Vulvar Kaposi sarcoma

　　1. Etiology

　　Currently, human herpesvirus-8 (also known as KSHV) is considered closely related to the occurrence of vulvar Kaposi sarcoma. Serological and biochemical studies suggest that the disease is related to cytomegalovirus infection, as it has a high incidence in AIDS patients, supporting the viral theory. Research on the etiology of vulvar Kaposi sarcoma has successively proposed hypotheses such as geographic factors, ethnic factors, climatic factors, gender factors, genetic factors, trauma and occupational factors, and infection factors, but none have been fully confirmed. It is now widely believed that virus infection or activation of latent oncogenic viruses in the case of impaired immune function of the body leads to the occurrence of Kaposi sarcoma.

　　2. Pathogenesis

　　The tumor is mostly dark red or purple red spots, papules, plaques, or nodules, and can also be grayish yellow. The spots are irregularly shaped with roughly clear boundaries. The surface of the plaques is uneven, with varying thickness, resembling the fusion of multiple nodules. The surface skin has ulcers with yellow exudate.

　　Microscopically, it is divided into three types: mixed cellularity, monocellularity, and anaplastic. It can also be divided into two histological types, hemangioma type and sarcoma type, according to the amount of vascular components in the tumor and the morphology of the spindle-shaped cells. In the early stage, increased blood vessel distribution can be seen in the dermis, accompanied by interstitial edema, inflammatory cell infiltration, and a large number of extracellular red blood cells and hemosiderin deposition. As the lesion progresses, inflammatory cells decrease, and spindle-shaped cell areas appear, mixed with vascular tumor-like areas. The spindle-shaped cell bundles resemble fibrosarcoma-like, but there are red blood cell-containing clefts, and PAS-positive clear bodies of different sizes can be seen inside and outside the cells. Further progression leads to the gradual occlusion of small blood vessels, the nuclei of spindle-shaped cells become larger and deeply stained, nuclear division increases, and finally a highly malignant sarcoma-like tumor is formed.

　　Under the electron microscope, the tumor is composed of differentiated tumor cells and tumor vessels. The tumor endothelial cells have characteristic Weible-Palade bodies, and the tumor pericytes are often surrounded by basement membrane, containing microfilaments, and even dense bodies and dense plaques can be seen. The tumor vascular endothelial cells are FⅧ positive, and some spindle-shaped cells are also positive. In addition, CD34 and CD31 are both positive.

　　First, introduction:

　　Lower limb lymphedema is caused by the obstruction of lymphatic fluid return, leading to the accumulation of lymphatic fluid in subcutaneous tissue, resulting in fibrosis, fatty sclerosis, and limb swelling in the later stage. The skin becomes thickened, rough, and hard like elephant hide, hence the name 'elephantiasis'. It can occur in the external genitalia, upper limbs, but it is most common in the lower limbs.

　　Second, clinical manifestations:

　　It is mainly manifested as swelling of one limb, starting from the ankle and extending to the entire lower limb. In the early stage, the skin is still normal, but in the late stage, the skin becomes thickened, dry, rough, and hyperpigmented, with warts or spiny-like projections appearing.

　　It can be divided into degrees according to the severity of lymphedema:

　　1. Mild:

　　The limb edema is凹陷性, and it can diminish or disappear after elevation of the limb. There is no fibrosis-like damage to the skin.

　　2. Moderate:

　　The edema does not indent when pressed, and the limb edema does not significantly subside after elevation. The skin has moderate fibrosis.

　　3. Severe:

　　Elephantiasis-like skin changes may appear.

　　It can manifest as slowly growing masses in the vulva accompanied by vulvar itching and burning sensation, or vulvar pain and vaginal discharge. During physical examination, red, brown, or blue spots or papules may be visible, which can also merge into plaques or nodules, appearing polypoid or papillary. Most of them have surface skin ulceration and are accompanied by lower limb lymphedema. Hall et al. (1979) reported large areas of slightly elevated squamous lesions in the labia majora, accompanied by satellite nodules in the skin of the inguinal, buttock, and thigh.

