Struma ovarii with follicular thyroid-type carcinoma and neuroendocrine component: case report

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1 Selvggi et l. World Journl of Surgicl Oncology 2012, 10:93 WORLD JOURNAL OF SURGICAL ONCOLOGY CASE REPORT Open Access Strum ovrii with folliculr thyroid-type crcinom nd neuroendocrine component: cse report Federico Selvggi 1, Domenico Risio 1, Mthew Wku 1, Dniel Simo 1, Domenico Angelucci 2, Alerto D Aulerio 1, Roerto Cotellese 1 nd Polo Innocenti 1* Astrct Strum ovrii (SO) is slow-growing ovrin neoplsm with thyroid tissue s its predominnt component. It is n uncommon neoplsm, usully symptomtic with n unknown risk of mlignnt trnsformtion. Due to difficulties in ssessing the rre iologicl nture nd the discrepncies in the reported cses, consensus on the pproprite tretment hs not een definitively reched. A 50-yer-old femle ws sujected to upper gut endoscopy which showed 30-mm mss locted in the gstric ntrum, suggestive of mesenchiml tumor. Incidentlly, pelvic CT scn lso documented solid mss in the right dnex, with morphologicl chrcteristics of ovrin neoplsm. The ptient underwent gstrectomy, totl hysterectomy, ilterl slpingo-oophorectomy with lymph node dissection, nd omentectomy. Histology documented the presence of gstric cvernous ngiom, nd, in the right dnex, foci of folliculr thyroid-type crcinom rising in SO with well-differentited neuroendocrine component. Here we report nd discuss the clinicl nd morphologicl presenttion of folliculr thyroid-type crcinom rising in SO. The neoplsm ws discovered incidentlly nd hd fvorle clinicl outcome t 1-yer follow-up. Keywords: Strum ovrii, Folliculr thyroid crcinom, Hysterectomy, Bilterl slpingo-oophorectomy Bckground Strum ovrii (SO) is monoderml vrint of ovrin tertom which predominntly contins thyroid tissue [1-4]. The incidence is from 0.1% to 0.3% of ll ovrin tertoms [5]. Ptients re usully symptomtic or predominntly presented with pelvic mss in 45% of cses or dominl pin [5]. Menstrul irregulrities nd clinicl hyperthyroidism hve een demonstrted in 9% nd 5% of cses, respectively [5,6]. Dignosis of SO is sed on histopthologicl criteri nd guidelines for primry thyroid glnd disese [2,5]. Mlignnt SO consists of ppillry thyroid crcinom (PTC) cells, folliculr vrint of PTC or folliculr thyroid crcinom (FTC) cells, nd mixed folliculr/ppillry crcinom cells [7,8]. The most common type is PTC, followed y FTC nd the recent form of highly differentited folliculr crcinom * Correspondence: innocenti@unich.it 1 Unit of Generl nd Lproscopic Surgery, Biomedicl Sciences Deprtment, G. d Annunzio University, Vi dei Vestini 31, Chieti 66100, Itly Full list of uthor informtion is ville t the end of the rticle (HDFCO), chrcterized y extrovrin dissemintion of thyroid elements [2,4,9]. The differentil dignosis etween enign nd mlignnt neoplsm is still extremely difficult, in prticulr for SO with folliculr growth pttern nd undefined cpsule. The incidence of mlignncy is lso difficult to ssess due to the rre nture of this cse nd the sence of stndrdized dignostic criteri [5,9]. Mlignnt trnsformtion hs een reported to vry from 5% to 37% of cses, metstsis is seen in 23% of cses nd it is minly intr-dominl [5]. Bsing our reserch on the review of specific scientific literture, we exmined this uncommon cse of folliculr thyroid-type crcinom rising in SO, unsuspected in the preopertive evlution. Cse report A 50-yer-old femle presented to our oservtion with epigstric pin. Her pst surgicl history ws uneventful except for ppendectomy. A 30-mm gstric solid mss, suggestive of mesenchiml tumor, ws documented y 2012 Selvggi et l.; licensee BioMed Centrl Ltd. This is n Open Access rticle distriuted under the terms of the Cretive Commons Attriution License ( which permits unrestricted use, distriution, nd reproduction in ny medium, provided the originl work is properly cited.

