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Hindawi Publishing Corporation Case Reports in Oncological Medicine Volume 2014, Article ID 910852, 4 pages http://dx.doi.org/10.1155/2014/910852 Case Report Advanced Ovarian Dysgerminoma Infiltrating Both Ovaries and Uterus in a 7-Year-Old Girl 1 1 1 1 1 Nexhmi Hyseni, Sadik Llullaku, Hysni Jashari, Kaltrina Zahiti, Fjolla Hyseni, 1,2 1,2 3 3 Fisnik Kurshumliu, Lumturije Luci, Fehim Muqolli, and Antigona Hasani Department of Pediatric Surgery, University Clinical Centre, Laxha e Spitalit PN, 10000 Pristina, Kosovo University Clinical Centre, Pathology Institute, 10000 Pristina, Kosovo Department of Anesthesiology and Reanimation, University Clinical Centre, 10000 Pristina, Kosovo Correspondence should be addressed to Nexhmi Hyseni; nexhmi email@example.com Received 28 November 2013; Accepted 16 January 2014; Published 23 February 2014 Academic Editors: C. Gennatas and L. Lu Copyright © 2014 Nexhmi Hyseni et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Introduction. Ovarian dysgerminoma is a rare malignant ovarian germ cell tumor with its peak incidence in young women. Abdominal pain, abdominal distention, and the presence of a palpable mass are common symptoms at presentation. Depending on the FIGO stage at presentation the prognosis of dysgerminomas aer ft surgical treatment, adjuvant chemotherapy, and radiotherapy is promising. Case Presentation. A 7-year-old girl was presented at our clinic with abdominal pain in all abdominal quadrants. Later the pain localized in the region of her right ovary. CT scan revealed a massive formation which was connected to her right ovary. Conclusion. Although malignant ovarian germ cell tumours are rare in children, physicians must always consider the possibility of MOGT-occurrences. eTh clinical symptoms might not be specific: abdominal pain, abdominal distention, nausea, and vomiting. In order to make a correct diagnosis the patients should undergo a complete clinical examination including radiological scans. Initial management is frequently surgery, followed by adjuvant chemotherapy and radiotherapy. Although disgerminoma is malignant tumor, the prognosis is promising. 1. Introduction 2. Case Presentation Dysgerminoma is a rare malignant ovarian germ cell tumor We report thecaseofa7-year-old Albanian girl whowas (MOGT) which is highly malignant and has its peak inci- presented with abdominal pain and a palpable mass in the dence in young women. Approximately one-third of all region of her right ovary. Initially the girl complained about dysgerminomasshowKITmutationsandtheseareassociated abdominal pain in all quadrants of the abdomen. Later the with advanced stage at presentation . Clinically the patients pain was localized on the right side. According to her mother present with abdominal pain, abdominal distention, and the pregnancy with her daughter was uncomplicated; the presence of a palpable mass, reduced appetite, vomiting, and girl had never been ill before, had no allergies, and was nausea as well as ovarian torsion [2, 3]. Conservative surgery, physically healthy looking except for light skin paleness. eTh postoperative chemotherapy, and postoperative radiother- laboratory results were normal except for haemoglobin (11, apy are effective therapeutic options. Fertility-preservation 8 g/dL) and haematocrit (34,6%). eTh radiological CT scan of surgery is often possible [ 3]and theoverall survival is 92.4% theabdomen andpelvisrevealedamassive, solid, andclearly . Preoperative elevation of tumor markers is significantly bordered formation with the dimensions12×8×15 cm. eTh related to poor prognosis for progression-free survival (PFS) suspicious formation showed a connection to the right ovary . Dysgerminomas reveal in 28% of cases presence of lymph that was hyperdense and had an inhomogeneous appearance. node metastasis, which is significantly associated with lower Some hypodense tissue within the tumour mass was identi- 5-year survival (82.8%) . Older patients were more likely fied as probably “fatty tissue” (Figures 1(a) and 1(b)). After to be diagnosedatanadvancedstage . an additional examination with intravenous contrast medium 2 Case Reports in Oncological Medicine (a) (a) (b) (b) Figure 2: After an additional examination with intravenous contrast Figure 1: CT scan of the abdomen and pelvis revealed a massive, medium the tumour mass showed a raised vascularization within solid, and clearly bordered formation with the dimensions12×8× the tumour. 