Vulvar synovial sarcoma

　　1. Etiology

　　Vulvar synovial sarcoma was once thought to originate from joint synovium, but the histological origin of vulvar synovial sarcoma remains unclear to this day. Typical synovial sarcomas have characteristics of biphasic differentiation of epithelium and mesenchyme.

　　2. Pathogenesis

　　The tumor presents as lobulated or nodular, often with a pseudocapsule and clear boundaries. The cut surface is grayish-white, soft, and often shows hemorrhage, necrosis, and cyst formation.

　　Microscopy shows two types of cells with different morphologies, forming a unique biphasic configuration. The epithelial cells are similar to cancer, with cuboidal to high columnar cells arranged in solid, nodular, or glandular, papillary structures. The spindle cells mostly have a fibrosarcomatous configuration. There are also transitional cells between the epithelial and spindle cells. According to the relative significance and differentiation degree of the epithelial and spindle cells, synovial sarcomas can be divided into biphasic, monophasic fibrous, monophasic epithelial, and low differentiation types. Under the electron microscope, the surface of the epithelial cells can be seen to have microvilli or pseudopodia facing the intercellular cavity or pseudo-glandular cavity. Electron-dense mucinous substances are commonly found in the lumen. The continuous basement membrane, which is not present in normal synovium, often separates the epithelial clusters or glandular structures from the surrounding spindle cells.

　　Almost all synovial sarcomas have a specific chromosomal translocation - t(x;18)(pll;q11), resulting in the fusion of the SSX gene on Xpll with the SYT gene on 18q11 to form the SYT-SSXl or SYT-SSX2 chimeric genes due to chromosomal balanced translocation. 61% to 64% of synovial sarcomas have SYT-SSXl, and about 36% have SYT-SSX2. All chimeric genes with SYT-SSXl are biphasic synovial sarcomas, while almost all monophasic synovial sarcomas have SYT-SSX2 chimeric genes (Ladanyi 2002). Detection of the SYT-SSX chimeric gene can be used to determine whether the surgical margin is positive, and it can also be used to diagnose small metastatic lesions, which is more sensitive and specific than histological examination.

　　Lymph node metastasis usually first reaches the group of lymph nodes closest to the tumor (first station), and then successively in those that are further away (second and third stations), while the tumor cells infiltrate and grow in each station, they also expand to the adjacent lymph nodes within the same group. However, there are also exceptions, some patients may bypass the lymph nodes in the pathway and directly metastasize to a more distant group of lymph nodes (second or third station). This type of metastasis is called skip metastasis in clinical practice. For example, in the case of cervical cancer, when there is no lymph node metastasis in the para-aortic lymph nodes in the pelvic retroperitoneum, the first lymph node metastasis is found in the neck. In addition, there may also be retrograde lymphatic drainage, which is transferred to the lymph nodes on the centrifugal side, which may be due to the obstruction of the lymphatic vessels in the direction of flow. For example, cervical cancer metastasizing to peritoneal lymph nodes.

　　Vulvar synovial sarcoma is a painless mass with slow subcutaneous growth, ranging in size from 1.2 to 20 cm, with the mass being mostly fixed and having clear boundaries, and it is easily misdiagnosed as a Bartholin's cyst. Vulvar synovial sarcoma is relatively rare. The age of onset is between 30 and 39 years.

　　Prognosis: Nielsen et al. reported that 2 cases had a relatively good prognosis, one with 4 years of tumor-free survival after radical vulvectomy, and the other recurred 3.5 years later, underwent resection and radiotherapy, and had 1 year of tumor-free survival. Zhang Rongyu et al. reported a case with only local resection, positive margins, recurrence 3 months after surgery, bone and inguinal lymph node metastasis, whole-body external radiotherapy, continued tumor progression, left iliac and pubic bone metastasis, left hemipelvic resection, pathological examination showed positive margins and vascular invasion, and the patient died 6 months later. Another case underwent radical vulvectomy with partial pubic and vaginal resection, recurrence with lung metastasis 10 months after surgery, and death 20 months later.

　　Saito et al. (2000) found that the prognosis of synovial sarcoma expressing E-cadherin or alpha-catenin is good, while the expression of beta-catenin is related to the progression of the tumor. Ladanyi et al. (2002) found that the SYT-SSXl fusion gene is mostly seen in patients with metastasis, with a 5-year survival rate of 53%, which is significantly worse than that of patients with SYT-SSX2 fusion gene (73%).

　　1, Chemical and immunohistochemical detection using PAS, colloidal gold, and Alizarin blue staining, followed by digestion with hyaluronidase, showing the presence of two different mucus types. Meshwork fiber staining can reveal a biphasic configuration, especially for those with lower differentiation, where fibroblasts are 100% positive for vimentin, and bcl-2, CD99, S-100 can also be positive. Some may also be positive for EMA and cytokeratin. Epithelioid cells, in addition to being positive for EMA and cytokeratin, are 40% positive for vimentin.

　　2, Cytogenetic testing, histopathological examination.

　　Firstly, dietetic recipes for vulvar synovial sarcoma

　　1, He Shou Wu egg soup

　　Composition: He Shou Wu 120 grams, eggs 4.

