Vaginal smooth muscle sarcoma

　　Pathogenesis

　　The tumor is a solid nodular mass, with sizes ranging from 1 to 15 cm, usually 4-6 cm, covered with mucosa on the surface, without a capsule or partially with a pseudocapsule, and shows infiltrative growth. The cross-section is grayish yellow or light red, with visible hemorrhage, necrosis, or softening resembling brain matter.

　　Microscopic examination shows the vaginal smooth muscle sarcoma cells arranged in a woven pattern, with three types visible: round cells, spindle cells, and mixed types, among which spindle cell sarcoma is the most common. The cells are round or spindle-shaped, with large nuclei, abundant and deeply stained chromatin, and occasionally巨细胞. More than 5 nuclei are observed in every 10 high-power fields (HPF). A smooth muscle tumor with fewer than 5 nuclei per 10 HPF is called cellular leiomyoma. Since the distinction between benign and malignant vaginal smooth muscle tumors is based on morphology, it is necessary to carefully assess the number of nuclear divisions, with at least 10-15 sections made from different planes, and 50 high-power fields observed at the most active nuclear division site, with the average calculated. In addition, it is also necessary to observe the changes in tumor cell atypia.

　　Electron microscopy shows longitudinal arrangement of intermediate filaments, dense spots, many pinocytic vesicles, and partial or complete basal membrane surrounding each cell.

　　Masson staining shows red myofibrils in the cytoplasm, and PTAH staining shows purple. The smooth muscle-specific actin (MSA) and vimentin are all positive. Most desmin (desmin), estrogen receptor (SR), and progesterone receptor (PR) are positive, and it can also be positive for calponin (calponinhl) and calcium-binding protein (hcaldesmin).

　　It often complications with uremia and cachexia. Uremia refers to chronic renal failure, which is a clinical syndrome composed of a series of symptoms and metabolic disorders caused by progressive and irreversible deterioration of renal function due to various kidney diseases, leading to the loss of renal function. The terminal stage of chronic renal failure is commonly known as uremia. Uremia is not an independent disease but a common clinical syndrome of various late-stage kidney diseases. It is a syndrome composed of a series of clinical manifestations that occur when chronic renal insufficiency enters the terminal stage. Cachexia (cachexia) comes from the Greek words 'kakos' and 'hexis', which literally means 'a bad condition'. It can be seen in various diseases, including tumors, AIDS, severe trauma, postoperative conditions, malabsorption, and severe sepsis, among which cachexia associated with tumors is the most common, known as tumor cachexia. It is a syndrome in which the tumor changes the metabolism of the body through various pathways, causing the body to be unable to absorb nutrients from the outside. The tumor takes nutrients from the body's own fat and protein to build itself, so the body loses a large amount of nutrients, especially essential amino acids and vitamins (formed by the decomposition of fat and protein). The oxidation process in the body is weakened, incomplete oxidation products accumulate, and nutrients cannot be fully utilized, causing a state mainly characterized by wasteful metabolism. There is insufficient heat, leading to anorexia, and only a small amount of food can be eaten or it is impossible to eat at all. The patient becomes extremely thin, with only bones left, resembling a skeleton, anemia, weakness, completely bedridden, unable to take care of oneself, extremely painful, and systemic failure.

　　1. Symptoms

　　Early stage is characterized by small submucosal hard nodules, usually asymptomatic. Some patients seek medical attention because they have felt the tumor themselves. The tumor exhibits diffuse infiltrative growth, usually presenting with vaginal pain, vaginal obstruction and a feeling of weight, discomfort due to pressure. Some may experience irregular vaginal bleeding or bleeding during sexual intercourse, increased leukorrhea, purulent or bloody vaginal discharge, and may have an unpleasant odor. In the late stage, there may be massive vaginal bleeding, and due to compression, infiltration of the bladder, urethra, and rectum, symptoms such as frequent urination, urgency, interruption of urination, difficulty in defecation or constipation may occur. About half of the cases have vaginal-rectal pain.