15 cm. eTh suspicious formation showed a connection to the right ovary that was hyperdense and had an inhomogeneous appearance. Some hypodense tissue within the tumour mass was identified as probably “fatty tissue.” the tumour mass showed a raised vascularization within the tumor (Figures 2(a) and 2(b)). The radiologist assumed an embryological origin of the tumour mass and suspected an “Immature teratoma of the right ovary with probable adhesion to the surrounding area.” eTh transversal infraumbilical laparotomy revealed a huge formation which involved both ovaries and the uterus. Aeft r intrasurgically consulting the gynaecologist the sur- Figure 3: Uniform tumor cells arranged in nests, separated by geons made the indication for a total hysterectomy with bilat- delicate b fi rous stroma rich in lymphocytes ( ×5; H&E stain). eral salpingo-oophorectomy. Aeft r extirpating the tumour mass suspicious lymph nodes were also removed and the sur- gical preparation was sent to the pathologist. eTh histopatho- logical examination revealed a dysgerminoma with FIGO central or slightly excentric nuclei with a vesicular chro- stage IIIc. The tumor mass was 692 g and measured 17× matin pattern and prominent nucleoli (Figures 3 and 5). 11 × 8.5 cm. On cut surface the tumor had multinodular The tumor islands were separated by collagenous stroma appearance with conu fl ent areas of necrosis and hemor- densely infiltrated by lymphocytes ( Figure 4). The tumor cells rhage. Histological examination revealed infiltrating islands were negative for CD45 (LCA) and S-100. PLAP (placental of uniform tumor cells with ample clear cytoplasm with alkaline phosphatase) was not available at our institution; Case Reports in Oncological Medicine 3 Figure 4: Medium-sized tumor cells with eosinophilic cytoplasm Figure 6: Lymph node metastasis with area of the tumor cells and central nuclei with vesicular chromatin (×10; H&E stain). partially replacing the lymph node structure (×2.5; H&E stain). uterus and one ovary without putting the child into risk of a further growing of the tumour and probably leading to metastasis into other organs. Similar to our case Nishio et al. made the indication for radical surgery at stages III and IV . In contrast to this Vicus et al. showed that fertility-sparing surgery in women with pure ovarian dysgerminoma led in 8 of 65 cases to 12 pregnancies and 12 live births. Drozynska ´ et al. published differences of dysgerminomas in young children (younger than 10 years) and older chil- dren (between 11 and 18 years) regarding histology, primary localization, and biochemical markers. eTh marker AFP was Figure 5: Higher magnification showing focal prominent nucleoli higher in younger patients (76% versus 44%), whereas 𝛽 - of the tumor cells (×20; H&E stain). HCG levels were increased in older patients (40% versus 9%). Compared to these n fi dings our case is rare regarding the age and the occurrence of a FIGO stage IIIc. however, the classical histological n fi dings in routine stains Lymph node metastasis is present in 28% of dysger- (H&E), lack of expression of hematopoietic markers, and minomas and is significantly associated with poor survival clinical findings were sufficient evidence of “seminoma- . In order to evaluate the prognosis and nd fi adequate like” germ-cell nature of this tumor, namely, dysgerminoma. therapy options lymphadenectomy is indicated. The result of Additionally, one lymph node was positive for metastatic the lymphadenectomy in our case was positive and showed tumor deposits (Figure 6). tumour cells within the lymph node which is according to Kumaretal. apredictor forpoorsurvival. 3. Discussion Despite these tumours being rare an overall survival of 97% can be achieved with conservative surgery and AreviewofAndres ´ et al. revealed the percentage of dys- platinum-based chemotherapy . Adjuvant chemotherapy germinomas (15%) in solid tumours in childhood . The in combination with initial surgery shows promising results mean age at diagnosis is14+2.7 years and the majority of concerning outcome and fertility . Quero-Hernandez ´ the patients are postpubertal  in contrast to our patient, et al. used mean 4 chemotherapy cycles including cisplatin, whowas 7years oldand didnot show anysigns of pubertyat etoposide, and bleomycin . We used also 4 chemotherapy presentation. Vicus et al. published data of the occurrence of cycles (including cisplatin, etoposide, and