Primary extrauterine endometrial stromal cell sarcoma: a case and review

Alla Zemlyak, Sonya Hwang, Eva Chalas, Colette R J Pameijer
Journal of gastrointestinal cancer · 2008 · doi:10.1007/s12029-009-9064-0 · PMID:19333789
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This case report and literature review describes an extrauterine endometrial stromal sarcoma in a 70-year-old woman, recommending complete resection as the primary treatment and progestin therapy for unresectable or metastatic low-grade tumors.

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Abstract

BACKGROUND: Extrauterine endometrial stromal sarcoma (ESS) is a rare neoplasm. Little is known about its pathophysiology or best treatment approach. CASE: We are describing a case of extrauterine ESS in a 70-year-old woman on hormone replacement therapy and with a history of endometriosis. We also present a brief review of the literature on ESS and its relationship to endometriosis and hormonal therapy. CONCLUSIONS: Complete resection should remain the treatment of choice for ESS. Unresectable or metastatic low-grade ESS may respond well to progestin therapy, but outcomes of high-grade ESS tend to be poor.
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Abstract

Background Extrauterine endometrial stromal sarcoma (ESS) is a rare neoplasm. Little is known about its pathophysiology or best treatment approach. Case We are describing a case of extrauterine ESS in a 70-year-old woman on hormone replacement therapy and with a history of endometriosis. We also present a brief review of the literature on ESS and its relationship to endometriosis and hormonal therapy.

Conclusions

Complete resection should remain the treatment of choice for ESS. Unresectable or metastatic low-grade ESS may respond well to progestin therapy, but outcomes of high-grade ESS tend to be poor.

References

Acharya S, Hensley ML, Montag AC, Fleming GF. Rare uterine cancers. Lancet Oncol. 2005;6(12):961–71. doi:10.1016/S1470-2045(05)70463-0. Chang KL, Crabtree GS, Lim-Tan SK, Kempson RL, Hendrickson MR. Primary extrauterine endometrial stromal neoplasms: a clinicopathologic study of 20 cases and a review of the literature. Int J Gynecol Pathol. 1993;12(4):282–96. doi:10.1097/00004347-199310000-00002. 282. Kovac D, Gasparovic I, Jasic M, Fuckar D, Dobi-Babic R, Haller H. Endometrial stromal sarcoma arising in extrauterine endometriosis: a case report. Eur J Gynaecol Oncol. 2005;26(1):113–6. Levine PH, Abou-Nassar S, Mittal K. Extrauterine low-grade endometrial stromal sarcoma with florid endometrioid glandular differentiation. Int J Gynecol Pathol. 2001;20(4):395–8. doi:10.1097/00004347-200110000-00014. Bosincu L, Massarelli G, Gossu Rocca P, Isaac MA, Nogales FF. Rectal endometrial sarcoma arising in endometriosis: report of a case. Dis Colon Rectum. 2001;44(6):890–2. doi:10.1007/BF02234715. Stenchever MA, Herbst AL, Droegemueller W. Comprehensive gynecology. 4th ed. St. Louis: Mosby; 2001. Pink D, Lindner T, Mrozek A, Kretzchmar A. Harm or benefit of hormonal treatment in metastatic low-grade endometrial stromal sarcoma: single center experience with 10 cases and review of the literature. Gynecol Oncol. 2006;101(3):464–9. doi:10.1016/j.ygyno.2005.11.010. Ihnen M, Mahner S, Janicke F, Schwartz J. Current treatment options in uterine endometrial sarcoma: report of a case and review of literature. Int J Gynecol Cancer. 2007;17:957–63. Christie DB, Day JD, Moore AB, Chapman JR, Nakayama DK, Conforti AM. Endometrial stromal sarcoma development after hysterectomy and tamoxifen therapy. Am Surg. 2008;74(8):726–8. Conflict of interest statement The authors declare that they have no conflicts of interest. Author information Authors and Affiliations Corresponding author Rights and permissions About this article Cite this article Zemlyak, A., Hwang, S., Chalas, E. et al. Primary Extrauterine Endometrial Stromal Cell Sarcoma: A Case and Review. J Gastrointest Canc 39, 104–106 (2008). https://doi.org/10.1007/s12029-009-9064-0 Received: Accepted: Published: Issue date: DOI: https://doi.org/10.1007/s12029-009-9064-0

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endometriosis

MeSH descriptors

Endometrial Neoplasms Sarcoma, Endometrial Stromal Aged Endometrial Neoplasms Endometrial Neoplasms Endometrial Neoplasms Endometriosis Endometriosis Estrogen Replacement Therapy Estrogen Replacement Therapy Female Humans Sarcoma, Endometrial Stromal Sarcoma, Endometrial Stromal Sarcoma, Endometrial Stromal Tamoxifen Tamoxifen

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