Epithelioid sarcoma of the vulva

Introduction

Introduction to vulvar epithelioid sarcoma Vulvar epithelioid sarcoma is a rare genital wart, a soft tissue of the clitoris, which is also common in the limbs of young men. basic knowledge The proportion of illness: 0.003% Susceptible people: no special people Mode of infection: non-infectious complication:

Cause

Causes of vulvar epithelioid sarcoma

(1) Causes of the disease

The histological origin of vulvar epithelioid sarcoma is unknown. Because of the similar epithelial-like cells under the microscope, Enzinger et al. (1970) officially named epithelioid sarcoma.

(two) pathogenesis

Vulvar epithelioid sarcoma is a solid mass, showing that one or more hard nodules have no capsule, the cut surface is shiny, can be fish-like, light red, grayish white or dark yellow, crisp and soft, but some fibers More lesions are more tough, and larger lesions may be accompanied by hemorrhage and necrosis, surrounded by dense fibrous tissue.

Under light microscopy, the tumor is characterized by central necrosis of the nodule. The tumor cells are fenced around the necrotic area. The periphery of the nodule is a hardened collagen fiber, which also shuttles between the tumor cells. The more the nodular central tumor cells are epithelial, There is a certain arrangement, and the closer to the peripheral part, the tumor cells are fusiform, the tumor cells are large in cytoplasm, eosinophilic, nuclear round, oval, vacuolated, nucleoli, mitotic, and calcification of tumor cells. , ossification, mucus change, cystic change, fissure.

Under the electron microscope, there is no basement membrane around the tumor cells, and the endoplasmic reticulum in the cytoplasm is rich in Golgi apparatus. The middle filament is rich and has a longitudinal arrangement of myofibroblasts. There are finger-like cells protruding and forming a bridge-like connection.

Immunohistochemistry showed bidirectional expression, that is, both epithelial and mesenchymal antibodies were positive, Vimentin, cytokeratin, and EMA were diffusely positive. Cytokeratin showed positive CK7, CK8, CKl3, CKl6, CKl8 and CKl9, most of the cells. CD34 and -catenin were positively expressed, and occasionally they were positive for desmin and HMB45, while S-100 and CD31 were mostly negative.

Prevention

Vulvar epithelioid sarcoma prevention

Regular physical examinations, early detection, early treatment, and follow-up work.

Complication

Vulvar epithelioid sarcoma complications Complication

Easy to concomitant infection.

Symptom

Vulvar epithelioid sarcoma symptoms Common symptoms Large labia majora or anus large... Nodular skin necrosis edema

Vulvar sarcoma occurs in the labia majora, clitoris, and around the urethra. At first, the mass is small, located under the skin, without any symptoms. It is found in physical examination or inadvertently. Later, the mass gradually enlarges and invades the skin to form ulcers. Pain bleeding may occur, some of which are progressively enlarged nodules, which may be single or multiple nodules, generally ranging in size from 1.5 to 4.0 cm, and as large as 9 cm, as small as 0.5 cm, in superficial tumors. Skin ulcers are caused in the early stage, and deep people tend to grow along the deep fascia. Hard and tough, nodular areas are not easy to move, sometimes with pain or tenderness.

Examine

Examination of vulvar epithelioid sarcoma

Secretory examination, tumor marker examination, immunological examination.

Histopathological examination.

Diagnosis

Diagnosis and diagnosis of vulvar epithelioid sarcoma

Vulvar epithelioid sarcoma is sometimes difficult to diagnose. It needs to be diagnosed in combination with clinical, laboratory and histopathological examination. Because the tumor grows slowly and has no special discomfort, it is easy to be misdiagnosed in clinical practice. Light microscopy, electron microscopy and various immunohistochemical indicators or special staining, if necessary, detect cytogenetic abnormalities, Kasamatsu et al (2001) reported that 1 case of vaginal epithelial sarcoma was misdiagnosed for 8 years, after the initial resection of the vulvar nodules The diagnosis was skin necrosis, and the local resection was followed by scar hyperplasia after recurrence. The second recurrence was accompanied by local superficial inguinal lymphedema. The biopsy was considered to be granulomatous lymphadenitis, and it was not diagnosed as epithelioid sarcoma until the re-reading.

Identification with Pap sac cyst, inflammatory granuloma, fibroids, lipoma, etc. In addition, this tumor also needs to be associated with poorly differentiated squamous cell carcinoma, adenocarcinoma and epithelioid peripheral neurofibromatosis, epithelioid leiomyosarcoma, epithelioid rhabdomyosarcoma. , epithelioid angiosarcoma, single-phase epithelial synovial sarcoma, non-pigmented malignant melanoma and malignant rhabdoid tumor.

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