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CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2012; 33(01): 1-6
DOI: 10.4103/0971-5851.96960
REVIEW ARTICLE

Endometrial stromal sarcoma: A review of the literature

Geetha Puliyath
Department of Obstetrics and Gynaecology, SUT Academy of Medical Sciences, Trivandrum, Kerala, India
,
M Krishnan Nair
Department of Obstetrics and Gynaecology, SUT Institute of Oncology, Pattom, Trivandrum, Kerala, India
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Abstract

Endometrial stromal sarcomas are rare malignant tumors of the uterus, and most of the information available in literature is based on small series or case reports. A proper preoperative diagnosis is difficult and in most cases the diagnosis is confirmed after hysterectomy for a presumed benign disease. Endometrial sampling, ultrasound, and magnetic resonance imaging can provide diagnostic clues. Total hysterectomy with bilateral salpingo-oopherectomy is the main line of management and for early disease complete cure is a reality. Ovarian conservation may be possible in young women with early stage disease and the role of lymphadenectomy is controversial. Adjuvant hormone therapy in the form of progesterone, gonadotropin releasing hormone analogues, and aromatase inhibitors are found to be effective in preventing recurrences. Hormone therapy, radiotherapy and surgical excision of the metastasis are recommended for recurrences.

Keywords

Endometrial stromal sarcoma - malignant uterine tumor - uterine sarcoma


Publication History

Article published online:
13 April 2022

© 2012. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/.)

