Uterine sarcoma is a malignant neoplasm of the body or cervix originating from undifferentiated connective tissue elements of the myometrium or endometrial stroma. Disease is manifested by cyclic and acyclic bleeding, abdominal pain, persistent whites with a putrid odor, general malaise. Uterine sarcoma is diagnosed by bimanual examination, hysteroscopy, ultrasound, diagnostic curettage, cytology and histology of biopsies, laparoscopy. With uterine sarcoma, an extended pangisterectomy is performed, combined with radiation and chemotherapy.
General information
In the structure of malignant neoplasms of the uterus, sarcoma is from 3 to 5-7% of cases. Uterine sarcoma is often combined with vaginal sarcoma, and can also develop in the node of an existing uterine fibroma. Disease occurs in women of any age (more often in pre- and postmenopausal), but it is observed even in girls, being a consequence of dysembriogenesis. By localization, sarcoma of the uterine body is diagnosed 10 times more often than its cervix. In its course, uterine sarcoma is extremely malignant.
Causes
The issues of epiopathogenesis are poorly studied. It is assumed that polyethological factors, including dysembrioplasias and recurrent injuries leading to the proliferation of regenerating tissues, play a decisive role in the development of a sarcomatous tumor.
Pathology is usually preceded by some pathological conditions. The most common (51-57%) tumor dysplasia occurs in focal nodular formations – uterine fibromyomas. Among the risk factors, gynecology also identifies disorders of embryogenesis, birth trauma, damage to the uterus during surgical termination of pregnancy and diagnostic curettage, pathology of the uterus with proliferative changes (endometriosis, endometrial polyps), etc.
Significant importance in the etiopathogenesis is given to chronic intoxication (including nicotine, alcohol, medicinal), occupational hazards, environmental problems, pelvic irradiation in cervical cancer. It is possible that the development of this disease is facilitated by anovulation and hyperestrogenism, as well as neuroendocrine disorders that occur during menopause.
Characteristic
Tumor dysplasia in sarcoma occurs more often in the smooth muscles of the uterus (leiomyosarcoma), interstitial connective tissue (endometrial stromal sarcoma) and other morphological structures. Myometrial sarcomas are usually round-shaped formations that are poorly delimited from the surrounding tissues. On the incision, sarcomatous nodes have a whitish, grayish-pinkish color, a soft crumbling consistency, sometimes the appearance of boiled meat or brain tissue. With necrosis and hemorrhage in the tumor tissue, the uterine sarcoma acquires a mottled color. Endometrial sarcomas are more often represented by limited (nodular, polypous) growths, less often they have a diffuse character.
As the uterine sarcoma grows, the myometrium infiltrates and can even reach the perimeter and walls of adjacent organs (bladder, intestine). With the invasion of the perinatal fiber, the clinic of parametritis can develop. Pathology is prone to rapid growth and early destruction (decay), which is accompanied by the formation of cystic cavities. With metastasis (hematogenic, lymphogenic), tumor cell dropouts are more often detected in the lungs (17%), liver (9%), retroperitoneal lymph nodes (8%), ovaries (7%), spine and bones (5%).
Classification
The main morphological forms are leiomyosarcomas, endometrial stromal sarcomas, mixed mesodermal tumors, carcinosarcomas, etc. Sarcomas originating from the myometrium occur in 47.2% of cases, from fibromatous nodes – in 25.3%, from the endometrium – in 27.5%.
According to the cellular composition, fibroblastic, spindle-cell, polymorphocellular, round-cell, muscle-cell, giant-cell, small-cell type of uterine sarcoma are distinguished.
