Mixed Müllerian tumor

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Mixed Müllerian tumor
Other namesMalignant mixed mesodermal tumor (MMMT)
gynecology

A malignant mixed Müllerian tumor, also known as malignant mixed mesodermal tumor (MMMT) is a

nulliparity. Less well-understood but potential risk factors include tamoxifen therapy and pelvic irradiation.[1]

Classification

There is debate over the naming of MMMT; the term

Müllerian duct – first from a stem cell into a population of cells, that then differentiates into epithelial and stromal components.[1][2]

There is evidence that some tumors are better explained by the composition theory, due to the aggressive nature of the epithelial cells involved which tend to metastasize much more readily than the sarcomal component. The behavior of MMMT overall is more related to the type and grade of the epithelium than the sarcoma, which suggests the sarcomal portion is an atypical "bystander" than primary driver of the tumor. Despite this, when purely endometrial tumors are compared to MMMTs, the MMMT tumor tends to have a worse prognosis.[1]

Morphology

Carcinosarcoma of the uterus

In gross appearance, MMMTs are fleshier than

cervical os. On histology, the tumors consist of adenocarcinoma (endometrioid, serous or clear cell) mixed with the malignant mesenchymal (sarcoma
) elements; alternatively, the tumor may contain two distinct and separate epithelial and mesenchymal components. Sarcomatous components may also mimic extrauterine tissues (e.g., striated muscle, cartilage, adipose tissue, and bone). Metastases usually contain only epithelial components.

Prognosis

Outcome of MMMTs is determined primarily by depth of invasion and stage. As with endometrial carcinomas, the prognosis is influenced by the grade and type of the adenocarcinoma, being poorest with serous differentiation. MMMTs are highly malignant; a stage I tumor has an expected five-year survival rate of 50%, while the overall five-year survival rate is less than 20%.[1]

Staging of uterine MMMTs is as follows:[3]

  • Stage I. Carcinoma is confined to the
    corpus uteri
    itself.
  • Stage II. Carcinoma involves the corpus and the cervix.
  • Stage III. Carcinoma extends outside the uterus but not outside the
    lesser pelvis
    .
  • Stage IV. Carcinoma extends outside the true pelvis or involves the mucosa of the bladder or the rectum.

A Cochrane review [4] indicates women with high stage uterine carcinosarcoma (stage 3 or 4) who were treated with combination chemotherapy including Ifosfamide, were at lower risk of disease progression and death than those women treated with Ifosfamide alone. However, more women experienced side effects with combination therapy than with ifosamide alone. Radiotherapy to the abdomen was not associated with improved survival.

References

External links