Vaginal rhabdomyosarcoma

　　1. Pathogenic cause

　　The pathogenic factors are not yet clear. Vaginal rhabdomyosarcoma originates from幼稚 primitive mesenchymal cells in the müllerian duct epithelial stroma.

　　2. Pathogenesis

　　Tumors are mostly pale red or grayish white, semi-transparent, mucoid, grape-like or polypoid masses, soft, edematous, and may be accompanied by hemorrhage or ulcers. The cut surface shows loose tissue with a mucoid appearance.

　　Microscopic examination of the typical grape cluster type can show three related areas: a cell formation layer close to the submucosa, a deeper cell area composed of more primitive tumor cells, and a central mucinous area with few cells. The cells are immature round and spindle-shaped cells, with nuclear atypia, and myoblasts can be seen.

　　The light microscopy and electron microscopy findings, as well as special staining, immunohistochemistry, RMS tumor markers, cell and molecular genetic tests of other types of RMS are the same as those of vulvar rhabdomyosarcoma.

　　In the late stage, bladder-vaginal fistula may occur. Bladder-vaginal fistula is a type of urinary fistula. Urinary fistula refers to an abnormal passage formed between the reproductive organs and the urinary system, manifested as urinary incontinence. Reproductive organ fistula is a very painful injury disease. Due to the inability to control urine, the vulva is immersed in urine for a long time, which not only brings physical pain to women but also causes patients to be afraid to approach the crowd, unable to participate in production and labor, and bears a great mental burden. For bladder-vaginal fistula, if the fistula is located at the internal orifice of the urethra and/or above, if the fistula is large, all urine leaks out of the vagina, and the patient is completely unable to urinate. If the fistula is small, and granulation tissue forms a valve-like shape around the fistula, the patient can often control a part of the urine, and when the bladder is overfilled, there is overflow of urine.

　　1. Symptoms:Vaginal rhabdomyosarcoma is asymptomatic in the early stage, and the progression of the disease is often manifested as vaginal bleeding and vaginal masses, accompanied by serous, mucous leukorrhea, sometimes bloody. For example, infants and young children may have irregular vaginal discharge or bleeding when crying, urinating, or coughing. In the absence of a history of trauma, RMS should be considered, especially when there are polypoid masses protruding outside the vaginal orifice. Adult women with the disease often manifest excessive menstruation or irregular vaginal bleeding, while elderly women often manifest irregular vaginal bleeding after menopause or vaginal discharge with an odor of purulent secretion. When the tumor necrotic ulceration, tissue fragments may be discharged from the vagina, and the lesion can involve the urethra and bladder, resulting in urinary system symptoms such as frequent urination, urgency, difficulty in urination, and hematuria.

　　2. Signs:It is most common in the upper third of the vagina, with the anterior wall being the most frequent. Tumors can present as one or more polypoid or grape-like, with sizes ranging from 0.2 to 15 cm, mostly 3 to 5 cm, with a smooth surface, pale red, semi-transparent, often fused into nodular lobulated, and can also be scattered. They are soft and脆，easily bleeding, and can fill the entire vagina, even protruding from the vaginal orifice, covering the clitoris and perineum. Tumors can also invade the cervix or spread to both sides of the vagina, palpable masses can be felt during pelvic examination, and regional lymph node and lung metastases can occur.

　　3. Types:Vaginal RMS is most commonly seen in grape cluster type, accounting for 67.1% to 78.6%, followed by embryonal type, accounting for 21.0% to 28.4%, while acinar type is limited to individual case reports. In staging, IRSG III stage is most common, accounting for about 84.1%.

　　Parents or caregivers should always pay attention to the cleanliness of the baby's vulva, change diapers in time, and check carefully whether there is redness or abnormal discharge in the baby's vagina each time the diaper is changed.

　　Vaginal rhabdomyosarcoma must be followed up closely after treatment, especially within the first 2 years after diagnosis, because local recurrence is likely (median recurrence 16 months), it is recommended to have vaginal, uterine cavity, and transvaginal ultrasound examinations under anesthesia every 2 months. Arndt et al. followed up for 5.8 years and found that 66.6% of the female reproductive tract rhabdomyosarcoma patients who recurred could be remedied by chemotherapy, surgery, and radiotherapy. For recurrent embryonal or grape cluster vaginal rhabdomyosarcoma, surgery is an exact treatment, and the prognosis of the group receiving radiotherapy in the remedial measures is better than that of other groups. The 5-year survival rate after recurrence mainly depends on the histological type of the mass, the stage at the initial diagnosis, and the way of recurrence (local recurrence, regional lymph node recurrence, or distant recurrence).

