Paget's disease of the vulva
Introduction
Introduction to vulvar Paget's disease Vulvar Paget's disease is a slowly progressive cancer with typical paget cells formed by vacuoles. About half of the patients have sweat glands. Occurred in the labia majora and perianal, genital itching and burning sensation are common symptoms. Examination revealed that the vulvar lesions are higher than the skin, local thickening, induration and desquamation on the skin surface, often hypopigmentation similar to white spots, some vulva Peggy Patients with specific diseases have underlying adenocarcinoma. basic knowledge Sickness ratio: 0.05% Susceptible people: no special people Mode of infection: non-infectious Complications: breast cancer vestibular large adenocarcinoma basal cell carcinoma bladder cancer gallbladder cancer cervical cancer colon cancer bladder cancer breast cancer
Cause
The cause of vulvar Paget's disease
Intraepithelial adenocarcinoma (30%):
The disease is an intraepithelial adenocarcinoma derived from exocrine and apocrine secretion of sweat glands. Because these lesions are expressed as apical secretion, it is believed that malignant cells are derived from undifferentiated basal cells, and "transformed cells" spread in the epithelium. After squamous epithelium, an additional type of cell may be reached which is converted to an additional type of cell during tumor formation.
Malignant cells (30%):
In most patients with secretory infiltrating organs such as sweat glands, bark gland or anorectal rectum, malignant cells migrate to the epidermis through the duct-like structure of the skin, and the lesions in the epidermis account for 70% of the epithelium. Thickening, echinoderma, infiltration of Paget cells in the deep layer of the epidermis, when Paget cells further proliferate, can spread to the entire epidermis, epidermal attachment (hair follicles, sebaceous glands, sweat glands), exudation scarring or keratinized desquamation on the vulva surface, And often spread to the anal area, even infiltrated into the inner side of the femur, urethral opening, vagina, cervix, anal and anal canal are rarely involved, local lymph nodes and other parts of the metastasis will also occur, mucus card red reaction is also positive, sulfur The sputum stain shows mucus.
Lymphocytes (30%):
Microscopically, in the deep layer of the epidermis, especially in the tip of the nail, a single or small group of scattered Paget cells can be seen. Paget cells are large round cells, rich in cytoplasm, transparent vacuoles, light staining, large nuclei, nuclear round shape. Or oval, some nucleus is folded, nucleoli are small but obvious, often located on one side, nuclear division is rare, nucleoplasmic ratio is 1:3, chromatin is fine and scattered, Paget cytoplasm contains acidic and neutral mucopolysaccharide It was positively stained with Alcian blue. PAS staining was positive before and after amylase treatment. There were no polynuclear keratinocytes in the epidermis. Paget cells did not enter the dermis from the epidermis, but could extend from the epidermis into the hair follicle epithelium, which may be accompanied by sweat gland cancer. Lymphocytes were visible in the dermis. And a small amount of plasma cells infiltrated.
Prevention
Vulvar Paget's disease prevention
1. There are many opportunities for malignant tumors in this disease with or after the disease. Patients should be examined systematically and followed up for a long time to observe the possibility of other tumors.
2. Arsenic should be used as little as possible to avoid strong radiation with long-wave ultraviolet rays. If it is multiple lesions, attention should be paid to the presence of other malignant tumors.
Complication
Vulvar Paget's disease complications Complications Breast cancer Vestibular large adenocarcinoma Basal cell carcinoma Bladder cancer Gallbladder cancer Cervical cancer Colon cancer Bladder cancer Breast cancer
The disease often coincides with other cancers at the same time or in succession. Common common cancers are: breast cancer, vestibular large adenocarcinoma, cutaneous basal cell carcinoma, bladder cancer, gallbladder cancer, vulvar invasive adenocarcinoma, combined with cervix, colon, bladder and Malignant tumors of the breast account for about 30%. When the anal mucosa is involved, it usually indicates a potential rectal adenocarcinoma.
