Endometrial stromal sarcoma (low-grade)
|Endometrial stromal sarcoma|
|Symptoms||Abnormal uterine bleeding, pelvic pain, mass of the uterus, spread|
|Usual onset||Females in their 50s|
|Risk factors||Estrogen, tamoxifen, pelvic irradiation|
Low-grade endometrial stromal sarcoma (LGESS) is a type of cancer in the uterus. It typically occurs in females in their 50s, and presents with abnormal uterine bleeding, pelvic pain, and mass of the uterus. Local, nodal or distant spread to lungs may be found at first presentation.
It is a subtype of endometrial stromal tumor belonging to the mesenchymal tumors of the main body of the uterus. It is distinct to and less aggressive than high-grade endometrial stromal sarcoma.
Low-grade endometrial stromal sarcoma consists of cells resembling normal proliferative phase endometrium, but with infiltration or vascular invasion. These behave less aggressively, sometimes metastasizing, with cancer stage the best predictor of survival. The cells express estrogen/progesterone-receptors.
- Polypoid mass extending into broad ligament, ovaries and fallopian tubes.
- Lymphatic tumor plugs as yellow, ropy or ball-like masses.
- Monotonous ovoid cells to spindly cells with minimal cytoplasm.
- Prominent arterioles. Angiolymphatic invasion common.
- Up to 10-15 mitotic figures per 10 HPF in most active areas.
- Tongue-like infiltration between muscle bundles of myometrium.
- May exhibit myxoid, epithelioid and fibrous change.
- May have foam cells or hyalinization in the stroma.
- muscle markers (muscle specific actin MSA, smooth muscle actin SMA and desmin) positive in areas of smooth muscle differentiation
- CD117- (c-kit -)
A recurrent chromosomal translocation, t(7;17)(p15;q21), occurs in endometrial stromal sarcoma. This translocation leads to the fusion of two polycomb group genes, JAZF1 and JJAZ1, with production of a fusion transcript with anti-apoptotic properties. Even normal endometrial stroma cells express the fusion gene, derived not by translocation, but by the "stitching" together of m-RNAs. Thus, it appears that a pro-survival gene in the normal endometrium is somehow subverted to become pro-neoplastic.
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