　　2. Signs and Symptoms

　　The common locations of the tumor are the upper segment of the posterior vaginal wall, followed by the lower segment of the posterior vaginal wall, and other walls can also appear. The mass often grows locally, may have a pseudocapsule, and vaginal examination can palpate hard nodular masses or infiltrative hard masses, which may be accompanied by bleeding, ulceration, hardening of the vaginal wall, narrowing, and in the late stage, lymphatic and hematogenous metastasis and distant organ metastasis to the lungs, liver, and other organs may occur.

　　Vaginal tumors can be divided into benign tumors and malignant tumors. Benign vaginal tumors include fibroma, leiomyoma, hemangioma, lipoma, neurinoma, mucinous tumor, and papilloma, which are quite consistent with their histological source names. Benign vaginal tumors generally do not produce obvious symptoms. Malignant vaginal tumors refer to lesions occurring in the vaginal wall tissue. There are primary and secondary types. Secondary cases are often metastatic from cervical cancer, vulvar cancer, endometrial cancer, rectal cancer, etc.; primary vaginal malignant tumors are less common, accounting for about 2% of gynecological malignant tumors.

　　Prognosis

　　Vaginal smooth muscle sarcoma is rare, and there is no large amount of 5-year survival rate data. Generally, the prognosis is poor, and there is still hope for long-term survival after extensive resection in early cases. If the pathological tissue examination shows active mitosis, more than 50 mitotic figures per 10 high-power fields, the recurrence rate is high, and the prognosis is poor. The survival time after radical treatment for vaginal smooth muscle sarcoma is 10-59 months, with an average of 24.9 months, and tumor-bearing or tumor-free survival for 8 years. The worst case is reported by Brown et al. (1960), where the patient died 1 month after diagnosis, and the best case is reported by Horn et al. (1998), where 6 cycles of doxorubicin chemotherapy were given after radical hysterectomy, vaginectomy, and vulvectomy, with tumor-free survival followed up for 15 years.

　　Factors affecting prognosis include:Risk factors for prognosis include: age, histological grade, FIGO stage, and treatment method. Age > 50 years, histological grade 3, FIGO stage III/IV, and first-line chemotherapy or radiotherapy are associated with poor prognosis. Age > 50 years, histological grade 3, FIGO stage III/IV, and first-line chemotherapy or radiotherapy are associated with poor prognosis.

　　1. Laboratory examination
　　Secretion examination, tumor marker examination, and histopathological examination.
　　2. Other auxiliary examinations
　　Vaginal colposcopy
　　Diagnosis is made based on clinical manifestations and pathological tissue examination. If the tumor invades the vaginal mucosa or has grown into the vagina, tissue can be taken for pathological examination. If the surface of the vaginal mucosa is still smooth, puncture biopsy or incisional biopsy can be performed to determine the diagnosis. Tasavoli et al. proposed the diagnostic criteria for vaginal smooth muscle sarcoma as: tumor diameter ≥ 3 cm, mitotic figures ≥ 10/10HPF; if mitotic figures are 5-10/10HPF, cells have moderate to severe atypicality.

　　Nutritional therapy for vaginal smooth muscle sarcoma

　　1. Astragalus and Poria cocos porridge

　　Ingredients: 30 grams of Astragalus membranaceus, 50 grams of Poria cocos, and 20 grams of glutinous rice.

　　Preparation: First, decoct Astragalus membranaceus and Poria cocos in water and add the washed glutinous rice to cook porridge. It has the effects of invigorating the Qi, benefiting the dampness, and detoxifying, and is used for patients with vaginal malignant tumors that have not healed after ulceration.

　　2. Job's tears and lotus seed porridge

　　Ingredients: 50 grams of Job's tears, 20 grams of lotus seeds, and red dates

　　Preparation: Boil lotus seeds until soft, add red dates and glutinous rice powder, and simmer for 15 minutes. Take two doses in the morning and evening, which has the effects of boosting Qi and nourishing blood, strengthening the spleen and removing dampness, and enhancing the body and preventing cancer.