2 Selvggi et l. World Journl of Surgicl Oncology 2012, 10:93 Pge 2 of 5 endoscopy. Due to its high vsculriztion nd the risk of gstrointestinl leeding, iopsy ws not indicted. The CT scn of the domen confirmed the presence of the gstric lesion, nd incidentlly showed pelvic mss of mm in the right dnex (Figure 1). The ptient ws symptomtic. The pelvic finding ws confirmed y US scn: the right ovrin mss hd prevlent solid component with rich supply of lood vessels. No signs or symptoms of hyperthyroidism were oserved. The ptient underwent right oophorectomy, nd fter intropertive histologicl dignosis of ovrin crcinom, totl dominl hysterectomy with ilterl slpingo-oophorectomy, retroperitonel lymph node dissection, nd omentectomy were finlly performed. The gstric lesion ws resected nd the digestive continuity ws restored y Billroth s II nstomosis. At surgery, no norml lymph nodes nd scites were noted. Histology did not revel signs of peritonel crcinomtosis in the pelvis. Mcroscopiclly the right ovry mss mesured 62x45 mm nd cut sections showed solid white-yellowish tissue with foclly hemorrhgic res. The folliculr thyroid-type crcinom ws chrcterized y the prolifertion of cells rrnged in folliculr nd treculr pttern (Figure 2). In the contest of ovrin tertom, the folliculr component showed n infiltrtive growth s oserved in thyroid crcinom with moderte differentition. The mitotic index ws not elevted nd ny necrotic tissue res hve een documented. The folliculr tumor order ws infiltrted ut the cellulr growth ws restricted within the cpsule. Vsculr invsion ws reported in pericpsulr cpillry structures (Figure 3). Together with the focl folliculr thyroid-type crcinom, the mture ovrin tertom showed well-differentited neuroendocrine component with cells rrnged in cordonllveolr structures (Figure 3). For immunohistochemistry, sections of formlin-fixed prffin-emedded smples were treted with H 2 O 2 /3% Figure 1 CT ppernce of gstric nd ovrin neoplsms: 30-mm solid mss with minute clcifictions locted etween the nterior wll of corpus nd gstric ntrum (, rrow). Pelvic mss of mm on right dnex suggestive of mixed cystic-solid ovrin tumor (). for 5 minutes to inhiit endogenous peroxidse nd then wshed in H 2 O. Antigen ws unmsked y tretment with EDTA t ph 9, or with citrte uffer t ph 6 in microwve oven (two 5-minutes courses). The slices were then held for 20 minutes t room temperture. After wshing in PBS/Tween-20, sections were incuted for 30 minutes with the primry ntiodies. Then, they were wshed nd stined with Bond TM Polymer Refine/HRP Detection Kit ccording to the mnufcturer s protocol (Leic, Wetzlr, Germny) or Bond TM Polymer Refine Red Detection Kit (Leic) for PgR, ER, TTF1, Chromogrnin A, Clcitonin, CD56, CDX2, CEA, CK20. For negtive controls, we sustituted non-immune ser for the primry ntiodies. The immunohistochemistry nlysis demonstrted positivity for PgR, TTF1, Chromogrnin A, CD56, CDX2, CEA, nd CK20. It ws negtive for ER nd clcitonin. Bsed on these specific immunophenotype profile, dignosis of folliculr thyroid-type crcinom rising in SO ws mde (Figures 2 nd 3). The multiple peritonel iopsies nd the lymph nodes