Endometrioid Stromal Sarcoma of the Ovary
Synonyms
Extrauterine endometrial stromal sarcoma of ovary; Low-grade endometrioid stromal sarcoma of ovary.
Definition
Endometrioid stromal sarcoma is a mesenchymal tumor of the ovary, demonstrating evidence of endometrial-type stromal differentiation in the absence of uterine origin.
Clinical Features
Incidence
These tumors are rare.
Age
Ovarian endometrioid stromal sarcomas can occur at a wide age range, being reported in women as young as 20 years of age and as old as 87. The majority are diagnosed in perimenopausal and early postmenopausal period at mean age of diagnosis of 50 years.
Symptoms
Patients with ovarian endometrioid stromal sarcoma usually present with nonspecific symptoms similar to those of other ovarian tumors. These include abdominal swelling, abdominal pain, and bowel obstruction. They can also be diagnosed incidentally by imaging for unrelated conditions or complaints.
Treatment
The mainstay of treatment for these tumors...
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References and Further Reading
Amador-Ortiz, C., Roma, A. A., Huettner, P. C., Becker, N., & Pfeifer, J. D. (2011). JAZF1 and JJAZ1 gene fusion in primary extrauterine endometrial stromal sarcoma. Human Pathology, 42(7), 939–946. https://doi.org/10.1016/j.humpath.2010.11.001.
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Chiang, S., Ali, R., Melnyk, N., McAlpine, J. N., Huntsman, D. G., Gilks, C. B., … Oliva, E. (2011). Frequency of known gene rearrangements in endometrial stromal tumors. The American Journal of Surgical Pathology, 35(9), 1364–1372. https://doi.org/10.1097/PAS.0b013e3182262743.
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Kikuchi, N., Sugita, S., Nakanishi, K., Sugawara, T., Segawa, K., Ito, Y., … Hasegawa, T. (2017). Ovarian high-grade endometrioid stromal sarcoma with YWHAE and NUTM2B rearrangements. Pathology International, 67(6), 327–329. https://doi.org/10.1111/pin.12542.
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Li, Z., Maeda, D., Kudo-Asabe, Y., Tamura, D., Nanjo, H., Hayashi, A., … Goto, A. (2018). MED12 is frequently mutated in ovarian and other adnexal leiomyomas. Human Pathology, 81, 89–95. https://doi.org/10.1016/j.humpath.2018.06.013.
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Xie, W., Bi, X., Cao, D., Yang, J., Shen, K., & You, Y. (2017). Primary endometrioid stromal sarcomas of the ovary: A clinicopathological study of 14 cases with a review of the literature. Oncotarget, 8(38), 63345–63352. https://doi.org/10.18632/oncotarget.18805.
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Allo, G. (2023). Mesenchymal Tumors, Pathology of the Ovary. In: Stolnicu, S., Ali-Fehmi, R. (eds) Gynecologic Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-97378-0_5660
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DOI: https://doi.org/10.1007/978-3-030-97378-0_5660
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