Primary cervical leiomyosarcomas (CLMS) constitute 21% of all cervical sarcomas. Because of their rarity, to our knowledge, fewer than 40 cases have been reported. The aim of this study is to evaluate the clinical and surgical–pathological features, prognosis, treatment options, and survival of primary CLMS.
Methods:
A systematic review of the medical literature was conducted to evaluate articles about primary CLMS. The literature was searched between 1959 and May 2019. On final evaluation, there were 29 articles (one consisted of 8 cases; one consisted of 3 cases) and 42 cases with the addition of our 4 cases.
Results:
Age (⩾48 versus ⩽47 years) (hazard ratio.HR], 4.528; 95% confidence interval.CI], 1.550–13.227; p=0.006) and mitoses count (<10/10 high-power field [HPF] versus ⩾10/10 HPF) (HR, 3.865; 95% CI, 1.046–14.278; p=0.043) are independent prognostic factors for recurrence and age (HR, 5.318; 95% CI, 1.671–16.920; p=0.005) and hysterectomy (performed versus not performed) (HR, 4.377; 95% CI, 1.341–14.283; p=0.014) are independent prognostic factors for death because of disease on multivariate analysis.
Conclusions:
Information on primary CLMS is sparse and obtained from rare case reports and case series. Hysterectomy must be the first choice of treatment in these patients according to our results on multivariate analysis. The type of hysterectomy does not have an effect on oncologic outcome. Radical hysterectomy is not obligatory and more data are needed to make more accurate conclusions.
WrightJDRosenblumKHuettnerPC, et al. Cervical sarcomas: an analysis of incidence and outcome. Gynecol Oncol2005; 99: 348–351.
2.
FadareO. Uncommon sarcomas of the uterine cervix: a review of selected entities. Diagn Pathol2006; 1: 30–35.
3.
FujiwakiRYoshidaMIidaK, et al. Epithelioid leiomyosarcoma of the uterine cervix. Acta Obstet Gynecol Scand1998; 77: 246–248.
4.
BansalSLewinSNBurkeWM, et al. Sarcoma of the cervix: natural history and outcomes. Gynecol Oncol2010; 118: 134–138.
5.
PratJ. FIGO staging for uterine sarcomas. Int J Gynaecol Obstet2009; 104: 177–178.
6.
WhitcombeDDValentePTAcostaOM, et al. Leiomyosarcoma of the uterine cervix associated with pregnancy: a case report and review of the literature. Gynecol Oncol Rep2016; 17: 45–48.
7.
KhoslaDGuptaRSrinivasanR, et al. Sarcomas of uterine cervix: clinicopathological features, treatment and outcome. Int J Gynecol Cancer2012; 22: 1036–1030.
8.
LackEE. Leiomyosarcomas in childhood: a clinical and pathologic study of 10 cases. Pediatr Pathol1986; 6: 181–197.
9.
GotohTKikuchiYTakanoM, et al. Epithelioid leiomyosarcoma of the uterine cervix. Gynecol Oncol2001; 82: 400–405.
10.
GiuntoliRLMetzingerDSDiMarcoCS, et al. Retrospective review of 208 patients with leiomyosarcoma of the uterus: prognostic indicators, surgical management, and adjuvant therapy. Gynecol Oncol2003; 89: 460–469.
11.
IrwinWPresleyAAndersenW, et al. Leiomyosarcoma of the cervix. Gynecol Oncol2003; 91: 636–642.
12.
RothbartMJMarkhamEH. Leiomyosarcoma of the cervix: report of a case. Am J Obstet Gynecol1974; 120: 853–854.
13.
KasamatsuTShiromizuKTakahashiM, et al. Leiomyosarcoma of the uterine cervix. Gynecol Oncol1998; 69:169–171.
14.
AbellMRRamirezJA. Sarcomas and carcinosarcomas of the uterine cervix. Cancer1973; 31: 1176–1192.
15.
KayaSBacanakgilHBSoymanZ, et al. Myxoid leiomyosarcoma of the cervix: a case report. J Obstet Gynaecol2016; 36: 989–991.
16.
FragaMPrietoOGarcia-CaballeroT, et al. Myxoid leiomyosarcoma of the uterine cervix. Int J Gynecol Pathol1998; 17: 89–90.
17.
LuBShiHZhangX. Myxoid leiomyosarcoma of the uterus: a clinicopathological and immunohistochemical study of 10 cases. Hum Pathol2017; 59: 139–146.
18.
ToyoshimaMOkamuraCNiikuraH, et al. Epithelioid leiomyosarcoma of the uterine cervix: a case report and review of the literature. Gynecol Oncol2005; 97: 957–960.
19.
GraysonWFourieJTiltmanAJ. Xanthomatous leiomyosarcoma of the uterine cervix. Int J Gynecol Pathol1998; 17: 89–90.
20.
TaylorHBNorrisHJ. Mesenchymal tumor of the uterus. Arch Pathol1966; 82: 40–44.
21.
BellSWKempsonRL. Problematic uterine smooth muscle neoplasms: a clinicopathologic study of 213 cases. Am J Surg Pathol1994; 18: 535–558.
22.
RobboySJKnorrisMNorrisHJ. Malignant potential and pathology of leiomyomatous tumors of uterus. Clin Consult Obstet Gynecol1990; 2: 2–9.
23.
HanniganEVGomezLG. Uterine leiomyosarcoma. Am J Obstet Gynecol1979; 134: 557–564.
24.