When assessing the prevalence of uterine sarcoma , stages IV are distinguished:
Stage I – the spread of uterine sarcoma is limited to the muscular or/and mucous layer:
- Ia – tumor invasion affects the myometrium or endometrium
- Ib – tumor invasion affects the myometrium and endometrium
Stage II – localization of sarcoma is limited to the body and cervix and does not go beyond them:
- IIa – there is a proximal or distal infiltration of the parametrium without transition to the walls of the pelvis
- IIb – the tumor passes to the cervix
Stage III – localization of sarcoma outside the uterus, but within the boundaries of the pelvis:
- IIIa – there is a one- or two-sided infiltration of the parametrium with a transition to the walls of the pelvis
- IIIb – tumor metastasis to regional lymph nodes, vagina, appendages, germination of large veins is noted
- IIIc – the germination of the serous cover of the uterus, the formation of conglomerates with neighboring structures without their damage is determined
Stage IV – germination of uterine sarcoma into adjacent organs and beyond the pelvis:
- IVa – the tumor grows into the bladder, rectum
- IVb – tumor metastasizes to distant organs
Symptoms
In the early stages, uterine sarcoma is a “mute” tumor that gives scant symptoms. With the development of uterine sarcoma in fibromatous nodes, the manifestations may not differ from the clinic of uterine fibroma (subserous, submucous, interstitial).
As uterine sarcoma progresses, depending on its localization, direction and growth rate, menstrual cycle disorders (menorrhagia, metrorrhagia), pelvic pain, abundant watery whiteness, which acquire a putrid odor when the infection is attached, are noted. Symptoms are most pronounced in sarcoma of submucous nodes and endometrium.
Late manifestations include anemia, cachexia, weakness, intoxication, ascites. With metastatization of sarcoma, pleurisy develops in the lungs; jaundice develops in the liver; pain in one or another department develops in the spine, as well as other manifestations characteristic of the affected organ.
Diagnostics
In the process of diagnosis, disease should be differentiated from benign fibromyoma. Uterine sarcoma should be considered with rapid growth of fibromatous nodes; acyclic bleeding; severe anemia that does not correspond to the degree of blood loss; increased ESR; recurrence of symptoms after removal of submucous nodes or polyps; detection of tumor nodes in the stump after supravaginal amputation of the uterus.
When examining the vagina, attention is drawn to the cyanotic coloration of the cervix, its edema, hypertrophy, and sometimes the detection of a sarcomatous node being born. With the help of gynecological examination (bimanual, rectovaginal), the localization of uterine sarcoma, the size and consistency of nodes, their displacement, the presence of infiltrates in the parametria, enlarged parietal lymph nodes, the condition of appendages is established.
According to ultrasound diagnostics, the nodular transformation of the uterus, its heterogeneous echogenicity, necrotic nodes, and the presence of pathological blood flow are revealed. During aspiration biopsy with cytological examination of smear prints, in some cases it is possible to detect the presence of atypical polymorphic cells. More accurate information is obtained with DDV followed by histological examination of endometrial scraping. In diagnostic terms, hysteroscopy, hysterocervicography, laparoscopy, lymphography, angiography are informative for uterine sarcoma.
All patients with uterine sarcoma are examined for urinary tract (excretory urography, chromocystoscopy, renography), intestines (rectoromanoscopy, irrigoscopy), lungs (chest X-ray), liver (ultrasound). Uterine sarcoma in the course of diagnosis is differentiated from uterine fibromyoma, ovarian tumors, endometrial polyps, primary tumors of adjacent localization.
Treatment
Combined treatment is most effective for uterine sarcoma. A radical intervention in uterine sarcoma is a pangisterectomy; in advanced cases – an extended hysterectomy – removal of the uterus, removal of appendages (adnexectomy), regional lymph nodes, infiltrate parametry and resection of adjacent organs.
The surgical stage is complemented by radiation therapy aimed at devitalizing tumor cells. Chemotherapy (doxorubicin, fluorouracil, cyclophosphamide, vincristine, dactinomycin) due to insufficient effectiveness is used as a palliative method for inoperable processes and recurrent uterine sarcoma.
Prognosis
The prediction of long-term results in uterine sarcoma is disappointing. On average, the five-year survival rate of patients with uterine sarcoma is about 40%: at the I st. – 47%, at the II st. – 44%, at the III st. – 40%, at the IV st. – 10%. A relatively favorable course is characterized by uterine sarcomas developing from fibromatous nodes (provided there are no metastases), the worst prognosis is noted for endometrial sarcomas.
Prevention
Prevention of uterine sarcoma consists in timely detection and correction of neuroendocrine disorders, treatment of endometritis, uterine fibromyomas, endometriosis, endometrial polyps. Preventive measures include the selection of contraception, prevention of abortions.
