A case of pleomorphic leiomyoma in Bartholin gland\u27s area in a 26-year-old woman
is reported. After diagnostic treatment, primary excision was done. A large, solid tumor
10  7.5 cm was extirpated. The tumor showed locally invasive behavior, which suggested
a malignant tumor of Bartholin gland, because of it\u27s localization and outlook. Pathohistological
examination and immunohistochemical reactions proved that it was a
mesenchymal tumor of smooth muscle origin with marked polymorphism, without mitosis,
with a myxoid stroma and with biological aggressivity, and the possibility of local
recurrence. Thus, a second more radical surgical procedure, was performed. In the excised
tissue, no residual tumor was found and all lymphnodes were negative

D. Čuržik

J. Topolovec

M. Milojković

M. Mrčela

S. Šijanović

Z. Hrgović

Z. Topolovec

English

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Coll. Antropol. 26 (2002) 2: 571–575UDC 618.16-006Original scientific paperPleomorphic Vulvar Leiomyoma withLocal Invasive BehaviorZ. Topolovec1, M. Mr~ela2, M. Milojkovi}1, S. [ijanovi}1, J. Topolovec3,D. ^ur`ik1 and Z. Hrgovi}11 Department for Gynecology and Obstetrics University Hospital »Osijek«, Osijek, Croatia2 Department for Pathology, University Hospital »Osijek«, Osijek, Croatia3 Byk Gulden Pharm., Konstanz, GermanyA B S T R A C TA case of pleomorphic leiomyoma in Bartholin gland's area in a 26-year-old womanis reported. After diagnostic treatment, primary excision was done. A large, solid tumor10  7.5 cm was extirpated. The tumor showed locally invasive behavior, which suggest-ed a malignant tumor of Bartholin gland, because of it's localization and outlook. Pa-thohistological examination and immunohistochemical reactions proved that it was amesenchymal tumor of smooth muscle origin with marked polymorphism, without mi-tosis, with a myxoid stroma and with biological aggressivity, and the possibility of localrecurrence. Thus, a second more radical surgical procedure, was performed. In the ex-cised tissue, no residual tumor was found and all lymphnodes were negative.IntroductionVulvar leiomyoma is very rare. Espe-cially rare is pleomorphic or bizarre leio-myoma in the vulvar area. Cases of bi-zarre or pleomorphic leiomyoma of someother localization (kidney, stomach, pros-tate) were still reported, but bizarre vagi-nal leiomyoma1 and vulvar (Bartholin'sgland area) leiomyoma2 were reported inonly few cases. This, usually benign tu-mor, is very hard to differ from leiomyo-sarcoma. The main criterion to find a dif-ference between them is the mitoticactivity, which is less than 3 per 10 highpower field (HPF) in atypical leiomyoma.This tumor has not only bizarre outlook,but can have unpredictable behavior3.The symptoms are often scanty (dys-pareunia and painful swelling in the areaof labia mayor and introitus). The ther-apy is a surgical procedure. Its degree de-pends on histological and clinical esti-mate of tumor behavior.Case ReportA 26-year-old woman, gravida 2, para1, was referred to the Department of Gy-necology and Obstetric at the UniversityHospital »Osijek« for the evaluation of571Received for publication March 24, 2002suspicious paravaginal tumor in distalvaginal third and right vulvar half area.The patient has recognized the tumor indistal vaginal third and right vulvar halfarea six months earlier. In the last fewweeks the swelling started to be morepainful, and caused dyspareunia, becauseof the narrow vaginal introitus. The pa-tient had no history of any earlier dis-eases. Clinically she had a hard tumormass, painful on palpation, located in dis-tal vaginal third and below the right la-bia mayor. Other gynecological and ultra-sound examination of the uterus andovaries, PAP smear and blood tests re-vealed normal findings. Surgical proce-dure was done. By vaginal and lateralperineal incision we got to the tumor,which was partly enucleated. In its cra-nial part the tumor tissue infiltrated thesurrounding tissue, and a sharp excisionof the tumor was done. Large solid tumorwith an uneven surface, 10  7.5 cm bigand two pieces of the tissue, which con-tent parts of the tumor were removed.The cross sectional tumor tissue is soft,solid yellow colored and partly