DM Gershenson

DT1 Trinh

FK Lim

HM Brammer 3rd

JB de Jolinière

JH Low

JS Berek

Kanwardeep Kawatra

KKM Kwoi

Lajya Devi Goyal

M Kawai

M Koshy

M Kotb Abbass

N Murugaesu

RJ Kurman

Sharanjit Kaur

Journal of Ovarian Research

English

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Goyal et al. Journal of Ovarian Research 2014, 7:91http://www.ovarianresearch.com/content/7/1/91CASE REPORT Open AccessMalignant mixed germ cell tumour of ovary- anunusual combination and review of literatureLajya Devi Goyal1, Sharanjit Kaur2* and Kanwardeep Kawatra3AbstractMixed germ cell tumours of the ovary are malignant neoplasms of the ovary comprising of two or more types of germcell components. Most of the malignant mixed germ cell tumours consists of dysgerminoma accompanied byendodermal sinus tumours, immature teratoma or choriocarcinoma. There are only few case reports of mixed germ celltumours with different combinations of malignant components.We report a very rare case of mixed germ cell tumours consisted of malignant components of endodermal sinus tumour,emryonal carcinoma, and benign component of teratomatuos and trophoblastic differentiation. This is the first case reportin the literature with both benign and malignant component of type described to best of our knowledge.Patient was an 18 year old girl, who presented with pain abdomen, abdominal mass and irregular bleeding. Ultrasoundand CT scan showed a huge mass with solid and cystic component. Tumour markers i.e alpha feto- protein (AFP),human chorionic gonadotropin (hCG), lactate dehydrogenate (LDH) and Ca-125 were raised. We performed fertilitysparing surgery by preserving one ovary, tube and uterus.Conclusion: Malingnant mixed germ cell tumours of ovary are highly aggressive neoplasm and early intervention andfertility sparing surgery is required for any adolescent girl presenting with rapidly enlarging pelvic mass.Keywords: Malignant mixed germ cell tumour, Endodermal sinus tumour, Teratoma, Embryonal cell carcinomaBackgroundOvarian germ cell tumours arise from primordial germ cellderived from the embryonal gonads. Malignant germ celltumour comprise less than 5% of all ovarian neoplasms.The incidence range from 1 to 6% in west and from 8 to19% in Asia [1]. The most common form of malignantgerm cell tumours are dysgerminoma (80%), endodermalsinus tumour (EST) (70%), and immature teratoma (53%)reported in a series [2]. Embryonal carcinoma, choriocar-cinoma and polyembryoma are very rare type of germ celltumour. Malignant mixed germ cell tumour is a type oftumour that consists of two or more malignant germcell component. Most common combination reported isdysgerminoma and EST [2] and rarer component includeembryonal carcinoma and immature terotoma [3,4].Tumour markers such as AFP, hCG and LDH contribute tothe diagnosis, prognosis and follow-up of the disease. Wereport a case of very rare mixed germ cell tumour consisted* Correspondence: jitsharan28@gmail.com2Department of Obstetrics and Gynaecology Nursing, University College ofNursing, Baba Farid University of Health Sciences, Faridkot 151203, Punjab,IndiaFull list of author information is available at the end of the article© 2014 Goyal et al.; licensee BioMed Central LCommons Attribution License (http://creativecreproduction in any medium, provided the orDedication waiver (http://creativecommons.orunless otherwise stated.of both malignant and benign component i:e EST, embry-onal carcinoma, mature teratomatuos components andtrophoblastic differentiation. There are only few case re-ports of mixed germ cell tumour with different combina-tions of malignant components but this is the first casereport in the literature with both benign and malignantcomponent of type described to the best of our knowledge.Case reportAn 18 year old girl presented with chief complaint of ab-dominal mass and pain of one month duration. She alsocomplained of fever and poor appetite. Her menstrualhistory revealed that she had experienced menarche atthe age of 12 and her cycles