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  • References

  • 1 Tavassoli FA, Devilee P. WHO Classification of Pathology and genetics of tumours of the breast and female genital organs. In: Tavassoli FA, Devilee P, editors, Lyon, France: IARC Press; 2003. p. 233-6.
  • 2 Micci F, Panagopoulos I, Bjerkehagen B, Heim S. Consistent rearrangement of chromosomal band 6p21 with generation of fusion genes JAZF1/PHF1 and EPC1/PHF1 in endometrial stromal sarcoma. Cancer Res2006;66:107-12.
  • 3 Halbwed I, Ullmann R, Kremser ML, Man YG, Moud NI, Lax S, et al. Chromosomal alterations in low-grade endometrial stromal sarcoma and undifferentiated endometrial sarcoma as detected by comparative genomic hybridization. Gynecol Oncol 2005;2:582-7.
  • 4 Cohen I. Endometrial pathologies associated with postmenopausal tamoxifen treatment. Gynecol Oncol 2004;2:256-66.
  • 5 Nicolas ML, Cathro HP, Kerr SE, Stelow EB. Cytomorphologicfeatures of low-grade endometrial stromal sarcoma. AJR Am J ClinPathol 2007;128:265-71.
  • 6 Ganjoei TA, Behtash N, Shariat M, Low AM. Grade endometrial stromal sarcoma of uterine corpus: A clinicopathological and survey study in 14 cases. World J Surg Oncol 2006;4:50.
  • 7 Jin Y, Pan L, Wang X, Dai Z, Huang H, Guo L, et al. Clinical characteristics of endometrial stromal sarcoma from an academic medical hospital in China. Int J Gynecol Cancer 2010;20:1535-9.
  • 8 Toprak U, Paaolu E, Karademir MA, Gülbay M. Sonographic, CT, and MRI findings of endometrial stromal sarcoma located in the myometrium and associated with peritoneal inclusion cyst. AJR Am J Roentgenol 2004;182:1531-3.
  • 9 Oliva E, Clement B, Young RH. Endometrial stromal tumours: An update on a group of tumours with a protean phenotype. Adv Anat Pathol 2000;7:257-81.
  • 10 Chu PG, Arber DA, Weiss LM, Chang KL. Utility of CD10 in distinguishing between endometrial stromal sarcoma and uterine smooth muscle tumors: An immunohistochemical comparison of 34 cases. Mod Pathol 2001;14:465-71.
  • 11 Zhu XQ, Shi YF, Cheng X, Zhao CL, Wu YZ. DetalImmunohistichemical markers in differential diagnosis of endometrial stromal sarcoma and cellular leomyoma. Gynaecol Oncol 2004;92:71-9.
  • 12 Agoff SN, Grieco VS, Garcia R, Gown AM Immunohistochemical distinction of endometrial stromal sarcoma and cellular leiomyoma. Appl Immunohistochem Mol Morphol 2001;9:164-9.
  • 13 Sumathi VP, McCluggage WG. CD 10 is useful in demonstrating endometrial stroma at ectopic sites and in confirming a diagnosis of endometriosis. J Clin Pathol 2002;55:391-2.
  • 14 D′Angelo E, Prat J. Uterine sarcomas: A review. Gynecol Oncol 2010;116:131-9.
  • 15 Lai CH, WuYC, Wu TI, Hsueh S, Chou HH, Chang TC. Prognostic factors and long term outcome for uterine endometrial stromal sarcoma. J Clin Oncol 2005;23:9057.
  • 16 Chan JK, Kawar NM, Shin JY, Osann K, Chen LM, Powell CB, et al. Endometrial stromal sarcoma: A population based analysis. Br J Cancer 2008;99:1210-5.
  • 17 Leath CA 3 rd , Huh WK, Hyde J Jr, Cohn DE, Resnick KE, Taylor NP, et al. A multi-institutional review of outcomes of endometrial stromal sarcoma. Gynec Oncol 2007;105:630-4.
  • 18 Grimer R, Judson I, Peake D, Seddon B. Guidelines for the management of soft tissue sarcomas. Sarcoma 2010;2010:506182.
  • 19 Gadducci A, Cosio S, Romanini A, Genazzani AR. The management of patients with uterine sarcoma: A debated clinical challenge. Crit Rev Oncol Hematol 2008;65:129-42.
  • 20 Li AJ, Giuntoli RL 2 nd , Drake R, Byun SY, Rojas F, Barbuto D, et al. Ovarian preservation in stage I low-grade endometrial stromal sarcomas. Obstet Gynecol 2005;106:1304-8.
  • 21 Amant F, De Knijf A, Van Calster B, Leunen K, Neven P, Berteloot P, et al. Clinical study investigating the role of lymphadenectomy, surgical castration, and adjuvant hormonal treatment in endometrial stromal sarcoma. Br J Cancer 2007;97:1194-9.
  • 22 Gadducci A, Sartori E, Landoni F, Zola P, Maggino T, Urgesi A, et al. Endometrial stromal sarcoma: Analysis of treatment failures and survival. Gynecol Oncol1996;63:247-53.
  • 23 Chu MC, Mor G, Lim C, Zheng W, Parkash V, Schwartz PE. Low-grade endometrial stromal sarcoma: Hormonal aspects. Gynecol Oncol 2003;90:170-6.
  • 24 Chan JK, Kawar NM, Shin JY, Osann K, Chen LM, Powell CB, et al. Endometrial stromal sarcoma: A population-based analysis. BrJ Cancer 2008;99:1210-5.
