Primary Peritoneal Serous Borderline Tumour Associated with Behçet's Disease: A Rare Case Report

TurkiyeKlinikleriJCaseRep2016;24(1) 18 rimaryPeritonealSerousBorderlineTumour(PPSBT)isarareen - titywithlimitedmalignantpotential.1 Realincidenceisnotknown asthelesionismostly foundasincidentalfindinginsurgicalopera - tionsmadeforanotherreason.Thisisamülleriantypeproliferationofperi- toneumwhichhasbeennamed“AtypicalEndosalpingiosis”or“Primary PapillaryPeritonealNeoplasm”inlasttwodecades.Thelong-termoutlook forthesepatientsisremarkablyfavorable .Itisdifficulttodeterminethe malignantpotentialinpre-operativeperiod .2 PrimaryPeritonealSerousBorderline TumourAssociatedwithBehçet’sDisease: A RareCaseReport AA BBSSTTRRAA CCTT Primary peritoneal serous borderline tumour is a rare epithelial proliferation can mostly present as an incidental finding at laparotomy. The risk factors for developing these tumours are not well known. Nulliparity and drugs used in infertility treatment appear to increase the risk, while oral contraceptives seem to have a protective effect. For the differential diagnosis, mesothelial pro - liferation, endosalpingiosis, endometriosis, high-grade primary papillary serous carcinoma and im - plants from primary ovarian serous carcinoma should be considered. Behçet’s Disease in association with malignancy has been reported sporadically in a few case reports. Preservation of the uterus and ovaries in young women is possible with conservative surgical approach, once the presence of an associated primary ovarian tumour has been excluded. Long term prognosis in primary peritoneal serous borderline tumour is very good similarly to ovarian borderline tumours. KKeeyy WWoorrddss:: Neoplasms, cystic, mucinous, and serous; peritoneum ÖÖZZEETT Primer peritoneal seröz “borderline” tümörler, nadir görülen bir epitelyal proliferasyon olup genellikle laparatomi sırasında tesadüfi olarak saptanırlar. Bu tümörler için risk faktörleri tam bi- linmemektedir. Nulliparite ve infertilite tedavisinde kullanılan ilaçlar risk faktörü olup oral kont - raseptifler koruyucu etki göstermektedirler. Ayırıcı tanıda mezotelyal proliferasyon, endosalpingios, endometriozis, yüksek dereceli primer peritoneal seröz karsinom ve overin primer seröz karsi- nomları düşünülmelidir. Malignensi ile birlikte Behçet hastalığı birkaç olguda sporadik olarak rapor edilmiştir. Primer ovarian bir tümörün varlığı dışlandığı durumlarda genç kadınlarda fertilitenin de korunması açısından uterus ile overleri koruyacak şekilde konservatif cerrahi tedavi uygulanabilir. Primer peritoneal seröz “borderline” tümör izleminde uzun dönem prognozu ovarian borderline tü - mörlere benzer şekilde oldukça iyidir. AA nnaahhttaarr KKeelliimmeelleerr::Tümörler, kistik, müsinöz, ve seröz; periton TTuurrkkiiyyee KKlliinniikklleerrii JJ CCaassee RReepp 22001166;;2244((11))::1188--2211 Engin KORKMAZER,a Sare KABUKÇUOĞLU b aDepartment of Gynecology and Obstetrics, Giresun University Faculty of Medicine, Giresun bDepartment of Pathology, Eskişehir Osmangazi University Faculty of Medicine, Eskişehir Ge liş Ta ri hi/Re ce i ved: 26.12.2013 Ka bul Ta ri hi/Ac cep ted: 21.12.2014 Ya zış ma Ad re si/Cor res pon den ce: Engin KORKMAZER Giresun University Faculty of Medicine, Department of Gynecology and Obstetrics, Giresun, TÜRKİYE/TURKEY [email protected] doi: 10.5336/caserep.2013-38574 Cop yright © 2016 by Tür ki ye Kli nik le ri Korkmazer et al. Medical Pathology Turkiye Klinikleri J Case Rep 2016;24(1) 19 Aim of this paper is to present microscopic findings of a case of PPSBT in a 23 year old woman together with the review of literature of this rare tumour . CASE REPORT Case is a 23-year-old, nulligravid, virgin, white race patient without any gynecological disease. In her medical history, she had Behçet’s Disease for 6 years. She has history of azothioprine, corticosteroid and interferon-alpha usage