Leiomyosarcoma is a malignant ( cancerous) smooth muscle tumor. A

benign tumor A benign tumor is a mass of cells (tumor) that does not invade neighboring tissue or metastasize (spread throughout the body). Compared to malignant (cancerous) tumors, benign tumors generally have a slower growth rate. Benign tumors have r ...

originating from the same tissue is termed leiomyoma. While leiomyosarcomas are not thought to arise from leiomyomas, some leiomyoma variants' classification is evolving. About one in 100,000 people are diagnosed with leiomyosarcoma (LMS) each year. LMS is one of the more common types of

soft-tissue sarcoma A soft-tissue sarcoma (STS) is a malignant tumour, a type of cancer, that develops in soft tissue. A soft tissue sarcoma is often a painless mass that grows slowly over months or years. They may be superficial or deep-seated. Any such unexplaine ...

, representing 10 to 20% of new cases. (Leiomyosarcoma of the bone is more rare.)

Sarcoma A sarcoma is a malignant tumor, a type of cancer that arises from transformed cells of mesenchymal (connective tissue) origin. Connective tissue is a broad term that includes bone, cartilage, fat, vascular, or hematopoietic tissues, and sa ...

is rare, consisting of only 1% of

cancer Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal bl ...

cases in adults. Leiomyosarcomas can be very unpredictable; they can remain

dormant Dormant, "sleeping", may refer to: Science *Dormancy Dormancy is a period in an organism's life cycle when growth, development, and (in animals) physical activity are temporarily stopped. This minimizes metabolic activity and therefore helps ...

for long periods of time and recur after years. It is a resistant cancer, meaning generally not very responsive to

chemotherapy Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs (chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemothe ...

or radiation. The best outcomes occur when it can be removed surgically with wide margins early, while small and still ''

in situ ''In situ'' (; often not italicized in English) is a Latin phrase that translates literally to "on site" or "in position." It can mean "locally", "on site", "on the premises", or "in place" to describe where an event takes place and is used in ...

''.

Mechanism

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 The uterus (from Latin ''uterus'', plural ''uteri'') or womb () is the organ in the reproductive system of most female mammals, including humans that accommodates the embryonic and fetal development of one or more embryos until birth. The ...

stomach The stomach is a muscular, hollow organ in the gastrointestinal tract of humans and many other animals, including several invertebrates. The stomach has a dilated structure and functions as a vital organ in the digestive system. The stomach i ...

small intestine The small intestine or small bowel is an organ in the gastrointestinal tract where most of the absorption of nutrients from food takes place. It lies between the stomach and large intestine, and receives bile and pancreatic juice through t ...

and retroperitoneum. Uterine leiomyosarcomas come from the smooth muscle in the muscle layer of the uterus. 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 The tunica media (New Latin "middle coat"), or media for short, is the middle tunica (layer) of an artery An artery (plural arteries) () is a blood vessel in humans and most animals that takes blood away from the heart to one or more parts o ...

). Thus, a leiomyosarcoma can have a primary site of origin anywhere in the body from a blood vessel. 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.

Diagnosis

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

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. 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 Docetaxel (DTX or DXL), sold under the brand name Taxotere among others, is a chemotherapy medication used to treat a number of types of cancer. This includes breast cancer, head and neck cancer, stomach cancer, prostate cancer and non-small-cell ...

/ trabectedin; pazopanib is the targeted therapy used in metastatic leiomyosarcoma as second line and is well tolerated. LMS of uterine origin often responds to hormonal treatments. As of 2020, several clinical trials for uterine LMS are active.

Notable cases

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. * Katie Price * Canadian public-health physician Sheela Basrur (1956–2008) developed uterine leiomyosarcoma in 2006. * American actress Diana Sands * 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. *

E. J. McGuire Edward John "E. J." McGuire (June 26, 1952 – April 7, 2011) was an American ice hockey coach who served as the director of the NHL Central Scouting Services. He earned his PhD in sports psychology from the University of Waterloo in 1990. Coach ...

, long-time professional ice hockey coach, scout, and vice president of the

National Hockey League The National Hockey League (NHL; french: Ligue nationale de hockey—LNH, ) is a professional ice hockey sports league, league in North America comprising 32 teams—25 in the United States and 7 in Canada. It is considered to be the top ranke ...

Central Scouting Bureau * Ellis Avery, American writer, two-time winner of the Stonewall Book Award *

Linda Uttley Linda Uttley (26 October 1966 – 27 November 2009) was an English rugby union footballer. She was born in Barnes, London and was the youngest of eight siblings. Her rugby career began in 1989 at Teddington Rugby Club. She moved to Wasps Rugby C ...

(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. *

Irene Hirano Irene Hirano Inouye (''née'' Yasutake; October 7, 1948 – April 7, 2020) was the founding President of the U.S.-Japan Council, a position she held ever since she helped create the organization in 2009 until her death. Hirano Inouye focused on b ...

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).

References

External links