Vulvar synovial sarcoma
Introduction
Introduction of vulvar synovial sarcoma Synovial sarcoma was first named in Sabrazes in 1934, and the prognosis of vulvar synovial sarcoma is relatively good. basic knowledge The proportion of illness: 0.0003% Susceptible people: no special people Mode of infection: non-infectious Complications: lymphoma
Cause
Causes of vulvar synovial sarcoma
(1) Causes of the disease
The vulvar synovial sarcoma has been mistaken for the synovial membrane of the joint, but the histological origin of the vulvar synovial sarcoma is still unclear. The typical synovial sarcoma has the characteristics of bidirectional differentiation of epithelial and mesenchymal.
(two) pathogenesis
The tumor is lobulated or nodular, with a pseudo-envelope, clear boundaries, grayish white surface, soft texture, often hemorrhagic necrosis and cyst formation.
Microscopic examination consists of two cells with different shapes. It has a unique biphasic configuration. The epithelial components are similar to cancer. The cubic to high columnar cells are arranged in solid, nested or adenoid, papillary-like structures, and spindle cells are mostly fibrosarcoma-like. Configuration, between epithelial and spindle cells, there are transitional cells. According to the relative significance and degree of differentiation of epithelial and spindle cells, synovial sarcoma can be divided into biphasic, single-phase fiber, single-phase epithelial And poorly differentiated, under the electron microscope, there are microvilli or pseudopods on the surface of epithelial cells, facing the intercellular cavity or pseudo gland cavity. The electrons are dense and mucous material in the cavity. The continuous substrate that is not in the normal synovium often has epithelial clusters or The adenoid structure is separated from the surrounding spindle cells.
Almost all synovial sarcomas have specific chromosomal translocations -t(x;18)(pll;q11), and the SSX gene of Xpll and the SYT gene of 18q11 are fused to SYT-SSXl due to chromosomal equilibrium translocation. SYT-SSX2 chimeric gene, 61% to 64% of synovial sarcoma with SYT-SSXl, about 36% of synovial sarcoma with SYT-SSX2, and SYT-SSX1 chimeric gene are biphasic synovial sarcoma. Almost all single-phase synovial sarcoma have the SYT-SSX2 chimeric gene (Ladanyi 2002). The SYT-SSX chimeric gene can be used to determine whether the surgical margin is positive, and can be used to diagnose micrometastases. The examination is more sensitive and specific.
Prevention
Vulvar synovial sarcoma prevention
Regular physical examination, early detection and determination of the scope of radical surgery, and good follow-up.
Complication
Vulvar synovial sarcoma complications Complications lymphoma
Lymph node metastasis.
Symptom
Vulvar synovial sarcoma symptoms common symptoms shame mass
It is a painless mass that grows slowly under the skin. Its size is 1.2-20cm. The mass is more fixed and the boundary is clear. It is easy to be misdiagnosed as a Papillary cyst.
Examine
Examination of vulvar synovial sarcoma
Histochemical and immunohistochemical assays were stained with PAS, colloidal gold and acin blue, and then digested with hyaluronidase. The presence of two different mucus was observed. Reticulated fiber staining revealed a biphasic configuration, especially for lower differentiation. Spindle cells vimentinl00% positive, bcl-2, CD99, S-100 can also be positive, and some can be positive for EMA and cytokeratin. Epithelioid cells are positive for 40% vimentin except EMA and cytokeratin.
Cytogenetic test, histopathological examination.
Diagnosis
Diagnosis and differentiation of vulvar synovial sarcoma
Diagnosis can be made based on clinical manifestations, symptoms and related tests.
Should be differentiated from the Papillary gland cyst.
