Muller mixed tumor

from Wikipedia, the free encyclopedia
Classification according to ICD-10
C53 Malignant neoplasm of the cervix uteri
C54 Malignant neoplasm of the corpus uteri
C55 Malignant neoplasm of the uterus, part unspecified
C56 Malignant neoplasm of the ovary
C57.0 Malignant neoplasm of the uterine tuba
C17 Malignant neoplasm of the small intestine
ICD-10 online (WHO version 2019)

Müller's mixed tumors are rare, extremely malignant ( malignant ) tumors in women that originate from pluripotent cells of the Müller duct, after which it was named. They occur predominantly in the body of the uterus and only rarely in the cervix . Diseases of the ovaries , fallopian tubes and the mesentery have also been described. The name mixed tumor results from the simultaneous occurrence of carcinomatous and sarcomatous components in the same tumor ( carcinosarcoma ).

Epidemiology

Müllerian mixed tumor of the corpus uteri

Müller's mixed tumors represent around 30 to 60 percent of uterine sarcomas , with only two to three percent of malignant diseases of the female reproductive organs being sarcomas, which usually occur in the uterus . Up to 2005, only about 50 cases of a Müller mixed tumor of the cervix uteri were described in the English-language literature . The mean age of onset is 65 years.

pathology

The tumors arise from pluripotent cells of the Müllerian duct of the normal uterine lining and usually develop with homologous and heterologous components. In the first variant, parts of squamous cell carcinoma or adenocarcinoma are found in the carcinomatous areas . The sarcomatous part contains exclusively tissue from atypical endometrium . In contrast, the heterologous forms in the sarcomatous parts contain several components, most commonly chondroblasts , rhabdomyoblasts , osteoblasts or fat cells . Adenosarcomas represent a special form in which only the mesenchymal part is malignant, while the adenomatous part is benign. Tumor typing is carried out according to the WHO classification ICD-O-3 with the morphology code 8950/3. Before an operation, the stages are clinically based on the FIGO classification . After surgical treatment, the staging is carried out according to the pTNM classification and, after a histological assessment by a pathologist, the stage designation is preceded by a small p . The degree of differentiation of the cancerous tissue is assessed according to the UICC ( Union Internationale Contre le Cancer ). In Müller's mixed tumor, there is a rapid rupture of blood and lymph vessels with early metastasis. In stages I and II, metastases are found in 17 to 35 percent of pelvic or para-aortic lymph nodes .

Stages according to TNM classification and FIGO (Fédération Internationale de Gynécologie et d'Obstétrique):

TNM FIGO criteria
TX Primary tumor cannot be assessed
T0 No evidence of a primary tumor
Tis Carcinoma in situ
T1 I. Tumor limited to the body of the uterus
1a IA Tumor confined to the endometrium or infiltrates less than half of the myometrium
1b IB Tumor infiltrates half or more of the myometrium
T2 II Tumor infiltrates the stroma of the cervix uteri, but does not spread beyond the uterus
T3 and / or N1 III local and / or regional spread
3a IIIA Tumor affects the serosa of the corpus uteri and / or the adnexa (direct spread or metastases)
3b IIIB Vaginal involvement and / or involvement of the parametria (direct spread or metastases)
3c or N1 IIIC Metastases in pelvic and / or para-aortic lymph nodes
3c1 IIIC1 Metastases in pelvic lymph nodes
3c2 IIIC2 Metastases in para-aortic lymph nodes with / without metastases in pelvic lymph nodes
T4 IV Tumor infiltrates the bladder and / or intestinal mucosa
Nx No statement can be made on regional lymph node metastases.
N0 No metastases in the regional lymph nodes.
N1 Metastases in the regional lymph nodes.
M0 No distant metastases detectable.
M1 The tumor has formed distant metastases. (except vagina, pelvic serosa, adnexa; including inguinal and abdominal lymph nodes other than para-aortic and / or pelvic lymph nodes)

therapy

The treatment of Müllerian mixed tumors consists almost exclusively of an operation. Radiation therapy and chemotherapy are not very effective. It consists of a careful examination of the abdominal cavity, the removal of a lavage cytology and an abdominal hysterectomy with removal of the fallopian tubes and ovaries . The large network ( greater omentum ) and the lymph nodes should also be removed, as they are of prognostic importance. Radiation therapy has no effect on survival but appears to reduce the incidence of local recurrences . Chemotherapy does not improve the prognosis. In the case of metastatic tumors and relapse, however, it can increase survival time. Possible active ingredients are paclitaxel , carboplatin , doxorubicin , gemcitabine and docetaxel .

