Vulvar epithelioid sarcoma

　　1. Etiology

　　The histological origin of vulvar epithelioid sarcoma is unknown. Since the microscopic view shows mainly epithelioid cells, Enzinger et al. (1970) officially named it epithelioid sarcoma.

　　2. Pathogenesis

　　Vulvar epithelioid sarcoma is a solid mass, with 1 or more firm nodular masses without capsules, showing a glossy surface that can be fish-like, pale red, grayish white, or dark yellow. It is脆 and soft, but some lesions with more fibers are more tenacious. Larger lesions may have hemorrhage and necrosis in the center, surrounded by dense fibrous tissue.

　　Under light microscopy, the characteristic of this tumor is central necrosis in the nodule, with tumor cells arranged in a lattice-like pattern around the necrotic area. The periphery of the nodule is fibrosis of collagen fibers, also scattered among the tumor cells. The tumor cells are more epithelioid and have a certain arrangement as they approach the center of the nodule, while near the periphery, the tumor cells are fusiform. The cytoplasm of the tumor cells is large and eosinophilic. The nuclei are round, oval, vacuolated, with prominent nucleoli and multiple mitotic figures. Tumor cells can appear calcified, ossified, mucinous, cystic, and fissured.

　　Under electron microscopy, there is no basement membrane around the tumor cells, with abundant rough endoplasmic reticulum and Golgi apparatus in the cytoplasm. Intermediate filaments are abundant and arranged in longitudinal arrays similar to fibroblasts. There are finger-like cell processes and bridge-like connections are formed.

　　Immunohistochemistry shows bidirectional expression, that is, both epithelial and mesenchymal antibodies are positive. Vimentin, cytokeratin, EMA are all diffusely positive. In the various types of cytokeratin, CK7, CK8, CKl3, CKl6, CKl8, and CKl9 are all positive. Most cells express CD34 and β-catenin positively, occasionally desmin, HMB45, and other positive expressions can be seen, while S-100, CD31 are mostly negative.

　　Early skin ulcers, deep ones often grow along the deep fascia, are hard and tough, and the nodular areas are not easy to move. Sometimes there is pain or tenderness. Easy to have complications such as infection. Associated with regional lymph node metastasis, the later stage often metastasizes to the lungs.

　　Vulvar sarcoma often occurs in the large labia, clitoris, and around the urethra. Initially, the mass is very small, located under the skin, and may have no symptoms, and can be found during physical examination or by chance. Later, the mass gradually increases, invades the skin to form ulcers, and when complications such as infection occur, pain and bleeding may occur. Some are manifested as progressively enlarged nodules, which may be single or multiple, generally 1.5-4.0cm in size, with some as large as 9cm and as small as 0.5cm. Superficial tumors may cause skin ulcers early, while deep ones often grow along the deep fascia, are hard and tough, and the nodular areas are not easy to move. Sometimes there is pain or tenderness.

　　Regular physical examinations, as early as possible to detect and treat early, do a good follow-up work prognosis: early diagnosis, early extensive resection of lesions will help improve the prognosis. Follow-up 0-312 months (average 56 months), 11 cases survived, accounting for 47.8%. Nine patients had local recurrence within 1-24 months after surgery, died 4.5-180 months after recurrence, all died of distant metastasis. Common sites of metastasis include the lungs and regional lymph nodes, and there may also be metastasis to the scalp, liver, and other sites. Factors related to poor prognosis include: proximal type, tumor >5cm, vascular invasion, distant metastasis, and high nuclear division number, etc.

　　The diagnosis of vulvar epithelioid sarcoma, in addition to relying on clinical manifestations, also requires related examinations, which are indispensable. The methods of examination are as follows:

      1, Secretion examination, tumor marker examination, immunological examination.

　　2, Histopathological examination.

　　I, Dietetic recipe for vulvar epithelioid sarcoma

　　1, Sophora Flavescent Egg: 2 eggs, 60 grams of brown sugar, 60 grams of Sophora Flavescent. Boil Sophora Flavescent to extract the juice, add the beaten eggs and brown sugar, and cook until done. Eat the egg and drink the soup. Once a day, for 6 days as a course of treatment. It has the effect of clearing heat and detoxifying, drying dampness, and healing sores, and can be used for the prevention and treatment of vulvar ulcers.

　　2, Longdan Coptis Egg: 10 grams of Longdan Coptis, 3 eggs, 30 milliliters of honey. Boil Longdan Coptis and remove the dregs, beat the eggs to make a fried egg, add honey, and take it on an empty stomach, once a day, for 5 days as a course of treatment. It has the effect of clearing heat, removing dampness, and healing sores.

