Vulvar prominent fibromyxoma

　　1. Etiology

　　Vulvar dermal fibrosarcoma originates from mesenchymal cells within the dermis with multipotent differentiation potential, and part of the disease is related to local trauma of the vulva, such as vaginal delivery.

　　2. Pathogenesis

　　The tumor presents as a single or multiple skin and subcutaneous nodules, with sizes ranging from 1cm×1cm to 8cm×10cm, and recurrent cases are often multiple. The surface is covered with tight or ulcerated skin, with clear boundaries and no capsule. Recurrent cases commonly show mucus variation and a softer texture, with rare bleeding or necrosis.

　　The microscopic examination is mainly divided into three subtypes: common type, mucus type, and fibrosarcoma variant, with the latter two being more common in recurrent cases. The tumor cells are fusiform, resembling fibroblasts, with a relatively uniform morphology and often arranged in a typical spoke-like pattern around a central point (collagen or small blood vessels). The atypia is mild, with a few mitotic figures visible, no necrosis. The tumor infiltrates diffusely in the dermis and subcutaneous tissue, extending along the intervals between the subcutaneous fat lobules, enveloping the subcutaneous fat, forming a scalloped or honeycomb-like image, or showing a multi-layer parallel arrangement. The spoke-like structure in recurrent cases is not obvious, with an increase in mitotic figures. The fibrosarcoma variant makes the cells denser, arranged in long bundles, with more prominent atypia and a higher number of mitotic figures (average 11/10HPF). If mucus variation occurs, the cells become loose and the blood vessels are exposed. 80% to 100% of the dermal fibrosarcomas express CD34, while most dermal fibrosarcomas are negative for FFXⅢa. The COL1A1-PDGFB chimeric gene formed by the fusion of the COL1A1 gene on chromosome 17 with the PDGFB gene on chromosome 22 due to t(17;22)(q22;q13) is a relatively specific marker for dermal fibrosarcoma. Wang et al. (1999) detected the paraffin sections of dermal fibrosarcoma using RT-PCR and found that about 83% (10/12) of the patients could detect the gene, considering that the COL1A1-PDGFB fusion gene could be used as an auxiliary diagnostic indicator, especially for atypical dermal fibrosarcoma. Vanni et al. (2000) detected the COL1A1-PDGFB chimeric gene in a case of vulvar dermal fibrosarcoma and found that it was a fusion transcript of the 37th exon of the COL1A1 gene and the 2nd exon of the PDGFB gene.

         After several years, it can grow rapidly, fuse into larger irregular masses, and the accelerated growth stage of the mass may be accompanied by local pain, tenderness, and ulcers. Complications: infection at the lesion site.

　　Generally, there are no自觉 symptoms, some have mild or moderate pain, and after slight trauma, it can break down and bleed. The common site is the labia majora, with the left side more common, some can involve the clitoris or mons pubis, mainly表现为gradually invasive growth, involving subcutaneous tissue. Initially, it is usually painless, nodular, solid nodules, which can grow rapidly and fuse into larger irregular masses after several years. The accelerated growth stage of the mass may be accompanied by local pain, tenderness, and ulcers, and the pain may radiate to the inner side of the thigh. Most patients seek medical attention during this period. Tumors detected by physical examination are usually solitary, presenting as elevated, hard masses, which can also be presented as a central mass surrounded by several small nodules, fixed on the surface, covered with skin, and not adherent to the deep muscle and fascia. The nodules are pale red or pale blue purple, and sometimes appear lobular. Recurrence is easy if not completely removed.

　　Note: Regular physical examination, early detection, early and reasonable surgical treatment, and good follow-up. Prognosis:

　　1. The 5-year survival rate of papillary dermal fibrosarcoma is 91% to 100%. The common type of papillary dermal fibrosarcoma is a low-grade malignant tumor, the malignant degree of the fibrosarcoma variant is higher, and the mucinous type has not yet been observed to differ significantly from the other two types.

　　1. Due to the irregular infiltration of the mass into the surrounding tissues, complete resection is difficult, and the local recurrence rate after surgical resection is 20% to 49%. Since 80% of the recurrences occur within 3 years after surgery, it is recommended to have a follow-up examination every 3 to 6 months in the first 3 years after surgery, and then once a year for long-term follow-up.

　　2. Although there are cases of metastasis to the lung or nearby lymph nodes, it is not common and usually occurs in the late stage, often as a result of multiple local recurrences. Systemic metastasis is rare, accounting for 0.3% to 0.5% of papillary dermal fibrosarcoma, and is generally hematogenous metastasis.

　　1. Secretion examination, detecting COL1A1-PDGFB fusion protein, fresh tissue can be used for dopa (DOPA) reaction or S-100 protein immunohistochemical staining.