　　1. Pathological examination

　　Under the microscope, the tumor grows like a polypoid mass on the surface of the epithelium, and below the epithelial layer there is a dense layer of undifferentiated tumor cells as a characteristic manifestation. The nuclei of tumor cells are large, and there is或多或少 eosinophilic cytoplasm. Nuclear division is often seen, and there may or may not be striations.

　　2. Imaging examinations

　　Chest X-ray and bone scan can determine whether there is bone and lung metastasis. CT scan can help doctors assess the extent of tumor invasion and formulate treatment plans. Ultrasound examination is very necessary as an auxiliary to CT. Magnetic resonance imaging can provide superior soft tissue contrast.

　　3. Other examinations

　　Immunohistochemistry, tumor markers, and molecular genetic testing.

　　1. Chinese wolfberry softshell turtle lean pork soup:30 grams of Chinese wolfberry, 1 softshell turtle (about 500 grams), 150 grams of lean pork. First place the turtle in hot water to move around, so that it urinates, then kill and cut open, remove the internal organs, wash and cut into pieces, add an appropriate amount of water, cook with Chinese wolfberry and lean pork until soft, take in 2 to 3 servings.

　　2. Chinese wolfberry sea cucumber lean pork sauté:15 grams of Chinese wolfberry, 250 grams of sea cucumber, 100 grams of lean pork. First soak the sea cucumber until it is fully permeated, wash and cut into slices, then cook with the lean pork in an appropriate amount of water until soft, season and eat, take in several servings.

　　3. Mushroom Cordyceps stewed chicken:20 grams of mushrooms, 15 grams of Cordyceps sinensis, 1 unegg-laying hen (about 1000 grams). Remove the mushroom stems, remove the feathers, heads, feet, and internal organs of the chicken, put the mushrooms and Cordyceps sinensis into the chicken's abdomen, seal with bamboo skewers, add an appropriate amount of water, simmer over low heat for 2 hours, season and serve. Can be taken in 2 to 3 servings.

　　4. Milk and egg white lotus seed paste:250 milliliters of fresh milk, 2 fresh eggs, 50 grams of Shiliuzi. Grind Shiliuzi into powder, add an appropriate amount of water to cook the lotus seed powder into a paste, add rock sugar or white sugar for seasoning, then add milk and egg whites and mix well, boil and serve. Take once a day or every other day.

　　5. Jinjin Valley ginger rabbit meat soup:Ji Nei Jin 12 grams, Gu Ya 30 grams, three slices of ginger, 100 grams of rabbit meat. Add an appropriate amount of water, boil together, add a little salt for seasoning, drink the soup and eat the meat. Take once a day or every other day.

　　6. Sharen Huai Shan stewed pig's stomach:Sharen 15 grams, Huai Shan Yao 50 grams, one pig's stomach. Break the Sharen, wash the pig's stomach clean and remove the fat. Put the Sharen and Huai Shan Yao into the pig's stomach, add an appropriate amount of water, simmer over low heat until the pig's stomach is tender, add a little salt for seasoning, drink the soup or eat with food.

　　7. Yan Wo stewed Xiyang Shen:Yan Wo 6 grams, Xiyang Shen 9 grams. Soak Yan Wo in warm water to remove the bird feathers, slice Xiyang Shen, add an appropriate amount of water, steam for 12 hours after stewing, and take it.

　　8. Pear juice sugarcane water chestnut dew:One part of pear juice, two parts of sugarcane juice, one part of water chestnut. Mix and serve cold, or heat and serve warm.

　　9. Huang Qi Gou Qi Zi stew water fish:Huang Qi 30 grams, Gou Qi Zi 20 grams, one water fish (about 500 grams). Wrap Huang Qi in gauze, remove the scales and internal organs of the fish, wash and cut into pieces. Add an appropriate amount of water, simmer until tender, remove the Huang Qi residue, add a little oil and salt for seasoning, and take it in several doses.

　　10. Turtle and pork trotter ginseng soup:One turtle (about 150-250 grams), 250 grams of pork trotter, 10 grams of Ren Shen. First, blanch the turtle with boiling water to make it urinate, cut off the head and claws, remove the internal organs, wash clean, and cut into pieces with the pork trotter. Add an appropriate amount of water, simmer slowly until tender, and take it in several doses.

