Pediatric Ovarian Tumors

Introduction

Introduction to pediatric ovarian tumors Ovarian tumors account for about 1% of pediatric malignancies. Rarely before the age of 5, about 20% occur in girls before menstruation, and 2/3 are from germ cells. The most common are teratoma. basic knowledge Sickness ratio: 0.5% Susceptible people: children Mode of infection: non-infectious Complications: precocious puberty

Cause

Pediatric ovarian tumor etiology

Germ cell tumor (25%):

Asexual cell tumor, endodermal sinus tumor, teratoma (mature - substantial, cystic; immature, monolayer epithelium - ovarian stroma, carcinoid, neuroectoderm, mixed), embryonal cancer, Malignant mixed germ cell tumor, polyembryoma, choriocarcinoma, gonadal tumor.

Genetic factors (30%):

It is one of the many causes of research in recent years. Most cases are autosomal dominant. In the past 10 years, molecular genetic research has made great progress. Narod et al found hereditary breast-ovarian malignant tumor (HBOC) syndrome. The specific gene that is susceptible to cancer in patients is on chromosome 17, now called BRCA1. Recently, another susceptibility gene, BRCA1, has been identified on chromosome 13. These two mutations make most epithelial ovarian malignancies inheritable. There are three main types of hereditary ovarian malignancies:

(1) High-risk patients: First, familial ovarian malignant tumor syndrome, such as mother or sister with ovarian malignant tumor, I am a high-risk patient.

(2) 50% risk: It is a breast-ovarian malignant tumor syndrome. There are one or two cancer patients in the mother or sister. The risk of ovarian malignancy is 50%.

(3) is a family history of cancer: the risk of ovarian malignant tumors, endometrial cancer, breast cancer and colorectal cancer may increase.

Pathology (30%):

(1) Histological classification:

(2) Histological classification: Histologically undifferentiated cells identified by Broder accounted for 0% to 25% of G1; undifferentiated cells accounted for 25% to 50% of G2; undifferentiated cells >50% were G3.

Prevention

Pediatric ovarian tumor prevention

Understanding the risk factors of tumors, developing appropriate prevention strategies can reduce the risk of tumors, and there are two basic clues to prevent tumors. Even if tumors have begun to form in the body, they can help the body to improve resistance. These strategies are as follows:

1. Avoid harmful substances (promoting factors) that can help us avoid or minimize exposure to harmful substances.

Some related factors of tumorigenesis are prevented before the onset, and many cancers can be prevented before they are formed. A report in the United States in 1988 compared the international malignant tumors in detail and proposed many external factors of known malignant tumors. In principle, it can be prevented, that is, about 80% of malignant tumors can be prevented by simple lifestyle changes, and traced back. In 1969, Dr. Higginson's research concluded that 90% of malignant tumors are caused by environmental factors," "Environmental factors", "lifestyle" refers to the air we breathe, the water we drink, the food we choose to make, the habits of activities, and social relationships.

2. Improve the body's immunity against tumors can help improve and strengthen the body's immune system and cancer.

The focus of our current cancer prevention efforts should first focus on and improve those factors that are closely related to our lives, such as quitting smoking, eating properly, exercising regularly, and losing weight. Anyone who follows these simple and reasonable lifestyles can Reduce the chance of cancer.

The most important thing to improve the function of the immune system is: diet, exercise and control troubles. Healthy lifestyle choices can help us stay away from cancer. Maintaining good emotional state and proper physical exercise can keep the body's immune system at its best. Tumors and prevention of other diseases are equally beneficial. Other studies have shown that appropriate activities not only enhance the body's immune system, but also reduce the incidence of colon cancer by increasing the peristalsis of the human intestinal system. Here we mainly understand the diet in preventing tumorigenesis. Some questions.

