8890/3 Leiomyosarcoma, NOS


Definitions

Tumours of the central nervous system
ICD-O-3 topography code: C70, C71 and C72
ICD10: C70
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma 1
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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Stomach
ICD-O-3 topography code: C16
ICD10: C16
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
2
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.

Smooth-muscle tumours with atypia and mitotic activity are designated leiomyosarcomas, and such tumours are distinctly rare in the stomach. Cases with ambiguous criteria of malignancy should be designated as having uncertain malignant potential, and complete excision and follow-up is necessary.



Corpus uteri
ICD-O-3 topography code: C54
ICD10: C54
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
3
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.

Leiomyosarcomas of the uterine corpus are typically solitary intramural masses with poorly defined margins. The diameter averages 8 cm. Zones of haemorrhage and necrosis are characteristic. The tumour may spread locally, regionally or by haematogenous dissemination. Local and regional spread may result in an abdominal or pelvic mass and gastrointestinal or urinary tract symptoms. The usual destination of haematogenous dissemination are the lungs.
Leiomyosarcoma is the most common pure uterine sarcoma. It principally arises in adults (median age 50-55 years). The tumour is highly malignant, the overall 5-year survival rate ranges from 15-25%. In most cases, recurrences are detected within two years. Major prognostic factor is the extent of tumour spread
4
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



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Skin
ICD-O-3 topography code: C44
ICD10: C44
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
5
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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Urinary system: Renal pelvis, ureter, bladder, urethra
ICD-O-3 topography code: C65-C68
ICD10: C65-C68
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
6
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.



Fallopian tube and Uterine ligaments
ICD-O-3 topography code: C57
ICD10: C57
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
7
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.



Kidney
ICD-O-3 topography code: C64
ICD10: C64
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
8
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.

Primary renal leiomyosarcoma is rare, but it is the most common renal sarcoma (50-60% of cases). It occurs chiefly in adults; men and women are equally affected. Tumours are typically large, solid, and focally necrotic. Renal leiomyosarcoma metastasizes to lung, liver, and bone. The prognosis is poor; most patients die within 1 year of diagnosis. Complete surgical excision is the only curative therapy. Small tumour size (<5cm), low histological grade, and limitation to the kidney are associated with a more favourable outcome
9
 
Eble JE, Sauter G, Epstein JI, Sesterhenn IA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs.
3rd Edition
IARC Press: Lyon 2004



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Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49
ICD10: C49
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
10
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.

Soft tissue leiomyosarcoma arises in intramuscular and subcutaneous locations. The most commonly affected site is the retroperitoneum, including the pelvis. Another distinctive subset of leiomyosarcomas arise in large blood vessels. Nonretroperitoneal leiomyosarcomas most frequently develop in the lower extremities. The tumours typically form fleshy masses. Larger lesions may contain areas of haemorrhage, necrosis, or cystic change. Tumour size depends on the location; retroperitoneal leiomyosarcomas are usually large (>10 cm) and may extend into adjacent organs, while nonretroperitoneal lesions tend to be smaller
11
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.
Soft-tissue leiomyosarcoma most commonly affects middle-aged or older persons. Retroperitoneal and inferior vena cava leiomyosarcomas show a distinct predilection for women, while tumours in other soft tissue sites affect both men and women in approximately equal proportion. The prognosis depends chiefly on tumour location and size; additional reliable prognostic factors are histological grading and osseous/vascular involvement. Retroperitoneal leiomyosarcomas are fatal in the great majority of cases. Leiomyosarcomas of large vessels also tend to have a poor prognosis, while nonretroperitoneal soft tissue leiomyosarcomas are generally more favourable in outlook
12
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.



Vulva
ICD-O-3 topography code: C51
ICD10: C51
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
13
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.

Vulvar leiomyosarcomas affect adult women. The tumours present as rapidly enlarging masses that may be associated with pain. Most cases are high grade tumours with necrosis, infiltrative margins, cytological atypia and mitotic activity
14
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



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Cervix uteri
ICD-O-3 topography code: C53
ICD10: C53
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
15
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.

Leiomyosarcoma is the most common primary cervical sarcoma. It forms either a mass replacing and expanding the cervix or a polypoid growth. Areas of necrosis or haemorrhage are common. The tumour arises in adults. Patients typically present with vaginal bleeding or discharge. The prognosis is poor
16
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003







Esophagus
ICD-O-3 topography code: C15
ICD10: C15
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
17
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.