　　3. Chestnut and yellow croaker

　　Ingredients: 2 yellow croakers, 10 chestnuts, scallions, ginger, garlic, cooking wine, soy sauce, salt, oil, and monosodium glutamate as needed.

　　Preparation: Remove the shell and wash the chestnuts. Heat oil in a wok, fry the fish on both sides until golden, then remove and set aside. Stir-fry scallion strips, ginger strips, and garlic slices, then add clear soup, chestnuts, salt, soy sauce, and bring the fish to a boil in the soup. Skim off the foam, cook until tender, and add monosodium glutamate before serving.

　　Benefits: Strengthen the spleen and stomach, activate blood circulation and reduce swelling. Yellow croaker is neutral and sweet in taste, promotes diuresis and reduces swelling, chestnuts are warm and sweet in taste, nourish the stomach and spleen, and activate blood circulation and stop bleeding.

　　4. Braised winter melon

　　Ingredients: 300g of winter melon, 12ml of cooking oil, 15ml of soy sauce, 30g of cornstarch, 9g of salt, 6ml of sesame oil, scallion strips, ginger juice, and garlic as needed.

　　Preparation: Peel the winter melon, remove the seeds and flesh, cut it into pieces, and boil it in a pot for 5 minutes, then remove and drain it. Mix soy sauce, salt, scallion strips, garlic slices, ginger juice, and cornstarch with warm water to make a sauce. (3) Heat oil in a pot over high heat, stir-fry the seasonings evenly, add the winter melon, stir-fry evenly, add sesame oil, and flip it over.

　　Benefits: Clear heat and promote diuresis. Winter melon is slightly cool and sweet and tasteless, combined with a little spicy ginger, scallions, and garlic, it has the effects of clearing heat and producing body fluid, detoxifying and promoting diuresis, lowering blood pressure, and lowering blood sugar.

　　5. Quick-fried cauliflower

　　Main ingredients: 150g of cauliflower, 15g of carrots, 15g of cucumber, 15ml of soybean oil, fine salt, monosodium glutamate, Sichuan pepper seeds, and sesame oil as needed.

　　Preparation: Break the cauliflower into small pieces, cut carrots into diamond-shaped slices, and blanch both in boiling water until just cooked, then cool them with cold water and drain. Slice the cucumber and place it in the dish. Drizzle with fried Sichuan pepper oil, let it sit for a while, then add fine salt, monosodium glutamate, and sesame oil, and mix well.

　　Benefits: Boost Qi and strengthen the spleen. Cauliflower is sweet and neutral in taste, nourishes the middle and boosts Qi, cucumber is sweet and cold in taste, and carrots are neutral and sweet, which have the effects of strengthening the spleen and stomach, and boosting Qi and blood.

　　6. Stir-fried chicken cubes with water chestnuts

　　Main ingredients: 1 young chicken, 10 fresh water chestnuts, 5g of mushrooms, and an appropriate amount of scallions, ginger, soy sauce, and other seasonings.

　　Preparation: Kill the chicken, remove the feathers, and cut the breast meat into cubes. Wash the fresh water chestnuts, peel them, and slice them. Wash the mushrooms and boil them in water until cooked, then remove them. Heat a little sesame oil in a pot, stir-fry the chicken cubes, then add water chestnuts, mushrooms, and seasonings to stir-fry together until the meat is cooked.

　　Benefits: Boost Qi and produce body fluid, nourish Yin and clear heat. Chicken is rich in nutrition and contains effective anti-cancer components. Water chestnuts are cool and sweet in taste, clear heat and quench thirst, nourish Yin and prevent cancer. Mushrooms have a strong anti-cancer effect.

　　7. Steamed mandarin fish with lingzhi

　　Ingredients: 15g of lingzhi, 1 mandarin fish, and an appropriate amount of scallions and ginger.