　　2. Histopathological examination.

　　I. Dietetic recipes for vulvar papillary dermal fibrosarcoma(The following information is for reference only, detailed information should be consulted with a doctor)

　　1. Kuduan Chicken Eggs: 2 eggs, 60 grams of brown sugar, 60 grams of Kuduan. Boil Kuduan to extract the juice, then add the beaten eggs and brown sugar, and cook until done. Eat the eggs and drink the soup. Take once a day, 6 days as a course of treatment. It has the effects of clearing heat and detoxifying, drying dampness and healing sores, and can be used for the prevention and treatment of vulvar ulcers.

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

　　3, Cabbage and Mung Bean Sprout Drink: Cabbage root 1 piece, mung bean sprout 30 grams. Clean the cabbage root and slice, clean the mung bean sprout and put them in a pot, add an appropriate amount of water, boil for 15 minutes, remove the dregs and take the juice, drink as tea, without拘time. It has the effect of clearing heat and eliminating dampness, which can be used for the treatment of vulvar ulcer.

　　4, Rock Candy Winter Melon Soup: Winter melon seed 30 grams, rock candy 30 grams. Clean the winter melon seed, grind into coarse powder, add rock candy, pour a bowl of boiling water, put it in an earthen pot, and simmer over low heat. Taken twice a day, for several days in a row. It has the effect of clearing heat, eliminating dampness, and stopping leukorrhea, which can be used for the treatment of vaginal itching.

　　5, Cang Ear Stomach: Stomach 1 piece, Cang Ear seed 30 grams, Huang Qi 10 grams. Clean the stomach, add water, and boil with the latter two herbs. Discard the herbs and eat the stomach. It has the effect of kidney-reinforcing, wind-dispelling, and itching-relieving. Used for the prevention and treatment of atrophic vulvar leukoplakia.

　　6, Goji Porridge: Goji berries 20 grams, rice an appropriate amount. Cook and eat as porridge.

　　7, HeShu Egg Soup

　　Composition: HeShu 120 grams, eggs 4 pieces.

　　Usage: Boil HeShu to make a concentrated decoction, then boil 4 eggs. This is a daily dose, taken twice a day.

　　8, Sesame Brown Sugar Porridge

　　Composition: Black sesame 200 grams, brown sugar 30 grams.

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

　　9, Walnut and Sesame Porridge

　　Composition: Walnut kernel 200 grams, sesame 100 grams, glutinous rice 100 grams.

　　Usage: Grind the walnut kernel and sesame separately. Cook glutinous rice with an appropriate amount of water, then add walnut kernel and sesame for consumption.

　　10, HeShu-ShanYao-YangRouSoup

　　Composition: HeShu 30 grams,山药100 grams, lamb meat 500 grams, ginger 9 grams.

　　What are the foods that are good for the body for external vulvar prominence fibrosarcoma

　　It is advisable to eat more foods with anti-external vulvar tumor and leukoplakia effects, such as sesame, almond, wheat, barley, loofah, black-bone chicken, cuttlefish, mangshan, pork pancreas, chrysanthemum, black plum, peach, litchi, polygonum aviculare, chicken blood, eel, abalone, crab, horseshoe crab, sardine, clam, tortoise shell.

　　For pain, it is advisable to eat horseshoe crab, red, lobster, sea asparagus, sea cucumber, tiger fish, beetroot, mung bean, radish, chicken blood.

　　For itching, it is advisable to eat amaranth, cabbage, rapeseed, taro, kelp, purple kelp, chicken blood, snake meat, pangolin.

　　To enhance physique and prevent metastasis, it is recommended to eat silver ear, black fungus, mushroom, monkey head fungus, gizzard, sea cucumber, Job's tears, walnut, crab, lizard, needlefish, etc.

　　After the surgery for the external vulvar prominence fibrosarcoma, consumption of Qi and blood injury, it is advisable to eat more Qi and blood-nourishing foods such as jujube, longan, mung bean, glutinous rice, litchi, mushroom, carrot, quail egg, lotus root starch, beans, etc.

　　6. Radiation therapy after vulvar lymphangioma surgery: it consumes Yin and fluid, so it is advisable to eat more Yin-nourishing and fluid-nourishing foods, such as spinach, Chinese cabbage, lotus root, radish, watermelon, banana, grapes, sea cucumber, sugarcane, lily, etc.

　　7. Chemotherapy after vulvar lymphangioma surgery: it can damage both Qi and blood, so it is advisable to often eat foods that nourish Qi and blood, such as mushrooms, walnuts, mulberries, Job's tears congee, red dates, longans, sea cucumber, etc.

