Vulvar malignant schwannoma

　　1. Etiology

　　Some scholars believe that the onset of vulvar malignant schwannoma is related to the heterozygous deletion of chromosome 17. The mutation or loss of the tumor suppressor gene p53 and NFl gene, and the increased activity of the ras gene lead to the occurrence of the disease. Thomas et al. proposed that if the fetus is exposed to a high concentration of nerve growth factor in the uterus, it will worsen the course of the disease. In addition, β-estradiol can promote the development of the disease.

　　2. Pathogenesis

　　Microscopic examination shows spiral or tuft-like arrangement of fusiform cells, abundant cytoplasm, large and deeply stained nuclei, twisted into wavy patterns. The atypia is obvious, and the nuclear division is frequent. The cell distribution is dense and sparse areas interlaced. Some (Lee 1997) may not have fusiform cells, only

　　Immunohistochemical examination shows most to be S-100 positive, some can be NSE, MBP positive.

　　Accompanied by systemic multiple neurofibromatosis

　　Fibromatosis is a tumor derived from fibrous tissue, with an incidence rate of 1.37% of benign soft tissue tumors. Tumors can occur in any part of the body's large muscles, with the aponeurosis of the rectus abdominis and its adjacent muscles on the abdominal wall being the most common, and it is more common during pregnancy and in the later stages of pregnancy. Those located outside the abdominal wall are more common in males, and they are more common in the scapular, thigh, and gluteal regions. The age of onset is mostly between 30 and 50 years, and children and adolescents are not uncommon. The etiology of this disease is not yet clear and may be related to trauma, hormones, and genetic factors.

　　Vulvar malignant schwannoma is manifested as gradually growing masses. Rapid growth often accompanied by pain, unable to walk, hard mass with tenderness, size 2.5-12cm, mostly occurring in the large labia, also seen in the small labia, mons pubis, clitoris, and perianal area. Those located in the large labia are often painful due to clothing stimulation, those located in the small labia have sexual pain, and those located around the perianal area are often accompanied by defecation pain, occasionally accompanied by difficulty urinating, decreased appetite, and weight loss.

　　Before treatment, attention should be paid to the hygiene of the vulva, and it is forbidden to engage in unclean sexual intercourse. Maintain good personal hygiene. It is best not to eat certain foods that are taboo for vulvar malignant schwannoma, such as coffee and other stimulating drinks. Avoid spicy and irritating foods such as scallions, garlic, ginger, and cassia. Avoid greasy, fried, moldy, and preserved foods. Avoid stimulants such as rooster and pork head meat. Avoid seafood. Avoid warm foods such as lamb, dog meat, chives, and pepper. Avoid smoking and drinking. It is recommended to eat more foods that have anti-vulvar tumor and leukoplakia effects, such as sesame seeds, almonds, wheat, barley, loofah, and black-bone chicken.

　　The diagnosis of vulvar malignant schwannoma depends not only on clinical manifestations, but also on related auxiliary examinations, such as immunohistochemical detection, p53 tumor suppressor gene detection, and histopathological examination.

　　Firstly, dietetic therapy for vulvar malignant schwannoma:

　　1, Sophora root and egg: 2 eggs, 60 grams of brown sugar, 60 grams of Sophora root. Boil Sophora root to get a concentrated decoction, add beaten eggs and brown sugar, cook until done, eat the egg and drink the soup. Once a day, 6 days as a course. It has the effect of clearing heat and detoxifying, drying dampness and healing sores, which can be used for the prevention and treatment of vulvar ulcer.

　　2, Gentian root and egg: 10 grams of gentian root, 3 eggs, 30 milliliters of honey. Boil gentian root, remove the dregs, beat the eggs into omelette, add honey, take on an empty stomach, once a day, 5 days as a course. It has the effect of clearing heat and removing dampness, healing sores.

　　3, Chinese cabbage and mung bean sprout drink: 1 root of Chinese cabbage, 30 grams of mung bean sprout. Clean the root of the Chinese cabbage and slice, clean the mung bean sprout and put them in the pot, add an appropriate amount of water, boil for 15 minutes, remove the dregs and take the juice, drink as tea, not restricted by time. It has the effect of clearing heat and removing dampness, which can be used for the treatment of vulvar ulcer.

　　4, Rock sugar winter melon soup: 30 grams of winter melon seeds, 30 grams of rock sugar. Clean the winter melon seeds, grind into coarse powder, add rock sugar, pour 1 bowl of boiling water, put into a pot, simmer over low heat. Take twice a day, for several days. It has the effect of clearing heat and promoting diuresis and stopping leukorrhea, which can be used for the treatment of vaginal itching.