　　First, traditional Chinese medicine treatment for vaginal rhabdomyosarcoma

　　1. Dialectical selection of prescription

　　After surgery, use Cang Zhu 16, Dan Shen 20, Sheng Di 12, Shan Yao 15, Sheng Ma 18, Huang Qi 12, Long Gu 16, Fu Shou 12, one dose per day, decocted in water and taken, to enhance immunity, promote wound healing, and improve the quality of life.

　　After the wound heals, the following can be used: Ban Zhilian 50, Shi Shang Bai 60, Yun Fu 16, Fa Ban Xia 15, Zhu Ru 12, Nuzhenzi 15, Shu Di 12, Hong Hua 6, Gan Cao 8, one dose per day, decocted in water and taken, continue for 5 days, then take the above prescription for 4 days, which helps to improve the efficacy.

　　2. Special prescription

　　(1) External washing formula

　　San Pin Formula: Bai Pi 45g, Ming Fan 60g, Xiong Huang 7.2g, Mo Yao 3.6g. Grind into fine powder and mix to make San Pin cakes the size of a 5-cent coin (thick 2mm, weight 0.2g), ultraviolet sterilization for standby. The patient lies on the gynecological examination bed, disinfect the vagina, and first protect the unbroken parts of the vagina and fornix with Vaseline gauze. Apply the San Pin cake on the tumor, after 5-7 days, there is necrotic tissue shedding, wait for the San Pin cake to absorb, and change the Chinese medicine 'Shuang Zi Fen' (Zicao, Zihua Ding, Cao He Che, Huang Bai, Cao Lian Cao each 30g, a little Bing pian as fine powder, high-pressure sterilization) before the tissue falls off; the medicine should be taken from 5-7 days after menstruation to 5 days before menstruation; this method has the effects of disinfection, anti-cancer, reducing swelling, and removing blood stasis. Contraindicated for those with serious liver and kidney function damage.

　　Huang Wou San No.1: Huang Bai 64%, Qing Fen 13%, Wugong 7%, Bing pian 3%, She Xiang 0.7%, Xiong Huang 12.3%, respectively ground into powder and sieved through a 100-mesh sieve, mixed evenly for standby. Use sterile tail thread cotton balls, dip the powder about 1g with a long-handled forceps and send it into the vaginal lesion; for mild cases, once a week; for severe cases, 2-3 times a week. Avoid sexual activity during treatment.

　　(2) Internal treatment formula

　　① Yicai Tablet: 5 catties of Strychnos nux-vomica, Trichosanthes kirilowii, and Paris polyphylla, 1 catty of licorice, the Strychnos nux-vomica skin removed, fried with sesame oil until crisp, mixed with the other three herbs into a fine powder, added with starch to make tablets, each tablet 0.3g, take 3 times a day; initially, take 3 tablets each time, if there are no adverse reactions, increase to 5 tablets each time, without course, take continuously. Most patients experience increased physical strength and appetite; taking an excessive amount or for a long time may cause symptoms such as tightness in the hands and feet, which will disappear after stopping the medicine. This formula has the effects of detoxifying, reducing swelling, and dissipating blood stasis.

　　② Pinellia ternata Formula: Appropriate amount of Pinellia ternata, made into oral tablets; take 3 times a day, the total amount is about 60g of crude drug, which has a significant effect of clearing heat, detoxifying, and anti-cancer.

　　(3) Prevention and treatment of toxic and side effects of radiotherapy and chemotherapy for malignant tumors The chemotherapy of malignant tumors often causes a series of toxic and side effects after radiotherapy; such as local pain, adhesion, tissue necrosis, decreased blood cells and platelets in the whole body, dysfunction of digestive function, damage to liver and kidney function, damage to the nervous system, etc. 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 and Codonopsis each 30g, Schisandra chinensis, Cuscuta chinensis, fried Astragalus, fried Astragalus, Ophiopogon japonicus, Angelica sinensis, Poria cocos, Amomum villosum, and Prunella vulgaris each 12g. Boil in water, take one dose per day, divided into 2-3 times for oral administration, starting 3 days before chemotherapy. This product has the functions of strengthening the body and anti-cancer, nourishing Yin and blood, promoting Qi and drying dampness, harmonizing the spleen and stomach to stop vomiting, and dissipating blood stasis and lump, which can prevent the toxic and side effects of chemotherapy and ensure the smooth completion of chemotherapy.

