This malignant tumor of the uterus is also known as a MMMT. It is a highly malignant tumor that usually arises in the post-menopausal age group. It commonly presents with post-menopausal bleeding, abdominal pain, and distension. This tumor is aggressive and may also present with symptoms of extra-uterine spread.
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EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Sarcomatoid carcinoma
INCIDENCE Most common uterine sarcoma
1.5% of all malignant uterine tumors
AGE RANGE-MEDIAN Median 65 years SEX (M:F) F EPIDEMIOLOGIC ASSOCIATIONS Prior pelvic irradiation Latent period of about 16 years
DISEASE ASSOCIATIONS CHARACTERIZATION Diabetes mellitus Infertility Obesity Hypertension
PATHOGENESIS CHARACTERIZATION Sarcomatoid carcinoma Most think this is an undifferentiated carcinoma which exhibits sarcomatoid transformation
CHARACTERIZATION Flow Cytometry Aneuploid with high S-phase fraction
CHARACTERIZATION General Sessile or pedunculated and usually fills the endometrial cavity
Extends to endocervix in 1/4 of cases
HISTOLOGICAL TYPES CHARACTERIZATION General
Malignant epithelium usually in the form of an endometrioid type adenocarcinoma
May also show squamous, clear cell, mucinous, and serous carcinoma
Mesenchymal component have homologous rease such as endometrial stromal sarcoma and fibrosarcoma and heterologous areas such as rhabdomyosarcoma
CHARACTERIZATION Special stains Immunoperoxidase
Positive for CK and EMA
Stromal component may show rhabdomyoblastic differentiation confirmed with desmin or muscle specific actin
May contain estrogen and progesterone receptors
Expression of CD10 in Malignant Mullerian Mixed Tumors and Adenosarcomas: An Immunohistochemical Study.
Mikami Y, Hata S, Kiyokawa T, Manabe T.
Department of Pathology, Kawasaki Medical School Hospital (YM, SH, TM), Kurashiki, Okayama.
Mod Pathol 2002 Sep;15(9):923-30 Abstract quote
CD10 has been demonstrated to be positive in endometrial stromal sarcoma (ESS) and thus is useful in establishing the diagnosis, but its expression in malignant mullerian mixed tumor (MMMT) and mullerian adenosarcoma remains to be clarified.
In this study, 12 cases of MMMT (9 uterine, 2 tubal, and 1 metastatic), 6 cases of mullerian adenosarcoma (three corporeal, two cervical, and one tubal), and 7 cases of primary uterine sarcomas had their tissues examined immunohistochemically for expression of CD10, desmin, myoglobin, alpha-smooth muscle actin (SMA), and cytokeratin. Of the primary uterine sarcomas, two were primary rhabdomyosarcomas (one cervical and one corporeal), two were ESSs, two were high-grade leiomyosarcomas, and one was a high-grade endometrial sarcoma. Sarcomatous components in all cases of MMMT and mullerian adenosarcoma, as well as all uterine sarcomas, were positive for CD10, showing moderate to marked staining intensity with varying distribution except in one MMMT, which showed weak and very focal staining. In four MMMTs, three adenosarcomas, and one rhabdomyosarcoma, myoglobin- and/or desmin-positive rhabdomyoblastic cells were positive for CD10. The immunoreactivity for CD10 showed the same distribution for alpha-SMA and myoglobin in three and two MMMTs, respectively. In five cases of MMMT, carcinomatous components were focally positive for CD10, and in two cases small populations of round or short spindle cells in sarcomatous components were positive for CD10, alpha-SMA, and cytokeratin (CAM5.2).
These results indicate that CD10 expression is not restricted to ESS but can be positive in MMMT and mullerian adenosarcoma as well as in a variety of uterine tumors including high-grade leiomyosarcoma and rhabdomyosarcoma. CD10 expression might be one of the characteristics of mullerian system-derived neoplastic mesenchymal cells.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES Leiomyosarcoma Absent CK and EMA staining
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSTIC FACTORS Surgical pathologic stage most important
Histological nature of the sarcomatous component has no bearing on prognosis
Early stage disease with 5YRS of 40-50%
Advanced disease with 5YRS of 25-30%
Pelvic lymph nodes may show metastases in 17% of cases where tumor grossly appears to be confined to the uterus PAP SMEAR An Abnormal Cervicovaginal Cytology Smear in Uterine Carcinosarcoma Is an Adverse Prognostic SignAnalysis of 25 Cases
Matthew J. Snyder, MD, etal.
Am J Clin Pathol 2004;122:434-439 Abstract quote
Carcinosarcoma of the uterus has been poorly characterized on cervicovaginal (Pap) smears, and we examine whether they effectively screen for carcinosarcoma and whether an abnormal Pap smear result has any clinical importance.
Twenty-five patients with histologically confirmed carcinosarcoma had a conventional Pap smear shortly before diagnosis. Eleven smears (44%) originally were read as abnormal (malignant or atypical), and 4 additional cases were read as abnormal on retrospective review (15/25 [60%]). All malignant elements were epithelial, and 2 cases (8%) had atypical spindle cells, but no diagnostic sarcoma.
