{"paper_id":"e027bd71-d1aa-4535-9147-5a939466cf09","body_text":"TurkiyeKlinikleriJCaseRep2016;24(1) \n18 \nrimaryPeritonealSerousBorderlineTumour(PPSBT)isarareen -\ntitywithlimitedmalignantpotential.1 Realincidenceisnotknown \nasthelesionismostly foundasincidentalfindinginsurgicalopera -\ntionsmadeforanotherreason.Thisisamülleriantypeproliferationofperi-\ntoneumwhichhasbeennamed“AtypicalEndosalpingiosis”or“Primary \nPapillaryPeritonealNeoplasm”inlasttwodecades.Thelong-termoutlook \nforthesepatientsisremarkablyfavorable .Itisdifficulttodeterminethe \nmalignantpotentialinpre-operativeperiod .2\nPrimaryPeritonealSerousBorderline\nTumourAssociatedwithBehçet’sDisease:\nA RareCaseReport \nAA BBSSTTRRAA CCTT  Primary peritoneal serous borderline tumour is a rare epithelial proliferation can mostly \npresent as an incidental finding at laparotomy. The risk factors for developing these tumours are not \nwell known. Nulliparity and drugs used in infertility treatment appear to increase the risk, while \noral contraceptives seem to have a protective effect. For the differential diagnosis, mesothelial pro -\nliferation, endosalpingiosis, endometriosis, high-grade primary papillary serous carcinoma and im -\nplants from primary ovarian serous carcinoma should be considered. Behçet’s Disease in association \nwith malignancy has been reported sporadically in a few case reports. Preservation of the uterus and \novaries in young women is possible with conservative surgical approach, once the presence of an \nassociated primary ovarian tumour has been excluded. Long term prognosis in primary peritoneal\nserous borderline tumour is very good similarly to ovarian borderline tumours.\nKKeeyy  WWoorrddss::  Neoplasms, cystic, mucinous, and serous; peritoneum    \nÖÖZZEETT  Primer peritoneal seröz “borderline” tümörler,  nadir görülen bir epitelyal proliferasyon olup \ngenellikle laparatomi sırasında tesadüfi olarak saptanırlar. Bu tümörler için risk faktörleri tam bi-\nlinmemektedir. Nulliparite ve infertilite tedavisinde kullanılan ilaçlar risk faktörü olup oral kont -\nraseptifler koruyucu etki göstermektedirler. Ayırıcı tanıda mezotelyal proliferasyon, endosalpingios,\nendometriozis, yüksek dereceli primer peritoneal seröz karsinom ve overin primer seröz karsi-\nnomları düşünülmelidir. Malignensi ile birlikte Behçet hastalığı birkaç olguda sporadik olarak rapor \nedilmiştir. Primer ovarian bir tümörün varlığı dışlandığı durumlarda genç kadınlarda fertilitenin de \nkorunması açısından uterus ile overleri koruyacak şekilde konservatif cerrahi tedavi uygulanabilir.\nPrimer peritoneal seröz “borderline” tümör izleminde uzun dönem prognozu ovarian borderline tü -\nmörlere benzer şekilde oldukça iyidir.\nAA nnaahhttaarr  KKeelliimmeelleerr::Tümörler, kistik, müsinöz, ve seröz; periton    \nTTuurrkkiiyyee  KKlliinniikklleerrii  JJ  CCaassee  RReepp  22001166;;2244((11))::1188--2211\nEngin KORKMAZER,a\nSare KABUKÇUOĞLU b\naDepartment of Gynecology and Obstetrics,\nGiresun University Faculty of Medicine,\nGiresun \nbDepartment of Pathology,\nEskişehir Osmangazi University \nFaculty of Medicine, Eskişehir \nGe liş Ta ri hi/Re ce i ved: 26.12.2013 \nKa bul Ta ri hi/Ac cep ted: 21.12.2014 \nYa zış ma Ad re si/Cor res pon den ce: \nEngin KORKMAZER \nGiresun University Faculty of Medicine,\nDepartment of Gynecology and Obstetrics,\nGiresun, \nTÜRKİYE/TURKEY \nekorkmazer@yahoo.com \ndoi: 10.5336/caserep.2013-38574 \nCop yright © 2016 by Tür ki ye Kli nik le ri \n\nKorkmazer et al. Medical Pathology \nTurkiye Klinikleri J Case Rep 2016;24(1) \n19 \nAim of this paper is to present microscopic \nfindings of a case of PPSBT in a 23 year old woman \ntogether with the review of literature of this rare \ntumour . \nCASE REPORT \nCase is a 23-year-old, nulligravid, virgin, white race \npatient without any gynecological disease. In her \nmedical history, she had Behçet’s Disease for 6 \nyears.\nShe has history of azothioprine, corticosteroid \nand interferon-alpha usage periodically before. She \npresented with persistent right inguinal pain. In \npelvic examination, 5 cm diameter smooth surfaced \nmass was palpated which begins from middle ab -\ndomen and grows to the right side. 95x58 mm \nparaovarian cystic lesion was detected in ultra -\nsound  scan. Tumour markers were CA-125: 18,4 \nU/ml [1,9-16,3], CEA: 0,571 mg/ml [0-34], AFP:\n0,988 IU/ml [0-5,8], Beta-HCG: <1,00 mIU/ml [0- \n30]. In pelviabdominal computed tomography an \n8,5x5x7 cm relatively smooth edged hypodens mass \nwas detected at right lateral adnex .