were regular with normalflow in the past but had irregular bleeding in last two cy-cles. Her physical examination revealed severe pallorand pedal edema. Her vital signs showed tachycardia(pulse rate 120/min), blood pressure 100/70 mm Hg andrespiratory rate 18/min. On abdominal examination ahuge mass up to the level of xiphisternum could be pal-pated. There was no guarding or rebound tenderness.td. This is an Open Access article distributed under the terms of the Creativeommons.org/licenses/by/4.0), which permits unrestricted use, distribution, andiginal work is properly credited. The Creative Commons Public Domaing/publicdomain/zero/1.0/) applies to the data made available in this article,Goyal et al. Journal of Ovarian Research 2014, 7:91 Page 2 of 4http://www.ovarianresearch.com/content/7/1/91Investigations revealed haemoglobin 4.9 gm/dl, totalcount 7700, platelet count 437 × 103 and on peripheralblood film there was microcytic hypochromic type ofanemia. Serum biochemistry was normal. USG revealeda huge solid cystic mass occupying the whole abdomen.Right ovary was not separately visualised from the massbut left ovary was normal looking. There was no evidenceof free fluid in abdomen. CT scan revealed no retroperi-toneal lymphadenopathy. Tumour markers levels wereCA-125 -259.3 IU/ml, Carcinoembroyonic antigen (CEA)4.3 ng/ml alpha feto protein (AFP) 489.9 ng/ml, humanchorionic gonadotropic levels (hCG) 3751.5 IU/ml andLactate dehydrogenate (LDH) 3600 IU/ml.Intraoperatively there was a huge mass arising fromright sided ovary with intact capsule. There was no freefluid in the abdominal cavity and peritoneal washingswere taken. Abdominal cavity was explored and therewas no evidence of malignant disease elsewhere. Left-sided ovary and uterus was normal looking. Tumour wasremoved and biopsy was taken from left ovary and infraco-lic omentectomy and pelvic and paraaortic lymhphadenect-omy was done for staging of the tumour. Frozen sectioncould not be done as the machine was out of order.On gross examination (Figure 1) tumour measured25 × 24 × 11 cm and weighed 4800 gms. External surfacewas smooth and bosselated with an intact capsule. Serialcut sections revealed a tumour with solid and cystic var-iegated cut surface showing dark-brown, grey-brown,myxoid and necrotic areas. Microscopy showed a germcell tumour of variable composition. Predominant com-ponent was that of yolk sac tumour showing reticular(Figure 2a) and microcystic (Figure 2b) areas withSchiller-Duval bodies (Figure 2c). Several multinucleatedtrophoblastic giant cells were also present (Figure 2d).Along with this, there were mature teratomatous com-ponents in the form of squamous islands (Figure 3a), cys-tic spaces lined by mucinous epithelium (Figure 3c) andFigure 1 Photograph showing gross tumour.hepatocytes (Figure 3b). Some areas also showed embry-onal carcinoma (Figure 3d). No extra capsular invasionwas seen. Lymph nodes and omentum were also free oftumour.Final histopathological diagnosis was: Mixed germ celltumour with components of yolk sac tumour, matureteratoma, embryonal carcinoma and trophoblastic giantcells.Patient was discharged in satisfactory condition andreceived four cycles of bleomycin, etoposide and cis-platin combination chemotherapy. Patient is on followup and on 3 and 6 month follow up patient did notshow any evidence of recurrence.DiscussionRare combination of uncommon germ cell components,huge size and capsule being intact, very high levels oftumour markers makes this case unusual.Most mixed germ cell tumour consist of combinationof dysgerminoma with endodermal sinus tumour ac-counting for one-third of the cases [2]. Other combin-ation include choriocarcinoma and immature teratomain decreasing order of frequency. Cases of mixed germcell tumour of endodermal sinus tumour and emryonalcarcinoma are rare and tumours with component of be-nign variety of trophoblastic and mature teratomatouscombination are exceptional. Recently a rare combin-ation of mixed germ cell and granulose cell