　　5, Cang'er stomach: 1 stomach, 30 grams of Cang'erzi, 10 grams of Huaijiu. Clean the stomach, add water and the latter two drugs, discard the medicine and eat the stomach. It has the effect of kidney-nourishing and wind-relieving itching. Used for the prevention and treatment of atrophic vulvar leukoplakia.

　　6, Goji berry congee: 20 grams of goji berries, an appropriate amount of rice. Cook into congee for consumption.

　　Secondly, what is good for the body to eat when suffering from vulvar malignant schwannoma:

　　1, It is advisable to eat more foods with anti-vulvar tumor and leukoplakia effects, such as sesame, almond, wheat, barley, loofah, black-bone chicken, cuttlefish, green mamba, pork pancreas, chrysanthemum, umeboshi, peach, lychee, spurge, chicken blood, eel, abalone, crab, horseshoe crab, sardine, clam, tortoise shell.

　　2, Pain is advisable to eat horseshoe crab, red, lobster, oyster, sea cucumber, tiger fish, beetroot, mung bean, radish, chicken blood.

　　3, Itching is advisable to eat amaranth, cabbage, mustard, taro, kelp, nori, chicken blood, snake meat, pangolin.

　　4, To enhance physical fitness and prevent metastasis, it is advisable to eat silver ear, black fungus, mushroom, truffle, gizzard, sea cucumber, Job's tears, walnut, crab, lizard, needlefish.

　　5, Postoperative vulvar malignant schwannoma surgery: It consumes Qi and injures blood, it is advisable to eat more Qi-nourishing and blood-nourishing foods, such as jujube, longan, mung bean, glutinous rice, lychee, mushroom, carrot, quail egg, lotus root powder, beans, etc.

　　6, Postoperative radiotherapy for vulvar malignant schwannoma: It consumes yin and fluid, it is advisable to eat more yin-nourishing and fluid-nourishing foods, such as spinach, small cabbage, lotus root, radish, watermelon, banana, grape, sea cucumber, sugarcane, lily, etc.

　　7. Chemotherapy after vulvar malignant schwannoma surgery: it may damage both Qi and blood, so it is advisable to eat foods that nourish Qi and blood regularly, such as mushrooms, shiitake mushrooms, walnuts, mulberries, Job's tears congee, jujube, longan, sea cucumber, etc.

　　Third, it is best not to eat the following foods for vulvar malignant schwannoma:

　　1. Avoid stimulants such as coffee.

　　2. Avoid spicy and刺激性 foods such as scallions, garlic, ginger, and cinnamon.

　　3. Avoid greasy, fried, moldy, and preserved foods.

　　4. Avoid irritants such as rooster and pork head meat.

　　5. Avoid seafood.

　　6. Avoid warm foods such as mutton, dog meat, chives, pepper, etc.

　　7. Avoid smoking and alcohol.

　　Western Treatment Methods for Vulvar Malignant Schwannoma

　　1. Surgical Treatment

　　Surgical treatment is the first choice for early-stage patients, including radical vulvectomy, unilateral vulvectomy, radical clitoridectomy, or anterior pelvic exenteration according to the site of onset. If gynecological examination or imaging suggests the possibility of lymph node metastasis, lymph node biopsy can be performed, and routine lymph node resection is not recommended because the tumor is mostly spread by blood.

　　2. Radiotherapy Treatment

　　Lambrou et al. (2002) were unable to perform radical resection due to the large vulvar mass, so preoperative external irradiation was given, and the mass shrank by about 50%, but the effect of radiotherapy is not yet clear, especially for patients with neurofibromatosis who need to be cautious because it may increase the possibility of secondary tumors.

　　3. Chemotherapy Treatment

　　For those who cannot be surgically removed or cannot be completely removed to achieve radical cure, chemotherapy can be given. Chemotherapy has a better effect on metastatic lesions than local recurrent lesions, and common chemotherapy regimens include VAC (vincristine, actinomycin D, and cyclophosphamide), doxorubicin (adriamycin), and ifosfamide. However, some scholars believe that there is no difference in overall survival rate and disease remission survival rate when using doxorubicin (adriamycin) alone or in combination with other drugs. Lambrou et al. suggest that patients with high risk of recurrence, such as those with tumors >5cm, located in deep tissues, highly malignant, etc., should receive postoperative doxorubicin (adriamycin) treatment.