　　Preparation: Grind the lingzhi into powder, then make several incisions on the cleaned mandarin fish, and cut the ginger and scallions into thin strips for later use. Spread the lingzhi powder evenly on the mandarin fish and inside the fish belly, and cover the fish with ginger and scallion strips. Place the prepared mandarin fish in a steamer and steam over high heat for 10 minutes. Remove the fish from the steamer, then add some soy sauce to the juice that the fish has released, and add a little salt according to your taste. Mix well and pour it over the fish.

　　Function: Fuling has the effect of invigorating the spleen, which can help patients quickly recover the digestive and absorptive function of the gastrointestinal tract, to assist in comprehensive recovery. The mandarin fish has a mild nature, tonifies the spleen and stomach, and when combined with Fuling, it also reflects the principle of 'clearing and nourishing' in traditional Chinese medicine, which is not easy to cause heat.

　　1. Differentiation and selection of prescriptions

　　1. Liver meridian damp-heat

　　Treatment method: clearing heat and promoting diuresis.

　　Prescription: Longdan Xiegan Decoction with modification, Longdan Cao 30g, Chaihu 15g, Zhizi 15g, Huangqin 15g, Shengdi 15g, Gancao 10g, Zexie 15g, Mutong 10g, Cheqianzi 10g, Danggui 15g, Banzhilian 15g, Baisao 15g, Fuling 10g, Yimi 10g. Add Suanjiang for dark tongue; add Chishao and Yinchen for bitter mouth and heart distress.

　　2. Qi stasis and blood stasis

　　Treatment method: regulating qi and activating blood, resolving blood stasis and dispersing masses.

　　Prescription: Guizhi茯苓 Wan with modification. Guizhi 30g,茯苓10g, Chishao 15g, Taoren 15g, Danpi 15g, Danggui 20g, Chuanxiong 15g, Yizhu 20g, Jixueteng 15g, Suanjiang, Yuanhu, Chuanlianzi, Houpu, Ganqi, Longkui each 10g, Huangqi, Jixueteng, Tuifuling, Shenglongmu each 15g, for those with severe pain, add Ru Xiang and Mo Yao.

　　3. Damp-heat toxicosis

　　Treatment method: clearing heat and promoting diuresis, detoxifying and dispersing nodules.

　　Prescription: Wuwei Xiaoyao Decoction with modification. Shuanghua 20g, Lianqiao, Zihua Ding, Yejuhua each 15g, Tiankui 10g, Tuifuling 10g, Qumai 15g, Banzhilian 15g, Danggui 15g, Chuanxiong 15g, Ru Xiang 10g, Mo Yao 10g, Baihua She Tongcao 15g, Yuxingcao 15g.

　　4. Liver and kidney yang deficiency

　　Treatment method: tonifying the kidney, detoxifying and reducing swelling.

　　Prescription: Zhipei Dihuang Wan with modification, Zhimu 20g, Huang Bai 20g, Shudi 25g,茯苓15g, Shanyao 25g, Shanyao 50g, Zexie 15g, Danpi 20g, Caoheche 15g, Dazai and Xiaozai each 10g, Banzhilian 20g, Gancao 10g.

　　2. Special prescription

　　1. External washing formula

　　(1) Sanpin Formula: Bai Pi 45g, Mingfan 60g, Xionghuang 7.2g, Mo Yao 3.6g. The fine powder is mixed to make Sanpin cakes the size of a 5 fen coin (thickness 2mm, weight 0.2g), disinfected with ultraviolet light and reserved. The patient lies on the gynecological examination bed, the vagina is disinfected, and first use Vaseline oil gauze to protect the unbroken parts of the vagina and fornix. Apply the Sanpin cake on the tumor, after 5-7 days, necrotic tissue falls off, wait for the Sanpin cake to absorb, and change the Chinese medicine 'Shuangzi powder' (Zicao, Zihua Ding, Caoheche, Huang Bai, Cao Liancao each 30g, a little Bingpian as fine powder, high-pressure disinfected) before the tissue falls off; medication should be taken from 5-7 days after menstruation to 5 days before menstruation; this method has the effects of disinfection, anticancer, reducing swelling, and removing blood stasis. Contraindicated for those with severe liver and kidney dysfunction.

