| Leiomyosarcoma |
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Leiomyosarcoma of the adrenal vein. Coronal view of abdominal MRI. Tumor (arrow) extends from the superior pole of the right kidney to the right atrium.
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| Classification and external resources |
| Specialty |
Hematology and Oncology |
| ICD-10 |
C49.M48 |
| ICD-O |
M8890/3 |
| DiseasesDB |
34362 |
| MedlinePlus |
000916 |
| eMedicine |
med/1180 |
| MeSH |
D007890 |
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[edit on Wikidata]
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Leiomyosarcoma (Gr. "smooth muscle connective tissue tumor"), also referred to as LMS, is a malignant (cancerous) smooth muscle tumor. It must not be confused with leiomyoma, which is a benign tumor originating from the same tissue. It is also important to note that leiomyosarcomas do not arise from leiomyomas.[1]
Leiomyosarcoma is an extremely 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]
Contents
- 1 Location
- 2 Treatment
- 3 Notable patients
- 4 See also
- 5 References
- 6 External links
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.[citation needed]
- 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
See also
References
- ^ Kumar, Vinay; Abbas, Abul; Aster, Jon (2015). Robbins and Cotran Pathologic Basis of Disease. Philadelphia, PA: Elsevier. pp. 1020–1021. ISBN 978-1-4557-2613-4.
- ^ Weaver MJ, Abraham JA (2007). "Leiomyosarcoma of the Bone and Soft Tissue: A Review". ESUN. 4 (2).
- ^ a b "Basic info". www.leiomyosarcoma.info. LMS leiomyosarcoma.
- ^ Arnold LM, Burman SD, O-Yurvati AH (April 2010). "Diagnosis and management of primary pulmonary leiomyosarcoma". J Am Osteopath Assoc. 110 (4): 244–6. PMID 20430913.
- ^ Piovanello P, Viola V, Costa G, et al. (2007). "Locally advanced leiomyosarcoma of the spleen. A case report and review of the literature". World J Surg Oncol. 5 (1): 135. doi:10.1186/1477-7819-5-135. PMC 2221972. PMID 18045454.
- ^ Sue Ghosh; Jonathan Hecht; Tanaz Ferzandi,; Christopher Awtrey (2007). "Leiomyosarcoma of the Uterus (ULMS): A Review". The Liddy Shriver Sarcoma Initiative. Retrieved 1 February 2016.
- ^ "About Chemotherapy". www.leiomyosarcoma.info. LMS leiomyosarcoma. August 2002.
- ^ Abu-Rustum, N. R.; Curtin, J. P.; Burt, M.; Jones, W. B. (1997). "Regression of uterine low-grade smooth-muscle tumors metastatic to the lung after oophorectomy". Obstetrics and gynecology. 89 (5 Pt 2): 850–852. doi:10.1016/S0029-7844(97)00033-1. PMID 9166348.
- ^ Hardman, M. P.; Roman, J. J.; Burnett, A. F.; Santin, A. D. (2007). "Metastatic Uterine Leiomyosarcoma Regression Using an Aromatase Inhibitor". Obstetrics & Gynecology. 110 (2 Pt 2): 518–520. doi:10.1097/01.AOG.0000267533.56546.c2. PMID 17666649.
- ^ "World's first skull and scalp transplant performed on cancer sufferer with huge hole in head".
- ^ "deadline.com".
- ^ "pforpadma.org".
- ^ "Leicester legend Weller mourned". BBC Online. 2004-11-13. Retrieved 2010-05-14.
- ^ "Jordan treated for cancer". BBC Online. 2002-08-11. Retrieved 2009-10-30.
- ^ Gillespie, Kerry (2008-04-12). "'Can't ever give up hope,' Basrur says". The Toronto Star. Retrieved 2010-01-08.
- ^ "That's My Time". www.irwinbarker.com. Archived from the original on 2008-10-02. Retrieved 5 March 2015.
In Irwin'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.”
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)
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Connective/soft tissue tumors and sarcomas (ICD-O 8800–9059) (C45–C49/D17–D21, 171/214–215)
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| Not otherwise specified |
- Soft-tissue sarcoma
- Desmoplastic small-round-cell tumor
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| Connective tissue neoplasm |
| Fibromatous |
| Fibroma/fibrosarcoma: |
- Dermatofibrosarcoma protuberans
- Desmoplastic fibroma
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| Fibroma/fibromatosis: |
- Aggressive infantile fibromatosis
- Aponeurotic fibroma
- Collagenous fibroma
- Diffuse infantile fibromatosis
- Familial myxovascular fibromas
- Fibroma of tendon sheath
- Fibromatosis colli
- Infantile digital fibromatosis
- Juvenile hyaline fibromatosis
- Plantar fibromatosis
- Pleomorphic fibroma
- Oral submucous fibrosis
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| Histiocytoma/histiocytic sarcoma: |
- Benign fibrous histiocytoma
- Malignant fibrous histiocytoma
- Atypical fibroxanthoma
- Solitary fibrous tumor
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|
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| Myxomatous |
- Myxoma/myxosarcoma
- Cutaneous myxoma
- Superficial acral fibromyxoma
- Angiomyxoma
- Ossifying fibromyxoid tumour
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| Fibroepithelial |
- Brenner tumour
- Fibroadenoma
- Phyllodes tumor
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| Synovial-like |
- Synovial sarcoma
- Clear-cell sarcoma
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|
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| Lipomatous |
- Lipoma/liposarcoma
- Myelolipoma
- Myxoid liposarcoma
- PEComa
- Chondroid lipoma
- Intradermal spindle cell lipoma
- Pleomorphic lipoma
- Lipoblastomatosis
- Spindle cell lipoma
- Hibernoma
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| Myomatous |
| general: |
|
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| smooth muscle: |
|
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| skeletal muscle: |
- Rhabdomyoma/rhabdomyosarcoma: Embryonal rhabdomyosarcoma
- Alveolar rhabdomyosarcoma
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- Leiomyoma
- Angioleiomyoma
- Angiolipoleiomyoma
- Genital leiomyoma
- Leiomyosarcoma
- Multiple cutaneous and uterine leiomyomatosis syndrome
- Multiple cutaneous leiomyoma
- Neural fibrolipoma
- Solitary cutaneous leiomyoma
- STUMP
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|
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| Complex mixed and stromal |
- Adenomyoma
- Pleomorphic adenoma
- Mixed Müllerian tumor
- Mesoblastic nephroma
- Wilms' tumor
- Malignant rhabdoid tumour
- Clear-cell sarcoma of the kidney
- Hepatoblastoma
- Pancreatoblastoma
- Carcinosarcoma
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| Mesothelial |
- Mesothelioma
- Adenomatoid tumor
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Tumors: female urogenital neoplasia (C51–C58/D25–D28, 179–184/218–221)
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| Adnexa |
| Ovaries |
Glandular and epithelial/
surface epithelial-
stromal tumor |
| CMS: |
- Ovarian serous cystadenoma
- Mucinous cystadenoma
- Cystadenocarcinoma
- Papillary serous cystadenocarcinoma
- Krukenberg tumor
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- Endometrioid tumor
- Clear-cell ovarian carcinoma
- Brenner tumour
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| Sex cord-gonadal stromal |
- Leydig cell tumour
- Sertoli cell tumour
- Sertoli-Leydig cell tumour
- Thecoma
- Granulosa cell tumour
- Luteoma
- Sex cord tumour with annular tubules
- Steroid cell tumor (NOS)
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| Germ cell |
- Dysgerminoma
- Nongerminomatous
- Embryonal carcinoma
- Endodermal sinus tumor
- Gonadoblastoma
- Teratoma/Struma ovarii
- Choriocarcinoma
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| Fibroma |
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| Fallopian tube |
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| Uterus |
| Myometrium |
- Uterine fibroids/leiomyoma
- Leiomyosarcoma
- Adenomyoma
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| Endometrium |
- Endometrioid tumor
- Uterine papillary serous carcinoma
- Clear cell carcinoma
- Endometrial intraepithelial neoplasia
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| Cervix |
- Cervical intraepithelial neoplasia
- SCC
- Glassy cell carcinoma
- Villoglandular adenocarcinoma
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| Placenta |
- Choriocarcinoma
- Gestational trophoblastic disease
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| General |
- Uterine sarcoma
- Mixed Müllerian tumor
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| Vagina |
- SCC
- Botryoid rhabdomyosarcoma
- Clear cell adenocarcinoma of the vagina
- Vaginal intraepithelial neoplasia
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| Vulva |
- SCC
- Melanoma
- Papillary hidradenoma
- Extramammary Paget's disease
- Vulvar intraepithelial neoplasia
- Bartholin gland carcinoma
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