necrotic.During the excision and enucleation wefind out local invasive tumor behavior,which, with concerning on localizationand macroscopic look suggested that itwas a malign tumor of Bartholin gland.By histological examination we found outthat the tumor was solid, made of bun-dles mostly with spindle cells. Spindlecells have round or oval nuclei with big,prominent, eosinophilic nucleoli. Nucleipartly show hyperchromasy in placeswhere there are cells with giant nuclei.Cytoplasm are eosinophilic elongated,with small hell vacuole. In this area cellshave two or more nuclei, with earliermentioned polymorphism (Figure 1).Between the cell bundles there is hya-line degeneration, and areas with plentyof myxoid Alcian positive intercellularsubstance (Figure 2).Vascularization is in shape of manyblood vessels with a thin wall. Mitosiswere not found, although the tumor wasexamined by three independent patholo-gists from three different countries. Thetumor has no capsule, on the marginsgrowth looks infiltrative (Figure 3).By immunohistochemical procedurescarried out in two independent institu-tion for gynecologic pathology proved pos-itive reaction on vimentin, smooth mus-cle actin and desmin, which refer to asmooth muscle origin of the tumor. Cito-keratin MNF 116, EMA, S–100, CD–34and CD–117 are negative.Based on established immunofenotypeof tumor cells and histological and macro-scopic examine, the conclusion is that it isa mesenchymal smooth muscle tumor,with marked polymorphism, without mi-tosis, with myxoid changes in the stromaand with infiltrative margins.572Z. Topolovec et al.: Pleomorphic Vulvar Leiomyoma, Coll. Antropol. 26 (2002) 2: 571–575Fig. 1. Pleomorphic leiomyoma – polymor-phism of cells and nuclei, HE 400  .According to the pathohistologistsopinions, the tumor's size, his outlook andcell pleomorphism, suggest that the tu-mor is biologically aggressive and localrecurrence or eventually metastasis canbe expected. The pathohistologists sug-gestion was to perform a second surgi-cally procedure seven weeks after enucle-ation and excision. This procedure was aresection of the tumor site, ipsilateral in-guinal and femoral lymphadenectomy,besides the parailiacal lymphadenectomyon the right side, by extraperitoneal ac-cess. In the excised material from the tu-mor site area a tubuloalveolar mucousglandula – glandula Bartholin was foundand histologically it was normal. In theinguinal, femoral and parailiacal lymph-nodes and in the material from re-excisedsite of the tumor by histological examina-tion the tumor tissue was not found. Fif-teen months later, the patient had no lo-cal or regional disease recurrence. Thepatient will be followed-up in gynecolo-gic-oncologic out patient department.DiscussionThere is still one case of bizarre leio-myoma in Bartholin glands area reportedin literature2, but this, usually benign tu-mor, sometimes is very difficult to differfrom leiomyosarcoma of the same area3.The appearance and quick growth ofleiomyoma of Bartholin gland's like itwas documented and in literature earlierdescribed, may be connected with a hor-mone replacement therapy4. Our patientdid not receive any hormone replacementtherapy.It is very often a simultaneous devel-opment of leiomyoma of the uterine cor-pus and leiomyoma from the labium ma-573Z. Topolovec et al.: Pleomorphic Vulvar Leiomyoma, Coll. Antropol. 26 (2002) 2: 571–575Fig. 2. Pleomorphic leiomyoma – tumorsarea with myxoid changes, HE 100  .Fig. 3. Pleomorph tumor cells near bloodvessel, outside of the tumor, point out atinvasive growth of tumor, HE 100  .ius5. According to clinical examinationand by ultrasound the same uterus tumorwas excluded.Of three main determinants of the ma-lign neoplasm of mesenchymal origin (>5cm, > 5 mitosis/10 HPF and infiltrativemargins)6, the tumor in our case was 10 7.5 cm with infiltrating margins, but mi-tosis were not found.There are three main histological cri-teria for the diagnosis of leiomyosarcoma:coagulation necrosis of the tumor cells,significant, usually diffuse, nuclear atypiaand frequent mitotic figures (MF), oftenatypical.However, disagreement about the im-portance of particular criteria on biologi-cal behavior of the tumor exist. Now, it isclear that one of the important