Vaginal endodermal sinus tumors are a rare malignant tumor occurring in the vagina of infants and young children, with less than 25% of patients surviving for two years after onset. Endodermal sinus tumors can occur in the ovary, vulva, vagina, and cervix.
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Vaginal endodermal sinus tumors
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1. What are the causes of vaginal endodermal sinus tumors?
2. What complications are easily caused by vaginal endodermal sinus tumors?
3. What are the typical symptoms of vaginal endodermal sinus tumors?
4. How to prevent vaginal endodermal sinus tumors
5. What laboratory tests need to be done for vaginal endodermal sinus tumors
6. Diet taboos for patients with vaginal endodermal sinus tumors
7. Conventional methods of Western medicine for the treatment of vaginal endodermal sinus tumors
1. What are the causes of vaginal endodermal sinus tumors?
First, Etiology
Vaginal endodermal sinus tumors may originate from embryonic cells, and Norris calls it mesonephric carcinoma. It is speculated that this tumor may be caused by a lack of embryonic tissue conductors during the decisive period of germ cell migration, resulting in misplaced germ cells entering the upper segment of the vagina.
Second, Pathogenesis
The tumor presents as polypoid or friable medullary or grape-like masses.
The microscopic histological structure of vaginal endodermal sinus tumors under the microscope is similar to that of ovarian endodermal sinus tumors. The morphology is diverse, with cells being ovoid or polyhedral with large nuclei and protuberances. Mitotic figures are cuboidal or flat. The basic features include:
1. Typical Schiller-Duval (S-D) bodies: structures similar to 'glomerular vascular loops' or the endodermal sinus structures of rodents.
2. Reticular structure.
3. Transparent spheroid.
4. Positive for amylase PAP and with an eosinophilic basement membrane-like structure.
Immunohistochemistry can determine AFP, that is, this tumor can secrete alpha-fetoprotein (AFP).
2. What complications are easily caused by vaginal endodermal sinus tumors?
Vaginal endodermal sinus tumors may cause lymph node metastasis in patients and can be disseminated to the lung through hematogenous spread. They may also be complicated by infection, and some may experience massive hemorrhage. Therefore, timely treatment of the disease is essential.
3. What are the typical symptoms of vaginal endodermal sinus tumors?
Early cases may be asymptomatic. With the development of the tumor, asymptomatic abnormal vaginal bleeding may occur, with bloodstained diapers or vaginal discharge. Under anesthesia, a vaginal examination can reveal polypoid, friable new growths inside the vagina without a stalk, with a diameter of at least 2cm and up to 10cm, mostly located at the upper segment of the posterior vaginal wall, with 10% to 15% originating from the cervix, and with a high degree of malignancy.
Clinical staging
Due to the different primary sites, it is difficult to establish a staging system suitable for all germ cell tumors. Although there is no specific staging system for vaginal endodermal sinus tumor, the staging system established by Brodeur for germ cell tumors is often used in clinical practice. This staging is based on the resectability of the primary tumor, the extent of regional lymph node metastasis, whether there is distant spread. When this system was established, tumor markers were not routinely detected, so the system does not include tumor markers. However, in fact, AFP is very helpful for the staging of vaginal endodermal sinus tumor. Due to the emphasis on organ protection and the use of chemotherapy, the staging system based on tumor resectability is somewhat outdated.
4. How to prevent vaginal endodermal sinus tumor
Prognosis
Vaginal endodermal sinus tumor has a high degree of malignancy and poor prognosis. Since this tumor is rare, there is no large amount of 5-year survival data. The median survival period is 11 months, 10% to 15% die within 2 years, and recurrences usually occur within 12 months. In a report of 50 cases, 3 patients survived more than 5 years, the longest being 23 years. In recent years, the 5-year survival rate has reached 18% (Aartsen, 1993). Since this tumor can secrete alpha-fetoprotein (AFP), the detection of serum alpha-fetoprotein levels is used as an indicator for monitoring the therapeutic effect and tumor recurrence.
