Adolescent and Pediatric Vaginal Sarcoma
Introduction
Introduction to adolescents and pediatric vaginal sarcoma Primary vaginal malignancies are uncommon, accounting for about 1% of gynecologic malignancies, and vaginal sarcomas account for 2% of vaginal malignancies. It is reported in the literature that embryonal rhabdomyosarcoma (glucopyosarcoma) is the most common in vaginal sarcoma and occurs mostly in infants and young children. Embryonic rhabdomyosarcoma occurs mostly in the anterior wall of the vagina, and sometimes the exact location of the lesion cannot be determined. The tumor is small papillary in the early stage, with a diameter of 2 to 3 mm, or a small nodular hyperplasia in the vaginal fold. It continues to develop into a pedunculated or pedunculated grape-like mass, sometimes as large as 3 cm in diameter. Embryonic rhabdomyosarcoma is a polypoid-like, edematous, translucent mass that forms a bead, such as a grape-like structure. There are many types of sarcoma, and grape sarcoma mostly occurs in the vagina, and grape-like growth begins from the submucosa. Vaginal sarcoma and vaginal grape sarcoma are similar to the naked eye and the microscopic view. The gross specimens are multiple polypoid-like structures. Hilgers reported 27 cases of intravaginal and extragonadlosarcoma, all showing embryonic striated muscle cells. Characteristics. basic knowledge The proportion of illness: 0.005% Susceptible people: female infants Mode of infection: non-infectious Complications: respiratory failure uremia
Cause
Adolescent and pediatric vaginal sarcoma
(1) Causes of the disease
Embryonic rhabdomyosarcoma is a subtype of mesoderm mixed tumors, and its origins vary from one source to another. Most of them are considered to be derived from mesoderm mesoderm tissue and have embryonic immature, hence the name embryonic rhabdomyosarcoma.
(two) pathogenesis
1. The naked eye
Embryonic rhabdomyosarcoma is a polypoid-like, edematous, translucent mass that forms a bead, such as a grape-like structure. There are many types of sarcoma, and grape sarcoma occurs mostly in the vagina, and grape-like growth begins from the submucosa (Figure 1). .
2. Mirror view
Vaginal sarcoma and vaginal grape sarcoma are similar to the naked eye and the microscopic view. The gross specimens are multiple polypoid-like structures. Hilgers reported 27 cases of intravaginal and extragonadlosarcoma, all showing embryonic striated muscle cells. Characteristics, typical cases have the characteristics of immature tumor cells:
1 has complete epithelial coverage.
2 There is a new layer under the epithelium.
3 undifferentiated round, fusiform, polymorphic cells.
4 There are mixed stromal tumors in the center (mainly seen in mesoderm mixed tumors). Immature round, fusiform or polymorphic cells are composed of cells under the epithelium. There are eosinophilic granules in the cytoplasm, and the edges are not neat. Dense staining, nuclear heterogeneity, nuclear size varies, but megakaryocytes, deformed nuclei are rare.
Prevention
Prevention of vaginal sarcoma in adolescents and children
To prevent or reduce recurrence, you must be aware of the following:
1. Early diagnosis.
2. Once diagnosed, the thoroughness of the first surgery is emphasized based on the extent of the infiltration.
3. Excision of the marginal tissue of the specimen, such as the discovery of tumor cells, must be supplemented by radiotherapy.
4. Follow up well.
Complication
Adolescent and pediatric vaginal sarcoma complications Complications, respiratory failure, uremia
Late distant lung metastasis, lymphatic metastasis caused respiratory failure, uremia.
Symptom
Adolescent and pediatric vaginal sarcoma symptoms Common symptoms hydronephrosis vaginal mucosa swelling vaginal bleeding bone transfer ascites urinary frequency dehydration weight loss respiratory failure lung metastasis
Embryonic rhabdomyosarcoma occurs mostly in the anterior wall of the vagina, and sometimes the exact location of the lesion cannot be determined. The tumor is small papillary at the beginning of the tumor, with a diameter of 2 to 3 mm, or a small nodular hyperplasia in the vaginal fold, which continues to develop into a pedicle or A sessile grape-like mass, sometimes as large as 3 cm in diameter, begins to grow from the subepithelial, begins at a central or multiple centers, continues to grow and expand, causes the vaginal mucosa to swell, form a cavity, and penetrate the vine-like tissue. The vaginal mucosa protrudes into the vagina, so that the vagina gradually enlarges, so that the tumor fills the entire vagina and protrudes beyond the vaginal opening. In a few cases, the lumps protrude and cover the external genitalia.
