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. It is therefore possible for leiomyosarcomas to 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, also 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 where there is 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. There is a wide differential diagnosis, which includes spindle cell carcinoma, spindle cell melanoma, fibrosarcoma, malignant peripheral nerve sheath tumor and even biphenotypic sinonasal sarcoma.

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.[9] 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 are - doxorubicin/ ifosfamide and doxorubicin combination/ gemcitabine and docetaxel/ trabectedin[10] pazopanib is the targeted therapy used in metastatic leiomyosarcoma as second line and is well tolerated.[11]

LMS of uterine origin do frequently, but not always, respond to hormonal treatments.[12][13]

Notable people who have had leiomyosarcoma include:

• Jim Boysen—software developer and recipient of world's first skull and scalp transplant[14]
• Leicester City footballer Keith Weller who made over 300 appearances for the Foxes, scoring 47 goals. Also made four appearances for England, scoring one goal[15]
• Katie Price (Jordan)[16]
• Canadian public health physician Sheela Basrur (1956–2008), who developed uterine leiomyosarcoma in 2006.[17]
• American actress Diana Sands.[18]
• Canadian comedian Irwin Barker, who was featured in a documentary, "That's my Time", which chronicled his battle with leiomyosarcoma. In Barker's own words. "Cancer has my body but not my spirit, and I'll continue to make jokes, not so much about cancer, but in spite of it."[19]
• E. J. McGuire Long time professional ice hockey coach, scout, and VP of the NHL Central Scouting Bureau
• Dr. Amy Reed (US physician who campaigned for discontinuing the use of morcellators, which have been shown to spread the disease)[20]
• Patricia Elliott, Tony-winning theatre and soap opera actress[21]
• Ellis Avery, America Writer, two-time winner of the Stonewall Book Award.

• Uterine sarcoma

• Gastrointestinal Stromal Tumors at eMedicine