DOCSLIB.ORG

Management of Bilateral Malignant Ovarian Germ Cell Tumors: Experience of a Single Institute

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Management of Bilateral Malignant Ovarian Germ Cell Tumors: Experience of a Single Institute MOLECULAR AND CLINICAL ONCOLOGY 5: 383-387, 2016 Management of bilateral malignant ovarian germ cell tumors: Experience of a single institute TING ZHAO1*, YAN LIU1*, HONGYUAN JIANG1, HAO ZHANG2 and YUAN LU1 Departments of 1Gynecology and 2Pathology, Obstetrics and Gynecology Hospital, Fudan University, Shanghai 200011, P.R. China Received September 30, 2015; Accepted April 25, 2016 DOI: 10.3892/mco.2016.915 Abstract. Bilateral malignant ovarian germ cell Introduction tumors (MOGCTs) are rare. Determination of the optimal treatment modalities is crucial, as these malignancies mainly Malignant ovarian germ cell tumors (MOGCTs) are rare, affect girls and young women who may wish to preserve accounting for ~5% of all ovarian malignancies (1). These their fertility. In order to review the prevalence, clinical tumors mainly affect girls and young women and are characteristics, treatment and outcome of bilateral MOGCTs, usually diagnosed at early stages. Bilateral involvement in we performed a retrospective review of patients who were MOGCT patients is very rare, with a reported prevalence of diagnosed with bilateral MOGCTs and underwent primary 4.3-6.9% (2,3). It is crucial to carefully consider the treat- surgery at the Obstetrics and Gynecology Hospital of Fudan ment modalities, as the affected patients are usually young University (Shanghai, China) between January, 2001 and and strongly wish to preserve their fertility. Due to the high December, 2014. Of the 130 patients investigated, 8 were diag- sensitivity of this type of tumor to chemotherapy, the National nosed with bilateral disease, most of whom were International Comprehensive Cancer Network of the United States has Federation of Gynecology and Obstetrics stage I. There was unequivocally suggested in their guidelines (version 2.2015) no significant difference in overall and disease-free survival that fertility-sparing surgery should be considered, even for between patients with unilateral and those with bilateral patients with advanced-stage disease (4). However, for cases disease. Cases with dysgerminoma, dysgerminoma coexisting with bilateral involvement, preserving fertility may be associ- with gonadoblastoma, yolk sac tumor and ovarian primary ated with increased risk, as cystectomy increases the possibility choriocarcinoma were included in this study. Fertility was of residual lesions. Due to the rarity of these tumors, published spared in 2 patients (1 with dysgerminoma and 1 with ovarian related articles in the English literature are sparse. An Italian primary choriocarcinoma). The patient with ovarian choriocar- study assessing multicenter data over 20 years aimed to cinoma experienced relapse and was finally salvaged by radical investigate this subject, but only found 8 patients with bilateral surgery and adjuvant chemotherapy. According to our results involvement, in 4 of whom fertility was preserved (5). and the published data, patients affected by bilateral MOGCTs The histological heterogeneity of MOGCTs adds to the have a satisfactory prognosis. The treatment modalities largely complexity of their management. MOGCTs may be divided depend on the histological type of the tumor. Fertility-sparing into dysgerminomas and non-dysgerminomas, of which the surgery may be safe for patients affected by dysgerminoma, most common types are yolk sac tumor, immature teratoma but should be considered with caution in patients with ovarian and mixed germ cell tumor. Embryonal carcinoma, non-gesta- primary choriocarcinoma. tional choriocarcinoma and polyembryoma are rare (4). The grade of malignancy differs between various histological types. The reported 5-year survival rates are also different: For bilateral dysgerminoma, immature terotoma and mixed germ cell tumor, the reported survival rates were 96, 94 and 87%, respectively (2). Due to the scarcity of bilateral MOGCTs and the varied histological types, it may be difficult to reach a conclusion on Correspondence to: Dr Yuan Lu, Department of Gynecology, whether conservative surgery (cystectomy) increases the risks Obstetrics and Gynecology Hospital, Fudan University, 419 Fangxie in these patients. The Obstetrics and Gynecology Hospital of Road, Shanghai 200011, P.R. China Fudan University (Shanghai, China) is one of China's biggest E-mail: [email protected] tertiary referral centers. The aim of this study was to retro- *Contributed equally spectively analyze the prevalence, clinical characteristics, management and outcome of patients affected by bilateral Key words: malignant ovarian germ cell tumors, bilateral, MOGCTs in this hospital, in order to accumulate more infor- management mation on this subject. We also conducted a review of the available literature to help elucidate this issue. 384 ZHAO et al: MANAGEMENT OF BILATERAL OVARIAN GERM CELL TUMORS Patients and methods and the remaining 2 patients of absence of menarche at the age of 17 years. One patient also complained of frequent urination Patient selection criteria. Patients meeting the following as an associated symptom. All the patients were nulliparous. criteria were included in the study: i) Histologically proven MOGCT; ii) primary surgery performed at the Obstetrics Diagnosis. Two patients presented with bilateral congenital and Gynecology Hospital of Fudan University between gonadal dysgenesis. The mass in the remaining 6 patients January, 2001 and December, 2014. A total of 130 patients was sized 4.7-30 cm. The tumor size of the bilateral ovaries fitting the requirements were identified, of whom 8 exhibited differed significantly in the same patient, with only one side bilateral involvement. Patient data, including age at diagnosis, presenting with an obviously enlarged or giant ovary, while presenting complaint, ultrasonography (USG) findings, serum the other ovary was only mildly or moderately enlarged. One tumor markers, surgical details, stage and histological type of patient was diagnosed with FIGO stage ⅢC disease, whereas tumor, adjuvant chemotherapy and follow-up data, including the remaining patients were all FIGO stage IB or IC. Four recurrence or death, were retrospectively collected from the patients were diagnosed with dysgerminoma, 2 with dysger- hospital medical records. The study protocol was approved minoma coexisting with gonadoblastoma, and the remaining by the Institutional Review Board of the Obstetrics and 2 with yolk sac tumor or ovarian primary choriocarcinoma. Gynecology Hospital of Fudan University. Written informed consent was acquired from the patients. Treatment. Laparotomy was performed in 5 and laparoscopy in 3 patients. Four patients underwent complete staging Histological classification and staging.The histological diag- procedures. Fertility was spared in 2 patients (details listed nosis was based on the International Classification of Diseases in Table I). All the patients received postoperative chemo- for Oncology, 3rd edition, of the World Health Organization (6). therapy, apart from 1 patient diagnosed with dysgerminoma Staging was performed according to the International coexisting with gonadoblastoma. The chemotherapy regimens Federation of Gynecology and Obstetrics (FIGO) classifica- were bleomycin, etoposide and cisplatin (BEP) in 4 patients; tion of ovarian tumors (7). The complete staging procedure cisplatin, vincristine and bleomycin (PVB) in 2 patients included the following steps: i) Collection of ascitic fluid or and carboplatin, dactinomycin and 5-fluorouracil (CDF) in washings of the peritoneal cavity; ii) careful examination of the 1 patient. The number of cycles ranged from 1 to 8. peritoneum, biopsy and excision of any nodules; iii) omentec- tomy; and iv) sampling or excision of the pelvic lymph nodes. Follow-up. During the median follow-up of 83 months Fertility-sparing surgery is defined as the preservation of the (range, 11-429 months), all the patients remained alive. Only uterus and at least part of a unilateral ovary, whereas radical 1 patient with bilateral ovarian primary choriocarcinoma surgery is defined as bilateral salpingo‑oophorectomy (BSO), experienced recurrence. The patient was a 24-year-old woman with or without total hysterectomy. who complained of irregular vaginal bleeding for ~1 month, with a serum β-human chorionic gonadotropin (β-HCG) level Statistical analysis. Statistical analysis was performed with elevated to 168.67 IU̸l (normal, <2.7 IU̸l). The patient was first SPSS software, version 16.0 (SPSS Inc., Chicago, IL, USA). diagnosed with ectopic pregnancy and a laparoscopic exami- Overall survival (OS) was defined as the time interval from nation was implemented. A 5-cm cyst was found in the left the date of primary surgery to death or last visit (months). ovary and a minor break was present in the right ovary which Disease‑free survival (DFS) was defined as the time interval was bleeding, and was considered to be a corpus luteum. The from the date of primary surgery to recurrence or last left ovarian cyst was excised and the corpus luteum-like right disease-free visit (months). The Kaplan-Meier method was ovarian lesion was also stripped. On histological examination, used to calculate survival and comparisons between survival both lesions were proven to be primary choriocarcinomas. rates were performed with the log-rank test. P-values <0.05 Following surgery, the CDF regimen was administered for were considered to indicate statistically significantdifferences . 6 cycles: Carboplatin i.p. on day 1, followed by dactinomycin and 5-fluorouracil i.v. on days 2-6. The β-HCG level
Load more