　　3. Cabbage and mung bean sprout drink: one root of cabbage, 30 grams of mung bean sprouts. Clean the cabbage root and slice it, clean the mung bean sprouts and put them in a pot, add an appropriate amount of water, boil for 15 minutes, remove the dregs and take the juice, drink it as tea, without restrictions on time. It has the effect of clearing heat and removing dampness, which can be used for the treatment of vulvar ulcers.

　　4. Rock sugar winter melon soup: 30 grams of winter melon seeds, 30 grams of rock sugar. Clean the winter melon seeds, grind them into coarse powder, add rock sugar, pour a bowl of boiling water, put it in a clay pot, and simmer over low heat. Take 2 times a day, and take it for several days. It has the effect of clearing heat and eliminating dampness, which can be used for the treatment of vulvar itching.

　　5. Cang'er stomach: one stomach, 30 grams of Cang'erzi, 10 grams of Fructus psoraleae. Clean the stomach, add water, and boil with the latter two drugs together, discard the medicine and eat the stomach. It has the effect of kidney-nourishing, wind-dispelling, and itching-stopping. It is used for the prevention and treatment of atrophic vulvar leukoplakia.

　　6. Goji berry congee: 20 grams of goji berries, an appropriate amount of rice. Cook and eat as congee.

　　Two, what to eat for vulvar epithelial sarcoma that is good for the body

　　1. Eat more foods that have anti-vulvar tumor and leukoplakia effects, such as sesame, almonds, wheat, barley, loofah, black-bone chicken, cuttlefish, black rat snake, pork pancreas, chrysanthemum, umeboshi, peach, lychee, purslane, chicken blood, eel, abalone, crab, horseshoe crab, sardine, clam, sea turtle.

　　2. For pain, eat horseshoe crab, red, lobsters, sea asparagus, sea cucumber, tiger fish, beetroot, mung beans, radish, chicken blood.

　　3. For itching, eat amaranth, cabbage, mustard greens, taro, kelp, purple kelp, chicken blood, snake meat, pangolin.

　　4. To enhance physical fitness and prevent metastasis, eat silver ear, black fungus, mushrooms, monkey head mushrooms, chicken gizzards, sea cucumber, Job's tears, walnuts, crabs, Agama, needlefish, etc.

　　5. After vulvar epithelial sarcoma surgery, it consumes Qi and injures blood, so it is advisable to eat more Qi-nourishing and blood-nourishing foods, such as jujube, longan, mung bean, glutinous rice, lychee, mushrooms, carrots, quail eggs, lotus root powder, beans, etc.

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

　　7. Chemotherapy after vulvar epithelial sarcoma surgery: it is easy to damage both Qi and blood, so it is advisable to often eat foods that nourish Qi and blood, such as mushrooms, walnuts, lotus seeds, mulberry, Congee, jujube, longan, sea cucumber, etc.

　　Three, it is best not to eat those foods with vulvar epithelial sarcoma

　　1. Avoid foods that cause itching and inflammation, such as fish, shrimp, crab, chicken heads, pork heads, goose meat, chicken wings, chicken feet, etc., as eating them will worsen the itching and inflammation of the vulva.

　　2. Eat less spicy and刺激性 foods. For example: onions, pepper, chili, Sichuan pepper, mustard greens, fennel, etc.

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

　　4. Quit smoking, drinking, coffee, and other stimulating beverages.

　　Precautions before the treatment of vulvar epithelial sarcoma

　　1. Develop good living habits, quit smoking and limit alcohol. Eat some cancer-preventive foods and alkaline foods with high alkaline content, and maintain a good mental state.

　　2. Have a good attitude towards stress, combine work and rest, and do not overwork.

　　3. Pay attention to the hygiene of the vulva, avoid long-term stimulation of secretions. Treat vulvar itching actively, and pay attention not to use strong irritant drugs to clean the vulva.

　　4. Strengthen physical exercise, enhance physical fitness, exercise more in the sun, and sweating can expel acidic substances in the body with sweat, avoiding the formation of an acidic constitution.

　　5. If nodules, ulcers, and white lesions on the vulva are found, medical treatment should be sought promptly.

　　Second, the Western medical treatment method for vulvar epithelioid sarcoma

　　After the diagnosis of treatment, immediate radical vulvectomy should be performed, at least a wide local excision of the lesion and inguinal lymph node dissection. Or cryotherapy, laser treatment. However, before treatment, a careful examination is required to exclude invasive cancer. In view of the high local recurrence rate and distant metastasis rate of 77% to 87% and 45% to 58% respectively for epithelioid sarcoma, wide local excision of the lesion should be preferred, including at least 2cm of surrounding normal tissue, and it is emphasized to retain the clitoris as much as possible. The treatment of regional lymph nodes is best achieved by suspicious lymph node biopsy resection. In addition, radiotherapy and chemotherapy are often used as adjuvant treatment after surgery, and the efficacy of radiotherapy and chemotherapy alone is not reliable.

