Malignant rhabdoid tumor of the vulva

　　1, Etiology

　　The histogenesis of malignant rhabdoid tumor of the vulva has been proposed to be from rhabdomyoblasts, neuroectoderm, histiocytes, epithelial cells, melanocytes, and mesenchymal cells, but no consensus has been reached, and further research is still ongoing.

　　2, Pathogenesis

　　The tumor is a round or irregular mass, with a rough surface and no capsule. The section is grayish-brown or brownish, with scattered hemorrhagic foci visible.

　　Under light microscopy, it shows a highly malignant sarcoma image, tumor cells are arranged in a diffuse or irregular nest, the characteristic feature is polygonal cells with rich eosinophilic cytoplasm, vesicular nuclei, prominent nucleoli, and呈“owl's eye”like. Some nuclei are eccentric, most of the cytoplasm contains transparent, faint eosinophilic spherical inclusions, PAS positive. Under electron microscopy, the inclusions are composed of intermediate filaments or microfilaments arranged in a whirlpool or concentric circular pattern.

　　Malignant rhabdoid tumor of the vulva was first reported in 1978 as 'poor prognosis rhabdomyosarcoma-like Wilm's tumor', although morphologically similar to rhabdomyoblasts, electron microscopy and immunohistochemistry did not find evidence of rhabdomyoblastic differentiation. The most common complication of malignant rhabdoid tumor of the vulva is metastasis, with the lung being the most common site, followed by retroperitoneal lymph nodes and liver.

　　Malignant rhabdoid tumor of the vulva mostly presents as painless subcutaneous nodules, active, recently increased in size, 2-6 cm in size, may be accompanied by tenderness, the course of the disease varies from several months to 1 year, most of which are located on the labia majora, and fewer on the mons pubis.

　　1. Prognosis: The prognosis is extremely poor. Patients generally die within 1 to 8 months after distant metastasis. Among the 8 patients followed up, 4 months to 11 years of survival were observed, with 2 cases surviving more than 5 years, and the median survival time was 9 months.

　　2. Epidemiology: Malignant striated muscle-like tumor of the vulva is rarely seen. The age of onset is between 19 to 49 years, with a median age of 39 years. The tumor is common in the kidneys of infants and children, while extrarenal malignant striated muscle-like tumors are rare.

　　3. Do a good job of the three-level prevention of tumors.

　　1. Tumor marker examination, immunohistochemical detection, and histopathological examination.

　　2. Routine gynecological examination of the vulva is a preliminary routine examination of the vulva, used to provide a basis for further diagnosis, and can also prevent and treat vaginal diseases in a timely manner. Normal vulva, pubic hair is downward-pointing, triangular distribution, the labia majora is pigmented, the labia minora is faintly red, the perineal area has no ulcers, dermatitis, skin tags, or hypopigmentation, and the clitoris length

　　Firstly, dietary therapy for malignant striated muscle-like tumor of the vulva

　　1. Sophora Root and Egg: 2 eggs, 60 grams of brown sugar, 60 grams of sophora root. Boil sophora root to make a strong decoction, add beaten eggs and brown sugar, and cook until done. Eat the egg and drink the soup. Take one dose daily, for 6 days as one course. It has the effect of clearing heat, detoxifying, drying dampness, and healing sores, which can be used for the prevention and treatment of vulvar ulcers.

　　2. Gentian Root and Egg: 10 grams of gentian root, 3 eggs, 30 milliliters of honey. Boil gentian root, remove the residue, beat the eggs into an omelet, add honey, and eat on an empty stomach. Take one dose daily, for 5 days as one course. It has the effect of clearing heat, removing dampness, and healing sores.

　　3. Chinese Cabbage and Mung Bean Sprout Drink: 1 root of Chinese cabbage, 30 grams of mung bean sprouts. Clean and slice the root of the Chinese cabbage, and clean the mung bean sprouts. Place them in a pot with water, boil for 15 minutes, remove the residue, and drink the juice as tea, at any time. It has the effect of clearing heat and removing dampness, which can be used for the treatment of vulvar ulcers.