criteriabeside the frequent mitotic figures andcellular atypia is the coagulation necrosisof the tumor cells, and perhaps the mostimportant particular prognostic factor forbiological behavior of leiomyosarcoma. Inpractice, there is a group of smooth mus-cle tumors that could not be put into truebenign nor into true malignant tumors.They are called smooth muscle tumors ofuncertain malignancy. The recognitioncriteria for this group of tumors definedby J. A. Benda and R. Zaino are:¿ cytological atypia, 2–5 MF/10 HPF;¿ hypercellularity, without atypia, 5–10 MF/10HPF;¿ normocellularity, without atypia,10–15 MF/HPF;¿ infiltrative margins, 5–10 MF/10HPF;¿ atypical mitotic figures and/or coag-ulation necrosis;¿ cells of epitheloid type, 2–5 MF/10HPF;¿ symplastic tumor, 2–5 MF/10 HPF;¿ vascular invasion, 2–5 MF/10 HPF;¿ intravenous leiomyomatosis, 2–5MF/10 HPF;¿ parasitic leiomyoma, 5–9 MF/10HPF3.The presented tumor content cytologi-cal atypia, hypercellularity, giant cellswhich are characteristic for symplastictumors and infiltrative margins, but donot content one of the most important ele-ment – frequent mitotic figures. This isthe main reason why we defined this tu-mor as pleomorphic (atypical) leiomyoma,and not as smooth-muscle tumor of un-certain malignant potential, although itsbiological behavior is more likely to be anuncertain malignant potential tumor.Also by WHO definition, bizarre leio-myoma are the tumors that content giantcells, with pleomorphic nuclei with littleor without mitotic activity7.Myxoid changes in vulvar leiomyomasare common and also related to its ag-gressive behavior and recurrence8. Thepresented tumor shows in hyaline stromaand in some cross-sections myxoid stro-ma.Because of all these elements, this tu-mor is, according to pathologists opinion,estimated as a biologically aggressive tu-mor, so we can expect, because of tumorsstroma myxoidity, infiltrative margins,cellular polymorphism and tumor size, lo-cal recurrence or even metastasis. This iswhy we did more radical surgical proce-dure.R E F E R E N C E S1. BIANKIN, S. A., V. E. O’TOOLE, C. FUNG, P.RUSSELL, Int. J. Gynecol. Pathol., 19 (2000) 186. —2. NERI, A., Y. PELED, D. BRASLAVSKI, Acta. Ob-stet. Gynecol. Scand., 72 (1993) 221. — 3. ILI]-FORKO, J., D. BABI], Uterine tumor of mesenchy-mal origin. In: JUKI], S. (Ed.): Pathology of female574Z. Topolovec et al.: Pleomorphic Vulvar Leiomyoma, Coll. Antropol. 26 (2002) 2: 571–575genital tract. (AGM, Zagreb, 1999). — 4. SIEGLE, J.C., L. J. CARTMELL, Reprod. Med., 40 (1995) 147. —5. ZLATKOV, V., N. DOGANOV, V. MACAVEERA,Rev. Fr. Gynecol. Obstet., 84 (1989) 351. — 6. TAVAS-SOLLI, F. A., J. H. NORRIS, Obstet. Gynecol., 53(1979) 213. — 7. SCULLY, R. E., T. A. BONFIGLIO,R. J. KURMAN, S. G. SILVERBERG, E. J. WILKIN-SON: World Health Organization: International his-tological classification of tumors. 2nd ed. (Springer-Verlag, Berlin, 1994). — 8. NEMOTO, T., M. SHINO-DA, K. KOMATSUZAKI, T. HARA, M. KOJMA, T.OGIHARA, Pathol. Int., 44 (1994) 454.Z. TopolovecDepartment for Ginecology and Obstetrics, University Hospital »Osijek«, Huttlerova 4,31000 Osijek, CroatiaPLEOMORFNI LEOMIOM VULVE S LOKALNO INVAZIVNIMPONA[ANJEMS A @ E T A KPrikaz pleomorfnog leomioma u podru~ju Bartolinijeve `lijezde u dvadeset{estogo-di{nje `ene. Nakon dijagnosti~kog postupka u~injena je primarna ekscizija. Odstra-njen je veliki solidni tumor, 10  7,5 cm. Tumor je pokazivao lokalno invazivno pona{a-nje, koje je zbog lokalizacije i izgleda tumora sugeriralo da se radi o malignom tumoruBartolinijeve `lijezde. Na osnovi patohistolo{kih i imunohistokemijskih pretraga zak-lju~eno je da se radi o mezenhimalnom tumoru porijeklom od glatkog mi{i}ja, s izra`e-nim polimorfizmom, bez mitoza, s miksoidnom stromom i biolo{kom agresivno{}u i mo-gu}no{}u nastanka lokalnog recidiva. Zbog toga je u~injen jo{ jedan kirur{ki zahvatradikalniji od prethodnog. U ekscidiranom tkivu nije na|en rezidualni tumor i svi lim-fni ~vorovi su bili negativni.575Z. Topolovec et al.: Pleomorphic Vulvar Leiomyoma, Coll. Antropol. 26 (2002) 2: 571–575

Pleomorphic Vulvar Leiomyoma with Local Invasive Behavior

Pleomorphic Vulvar Leiomyoma with Local Invasive Behavior

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