were free from metsttic cncer cells. In ddition, peritonel wshing reveled mesothelil cells, leukocytes ut not tumor cells on cytologicl exmintion. The definitive dignosis of gstric neoplsm ws cvernous hemngiom involving sumucos (Figure 3). The immedite postopertive course ws chrcterized y nemi treted y medicl supporting therpy nd lood trnsfusions. Postopertive thyroid scn nd thyroid function were norml. She ws referred for thyroidectomy ut she refused the opertion. Thyrogloulin levels were monitored in the postopertive period. Nine months fter tretment, lortory evlution reveled norml serum TSH (0.95 mu/l) nd thyrogloulin (6.17 ng/ml) levels. Locl nd distnt recurrences were not oserved t the CT scn evlution fter follow-up period of 1-yer nd the ptients ws scheduled for further follow-up. During this period the US of the neck reveled norml thyroid glnd. Discussion Although SO hs elicited considerle interest since it ws first descried, mny dignostic spects re still unknown. SO, defined s contining 50% or more thyroid tissue, ccounts for pproximtely 5% of ll ovrin tertom, with n incidence of mlignnt trnsformtion reported in 5% to 37% of SO [1,10,11]. Most cses re found incidentlly nd for this reson the only clinicl dt re otined from retrospective reports. Previous studies hve demonstrted tht the mjority of ptients with SO re symptomtic or ccompnied y non-specific symptoms similr to other ovrin neoplsms. At the time of dignosis, the most common symptoms were lower dominl pin, plple dominl mss, scites, nd norml vginl leeding [5,12]. Unusul clinicl presenttions such s hyperthyroidism nd Meigs' syndrome hve een lso documented [2].

3 Selvggi et l. World Journl of Surgicl Oncology 2012, 10:93 Pge 3 of 5 c d Figure 2 Histologicl nd immunohistochemicl profile of thyroid type crcinom in SO. The sections show folliculr thyroid cells with the mucoid glndulr component (H&E), 10 (); strong nd diffuse CD56 rectivity, 20 (); Chromogrnin A expression in ovrin strum, showing the neuroendocrine cells 20 (c); Intrfolliculr mteril nd the immunorectivity for thyrogloulin, 20 (d). The incidence of hyperthyroidism ws reported to e 5% to 8% nd 17% to 33% of the cses hd scites t dignosis [4-6,12,13]. The pthophysiology of hyperthyroidism in SO is still unknown. Mtsud nd collegues hve reported tht mlignnt SO cn e dignosed efore opertion y the evlution of free T3, nd T4, Thyrogloulin, nd TSH. The extremely high levels of Thyrogloulin in locl ovrin venousloodcompredwiththtinperipherlloodprovide evidence of the production of Thyrogloulin in the ovrin tumor nd the normliztion of it s serum levels is c d Figure 3 Histologicl nd immunohistochemicl profile of thyroid type crcinom in SO. Microscopicl reltionship etween SO nd ovrin tertom: on the right side mture one tissue with osteolsts; on the left, folliculr thyroid cncer cells, 20 (). Vsculr invsion: cpillry structures re invded y cncer cells in the pericpsulr re (, lck rrows). Neuroendocrine tumor morphology with cordonl-lveolr pttern (c). Cvernous hemngiom of the stomch rising from sumucos: on the lower prt of the section, norml gstric mucos nd muscolris mucose, 20 (d).