5. What laboratory tests are needed for vaginal endodermal sinus tumor
Measurement of serum AFP
Serum AFP containing asparagine linked to glycan is the most reliable tumor marker for malignant tumors, especially hepatocellular carcinoma and endodermal sinus tumor. Studies have found that AFP produced by different tumors can be separated into different isomers due to the differences in their glycan structure, which can be separated by Lectin affinity electrophoresis. The reagents include conA, LCA, E-PHA, and alloA. Serum AFP is divided into two parts by ConA, namely the non-reactive type (C1) and the reactive type (C2). Endometroid adenocarcinoma of the uterus, vaginal endodermal sinus tumor, and ovarian endodermal sinus tumor can all detect C1 and C2, while only a small amount of C1 is detected in umbilical cord blood. It is divided into three parts by LCA, namely L1, L2, and L3. Vaginal endodermal sinus tumor contains L2 and L3. The isomers of AFP from these four tissue sources are different. P2, P3, P4, P5, and a sub-type P3f can be measured by E-PHA, and vaginal endodermal sinus tumor shows P2, P3f, P4, and P5, which is significantly different from that of ovarian endodermal sinus tumor. Two main parts, A1 and A3, and a sub-type A1s can be detected by alloA, and vaginal endodermal sinus tumor shows A3, A1, and A1s, which is significantly different from AFP from other sources. Therefore, the isomer spectrum of serum AFP can be determined by Lectin affinity electrophoresis to determine the source of AFP, and the determination of serum AFP can evaluate the therapeutic effect and monitor the recurrence after treatment.
1. Ultrasound examination:It can show the size and shape of the mass inside the vagina, but the positive rate is not high. In the 3 cases reported by Handel, 2 were negative in ultrasound examination.
2. CT examination:It can show whether there is a space-occupying lesion in the vagina and whether the bladder, rectum, lymph nodes, or distant organs have metastasis.
3. MRI examination:The images provided by MRI are more accurate and comprehensive than CT and ultrasound. It can better show the location, size, and extent of the tumor, and can accurately show the edge of the tumor. Its non-invasive and non-ionizing radiation is particularly suitable for pelvic examination in infants and young children, and its contrast in soft tissues is better than that of CT examination.
4. Cystoscopy:It can show whether the bladder and urethra are invaded by the tumor.
5. Colposcopy:Regardless of the results of imaging examination, for infants and young children with unexplained vaginal bleeding, a colposcopy should be performed to see the exophytic, friable, and swollen mass originating from the vaginal wall. When a mass is found during vaginal examination and colposcopy, tissue biopsy should be performed.
6. Dietary taboos for patients with vaginal endodermal sinus tumor
1. Astragalus and Poria cocos porridge
Ingredients: 30g Astragalus membranaceus, 50g Poria cocos, and 20g glutinous rice.
Preparation: First, decoct Astragalus membranaceus and Poria cocos in water and add the washed glutinous rice to cook porridge. It has the effects of invigorating Qi, promoting diuresis, and detoxifying, and is used for patients with external malignant tumors of the vulva that do not heal.
2. Job's tears and lotus seed porridge
Ingredients: 50g Job's tears, 20g lotus seeds, and red dates.
Preparation: First, cook the lotus seeds until soft, add red dates and glutinous rice powder, and cook for 15 minutes. Take two servings in the morning and evening, which has the effects of invigorating Qi and nourishing blood, strengthening the spleen and promoting diuresis, and enhancing the body and anti-cancer effects.
3. Chestnut and yellow croaker
Ingredients: 2 yellow croaker, 10 chestnuts, scallions, ginger, garlic, cooking wine, soy sauce, salt, oil, and monosodium glutamate as needed.
Preparation method: Remove the shell of the chestnut, wash it clean. Heat oil in a pan, fry the fish on both sides, remove and set aside. Stir-fry scallion slices, ginger slices, and garlic slices, add clear soup, chestnut, salt, and soy sauce, put the fish in the soup and boil, remove the foam, cook until tender, and add monosodium glutamate when done.
Effects: Invigorates the spleen and stomach, promotes blood circulation and reduces swelling. Yellow croaker has a mild taste and sweet taste, promotes urination and reduces swelling, chestnut has a warm taste and sweet taste, nourishes the stomach and strengthens the spleen, promotes blood circulation and stops bleeding.
