Vulvar papillary sarcoma of the soft tissue

　　1. Etiology

　　There are several hypotheses about the origin of vulvar papillary sarcoma of the soft tissue, including paraganglia, Schwann cells, chemoreceptors, renin-producing cells, and skeletal muscle-derived, among others. Most studies support the myogenic theory, but some scholars hold different opinions (Ordonez 1999). In the WHO (1999) classification, it is classified as other types.

　　2. Pathogenesis

　　The mass is often round, elliptical, or nodular, often with an incomplete capsule, cut surface grayish red, soft, easily broken, and commonly hemorrhagic and necrotic.

　　Microscopic examination shows that the tumor cells are separated into nests by vascular and fibrous septa, and present in three morphologies: acinar structure, solid nests, and wreath-like structure. There are two types of tumor cells: clear cytoplasmic cells and weakly acidic granular cytoplasmic cells. HE staining can find PAS-positive, anti-amylase eosinophilic crystals in the cytoplasm. Electron microscopy confirms that the crystals are secretory products formed in the Golgi apparatus.

　　Immunohistochemistry shows that part of desmin, actin, myoglobin, MyoD1, myogenin are positive, while S-100 is negative.

　　Ladanyi et al. (2001) found that due to the presence of relatively specific chromosomal changes - der(17)t(X;17)(p11.2;q25) in papillary sarcoma of the soft tissue, the TFE3 gene on Xp11.2 fuses with the ASPL gene on 17q25 to form the TFE3-ASPL fusion gene. Detection of this chromosomal translocation or fusion gene by FISH or RT-PCR methods is helpful for the diagnosis of the tumor. Recently, Ladanyi et al. (2002) proposed a specific antibody for the TFE3 gene fusion site, using immunohistochemistry to detect the expression of the TFE3 gene in the nucleus to assist in the diagnosis of papillary sarcoma of the soft tissue.

　　Vulvar adenoid cystic soft tissue sarcoma is a rare highly malignant tumor, mainly located in the deep soft tissue of the limbs, mainly metastasizes to the lung through hematogenous spread. Vulvar adenoid cystic soft tissue sarcoma is extremely rare, occurring more frequently during adolescence. Immunohistochemistry shows partial desmin, actin, myoglobin, MyoD1, myogenin as positive, while S-100 is negative. Due to the friction of the sarcoma and the breakdown, combined with the decline in body resistance and immunity, it is easy to develop infections.

　　Vulvar adenoid cystic soft tissue sarcoma is generally found during physical examination or unintentionally, presenting as painless masses with slow growth, the course of the disease varies from several months to several years, may be accompanied by ulcers, 3 patients were found in the large labia, right small labia, and right lower part of the urethra, with sizes of 2-4 cm.

　　1. Prevention of vulvar adenoid cystic soft tissue sarcoma:Regular physical examinations, aim to detect and treat early, and do a good job of follow-up.

　　2. Prevention:Regular physical examinations, aim to detect and treat early, and do a good job of follow-up.

　　3. Preoperative preparation:Consume high-protein, low-fiber diet before surgery, and avoid high-fiber foods in the week before surgery to ensure that there is no bowel movement within one week after surgery, reducing the risk of perineal wound infection caused by defecation.

　　1. Histopathological examination.

　　1. Histopathological examination. CT has good sensitivity and specificity for diagnosing pulmonary metastatic lesions, showing clear boundaries and increased density of nodules or masses. Enhanced CT shows curved and dilated blood vessels within the tumor. Tumor marker examination, ulcer secretion examination, combined with immunohistochemical examination.

　　Firstly, dietary therapy for vulvar adenoid cystic soft tissue sarcoma

　　1. Silver Ear and Lotus Root Starch Soup

　　25 grams of silver ear, 10 grams of lotus root starch, and appropriate amount of rock sugar. After the silver ear is soaked and softened, add appropriate amount of rock sugar and simmer until烂. Dissolve the lotus root starch and take it.

　　Effects: It has the effects of clearing heat, moistening dryness, and stopping bleeding. Suitable for those with excessive menstrual flow and bright red blood.

　　2. Eliminating Tumor Egg

　　Boil 2 eggs, 5 wall lizards of traditional Chinese medicine, and 9 grams of rhizoma curcumae with 400 grams of water. After the eggs are cooked, peel them and continue to boil, discard the medicine and eat the eggs. Take 1 dose every night.

　　Effects: Dissipate nodules and relieve pain, expel wind and calm惊. Suitable for qi stagnation and blood stasis.