Human epidemiology and animal studies have shown that vitamin A plays an important role in reducing the risk of cancer. Vitamin A supports normal mucosa and vision. It directly or indirectly participates in most of the body's tissue functions. Vitamin A is present in animal tissues. In the liver, whole eggs and whole milk, the plant is in the form of -carotene and carotenoids, which can be converted into vitamin A in the human body. Excessive intake of vitamin A can cause adverse reactions in the body and -carrot This is not the case with carotenoids, and the low vitamin A content in the blood increases the risk of malignant tumors. Studies have shown that those with low levels of vitamin A intake in the blood increase the likelihood of lung cancer, while those with low blood levels in smokers Levels of vitamin A ingestors have the potential to double lung cancer. Vitamin A and its mixture can help remove free radicals in the body (free radicals can cause damage to genetic material), and secondly stimulate the immune system and help differentiate cells in the body. Ordered tissue (while the tumor is characterized by disorder), some theories suggest that vitamin A can help early carcinogens Invasion mutated cells become reversed the occurrence of the normal growth of cells.

In addition, some studies suggest that supplementation with -carotene alone does not reduce the risk of cancer, but rather increases the incidence of lung cancer. However, when -carotene binds to vitamin C, E and other antitoxin substances, its protective effect. It is shown, because it can increase free radicals in the body when it is consumed by itself. In addition, there are interactions between different vitamins. Both human and mouse studies have shown that the use of -carotene can reduce 40% of vitamins in the body. At E-level, a safer strategy is to eat different foods to maintain a balanced vitamin to protect against cancer, as some protective factors have not been discovered so far.

Vitamin C, E is another anti-tumor substance that prevents the harm of carcinogens such as nitrosamines in food. Vitamin C protects sperm from genetic damage and reduces the risk of leukemia, kidney cancer and brain tumors in their offspring. Vitamin E can reduce the risk of skin cancer. Vitamin E has the same anti-tumor effect as vitamin C. It is a scavenger that protects against toxins and scavenges free radicals. The combination of vitamins A, C and E protects the body against toxins. Better than applying it alone.

At present, research on phytochemistry has attracted widespread attention. Phytochemistry is a chemical found in plants, including vitamins and other substances found in plants. Thousands of plant chemicals have been found, many of which have anticancer properties. The protective mechanism of these chemicals not only reduces the activity of carcinogens but also enhances the body's immunity against carcinogens. Most plants provide antioxidant activity that exceeds the protective effects of vitamins A, C, and E, such as a cup of cabbage. Contains 50mg of vitamin C and 13U of vitamin E, but its antioxidant activity is equivalent to the antioxidant activity of 800mg of vitamin C and 1100u of vitamin E. It can be inferred that the antioxidant effect in fruits and vegetables is far better than what we know. The effect of vitamins is strong, and no doubt natural plant products will help prevent cancer in the future.

Complication

Pediatric ovarian tumor complications Complication precocious

Causes precocious puberty, which can cause tumor and pedicle torsion, bleeding and necrosis, which can cause urination and difficulty in defecation.

Symptom

Pediatric ovarian tumor symptoms Common symptoms Estrogen in the urine increased appetite, abdominal distension, acute abdomen, leukocytosis, abdominal pain, lower abdomen, bulging, calcification, nausea and weakness

1. The tumor with less clinical manifestations generally does not produce symptoms. Occasionally, the affected side has a feeling of sinking or holding pain in the lower abdomen. It can clearly touch the abdominal mass. The surface is smooth, no tenderness, and there is sac sexy. Most benign tumors form a fallopian tube. The long stem pedicle, because the tumor and the surrounding tissue are more non-adhesive, so the mobility is greater, often the mass can be moved from the lower abdomen side to the upper abdomen.

Malignant tumors grow rapidly, the tumors are irregular, no mobility, and may be associated with ascites. In the short term, systemic symptoms such as weakness, fever, loss of appetite, etc. occur.

Functional ovarian tumors, such as granulosa cell tumors, can cause symptoms of precocious puberty due to the production of large amounts of estrogen. Female characteristics such as physique, breast, and external genitalia are rapidly developed, and menstruation occurs, but ovulation does not occur, and bone development can exceed normal range. Estrogen in the urine is increased, and gonadotropin in the urine is also elevated, which exceeds the general rule and reaches the human level.

A medium-sized, long ovarian mass (including a sacral ovarian cyst) can cause tumor and pedicle torsion. Once reversed, hemorrhage and necrosis can occur. Clinically, it is acute abdomen. The child may have abdominal pain and nausea. Or vomiting, abdominal muscle tension in the tumor site, tenderness is obvious, children may have elevated body temperature and leukocytosis, when the tumor is larger, oppressing adjacent organs, can cause urination and difficulty in defecation.