　　1. For sarcomas with late stages of antineoplastic chemotherapy and active nuclear division on tissue, combined chemotherapy before and after radical surgery can improve the prognosis. Currently, commonly used antineoplastic chemotherapy regimens for the treatment of soft tissue sarcomas include:

　　(1) VAC regimen: Vincristine (vincristine) 1.5mg, intravenous injection, days 1 and 8; Actinomycin or Actinomycin D (Krestin) 400-600; intravenous injection, days 1 to 4; Cyclophosphamide (cyclophosphamide) 300mg, intravenous injection, days 1, 4, 8. Repeat every 3 to 4 weeks, but subject to the recovery of bone marrow function. It has been reported that this regimen can extend the survival period of pelvic sarcoma by 4 to 5 times (Rivard, 1975).

　　(2) ADIC regimen: Doxorubicin (adriamycin) 60mg, intravenous infusion, day 1; Dacarbazine (thiotepa) (DTIC) 250mg/?, intravenous infusion, days 1 to 5. Effective rate 42% (Beretta, 1983).

　　(3) CYVADIC regimen: Cyclophosphamide 500mg, intravenous infusion, day 2; Vincristine 1.5mg, intravenous injection, days 1 and 8; Dacarbazine (thiotepa) 250mg/m2, intravenous infusion, day 2. Treatment interval 4 weeks. Effective rate 47%.

　　(4) IVA regimen: Ifosfamide (ifosfamide) 3mg/m2, intravenous infusion, days 1 to 2. Sodium mesylate (mesna) 600mg/m2, intravenous injection, days 1 to 2 (0, 4, 8h/d). Vincristine 1.5mg/m2 (maximum dose 2mg), intravenous infusion, day 1. Actinomycin D 1.5mg/m2 (maximum dose 2mg), intravenous infusion, day 1.

　　2. For patients with localized malignant lymphoma, surgery should be performed first, followed by chemotherapy. The commonly used regimen is

　　(1) COP regimen: Cyclophosphamide 800mg., intravenous injection, days 1 and 15; Vincristine 1.4mg, intravenous injection, day 1; Prednisone (Prednisone) 100mg, oral, days 1-5. Repeat one course every 3 weeks, with an efficacy rate over 80% (Pan Qichao, 1989).

　　(2) CHOP regimen: Cyclophosphamide 750mg. Intravenous infusion, day 1; Doxorubicin (Adriamycin) 50mg, intravenous infusion, day 1; Vincristine 1.4mg/m2, intravenous injection, day 1; Prednisone (Prednisone) 100mg, oral, days 1-5. Repeat one course every 3 weeks, with an efficacy rate of over 90%.

　　3. Adjuvant Radiotherapy: Adjuvant radiotherapy is beneficial, can reduce the local recurrence rate after surgery, and can also achieve short-term cure when combined with chemotherapy.

　　4. Cryotherapy: Cryotherapy can be used when it is not suitable to excise multiple lesions.

　　5. Laser Therapy: Before treatment, a colposcopy should be performed for a comprehensive examination to clarify the edge of the disease. The depth of treatment is determined according to the size of the lesion and the VIN level. Generally, the depth of laser treatment on hairless areas is 1mm. In areas with hair, the treatment depth should be less than 2mm.

　　6. Others: including local application of 5-Fu ointment, subcutaneous injection of interferon, and the application of retinoic acid, etc.

　　The epithelioid sarcoma occurring on the vulva is more malignant than that occurring outside the reproductive tract, and the prognosis is poor.

　　This disease is prone to recurrence and metastasis, and recurrence is often multifocal. The recurrence rate is 65% to 77%, even up to 85%, mainly depending on whether the extent of the first resection is sufficient. 45% to 75% of cases can occur metastasis, the most common initial site of metastasis is lymph nodes (48%), followed by the lung (25%), and can also metastasize to the scalp, bone, and brain and other tissues. Other adverse prognostic factors include age at diagnosis, tumor size (greater than 5cm), involvement of deep tissues, lymph node metastasis (early metastasis, poor prognosis), location (distal type has better prognosis, while proximal type is more aggressive), and female 5-year survival rate can reach 80%.