Leiomyosarcoma

Smooth muscle cells make up the involuntary muscles, which are found in most parts of the body, including the uterus, stomach and intestines, the walls of all blood vessels, and the skin, so leiomyosarcomas can appear at any site in the body. They are most commonly found in the uterus,[5] stomach,[6] small intestine and retroperitoneum.[7]

Uterine leiomyosarcomas come from the smooth muscle in the muscle layer of the uterus.[8] Cutaneous leiomyosarcomas derive from the pilo-erector muscles in the skin. Gastrointestinal leiomyosarcomas might come from smooth muscle in the GI tract, or alternatively, from a blood vessel. At most other primary sites—retroperitoneal extremity (in the abdomen, behind the intestines), truncal, abdominal organs, etc.—leiomyosarcomas appear to grow from the muscle layer of a blood vessel (the tunica media). Thus, a leiomyosarcoma can have a primary site of origin anywhere in the body from a blood vessel.[4]

The tumors are usually hemorrhagic and soft and microscopically marked by pleomorphism, abundant (15–30 per 10 high-power fields) abnormal mitotic figures, and coagulative tumor cell necrosis. The differential diagnosis, which includes spindle cell carcinoma, spindle cell melanoma, fibrosarcoma, malignant peripheral nerve sheath tumor and even biphenotypic sinonasal sarcoma, is wide.

Histopathology of leiomyosarcoma shows variable atypia, often with cytoplasmic vacuoles at both ends of nuclei, and frequent mitoses.[9]

Diagnosis of LMS is made by performing a soft-tissue biopsy and examining its histopathology.

Surgery, with as wide a margin of removal as possible, has generally been the most effective and preferred way to attack LMS. If surgical margins are narrow or not clear of tumor, however, or in some situations where tumor cells were left behind, chemotherapy or radiation has been shown to give a clear survival benefit.[10] While LMS tends to be resistant to radiation and chemotherapy, each case is different and results can vary widely.

For metastatic (widespread) disease, chemotherapy and targeted therapies are the first choices. Chemotherapy regimens include: doxorubicin/ifosfamide and doxorubicin combination/gemcitabine and docetaxel/trabectedin;[11] pazopanib is the targeted therapy used in metastatic leiomyosarcoma as second line and is well tolerated.[12]

LMS of uterine origin often responds to hormonal treatments.[13][14] As of 2020, several clinical trials for uterine LMS are active.[15]

People who have had leiomyosarcoma include:

• Leicester City footballer Keith Weller, who made over 300 appearances for the Foxes, scored 47 goals. Also, he made four appearances for England, scoring one goal.[16]
• Katie Price[17]
• Canadian public-health physician Sheela Basrur (1956–2008) developed uterine leiomyosarcoma in 2006.[18]
• American actress Diana Sands[19]
• The first year of treatment for leiomyosarcoma of Canadian comedian Irwin Barker was the subject of a 2008 television documentary, That's My Time; he died in 2010.[20]
• E. J. McGuire, long-time professional ice hockey coach, scout, and vice president of the National Hockey League Central Scouting Bureau
• Ellis Avery, American writer, two-time winner of the Stonewall Book Award
• Linda Uttley (1966–2009), English rugby union footballer in the Women's England Team, was diagnosed with leiomyosarcoma in 2007 and died in 2009 at the age of 43.[21]
• Irene Hirano Inouye, founding President of the U.S.-Japan Council, a position she held ever since she helped create the organization in 2009 (see her Wikipedia entry for more information).

• Uterine sarcoma

• Gastrointestinal Stromal Tumors at eMedicine