Uterine Leiomyosarcoma - Causes, Symptoms, Diagnosis, and Treatment

Introduction

Leiomyosarcoma is a soft tissue tumor that can develop in any body part, like the digestive system, uterus, blood vessels, or urinary system. Leiomyosarcomas are very aggressive. It grows fast and invades nearby tissues. The diagnosis is usually difficult because there are no visible symptoms during the early stages of uterine leiomyosarcomas. Among the leiomyosarcomas, uterine leiomyosarcomas are common.

What Is Uterine Leiomyosarcoma?

Uterine leiomyosarcoma is a rare tumor forming in the smooth muscle lining of the uterus wall. It is a malignant tumor and can spread to other areas in the body, like the lungs or liver, which can cause life-threatening complications. Uterine leiomyosarcoma has a high rate of recurrence. After the treatment of leiomyosarcoma, there is a chance of recurrence in almost 50 percent of the cases.

What Are the Causes of Uterine Leiomyosarcoma?

The cause of uterine leiomyosarcoma is not clear. They often do not show any symptoms and usually occur very spontaneously. Some of the known causes are-

• Immunologic abnormalities.

• Genetic factors and abnormal gene mutations.

• Inherited conditions like Gardner syndrome (condition of having several colorectal polyp growths), Werner syndrome (an inherited disorder that characteristics rapid aging early in young adulthood), neurofibromatosis (tumors present in the brain and spinal cord), and several immunodeficiency syndromes.

• Environmental factors (ultraviolet radiations and ionizing radiations).

• Certain chemicals.

• Unhealthy diet.

• Due to stress.

• History of radiation therapy.

What Are the Symptoms of Uterine Leiomyosarcoma?

Signs and symptoms of uterine leiomyosarcoma depend on the tumor's location, size, and spread. Most of the leiomyosarcoma of the uterus are asymptomatic. Some of the common signs and symptoms are mentioned below.

• Abnormal vaginal bleeding.

• Post menopause bleeding.

• Pain in the pelvic region.

• Stomach pain.

• Abdominal bloating.

• Abnormal vaginal discharge.

• Alteration in bowel and bladder movements.

• Fatigue and weakness.

• Weight loss.

• Loss of appetite.

• Fever.

• Nausea and vomiting.

What Are the Stages of Uterine Leiomyosarcoma?

Uterine leiomyosarcoma’s stages based on the federation of obstetrics and gynecology -

• Stage 1 - When the tumor is only present in the uterus.

• Stage 2 - The tumor has spread to the cervix.

• Stage 3 - The tumor has spread to the abdomen, pelvis, and lymph nodes.

• Stage 4 - There is distant metastasis of the tumor. For example, the urinary bladder, rectum, lungs, or bones.

How Is Uterine Leiomyosarcoma Diagnosed?

Uterine leiomyosarcoma is diagnosed accidentally while operating on the fibroids, leiomyoma, or benign smooth muscle tumors in the uterus. Leiomyosarcoma is diagnosed when this tumor is pathologically examined. Other diagnosing aids are -

• Computed Tomography (CT) Scan - To locate the size, location, and extension of the tumor, imaging techniques are used. CT scan uses high x-ray radiation and computer technology to produce image slices or a cross-sectional image of the affected region.

• Magnetic Resonance Imaging (MRI) - Using a large magnetic field, radio waves, and computer technology, a three-dimensional image of the uterus and tumor is produced.

• Positron Emission Tomography (PET) - By using a radioactive drug, abnormal metabolic activities in the body are found by PET scan.

• Ultrasound - High-frequency sound waves are produced to create echoes which will get reflected and create images of the internal organs and tissues.

• Fine Needle Aspiration Biopsy - A thin hollow needle with a syringe is inserted into the tumor. A tissue sample is removed and observed under a microscope to confirm metastatic uterine leiomyosarcoma.

• Incisional Biopsy - When the fine needle aspiration biopsy is inconclusive, an incisional biopsy is done. In an incisional biopsy, a small tissue sample is removed surgically, processed, and observed under a microscope.

What Is the Treatment for Uterine Leiomyosarcoma?

The treatment for uterine leiomyosarcoma depends on the tumor location, the extent of spread, the degree of malignancy, involvement of the lymph nodes, and the patient’s age and health. The treatment for uterine leiomyosarcoma is -

• Surgical Therapy - The first treatment option for uterine leiomyosarcoma is to remove the complete tumor with any affected tissues. A hysterectomy (complete removal of the uterus) is usually performed. Bilateral salpingo-oophorectomy (when the ovary and fallopian tube on both sides are removed) is indicated for women who have metastases of uterine leiomyosarcoma.

• Chemotherapy - Cancer cells are killed, and the growth of the tumor is reduced by using high-dosage drug therapy. Chemotherapy can be used as an adjuvant to the surgical therapy. Before surgery, to shrink the tumor and after surgery to prevent the relapse of the tumor. A combination therapy is used, like Docetaxel, Doxorubicin, and Gemcitabine or Doxorubicin, Ifosfamide, or Cisplatin, followed by radiation therapy.

• Radiation Therapy - In the complete removal of the tumor, radiation therapy is usually not indicated. If the tumor has not been completely removed due to some reasons, then radiation therapy is indicated.

• Targeted Therapy - Using drugs on a specific site to kill the cancerous cells.

What Are the Side Effects After Treatment of Uterine Leiomyosarcoma?

Based on the treatment received, the side effects can vary from person to person. Common side effects seen are mentioned below -

• After surgical removal of the leiomyosarcoma, people may have infections, bleeding or blood clots, allergic reactions to anesthesia, or pain.

• During the chemotherapy, people may have tiredness, hair loss, anemia, diarrhea or constipation, mood swings, infections, nausea, and vomiting.

• After radiation therapy, tiredness, nausea, and itchy or dry skin can be seen.

What to Do if There Is a Relapse of Uterine Leiomyosarcoma?

If the tumor is completely removed, the chances of recurrence are a little low. Still, complete tumor removal is impossible if the tumor has metastasized to other organs and tissues like the lungs or liver. So there will be a recurrence of the tumor. In such cases, palliative chemotherapy is advised. Ifosfamide, Gemcitabine or Doxorubicin is given in such cases.

Conclusion

Uterine leiomyosarcoma is a rare tumor, but it is a malignant tumor. Due to its aggressive nature, the prognosis is questionable. But if it is diagnosed at an early stage, then the prognosis is good. The treatment of uterine leiomyosarcoma is decided by the opinion given by a series of doctors like a physician, gynecologist, radiologist, and medical oncologist. Uterine leiomyosarcoma has a high risk of recurrence. So routine examination is needed to prevent the recurrence of the tumor.