8890/0 Leiomyofibroma



Definitions

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

Leiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.



Esophagus
ICD-O-3 topography code: C15

/span>eiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.

In the oesophagus, leiomyoma is the most common mesenchymal tumour and occurs over a wide range of ages from childhood onwards and is twice as frequent in males as in females 1
 
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



. Leiomyomas are typically located in the distal to middle oesophagus and often manifest as dysphagia. The tumour varies from a minimal intramural nodule to a larger mural nodule to an externally extending ediastinal mass, but most are 1?3cm in size 2
Click to access Pubmed
Hatch GF, Wertheimer-Hatch L, Hatch KF, Davis GB, Blanchard DK, Foster RS, Skandalakis JE (2000)
Tumors of the esophagus.
World J Surg 24: 401-11



3
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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



4
Click to access Pubmed
Seremetis MG, Lyons WS, deGuzman VC, Peabody JW (1976)
Leiomyomata of the esophagus. An analysis of 838 cases.
Cancer 38: 2166-77



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Stomach
ICD-O-3 topography code: C16

Leiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.

Gastric intramural leiomyoma
This tumour is rare, approximately 50 times less common than gastrointestinal stromal tumor (GIST). These tumours usually occur in older adults and clinically present similarly to GISTs. The tumours vary from minimal mural nodules to masses of >5cm in size. However, minimal leiomyomas have been commonly found in oesophagogastric resections for carcinoma
7
Click to access Pubmed
Abraham SC, Krasinskas AM, Hofstetter WL, Swisher SG, Wu TT (2007)
"Seedling" mesenchymal tumors (gastrointestinal stromal tumors and leiomyomas) are common incidental tumors of the esophagogastric junction.
Am J Surg Pathol 31: 1629-35



. Histologically, these tumours are composed of well-differentiated smooth-muscle cells with no or little atypia and mitotic activity, or isolated mitosis.
Smooth-muscle tumours with atypia and mitotic activity are designated leiomyosarcoma, and such tumours are distinctly rare in the stomach.



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

Leiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.

Leiomyoma of the colon and rectum
A distinctive type of leiomyoma that usually occurs in the colon and rectum is that of the muscularis mucosae. These tumours are roundish nodules that usually measure < 1 cm and not more than 2 cm. They are usually incidental endoscopic findings in older adults and are clinically indolent. Histologically, these tumours are composed of bundles of well-differentiated smooth muscle that merge into the muscularis mucosae. Although focal nuclear atypia may be present, mitotic activity is not detectable.

Intramural leiomyomas are rare. They usually measure 1?3 cm, and are composed of well-differentiated smooth muscle cells with limited, if any, atypia and virtually no mitotic activity, although tumours with both atypia and mitotic activity are more appropriately designated as leiomyosarcomas
8
 
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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Hypopharynx, Larynx and Trachea
ICD-O-3 topography code: C13, C32, C33

Leiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.



Breast
ICD-O-3 topography code: C50

Leiomyoma and leiomyosarcoma are, respectively, morphologically benign and malignant tumours that show distinct smooth-muscle differentiation.





Skin
ICD-O-3 topography code: C44

Leiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.

Skin: Pilar leiomyoma



Kidney
ICD-O-3 topography code: C64

Leiomyoma is a well-circumscribed, benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern
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



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Renal leiomyomas commonly arise from the renal capsule, but also from the muscularis of the renal pelvis or from cortical vascular smooth muscle. The tumours are firm, solid, and usually small. Most are incidentally discovered at autopsy
11
 
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

/span>eiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern
12
 
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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> Press: Lyon 2002


. Myolipoma of soft tissue is extremely rare. It occurs in adults, with a male to female ratio of 1:2. Tumours do not recur; complete surgical resection is curative 14
 
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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Corpus uteri
ICD-O-3 topography code: C54

Leiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern. Scattered adipocytes are a relatively common observation; a leiomyoma containing a striking number of adipocytes is termed a lipoleiomyoma.

Leiomyomas of the uterine corpus arise from the myometrium. They typically present as multiple, spherical firm nodules. Most frequently, they are intramural. Submucosal leiomyomas deform the overlying endometrium and may bulge into the endometrial cavity. They are frequently ulcerated and haemorrhagic. Subserosal leiomyomas can become pedunculated and may lose their attachment to the uterus. Degenerative changes are common. Most tumours are more cellular than the surrounding myometrium
15
 
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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Small intestine
ICD-O-3 topography code: C17

Leiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.

Intestinal leiomyoma
Intramural leiomyomas similar to those seen in the stomach are very rare (about 1 per 100 gastrointestinal stromal tumors (GISTs)). Muscularis mucosae leiomyoma, like that found more commonly in the colon and rectum, has also been reported
16
 
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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Gallbladder and extrahepatic biliary tract
ICD-O-3 topography code: C23-C24.0

Leiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.



Cervix uteri
ICD-O-3 topography code: C53

Leiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.

Leiomyoma is the most common benign mesenchymal tumour of the cervix
17
 
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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Urinary system: Renal pelvis, ureter, bladder, urethra
ICD-O-3 topography code: C65-C68

Leiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.



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

Leiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.



Prostate gland
ICD-O-3 topography code: C61

Leiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.



Vagina
ICD-O-3 topography code: C52

Leiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.



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

Leiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.



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

Leiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.



Vulva
ICD-O-3 topography code: C51

Leiomyoma is a well-circumscribed benign smooth muscle neoplasm histologically characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern.