Endometrial stromal sarcoma (ESS) is a rare uterine neoplasm infrequently arising in extra-genital sites. Herein, we report an extremely rare case of primary extra-genital ESS of transverse mesocolon occurring in a 51-year-old female presenting with gradually increasing abdominal mass. The clinical diagnosis considered was a gastrointestinal stromal tumor. Intra-operatively, the mass was confined exclusively to the transverse mesocolon. Microscopy revealed a cellular tumor composed of oval to elongate neoplastic cells with hyperchromatic nuclei, inconspicuous nucleoli and were immunoreactive for CD10, progesterone receptor (PR), estrogen receptor (ER), and PAX8; negative for KIT, CD34, SMA, S100, synaptophysin, chromogranin, WT1, and calretinin. A distinct arborizing network of arterioles along with foci of endometriosis was also seen. We present this case for its extreme rarity and the challenges entailed in its diagnosis.
ChanJKKawarNMShinJY, et al.Endometrial stromal sarcoma: a population-based analysis. Br J Cancer. 2008;99(8):1210–1215. doi:10.1038/sj.bjc.6604527
2.
CarcangiuMKurmanRJCarcangiuMLHerringtonCS. WHO Classification of Tumours of Female Reproductive Organs. International Agency for Research on Cancer; 2014.
3.
BackJAChoiMGJuUCKangWDKimSM. A case of advanced-stage endometrial stromal sarcoma of the ovary arising from endometriosis. Obstet Gynecol Sci. 2016;59(4):323–327. doi:10.5468/ogs.2016.59.4.323
4.
MourraNTiretEParcYde Saint-MaurPParcRFlejouJF. Endometrial stromal sarcoma of the rectosigmoid colon arising in extragonadal endometriosis and revealed by portal vein thrombosis. Arch Pathol Lab Med. 2001;125(8):1088–1090. doi:10.1043/0003-9985(2001)125<1088:ESSOTR>2.0.CO;2
5.
BosincuLMassarelliGCossu RoccaPIsaacMANogalesFF. Rectal endometrial stromal sarcoma arising in endometriosis: report of a case. Dis Colon Rectum. 2001;44(6):890–892. doi:10.1007/bf02234715
6.
BuchholzVKiroffGTrochslerMKanhereH. An unexpected diagnosis of primary omental endometrial stromal sarcoma in a patient with acute right abdominal pain: a case report and review of literature. Int J Surg Case Rep. 2017;36(7):8–14. doi:10.1016/j.ijscr.2017.04.017
7.
AlcazarJGuerrieroSAjossaS, et al.Extragenital endometrial stromal sarcoma arising in endometriosis. Gynecol Obstet Invest. 2012;73(4):265–271. doi:10.1159/000336522
8.
KhanACraigMJarmulowiczMDavidsonB. Liver tumours due to endometriosis and endometrial stromal sarcoma. HPB (Oxford).2002;4(1):43–45. doi:10.1080/136518202753598735
9.
MorrisonCRamirezNCChanJKCWakelyP. Endometrial stromal sarcoma of the retroperitoneum. Ann Diagn Pathol. 2002;6(5):312–318. doi:10.1053/adpa.2002.35746
10.
WuHLuoYYinNGuiWCaoY. Vaginal endometrial stromal sarcoma: a literature review. Eur J Obstet Gynecol Reprod Biol X. 2019;4(100094):1–5. doi:10.1016/j.eurox.2019.100094
MasandRPEuscherEDDeaversMTMalpicaA. Endometrioid stromal sarcoma: a clinicopathologic study of 63 cases. Am J Surg Pathol. 2013;37(11):1635–1647. doi:10.1097/PAS.0000000000000083
13.
NorrisHJTaylorHB. Mesenchymal tumors of the uterus. I. A clinical and pathological study of 53 endometrial stromal tumors. Cancer.1966;19(6):755–766. doi:10.1002/1097-0142(196606)19:6 < 755::aid-cncr2820190604 > 3.0.co;2-u
14.
GhosalTRoyAKurianS. Primary extrauterine endometrial stromal sarcoma: located in pelvic and abdominal tissue and arising in endometriosis. Indian J Pathol Microbiol. 2014;57(3):447. doi:10.4103/0377-4929.138757
15.
McCluggageWGSumathiVPMaxwellP. CD10 Is a sensitive and diagnostically useful immunohistochemical marker of normal endometrial stroma and of endometrial stromal neoplasms. Histopathology. 2001;39(3):273–278. doi:10.1046/j.1365-2559.2001.01215.x
16.
ReichORegauerSUrdlWLahousenMWinterR. Expression of oestrogen and progesterone receptors in low-grade endometrial stromal sarcomas. Br J Cancer. 2000;82(5):1030–1034. doi:10.1054/bjoc.1999.1038
17.
BhargavaRShiaJHummerAJThalerHTTornosCSoslowRA. Distinction of endometrial stromal sarcomas from “hemangiopericytomatous” tumors using a panel of immunohistochemical stains. Mod Pathol. 2005;18(1):40–47. doi:10.1038/modpathol.3800248
18.
ChuPGArberDAWeissLMChangKL. Utility of CD10 in distinguishing between endometrial stromal sarcoma and uterine smooth muscle tumors: an immunohistochemical comparison of 34 cases. Mod Pathol. 2001;14(5):465–471. doi:10.1038/modpathol.3880335
19.
BirdCCWillisRA. The production of smooth muscle by the endometrial stroma of the adult human uterus. J Pathol Bacteriol. 1965;90(1):75–81. doi:10.1002/path.1700900108
20.
ZhuQSunY-QDiX-QHuangBHuangJ. Metastatic low-grade endometrial stromal sarcoma with sex cord and smooth muscle differentiation: a case report. World J Clin Cases. 2019;7(2):221–227. doi:10.12998/wjcc.v7.i2.221
21.
WongNACSMeleghZ. Gastrointestinal stromal tumors can express CD10 and epithelial membrane antigen but not oestrogen receptor or HMB45. Histopathology. 2011;59(4):781–785. doi:10.1111/j.1365-2559.2011.03979.x
22.
SallaCKonstantinouPChatzipantelisP. CK19 And CD10 expression in pancreatic neuroendocrine tumors diagnosed by endoscopic ultrasound-guided fine-needle aspiration cytology. Cancer. 2009;117(6):516–521. doi:10.1002/cncy.20048
23.
NevesCBorges SoaresACostaAF, et al.CD10 (neutral endopeptidase) expression in myoepithelial cells of salivary neoplasms. Appl Immunohistochem Mol Morphol. 2009;18(2):172–178. doi:10.1097/PAI.0b013e3181b8f7c5
24.
YadaKKashimaKDaaTKitanoSFujiwaraSYokoyamaS. Expression of CD10 in basal cell carcinoma. Am J Dermatopathol. 2004;26(6):463–471. doi:10.1097/00000372-200412000-00004
ChangKLCrabtreeGSLim-TanSKKempsonRLHendricksonMR. Primary extrauterine endometrial stromal neoplasms: a clinicopathologic study of 20 cases and a review of the literature. Int J Gynecol Pathol. 1993;12(4):282–296.
27.
YoungRHPratJScullyRE. Endometrioid stromal sarcomas of the ovary. A clinicopathologic analysis of 23 cases. Cancer. 1984;53(5):1143–1155. doi:10.1002/1097-0142(19840301)53:5 < 1143::aid-cncr2820530521 > 3.0.co;2-f