The main symptoms are vaginal protrusions and vaginal bleeding, which are sometimes found occasionally when the baby takes a bath or change diapers. Sometimes the child coughs. When crying, the mass is removed from the vagina due to the increase of abdominal pressure. When the tumor appears, Generally no pain, such as vaginal anterior wall lesions continue to infiltrate the pelvic organs, involving the urethra, bladder, and frequent urination, urinary retention, hydronephrosis and other symptoms, when the tumor develops to the advanced stage, also like other malignant tumors, loss of appetite , weight loss, nausea, vomiting, dehydration, hypothermia, etc., and often die due to cachexia, respiratory failure or uremia. If the mass extends up to the pelvic cavity, the mass can be touched in the pelvis, sometimes the abdomen is enlarged with ascites, such as The tumor metastasizes to the lymph nodes, often with enlarged lymph nodes on both sides of the groin, or symptoms of pulmonary metastases.
Vaginal embryonal rhabdomyosarcoma is mainly local metastasis, and also metastasized to the lungs through the blood, and is transferred to the spine and cranial bone. The tumor begins at the anterior wall of the vagina and gradually infiltrates the adjacent organs or surrounding tissues, especially The urethra, the posterior wall of the bladder, the vaginal septum of the bladder are more common, the bladder and vaginal septum are loose, and there is no resistance to tumor penetration. In the literature, 15 cases of autopsy results were reported. Half of the patients were limited to pelvic growth, and Mayo Hospital reported 7 cases. The patients who died of metastasis confirmed autopsy with acute or chronic hydronephrosis, including 3 cases of distant metastasis to inguinal lymph nodes, 3 cases of lung metastasis, 1 case of bone metastasis, and tumors that were transferred from the vaginal wall to the posterior. The vaginal septum is relatively solid and has a protective barrier to the infiltration of advanced tumors. Even if the rectal metastasis is not directly caused by the vaginal wall, it is first invaded into the rectal vaginal fossa, and then reaches the rectum, and the rectal metastasis is occasionally seen.
Examine
Examination of adolescents and pediatric vaginal sarcoma
Vaginal device examination: the genitals of adolescents and children are not yet mature, especially the genital genitals in children are located in the deep pelvic cavity. Gynecological examination is difficult. Generally, abdominal and anal examinations are performed routinely. When anal examination is performed on children, it is best to use the little fingers to reach the rectum. If the condition requires, a vaginal examination should still be performed.
1. Indications for vaginal examination
Genital bleeding, infection, suspected genital foreign body, tumor, trauma, etc. are the absolute indications for vaginal examination, and must be checked immediately to avoid delay in diagnosis, congenital malformation, developmental disorders, acute abdomen, anorectal disease, enuresis, etc. The certificate needs to be checked in conjunction with other specialists.
2. Vaginal examination equipment
Check the vaginal and cervix of children generally use vaginoscope (vaginoscope), you can also use otolaryngoscope, etc., according to the size of the hymen hole, choose the corresponding diameter of the vaginal speculum, the general baby with a small vaginal speculum, smaller For children, the medium size is larger for children. In addition, it is not advisable to use a cotton swab when taking the upper part of the vagina, so that the cotton may fall off and fall into the vagina when it is taken out. It is best to use a sterile glass pipette or a plastic tube instead.
3. Vaginal examination method
Before performing vaginal speculum examination, first of all, you need to get the consent of your family and get cooperation. Generally, you don't need anesthesia. However, if the sick child can't cooperate well and the hymen hole is too small, you can do it under general anesthesia, take the supine position, assistant. Fix the sick child's hip joint and knee joint, make the two legs flex, the abdominal wall is slack, slowly put into the vaginal speculum, the operation must be gentle, so as not to cause damage and bleeding, it must be noted that the child's vagina is immature, limited capacity, relative Short and narrow, the Qianlong has not yet formed. In the early childhood, the vaginal length is only 4.5-5.5cm on average. The vaginal wall is thin and easy to be damaged. In the late childhood, the vaginal length is 7.5cm on average. The cervix is flat and resembles a button-like protrusion. At the menarche, the shape of the cervix is like the shape of an adult.
Histopathological examination.
Diagnosis
Diagnosis and differentiation of adolescents and pediatric vaginal sarcoma
According to clinical manifestations and pathological features, it is generally not difficult to diagnose, but it is not easy to diagnose early in clinical practice. When a child has a vaginal mass in the vagina, the mass is often quite large, and even has destructive infiltration or metastasis, sometimes histopathological examination. It is quite benign and often misunderstood as a benign tumor. Scholars believe that there are longitudinal and transverse striped muscle fibers in the eosinophilic cytoplasm, which is the main basis for the diagnosis of rhabdomyosarcoma, but it is difficult to find this striped structure in practice. Mainly because of the small number of tumor cells and the sparse structure, electron microscopy helps to confirm the appearance of striped muscle structure. The presence of immature cells in tissue sections is more important for diagnosis.
Must be differentiated from vaginal polypoid adenocarcinoma, benign renal papillary adenoma, middle renal ductal adenocarcinoma and vaginal hematoma.