4. Braised winter melon
Ingredients: 300g winter melon, 12ml cooking oil, 15ml soy sauce, 30g cornstarch, 9g salt, 6ml bright oil, scallions, ginger, garlic, and some cooking wine.
Preparation method: Peel off the outer skin of the winter melon, remove the seeds and pulp, cut into pieces, and boil in a pot for 5 minutes, then remove and drain the water. Mix soy sauce, salt, scallion slices, garlic slices, ginger juice, and cornstarch with warm water to make the sauce. Heat oil in the pot over high heat, pour in the sauce and stir evenly, add the winter melon, stir-fry evenly, and turn over with bright oil when done.
Effects: Clear heat and promote diuresis. Winter melon is slightly cool in nature and sweet and bland in taste. Accompanied by a small amount of spicy scallions, ginger, and garlic, it has the effects of clearing heat and generating body fluid, detoxifying and promoting diuresis, lowering blood pressure, and lowering blood sugar.
5. Quick-fried cauliflower
Main ingredients: 150g cauliflower, 15g carrots, 15g cucumber, 15ml soybean oil, fine salt, monosodium glutamate, Sichuan pepper seeds, and sesame oil as needed.
Preparation method: Break the cauliflower into small pieces, cut the carrots into diamond-shaped slices, and blanch them in boiling water until done, then rinse with cold water and drain. Slice the cucumber and place it in the dish. Drizzle with fried Sichuan pepper oil, let it sit for a while, and then add fine salt, monosodium glutamate, and sesame oil to mix well.
Effects: Tonify Qi and invigorate the spleen. Cauliflower is sweet and neutral in taste, tonifying the middle and invigorating Qi. Cucumber is sweet and cold in nature, clearing heat. Carrots are neutral in nature and sweet in taste, with the effects of invigorating the spleen and stomach, and nourishing Qi and blood.
6. Chicken cubes with water chestnuts
Main ingredients: 1 young chicken, 10 fresh water chestnuts, 5g mushrooms, appropriate amounts of scallions, ginger, soy sauce, and other seasonings.
Preparation method: Kill the chicken, pluck the feathers, and cut the breast meat into cubes. Wash the fresh water chestnuts, peel them, and slice them. Wash the mushrooms and cook them in water until done and remove them. Pour a moderate amount of sesame oil into the pot and stir-fry the chicken cubes, then add water chestnuts, mushrooms, and seasonings to stir-fry together until the meat is cooked.
Effects: Tonify Qi and generate body fluid, nourish Yin and clear heat. Chicken is rich in nutrients and contains effective anti-cancer components. Water chestnuts are cool in nature and sweet in taste, clearing heat, quenching thirst, nourishing Yin, and anti-cancer. Mushrooms have a strong anti-cancer effect.
7. Conventional Western treatment methods for vaginal endodermal sinus tumors
1. Treatment
Like other rare diseases, due to the limited number of available cases, the biological behavior of the disease has not yet been fully understood, and therefore, there is no ideal treatment plan. From the existing cases, the disease can directly invade surrounding tissues or spread to distant sites through blood and lymph. The prognosis is poor, and untreated cases may die within 2 to 4 months after the appearance of symptoms.
Before 1965, surgery and (or) radiotherapy were used, with poor results. Radical surgery ranged from vaginal resection to complete pelvic clearance, causing loss of sexual function and fertility, and sometimes loss of the bladder and rectum. Long-term radiotherapy can cause destruction of ovarian function, abnormal growth of pelvic bones, femoral head necrosis, and bone marrow destruction, and can also lead to the occurrence of new tumors.
Since 1970, chemotherapy drugs have been part of the entire treatment for endodermal sinus tumors. The VAC regimen is currently considered the most effective chemotherapy regimen. The recommended treatment plan is conservative surgery (local resection or partial vaginal resection) combined with chemotherapy, which can improve the prognosis, reduce the incidence of complications, and preserve the fertility of the child. Conservative resection can eliminate tumor cells on the vaginal wall, making chemotherapy more effective. Even if there is effective postoperative chemotherapy, residual tumor tissue on the vaginal wall can easily lead to local recurrence.