2. Types of tissue This article only briefly describes ovarian tumors that are more common in children:

(1) dysgerminoma (dysgerminoma): also known as germ cell carcinoma, is the most common malignant germ cell tumor in children and adolescence, morphologically and biologically equivalent to testicular germ cell carcinoma and pineal region, anterior mediastinum, Retroperitoneal extragonadal cell carcinoma.

Abdominal masses have relatively rapid development of symptoms and signs. In addition to tumor torsion, abdominal pain is not common. 75% of cases are diagnosed as stage I. There may be local spread, regional lymph node metastasis and long-distance transfer to the lung, liver or supraorbital lymph nodes. Asexual cell tumor is a large nodular tumor, up to 20cm in diameter, mostly occurs on the right side, bilaterally occurring 5% to 10%, 14% to 25% is mixed type dysplasia, that is, other reproduction Cell tumor components, such as gonadal cell tumors, immature teratomas, endodermal sinus tumors, mature teratomas and choriocarcinoma.

If the tumor is limited to the ovary, only the affected side of the ovary and fallopian tube resection, with or without radiotherapy, the survival rate is similar, up to 80%, if the II ~ IV period, comprehensive treatment is required.

Ovarian tumor staging according to FIGO (international federation of gynaecology and obstetrics):

Stage I: The tumor is confined to the ovary

Stage II: The tumor invades the unilateral or bilateral ovaries and has pelvic diffusion.

Stage III: The tumor invades the unilateral or bilateral ovaries and has intraperitoneal metastases that spread to the pelvic and/or retroperitoneal lymph nodes.

Stage IV: long distance transfer.

(2) Endodermal sinus tumor: almost all of the highly malignant embryonal epithelial tumors, characterized by embryonic cells, which are loosely networked and resemble the special perivascular structures of the mouse placenta endodermal sinus. There are also PAS-positive hyaline corpuscles inside and outside the cell. This tumor, also known as yolk sac tumor, contains many small vesicles similar to yolk sac vacuoles.

The tumor has a high degree of malignancy and rapidly spreads to the lymphatic and abdominal tissues. Therefore, the course of disease is short, often accompanied by abdominal pain. Most of the diagnosis is in stage III. The average age at diagnosis is 18 to 19 years old. It can be detected that serum AFP is increased. Infants (<6 months) may also have an increase in AFP.

Surgery plus multiple drug combinations such as VAC regimen (vincristine, actinomycin D, cyclophosphamide) or PVB (cisplatin, vinblastine sulfate and bleomycin) can improve survival rates by 45% to 72%.

(3) embryonal carcinoma (embryonal carcinoma): about 4% of ovarian malignant tumors, the average age of diagnosis is 14 years old, except for abdominal masses, half of the patients have abdominal pain, the tumor surface is smooth, the maximum diameter can reach 10 ~ 20cm, although Microscopically like endodermal sinus tumor, but the cells do not differentiate, mitotic figures, hemorrhage and necrosis are more significant, no Schiller-Duval body, the incidence of clinical endocrine performance is higher, including pregnancy-positive reaction, HCG increased, diagnosis 60 % is a stage I lesion, occasionally bilateral lesions, stage I lesions only for the affected side of the ovary and fallopian tube resection, the survival rate of up to 50%, radiotherapy effect is not obvious, chemotherapy can refer to endodermal sinus tumor.

(4) Teratoma: It is the most common in germ cell tumors and can be divided into mature type (99%) and immature type (1%). Mature type includes:

1 typical cystic and solid teratoma, often including 3 germ layers;

2 single germ layer lesions include thyroid tissue, carcinoid, neuroectodermal tumor or thyroid carcinoid (thyroid carcinoid), in addition to neuroectodermal lesions, these tumors in children and adolescence are benign, but reported malignant changes, immature The type accounts for 7.4% of children with malignant ovarian tumors. The average age at diagnosis is 11 to 14 years old. Half of them occur before menstruation. Except for abdominal masses, they often have abdominal pain. Because of rapid growth and infiltration of the capsule, 50% of patients under operation The tumor has exceeded the ovary, and the tumor can spread to the peritoneum, regional lymph nodes, lungs and liver. If the tumor is broken, the prognosis is poor.