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

　　2, Sesame brown sugar porridge

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

　　Usage: Clean black sesame seeds, slightly fry, store in a bottle for later use or crush and store in a bottle. Take 2 tablespoons with an appropriate amount of brown sugar, dip in steamed buns or dilute with hot water for drinking.

　　3, Walnut sesame seed porridge

　　Composition: Walnuts 200 grams, sesame seeds 100 grams, glutinous rice 100 grams.

　　Usage: Grind walnuts and sesame seeds into powder. Cook glutinous rice with an appropriate amount of water, then add walnuts and sesame seeds and serve.

　　4, He Shou Wu Chinese yam lamb soup

　　Composition: He Shou Wu 30 grams, Chinese yam 100 grams, lamb meat 500 grams, ginger 9 grams.

　　Secondly, what foods are good for vulvar synovial sarcoma

　　1, It is advisable to eat more foods that have an anti-vulvar tumor and leukoplakia effect, such as sesame seeds, almonds, wheat, barley, cucurbita moschata, black-bone chicken, cuttlefish, amethystine snake, pig pancreas, chrysanthemum, umeboshi, peach, lychee, portulaca oleracea, chicken blood, eel, abalone, crab, cuttlefish, sardine, mermaid, clam, tortoise shell.

　　2, For pain, it is advisable to eat cuttlefish, red crabs, lobsters, clams, sea cucumber, tiger fish, beetroot, mung beans, radish, chicken blood.

　　3, It is advisable to eat amaranth, cabbage, rapeseed, taro, kelp, purple kelp, chicken blood, snake meat, pangolin, etc. for itching.

　　4, To enhance physical fitness and prevent metastasis, it is advisable to eat silver ear, black fungus, shiitake mushrooms, hou-tuo jelly fungus, chicken gizzards, sea cucumber, Job's tears, walnuts, crabs, lizard, needlefish, etc.

　　5, After surgery for vulvar tumors, qi is consumed and blood is injured, so it is advisable to eat more foods that tonify qi and nourish blood, such as jujube, longan, adzuki beans, glutinous rice, lychee, shiitake mushrooms, carrots, quail eggs, lotus root starch, beans, etc.

　　6, Radiotherapy after surgery for vulvar tumors: it consumes yin and fluid, so it is advisable to eat more nourishing yin and fluid foods, such as spinach, minor bok choy, lotus root, radish, watermelon, banana, grapes, sea cucumber, sugarcane, lily, etc.

　　7, Chemotherapy after surgery for vulvar tumors: it can damage both qi and blood, so it is advisable to eat foods that tonify qi and blood, such as black fungus, shiitake mushrooms, walnuts, mulberries, job's tears congee, red dates, longans, sea cucumber, etc.

　　8, Shitake mushrooms, 90 grams, stir-fried with a moderate amount of vegetable oil and a little salt, then cooked into soup. It can help prevent cancer.

　　9. Mushrooms Appropriate amount of mushrooms, decocted into soup, cooked, or ground into powder for oral administration.

　　10. Water Chestnuts Fresh water chestnuts 20-30, add an appropriate amount of water, slowly cooked into a thick soup, divided into 2-3 times to take. It has a certain curative effect on gastric cancer and cervical cancer.

　　11. Oolong Tea Regular drinking of oolong tea has a certain anticancer effect.

　　12. Bee Honey Regular consumption of bee honey can enhance human immunity and has anticancer effects.

　　13. Sprouts The chlorophyll in sprouts can prevent colorectal cancer and some other cancers.

　　14. Other milk or goat's milk, not only rich in vitamins but also containing certain anticancer substances; fresh vegetables such as radishes, cabbage, pumpkins, peas, lettuce, etc., have a certain effect on offsetting the nitrosamines in food; carrots, spinach, tomatoes, seaweed, etc., are rich in vitamin A and have a certain anticancer effect.

　　Third, it is best not to eat certain foods for vulvar synovial sarcoma

　　1. Avoid stimulant drinks such as coffee.

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

　　3. Avoid smoking and drinking.

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

　　5. Avoid fowl such as roosters and geese.

　　6. Avoid seafood and刺激性, allergenic foods when pruritus is severe.

　　1. Prevention Regular physical examinations, early detection, and determination of the scope of radical surgery, and follow-up care should be done well.

　　2. Vulvar Synovial Sarcoma Western Medical Treatment Methods Surgical treatment is the preferred treatment method. The radical surgical scope is determined according to the size and differentiation degree of the sarcoma. If there is lymph node metastasis, simultaneous lymph node dissection should be performed.

　　3. Radiotherapy For those with unsatisfactory surgical margins, postoperative radiotherapy can be added. Radiotherapy has a good effect on monophasic epithelial type.

　　4. Chemotherapy The role of preoperative adjuvant chemotherapy, including arterial catheter chemotherapy, in the treatment of synovial sarcoma is gradually being recognized by most scholars, especially for patients with a diameter greater than 10 cm (Thompson 2000).

　　5. Immunotherapy Bennicelli et al. (2002) proposed that the specific SYT-SSX fusion gene is the target of immunotherapy. Tumor-specific cytotoxic T cells that recognize and kill cells with the SYT-SSX fusion gene, generated by the peptide sequence of the translocation breakage region, can identify and kill cells with the SYT-SSX fusion gene.