　　② Yangxue Shengbai Drink: Codonopsis, Astragalus, Angelica sinensis, prepared Rehmannia, Schisandra chinensis, Uncaria tomentosa, Poria cocos, each 15g, Bupleurum 10g, Cinnamon bark 6g, Prepared Pinellia 9g, for Yin deficiency, add raw Rehmannia and Osteodermata each 15g; for Yang deficiency, add Morinda officinalis and Curculigo orchioides each 12g; for insufficient heart and blood, add Ziziphus jujuba and Cuscuta chinensis each 12g; for damp-heat, add Phellodendron amurense and Alisma orientale each 10g. This formula has the effects of invigorating Qi and strengthening the spleen, balancing Yin and Yang, and promoting blood circulation and detoxification, and is used for leukopenia caused by chemotherapy.

　　3. Other therapies

　　(1) Rubbing method: Apply the Curcuma oil ointment, after each radiotherapy, apply the ointment to the radiotherapy site and let it dry. Instruct the patient to wear loose underwear to prevent it from sticking to the skin; it is suitable for skin burns caused by radiotherapy.

　　(2) Chinese medicine injection method

　　① Huaihuanglian Injection, 4ml each time, once a day, intramuscular injection, 30 days as a course of treatment, 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 high-pressure syringe connected with a needle. According to the lesion, inject at the tumor site, twice a week, 5-10% Brucea javanica oil 4-8ml each time. After injection, stop taking medicine according to the situation of lesion shedding, necrosis, and new growth; it has the effect of removing decayed and necrotic cancer tissue and promoting the growth of new tissue.

　　Second, Western medical treatment methods for vaginal rhabdomyosarcoma

　　1. Surgical treatment

　　(1) If the cancer lesion is located in the upper third of the vagina, it is possible to perform a total hysterectomy, most of the vaginal resection, and pelvic lymph node dissection.

　　(2) For cases where the tumor mass is located in the lower 1/3 of the vagina, excision of the vulva, lower vaginal segment (and possibly part of the urethra if necessary) and bilateral inguinal lymph nodes (and possibly pelvic lymph nodes if necessary) dissection should be performed.

　　(3) For cases where the tumor mass is located in the middle of the vagina or is multifocal, total hysterectomy, total vaginal resection, and inguinal, iliac pelvic lymph node dissection should be performed.

　　(4) For cases where the tumor mass involves the urethra, bladder, or rectum, a anterior or posterior pelvic organ resection and pelvic or inguinal lymph node dissection can be performed. And artificial urethra, artificial bladder, or artificial anus reconstruction can be performed.

　　(5) For children or young patients with fertility requirements, vaginal reconstruction with skin flaps is performed after vaginal resection.

　　(6) Radical surgical resection is the fastest and most reliable method for the treatment of rhabdomyosarcoma. Patients with grossly visible lesions after the first surgery or those who have not achieved complete therapeutic effects within six months of induction chemotherapy and radiotherapy should advocate for a second surgery to perform radical resection of these lesions.

　　2. Chemotherapy (radiation therapy)

　　(1) Chemotherapy:

　　In the past, chemotherapy was only used to eliminate residual lesions after surgery. To preserve organ function and improve prognosis, preoperative multi-drug chemotherapy is now used to reduce the need for radical surgery, and chemotherapy is continued after surgery. Since a considerable number of children have distant metastases at the time of consultation, and most tumor recurrences occur within 2 years after surgery, chemotherapy is very important and usually needs to be continued for 2 years or more.

　　① American Rhabdomyosarcoma Study Group (IRS): Chemotherapy is started within 42 days after the biopsy of the tumor mass or within 21 days after surgery in I-Ⅲ period patients. ForⅣ period patients, chemotherapy is given as soon as diagnosed.

　　Ⅰ period: VA (vincristine and actinomycin D) regimen chemotherapy for 1 year - actinomycin D (neomycin) 0.015mg/(kg/day), intravenous injection, for 5 consecutive days, repeated every 9 weeks. Vincristine 2mg/m2, intravenous injection, once a week for 5 weeks before the administration of actinomycin D (neomycin).