\nA 23-year-old white virgin nulligravid woman \nwas admitted to the hospital because of persistent \nright inguinal pain which had started 2 months \nearlier associated without any  other symptom. She \nhad Behçet’s disease for 6 years with  history of \nazothiopurine corticosteroids and interferon alpha \nusage for it periodically.\nThe patient underwent laparoscopic surgery.\nIn intraoperative exploration we found an adnexial\nmass which origins from peritoneum and extends \nto abdominal cavity with multiple adhesions to \novaries and uterus. Rest of the peritoneal surfaces \nwas normal. We performed laparosopic cyst exci-\nsion. After the cyst excision patient was discharged \nat postoperative 3 rd day.\nMicroscopic examination disclosed tumour \nclusters of branching papillae or glandular struc -\ntures, which had prominent psammomatous calci-\nfications (Figures 1, 2) .The tumour cells lack \nsignificant cytologic atypia and mitoses. Stromal in -\nvasion is not identified.A typical proliferating \nserous tumour has developed in 4 mm 2 of the peri-\ntoneum. In that area there are also psammomatous \ncalcifications , peritonitis, chronic inflammatory \nprocess showing abscess formation and peritoneal\ninclusion cysts (Figure 3).\nA n informed consent form obtained from pa -\ntient.\nDISCUSSION \nPPSBT is usually seen in women younger than 40 \nyears and has a really good prognosis. It can pres -\nents various manifestations (infertility, pelvic pain,\nchronic pelvic inflammatory disease, amenorrhea,\npelvic mass) mostly found incidentally.3 Usually \npelvic mass and high serum CA-125 levels imitate \nan ovarian malignancy. \nFIGURE 1: Atypical proliferating serous tumour development (arrows) \n(haematoxylin and eosin stain; original magnification, x80).\nFIGURE 2: Psammoma bodies (arrows) are noted in the core of the papilla \nand stroma (haematoxylin and eosin stain; original magnification, x200).\n\nKorkmazer ve ark. Tıbbi Patoloji\nTurkiye Klinikleri J Case Rep 2016;24(1) \n20 \nThe gross characteristics of PPSBT observed \nduring the surgery have variable appearances, with \nfocal-to-diffuse lesions that may be miliary, granu -\nlar, or nodular or may resemble adhesions .2,4,5 \nMicroscopic findings of peritoneal/serosal fo -\ncuses are divided into two categories as lesions with \nor without fibroblastic reaction surrounding epithe -\nlial cell proliferations.Epithelial component can be \nchanged by the proliferation of surrounding mi-\ncrostructural cellular units. Due to its complexity \nand severity lesion may include unique cells or small\nsolid cell clusters . There is no significant histological\natypia or mitosis in cells. Destructive stromal inva -\nsion is generally not defined. Lesions without fi-\nbroblastic reaction have wide papillary groups with \nepithelial cells that are located near peritoneal sur -\nface or can be separated easily from peritoneal sur -\nface and have moderate cellular atypia. Papillae are \nformed of flat, cuboidal or columnar cells organized \nin simple or stratified manner. These cells separate \nfrom stroma by forming clefts. In those lesions usu -\nally there are several psammoma bodies .2,6,7 \nPPSBT is invariably extensive and thought to \ndevelop from a pre-existing endosalpingiosis which \nis present in 70-80% of cases. Endosalpingiosis is a \nmetaplastic lesion in the peritoneal cavity and is a \nprecursor for PPSBT .1,7 \nBehçet’s Disease was first described by Hulusi\nBehçet in 1937 as a triad of recurrent aphthous ul-\ncers of the mouth and genitalia and relapsing \nuveitis and also systematic vasculitis of unknown \norigin that may involve all systems.8 Several stud -\nies have shown that there’s no increase in malig -\nnancy risk in Behçet’s Disease compared to normal\npopulation.9,10 \nBell and Scully described 25 cases originally \ndiagnosed as PPSBT, atypical endosalpingiosis, or \nserous cystadenoma/cystadenofibroma with peri-\ntoneal implants.1 The patients were all women,\naged 19 to 53 years, and had a variable clinical his -\ntory, which included abdominal/ pelvic pain (7 pa -\ntients), infertility (6 patients), chronic pelvic \ninflammatory disease, small bowel obstructive \nsymptoms, and amenorrhea. Eight of the patients’\nperitoneal lesions were incidental to laparotomy \nfor other reasons (benign ovarian serous tumour,\nleiomyoma, ventral hernia, and cesarean section).