　　(2) Huangwu San No.1: Huang Bai 64%, Qingfen 13%, Wugong 7%, Bingpian 3%, She Xiang 0.7%, Xionghuang 12.3%. The powder is ground and sieved through a 100-mesh sieve, mixed evenly and reserved. Use sterile tail thread cotton balls, dip about 1g of medicine powder with a long forceps and send it into the vaginal lesion; mild cases once a week, severe cases 2-3 times a week. Avoid sexual life during treatment.

　　2. Internal treatment formula

　　(1) Yicai tablet: Raw Strychnos nux-vomica, Tuber trigonum, and Rhizoma fritillaria thunbergii each 5 jin, licorice 1 jin. Remove the skin of Strychnos nux-vomica, fry with sesame oil until crisp, mix with the other three herbs into a fine powder, add starch to make tablets, each tablet 0.3g, take 3 times a day; start with 3 tablets each time, increase to 5 tablets if there are no adverse reactions, without a specific course, continue taking medication. Most patients experience increased physical strength and appetite; symptoms such as tightness in the hands and feet may occur with excessive or prolonged medication, which will disappear after stopping the medication. This formula has the effects of detoxifying, reducing swelling, and removing blood stasis.

　　(2) Pinellia ternata formula: Appropriate amount of Pinellia ternata, made into oral tablets; take 3 times a day, with a total dose of about 60g of crude drug. It has a significant effect of clearing heat, detoxifying, and anti-cancer.

　　3. Formulas for Preventing and Treating the Toxic and Side Effects of Radiotherapy and Chemotherapy

　　After chemotherapy and radiotherapy for malignant tumors, a series of toxic and side effects often occur; such as local pain, adhesion, tissue necrosis, decreased blood cells and platelets, dysfunction of digestive function, liver and kidney damage, and nervous system damage. The application of traditional Chinese medicine formulas for the prevention and treatment of these toxic and side effects has a good effect. The following formulas can be selected.

　　(1) Shenqi Fangdu decoction: Astragalus membranaceus and Codonopsis pilosula each 30g, Schisandra chinensis, Cynanchum bungei, fried Bupleurum chinense each 15g, Ophiopogon japonicus 20g, Angelica sinensis, Poria cocos, Citrus reticulata, and raw Pinellia ternata each 12g. Decoct and take one dose daily, divided into 2-3 servings. Start taking 3 days before chemotherapy. This product has the functions of reinforcing the body and anti-cancer, nourishing Yin and blood, promoting Qi and drying dampness, harmonizing the spleen and stomach to stop vomiting, and removing blood stasis and dissipating masses. It can prevent the toxic and side effects of chemotherapy and ensure the smooth completion of chemotherapy.

　　(2) Yangxue Shengbai Drink: Codonopsis pilosula, Astragalus membranaceus, Angelica sinensis, Rehmannia glutinosa, Schisandra chinensis, Spatholobus suberectus, Smilax glabra each 15g, Bupleurum chinense 10g, Cynanchum bungei 6g, Coptis chinensis 6g, and 9g of fried Bupleurum chinense and fried Poria cocos. Add raw Rehmannia and Osteodermia each 15g for Yin deficiency; add Morinda officinalis and Curculigo orchioides each 12g for Yang deficiency; add Ziziphus jujuba and Cuscuta chinensis each 12g for insufficient heart and blood; add Phellodendron amurense and Alisma orientale each 10g for damp-heat. This formula has the effects of invigorating the spleen and stomach, balancing Yin and Yang, promoting blood circulation and detoxifying. It is used for leukopenia caused by chemotherapy.