　　8. Vitamin A and C have the function of preventing cell malignancy and metastasis and increasing the stability of epithelial cells; vitamin C can also prevent general symptoms of radiation injury and can increase the level of white blood cells; vitamin E can promote cell division and delay cell aging; vitamin B1 can promote the appetite of patients and alleviate the symptoms caused by radiotherapy. Therefore, it is recommended to eat more foods rich in the above vitamins, such as fresh vegetables, fruits, sesame oil, grains, beans, and animal viscera.

　　Third, what foods should not be eaten for vulvar papillary dermal fibrosarcoma

　　1. Avoid stimulants such as coffee.

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

　　3. Avoid smoking and drinking.

　　4. Avoid fatty, fried, moldy, and preserved foods.

　　5. Avoid foods that promote hair growth, such as roosters and geese.

　　6. Avoid astringent and astringent foods, as they are prone to blood stasis and blood stasis, which should be avoided. Acrid and warm dispersing agents are beneficial for circulation, and can be eaten: but not too much, as excessive pungent and spicy stimulation will also worsen pain.

　　First, prevention: regular physical examination, early detection, early reasonable surgery, and follow-up care.

　　1. The timing of surgery is very important in the Western medical treatment of vulvar papillary dermal fibrosarcoma. The design of the first operation in the early stage is reasonable and can achieve radical resection. Vulvar local extensive resection is the first-line treatment. The resection includes 3 to 5 cm of normal skin around the tumor, and deep to the fascia of the pubic symphysis and the urethro-genital diaphragm 1 to 2 cm deep. Skin grafting is required for larger tumors. The effect of radiotherapy is not good, and for recurrent cases, more extensive resection is even more necessary. Frozen section examination during surgery is helpful to determine whether the resection is complete. Some scholars propose using the Mohs micrographic surgery technique, which can reduce local recurrence and preserve normal vulvar tissue, but due to the need for highly accurate and professional surgical techniques, it is still difficult to be widely promoted.

　　2. As lymph node metastasis is rare, there is no need for routine resection. For patients with positive surgical margins, postoperative radiotherapy is recommended, while chemotherapy is not advocated unless there is distant metastasis. For patients with recurrence or multiple recurrences, radical resection and reconstruction are still the first choice. There are reports that vulvar papillary dermal fibrosarcoma patients have a recurrence rate of 1 time every 2 to 4 years within 20 years, all of whom underwent radical resection, and there was no tumor recurrence after the last operation, with a follow-up of 7 years. STI-571 is a relatively specific inhibitor of platelet-derived growth factor receptor tyrosine kinase, and studies have found that oral STI-571 has efficacy in the treatment of locally advanced and metastatic papillary dermal fibrosarcoma in phase I clinical trials, but further research is needed on the application of this drug in papillary dermal fibrosarcoma.

　　Secondly, precautions to be taken before the treatment of vulvar desmoid fibrosarcoma

　　1, Regular physical examinations, early detection, early and reasonable surgical treatment, and follow-up. There are currently no very effective traditional Chinese medicine treatment methods and Chinese herbal medicine for vulvar desmoid fibrosarcoma. The timing of surgical treatment for vulvar desmoid fibrosarcoma is very important in Western medicine, with the early first operation designed reasonably to achieve radical treatment. Local extensive resection is the preferred treatment. The surgical resection includes surrounding 3 to 5 cm of normal skin, deep to 1 to 2 cm of the fascia of the pubic symphysis and the urogenital diaphragm. Skin grafting is required if the tumor is large. Radiotherapy is not effective, and more extensive resection is required for recurrent cases. Frozen section examination during surgery is helpful to determine whether the resection is complete. Some scholars propose using the Mohs micrographic surgery technique, which can reduce local recurrence and preserve normal vulvar tissue, but due to the need for highly accurate and professional surgical skills, it is still difficult to be widely promoted.

　　1, Routine lymph node dissection is not necessary due to the rarity of lymph node metastasis. For patients with positive surgical margins, radiotherapy is administered after surgery, and chemotherapy is not recommended unless there is distant metastasis. For patients with recurrence or multiple recurrences, resection and reconstruction are still the preferred treatment. There are reports that vulvar desmoid fibrosarcoma patients have recurrences once every 2 to 4 years over 20 years, all undergoing radical surgical resection, and surviving without tumor for 7 years after the last resection.

　　2, STI-571 is a relatively specific inhibitor of platelet-derived growth factor receptor tyrosine kinase. Some studies have found that oral STI-571 has efficacy in the treatment of locally advanced and metastatic desmoid fibrosarcoma in phase I clinical trials, but further research is needed on the application of this drug in desmoid fibrosarcoma.