Huang believes that conservative surgery should at least involve a partial vaginal resection. Simple tumor resection, even combined with effective chemotherapy, cannot prevent local recurrence. The level of serum AFP is a useful indicator for diagnosis and monitoring recurrence.
There have been reports of good efficacy from chemotherapy alone. Bochner reported a 16-month-old girl with an intravaginal endodermal sinus tumor who was treated with the PEB regimen for two courses. Pelvic CT showed significant shrinkage of the tumor. After continuing two more courses of chemotherapy, colposcopic examination revealed a small residual tumor on the posterior vaginal wall, biopsy showed necrotic tissue and a small amount of visible tumor tissue; two more courses of chemotherapy were administered, and the serum AFP was normal. CT showed a small residual mass on the vaginal wall, and laparotomy was performed to remove the residual lesion. Pathological tissue sections showed only fibrotic tissue and no tumor cells were seen. Follow-up for 6 years showed no recurrence. He believes that preoperative chemotherapy can reduce the tumor burden and make conservative surgery more feasible. Shen Keng from Peking Union Medical College Hospital reported two patients who were treated with chemotherapy alone (PVB regimen and PEB regimen, respectively) and the tumor tissue disappeared, and no tumor cells were found on biopsy. They were followed up for 19 and 22 months, respectively, and no recurrence was found (Table 2). Hander reported a 15-month-old girl who presented with painless vaginal bleeding of unknown etiology. Ultrasound examination did not show any abnormalities, CT showed a 2.5cm×3cm mass in the vagina, and it was suspected that the pelvic wall was eroded. Chest and abdominal CT did not show any metastases. The serum AFP was 8913ng/ml. Under general anesthesia, cystoscopy, colposcopy, and biopsy were performed, and rapid frozen sections showed vaginal grapefruits sarcoma. Routine pathological sections showed AFP-positive and Schiller-Duval bodies, and the diagnosis was intravaginal endodermal sinus tumor. After three courses of induction chemotherapy (cisplatin, etoposide, bleomycin), CT examination showed no residual lesions, and the serum AFP dropped to 8ng/ml. A repeat biopsy under anesthesia showed no residual tumor. Four months after chemotherapy, AFP increased, and CT showed a 2cm mass at the junction of the uterus and vagina. Total vaginal tumor resection was performed, and five more courses of chemotherapy were administered, and the serum AFP dropped to normal. MRI examination showed no residual mass. AFP increased again to 16000ng/ml within two months. Chemotherapy with vincristine, actinomycin D, and cyclophosphamide was then administered, and the serum AFP temporarily returned to normal. After five courses of chemotherapy, AFP began to rise, and pelvic MRI showed the tumor increased.补救化疗(ifosfamide, carboplatin, etoposide [epipodophyllotoxin] for one course) was administered, and anterior pelvic debridement was performed, including salpingo-oophorectomy, total abdominal hysterectomy, total vaginal resection, partial cystectomy, and total urethrectomy. Pelvic radiation was given during surgery, and the sigmoid colon was used to reconstruct the vagina, part of the sigmoid colon was used to repair the bladder, and the appendix was used as the urethra. Postoperative, large doses of chemotherapy drugs carboplatin, etoposide (epipodophyllotoxin), and melphalan (left旋溶肉瘤素) were administered, and previously frozen autologous peripheral blood stem cells were infused. AFP remained normal, and follow-up for 6 years showed no tumor recurrence.
Young reported 6 cases of patients who were treated with vincristine, actinomycin D, and cyclophosphamide after surgical treatment, and two of them also received radiotherapy. All patients survived for 2 to 9 years after surgery, so chemotherapy, with or without radiotherapy, can effectively control this tumor.
2. Prognosis
The malignant degree of vaginal endodermal sinus tumor is high, and the prognosis is poor. Since this tumor is very rare, there is no large amount of 5-year survival data. The median survival time is 11 months, and 10% to 15% die within 2 years. Recurrences often occur within 12 months. Among the 50 reported cases, 3 survived for more than 5 years, the longest being 23 years. In recent years, the 5-year survival rate has reached 18% (Aartsen, 1993). Since this tumor can secrete alpha-fetoprotein (AFP), the detection value of serum alpha-fetoprotein is used as an indicator for monitoring the effectiveness of treatment and tumor recurrence.
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