Leiomyosarcoma

Leiomyosarcoma (Gr. "smooth muscle connective tissue tumor"), also referred to as LMS, is a malignant (cancerous) smooth muscle tumor. A benign tumor originating from the same tissue is termed leiomyoma. It is also important to note that leiomyosarcomas do not arise from leiomyomas.^[1]

Leiomyosarcoma is a rare form of cancer, and accounts for 5–10% of soft-tissue sarcomas, which are in themselves relatively rare.^[2] Leiomyosarcomas can be very unpredictable. They can remain dormant for long periods of time and recur after years. It is a resistant cancer, meaning generally not very responsive to chemotherapy or radiation. The best outcomes occur when it can be removed surgically with wide margins early, while small and still in situ.^[3]

Location

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,^[4] stomach, small intestine and retroperitoneum.^[5]

Uterine leiomyosarcomas come from the smooth muscle in the muscle layer of the uterus.^[6] 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.^[3]

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.

Treatment

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.^[7] While LMS tends to be resistant to radiation and chemotherapy, each case is different and results can vary widely.

LMS of uterine origin do frequently, but not always respond to hormonal treatments.^[8][9]

Notable patients

Notable people who have had leiomyosarcoma include:

• Jim Boysen—software developer and recipient of world's first skull and scalp transplant^[10]
• Padma L. Atluri—writer for television series Men In Trees, 90210.^[11][12]
• Leicester City footballer Keith Weller who made over 300 appearances for the Foxes, scoring 47 goals. Also made 4 appearances for England, scoring 1 goal ^[13]
• Katie Price (Jordan)^[14]
• Canadian public health physician Sheela Basrur (1956–2008), who developed uterine leiomyosarcoma in 2006.^[15]
• American actress Diana Sands.
• 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."^[16]
• E. J. McGuire Long time professional ice hockey coach, scout, and VP of the NHL Central Scouting Bureau
• Durham, NC Kenneth Winston Cherry-Kenneth was first employed at his family’s seafood restaurant, the Breezeway, at Topsail Beach, a place that he loved. He then began a career in Insurance as the Medical Underwriter for Mid-South Insurance Company in Fayetteville, which he dedicated himself to for twenty years. </ref> http://www.hallwynne.com/kenneth-cherry/

See also

• Uterine sarcoma

References

External links

• Two Detailed Review Articles on Leiomyosarcoma
• LMS leiomyosarcoma.info Comprehensive reference site for information on leiomyosarcoma
• National Leiomyosarcoma Foundation
• Leiomyosarcoma Direct Research Foundation
• Sloan Kettering website
• #2434 (Pathology images)
• Gastrointestinal Stromal Tumors at eMedicine
• Live Leiomyosarcoma Video (Gupta Gastro Associates Unique Findings)