  • 25 Li AJ, Giuntoli RL 2 nd , Drake R, Byun SY, Rojas F, Barbuto D, et al. Ovarian preservation in stage I low-grade endometrial stromal sarcomas. Am J Obstet Gynecol 2005;106:1304-8.
  • 26 Goff BA, Rice LW, Fleischhacker D, Muntz HG, Falkenberry SS, Nikrui N, et al. Uterine leiomyosarcoma and endometrial stromal sarcoma: Lymph node metastases and sites of recurrence. Gynecol Oncol 1993;50:105-9.
  • 27 Reich O, Winter R, Regauer S. Should lymphadenectomy be performed in patients with endometrial stromal sarcoma? Gynecol Oncol 2005;97:982; author reply 982-3.
  • 28 Riopel J, Plante M, Renaud MC, Roy M, Têtu B. Lymph node metastases in low-grade endometrial stromal sarcoma. Gynecol Oncol 2005;96:402-6.
  • 29 Shah JP. Lymphadenectomy and ovarian preservation in low-grade endometrial stromal sarcoma. Obstet Gynecol 2008;112:1102-8.
  • 30 NCCN clinical practice guidelines in oncology. Soft tissue sarcoma. Version 2. 2011. National Comprehensive Cancer Network, Inc.;2011.
  • 31 Reich O, Regauer S. Aromatase expression in low-grade endometrial stromal sarcomas: An immunohistochemical study. Mod Pathol 2004;17:104-8.
  • 32 Spano JP, Soria JC, Kambouchner M, Piperno-Neuman S, Morin F, Morere JF, et al. Long-term survival of patients given hormonal therapy for metastatic endometrial stromal sarcoma. Med Oncol 2003;20:87-93.
  • 33 Alkasi O, Meinhold-Heerlein I, Zaki R, Fasching P, Maass N, Jonat W, et al. Long-term disease-free survival after hormonal therapy of a patient with recurrent low grade endometrial stromal sarcoma: A case report. Arch GynecolObstet 2009;279:57-60.
  • 34 Chu MC, Chu, Mor G, Lim C, Zheng W, Prakash V, Schwart PE. Low-grade endometrial stromal sarcoma: Hormonal aspects. Gynaec Oncol 2003; 90:170-6.
  • 35 Navarro D, Cabrera JJ, León L, Chirino R, Fernández L, López A, et al. Endometrial stromal sarcoma expressionof estrogen receptors, progesterone receptors and estrogen-induced srp27 (24K) suggests hormone responsiveness. J Steroid Biochem Mol Biol 1992;41:589-96.
  • 36 Reich O, Regauer S. Hormonal therapy of endometrial stromal sarcoma. Curr Opin Oncol 2007;19:347-52.
  • 37 Garg G, Awoniyi O, Awonuga, Eugene P, Toy. Primary extrauterine endometrial stromal sarcoma: Response to hormone therapy. Rare Tumors 2009;1:2.
  • 38 Linder T, Pink D, Kretzschmar A. Hormone treatment of endometrial stromal sarcomas: A possible indication for aromatase inhibitors. J Clin Oncol 2005;23(Suppl):9057.
  • 39 Pink D, Lindner T, Mrozek 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;1013:464-9.
  • 40 Casali PG, Blay JY. Soft tissue sarcomas: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol 2010;21:198-203.
  • 41 Reich O, Regauer S. Estrogen replacement therapy and tamoxifen are contraindicated in patients with endometrial stromal sarcoma. Gynecol Oncol 2006;102:413-4.
  • 42 Maluf FC, Sabbatini P, Schwartz L, Xia J, Aghajanian C. Endometrial stromal sarcoma: Objective response to letrozole. Gynaec Oncol 2001;82:384-8.
  • 43 Weitmann HD, Knocke TM, Kucera H, Pötter R. Radiation therapy in the treatment of stromal sarcoma. Int J Radiat Oncol Biol Phys 2001;41:739-48.
  • 44 Reich O, Nogales FF, Regauer S. Gonadotropin-releasing hormone receptor expression in endometrial stromal sarcomas: An immunohistochemical study. Mod Pathol 2005;18:573-6.
  • 45 Kim GY, Sung P, Han J, Park JO, Lee KS. Pulmonary metastases of uterine endometrial storm al sarcoma: Diffusemicronodular and ground glass opacities: A case report. J Korean Med Sci 2004;19:901-3.
  • 46 Ihnen M, Mahner S, Jänicke F, Schwarz J. Current treatment options in uterine endometrial stromal sarcoma: Report of a case and review of the literature. Int J Gynecol Cancer 2007;17:957-63.
  • 47 Dupont NC, DiSaia PJ. Recurrent endometrial stromal sarcoma: Treatment with a progestin and gonadotropin releasing hormone agonist. Sarcoma 2010;2010:353679.
  • 48 Lin YC, Kudelka AP, Tresukosol D, Malpica A, Carrasco CH, Lawrence DD, et al. Prolonged stabilization of progressive endometrial stromal sarcoma with prolonged oral etoposide therapy. GynecolOncol 1995;58:262-5.
  • 49 Malouf GG, Duclos J, Rey A, Duvillard P, Lazar V, Haie-Meder C, et al. Impact of adjuvant treatment modalities on the management of patients with stages I-II endometrial stromal sarcoma Ann Oncol 2010;21:2102-6.
  • 50 Bodner K, Adler BB, Obermair A, Windbichler G, Petru E, Mayerhofer S, et al. Prognostic parameters in endometrial stromal sarcoma: A clinicopathologic study in 31 patients. Gynecol Oncol 2001;81:160S-5S.