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Case Report

Submitted: 31 Jul 2012Accepted: 18 Sep 2012

Malignant Mixed Epithelial Tumour of Ovary-Serous Papillary Cystadenocarcinoma and Transitional Cell Carcinoma with Tubo-Ovarian Torsion: A Rare Tumour with Rare Presentation

Shashikant AdlekhA1, Tandra ChAdhA2

1 Department of Pathology, Sree Narayana Institute of Medical Sciences, Ernakulam-483594, Kerala, India

2 Department of Microbiology, Sree Narayana Institute of Medical Sciences, Ernakulam-483594, Kerala, India

Abstract Ovarian torsion can be associated with various pathophysiological factors. Most commonly, benign epithelial ovarian tumours present with torsion. We present an unusual case of mixed malignant epithelial ovarian tumour with a predominant component of high-grade serous cystadenocarcinoma (85%) and transitional cell carcinoma (TCC) (15%) in a patient who presented with acute lower abdomen. The tumour was associated with tubo-ovarian torsion.

Keywords: malignant epithelial neoplasms, torsion abnormality, acute abdomen, serous carcinoma, transitional cell carcinoma

Introduction

Ovarian torsion is one of the significantcauses of acute lower abdomen in women. Theclinical presentation is vague and non-specificand necessitates urgent diagnosis and surgicalintervention to save the adnexa from gettinginfarcted. Ovarian tumours, more commonlybenign and rarely malignant, are implicated intorsion. Mixed epithelial tumours account forless than 4% (reported incidence varies from0.5%to4%)ofallovarianepithelialtumours,andincidence ofmixedmalignant epithelial tumourismuchrarer(1).Asthebehaviourofthetumourdepends on the predominant component inmixedmalignanttumours,itisimperativetoruleout amixed carcinoma in theovarianneoplasmspecimen by sectioning multiple areas andevaluatingthemmicroscopically.

Case Report

A 55-year-old post-menopausal femalepresented with sudden onset acute lowerabdominal pain. Greyscale and colour Dopplerultrasonography of the abdomen showed a“whirlpool appearance” and suggested left tubo-ovarian torsion with no evidence of enlargedlymph nodes or any lesion in the liver, kidney,

or bladder. Emergency surgery was performedand the left tubo-ovarian mass was sent forhistopathological examination. The left ovarymeasured 6 × 2.5 × 1 cm and showed markedhaemorrhage and infarction on its surface. Onsectioning the ovary, a unilocular cyst, with themajor portion infarcted, and a grey-white solidarea (Figure 1) were seen amidst the infarctedarea. The solid area did not show any papillaryprocesses.Multiplesectionsweretakenfromthegrey-whitesolidareasoftheovary.Thefallopiantube measured 4 cm in length and was grosslyinfarcted over themajority of its area. Sections(Figure 2,3) from the infarcted portion of thefallopiantubeandovaryandthegrey-whitesolidareas of the ovary showed the tumour to be amixedmalignantepithelialovariantumour(high-grade serous and high-grade transitional cellcarcinoma (TCC), associated with tubo-ovariantorsion.Theserouscarcinomacomponentshowedfineandirregularlybranchingpapillaelinedwithmalignant cuboidal to columnar epithelial cellsof high nuclear grade and presence of mitoses.Occasional slit-likeor irregularglandsandsolidcomponentsofserousadenocarcinomawerealsoseen. The TCC component showed undulatingbands, a diffuse pattern, and occasional broad

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Figure 1: Cutopen specimenof ovary showingmarkeddegreeofinfarctionandfocalgreywhitesolidarea(redarrow).

Figure 2: Section showing closely packedmalignant transitional cell nests(Haematoxylin and eosin, 100×magnification).

Figure 3: Section showing a cystic cavity linedby proliferating papillae and micropapillae of serous adenocarcinomawith invasion of stroma by solidnests and glandular structures-(Haematoxylin and eosin, 100×magnification).

papillae lined with cells with clear to oxyphiliccytoplasm, a marked degree of anisonucleosis,and prominent nucleoli in many of the cells.Serous carcinoma accounted for 85% and TCCaccountedfor15%ofthemixedepithelialovariantumour. Immunohistochemical studies of thesectionsrevealedpositivestainingforCK7,WT1,and CA125, and negative staining for CK20,thus ruling out metastasis from extra-ovarianurothelialtumours.Benigntransitionalcellnestswere not seen. Psammoma bodies were seen ina few sections. The patient was referred to theoncologyunitforfurthermanagement.

