Vulvar malignant granulocytic tumors

　　1. Etiology

　　It was previously believed that malignant granulocytic tumors originated from myoblasts (termed malignant granulocytic myoblasts), histiocytes, fibroblasts, and undifferentiated mesenchymal cells. However, recent studies using immunohistochemistry and electron microscopy have shown that they are derived from Schwann cells.

　　2, Pathogenesis

　　The skin surface of the tumor is intact. The mass boundary is unclear, so the tumor often has surrounding fat and muscle. The cut surface of the tumor is grayish yellow.

　　Under light microscopy, the tumor cells are of unequal size, forming nests or strands, with round, polygonal, oval, or spindle-shaped cytoplasm. The cytoplasm is rich and filled with similar-sized, unevenly distributed eosinophilic granules, and is positive for PAS. The nuclei are of unequal size and deeply stained, with mitotic figures >2/10HPF, and coagulative necrosis can be seen. The diagnosis is more definite if metastasis is observed. Under electron microscopy, the cytoplasmic granules are autophagic vesicles, and myelinated or unmyelinated axon-like structures can also be seen.

　　The tumor cells are positive for S-100 protein and neuron-specific enolase (NSE), and CD68 and CD57 (Leu-7) can be positive, but the specificity is not strong.

　　Vaginal malignant granulosa cell tumor can lead to the following complications:

　　Granulocytic myoblastoma of the respiratory tract: Due to the tumor cells' ability to invade the upper respiratory tract and gastrointestinal tract, it can lead to granulocytic myoblastoma in the respiratory tract. Generally, there are no obvious symptoms, but when the tumor grows, it may present with a sensation of foreign body in the upper respiratory tract, with symptoms such as runny nose and nasal congestion. It can metastasize to the lungs, liver, and bones through lymphatic vessels.

　　Clinically, there are no specific symptoms, and it is often discovered incidentally, most commonly in the labia, occasionally in the clitoris. It is usually solitary, and multiple nodules are rare. It usually presents as painless, solitary nodules in the dermis or subcutaneous tissue, with sizes ranging from 1.5 to 12cm and a hard texture.

　　Preventive measures should be taken according to the three levels of tumor prevention. Clinical factors significantly associated with the invasiveness and poor prognosis of this tumor include tumor size >4cm, history of local recurrence, rapid growth of the mass, invasion of surrounding tissues, and advanced age. In addition, high Ki-67 and p53 positivity also suggest a poor prognosis.

　　The prognosis of this tumor is poor, with rapid growth and a high local recurrence rate. Among the 6 cases, except for 1 case with no local recurrence after a follow-up of only 16 months, the other 5 cases all had local recurrence, which occurred between 2 to 6 years post-operation (median recurrence time of 3 years). The tumor is prone to distant metastasis and can metastasize through lymphatic vessels to regional lymph nodes or via hematogenous routes to the lungs, liver, and bones. Among the 6 patients, 1 had lymph node and lung metastasis, and 3 had regional lymph node metastasis. Two cases were followed up for 90 months and 35 months, respectively, and both survived (Horowitz 1995, Magori 1973); 1 case died after a follow-up of 91 months (Horowitz 1995); 1 case survived with the tumor for 24 months (Fanburg 1998); the other 2 cases survived without tumor, with follow-up times of 16 months and 68 months, respectively (Ramos 2000, Robertson 1981). Due to previous reports of distant metastasis occurring 10 years later, long-term follow-up is necessary.

　　The examination methods for external genital malignant granulosa cell tumor should pay attention to:

      1, Tumor marker examination, p53 tumor suppressor gene detection, immunohistochemical detection.

　　2, Histopathological examination.

　　One, Dietetic recipes for external genital malignant granulosa cell tumor

　　1, Fresh mushrooms 90 grams of fresh mushrooms, fried with a moderate amount of vegetable oil and a little salt, then boiled into a soup for consumption.

　　2, Enoki mushrooms An appropriate amount of enoki mushrooms, decocted into a soup, cooked, or ground into powder for consumption.

　　3, Fresh water chestnuts 20-30 fresh water chestnuts, add an appropriate amount of water, simmered into a thick soup, taken in 2-3 doses.

　　4, Oolong tea Regular consumption of oolong tea has a certain anticancer effect.