\nBiscotti and Hart studied 17 cases of PPBST. The \npatients were all girls or women aged 16 to 67 \nyears. Nine of Biscotti and Hart’s cases were inci-\ndental to surgery performed for other reasons, such \nas caesarean section, endometriosis, or adhesions .1,7 \nThe pathological differential diagnosis inc- \nludes endometriosis, endosalpin giosis, benign \nreactive mesothelial proliferations such as ade -\nnomatoid tumour or florid mesothelial hyperpla -\nsia, and borderline mesothelial proliferations \nwith similar histological features, which include \nbenign or well-differentiated papillary mesothe -\nlioma .4,5,11-14 \nPrimary peritoneal neoplasms showed signifi-\ncantly less expression of estrogen receptor and \nprogesterone receptor than ovarian primary tu -\nmours did .11 Calretinin, which is a mesothelial cell\nmarker was negative in our case.\nPPSBT is a very rare case and should be con -\nsidered in the differential diagnosis of epithelial\nproliferations with associated psammoma bodies on \nthe peritoneal surfaces of viscera. Correlation with \nclinical presentation, radiographic findings, surgi-\ncal staging, histopatologic and immunohistochem -\nical findings of the epithelial proliferation is \nessential for the correct diagnosis. Conservative \nsurgery can be performed in young fertile female \npatients.\nFIGURE 3: Peritoneal inclusion cyst (PIC), Haemorrhagic Necrosis (HN),\nChronic inflammation (INF), Thrombosis Vessel (TV) (haematoxylin and eosin \nstain; original magnification, x80) \n\nKorkmazer et al. Medical Pathology \nTurkiye Klinikleri J Case Rep 2016;24(1) \n21 \n1. Bell DA, Scully RE. Serous borderline tumours \nof the peritoneum. Am J Surg Pathol 1990;\n(14):230-9.\n2. Hutton RL, Dalton Sr. Primary peritoneal\nserous borderline tumours. Arch Pathol Lab \nMed 2007;131(1):138-44.\n3. Tinelli A, Malvasi A, Pellegrino M. An inciden -\ntal peritoneal serous borderline tumor during \nlaparoscopy for endometriosis. Eur J Gy -\nnaecol Oncol 2009;30(5):579-82.\n4. Arraiza M, Metser U, Vajpeyi R, Khalili K, Han -\nbidge A, Kennedy E, et al. Primary cystic peri-\ntoneal masses and mimickers: spectrum of\ndiseases with pathologic correlation. Abdom \nImaging 2014 Oct 1. [Epub ahead of print]\n5. Levy AD, Shaw JC, Sobin LH. Secondary tu -\nmors and tumorlike lesions of the peritoneal\ncavity: imaging features with pathologic cor -\nrelation. Radiographics 2009;29(2):347-73.\n6. Biron-Shental T, Klein Z, Edelstein E, Al-\ntaras M, Fishman A. Primary peritoneal bor -\nderline tumour. A case report and review of\nthe literature. Eur J Gynaecol Oncol 2003;\n24(1):96-8.\n7. Go HS, Hong HS, Kim JW, Woo JY. CT ap -\npearance of primary peritoneal serous border -\nline tumour: a rare epithelial tumour of the \nperitoneum. Br J Radiol 2012;85(1009):e22-5.\n8. Biscotti CV, Hart WR. Peritoneal serous mi-\ncropapillomatosis of low malignant potential\n(serous borderline tumours of the peritoneum):\na clinicopathologic study of 17 cases. Am J \nSurg Pathol 1992;16(5):467-75.\n9. Behçet H. Uber rezidivierende aphtose, durch \nein virus verursachte Geschwure am Mund,\nam Auge und an den Genitalien. Dermatol\nWschr 1937;105:1152-7.\n10. Arimura K, Arima N, Matsushita K, Akimoto M,\nPark CY, Uozumi K, et al. High incidence of\nmorphological myelodysplasia and apoptotic \nbone marrow cells in Behçet’s disease. J Clin \nImmunol 2007;27(2):145-51.\n11. Tada Y, Koarada S, Haruta Y, Mitamura M,\nOhta A, Nagasawa K. The association of Be -\nhçet's disease with myelodysplastic syndrome \nin Japan: a review of the literature. Clin Exp \nRheumatol 2006;24(5 Suppl 42):S115-9.\n12. Halperin R, Zehavi S, Hadas E, Habler L,\nBukovski I, Schneider D. Immunohistochemi-\ncal comparison of primary peritoneal and pri-\nmary ovarian serous papillary carcinoma. Int J \nGynecol Pathol 2001;20(4):341-5.  \n13. Bell DA, Scully RE. Benign and borderline \nserous lesions of the peritoneum in women.\nPath Annu 1989;24 Pt 2:1-21.\n14. Bell KA, Smith Sehdev AE, Kurman RJ. Re -\nfined diagnostic criteria for implants associ-\nated with ovarian atypical proliferative serous \ntumours (borderline) and micropapillary \nserous carcinoma. Am J Surg Pathol 2001;\n25(4):419-32.\n15. Smith Sehdev AE, Sehdev PS, Kurman RJ.\nNoninvasive and invasive micropapillary (low- \ngrade) serous carcinoma of the ovary: a clini-\ncopathologic analysis of 135 cases. Am J Surg \nPathol 2003;(27(6):725-36.\nREFERENCES","source_license":"CC0","license_restricted":false}