MayerhofferKObermairAWindbichlerG, et al. Leiomyosarcoma of the uterus: a clinicopathological multicenter study of 71 cases. Gynecol Oncol1999; 74: 196–201.
25.
GadduciALandoniFSartoriE, et al. Uterine leiomyosarcoma: analysis of treatment failures and survival. Gynecol Oncol1996; 62: 25–32.
26.
LarsonBSilfverswardCNilssonB, et al. Prognostic factors in uterine leiomyosarcoma: a clinical and histopathological study of 143 cases. Acta Oncol1990; 29: 185–191.
27.
ZivanovicOJacksLMIasonosA, et al. A nomogram to predict postresection 5-year overall survival for patients with uterine leiomyosarcoma. Cancer2012; 118: 660–669.
28.
MajorFJBlessingJASilverbergSG, et al. Prognostic factors in early-stage uterine sarcoma: a Gynecologic Oncology Group study. Cancer1993; 71(4 Suppl): 1702–1709.
29.
CasanovaJHuangKGThepsuwanJ, et al. Localized leiomyosarcoma of the uterine cervix with rapid lung metastases. Gynecol Minim Invasive Ther2015; 4: 95–97.
30.
KappDSShinJYChanJK. Prognostic factors and survival in 1396 patients with uterine leiomyosarcomas: emphasis on impact of lymphadenectomy and oophorectomy. Cancer2008; 112: 820–830.
31.
RobertsMEAynardiJTChuCS. Uterine leiomyosarcoma: a review of the literature and update on management options. Gynecol Oncol2018; 151: 562–572.
32.
HensleyMLWathenJKMakiRG, et al. Adjuvant therapy for high-grade, uterus-limited leiomyosarcoma: results of a phase 2 trial: SARC 005. Cancer2013; 119: 1555–1561.
33.
HensleyMLEnserroDHatcherH, et al. Adjuvant gemcitabine plus docetaxel followed by doxorubicin versus observation for high-grade uterine leiomyosarcoma: a phase III NRG oncology/gynecologic oncology group study. J Clin Oncol2018; 36: 3324–3330.
34.
SeddonBStraussSJWhelanJ, et al. Gemcitabine and docetaxel versus doxorubicin as first-line treatment in previously untreated advanced unresectable or metastatic soft-tissue sarcomas: GeDDiS: a randomised controlled phase 3 trial. Lancet Oncol2017; 18: 1397–1410.
35.
BlausteinAImmermanB. Leiomyosarcoma of the cervix. Obstet Gynecol1963; 22: 224–227.
36.
Abdul-KarimFWBaziTMSorensenK, et al. Sarcoma of the uterine cervix: clinicopathologic findings in three cases. Gynecol Oncol1987; 26: 103–111.
37.
SturdyDE. Leiomyosarcoma of cervical stump following subtotal hysterectomy. Br J Surg1959; 46: 369–370.
38.
AminimoghaddamSArabianSHaghighiS, et al. Cervical leiomyosarcoma: a case report. Obstet Gynecol Cancer Res2016; 1: e7523.
39.
BhatiaVTaksandeRNatekarA, et al. Cervical leiomyosarcoma: a rare entity. South Afr J Gynaecol Oncol2015; 7: 64–66.
40.
CooleySStunnellHWalshC, et al. Cervical leiomyosarcoma: a case report. J Obstet Gynaecol2005; 25: 222.
41.
Dhull AKC AKaushalVMarwahN. The uncovered story of leiomyosarcoma of the cervix: a rare case report and review of literature. BMJ Case Rep Epub 2013 Mar 20.
42.
DoshiBShettySSafayaA. Leiomyosarcoma of cervix. J Obstet Gynaecol India2013; 63: 211–212.
43.
HaldarKIzuwah-NjokuNWarrenM. Cervical leiomyosarcoma diagnosed after uterine artery embolization. Int J Gynaecol Obstet2008; 101: 197–198.
44.
JayaramVKParikshithJNarayananGS, et al. Multimodality management of leiomyosarcoma of the cervix. Ecancermedicalscience2018; 12: 830.
45.
MasbahOMellasNBekkouchI, et al. Epithelioid leiomyosarcoma of the uterine cervix: a case report and review of the literature. J Afr Cancer2012; 4: 53–56.
46.
MehraRSidhuSPKPandeyAK, et al. A rare case of leiomyosarcoma of the cervix and challenges in its management: a case report and brief review of literature. Int J Res Med Sci2015; 3: 320–324.
47.
MittalSVermaYPKumarT. Primary leiomyosarcoma of cervix: now it counts!Int J Curr Res2017; 9: 49398–49401.
48.
SahuLBupathyABadheBA. Leiomyosarcoma of the uterine cervix in a young woman. J Obstet Gynaecol Res2008; 34: 717–720.
49.
GroverSAbrahamMMahajanMK. Leiomyosarcoma of the cervix. J Obstet Gynecol India2009; 59: 364–366.
50.
SharmaCShekharSRaneSU, et al. Cervical leiomyosarcoma: what is the optimum management?Eur J Obstet Gynecol Reprod Biol2012; 165: 368–369.
51.
ThambiRSubithaKPothenL, et al. Leiomyosarcoma of uterine cervix. Nat J Lab Med2016; 5: 72–73.
52.
ZhiqiangLBinSMinF, et al. Leiomyosarcoma of cervical stump following subtotal hysterectomy: a case report and review of literature. Eur J Gynaecol Oncol2016; 37: 148–151.
