9: A Monophasic Synovial Sarcoma at Base of the Tongue and Mandible
Furthermore, in July 2016, the company gained access to the EMA's newly-established Priority Medicines regulatory initiative for the company's SPEAR T-cell therapy, which targets NY-ESO for the treatment of patients with metastatic synovial sarcoma.