词条 | Endometrial stromal sarcoma |
释义 |
| name = Endometrial stromal sarcoma | synonyms = | image = EndometrialStromalSarcomaLowGrade.JPG | caption = Micrograph of a low-grade endometrial stromal sarcoma. H&E stain. | pronounce = | field = Oncology, gynecology | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }}Endometrial stromal sarcoma is a malignant subtype of endometrial stromal tumor arising from the stroma (connective tissue) of the endometrium rather than the glands. There are three grades for endometrial stromal tumors, as follows.[1] It was previously known as endolymphatic stromal myosis because of diffuse infiltration of myometrial tissue or the invasion of lymphatic channels.[2] Low-grade endometrial stromal sarcomaLow-grade endometrial stromal sarcoma consists of cells resembling normal proliferative phase endometrium, but with infiltration or vascular invasion. These behave less[3] aggressively, sometimes metastasizing, with cancer stage the best predictor of survival. The cells express estrogen/progesterone-receptors. Undifferentiated uterine sarcomaUndifferentiated uterine sarcoma, or undifferentiated (high-grade) endometrial stromal sarcoma, does not resemble normal endometrial stroma and behaves much more aggressively, frequently metastasizing. The differential includes leukemia, lymphoma, high-grade carcinoma, carcinosarcoma, and differentiated pure sarcomas. PathologyMacroscopy
Microscopy
Immunochemistry
Genetic featuresA 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.[4] References1. ^Sternberg's Diagnostic Surgical Pathology, 5th edition, p. 2242-2245. 2. ^Kumar V, Abbas A, Fausto N, Aster J (2010). Robbins and Cotran Pathologic Basis of Disease. 8th edition. Philadelphia: Elsevier Saunders 3. ^https://www.cancer.org/cancer/uterine-sarcoma/about/what-is-uterine-sarcoma.html 4. ^{{cite journal |vauthors=Li H, etal |title=Gene fusion and RNA trans-splicing in normal and neoplastic cells |journal=Cell Cycle |year=2009| doi = 10.4161/cc.8.2.7358 |volume=8 |issue=2 |pages=218–222}} External links{{Medical resources| DiseasesDB = | ICD10 = | ICD9 = | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = | eMedicineTopic = | MeshID = }}
1 : Gynaecological cancer |
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