Learn about your diagnosis

Uterus – Carcinosarcoma

 

endometrial carcinosarcoma

by Emily Goebel, MD FRCPC, reviewed on March 24, 2019

Quick facts:
  • Endometrial carcinosarcoma is an aggressive form of cancer that starts in the endometrium, the tissue that lines the inside of the uterus.
  • It is called carcinosarcoma because unlike most tumours, it made up of two parts, a carcinoma and a sarcoma, that look different when examined under the microscope.

In this article you will learn about:

  • The normal uterus and endometrium
  • What is endometrial carcinosarcoma?
  • How do pathologists make this diagnosis?
  • Immunohistochemistry
  • Histologic grade (FIGO)
  • Myometrial invasion
  • Cervical stromal involvement
  • Other tissues involved
  • Lymph nodes
  • Lymphovascular invasion
  • Margins
  • Tumour size
  • Pathologic stage
The normal uterus and endometrium

The uterus is a pear-shaped hollow organ found in the female pelvis between the rectum (the end of the large bowel) and the urinary bladder. The upper part of the uterus (fundus) is attached to the fallopian tubes while the lower part is connected to the vagina through the uterine cervix.

The walls of the uterus are made up of three layers:

  • Endometrium – The endometrium is the tissue on the inner surface of the uterus. The endometrium is made up of endometrial glands lined by one layer of cells that form a barrier called the epithelium. The epithelium is surrounded by supporting tissue called endometrial stroma.
  • Myometrium – The myometrium is the middle layer and is made up of smooth muscle which allows the uterus to change size and contract.
  • Perimetrium – The perimetrium is a thin layer of tissue that surrounds the outside of the uterus.
What is endometrial carcinosarcoma?

Endometrial carcinosarcoma is a type of cancer that develops in the endometrium.  It is called ‘carcinosarcoma’ because unlike most tumours, it made up of two parts, a carcinoma and a sarcoma, that look different when examined under the microscope.

The carcinoma part is made up of abnormal epithelial cells which are the type of cell that normally line the inside of the endometrium.  The sarcoma part is made up of abnormal spindle cells which the type of cell that are normally found in connective tissue. Both parts of the tumour are cancerous.

How do pathologists make this diagnosis?

When your pathologist examines the tumour under the microscope they will often describe the look of both the carcinomatous and sarcomatous parts in the tumor.  The carcinomatous part can look like other more common types of cancer that start in the endometrium including endometrioid carcinoma, serous carcinoma, or clear cell carcinoma.

The sarcomatous part can have homologous or heterologous elements. Homologous means that this sarcomatous component looks like the connective tissue that is normally found in the uterus, such as stroma or smooth muscle.  Heterologous means that the sarcomatous part looks like connective tissue that is not normally found inside the uterus, such as skeletal muscle (which pathologists call rhabdomyosarcoma) or cartilage (which pathologists call chondrosarcoma).  The presence of heterologous elements is important because it may be associated with worse prognosis.

Another name for carcinosarcoma is malignant mixed mullerian tumor (MMMT).

Endometrial carcinosarcoma is usually diagnosed after a small sample of tissue is removed from your endometrium in a procedure called a biopsy or curettage.. Once the diagnosis is made, the tumour is removed in a resection specimen known as a hysterectomy.

Immunohistochemistry

Your pathologist may perform a test called immunohistochemistry on your tissue sample to confirm the diagnosis.

The carcinoma part is usually positive for proteins called cytokeratins. The sarcoma part may or may not be positive for cytokeratins, and can be positive for proteins called desmin or actin.

The entire tumor is usually strongly positive of p53.  If there is a part of the tumour that looks like rhabdomyosarcoma (see above), it will be positive for the muscle markers myogenin and MyoD1.

Histologic grade (FIGO)

Grade is a word pathologists use to describe how different the cancer cells look compared to the normal cells in that same location. Low grade tumours look very similar to normal cells while high grade tumours look very little like the normal cells usually found in that location.

Why is this important? All endometrial carcinosarcomas are high grade tumours and are not usually given a FIGO grade. As such, under the section for grade in your report you may see “not applicable”.  If the tumour is graded, it will be given the highest grade (grade 3).

Myometrial invasion

The myometrium is a thick band of muscle just below the endometrium. The movement of cancer cells from the endometrium into the myometrium is called myometrial invasion.

The amount of myometrial invasion will be described in millimeters and as a percentage of the total myometrial thickness. Myometrial invasion will only be described in your report after the entire tumour has been removed.

Why is this important? Myometrial invasion is an important prognostic feature and is used to determine the tumour stage (see Pathological stage below).

Cervical stromal involvement

The cervix is a structure at the very bottom of the uterus. The cervix connects directly with the endometrium. The wall of the cervix is made up of a type of tissue called stroma.

Carcinosarcoma may grow from the endometrium into the cervix. After the tumour is removed completely, your pathologist will carefully examine the tissue from the cervix to see if there are any cancer cells in the cervical stroma.

Why is this important? Finding cancer cells in the cervical stroma increases the tumour stage (see Pathologic stage below).

