Endometrial stromal sarcoma

Introduction to endometrial stromal sarcoma Endometrial stromal sarcoma (ESS) is a tumor derived from endometrial stromal cells, accounting for 30% to 40% of uterine sarcoma. According to the histological and clinical features of the tumor, it is divided into two categories, namely Low grade endometrial stromal sarcoma (LGESS) and high grade endometrial stromal sarcoma (HGESS), the former accounted for about 80%, the disease progresses slowly, the prognosis is good, and the latter is highly malignant, the condition Rapid development, easy invasion and metastasis, poor prognosis. The pathological features of the two are also different. Low-grade malignant endometrial stromal sarcoma has many names in the past. Doran and Lockyer first described the disease in 1909. In 1940, Goodall named the disease endolymphaticstromal endometriosis. In 1946, Henderson proposed Endometrial stromal disease (stromalendometriosis), and scholars named the disease as endolymphaticstromal myosis (endolymphaticstromalmyosis), etc., more confusion. In recent years, most scholars have advocated the concept of low-grade endometrial stromal sarcoma, which can not only explain the origin of the tumor, but also indicate the nature of the tumor, and avoid confusion with endometriosis and adenomyoma. basic knowledge The proportion of illness: 0.0006% Susceptible people: women Mode of infection: non-infectious Complications: anemia

Endometrial stromal sarcoma etiology

(1) Causes of the disease

Mesenchymal cells originating in the endocervix of the cervix, most of the highly malignant endometrial stromal sarcomas are from the endometrial stroma; low malignant endometrial stromal sarcoma grows only along the dilated lymphatic vessels or blood vessels, which still originate from Interstitial cells of the intima.

(two) pathogenesis

1. Low grade endometrial stromal sarcoma

(1) General form:

1 Low-grade malignant endometrial stromal sarcoma has two general forms:

A. The tumor forms polypoid or nodules. From the endometrial process to the uterine cavity or to the cervix, the tumor volume is larger than the general polyp, the pedicle is wide, the texture is soft and brittle, and the surface is smooth or broken and secondary infection.

B. Tumor-like leiomyoma, located in the myometrium of the uterus, often infiltrates the myometrium, showing nodular or diffuse growth, and the boundary between the myometrium and the myometrium is unclear.

2 tumor section texture soft, uniform, like raw fish-like, tissue edema, with hemorrhage, necrosis, you can see dark red, brown or gray-yellow areas; also see cystic areas, but bleeding, necrosis is not as high as the endometrium Interstitial sarcoma is more common.

3 para-uterine tissue or extrauterine pelvic cavity can be seen in the tumor-like lymphatic tumor, such as rubber, elastic, which is a common feature of low-grade endometrial stromal sarcoma.

(2) Microscopic features (Figure 1):

1 tumor cells like proliferative endometrial stromal cells, uniform size, oval or small fusiform.

2 nuclear fission elephant 5 ~ 10 /10 HPFs.

3 There are many blood vessels in the tumor, and the tumor grows along the dilated lymphatic vessels, which infiltrate the surrounding smooth muscle tissue.

4 has a wide range of interstitial hyaline degeneration.

5 parts of the tumor contain Call-Exner small body-like structure, and some tumors contain epithelioid-like differentiation areas, forming endometrioid glands, small tubes, cell nests and cords. If these components are more, ovarian cord-like components are formed. Ovarian sex cord-like), this ingredient is vimentin, desmin, actin positive, indicating that it is a muscle-like differentiation component, not an epithelial component.

6 estrogen receptor (ER) and progesterone receptor (PR) can be positive, DNA ploidy is mostly diploid.

2. Highly malignant endometrial stromal sarcoma

(1) Gross morphology: similar to low-grade malignant endometrial stromal sarcoma, but the tumor volume is larger, hemorrhagic necrosis is more obvious, and some lesions are similar to endometrial cancer and uterus mesodermal mixed tumor, lacking sputum lymphatic vessels The characteristics of the tumor.

(2) Microscopic features (Figure 2):

1 The tumor cells are fusiform or polygonal, different in size and distinct in shape, and can be found in giant cells of the tumor.

2 mitotic figures 10 /10 HPFs, often more than 20 ~ 30 /10 HPFs.

3 tumor cells can be arranged into epithelioid cell nests, cords and flaky.

4 tumor cells can grow along the lymphatic sinus or sinusoids or invade the muscle layer.

Highly malignant endometrial stromal sarcoma has a high degree of malignancy, rapid growth, frequent local recurrence and distant metastasis, and may have a gross invasion of the muscular layer.

Endometrial stromal sarcoma prevention

It is very important to maintain a good attitude, to maintain a good mood, to have an optimistic, open-minded spirit, and to be confident in the fight against disease. Don't be afraid, only in this way can you mobilize your subjective initiative and improve your body's immune function.

Endometrial stromal sarcoma complications Complications anemia

Low-grade endometrial stromal sarcoma with para-uterine intravascular tumor thrombus and lung metastases is particularly common, followed by local infiltration and lymphatic metastasis. Highly malignant endometrial stromal sarcoma is locally invasive, often with muscularis infiltration and Destructive growth.