4 Selvggi et l. World Journl of Surgicl Oncology 2012, 10:93 Pge 4 of 5 relted to surgicl resection of the tumor [13]. In our cse theptientdidnotshownyspecificsymptomsndthe dignosis of SO ws incidentl nd only estlished fter surgicl removl. Thyroid tissue in SO is morphologiclly, iochemiclly identicl to tht of cervicl thyroid glnd. For this reson the dignosis of mlignnt SO is in conformity to the sme criteri used for thyroid crcinom, such s the presence of ground glss nuclei, vsculr invsion, nd mitotic ctivity [3]. Unfortuntely, the concept of mlignnt SO in the literture is confusing due to lck of stndrdized prognostic prmeters. The dignosis of well-differentited vrint is prticulrly difficult due to lck of well-defined tumor cpsule in the ovrin tissue. In these cses, the presence of infiltrtion y tumor cells into the surrounding ovrin tissue, nd involvement of vsculr system, or metstsis, highly supported the dignosis of mlignncy [2]. Although the histologicl spects of mlignncy re often documented, the mjority of ptients tht underwent surgicl tretment for thyroid type crcinom rising in SO did not show cliniclly ggressive outcome with high rtes of recurrences nd metsttic spred. The iologicl ehvior remins often enigmtic nd seems not to e correlted with the oserved long-term clinicl outcomes. With the efforts of understnding the prognostic prmeters nd defining more pproprite tretment cre, moleculr nd morphologicl consensus is dvocted to stndrdize dignostic criteri of mlignncy in cses of thyroid type crcinom in SO. Distnt metstsis hs een reported to e rre feture of SO in pproximtely 5% of cses [12]. Other clinicl studies demonstrted higher intr-dominl metsttic rte of 23% [5]. Common metsttic sites re the omentum, peritoneum, lymph nodes, fllopin tues, nd the contrlterl ovry [12]. Some ptients presented with distnt metstses to the lungs, one, rin, liver, nd mesenteric surfces of the spleen nd diphrgm [8,11]. In clinicl dignosis, thyroid type crcinom in SO hs to e differentited from cystdenom nd other primry ovrin cncers or metsttic tumors. Generlly, SO ppered s smoothmrgin multicystic mss with high ttenution signls during pre-contrst on CT scn. Signl intensities on T1-weighted imges were usully intermedite to high, nd those on T2-weighted imges were low s recently reported y Shen nd collegues [14]. The mjority of tumors showed mixed cystic nd solid mss, with cpsule wll thickness of 3 mm on verge, nd contined trnsprent, green,orrownfluids[14].toourknowledgefivelethl cses hve een reported in literture with recurrences nd metsttic disese [8]. In our cse no metstsis hve een documented during 1-yer follow-up fter surgery. The tretment of mlignnt SO remins controversil nd no consensus exists on the surgicl nd non-surgicl modlities. Surgicl tretment consists in totl dominl hysterectomy plus ilterl slpingo-oophorectomy with omentectomy nd lymph nodes smpling, resonle therpy for postmenopusl women with dignosis of crcinom. In other conditions, especilly when fertility hs to e preserved, conservtive surgery concerning unilterl oophorectomy might e proposed together with strict follow-up [5]. This hypothesis should e crefully discussed with the ptients. Lproscopic surgery my offer some dvntges in surgicl stging nd its miniinvsive removl ut this technique is not stndrdized for these uncommon conditions [6]. After the initil surgery, some uthors hve dvocted ner totl thyroidectomy nd rdioctive iodine ltion to detect nd tret recurrent disese [11]. Totl thyroidectomy is mndtory to exclude primry thyroid neoplsm in the differentil dignosis of SO. Totl thyroidectomy followed y ¹³¹ I rdio ltion therpy should e reserved for ptients with recurrence or residul disese [5,10]. Thyrogloulin is well estlished mrker for monitoring the recurrence of mlignncy. An incresed serum levels of Thyrogloulin represent the erly detection of recurrence s reported in mny studies [6,11]. SO contining thyroid type crcinom must e distinguished from ppillry or folliculr thyroid crcinom metsttic to the ovry [4]. It is mndtory to study the thyroid glnd for the differentil dignosis of primry or secondry tumor of the ovry. De Simone nd collegues hve proposed thyroidectomy to confirm norml thyroid glnd, y excluding primry thyroid crcinom, nd potentite rdioctive iodine therpy [3]. No consensus hs een reched in performing prophylctic totl thyroidectomy fter the dignosis of thyroid type crcinom in SO. In this sitution, the comintion of surgicl removl with susequent thyroidectomy nd rdiotherpy do not represent stndrdized therpy ut it is comprehensive therpeutic modlity, still not supported y scientific dt in reducing the risk of recurrence nd increse prognostic indices of selected cses. Competing interests The uthors declre tht they hve no competing interests. Authors contriutions FS, MW, RC, nd PI nlyzed the dt nd wrote the mnuscript. DR, AD, nd DS prticipted in the cquisition nd interprettion of rdiologicl dt. DA crried out the histologicl nd io-moleculr studies. FS nd PI contriuted to the finl version nd crried out the clinicl cse report. All uthors red nd pproved the finl mnuscript. Acknowledgements Written informed consent ws otined for puliction of this clinicl cse with ccompnying imging. The work ws supported y funding to F.S. from G. d Annunzio University, Chieti, Itly; Support Grnt Ex-Legge 240/2010 (Progetto Specile Multisse RETI PER L ALTA FORMAZIONE - P.O. F.S.E , Pino Opertivo ).

