What is Epithelioid Leiomyosarcoma?
Epithelioid leiomyosarcoma is a specific type of smooth muscle cell tumour. Smooth muscles are also named involuntary muscles. Smooth muscles are found in the hollow organs, these include the stomach, intestines, urinary bladder, and uterus. These are also found in the walls of the blood vessels, and respiratory, gastrointestinal, and genitourinary tract. Epithelioid leiomyosarcoma can occur in the abdomen, retroperitoneum, large blood vessels, and uterus, but the most common site of its development is the uterus. Leiomyosarcoma is the most common pure uterine sarcoma, which accounts for around 1% of all uterine malignancies. Epithelioid and myxoid are the most common morphological variants of uterine leiomyosarcoma.
Disease Etiology
Epithelioid leiomyosarcoma has no known etiology; in most cases, a genetic mutation is considered the underlying cause, while in very rare cases a preexisting uterine fibroid may be responsible for uterine epithelioid leiomyosarcoma.
Disease Epidemiology
The mean age of development of epithelioid leiomyosarcoma is 51 years.
Sign and Symptoms
Signs and symptoms vary according to the site of the origin of the malignant tumour (cancer). Common signs and symptoms are; weight loss, Lethargy (fatigue), and Fever.
- Signs and symptoms of uterine epithelioid leiomyosarcoma include abnormal vaginal bleeding and pelvic mass. In most cases, it is diagnosed incidentally during a hysterectomy (surgical removal of the uterus) for fibroids (benign tumours of the uterus). They mostly appear in the 5Th decade of life.
- Retroperitoneal Epithelioid leiomyosarcoma presents with the sign and symptoms of an abdominal mass, abdominal heaviness, and pain.
- Leiomyosarcoma of the colon (large intestine) presents with abdominal pain and gastrointestinal bleeding (bleeding during or after defecation, with stool). They mostly appear in the 5th or 6th decades of life.
- Intraluminal vascular leiomyosarcoma may present with the symptoms of thrombosis (formation of a blood clot within the major veins of the heart) in the inferior vena cava.
Diagnosis
Diagnostic tests are:
- Diagnosis through radiographic and imaging technology by using CT-scan, PET-scan, MRI, and X-ray.
- Tumour or tumour site biopsy
Treatment
Treatment options include surgical removal of the tumour, Chemotherapy, Radiotherapy, and immunotherapy. The selection of the right treatment depends upon the type and staging (site and size) of the tumours. It also depends on the treatment efficacy and general health of the patient.
What Support can we Give for Epithelioid Leiomyosarcoma?
Epithelioid Leiomyosarcoma is rare cancer, meaning it is not as well known as other forms of cancer. Without a Ribbon is an Australian organisation that provides support for individuals who suffer from rare cancers. So, we provide a designated platform for Warriors to obtain information specific to their Rare Cancer. We also provide annual opportunities for our Warriors to meet and learn from each other. If you suffer from rare cancer such as Epithelioid Leiomyosarcoma, we can help and support you through your journey thanks to the generous donations we receive. Click the link below to sign up and become a Warrior today!
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