Vulvar acinar soft tissue sarcoma

Introduction of vulvar alveolar soft tissue sarcoma Vulvar alveolar soft tissue sarcoma is a rare and highly malignant tumor. It is mainly composed of deep soft tissues of the extremities, and is mainly metastatic to the lungs. Vulvar alveolar soft tissue sarcoma is extremely rare, occurring in adolescence. Vulvar alveolar soft tissue sarcoma is usually found in physical examination or inadvertently. It is a painless mass that grows slowly. The course of the disease varies from several months to several years, and may be accompanied by ulcers. basic knowledge The proportion of illness: 0.003% Susceptible people: women Mode of infection: non-infectious Complications: sarcoma

The cause of vulvar alveolar soft tissue sarcoma

Causes:

The vulvar vesicular soft tissue sarcoma has several hypotheses such as paraganglia, Schwann cells, chemoreceptors, renin-producing cells and skeletal muscle origin. Most studies support the myogenic theory, but some scholars disagree. (Ordonez 1999), WHO (1999) classified it into other categories.

Pathogenesis:

The masses are mostly round, elliptical or nodular, often with incomplete capsules, cut face gray-red, soft, easy to break, common hemorrhagic necrosis. Microscopic examination is characterized by vascular fibrosis dividing tumor cells into a nest, which is characterized by acinar-like structure, solid nested structure, and floral ring structure. The tumor cells have clear cytoplasmic cells and weak eosinophilic granulocyte cytoplasmic cells 2 HE staining can find PAS-positive, anti-amylase eosinophilic crystals in the cytoplasm. Electron microscopy confirmed that the crystals are secreted products formed in the Golgi.

Immunohistochemistry showed partial desmin, actin, myoglo bin, MyoDl, myogenin positive, and S-100 negative.

Ladanyi et al (2001) found that in the alveolar soft tissue sarcoma, the TFE3 gene on Xp11.2 and 17q25 were present due to the presence of a more specific chromosomal alteration, der (17)t(X;17)(p11.2;q25). The fusion of ASPL gene forms the TFE3-ASPL chimeric gene, and the detection of the chromosomal translocation or chimeric gene by FISH or RT-PCR is helpful for the diagnosis of this tumor. Recently, Ladanyi et al. (2002) proposed a fusion of TFE3 gene. Point-specific antibodies were used to detect the expression of TFE3 gene in the nucleus by immunohistochemistry to assist in the diagnosis of alveolar soft tissue sarcoma.

Vulvar alveolar soft tissue sarcoma prevention

Regular physical examination, early detection, early treatment, and good follow-up.

Complications of vulvar alveolar soft tissue sarcoma Complications sarcoma

Vulvar alveolar soft tissue sarcoma is a rare and highly malignant tumor. It is mainly composed of deep soft tissues of the extremities, and is mainly metastatic to the lungs. Vulvar alveolar soft tissue sarcoma is extremely rare, occurring in adolescence. Immunohistochemistry showed partial desmin, actin, myoglo bin, MyoDl, myogenin positive, while S-100 was negative. Because the sarcoma is ruptured by friction, and the body's resistance and immunity are reduced, it is easy to merge.

Vulvar alveolar soft tissue sarcoma symptoms Common symptoms Large labia majora or anus large...

Vulvar alveolar soft tissue sarcoma is usually found in physical examination or inadvertently, showing a painless mass that grows slowly. The course of the disease varies from several months to several years, and may be accompanied by ulcers. Three patients are seen in the labia majora and the right labia minora. And the lower right side of the urethral opening, the size is 2 ~ 4cm.

Examination of vulvar alveolar soft tissue sarcoma

Histopathological examination.

CT has good sensitivity and specificity for diagnosing lung metastasis lesions. It can be seen that nodules or masses with clear boundary density increase, CT can be seen in the curved and dilated blood vessels in the tumor, tumor marker examination, ulcer secretion examination, combined immunity Group inspection.

Diagnosis and diagnosis of vulvar alveolar soft tissue sarcoma

Diagnosis can be based on clinical manifestations, symptoms, CT imaging, and histopathological examination.

Due to the lack of symptoms and slow growth of genital alveolar soft tissue sarcoma, it is easy to be misdiagnosed as benign lesions such as Pap sac cyst and granulosa cell tumor. According to the microscopic acinar-like structure, the capillary-rich interstitial and characteristic cytoplasm Eosinophilic crystallization, combined with immunohistochemistry and expression of specific chimeric genes, should not be difficult to make a differential diagnosis.

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