8931/3 Stromal endometriosis



Definitions

Ovary
ICD-O-3 topography code: C56
ICD10: C56
           

An infiltrating mesenchymal tumor arising from the uterine corpus, cervix, vagina, and the ovary. It is characterized by the presence of oval to spindle-shape cells that resemble the cells of the endometrial stroma, without evidence of significant atypia and pleomorphism. Numerous small vessels are also present. It is usually characterized by indolent growth and late recurrences.



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

An infiltrating mesenchymal tumor arising from the uterine corpus, cervix, vagina, and the ovary. It is characterized by the presence of oval to spindle-shape cells that resemble the cells of the endometrial stroma, without evidence of significant atypia and pleomorphism. Numerous small vessels are also present. It is usually characterized by indolent growth and late recurrence.



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

Low grade endometrial stromal sarcoma is an infiltrating mesenchymal tumour arising from the uterine corpus, uterine cervix, vagina, and the ovary. It is typically densely cellular and characterized by the presence of oval to spindle-shaped cells that resemble the cells of the endometrial stroma, without evidence of significant atypia and pleomorphism. A rich network of thin-walled small vessels supports the cells.
Cervical low grade endometrial stromal sarcoma is very rare. It may arise from cervical endometriosis. The tumours are usually indolent. They have a propensity for late recurrence1
 
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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Corpus uteri
ICD-O-3 topography code: C54
ICD10: C54
           

Low-grade endometrial stromal sarcoma is an infiltrating mesenchymal tumour arising from the uterine corpus, uterine cervix, vagina, and the ovary. It is typically densely cellular and characterized by the presence of oval to spindle-shaped cells that resemble the cells of the endometrial stroma, without evidence of significant atypia and pleomorphism. A rich network of thin-walled small vessels supports the cells. The tumour typically infiltrates deeply into the myometrium; about 50% of cases extend into the serosa.
Low-grade endometrial stromal sarcomas are usually indolent tumours. They have a propensity for late recurrence (>20 years in some cases). Resection, radiation therapy, progestin therapy or a combination thereof is frequently successful; 5-year survival ranges from 67% to nearly 100%
2
Click to access Pubmed
Nordal RN, Kjorstad KE, Stenwig AE, Tropé CG (1993)
Leiomyosarcoma (LMS) and endometrial stromal sarcoma (ESS) of the uterus. A survey of patients treated in the Norwegian Radium Hospital 1976-1985.
Int J Gynecol Cancer 3: 110-115



3
Click to access Pubmed
Chang KL, Crabtree GS, Lim-Tan SK, Kempson RL, Hendrickson MR (1990)
Primary uterine endometrial stromal neoplasms. A clinicopathologic study of 117 cases.
Am J Surg Pathol 14: 415-38



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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