In the oesophagus , malignant smooth-muscle tumours are very rare. They are recognized by a histological resemblance to smooth muscle, nuclear atypia, mitotic activity, and immunohistochemical presence of SMA and desmin. Many reported examples have presented as polypoid intraluminal masses, and prognosis has been poor
18
Click to access Pubmed
Miettinen M, Sarlomo-Rikala M, Sobin LH, Lasota J (2000)
Esophageal stromal tumors: a clinicopathologic, immunohistochemical, and molecular genetic study of 17 cases and comparison with esophageal leiomyomas and leiomyosarcomas.
Am J Surg Pathol 24: 211-22



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Liver and intrahepatic bile ducts
ICD-O-3 topography code: C22
ICD10: C22
           

An uncommon, aggressive malignant smooth muscle neoplasm, usually occurring in post-menopausal women. It is characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid, granular cell, inflammatory and myxoid leiomyosarcomas.

Hepatic leiomyosarcoma
Conventional leiomyosarcomas are almost always metastatic when involving the liver, and search for a primary tumour in locations such as the retroperitoneum is always necessary. EBV-associated leiomyosarcomas/smooth-muscle tumours can form primary hepatic masses. These tumours occur in patients with immunosuppression, either acquired (HIV/AIDS-associated) or iatrogenic (usually transplant-associated). In liver-transplant patients, occurrence from donor cells has been reported in allografts, and occurrence from recipient cells outside the graft.

Histologically, these tumours are typically less differentiated than typical leiomyosarcomas, composed of oval to spindled mesenchymal cells with immunoreactivity for α smooth muscle actin, but usually not for desmin. Demonstration of nuclear EBER is diagnostic.

Tumour behaviour is unpredictable, and multifocal lesions do not necessarily indicate metastasis, but are a feature of this tumour
19
Click to access Pubmed
Timmons CF, Dawson DB, Richards CS, Andrews WS, Katz JA (1995)
Epstein-Barr virus-associated leiomyosarcomas in liver transplantation recipients. Origin from either donor or recipient tissue.
Cancer 76: 1481-9



. In some cases, antiviral treatment and restoration of immune response have resulted in tumour regression and long-term survival 20
Click to access Pubmed
Bonatti H, Hoefer D, Rogatsch H, Margreiter R, Larcher C, Antretter H (2005)
Successful management of recurrent Epstein-Barr virus-associated multilocular leiomyosarcoma after cardiac transplantation.
Transplant Proc 37: 1839-44



21
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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Breast
ICD-O-3 topography code: C50
ICD10: C50
           

See Leiomyoma as these two tumours are discussed together.





Gallbladder and extrahepatic biliary tract
ICD-O-3 topography code: C23-C24.0
ICD10: C23-C24
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
28
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.

Leiomyosarcoma of the gallbladder and extrahepatic biliary tract
Epstein-Barr virus (EBV)-associated leiomyosarcoma/smooth muscle tumour rarely involves the gallbladder wall
29
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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Nasal cavity and Sinuses
ICD-O-3 topography code: C30-C31
ICD10: C30-C31
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
30
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.



Colon and rectum
ICD-O-3 topography code: C18-C20
ICD10: C18-C20
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
31
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.



Small intestine
ICD-O-3 topography code: C17
ICD10: C17
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
32
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.

Intestinal leiomyosarcomas
They are rare as leiomyomas. Prognosis is better when grade and mitotic rate are low
33
Click to access Pubmed
Miettinen M, Sobin LH, Lasota J (2009)
True smooth muscle tumors of the small intestine: a clinicopathologic, immunhistochemical, and molecular genetic study of 25 cases.
Am J Surg Pathol 33: 430-6



34
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



.



Vagina
ICD-O-3 topography code: C52
ICD10: C52
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
35
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.



Prostate gland
ICD-O-3 topography code: C61
ICD10: C61
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
36
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.



Hypopharynx, Larynx and Trachea
ICD-O-3 topography code: C13, C32, C33
ICD10: C13, C32, C33
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
37
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.



Bone
ICD-O-3 topography code: C40-C41
ICD10: C40-C41
           

Leiomyosarcoma is an uncommon, aggressive malignant smooth muscle neoplasm, histologically characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid leiomyosarcoma, myxoid leiomyosarcoma, granular cell and inflammatory leiomyosarcoma
38
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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