　　1. Classical type (European type)

　　Patients are more common in the age group of 50 to 70, with the earliest lesions most commonly occurring in the distal lower limbs and hands and forearms, gradually extending to the trunk, presenting as pale red, pale blue-black, purple-red, or purple spots or plaques. Later, they enlarge and coalesce to form large plaques or nodules, which are hard like rubber, locally swollen, and can develop significant lymphedema later. Later, plaques and nodules can appear on the face, ears, trunk, and oral cavity, especially more commonly on the soft palate. In the case of slow progression of the disease, new nodules can appear, often spontaneously bleeding or bleeding after trauma, and gradually increasing in size, which can lead to ulcers, even gangrene, necessitating amputation. However, especially in the early stage, remission periods can also occur, with almost all nodules naturally regressing, leaving atrophy and scars, accompanied by itching, burning, or pain.

　　Intravisceral lesions account for 10%, gastrointestinal tract is the most common, in addition, the heart, lungs, liver, adrenal glands, and abdominal lymph nodes can also be involved. The skeletal changes are characteristic, including osteoporosis, cyst formation, and even erosion of the cortex, which has diagnostic value and indicates that the lesions are widespread. In children, eyelid and conjunctival lesions can occur, with invasion of the lymphocytic system, commonly presenting with monocytosis, followed by eosinophilia. The mortality rate of the disease is 10% to 20%.

　　2. African type

　　Highly prevalent in tropical African regions, divided into 4 subtypes, with the nodular subtype being self-resolving; the bright red subtype is prone to ulceration, bleeding, and secondary infection; the infiltrative subtype presents with deep infiltration, fibrosis, non-pitting edema, and often bone destruction; the lymphadenopathy subtype is more common in children and young adults, with extremely poor prognosis, chemotherapy can alleviate the condition, and skin lesions may or may not be present.

　　3. Kaposi sarcoma in AIDS patients

　　Commonly seen in people aged 25 to 50, with rapid progression and high mortality rate. Initially, it appears as red spots surrounded by pale halos, which turn purple or brown after a week, the pale halo disappears, and then nodules or masses form. The tumor size ranges from a few millimeters to 1 cm, commonly seen in the neck, trunk, and upper limbs, less in the lower limbs. Due to severe defects in the patient's cellular immune function, multiple lesions occur, widespread throughout the body, and symmetrically distributed.

　　4. Kaposi sarcoma related to transplantation

　　Occurring after organ transplantation when immunosuppressants are used, skin lesions are widespread over the entire skin, mucous membranes, lymph nodes, and internal organs are involved, with rapid progression of the disease. The skin lesions can heal spontaneously after discontinuation of immunosuppressants.

　　Prevention of lower limb lymphedema: The prevention and control of mosquito-borne diseases and filariasis are the main measures to prevent lymphedema caused by filarial infection. For lymphangitis caused by hemolytic streptococcal infection, it should be thoroughly treated in the initial attack, with sufficient dosage of antibiotics and appropriate prolongation of the course of treatment. Tinea pedis is a common factor for pathogenic bacteria to invade, and should be actively treated. Regular physical examinations should be attended to enhance immunity and physical fitness, and medical treatment should be sought promptly upon the discovery of the disease.

　　First, tumor marker examination

　　肿瘤标志物(TumorMarker)是反映肿瘤存在的化学类物质。

　　Tumor markers (Tumor Marker) are chemical substances that reflect the presence of tumors.

　　1. The tumor tissue produces, including: differentiation antigens; embryonic antigens (AFP, CEA); isoenzymes (NSE); hormones (HCG); tissue-specific antigens (PSA, freePSA): mucin, glycoprotein, glycolipid (CA125); oncogenes and their products; polyamines, etc.

　　2. The interaction between the tumor and the host produces, including: serum ferritin; immune complexes; acute phase proteins; isoenzymes; interleukin receptors; tumor necrosis factor, etc.

　　Secondly, histopathological examination:

　　1. Grossly, the tumor is mostly dark red or purple-red macules, papules, plaques, or nodules, and can also be grayish yellow. The macules are irregular in shape, with roughly clear boundaries. The surface of the plaques is uneven in height and thickness, similar to multiple nodules merging together. The surface skin has ulcers, accompanied by yellow exudate.