　　3. Two Fresh Soup

　　Cut 120 grams of fresh lotus root into slices, chop 120 grams of fresh reed root, and boil the juice into tea for drinking.

　　Effects: Nourish yin and cool blood, remove blood stasis and stop bleeding. Suitable for those with excessive menstrual flow and blood heat stasis.

　　Secondly, what should be eaten for the treatment of vulvar adenoid cystic soft tissue sarcoma?

　　1. It is recommended to consume more foods with anti-external genital tumor properties, such as sesame seeds, almonds, wheat, barley, loofah, black-bone chicken, cuttlefish, green mamba, pork pancreas, chrysanthemum, umeboshi, peaches, lychees, purslane, chicken blood, eel, abalone, crab, horseshoe crab, sardine, clam, turtle shell. For pain, it is recommended to consume horseshoe crab, red, lobsters, oysters, sea cucumber, tiger fish, beetroot, mung beans, radish, chicken blood. For itching, it is recommended to consume amaranth, cabbage, turnip, kelp, nori, chicken blood, snake meat, pangolin.

　　2. To relieve pain, it is recommended to consume horseshoe crab, red, lobsters, oysters, sea cucumber, tiger fish, beetroot, mung beans, radish, chicken blood.

　　3. To relieve itching, it is recommended to consume amaranth, cabbage, turnip, kelp, nori, chicken blood, snake meat, pangolin.

　　4. To enhance physical fitness and prevent metastasis, it is recommended to consume silver ear, black fungus, mushrooms, hedgehog mushrooms, gizzard, sea cucumber, Job's tears, walnuts, crabs, lizard, needlefish.

　　5. After surgery for vulvar invasive ductal carcinoma of the soft tissue, it may consume Qi and injure blood, so it is advisable to eat more foods that tonify Qi and nourish blood, such as jujube, longan, mung bean, glutinous rice, lychee, mushroom, carrot, quail eggs, lotus root powder, beans, etc.

　　6. Radiotherapy after surgery for vulvar invasive ductal carcinoma of the soft tissue: it may consume Yin and damage body fluid, so it is advisable to eat more foods that nourish Yin and body fluid, such as spinach, green vegetables, lotus root, radish, watermelon, banana, grapes, sea cucumber, sugarcane, lily, etc.

　　7. Chemotherapy after surgery for vulvar invasive ductal carcinoma of the soft tissue: it may damage both Qi and blood, so it is advisable to often eat foods that nourish Qi and blood, such as wood ear, mushroom, walnuts, mulberries, congee of Job's tears, jujube, longan, sea cucumber, etc.

　　III. Foods to avoid for vulvar invasive ductal carcinoma of the soft tissue

　　1. Avoid foods that cause itching. For example: fish, shrimp, crab, chicken head, pork head meat, goose meat, chicken wings, chicken feet, etc., which will worsen the itching and inflammation of the perineum after eating.

　　2. Eat less spicy and stimulating foods. For example: onions, pepper, chili, Sichuan pepper, mustard, fennel.

　　3. Avoid eating fried and greasy foods. For example: fried dough sticks, butter, butter, chocolate, etc., which have the effect of promoting dampness and increasing heat, which will increase the secretion of leukorrhea and is not conducive to the treatment of the disease.

　　4. Quit smoking, drinking, and caffeine and other stimulating drinks.

　　I. Precautions before the treatment of vulvar invasive ductal carcinoma of the soft tissue

　　1. Prevention:Regular physical examinations, aim to detect and treat early, and do a good job of follow-up.

　　2. Preoperative preparation:Consume high-protein, low-fiber diet before surgery, and avoid high-fiber foods in the week before surgery to ensure that there is no bowel movement within one week after surgery, reducing the risk of perineal wound infection caused by defecation.

　　II. Traditional Chinese Medicine Treatment Methods for Vulvar Invasive Ductal Carcinoma of the Soft Tissue

　　1. Home remedies

　　(1) Ba Zhen Decoction with modifications. 15g of Dang Gui, 15g of Shu Di Huang, 15g of Bai Shaoyao, 10g of Chuan Xiong, 15g of Tang Shen, 15g of Bai Zhu, 50g of Huang Qi, 10g of Ze Xie, 15g of Tian Kui Zi, 15g of Shan Ci Gu. For blood deficiency, add 30g of Ji Xue Teng and 10g of Shou Wu; for spleen deficiency with poor appetite, add 15g of Shan Yao, 10g of Chen Pi, and 15g of Bian Dou; if pain is present, add 3g of Xi Xin and 20g of Yuan Hu. Decocted in water for oral administration, one dose per day.