tumour hasbeen reported in the literature [5].The average age of presentation of germ cell tumour is13.8 years (4–27 years) [6]. Most common clinicalpresentation includes abdominal mass with or without ab-dominal pain or fever. Embryonal carcinoma may secreteestrogen and can present with precocious puberty or ir-regular vaginal bleeding [7]. The gross appearance variesaccording to the individual constituents of the tumour. Im-aging modalities can be used to establish the diagnosis butdifferent types of tumour may show overlapping featuresand the definitive diagnosis is made by histopathology. Inthis case very high levels of tumour markers AFP and hCGcould be attributed to EST and embyonal carcinoma, butwe could not find any evidence of dysgerminoma compo-nent on histopathology as LDH was also highly raised. Im-munohistochemistry is a wide-used biological techniquethat help in the diagnosis and development of new man-agement modalities [8,9]. Trinh DT et al. described theutility of CD117, CD133, SALL4, OCT4, TCL1 andglypican-3 in malignant germ cell tumors of the ovary [9].Ovarian endodermal sinus tumours are highly aggres-sive but with surgery and combination chemotherapy,the five year survival with stage 1 tumours and more ad-vanced disease are 92% and 29% to 44% respectively[10]. Embryonal carcinoma of ovary is usually presentalong with other components. Survival in embryonalFigure 2 Yolk sac tumour. Yolk sac tumour showing reticular (a) and microcystic (b) areas with Schiller-Duval bodies (c). showingmultinucleatedtrophoblastic giant cells (d).Figure 3 Mixed germ cell tumour. Showing mature teratomatous components in the form of squamous islands (a), cystic spaces lined bymucinous epithelium (c) and hepatocytes (b). Some areas also showed embryonal carcinoma (d).Goyal et al. Journal of Ovarian Research 2014, 7:91 Page 3 of 4http://www.ovarianresearch.com/content/7/1/91Goyal et al. Journal of Ovarian Research 2014, 7:91 Page 4 of 4http://www.ovarianresearch.com/content/7/1/91carcinoma the first reported series of 15 patients was39%, with 50% of stage I patients being disease free at3.75 to 15 years post-surgery and chemotherapy [11].More recent data illustrate the improved survival achiev-able with conservative surgery and combination chemo-therapy, with survival figures of 98% and 94% for earlyand advanced stage tumours [12]. Although standardprognostic system for ovarian germ cell tumour is cur-rently unavailable but poor prognostic indicators includelarge size, unfavourable histological type, and advancedstage at presentation. Elevation of both AFP and hCGlevels is a strong predictor of poor survival shown in arecent analysis [13]. Unilateral oophorectomy and surgi-cal staging is the minimal surgery in ovarian germ celltumour if conservation of fertility is of concern. Preser-vation of fertility is recommended even in metastaticdisease as these tumours are highly sensitive to chemo-therapy. Hysterectomy and bilateral salpingioophorect-omy do not change the outcome [8]. All patients exceptthose with FIGO stage 1a require combination chemo-therapy. Usually 3 to 4 course of combination chemo-therapy containing bleomycin, etoposide and cisplatin(BEP) or vincristine, dactinomycin, and cylophospha-mide (VAC) are commonly used. The serum markers,may become negative during chemotherapy, but thismay reflect regression of only a particular component ofthe mixed lesion. Therefore, in these patients a second-look laparotomy may be indicated if there is residual dis-ease following chemotherapy.ConclusionMalingnant mixed germ cell tumours of ovary are highlyaggressive neoplasm and early intervention and fertilitysparing surgery is required for any adolescent girl pre-senting with rapidly enlarging pelvic mass.ConsentWritten informed consent was obtained from the patientfor the publication of this report and any accompanyingimages.Competing interestsThe authors declare that they have no competing interests.Authors’ contributionsLD who dealt with the case and drafted the manuscript. KK Assisted in Histopathology Report of the sample and SK carried out all the documentary andarticle work out. All authors read and approved the final manuscript.Author details1Department of Obstetrics and Gynaecology, Guru Gobind Singh MedicalCollege, Faridkot 151203, Punjab, India. 