　　2. Microscopically, it is divided into three types: mixed cellularity, monocellularity, and anaplasia. It can also be divided into two histological types, vascular tumor type and sarcoma type, according to the number of vascular components in the tumor and the morphology of the spindle-shaped cells.

　　Dietary taboos for patients with vulvar Kaposi sarcoma

　　Firstly, dietetic recipes for vulvar Kaposi sarcoma

　　1. He Shou Wu and Egg Soup

　　Usage: Boil He Shou Wu to get a concentrated decoction, boil 4 eggs. This is a daily dose, taken twice a day.

　　2. Sesame Brown Sugar Porridge

　　Composition: 200 grams of black sesame, 30 grams of brown sugar.

　　Usage: Clean the black sesame, slightly roast, put it in a bottle for storage or crush it and put it in a bottle. Use 2 tablespoons with an appropriate amount of brown sugar, dip it in steamed bread or use boiling water to serve.

　　3. Walnut and Sesame Porridge

　　Composition: 200 grams of walnut kernel, 100 grams of sesame, 100 grams of glutinous rice.

　　Usage: Grind the walnuts and sesame into powder. Boil the glutinous rice with an appropriate amount of water, then add the walnuts and sesame and it can be eaten.

　　4. He Shou Wu and Yam Lamb Soup

　　Composition: 30 grams of He Shou Wu, 100 grams of yam, 500 grams of lamb meat, 9 grams of ginger.

　　Secondly, what foods are good for vulvar Kaposi sarcoma?

　　1. It is recommended to eat more foods with anti-external genital tumor and leukoplakia effects, such as sesame, almond, wheat, barley, loofah, black-bone chicken, cuttlefish, black mamba, pork pancreas, chrysanthemum, black plum, peach, lychee, purslane, chicken blood, eel, abalone, crab, horseshoe crab, sardine, clam, turtle shell.

　　2. It is recommended to eat horseshoe crab, red, lobster, clam, sea cucumber, tiger fish, beetroot, mung bean, radish, chicken blood for pain.

　　3. It is recommended to eat amaranth, cabbage, rapeseed, taro, kelp, nori, chicken blood, snake meat, pangolin for itching.

　　31. To enhance physical fitness and prevent metastasis, eat silver ear, black fungus, mushrooms, maitake, gizzard, sea cucumber, Job's tears, walnuts, crabs, lizard, needlefish, etc.

　　30. After surgery for perineal Kaposi's sarcoma, consuming Qi and injuring blood, it is advisable to eat more Qi-nourishing and blood-nourishing foods, such as jujube, longan, adzuki beans, glutinous rice, lychee, mushrooms, carrots, quail eggs, lotus root powder, beans, etc.

　　29. Radiotherapy after surgery for perineal Kaposi's sarcoma: consumes Yin and fluid, it is advisable to eat more Yin-nourishing and fluid-nourishing foods, such as spinach, small mustards, lotus root, radish, watermelon, banana, grapes, sea cucumber, sugarcane, lily, etc.

　　28. Chemotherapy after surgery for perineal Kaposi's sarcoma: easy to damage both Qi and blood, it is advisable to often eat foods that nourish Qi and blood, such as mushrooms, walnuts, lotus seeds, sorghum porridge, jujube, longan, sea cucumber, etc.

　　27. Mushrooms Fresh mushrooms 90 grams, fried with a little vegetable oil and a little salt, then cooked into a soup for consumption. It can prevent cancer.

　　26. Mushrooms An appropriate amount of mushrooms, decocted into a soup, cooked, or ground into powder for oral administration.

　　25. Fresh water chestnuts 20-30 fresh water chestnuts, add an appropriate amount of water, simmered over low heat into a thick soup, divided into 2-3 times to take. It has certain efficacy for gastric cancer and cervical cancer.

　　24. Oolong tea Regular consumption of oolong tea has a certain anticancer effect.

　　23. Bee milk Regular consumption of bee milk can enhance human immunity and has anticancer effects.

　　22. Sprouts The chlorophyll in sprouts can prevent colorectal cancer and other cancers.

　　21. Other milk or goat's milk, not only rich in vitamins but also containing certain anticancer substances; fresh vegetables such as radishes, cabbage, pumpkins, peas, lettuce, etc., have a certain effect on neutralizing nitrosamines in food; carrots, spinach, tomatoes, seaweed, etc., are rich in vitamin A and have a certain anticancer effect.