　　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 a bowl of boiling water, place in an earthen pot, and simmer over low heat. Take two doses daily, for several days in a row. It has the effect of clearing heat, removing dampness, and stopping leukorrhea, which can be used for the treatment of vaginal itching.

　　5. Sow Thistle Stomach: 1 stomach, 30 grams of sow thistle seed, 10 grams of bone-setting herb. Clean the stomach, add water, and boil with the last two herbs. Discard the herbs and eat the stomach. It has the effect of tonifying the kidney, removing wind, and stopping itching. It is 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 and consume.

　　Secondly, what is good for the body to eat when suffering from malignant striated muscle-like tumor of the vulva

　　1. It is recommended to eat more foods that have an anti-external genital tumor effect, such as sesame, almond, wheat, barley, loofah, black-bone chicken, cuttlefish, grass snake, pork pancreas, chrysanthemum, umeboshi, peach, lychee, portulaca, chicken blood, eel, abalone, crab, horseshoe crab, sardine, clam, and tortoise shell.

　　2. For pain, it is recommended to eat horseshoe crab, red shrimp, lobster, clam, sea cucumber, tiger fish, beetroot, mung bean, radish, and chicken blood.

　　3. For itching, it is recommended to eat amaranth, Chinese cabbage, rapeseed, taro, kelp, seaweed, chicken blood, snake meat, pangolin.

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

　　5. After the surgery of vulvar malignant rhabdomyosarcoma, it consumes Qi and injures blood, so it is advisable to eat more Qi and blood-nourishing products such as jujube, longan, adzuki bean, glutinous rice, lychee, mushroom, carrot, quail egg, lotus root starch, beans, etc.

　　6. After the surgery of vulvar malignant rhabdomyosarcoma, radiotherapy consumes Yin and damages fluid, so it is advisable to eat more nourishing Yin and fluid products such as spinach, minor vegetables, lotus root, radish, watermelon, banana, grape, sea cucumber, sugarcane, lily, etc.

　　Thirdly, what foods should not be eaten after the surgery of vulvar malignant rhabdomyosarcoma

　　1. Avoid smoking, alcohol, and spicy刺激性 food.

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

　　3. Avoid stimulants such as rooster and goose.

　　4. Avoid seafood and stimulating, allergic foods when itching is severe.

　　5. Avoid warm foods such as mutton, chive, ginger, pepper, cinnamon, etc. when there is ulceration and bleeding.

　　It is necessary to have a detailed understanding of the symptoms of the disease and related contraindications.

　　There are currently no very effective traditional Chinese medicine treatment methods and herbal medicines for the treatment of vulvar malignant rhabdomyosarcoma. The western medical treatment methods for vulvar malignant rhabdomyosarcoma believe that Brand et al. (2001) consider that initial surgical resection may provide the best opportunity for cure, and suggest that extensive local resection or unilateral vulvar resection be the first choice, and adjuvant radiotherapy can be performed for patients with positive surgical margins to improve local control rates. For patients with local recurrence, repeated extensive local resection and lymph node dissection is still the first choice, while adjuvant chemotherapy and radiotherapy are not very effective.

　　This tumor is prone to local recurrence and distant metastasis. Among the 5 patients who underwent local resection, 3 recurred within 2 months, 1 recurred at 6 years old, and 1 had no recurrence after only 8 months of follow-up. Among the 3 patients who underwent unilateral vulvar resection and inguinal lymph node dissection, local recurrence or lung metastasis occurred 1, 3, and 26 months after surgery, respectively. Among the patients with recurrence, 5 underwent repeated extensive local resection and lymph node dissection, and 1 recurred again after 10 months, undergoing extensive local resection and radiotherapy for the third time. The patient was followed up for 61 months after the third operation, and there was no tumor-free survival.