Cervical involvement was the only histologic parameter correlating with an abnormal Pap smear result (P = .04). Univariate analysis found stage III or IV disease was an adverse prognostic sign compared with stage I or II disease (mean survival, 8 vs 36 months, respectively; P = .001), and multivariate analysis indicated that an abnormal Pap smear result correlated with worse survival (P = .023).
The conventional Pap smear is insensitive (60%) for detecting carcinosarcoma, but when the result is abnormal, the Pap is an important stage-independent adverse prognosticator.
Proliferation indices and p53-immunocytochemistry in uterine mixed mullerian tumors.
Nicotina PA, Ferlazzo G, Vincelli AM.
Department of Human Pathology, University of Messina, Italy.
Histol Histopathol 1997 Oct;12(4):967-72 Abstract quote
Mixed mullerian tumor (MMT) is a biphasic malignancy of elderly women. It, including both a carcinomatous and a sarcomatous component (CC and SC), is regarded as a female genital tract carcinosarcoma (FGTCS). Since current methods to grade CC and SC are not still univocal, the authors estimate mitotic index (MI) and MIB 1-immunolabeling index (MIB 1-LI) as common prognostic indices for the MMT components. They also compare above prognostic indices with p-53 immunocytochemistry, in MMTs.
The present study thus points out that: (a) MI of CC and SC areas is consistent with the respective conventional tumor grades; (b) MI averages of CC are higher than those observed in the SC areas; (c) MI and MIB 1-LI of the CC-tumor cells correlate reciprocally in a very significant fashion; (d) A diffuse strong p53 nuclear immunostaining (> 50% cells) is often patent where the highest MI and MIB 1-LI are found.
In conclusion, the authors propose MI and MIB 1-LI as two complementary useful indices to assess prognosis of MMTs. They also suggest p53 nuclear immunolabeling should be regarded as an independent biomarker of unfavourable MMT behaviour.
Is vascular and lymphatic space invasion a main prognostic factor in uterine neoplasms with a sarcomatous component? A retrospective study of prognostic factors of 60 patients stratified by stages.
Rovirosa A, Ascaso C, Ordi J, Abellana R, Arenas M, Lejarcegui JA, Pahisa J, Puig-Tintore LM, Mellado B, Armenteros B, Iglesias X, Biete A.
Department of Radiation Oncology, Hospital Clinic i Universitari, Barcelona, Spain.
Int J Radiat Oncol Biol Phys 2002 Apr 1;52(5):1320-9 Abstract quote
BACKGROUND: Sarcomatous neoplasms of the uterine corpus are still a challenge in terms of obtaining prognostic factors and the most optimum complementary treatment to surgery. The most important prognostic factor is stage; relapses usually appear during the first 2 years, and most patients die within the first 3 years. We have performed a multivariate study of prognostic factors, stratifying patients by stage, to determine their impact on overall survival, disease-free survival, local relapse-free survival, and distant metastasis-free survival. Special emphasis has been given to vascular and lymphatic space invasion (VLSI).
METHODS: Sixty patients diagnosed with uterine neoplasms with a main sarcomatous component were treated at Hospital Clinic i Universitari of Barcelona between January 1975 and June 1999. Pathologic type: 32 carcinosarcomas, 14 leiomyosarcomas, 9 adenosarcomas, and 5 endometrial stromal sarcomas. Treatment: 58/60 surgery, 35/60 postoperative radiotherapy, 2/60 exclusive chemotherapy, and 3/60 complementary chemotherapy. FIGO stages: 43 Stage I, 4 Stage II, 11 Stage III, and 2 Stage IV. Variables analyzed: age, stage, vascular and lymphatic space invasion, myometrial invasion, mitotic index, tumor size, unicentricity/multicentricity, necrosis, and radiotherapy. Statistics: the S and Cox proportional risk models. The partial effect of each risk factor was calculated by hazard ratio (HR) with a confidence interval of 95%.
RESULTS: Early stages: Multivariate analysis showed that tumor size larger than 8 cm and VLSI had an impact on overall survival (HR = 4.01 and HR = 24.45, respectively). VLSI was present in 23% of the cases. Myometrial invasion greater than 50% had an impact on disease-free survival and local relapse-free survival (HR was 9.75 and 3.20, respectively). VLSI had an impact on distant metastasis-free survival (HR = 2.92). Advanced stages: VLSI was present in 89% of the cases. Only leiomyosarcoma type made the overall survival worse (HR = 10.54).
CONCLUSIONS: Vascular and lymphatic space invasion was a relevant prognostic factor in our series, with an impact on overall survival and distant metastasis-free survival in early stages. In advanced stages, VLSI had no impact on survival, but was present in 89% of cases. Myometrial invasion >50% had an impact on local relapse. Advanced stages had a more aggressive behavior, and there was a higher incidence of poor prognostic factors in these stages. Nevertheless, prospective studies are still needed on prognostic factors and on the best treatment option.
Tumors confined to uterus (Stage I and II) treated with TAH-BSO
Follow with postoperative pelvic irradiation
Extrauterine spread (Stage III and IV) treated with TAH-BSO, excision of extra-uterine tumor, and postoperative pelvic irradiation-may add chemotherapy
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Last Updated September 9, 2004
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