　　Thirdly, what foods should be avoided for perineal Kaposi's sarcoma

　　19. Avoid stimulant beverages such as coffee.

　　18. Avoid spicy and irritating foods such as scallions, garlic, ginger, and cassia bark.

　　17. Avoid smoking and drinking.

　　16. Avoid greasy, fried, moldy, and salted foods.

　　15. Avoid birds such as roosters and geese.

　　14. Avoid seafood and irritant, sensitizing foods when itching is severe.

　　Precautions before the treatment of perineal Kaposi's sarcoma

　　Firstly, prevention

　　Carry out the third-level prevention of tumors.

　　Secondly, Western medical treatment methods for perineal Kaposi's sarcoma

　　1. Surgical treatment:

　　Early small lesions can be surgically removed, and surgery is limited to local biopsy or resection of solitary nodules. For infiltration and recurrence, a combination of surgery, radiotherapy, chemotherapy, and immunotherapy can be used.

　　2. Other treatments:

　　Local masses can be treated with radiotherapy, laser resection, cryotherapy, and intratumoral injection of chemotherapy drugs such as vinblastine sulfate (vincristine).

　　3. Radiotherapy:

　　Kaposi's sarcoma is a malignant tumor that is relatively sensitive to radiotherapy. Radiotherapy has a good therapeutic effect on isolated patches and plaques, but its therapeutic effect on larger confluent and edematous lesions is poor. Macasaet et al. (1995) reported that 2Gy of cobalt external irradiation was administered three times a week for a total of 4 weeks to treat perineal Kaposi's sarcoma. However, due to the patient's refusal to continue treatment in the second week, the efficacy could not be observed.

　　4, Chemotherapy:

　　Chemotherapy drugs include actinomycin D, dacarbazine (lomustine), carmustine (carmustine), bleomycin, doxorubicin (adriamycin), etoposide (etoposide), sulfate vincristine (vinblastine), vincristine, etc., which can be administered intravenously alone or in combination, with a response rate of 50% to 88%. The combination of actinomycin D, vincristine, and imidazolecarboxamide is relatively optimal. Vincristine (0.1mg/ml) is administered by intramuscular injection every 2 weeks, not more than 3ml per time, which may have a temporary effect. The cause of AIDS is mainly for AIDS treatment.

　　Other combined chemotherapy regimens include bleomycin, doxorubicin (adriamycin), and vincristine; bleomycin and sulfate vincristine (vinblastine). Currently, pegylated liposomal anthracycline antibiotics are recommended for the treatment of Kaposi's sarcoma to increase the local drug concentration of the tumor, while reducing toxic and side effects. Evans et al. reported that low-dose etoposide (topoisomerase I inhibitor) 50mg/d is effective in 36.1% of patients with recurrence or progression after combination chemotherapy with paclitaxel or liposomal doxorubicin or without etoposide (topoisomerase I inhibitor), and it is considered safe and effective to take low-dose etoposide (topoisomerase I inhibitor) orally.

　　5, Immunotherapy:

　　It mainly includes recombinant interferon α, granulocyte-macrophage colony-stimulating factor, white blood cell immunomodulators, and biological response modifiers interleukin-2 (IL-2).

　　6, Antiviral Treatment:

　　There are zidovudine (azidothymidine), didanosine (ddI), and ddC, etc. By inhibiting the activity of the reverse transcriptase of the HIV virus and blocking the replication of the virus in the body, the condition of immune deficiency can be improved.

　　7, Other:

　　Antivascular Formation Agents TNP-470, IM-862, etc., hormonal drugs such as chorionic gonadotropin (HCG), retinoic acid, etc., have entered phase I/II clinical trials. In addition, matrix metalloproteinase inhibitors such as Col-3, TIMPs, IL-12, and inhibitors of vascular endothelial growth factors such as PTK787-ZK, antisense vascular endothelial growth factor are under further research.