　　Ⅱ and Ⅲ periods: VAC (vincristine, actinomycin D, and cyclophosphamide) chemotherapy for 2 years - vincristine 1.5mg/m2, intravenous injection, on the first day, once a week; actinomycin D (neomycin) 0.015mg/(kg/day), intravenous injection, course days 1-5, repeated once every 4 weeks; cyclophosphamide 2.2g/m2, intravenous injection, on the first day, repeated once every 4 weeks, with mesna (sodium thiosulfate) for detoxification. Doxorubicin (adriamycin) (30mg/(m2/day), intravenous injection) or/and cisplatin (90mg/m2, intravenous injection) can be added in the case of alveolar/undifferentiated types.

　　Ⅳ period: After 12 weeks of IE (ifosfamide plus etoposide) chemotherapy, VAC regimen is used again - ifosfamide 1.8g/m2/day and etoposide (podophyllotoxin) 100mg/(m2/day), intravenous injection, course days 1-5, repeated once every 3 weeks, a total of 4 times, and mesna (sodium thiosulfate) for detoxification. Then, the VAC regimen is given.

　　② International Society of Pediatric Oncology (SIOP):

　　Stage I pT1: Chemotherapy with VA regimen - vincristine 1.5mg/m2, intravenous injection, on the first day of the cycle; actinomycin D (neomycin) 1.5mg/m2, intravenous injection, on the first day of the cycle, for a total of 2 cycles.

　　Stage I pT3, II: The IVA regimen - vincristine, actinomycin D (neomycin), and ifosfamide [V1.5mg/(m2/d1), A1.5mg/(m2/d1), I 3g/(m2/d1-3)]. If the chemotherapy regimen is not effective, VCE - vincristine, carboplatin, and teniposide (thiotepa, VM26) [V1.5mg/(m2/dl), C600mg/(m2/d3), VM26150mg/(m2/d4)] is administered. A total of 4 to 6 cycles.

　　Stage III, IV: The CEIVAE regimen, also known as IVA, CEV (carboplatin 500mg/(m2/d1), epirubicin 150mg/(m2/d1), and vincristine 1.5mg/(m2/d1), IVE [vincristine, ifosfamide, and etoposide (podophyllotoxin) 200mg/(m2/d1-3)] are alternated. A total of 4 cycles.

　　In addition, there are research groups such as the German Soft Tissue Sarcoma Study Group (CWS) and the Italian Soft Tissue Sarcoma Study Group, as each research group uses different rhabdomyosarcoma classifications and different chemotherapy regimens, the results cannot be compared with each other. Raney et al. reported that the 3-year survival rate was 91% for locally advanced intermediate-risk rhabdomyosarcoma treated with vincristine, actinomycin D (neomycin), doxorubicin (adriamycin), etoposide (podophyllotoxin), and ifosfamide.

　　③ For patients newly diagnosed with stage IV and acinar/undifferentiated types, the efficacy of topotecan is up to 58% (Ruymam, 2000), so in the ongoing IRSV study, VTC (vincristine, topotecan, and cyclophosphamide) or VCPT (vincristine and irinotecan [CPT-11]) are used to treat stage IV and acinar/undifferentiated patients.

　　④ Second-line chemotherapy and rescue chemotherapy: For patients who have relapsed after multiple chemotherapy regimens, due to decreased tolerance to chemotherapy drugs and multi-drug resistance, there is no ideal chemotherapy drug. Re-treatment after relapse needs to be based on the initial treatment to help select drugs, generally selecting drugs that were effective during the initial treatment combined with drugs that have not been used. For example, after relapse from VAC chemotherapy, VadrC (adr for doxorubicin) can be used, and for relapse after VadrC chemotherapy, ifosfamide, carboplatin, and etoposide (podophyllotoxin) can be used, but elronitibine can be used for all relapse cases, as even patients resistant to topotecan (topotecan) can still respond to elronitibine.

　　(2) Radiotherapy:

　　Radiotherapy is a very important method for treating childhood rhabdomyosarcoma and certain undifferentiated soft tissue sarcomas. For sites where residual tumor cannot be completely resected by surgery, radiotherapy can further kill residual tumor cells that cannot be removed by simple surgery. Vaginal applicators (candles) or interstitial irradiation can be used for exophytic tumors. Tumors in the lower part of the vagina or the entire vaginal lesion can be treated with vaginal candles or interstitial implantation. If the tumor is only located on one side of the vagina and is large, interstitial implantation can be performed to reduce the tumor size, followed by vaginal candle irradiation, while appropriate lead blocks are used for areas not requiring irradiation. The dose reference point is generally selected at the base of the tumor. For rhabdomyosarcoma, the minimum tumor radiation dose is 4000 cGy, while older children should receive 4500 cGy. For tumors with a diameter greater than 5 cm, the radiation dose should be 5000-5500 cGy. For stage I and II patients, radiotherapy and chemotherapy can be performed simultaneously, while for stage III and IV patients, radiotherapy should be delayed until 6 weeks to evaluate the efficacy of chemotherapy and reduce mucositis and other damage. For children with localized tumors, one course of the 'VAC' regimen should be repeated every 4 weeks. If the tumor shrinks by 50% or more after 2 courses of 'VAC' chemotherapy, 2 more 'VAC' courses can be used. The efficacy is estimated through repeated biopsies. If local tumors are still persisting, radiotherapy should start from the 16th week.