5 Selvggi et l. World Journl of Surgicl Oncology 2012, 10:93 Pge 5 of 5 Author detils 1 Unit of Generl nd Lproscopic Surgery, Biomedicl Sciences Deprtment, G. d Annunzio University, Vi dei Vestini 31, Chieti 66100, Itly. 2 Unit of Pthology, G. d Annunzio University, Chieti, Itly. Received: 24 Jnury 2012 Accepted: 9 April 2012 Pulished: 21 My 2012 References 1. Nvrro MD, Tn MAL, Lovecchio JL, Hjdu SI: Cse report: mlignnt strum ovrii. Ann Clin L Sci 2004, 34(1): Roth LM, Tlermn A: The enigm of strum ovrii. Pthology 2007, 39(1): Dogny M, Gungor T, Cvkytr S, Sirvn L, Mollmhmutoglu L: Mlignnt strum ovrii with focus of ppillry thyroid cncer: cse report. Arch Gynecol Ostet 2008, 277: Slmn WD, Singh M, Twij Z: A cse of ppillry thyroid crcinom in strum ovrii nd review of the literture. Ptholog Res Int 2010, : Mkni S, Kim W, G AR: Strum Ovrii with focus of ppillry thyroid cncer: cse report nd review of the literture. Ginecol Oncol 2004, 94: Kremer B, Grischke EM, Steler A, Hirides P, Rothmund R: Lproscopic excision of mlignnt strum ovrii nd 1 yer follow-up without further tretment. Fertil Steril 2011, 95(6):2124. e9-e Celestino R, Mglhães J, Cstro P, Triller M, Vingre J, Sores P, Sorinho-Simões M: A folliculr vrint of ppillry thyroid crcinom in strum ovrii. Cse report with unique moleculr ltertions. Histopthology 2009, 55(4): Mrcy PY, Thrit J, Benisvy D, Azur P: Lethl, mlignnt, metsttic strum ovrii. Thyroid 2010, 10(9): Zhng X, Axiotis C: Thyroid-Type Crcinom of Strum Ovrii. Arch Pthol L Med 2010, 134: Boutross-Tdross O, Sleh R, As SL: Folliculr vrint ppillry thyroid crcinom rising in strum ovrii. Endocr Pthol 2007, 18: Yss L, Sdow P, Mrqusee E: Mlignnt strum ovrii. Nt Clin Prct Endocrinol Met 2008, 4(8): Yoo S-C, Chng Ki-Hong, Lyu Mi-Ok, Chng S-J, Ryu H-S, Kim H-S: Clinicl chrcteristics of strum ovrii. J Gynecol Oncol 2008, 19(2): Mtsud K, Mehm T, Knzw K: Mlignnt strum ovrii with thyrotoxicosis. Gynecol Oncol 2001, 82: Shen J, Xi X, Lin Y, Zhu W, Yun J: Dignosis of Strum ovrii with medicl imging. Adom Imging 2011, 36: doi: / Cite this rticle s: Selvggi et l.: Strum ovrii with folliculr thyroid-type crcinom nd neuroendocrine component: cse report. World Journl of Surgicl Oncology :93. Sumit your next mnuscript to BioMed Centrl nd tke full dvntge of: Convenient online sumission Thorough peer review No spce constrints or color figure chrges Immedite puliction on cceptnce Inclusion in PuMed, CAS, Scopus nd Google Scholr Reserch which is freely ville for redistriution Sumit your mnuscript t