Cancer Treatment Communications (2014) 2, 12–15

http://dx.doi.org/12213-0896 & 2014 T(http://creativecom

☆Précis: Malignanextremely rare andpriate management

nCorresponding auE-mail addresses

jozo.visnovsky@gma1Tel: +421 902 30

Malignant mixed Mullerian tumor of thecervix – case report$

Erik Kudelaa,1, Pavol Slavikb, Jozef Visnovskya,n, Pavol Buocika,Jana Sivakovaa, Petra Sumichrastovaa, Lukas Plankb, Jan Dankoa

aDepartment of Gynecology and Obstetrics, Jessenius Faculty of Medicine, Comenius University, SlovakiabInstitute of Pathologic Anatomy, Jessenius Faculty of Medicine, Comenius University, Slovakia

Received 26 August 2013; received in revised form 21 January 2014; accepted 23 February 2014

KEYWORDSMalignant mixed Mul-lerian tumor;Cervix

0.1016/j.ctrc.2014he Authors. Publismons.org/licenses

t mixed Mulleriabecause of theiris vital.thor.: kudela.erik@gmail.com (J. Visnovsk0 017; fax: +421

AbstractObjective: To report a rare case of cervical malignancy necessitating complex treatment.Case: A sixty-five year old woman was admitted to the hospital with a diagnosed tumor arisingfrom the upper lip of the cervix. Patient underwent the amputation of the cervix with thecurettage of the cervical channel. Because of the diagnosis of malignant mixed Mullerian tumor(MMMT) a radical hysterectomy with pelvic lymphadenectomy was performed afterwards.The composition of the tumor concerning basaloid carcinoma combined with homologoussarcoma was published only in five cases in the known literature.Conclusions: Because of rarity of MMMT there are no consensus guidelines regardingtreatment. We present the case with successful management and 4-year disease free survival.& 2014 The Authors. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/3.0/).

1. Introduction

About 80–90 % of cervical malignancies represent squamous cellcarcinomas followed by adenocarcinomas (10–20%) where inci-dence is on the rise nowadays. Other types of tumors arerelatively rare. Malignant mixed Mullerian tumors of the cervixare extremely rare (0.005% of all malignancies of the cervix)

.02.001hed by Elsevier Ltd. This is an op/by-nc-nd/3.0/).

n tumors of the cervix areaggressive behavior appro-

il.com (E. Kudela),y).43 4203 242.

first described in 1951 by Ferriera and colleagues [1,2]. MMMT´sinclude both malignant epithelial and mesenchymal compo-nents. The most common components are squamocellularcarcinoma combined with homologous sarcoma. So far onlyabout 50 cases can be found in the literature [3] and from theseonly a smaller part was thoroughly described. Because of such anumber of cases there are no consensus guidelines regardingtreatment, prognosis and outcome.

2. Case report

A sixty-five year old woman (hypertensive, diabetic, P2) wasadmitted to the hospital with a diagnosed tumor arisingfrom the upper lip of the cervix. She complained of threemonths lasting intermittent vaginal bleeding one year after

en access article under the CC BY-NC-ND license

Fig. 1 PAP staining of the cytology sample from the site ofcervical tumor. Visible cells with morphologically large atypicnuclei suspicious from malignity.

Fig. 2 (A–C) Haematoxilin staining of the tumor with emphasison the border of two different components (basaloid carcinomaand homologous sarcoma).

13Malignant mixed Mullerian tumor of the cervix – case report

the menopause. No other symptoms were present. Duringthe vaginal examination at the Department an ExophyticFragile tumor 3� 1.5 cm was confirmed. The cytologicalsample from the site of the tumor was suspicious frommalignity (Fig. 1). Patient underwent the amputation of thecervix with the curettage of the cervical channel. Becauseof the diagnosis of malignant mixed Mullerian tumor aradical hysterectomy with pelvic lymphadenectomy wasperformed afterwards. Subsequently whole pelvis irradia-tion ( 60 Gy in 30 fractions) and cis-platin based adjuvantchemotherapy were carried out without any complications.The patient is now five years after the operation with nosigns of recurrence.

2.1. Pathological findings

Grossly the tumor occupied the anterior lip of the cervix withregressive changes. Microscopically the tumor was composedof two different parts. The epithelial part was composed ofbasaloid squamocellular carcinoma (HMW CK+, EMA+)whereas the mesenchymal part was represented by homo-logous high-grade stromal sarcoma (CD 10+, vimentin+,desmin+/�, MSA�, SMA�, S100�) (see Figs. 2 and 3) .The endocervix was intact, without the infiltration, andsamples from the endocervical curettage were negative asfar as the malignancy was concerned. Uterus, fallopian tubesand ovaries were intact. 30 Lymph nodes removed from theiliac and obturatory regions were examined with no sign ofmetastatic process. In conclusion the tumor was staged aspT1bpN0pMx G3.