Typical tumors have an envelope with a maximum diameter of 15-20 cm. The cut surface has cystic and parenchymal parts. According to the neuroepithelial content of ovarian tumors, it is divided into different grades:

Level 0: Only mature organizations.

Grade 1: Mainly mature tissue, some immature components, one slice only sees the neuroepithelial at a low power field.

Grade 2: a moderate amount of immature components, one slice can be seen in 1 to 3 low power field neuroepithelial.

Level 3: A large number of immature components, one slice visible 4 low power field neuroepithelial.

In summary, the level of immature is judged according to the amount of neuroepithelial, and the grade above 0 is malignant.

If the lesion is in stage II or lower, only the affected side of the ovary and fallopian tube can be removed. Otherwise, chemotherapy should be added, and VAC and cisplatin should be used to control local recurrence and metastasis.

(5) Malignant mixed germ cell tumor: 20% of ovarian germ cell tumors in children and adolescents and 8% of malignant ovarian germ cell tumors. The average age at diagnosis is 16 years old, 40% is menstruation. Before the menarche, AFP and HCG should be measured before surgery. Because the tumor can contain endodermal sinus tumor and embryonic cancer components, bilateral lesions can be as much as 20%, so the contralateral ovaries must be examined during surgery.

The prognosis is determined by the tissue structure, and the survival rate is about 50%. The prognosis can be improved by chemotherapy such as vincristine, actinomycin D, cyclophosphamide, and cisplatin, vinblastine sulfate, and bleomycin.

(6) Granulosa-theca cell tumor: 3% of pediatric ovarian tumors, the average age at diagnosis is 8 years old, 60% of the sick children have precocious puberty, mostly unilateral lesions, malignant tendency Low, do the affected ovarian and fallopian tube resection, radiotherapy, chemotherapy only for advanced and recurrent cases.

(7) Epithelial tumors: rare in prepubertal girls, pathologically can be divided into serous, mucinous, endometriosis (endometriosis) or clear cell tumors, showing varying degrees of cell morphology can be benign or malignant The edges are up to malignant.

Examine

Pediatric ovarian tumor examination

1. Ascites cytology examination of the lower abdomen axillary puncture, such as less ascites can be puncture through the posterior iliac crest, ascites water to check cancer cells.

2. Tumor marker determination

(1) CA125: It has important reference value for the diagnosis of ovarian epithelial cancer, especially serous cystadenocarcinoma, followed by endometrial carcinoma, the positive rate of serous cystadenocarcinoma is above 80%, and more than 90% CA125 The level is slowed down or worsened, so it can also be used as post-treatment monitoring. The positive rate of advanced ovarian cancer is high, but the positive rate of stage I ovarian malignant tumor is only 50%. Clinically, CA12535U/ml is a positive standard, CA125 is not Specificity, some gynecological non-malignant diseases such as acute pelvic inflammatory disease, endometriosis, pelvic and abdominal tuberculosis, ovarian cysts, uterine fibroids and some non-gynecological diseases also have elevated CA125 values.

(2) AFP: specific for ovarian endodermal sinus tumors, mixed tumors containing endodermal sinus tumor components, dysgerminoma and embryonal tumors, some immature teratomas can also be elevated, AFP can be used as germ cell tumor Important markers before and after treatment and follow-up, normal value <29g / L.

(3) HCG: The abnormality of HCG in blood of patients with germ cell tumor of primary ovarian choriocarcinoma is negative, and the HCG value of serum B subunit of normal non-pregnant women is negative or <3.1 mg/ml.

(4) CEA: Some ovarian malignant tumors, especially mucinous cystadenocarcinoma, have abnormally elevated CEA, but they are not specific antigens of ovarian tumors.

(5) LDH: elevated LDH in serum of some ovarian malignant tumors, especially anaplastic tumors are often elevated.

But it is not an ovarian tumor-specific indicator.