　　3. Other Therapies

　　1. Rubbing Method:Apply Yìshù oil ointment after each radiotherapy session, and dry the ointment on the irradiated area. Instruct the patient to wear loose underwear to prevent the skin from sticking; it is suitable for skin burns caused by radiotherapy.

　　2. Traditional Chinese Medicine Injection Method

　　(1) Huan葵 injection, 4ml each time, once a day, intramuscular injection, 30 days as a course, with the effects of clearing heat and dampness, detoxifying and reducing swelling.

　　(2) Add 10% Brucea javanica oil to the disinfected vagina, and use a 5ml syringe with a needle attached. According to the lesion, inject at the tumor site twice a week, 5-10% Brucea javanica oil 4-8ml each time. After injection, stop medication based on the condition of the lesion's shedding, necrosis, and regeneration; it has the effect of shedding necrotic cancer tissue and promoting the growth of new tissue.

　　4. Traditional Chinese Medicine

　　1. Gynecological Pill:This product has the effects of promoting blood circulation and removing blood stasis, degrading corrupt tissues and promoting muscle regeneration, reducing swelling and alleviating pain, drying dampness and contraction, detoxifying and killing parasites. For external use, apply once or twice a week, one pill per time, with a course of four times. Abstain from sexual activity and avoid spicy foods, fish, crabs, chickens, ducks, and other pungent items during treatment.

　　2, Mahuangxian suppository:It has the effects of clearing heat, drying dampness, relieving itching, and dissolving blood stasis to detoxify. Topically, take one pill into the vagina every night.

　　Fifth, Surgical Treatment

　　Local extensive resection must be performed for vaginal smooth muscle sarcoma. The incision edge should be at a certain distance from the tumor to be considered as the tumor being excised. In order to completely excise the tumor, sometimes it is necessary to perform posterior pelvic, anterior pelvic, or total pelvic visceral resection.

　　Sixth, Chemotherapy (Radiation Therapy)

　　Chemotherapy regimens that are effective for smooth muscle sarcomas include:

　　1, AD:Doxorubicin (adriamycin) 60mg/㎡ intravenously, on the first day; dacarbazine (dacarbazine, DTIC) 250mg/㎡ intravenously, on the first to fifth days. The interval between courses is 3 weeks.

　　2, CVAD:Cyclophosphamide 500mg/㎡ intravenously, on the second day; vinblastine 1.5mg/㎡ intravenously, on the first and eighth days; doxorubicin (adriamycin) 50mg/㎡ intravenously, on the second day; dacarbazine 250mg/㎡ intravenously, on the first to fifth days. The interval between courses is 3 weeks.

　　3, MeCA:Semustine (Me-OCNU) 150mg/㎡ orally, on the first day; doxorubicin (adriamycin) 60mg/㎡ intravenously, on the first day. Repeat one course of treatment every 6 weeks.

　　4, CAD:50mg/㎡ of cyclophosphamide intravenously, on the second day; 45mg/㎡ of doxorubicin (adriamycin) intravenously, on the first day; 400mg/㎡ of dacarbazine, intravenously, on the first and second days. Repeat one course of treatment every 3 weeks.

　　In the treatment of vaginal smooth muscle sarcoma, regional arterial perfusion chemotherapy is used, and the local control rate can reach 80%. The use of doxorubicin arterial perfusion chemotherapy has low systemic toxicity, 50% of tumors show necrosis, and the necrosis of tumors increases to 85% after adding X-ray radiotherapy of 35Gy after chemotherapy.

　　Intraperitoneal arterial perfusion chemotherapy is a good chemotherapy method for treating vaginal smooth muscle sarcoma, which can increase the drug concentration in local tissues and improve efficacy, and has low systemic toxicity.

　　In the case of local advancement in the late stage of smooth muscle sarcoma of the posterior vaginal wall, good short-term efficacy can also be achieved by adding external radiotherapy to the pelvic floor area after posterior pelvic visceral resection.