periodically before. She presented with persistent right inguinal pain. In pelvic examination, 5 cm diameter smooth surfaced mass was palpated which begins from middle ab - domen and grows to the right side. 95x58 mm paraovarian cystic lesion was detected in ultra - sound scan. Tumour markers were CA-125: 18,4 U/ml [1,9-16,3], CEA: 0,571 mg/ml [0-34], AFP: 0,988 IU/ml [0-5,8], Beta-HCG: <1,00 mIU/ml [0- 30]. In pelviabdominal computed tomography an 8,5x5x7 cm relatively smooth edged hypodens mass was detected at right lateral adnex . A 23-year-old white virgin nulligravid woman was admitted to the hospital because of persistent right inguinal pain which had started 2 months earlier associated without any other symptom. She had Behçet’s disease for 6 years with history of azothiopurine corticosteroids and interferon alpha usage for it periodically. The patient underwent laparoscopic surgery. In intraoperative exploration we found an adnexial mass which origins from peritoneum and extends to abdominal cavity with multiple adhesions to ovaries and uterus. Rest of the peritoneal surfaces was normal. We performed laparosopic cyst exci- sion. After the cyst excision patient was discharged at postoperative 3 rd day. Microscopic examination disclosed tumour clusters of branching papillae or glandular struc - tures, which had prominent psammomatous calci- fications (Figures 1, 2) .The tumour cells lack significant cytologic atypia and mitoses. Stromal in - vasion is not identified.A typical proliferating serous tumour has developed in 4 mm 2 of the peri- toneum. In that area there are also psammomatous calcifications , peritonitis, chronic inflammatory process showing abscess formation and peritoneal inclusion cysts (Figure 3). A n informed consent form obtained from pa - tient. DISCUSSION PPSBT is usually seen in women younger than 40 years and has a really good prognosis. It can pres - ents various manifestations (infertility, pelvic pain, chronic pelvic inflammatory disease, amenorrhea, pelvic mass) mostly found incidentally.3 Usually pelvic mass and high serum CA-125 levels imitate an ovarian malignancy. FIGURE 1: Atypical proliferating serous tumour development (arrows) (haematoxylin and eosin stain; original magnification, x80). FIGURE 2: Psammoma bodies (arrows) are noted in the core of the papilla and stroma (haematoxylin and eosin stain; original magnification, x200). Korkmazer ve ark. Tıbbi Patoloji Turkiye Klinikleri J Case Rep 2016;24(1) 20 The gross characteristics of PPSBT observed during the surgery have variable appearances, with focal-to-diffuse lesions that may be miliary, granu - lar, or nodular or may resemble adhesions .2,4,5 Microscopic findings of peritoneal/serosal fo - cuses are divided into two categories as lesions with or without fibroblastic reaction surrounding epithe - lial cell proliferations.Epithelial component can be changed by the proliferation of surrounding mi- crostructural cellular units. Due to its complexity and severity lesion may include unique cells or small solid cell clusters . There is no significant histological atypia or mitosis in cells. Destructive stromal inva - sion is generally not defined. Lesions without fi- broblastic reaction have wide papillary groups with epithelial cells that are located near peritoneal sur - face or can be separated easily from peritoneal sur - face and have moderate cellular atypia. Papillae are formed of flat, cuboidal or columnar cells organized in simple or stratified manner. These cells separate from stroma by forming clefts. In those lesions usu - ally there are several psammoma bodies .2,6,7 PPSBT is invariably extensive and thought to develop from a pre-existing endosalpingiosis which is present in 70-80% of cases. Endosalpingiosis is a metaplastic lesion in the peritoneal cavity and is a precursor for PPSBT .1,7 Behçet’s Disease was first described by Hulusi Behçet