Kaposi's sarcoma of the vulva
Introduction
Introduction to vulvar Kaposi's sarcoma Vulvar Kaposi's sarcoma is a mass with slow vulvar growth accompanied by genital itching and burning sensation. It has malignant tumors of vulvar pain and vaginal discharge, surface skin ulceration and lymphedema of lower extremities. Kaposi sarcoma is usually classified into classic type. Or Kaposi sarcoma of European type, African type or regional Kaposi sarcoma, immunosuppressive-induced type and AIDS-related type and transplant-related Kaposi sarcoma. basic knowledge The proportion of illness: 0.005% Susceptible people: women Mode of infection: non-infectious Complications: lower extremity lymphedema
Cause
The cause of vulvar Kaposi's sarcoma
(1) Causes of the disease
It is currently believed that human herpes simplex virus-8 (also known as KSHV) is closely related to the occurrence of vulvar Kaposi's sarcoma. It is related to the cytomegalovirus infection from serological and biochemical aspects, because of the high incidence among AIDS patients. Therefore, it supports the theory of virology. The etiology of vulvar Kaposi's sarcoma has raised the hypothesis of geographical factors, ethnic factors, climatic factors, gender factors, genetic factors, trauma and occupational factors, infection factors, etc., but they have not been fully It is generally confirmed that in the case of damage to the body's immune function, it may cause viral infection or activate a potential tumorigenic virus, causing Kaposi sarcoma.
(two) pathogenesis
Most of the tumors are dark red or purple red patches, papules, plaques or nodules. They can also be grayish yellow, the patches are irregular, the boundaries are generally clear, the surface of the plaque is uneven, the thickness is different, similar to multiple nodules. Mutual fusion, surface skin ulcers, with yellow exudate.
There are three types under the microscope: mixed cell, single cell and anaplastic, and can be divided into two types of histological types, hemangiomas and sarcoma, according to the number of vascular components in the tumor and the shape of the spindle cells. Increased vascular distribution, with interstitial edema, inflammatory cell infiltration, and more extravascular red blood cells and hemosiderin deposition, when the lesion progresses, the inflammatory cells decrease, the spindle cell area appears, mixed with the hemangioma-like area, The spindle cell bundle resembles a fibrosarcoma, but there are fissures containing red blood cells. PAS-positive transparent bodies of different sizes can be seen inside and outside the cells. When progressing, small blood vessels gradually occlude, and the nuclei of the spindle cells become large and deeply stained. The number of mitotic figures increases, eventually forming a highly malignant sarcoma.
Under electron microscopy, the tumor is composed of different differentiated tumor cells and tumor blood vessels. The tumor-like endothelial cells have characteristic Weible-Palade bodies, and the tumor-like pericytes are mostly surrounded by basement membranes, containing microfilaments, and even dense bodies. And dense spots.
Tumor vascular endothelial cells, FVIII positive, some spindle cells were also positive, in addition, both CD34 and CD31 were positive.
Prevention
Vulvar Kaposi sarcoma prevention
Do a good job in the tertiary prevention of cancer. Regularly participate in physical examinations, enhance immunity, and find medical conditions in a timely manner.
Complication
Vulvar Kaposi sarcoma complications Complications lower limb lymphedema
Lower limb lymphedema.
Symptom
Vulvar Kaposi sarcoma symptoms Common symptoms Itching edema Osteoporosis Bone destruction Papular polyps Secondary infection Cyst nodules Vulva burning irritations
It can be expressed as a slow-growing block of the vulva with genital itching and burning sensation, or vulvar pain and vaginal discharge. Red, brown or blue patches or papules can be seen during physical examination, and can also be fused into plaques or nodules. It is polypoid or papillary, mostly with surface skin ulceration and associated with lower extremity lymphedema. Hall et al (1979) reported squamous lesions with a slight uplift of the labia majora, satellite nodules with groin, buttocks and thigh skin.
1. Classic type (European type)
Most patients occur in the 50 to 70 years old. Early damage is most common in the distal extremities and the hands and forearms. It gradually develops into the trunk, showing reddish, pale blue, black, red or purple spots or plaques. Large plaques or nodules, nodules are as hard as rubber, local swelling, and later lymphedema can be invented. Later plaques and nodules can appear on the face, ears, trunk and mouth, especially in soft palate. When the condition is slow, new nodules may occur, often bleeding after blood or trauma, and gradually increase, ulcers may occur, and even gangrene may occur, requiring amputation. However, especially in the early stage, remission period may occur, and almost all nodules may occur. Naturally receding, leaving atrophy and scarring, accompanied by itching, burning or pain.