3. Discussion

MMMT of the cervix usually occurs in postmenopausalwomen, although the age varies from 12 to 93 years [4,5].Most of the patients complained of vaginal bleeding and hada detectable mass in the uterine cervix. Grossly the cervicaltumors ranged between 1.1 and 10 cm [4]. Microscopicallythe documented epithelial components of tumors wererepresented by squamocellular carcinoma (keratinizing,

non-keratinizing, basaloid, papillary) and adenocarcinoma.Documented mesenchymal components were eitherhomologous or heterologous (rhabdomyosarcoma, chondro-sarcoma). One case report described also neuroendocrinecomponent of MMMT. There are various etiologic factorsconcerning radiation exposure to the pelvic area, previous

HMW CK CD 10

Vimentin

Fig. 3 Immunohistochemical findings. Positive CK staining in basaloid carcinoma (A), and positive vimentin and CD10 staining insarcomatous component (B, C).

Table 1 Literature review of basaloid MMMT's of the cervix (RT—radiotherapy, CHT—chemotherapy, TAH (total abdominalhysterectomy), BSO (bilateral salpingo-oophorectomy), ACC—adenocarcinoma, SCC—squamocellular carcinoma, NA—not available).

Reference Age Presentation Treatment Carc. comp. Sarc. comp Outcome

[4] 71 Cervical mass Local excision,RT+CHT

Basaloid carcinoma withACC like pattern

Low-grade myxoid fibrosarcoma NA

[4] 70 Abnormal PAP TAH, BSO Basaloid carcinoma withACC like pattern

Low-grade myxoid fibrosarcoma NA

[4] 87 Abnormal PAP Local excision, RT Basaloid carcinoma Homologous sarcoma NA[6] 68 NA NA Basaloid carcinoma,

keratinizing SCCSpindle cell sarcoma NA

[6] 55 NA NA Basaloid carcinoma Spindle/giant cell sarcoma NA[6] 36 NA NA Basaloid carcinoma,

kratinising SCCSpindle and myxoid cell sarcoma NA

[6] 93 NA NA Basaloid carcinoma,kratinising SCC

Spindle/giant cell sarcoma NA

E. Kudela et al.14

chemotherapy and HPV infection. The most common HPVtypes detected in MMMTs are HPV 16 and 18. Grayson et al.detected HPV16-DNA in the nuclei of both epithelial andsarcomatous components in three cases. These observationslend support to metaplastic theory of histogenesis [6].

Our case represents only seventh documented case in theliterature of MMMT (according to the review of Khannaet al.) composed of basaloid carcinoma and homologoussarcoma (see Table 1). MMMTs of the cervix compared totheir counterparts in the corpus are more commonly

15Malignant mixed Mullerian tumor of the cervix – case report

confined to the uterus at presentation and have a betterprognosis because of their early detection [4]. Behavior ofthese tumors is dominated by the carcinomatous compo-nent. Cervical MMMTs are according to a review article byKhanna et al. managed with radical hysterectomy, che-motherapy (cisplatin-based) and radiotherapy. According toTable 1 appropriate data concerning treatment modalitiesand outcome are missing in the literature. Some authorsadvise the removal of pelvic and aortic lymph nodes,omentectomy and peritoneal cytology in case of abdominaldissemination. The role of adjuvant radiotherapy andchemotherapy is also uncertain, but some authors haveshown the advantage of radiotherapy for disease-specificsurvival in early stage tumors. Aggressive primary therapycan result in curing early-stage tumors whereas extracervi-cal disease has very poor prognosis [7]. Taxanes andcisplatin-based chemotherapy, ifosfamid, along with pelvicirradiation, may lead to increased survival in people withmetastatic carcinosaromas [8].

Median follow-up for patients with MMMTwas 16 months,median time to death 10.5 months [9]. The follow-up of ourpatient continues for 60 months (control every 6 months)with negative ultrasound of small pelvis (every 6 moths),negative oncocytology result and negative oncomarkers(Ca125, CEA).

In summary, we present a case of MMMTof the cervix withbasaloid and high-grade homologous sarcoma components.Despite its aggressive nature, the patient was successfullymanaged with no signs of recurrence 5 years after thesurgery.

Acknowledgments

Funding was received from the Comenius University GrantsNo. 303/2011, 242/2012, 77/2013 and Scientific Grant

Agency of the Ministry of Education of the Slovak RepublicVEGA 1/0271/12.

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