2Department of Obstetrics andGynaecology Nursing, University College of Nursing, Baba Farid University ofHealth Sciences, Faridkot 151203, Punjab, India. 3Department of Pathology,Christian Medical College, Ludhiana 141001, Punjab, India.Received: 12 May 2014 Accepted: 21 September 2014References1. Lim FK, Chanrachakul B, Chong SM, Ratnam SS: Malignant ovarian germcell tumours: experience in the National University Hospital ofSingapore. Ann Acad Med Singapore 1998, 27(5):657–661.2. Gershenson DM, Del Junco G, Copeland LJ, Ruthledge FN: Mixed germ celltumors of the ovary. Obstet Gynecol 1984, 64(2):200–206.3. Koshy M, MBBS MR, Vijayananthan A, MBBS MR, Vadiveloo V: Malignantovarian mixed germ cell tumour: a rare combination. Biomed ImagingInterv J 2005, 1(2):e10.4. Kwoi KKM, Loke TKL, Hui JPK, Lai MHY, Chan JCS: Malignant mixed germ celltumour of the ovary in 10 year old girl. J HK Coll Radiol 2008, 11:92–95.5. Kotb Abbass M, Dalia Ahmed E, Hekma Saad F, Hanaa A-L, Mohamed A-K:Precocious puberty secondary to a mixed germ cell-sex cord-stromaltumor associated with an ovarian yolk sac tumor: a case report. J MedCase Rep 2012, 6:162.6. Brammer HM 3rd, Buck JL, Hayes WS, Sheth S, Tavassoli FA: From thearchives of the AFIP. Malignant germ cell tumors of theovary:radiologic-pathologic correlation. Radiographics 1990, 10:715–724.7. Berek JS, Hacker N, Berek JS, Hacker NF: Practical Gynecologic Oncology.Fifthth edition. Philadelphia: Williams and Wilkins; 2007.8. de Jolinière JB, Ben Ali N, Fadhlaoui A, Dubuisson JB, Guillou L, Sutter A,Betticher D, Hoogewoud HM, Feki A: Two case reports of a malignantgerm cell tumor of ovary and a granulosa cell tumor: interest of tumoralimmunochemistry in the identification and management. Front. Oncol2014. doi:10.3389/fonc.2014.00097.9. Trinh DT1, Shibata K, Hirosawa T, Umezu T, Mizuno M, Kajiyama H, KikkawaF: Diagnostic utility of CD117, CD133, SALL4, OCT4, TCL1 and glypican-3in malignant germ celltumors of the ovary. J Obstet Gynaecol Res 2012,38(5):841–848.10. Kawai M, Kano T, Furuhashi Y, Misawa T, Nakashima N, Hattori S: Prognosticfactors in yolk sac tumours of ovary. A clinicopathological analysisof 29cases. Cacer 1991, 67:184–192.11. Kurman RJ, Norris HJ: Embryonal carcinoma of the ovary. Aclinicopathological entity distinct from endodermal sinus tumourresembling embryonal carcinoma of the adult testis. Cancer 1976,38:2420–2433.12. Low JH, Perrin LC, Crandon AJ, Hacker NF: Conservative surgery topreserve ovarian function in patients with malignant ovarian germ celltumours. A review of 74 cases. Cancer 2000, 89:391–398.13. Murugaesu N, Schmid P, Dancey G, Agarwal R, Holden L, McNeish I, SavageMP, Newlands ES, Rustin Gordon JS, Seckl JM: Malignant ovarian germ celltumors: identification of novel prognostic markers and long-term outcomeafter multimodality treatment. J Clin Oncol 2006, 24:4862–4866.doi:10.1186/s13048-014-0091-5Cite this article as: Goyal et al.: Malignant mixed germ cell tumour ofovary- an unusual combination and review of literature. Journal of OvarianResearch 2014 7:91.Submit your next manuscript to BioMed Centraland take full advantage of: • Convenient online submission• Thorough peer review• No space constraints or color ﬁgure charges• Immediate publication on acceptance• Inclusion in PubMed, CAS, Scopus and Google Scholar• Research which is freely available for redistributionSubmit your manuscript at www.biomedcentral.com/submit

Malignant mixed germ cell tumour of ovary- an unusual combination and review of literature

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Malignant mixed germ cell tumour of ovary- an unusual combination and review of literature

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