Visceral lesions account for 10%, the gastrointestinal tract is the most common, in addition, heart, lung, liver, adrenal gland and abdominal lymph nodes can also be affected, bone changes are characterized, osteoporosis, cyst formation and even erosion of the cortex, has diagnostic value, and shows The lesions have been extensive, and eyelid and membrane damage can occur in children. The lymphocyte system is invaded, usually with mononuclear cells, followed by eosinophilia, and the mortality rate of this disease is 10% to 20%.
2. African type
The incidence rate in tropical Africa is high, divided into 4 subtypes, and the subtypes of the nodules can be relieved by themselves; the bright red subtype is easy to break and hemorrhage and secondary infection; the infiltrating subtype is deep infiltration, fibrosis, non-depression edema, There are many bone destruction; the subtype of lymphadenopathy is more common in children and young people, the prognosis is very poor, chemotherapy can be relieved, and skin lesions are optional.
3. Kaposi sarcoma of AIDS patients
More common in 25 to 50 years old, rapid progress, high mortality, early red spots, surrounded by pale halo, 1 week later into purple or brown spots, pale halo disappeared, after the formation of nodules or masses, tumors of several millimeters to 1cm, The neck, torso, upper limbs are more common, and the lower limbs are less. Due to serious defects in the patient's cellular immune function, the damage is frequent, spread throughout the body, and symmetrically distributed.
4. Kaposi sarcoma associated with transplantation
When immunosuppressive agents are applied after organ transplantation, the skin lesions are spread throughout the skin, mucous membranes, lymph nodes and internal organs, and the disease progresses rapidly. The skin lesions can be self-healing after the immunosuppressive agents are stopped.
Examine
Examination of vulvar Kaposi's sarcoma
The following checks can be done to rule out other diseases:
First, tumor marker inspection:
Tumor Marker is a chemical substance that reflects the presence of a tumor.
1, classification:
(1) Tumor tissue production, including: differentiation antigen; embryonic antigen (AFP, CEA); isoenzyme (NSE); hormone (HCG); tissue-specific antigen (PSA, freePSA): mucin, glycoprotein, glycolipid (CA125); oncogenes and their products; polyamines and the like.
(2) The tumor is produced by interaction with the host, including: serum ferritin; immune complex; acute phase protein; isoenzyme; interleukin receptor; tumor necrosis factor.
Second, histopathological examination:
1. General:
Most of the tumors are dark red or purple red patches, papules, plaques or nodules, and may also be grayish yellow. The patches are irregular and the boundaries are generally clear.
The surface of the plaque is uneven and uneven, similar to multiple nodules. Surface skin ulcers with yellow exudate.
2, under the microscope:
There are three types, mixed cell, single cell and anaplastic, and can be divided into two histological types, hemangiomas and sarcoma according to the number of vascular components in the tumor and the shape of spindle cells.
Increased vascular distribution in the early dermis, with interstitial edema, inflammatory cell infiltration, and more extravascular red blood cells and hemosiderin deposition. As the lesion progresses, the inflammatory cells decrease, and the spindle cell area appears, which is intermingled with the angiotum-like area. The spindle cell bundle resembles a fibrosarcoma, but there are fissures containing red blood cells, and PAS-positive transparent bodies of varying sizes can be seen inside and outside the cell.
Further progress, small blood vessels gradually occluded, the nucleus of spindle cells became larger, deep stained, and mitotic figures increased, eventually forming a highly malignant sarcoma.
Diagnosis
Diagnosis and diagnosis of vulvar Kaposi sarcoma
Diagnosis should be combined with clinical and histopathological examination. Early damage such as color, typical site of the disease, and slower increase, the diagnosis is easier, but it is more difficult to start edema.
The shape of the tumor is variable and can be mistaken for purulent granuloma, but the latter lacks the spindle-shaped cell component, and is also differentiated from bacillary angiomatosis and other sarcomas. In addition, it is necessary to pay attention to the pseudo-Kaposi formed by arteriovenous fistula. The difference in sarcoma, although the histology also shows capillary, fibroblast proliferation and red blood cell exudation, but no vascular fissures and atypical spindle cells, and can be expressed as elevated skin temperature, hairy, sweating and Hearing continuous vascular murmurs or tremors in the affected area.