in 1937 as a triad of recurrent aphthous ul- cers of the mouth and genitalia and relapsing uveitis and also systematic vasculitis of unknown origin that may involve all systems.8 Several stud - ies have shown that there’s no increase in malig - nancy risk in Behçet’s Disease compared to normal population.9,10 Bell and Scully described 25 cases originally diagnosed as PPSBT, atypical endosalpingiosis, or serous cystadenoma/cystadenofibroma with peri- toneal implants.1 The patients were all women, aged 19 to 53 years, and had a variable clinical his - tory, which included abdominal/ pelvic pain (7 pa - tients), infertility (6 patients), chronic pelvic inflammatory disease, small bowel obstructive symptoms, and amenorrhea. Eight of the patients’ peritoneal lesions were incidental to laparotomy for other reasons (benign ovarian serous tumour, leiomyoma, ventral hernia, and cesarean section). Biscotti and Hart studied 17 cases of PPBST. The patients were all girls or women aged 16 to 67 years. Nine of Biscotti and Hart’s cases were inci- dental to surgery performed for other reasons, such as caesarean section, endometriosis, or adhesions .1,7 The pathological differential diagnosis inc- ludes endometriosis, endosalpin giosis, benign reactive mesothelial proliferations such as ade - nomatoid tumour or florid mesothelial hyperpla - sia, and borderline mesothelial proliferations with similar histological features, which include benign or well-differentiated papillary mesothe - lioma .4,5,11-14 Primary peritoneal neoplasms showed signifi- cantly less expression of estrogen receptor and progesterone receptor than ovarian primary tu - mours did .11 Calretinin, which is a mesothelial cell marker was negative in our case. PPSBT is a very rare case and should be con - sidered in the differential diagnosis of epithelial proliferations with associated psammoma bodies on the peritoneal surfaces of viscera. Correlation with clinical presentation, radiographic findings, surgi- cal staging, histopatologic and immunohistochem - ical findings of the epithelial proliferation is essential for the correct diagnosis. Conservative surgery can be performed in young fertile female patients. FIGURE 3: Peritoneal inclusion cyst (PIC), Haemorrhagic Necrosis (HN), Chronic inflammation (INF), Thrombosis Vessel (TV) (haematoxylin and eosin stain; original magnification, x80) Korkmazer et al. Medical Pathology Turkiye Klinikleri J Case Rep 2016;24(1) 21 1. Bell DA, Scully RE. Serous borderline tumours of the peritoneum. Am J Surg Pathol 1990; (14):230-9. 2. Hutton RL, Dalton Sr. Primary peritoneal serous borderline tumours. Arch Pathol Lab Med 2007;131(1):138-44. 3. Tinelli A, Malvasi A, Pellegrino M. An inciden - tal peritoneal serous borderline tumor during laparoscopy for endometriosis. Eur J Gy - naecol Oncol 2009;30(5):579-82. 4. 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Behçet H. Uber rezidivierende aphtose, durch ein virus verursachte Geschwure am Mund, am Auge und an den Genitalien. Dermatol Wschr 1937;105:1152-7. 10. Arimura K, Arima N, Matsushita K, Akimoto M, Park CY, Uozumi K, et al. High incidence of morphological myelodysplasia and apoptotic bone marrow cells in Behçet’s disease. J Clin Immunol 2007;27(2):145-51. 11. Tada Y, Koarada S, Haruta Y, Mitamura M, Ohta A, Nagasawa K. The association of Be - hçet's disease with myelodysplastic syndrome in Japan: a review of the literature. Clin Exp Rheumatol 2006;24(5 Suppl 42):S115-9. 12. Halperin R, Zehavi S, Hadas E, Habler L, Bukovski I, Schneider D. Immunohistochemi- cal comparison of primary peritoneal and pri- mary ovarian serous papillary carcinoma. Int J Gynecol Pathol 2001;20(4):341-5. 13. Bell DA, Scully RE. Benign and borderline serous lesions of the peritoneum in women. Path Annu 1989;24 Pt 2:1-21. 14. Bell KA, Smith Sehdev AE, Kurman RJ. 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