Other tissues involved

Several other organs and tissues are directly attached or very close to the uterus including the ovaries, fallopian tubes, vagina, bladder, and rectum.

Why is this important? Cancer cells directly growing into any of these structures by carcinosarcoma is associated with poor prognosis and will be described in your report.

Lymph nodes

​Lymph nodes are small immune organs located throughout the body. Cancer cells can travel from the tumour to a lymph node through lymphatic channels located in and around the tumour (see Lymphovascular invasion above). The movement of cancer cells from the tumour to a lymph node is called a metastasis.

Your pathologist will carefully examine all lymph nodes for cancer cells. Lymph nodes that contain cancer cells are often called positive while those that do not contain any cancer cells are called negative. Most reports include the total number of lymph nodes examined and the number, if any, that contain cancer cells.

Lymph nodes on the same side as the tumour are called ipsilateral while those on the opposite side of the tumour are called contralateral.

Lymph nodes examined are usually divided into those found in the pelvis and those found around a large blood vessel in the abdomen called the aorta. The lymph nodes found around the aorta are called para-aortic.

If cancer cells are found in a lymph node, the size of the area involved by cancer will be measured and described in your report.

  • Isolated tumour cells – The area inside the lymph node with with cancer cells is less than 0.2 millimeters in size.
  • Micrometastases – The area inside the lymph node with with cancer cells is more than 0.2 millimeters but less than 2 millimeters in size.
  • Macrometastases – The area inside the lymph node with with cancer cells is more than 2 millimeters in size.

Why is this important? Cancer cells found in a lymph node is associated with a higher risk that the cancer cells will be found in other lymph nodes or in a distant organ such as the lungs. The number of lymph nodes with cancer cells is also used to determine the nodal stage (see Pathologic stage below).

Lymphovascular invasion

Blood moves around the body through long thin tubes called blood vessels. Another type of fluid called lymph which contains waste and immune cells moves around the body through lymphatic channels.

Cancer cells can use blood vessels and lymphatics to travel away from the tumour to other parts of the body. The movement of cancer cells from the tumour to another part of the body is called metastasis.

Before cancer cells can metastasize, they need to enter a blood vessel or lymphatic. This is called lymphovascular invasion.

Why is this important? Lymphovascular invasion increases the risk that cancer cells will be found in a lymph node or a distant part of the body such as the lungs.

Margins

A margin is the normal tissue that surrounds a tumour and is removed with the tumour at the time of surgery. The margins will only described in cases where the tumour extends into the cervical stroma or other tissues surrounding the uterus and after the entire tumour has been removed.

The type and number of margins described in your report will depend on the type of surgical procedure performed to remove the tumour.

Why is this important? Cancer cells found at the margin (positive margin) increase the risk that the tumour will grow back after treatment.

Tumour size

This is the size of the tumour measured in millimeters. The tumour size is only measured after the entire tumour has been removed.

Pathologic stage

​The pathologic stage for carcinosarcoma is based on the TNM staging system, an internationally recognized system originally created by the American Joint Committee on Cancer.

This system uses information about the primary tumour (T), lymph nodes (N), and distant metastatic disease (M)  to determine the complete pathologic stage (pTNM). Your pathologist will examine the tissue submitted and give each part a number. In general, a higher number means more advanced disease and worse prognosis.

Pathologic stage is not reported on a biopsy specimen. It is only reported when the entire tumour has been removed in an excision or resection specimen.

Tumour stage (pT) for carcinosarcoma:

Endometrial carcinosarcoma is given a tumour stage between T1 and T4 based on the depth of myometrial invasion and growth of the tumour outside of the uterus.

  • T1 – The tumour only involves the uterus.
  • T2 – The tumour has grown to involve the cervical stroma.
  • T3 – The tumour has grown through the wall of the uterus and is now on the outer surface of the uterus OR it has grown to involve the fallopian tubes or ovaries.
  • T4 – The tumour has grown directly into the bladder or the colon.

Nodal stage (pN) for carcinosarcoma:

Endometrial carcinosarcoma is given an nodal stage from N0 to N2 based on the examination of lymph nodes from the pelvis and abdomen.

  • N0 – No cancer cells were found in any of the lymph nodes examined.
  • N1mi – Cancer cells were found in at least one lymph node from the pelvis but the area with cancer cells was not larger than 2 millimeters (only isolated cancer cells or micrometastasis).
  • N1a – Cancer cells were found in at least one lymph node from the pelvis and the area with cancer cells was greater than 2 millimeters (macrometastasis).
  • N2mi – Cancer cells were found in at least one lymph node outside the pelvis but the area with cancer cells was not larger than 2 millimeters (only isolated cancer cells or micrometastasis).
  • N2a – Cancer cells were found in at least one lymph node outside the pelvis and the area with cancer cells was greater than 2 millimeters (macrometastasis).
  • NX – No lymph nodes were sent for examination.

Metastatic stage (pM) for carcinocarcoma:

Endometrial carcinosarcoma is given a metastatic stage of M0 or M1 based on the presence of cancer cells at a distant site in the body (for example the lungs). The metastatic stage can only be determined if tissue from a distant site is sent for pathological examination.

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