Endometrial stromal sarcoma symptoms Common symptoms Vaginal bleeding bloating squamous cell carcinoma abdominal pain uterine cavity separation

1. The most common symptoms of symptoms are irregular vaginal bleeding, increased menstruation and vaginal discharge, anemia, lower abdominal pain and so on.

2. Signs can be found in the cervix or vagina, soft and brittle, easy to hemorrhagic polypoid mass, such as tumor rupture and infection, can have extremely stinky vaginal secretions, often combined with anemia, uterus enlargement, pelvic swelling Things.

3. The pelvic examination of the uterus is increased to varying degrees. The early pelvic examination is similar to the uterine wall fibroids. When the tumor develops, the cervix can be seen as a polypoid or cauliflower-like prolapse.

There are data showing that common symptoms of endometrial stromal sarcoma include vaginal bleeding (69.6%), compression symptoms (39.1%), lower abdominal mass (30.4%), vaginal discharge (17.4%), lower abdominal pain (17.4%). ).

4. Staged endometrial stromal sarcoma originated from the endometrial stroma. Some scholars believe that its developmental changes have many similarities with endometrial cancer. It is recommended to use the FIGO staging criteria in stages:

(1) Clinical staging (FIGO 1971, Table 1): The International Federation of Obstetricians and Gynecologists (FIGO) stipulates that clinical endometrial cancer is scheduled for endometrial cancer by October 1989, and radiotherapy is not available for surgery. The procedure was followed by 1971 clinical staging.

(2) Surgical pathological staging: FIGO recommended the use of endometrial cancer surgery-pathological staging in October 1988.

Provisions regarding staging:

1 Because endometrial cancer has been surgically staged, the previously used segmental diagnosis to distinguish between stage I or phase II methods is no longer applied.

2 A small number of patients began to choose radiotherapy, still using the clinical staging of the 1971 FIGO, but should be noted.

3 The thickness of the muscle layer should be measured together with the depth of cancer invasion.

Histopathological grade:

G1: Non-squamous or mulberry-like solid growth type 5%.

G2: Non-squamous or non-mulberry-like solid growth types account for 6% to 50%.

G3: Non-squamous or non-mulberry-like solid growth type >50%.

Note on pathological classification:

A. Pay attention to nuclear atypicality. If it is not consistent with the structural classification, G1 or G2 should be upgraded by one level.

B. Grading of serous adenocarcinoma, clear cell adenocarcinoma and squamous cell carcinoma should first consider the grading of the nucleus.

C. Adenocarcinoma with squamous components should be graded according to the nuclear fraction of glandular components.

Endometrial stromal sarcoma examination

1. Pre-surgical scraping has certain value for endometrial stromal sarcoma. The positive rate of diagnosis and curettage is about 80%, which is higher than 40% of uterine leiomyosarcoma, but lower than that of uterine malignant mesodermal mixture. 80% to 90% of the tumor, it is also believed that endometrial stromal sarcoma has a wide base of polypoid lesions, and the diagnosis of scraping has certain limitations.

2. Tumor marker examination.

3. Color Doppler measurement of blood flow signals and blood flow resistance of uterus and tumors is helpful for diagnosis. Peking University People's Hospital concluded that the blood flow resistance index (RI) of 11 cases of LG-ESS averaged 0.42, therefore, it is recommended. For those with low blood flow, it is highly suspected of uterine sarcoma.

Diagnosis and differentiation of endometrial stromal sarcoma

Diagnostic criteria

Clinical irregular vaginal bleeding, when the pelvic examination see polypoid-like protrusions in the cervix, in the diagnosis of cervical polyps, endometrial polyps and submucosal fibroids, should be alert to the possibility of endometrial stromal sarcoma, visible during surgery The tumor forms polyps or nodules from the endometrial process to the uterine cavity or from the cervix. The tumor volume is larger than that of the general polyps, the pedicle is wide, the texture is soft and brittle, and the tumor in the muscular layer is nodular or diffuse, but the boundary is not Clear, not easy to completely remove; tumor section is fish-like, may have bleeding, necrosis and cystic changes, for suspicious cases, should be frozen section examination, but the final diagnosis depends on paraffin section examination.

Differential diagnosis

1. There are many differences between low-grade malignant endometrial stromal sarcoma and highly malignant endometrial stromal sarcoma. It is often difficult to distinguish clinically, mainly relying on pathological examination for identification.

2. Identification of endometrial polyps with endometrial polyps often multiple, pedicle, and thin, small, and endometrial stromal sarcoma is mostly a single polypoid mass, wide or thick pedicle, often accompanied There are hemorrhagic necrosis and infection, and more vaginal discharge.

3. Identification of endometrial stromal sarcoma with uterine submucosal fibroids can be released from the uterine cavity to the vagina, clinical manifestations and submucosal fibroids are similar, differential diagnosis is more difficult, more need surgical pathological diagnosis, but endometrial stromal sarcoma growth Fast, blood flow signals are abundant, there is a low-resistance spectrum, and some patients may have elevated serum CA125.

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