　　(2) Wu Wei Xiao Du Decoction with modifications. 15g of Pu Gong Ying, 15g of Ye Ju Hua, 15g of Di Ding, 20g of Tian Kui Zi, 20g of Jin Yin Hua, 15g of Bai Hua She Tong Cao, 30g of Yi Yi Ren, 30g of Jiang Can, 15g of Chong Lou, 15g of Dang Gui, 15g of Bai Zhu, 40g of Huang Qi, 10 strips of Wu Tong. For pain, add 10g of Mo Xiang and 10g of Mo Yao; for constipation, add 5g of Da Huang and 20g of Rong Cong Rong; for low fever, add 20g of Di Gu Pi. Decocted in water for oral administration, one dose per day.

　　(3) Long Dan Xie Gan Decoction with modifications. 15g of Long Dan Cao, 10g of Chai Hu, 15g of Huang Qin, 15g of Zhui Zi, 10g of Ze Xie, 10g of Che Qian Zi, 15g of Sheng Di Huang, 15g of Dang Gui, 15g of Tu Fu Ling, 15g of Ku Shen, 15g of Zao Jiao Ci, 15g of Shan Ci Gu, 10g of Gan Cao, 30g of Huang Qi. For severe local pain, add 6g of Mo Xiang and 6g of Mo Yao; for itching, add 15g of Fang Feng, 15g of Bai Xian Pi, and 5g of She Tui. Decocted in water for oral administration, one dose per day.

　　2. Prescriptions:

　　(1) 12g of Rehmannia glutinosa, 9g of White peony root, Angelica sinensis, Bupleurum chinense, Scutellaria baicalensis, Gentiana scabra, Forsythia, Fritillaria thunbergii, 15g of Polygonum cuspidatum, 30g of Hedyotis diffusa, 30g of Coix, 6g of Saposhnikovia divaricata, 6g of Cynanchum otophyllum, 6g of Ligusticum chuanxiong. Suitable for the type of damp-heat descending. Decocted, 1 dose per day.

　　(2) 6g of Ligusticum chuanxiong, Angelica sinensis, Gentiana scabra, Scutellaria baicalensis, Atractylodes macrocephala, Bupleurum chinense, 9g of Rehmannia glutinosa, Fritillaria thunbergii, Platycodon grandiflorus, 24g of Lonicera japonica, Trichosanthes kirilowii, Ophiopogon japonicus, 12g of White peony root, 15g of Rehmannia glutinosa. Suitable for the type of liver meridian depression with heat. Decocted, 1 dose per day.

　　(3) 9g of Coptis, Scutellaria baicalensis, Phellodendron amurense, Moutan bark, Red peony root, 24g of Taraxacum mongolicum, Viola yedoensis, Hedyotis diffusa. Suitable for the type of fire-toxin hyperactivity, decocted, 1 dose per day.

　　(4) 12g of Herba Epimedii, 12g of Nymphoides peltata, 15g of Lonicera japonica, 15g of Viola yedoensis, 15g of Polygonum cuspidatum, 9g of Forsythia, 12g of Citrus reticulata, 12g of Carthamus tinctorius, 30g of Semilobum, 30g of Lysimachia christinae, 30g of Solanum lyratum, 15g of Taraxacum mongolicum. Decocted, 1 dose per day.

　　(5) Diabola Bufo Decoction: 15g of Diabola, Bufo, Poria, Poria cocos, Codonopsis, 18g of Hedyotis diffusa, Coix, Semilobum, 10g of Tripterygium, Atractylodes, 12g of Curcuma, 3g of Glycyrrhiza. Decocted 3 times, taken 3 times. If there is no obvious reaction, it can be taken continuously for 2-3 months or more.

　　Third, Western Medical Treatment Methods for Vulvar Adenoid Soft Tissue Sarcoma

　　Surgical Treatment: Radical vulvectomy with lymph node dissection can achieve local control for local vulvar masses. Shen et al. (1982) performed radical vulvectomy, right inguinal lymph node dissection, and left superficial inguinal lymph node dissection. The patient survived without tumor for 2 years after surgery. He Wen Cui et al. (1993) first gave 8 days of combined intravenous chemotherapy with fluorouracil and nitrocaphane (dis瘤芥) to a patient with lung metastasis, and the local vulvar ulcer healed, and the mass significantly shrank, but there was no obvious change in the lung metastasis focus. Continue the original chemotherapy plan and perform local vulvar tumor resection. The patient died on the 13th day after surgery due to acute severe hepatitis and hepatic encephalopathy.