vaginal rhabdomyosarcoma
Introduction
Introduction to vaginal rhabdomyosarcoma Vaginal rhabdomyosarcoma (RMS) is a highly malignant tumor. The predilection area is related to age, and infants and young children are more common in the vagina. The patient developed irregular vaginal discharge or bleeding, accompanied by serous, mucinous vaginal discharge, sometimes bloody. There may be polypoid masses protruding from the vagina, mostly eddy or edema-like grape-like or polypoid tissue blocks. basic knowledge The proportion of illness: 0.004% Susceptible people: women Mode of infection: non-infectious Complications: hematuria
Cause
Causes of vaginal rhabdomyosarcoma
Cause:
Most of the tumors are pale red or grayish white, translucent mucus-like, grape-like or polypoid mass, soft, edematous, may be accompanied by bleeding or ulceration, and the cut surface shows loose tissue with mucus.
Microscopic examination of the typical grape cluster type can show three related different regions: the cell layer is formed immediately below the mucosa, the deeper layer is the cell area composed of the original tumor cells, and the center is the cell-stained area with rare cells. Immature round and spindle cells with nuclear dysplasia, visible striated muscle cells.
The remaining various types of RMS were observed under light and electron microscopy as well as special staining, immunohistochemistry, RMS tumor markers, and cellular and molecular genetic examinations were similar to vulvar rhabdomyosarcoma.
Prevention
Vaginal rhabdomyosarcoma prevention
Parents or nurses should always pay attention to the baby's vulva clean, change the diaper in time, every time you change the diaper, you should carefully check the baby's vagina for redness or abnormal secretions.
Vaginal rhabdomyosarcoma must be followed closely after treatment, especially within 2 years after diagnosis, because local recurrence is very likely (median recurrence 16 months), it is recommended that colposcopy, hysteroscopy and transvaginal ultrasonography should be performed every 2 months after anesthesia After Arndt et al. followed up for 5.8 years, 66.6% of patients with recurrent female genital rhabdomyosarcoma were remedied by chemotherapy, surgery and radiotherapy. For recurrent embryonic or grape cluster vaginal rhabdomyosarcoma, surgery is an exact treatment. The remedy used in the radiotherapy group had a better prognosis than the other groups. The 5-year survival rate after recurrence mainly depends on the histological type of the mass, the initial diagnosis and the mode of recurrence (local recurrence, regional lymph node recurrence or distant recurrence). ).
Complication
Vaginal rhabdomyosarcoma complications Complications
Late stage can be complicated by vaginal fistula.
Symptom
Vaginal rhabdomyosarcoma symptoms Common symptoms Urinary frequency urinary urgency purulent discharge Vaginal bleeding hematuria
1. Symptoms: Vaginal rhabdomyosarcoma is asymptomatic in the early stage. The development of the disease often manifests as vaginal bleeding and vaginal mass, accompanied by serous, mucous vaginal discharge, sometimes bloody, such as infants crying, urinating or coughing, not appearing Regular vaginal drainage or bleeding, in the absence of a history of trauma should take into account the possibility of RMS, especially when polyps protrude from the vaginal opening, adult women often present with a disease of menorrhagia, or irregular vagina Bleeding, elderly women often manifest as irregular vaginal bleeding after menopause or vaginal discharge of odorous purulent secretions. When tumor necrosis is broken, the vagina may have tissue fragments discharged, the lesion may involve the urethra, and the bladder may appear in the urinary system. The corresponding symptoms, such as frequent urination, urgency, difficulty urinating, hematuria.
2. Signs: Most common in the upper third of the vagina, the former wall is mostly, the tumor can be one or more polypoid or grape-like, the size is 0.2 ~ 15cm, mostly 3 ~ 5cm, the surface is smooth, reddish, half Transparent, often blending with nodules and lobes, can also be scattered, soft and brittle, easy to bleed, can fill the entire vagina, and even protrude from the vaginal opening, covering the clitoris and perineum, the tumor can also invade the cervix or Spread to the sides of the vagina, pelvic examination can lick the block, regional lymph nodes and lung metastases can occur.
3. Type: Vagina RMS is most common in grape clusters, accounting for 67.1% to 78.6%, followed by embryo type, accounting for 21.0% to 28.4%, while acinar type is limited to individual cases, and IRS GIII is the most in staging. See, about 84.1%.
Examine
Examination of vaginal rhabdomyosarcoma
Pathological examination
Under the microscope, tumor-like polypoid masses grow on the epithelial surface, and a dense, undifferentiated tumor cell layer beneath the epithelial layer is characteristic. The tumor cell nucleus is larger, and more or less eosinophilic cytoplasm is seen. Easy to see mitotic figures, with or without horizontal stripes.
2. Imaging examination
Chest radiography and bone scan can determine whether there is bone metastasis and lung metastasis. CT scans enable doctors to assess the extent of tumor invasion and develop treatment options. Ultrasound is necessary as an aid to CT. Magnetic resonance imaging provides superior soft tissue control.
3. Other inspections
Immunohistochemistry, tumor markers, molecular genetics examination.
Diagnosis
Diagnosis and differentiation of vaginal rhabdomyosarcoma
diagnosis
According to the characteristics of the age of onset and clinical manifestations, the possibility of this disease should be thought of, and then further examination to make a diagnosis, there is a certain difficulty in vaginal examination in infants and young children, pediatric speculum or urethroscopic examination can be applied, if necessary, under the anesthesia for vagina Check, any suspected lesions should be biopsied to confirm the diagnosis, for better differentiation RMS, conventional light microscopy or special staining can be clearly diagnosed by striated muscle differentiation, low tumor differentiation, no obvious or typical muscle differentiation cells Must be checked by electron microscopy.
Differential diagnosis
1. Superficial interstitial reaction can occur in the vagina, cervix, pale edema, polypoid, size, development is slow, most occur in pregnancy, rarely recurrence after resection, the lesion can be seen 0.2 ~ 0.5 The mm-thick subepithelial mucus-like interstitial zone has a large number of astrocytes, which have a singular nucleus similar to that of fibroblasts after radiotherapy. The cells have no horizontal stripes and no nuclear division.
2. Vaginal polyps are often single-shot, pedicled on the base, no grape-like appearance, tough texture, not easy to hemorrhage. There are only a few abnormal cells with deep nuclear staining in the interstitial, mitosis is rare, and there is no amount of immature mesenchymal cells. And cells differentiated from striated muscle, there is no new tissue layer.
3. Benign rhabdomyosarcoma is very rare, mainly in adult women, rare in infants and young children, the tumor is hard and nodular, the boundary is clear, the development is slow, and the histological differentiation is good. The main feature is that the embryonic differentiation is more regular. Embryonic striated muscle, or mature adult striated muscle, cytoplasm with horizontal stripes, no amount of immature undifferentiated mesenchymal cells or immature myoblasts and mucinous stroma.
4. Vaginal endodermal sinus tumor occurs in infants and young children, may be nodules or polyps, rich in mucinous interstitial, similar to the grape cluster RMS part, but endodermal sinus tumor can be seen except mucinous interstitial Epithelial differentiation, histological identification is not difficult.