(6) Sex hormones: granulosa cell tumor, follicular tumor can produce higher levels of estrogen; when luteinized, it can also secrete testosterone, serous, mucinous or fibroepithelial neoplasia can sometimes secrete a certain amount of estrogen.

3. Flow cytometry Cell DNA assay Flow cytometry (Fcm) method Flow cytometry analysis of DNA square map to understand tumor DNA content, ovarian malignant tumor DNA content and tumor histological classification, classification, clinical stage, recurrence In terms of survival rate, Khoo performed Fcm examination on 53 cases of stage III ovarian malignant tumors, and found that 30% of the tumors were DNA diploids, and the survival rate of those patients with diploid tumors (65%) was significantly better than that of heterodiploid (31). %), Vergote explored the relationship between the prognosis of 290 cases of stage I epithelial ovarian malignant tumors and DNA ploidy, and found that diploid tumors are mostly stage I G1, mucinous or endometrioid carcinoma, while heterodiploid tumors are mostly Ic phase G3, serous or clear cell carcinoma, low-risk group (diploid tumor) without recurrence, while high-risk group (heterodiploid tumor) recurred 25%, Trope et al considered DNA ploidy analysis is second only to tissue An important prognostic indicator for grading.

Film degree exam:

1. Ultrasonography is an important means to diagnose ovarian tumors. It can determine the size, location, texture, relationship with the uterus and the presence or absence of ascites. The judgment of benign and malignant depends on experience, up to 80% to 90%, but the tumor Ultrasound diagnosis is difficult in patients below 2cm, vaginal ultrasonography, especially vaginal color Doppler ultrasonography can show changes in blood flow within the tumor, providing a reference for the identification of benign and malignant.

2. CT, and MRI examination to determine the tumor size, texture, and the relationship between the pelvic organs, especially for the pelvic and aortic lymph node enlargement has a certain value.

3. Lymphangiography can show the iliac vessels and para-aortic lymph nodes and their metastatic signs, providing preoperative evaluation and lymph node dissection preparation.

4. Select the following check if necessary

Gastroscope, colonoscopy: to identify ovarian metastatic carcinoma of the primary gastrointestinal cancer.

Intravenous pyelography: understanding the secretion and excretion function of the kidney, urinary tract compression and obstruction symptoms.

Radioimmunoimaging: Tumor-positive imaging was performed using a radionuclide-labeled antibody as a tumor-positive imaging agent.

Laparoscopy: For pelvic masses that are difficult to be clinically diagnosed, laparoscopic biopsy is performed in patients with ascites, and ascites is used for qualitative and preliminary clinical staging of pathology and cytology.

Diagnosis

Diagnosis and diagnosis of ovarian tumors in children

diagnosis

According to the history, the growth of the mass and the greater mobility, it can be diagnosed as ovarian tumor, but a few lumps fixed in the pelvic cavity can not exclude ovarian tumors. Ovarian teratoma can show calcification on the abdominal plain film, bones and teeth. Shadow.

Check the lower abdomen mass, emphasize the urinary or catheterization of the child, and empty the bladder. The abdominal examination combined with the rectal examination for double examination to exclude the filling of the bladder, while pushing the mass, pay attention to whether the uterus is pulled or not. Determine the relationship with the uterus.

Ultrasound and CT examinations can assist with localization and characterization. Chest radiography can detect lung and thoracic lymph node metastasis. The measurement of tumor markers, AFP, HCG and LDH, is also important to determine treatment planning and monitor tumor behavior.

After the clinical stage, laparotomy was performed to remove the tumor and determine its tissue type.

In the Children's Hospital from 1956 to 1980, 4524 cases of solid tumors confirmed by pathological sections had 144 cases (3.2%) of ovarian tumors, including 91 cases (63.2%) over 7 years old.

Differential diagnosis

When the mass is larger and grows into the abdominal cavity, it is feasible to have barium meal angiography, which should be differentiated from other intra-abdominal or retroperitoneal masses. Clinically, patients who have misdiagnosed ectopic kidneys as ovarian tumors and have surgical exploration should pay attention.

When the ovarian tumor is reversed, it must be differentiated from other acute abdomen such as appendicitis and Meckel's diverticulitis.

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