Symptom
Vulvar Paget's disease symptoms Common symptoms Itching eczema scarring vulva burning irritating epidermis keratosis genital itching
Generally, there are no symptoms, a few have different degrees of itching and burning, the course of the disease is slow, and it can be prolonged for many years. The lesion begins in the vulva, the genital folds and the perianal, and then spread to the labia minora, which often occurs when standing for a long time. The naked eye observed the lesion as a clear red eczema-like plaque with exudative scarring or keratinized desquamation on the surface, and often spread to the anal area, even infiltrating into the femoral lining, urethra, vagina, cervix, anus and anal canal. It is rarely involved. After peeling off the skin, the bright red particles are exposed. The skin lesions are clear and the edges are irregular. The red and white lesions are the ulcer area and the highly keratinized area, and some lesions are soft and Homogeneous erythema, surgical specimens with invasive lesions are more common in vestibular lesions, such as ulcers, often marked by invasive growth, about 20% of patients with this disease with vulvar invasive adenocarcinoma, and clinical infiltration Type of cancer is more common in invasive carcinoma under the microscope. Patients with Paget's disease in the vulva have increased the risk of adenocarcinoma in other areas, especially in the anal area and breast area. Invasive adenocarcinoma of recurrent Paget's disease.
A wide range of lesions usually bulge and often begin in the area where the hair is grown.
Examine
Examination of vulvar Paget's disease
Secretory examination, tumor marker examination, polymerase chain reaction.
Histopathological examination.
Diagnosis
Diagnosis and identification of vulvar Paget's disease
diagnosis
The diagnosis of Paget disease depends on pathological biopsy. Sometimes the smear cytology can see paget cells, but the lesions limited to the deep part of the epidermis are often false negative. It should be noted that the disease can occur in multiple centers. The actual size of the lesion is better than the naked eye. The red patches are more extensive.
Differential diagnosis
Vulvar eczema
Non-infectious inflammatory skin diseases caused by allergies, neurological dysfunction, congenital allergic quality, etc., are more common in clinical practice. They are classified into acute and chronic eczema according to the course of the disease. The acute manifestations are severe pruritus in the vulva. And inflammatory rash; chronic itching can be repeated, local skin thickening, rough or lichen-like sclerosis, the main inflammatory changes under the microscope, effective hormone therapy.
2. Vulvar Bowen disease
More common in middle-aged, any part of the body can occur, the lesions often more than 1cm, dark red plaques, clear and irregular boundaries, the surface often covered with keratinized scales, erosive scars and granulation tissue formation, microscopic tumor Cellular abnormalities are more obvious, mitotic figures are more common, lesions affect the terminal hair follicles, outer hair root sheaths, sebaceous gland ducts, and easily develop into invasive cancer.
3. Eczema-like cancer primary malignant melanoma
Paget disease must be differentiated from epidermal disseminated melanoma. Melanocytes often invade the dermis, PAS and Alcian blue staining is negative, dopa staining is positive, and Paget cells do not invade the dermal mesenchyme, PAS and Alcian blue staining positive, dopa staining negative.
4. Pigmented sweat duct tumor
Is a kind of tumor, a considerable number of patients have a family history, more common in adolescent and middle-aged women, associated with endocrine, can be located alone in the vulva, can also be in the upper and lower eyelids and other parts of the face, for a waxy luster of flat papules The color is almost skin color. Under the microscope, the tumor cells are often located in a small area of the dermis. There are very large catheters in the fibrous interstitial, which are shaped like commas or scorpions. There may be cystic catheter cavities near the epidermis.
5. Candida albicans infection
Can appear refractory genital itching symptoms, skin lesions appear after the vulva skin is scratched, easy to be confused with the disease, pathological biopsy can be identified.
6. Vulvar intraepithelial neoplasia
The disease sometimes has eczema-like changes accompanied by itching symptoms, and a biopsy can confirm the diagnosis.