　　5, Bee honey Regular consumption of bee honey can enhance the body's immunity and has anticancer effects.

　　6, Sprouts The chlorophyll in sprouts can prevent colorectal cancer and other cancers.

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

　　Two, What is good for the body when eating external genital malignant granulosa cell tumor

　　1, Foods should be as diverse as possible, with a high protein, high vitamin, low animal fat, and easily digestible diet, as well as fresh fruits and vegetables. Avoid consuming stale, deteriorated, or刺激性 foods, carbonated drinks, and gas-producing foods. Eat less smoked, roasted, pickled, fried, and overly salty foods. Combine coarse and fine grains in staple foods to ensure nutritional balance and prevent bloating, diarrhea, and constipation.

　　2, To prevent the decrease of white blood cells and platelets caused by chemotherapy, it is advisable to consume more blood and meat, such as animal organs, egg yolks, lean meat, fish, eel, chicken, and bones; at the same time, medicated diet can be used in combination, such as Codonopsis, Astragalus, Angelica sinensis, jujube, peanuts, etc.

　　3, To enhance immune function, foods such as mushrooms, shiitake mushrooms, hedgehog mushrooms, and black fungus can be consumed.

　　4, To increase appetite and prevent vomiting, one can change the menu, alter cooking methods, enhance the color, aroma, and taste of food; eat small and frequent meals, consume some light and refreshing cold dishes; add some ginger to the diet to stop vomiting; also, medicated diet can be used to improve appetite and invigorate the spleen, such as hawthorn meat cubes, Astragalus, yam, radish, and dried tangerine peel.

　　5, Diet should be light and nutritious, correcting malnutrition and abnormal eating habits. It is not advisable to consume刺激性, seafood, etc. frequently.

　　6, Suitable foods: milk, spinach, yam, cabbage, rapeseed, mushrooms, lean meat, eggs, crucian carp, apples, pears, jujube, peanuts, black rice, etc.

　　Third, for malignant vulvar granulosa cell tumors, it is best not to eat those foods

　　1. Forbidden foods: crab, mackerel, crucian carp, goose meat, chili, oranges, etc.;

　　2. Avoid smoking and alcohol;

　　3. Avoid刺激性 foods such as scallions, garlic, peppers, and cinnamon;

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

　　5. Avoid warm and blood-activating foods such as mutton, dog meat, leeks, pepper, etc.

　　First, Adult Type

　　Granulosa cell tumors should be followed up every 3 months initially, and then re-examined every 6 months after 1 year. It is important to adhere to long-term follow-up. Since recurrence has been reported after 5 years, 10 years, and even 20 years after surgery for adult granulosa cell tumors, Lauszus et al. (2001) reported that the 5-year survival rate of clinical stage I granulosa cell tumors is 94%, 10 years is 82%, and 20 years is 62%, so follow-up for 5 to 10 years, or even longer, is recommended.

　　Second, Juvenile Type

　　Granulosa cell tumors: About 5% of juvenile granulosa cell tumors are malignant, characterized by rapid recurrence, generally spreading widely in the abdominal cavity within 2 years after initial diagnosis, so strict follow-up after surgery is more important for these patients. The follow-up time should also be once every 3 months, and once every 6 months after 1 year is recommended.

　　Third, Follow-up Content

　　1. General physical examination: Pay special attention to the abdomen, feel for lumps and the occurrence of ascites.

　　2. A careful and thorough gynecological pelvic examination should be sensitive to the thickening and nodules appearing in the pelvic floor, uterine rectal pouch lateral fornix, etc., to understand whether there are recurrent foci.

　　3. Endocrine Examination: Monitor the changes of vaginal smear, detect the changes of hormone levels in blood and urine.

　　4. Imaging Examination: X-ray chest film examination to understand whether there is distant metastasis; pelvic B-ultrasound MRI, CT can understand whether there is metastasis of pelvic and abdominal organs and whether there is recurrence in the surgical area.

　　5. Tumor Marker Measurement: This is the most meaningful follow-up monitoring method. Valuable results have been achieved abroad, as reported by Long et al. (2000). The ultra-sensitive enzyme-linked immunosorbent assay (ELISA) for anti-Müllerian hormone (AMH) can detect early recurrent foci or preclinical lesions.