　　In recent years, a method of radioactive implantation has been practically applied, especially suitable for children with small tumors located in critical sites such as the head and neck, bladder, vagina, limbs, etc. Due to the strict control of radiation dose, the surrounding tissues are lightly affected, resulting in low incidence of fibrosis.

　　For metastatic rhabdomyosarcoma, under the condition of bone marrow tolerance, simultaneous radiotherapy can be performed on the vagina and the metastatic sites. Radiotherapy should start in conjunction with chemotherapy 18.5 weeks after chemotherapy, and the scope of radiotherapy should be the primary tumor plus a 2cm margin. If there is metastasis in the local lymph nodes, these lymph nodes should also be included. Radiotherapy to the vagina is 50.4 Gy, while the lung should be limited to 14.4 Gy, and the lesion in the bone marrow cannot be radiotherapeutically treated. For female reproductive tract rhabdomyosarcoma, short-distance irradiation should be used as much as possible after the ovaries have been transposed. If the dose to the ovaries is 1-4 Gy, it does not affect reproductive function, but if it is greater than 5 Gy, it must be transposed.

　　3. Other special therapies

　　(1) Immunotherapy and gene therapy: To prevent the inhibitory effect of bone marrow, granulocyte colony-stimulating factor is injected subcutaneously 24-48 hours after high-dose chemotherapy until the absolute neutrophil count exceeds 1000 cells/μl. However, granulocyte colony-stimulating factor may increase the damage to stem cells caused by alkylating agents. Breitfeld et al. (2001) suggested increasing the ability of stem cells to repair alkylating agent damage by transferring genes encoding DNA repair enzymes. The addition of interleukin-2 (IL-2), an immunomodulator, to high-dose chemotherapy can improve the survival rate of patients with recurrence.

　　(2) Stem Cell Transplantation: Currently, the efficacy of autologous stem cell transplantation for rhabdomyosarcoma is being evaluated, which allows us to use chemotherapy at doses much higher than normal. Before chemotherapy starts, the patient's own stem cells are removed and preserved; they are then infused back into the patient's body after chemotherapy ends.

　　4. Combined Therapy:For children with stage I and II rhabdomyosarcoma, surgery should be performed as soon as possible after clear diagnosis, and radiotherapy and chemotherapy should be performed according to the specific type and stage after surgery. For stage III and IV rhabdomyosarcoma, combined chemotherapy can be started as soon as it is diagnosed to prepare for surgery. The operation is performed after 14 weeks of chemotherapy. VAC and VDC regimens are alternated for two years after surgery.

　　III. Prognosis

　　Vaginal rhabdomyosarcoma has a good prognosis, and within 2 years after diagnosis, due to the high probability of local recurrence (median recurrence 16 months), it is recommended to perform colposcopy, hysteroscopy, and transvaginal ultrasound every 2 months under anesthesia. The 10-year survival rate is 90%. Histological type is not a prognostic factor for vaginal RMS.

　　1. Histological Type:Tumor Size

　　2. Stage at initial diagnosis:Patients aged 1 to 9 years at diagnosis have a better prognosis than those aged 9 years.

　　3. Metastasis:Patients with distant metastasis at the time of consultation have a significantly worse prognosis than those without distant metastasis. Distant metastasis has a poorer prognosis than local metastasis.

　　4. Staging:Patients with vaginal RMS in IRS III and IV studies have significantly higher 5-year survival rates and disease remission survival rates than those in IRS I and II studies.

　　5. Early treatment response:Patients with a lack of beneficial response to initial radiotherapy and chemotherapy have poor long-term prognosis. For stage IV patients, those who achieve complete remission 18 weeks before the start of 3 cycles of chemotherapy have a significantly higher 3-year overall survival rate than those who achieve complete remission after 3 cycles of chemotherapy.