bleomycin) and pureovariandysgerminomas:72.3%stageI,4.6%stageII,and radiotherapy. She is still in complete remission approximately 21.5% stage III disease. The initial treatment was surgery with oneyearaeft rpresentation. We believethatour report on the 72.2% of the patients undergoing unilateral oophorectomy challenges of diagnosis and treatment in this case can help and 21.5% bilateral oophorectomy +/− hysterectomy . The clinicians to better understand and manage these pathologies. indication for bilateral oophorectomy and hysterectomy in our case was intrasurgically made with a gynaecologist. The reason therefore was a macroscopically visible infiltration of 4. Conclusion both ovaries and the uterus which coalesce to a huge tumour mass. Although fertility-sparing surgery is the main aim and Our case shows that inconspicuous symptoms like abdominal is possible in 70% of cases , the indication for bilateral pain, abdominal distention, nausea, and vomiting might oophorectomy and hysterectomy in our case was obvious. occur due to a malignant ovarian germ cell tumour. Although We couldnot seeany possibilitiesofsparing theinfiltrated dysgerminomas rarely occur in childhood, the physician 4 Case Reports in Oncological Medicine should not exclude the possibility of dysgerminomas appear- a single institutional experience,” Gynecologic Oncology,vol.117, no. 1, pp. 23–26, 2010. ing in this age. er Th efore a complete clinical examination with radiological scans is necessary in order not to miss growing  S. Nishio, K. Ushijima, A. Fukui et al., “Fertility-preserving treatment for patients with malignant germ cell tumors of the malignant formations. In cooperation with gynaecologists ovary,” Journal of Obstetrics and Gynaecology Research,vol.32, and paediatricians the best individual therapy option should no. 4, pp. 416–421, 2006. be found. Adjuvant chemotherapy and radiotherapy show  A. Quero-Hernandez, ´ R. Estrada-Correa, H. Tenorio-Rod- favourable outcome and future genetic examinations will r´ıguez, and R. M. Alvarez-Sol´ıs, “Malignant germ cell ovarian hopefully reveal new biological and genetic targets to improve tumors: clinical characteristics, treatment and outcome,” Ciru- overall survival and fertility. gia Y Cirujanos,vol.75, no.2,pp. 81–85, 2007. Abbreviations MOGT: Malignant ovarian germ cell tumor PFS: Progression-free survival. Conflict of Interests eTh authors declare that they have no conflict of interests. Authors’ Contribution Nexhmi Hyseni was responsible for conception and editing of paper. Sadik Llullaku, Hysni Jashari, Kaltrina Zahiti, and Fjolla Hyseni were responsible for literature search and preparation of paper. Other authors did surgery of patients andhelpedinwriting thepaper.All authorsreadand approved the n fi al paper. References  L. Cheng, L. M. Roth, S. Zhang et al., “KIT gene mutation and amplification in dysgerminoma of the ovary,” Cancer,vol.117, no. 10, pp. 2096–2103, 2011.  S.-M. Chu, Y.-C. Ming, H.-C. Chao et al., “Ovarian tumors in the pediatric age group: 37 cases treated over an 8-year period,” Chang Gung Medical Journal,vol.33, no.2,pp. 152–156, 2010.  D. Biswajit, C. N. Patil, and T. G. Sagar, “Clinical presentation and outcome of pediatric ovarian germ cell tumor: a study of 40 patients,” Journal of Pediatric Hematology/Oncology,vol.32, no. 2, pp. e54–e56, 2010.  S. Tangjitgamol, J. Hanprasertpong, S. Manusirivithaya, V. Wootipoom, T. Thavaramara, and R. Buhachat, “Malignant ovarian germ cell tumors: clinico-pathological presentation and survival outcomes,” Acta Obstetricia et Gynecologica Scandinav- ica, vol. 89, no. 2, pp. 182–189, 2010.  S.Kumar,J.P.Shah, C. S. Bryant et al., “ep Th revalenceand prognostic impact of lymph node metastasis in malignant germ cell tumors of the ovary,” Gynecologic Oncology,vol.110,no. 2, pp.125–132,2008.  E. Drozynska, ´ K. Połczyn´ska,S.Popadiuketal.,“Characteristics of extracranial malignant germ cell tumours in two age groups of children (0–10 and 10–18 years). Multicentre experiences,” Medycyna Wieku Rozwojowego,vol.15, no.1,pp. 16–24, 2011.  M.M.Andre´s,E.Costa,A.Can˜ete,L.Moreno, andV.Castel, “Solid ovarian tumours in childhood: a 35-year review in a single institution,” Clinical and Translational Oncology,vol.12, no. 4, pp. 287–291, 2010.  D.Vicus,M.E.Beiner, S. Klachook,L.W.Le, S. Laframboise, and H. 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Published: Feb 23, 2014
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