Discussion

Mixed epithelial ovarian tumours, bydefinition, are composed of admixtures of twoor more of the five major cell types: serous,mucinous,endometrioid,clearcell,andBrenner/transitional. According to the World HealthOrganisation,theminorcomponentmustaccountfor at least 10%of the tumour in amicroscopicexamination inorder tobeclassifiedasamixedtumour(1). The origin of mixed epithelial ovariantumours has been controversial. Kurman et al.(2)andMalpicaetal.(3)havestatedthatovariancancers are of de novo origin. They dividedovarian cancers into type I and type II, basedon their clinicalbehaviour.Type I includes low-grade endometrioid, clear cell, mucinous, andtransitional carcinomas, which behave in anindolent manner and are confined to the ovaryat thetimeofpresentation.TypeII tumoursarehighly aggressive, evolve rapidly, and presentin advanced stages. They include high-gradeserous carcinomas,undifferentiated carcinomas,and malignant mesodermal mixed tumours(carcinosarcoma). The frequent combinationsencounteredinmixedepithelialtumoursincludeserous/endometrioid and serous/TCC types(4–7). The least differentiated componentdetermines the tumour grade and thedominantcelltypegenerallydictatesbehaviour(8). It is quite important to identify thetransitional cell component, as tumours witha predominant TCC component respond muchbetter to chemotherapy compared to othersurface epithelial tumours. Ovarian TCC is arare subtype of epithelial ovarian cancer (9).PureformsofTCCaccountforonly1%ofsurfaceepithelial carcinomas, mixed carcinomas withpredominant TCC components account for 5%,and carcinomas with minor TCC componentsaccountfor3%(10).Itissometimesverydifficult

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to distinguish moderate-to-high-grade TCCfrom poorly differentiated serous carcinomaand undifferentiated carcinoma. TCCs arecharacterised by undulating thick bands andvaried patterns, such as insular, trabecular, andeven minor components of undifferentiatedcarcinoma. TCCs have broad papillae comparedto the fine papillae found in serous carcinomas.In addition, TCCs are lined with cells, some ofwhich have the transitional cell characteristicsof pale and granular or oxyphilic cytoplasm,roundtooblongnucleiwithasinglenucleolusora longitudinal nuclear groove, and the presenceof undulating bands lined with these cells.Immunostaining does not help to distinguishbetween TCC and poorly differentiated serouscarcinomas,asTCCstainspositivelyforallserousimmunomarkers,suchasCK7,CA125,andWT1,andnegativelyforallurothelialmarkers,suchasuroplakin,thrombomodulin,andCK20.TCCalsoneedstobedifferentiatedfromthemorecommonmalignant Brenner tumour by the absence ofbenign Brenner tumour components/benigntransitional cell nest and stromal calcification,comparedtothepresenceofthesecomponentsinmalignantBrennertumours. Ovarian torsion usually occurs unilaterallyinapathologicallyenlargedovary,anditismorecommon on the right side (60%). Torsion caninvolve the ovary alone, but it more commonlyaffects the ovary and oviduct (adnexal torsion).It frequently arises from one ofmany anatomicchanges. Torsion is associated with ovariantumour in 50–60% of cases. Dermoid tumoursarethemostcommonbenigntumoursassociatedwith torsion. Malignant tumours are much lesslikely to be associated with torsion comparedto benign tumours (11,12) due to the presenceof cancerous adhesions that fix the ovary tosurrounding tissue. Adnexal torsion is notlimitedtowomenofreproductiveage(13);post-menopausal women with an adnexal mass maybe affected with torsion. Approximately 17% ofovarian torsion patients present with suddenonset of severe unilateral lower abdominal painthat worsens intermittently over many hours.Early diagnosis of ovarian (adnexal) torsion isimperative,andcolourDopplerultrasonographyhasavitalroleintheexaminationofwomenwithlowerabdominalandpelvicpain(14,15). This case showed a varied and rarepresentationofthemuchrarersurfaceepithelialovariantumour.

Acknowledgement

None.

Conflict of Interest

None.

Funds

None.

Authors’ Contributions

Conception and design, drafting of the article,critical revision of the article for the importantintellectual content, administrative, technical orlogistic support, provision of studymaterials orpatientandfinalapprovalofthearticle:SA,TC

Correspondence

DrShashikantAdlekhaMD Pathology (RDVV), DNB Pathology (NationalBoard,NewDelhi)DepartmentofPathologySreeNarayanaInstituteofMedicalSciencesNorthKuthiyatode,Chalakka-683594District:ErnakulamKerala,IndiaTel:+9172-93052109Fax:+048-43615523Email:shuntyad@yahoo.co.in

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