forecast

The prognosis of these tumors is poor and depends on the type of sarcoma, the tumor stage and the location where the tumor grew. The 5-year survival rate is 40 to 50 percent in stage I and drops to 25 to 30 percent in more advanced stages. Other important prognostic criteria are the depth of myometrial infiltration , the presence of pelvic lymph node metastases , which are detected in 17 percent of tumors clinically restricted to the uterus, and the growth of the tumor into blood vessels and spread to the cervix uteri .

history

The Müllerian mixed tumor was first described by Ferriera in 1951.

Individual evidence

  1. ^ A b Heinrich Schmidt-Matthiesen , Gunther Bastert , Diethelm Wallwiener : Gynecological Oncology - Diagnostics, Therapy and Follow-Up Care on the Basis of the AGO Guidelines. 10th edition. Schattauer Verlag, 2002, ISBN 3-7945-2182-X , p. 105.
  2. ^ A. Maheshwari, S. Gupta, T. Shet, R. Wuntkal, HB Tongaonkar: Diagnostic dilemma in a case of malignant mixed mullerian tumor of the cervix. In: World Journal of Surgery 4 (2006), 36, PMID 16813659 , doi: 10.1186 / 1477-7819-4-36
  3. ^ JD Wright, K. Rosenblum, PC Huettner, DG Mutch, JS Rader, MA Powell, RK Gibb: Cervical sarcomas: An analysis of incidence and outcome. In: Gynecol Oncol . 99 (2005), pp. 348-351, PMID 16051326 , doi: 10.1016 / j.ygyno.2005.06.021
  4. ↑ a b c P. Hantschmann: Rare uterine malignancies. In: Journal Oncology . 8 (2008).
  5. ↑ a b c G. Leppien, H. Schmidt-Matthiesen: Sarcomas of the female genital organs. In: Karl Heinrich Wulf , Heinrich Schmidt-Matthiesen : Clinic of gynecology and obstetrics. Volume 11, Special Gynecological Oncology I. 3rd edition. Urban and Schwarzenberg, 1991, ISBN 3-541-15113-7 , pp. 257-268.
  6. ↑ LC Hanker, M. Kaufmann: Rare gynecological tumors. In: Oncologist. 15 (2009), pp. 261-269, doi: 10.1007 / s00761-009-1588-1
  7. ↑ L.-C. Horn, K. Schierle, D. Schmidt, U. Ulrich: New TNM / FIGO staging system for cervical and endometrial carcinoma as well as malignant Müllerian mixed tumors (MMMT) of the uterus. In: obstetric women's health. 69 (2009), pp. 1078-1081, doi: 10.1055 / s-0029-1240644
  8. ↑ L.-C. Horn, K. Schierle, D. Schmidt, U. Ulrich, A. Liebmann, C. Wittekind: Updated TNM / FIGO staging system for cervical and endometrial carcinoma and malignant mixed tumors (MMMT) of the uterus. Facts and background. In: Pathologist. 2010, PMID 20084383
  9. ↑ B. Larson, C. Silfversward, B. Nilsson et al .: Mixed Mullerian tumors of the uterus - prognostic factors: a clinical and histopathologic study of 147 cases. In: Radiotherapy and Oncology . 17, pp. 123-132 (1990), PMID 2157241
  10. ↑ W. Schweizer, R. Demopoulos, U. Beller, N. Dubin: Prognostic factors for malignant mixed müllerian tumors of the uterus. In: Int J Gynecol Pathol. 9: 129-136 (1990).
  11. ^ HP Ferriera: A case of mixed mesodermal tumor of the uterine cervix. In: J Obstet Gynaecol Br Emp. 58 (1951), pp. 446-448, PMID 14861690 .