Recommended publications
  • Gonadal (Testicular and Ovarian Tumors)
    Gonadal (Testicular and Ovarian Tumors) Gonadal (Testicular and Ovarian Tumors) Authors: Ayda G. Nambayan, DSN, RN, St. Jude Children’s Research Hospital Erin Gafford, Pediatric Oncology Education Student, St. Jude Children’s Research Hospital; Nursing Student, School of Nursing, Union University Content Reviewed by: Guillermo L. Chantada, MD, Hospital JP Garrahan, Buenos Aires, Argentina Cure4Kids Release Date: 1 September 2006 At 4 weeks gestation, germ cells begin to develop in the yolk sac in an undifferentiated state. These primordial germ cells migrate to the gonadal ridge by the 6th week of gestation, and descend into the pelvis or scrotal sac.This migratory route explains the midline location of most extra-gonadal germ cell tumors (intracranial, mediastinal, retroperitoneal or sacrococcygeal). (A -1 ) Germ cell neoplasms arise either from the primordial germ cells (gonadal) or indirectly through embryonic or extra-embryonic differentiation (extra-gonadal). The stromal tumors arise from the primitive sex cords of either the ovary or the testicles, and the rare epithelial tumors arise from the coelomic epithelium that have undergone neoplastic transformations. Though the morphology of each type of germ cell tumor is similar in all locations (whether gonadal or extragonadal), the morphologic type and biological characteristics vary depending on the site of origin and age of the patient. There are three (A – 2) biologically distinct subsets of germ cell tumors: - tumors of the adolescent testis and ovary - extragonadal germ cell tumors of older children - tumors of infants and young children Testicular Germ Cell Tumors: Approximately 7% of all germ cell tumors are testicular and 75% of all (A – 3) testicular tumors have a germ cell origin.
    [Show full text]
  • Frequency of Malignant Ovarian Germ Cell Tumor and Distribution of Demographic Features in a Main Tertiary Hospital in Iran
    ©2018 ASP Ins., Afarand Scholarly Publishing Institute, Iran ISSN: 2476-5848; Journal of Obstetrics, Gynecology and Cancer Research. 2018;3(2):59-63. Frequency of Malignant Ovarian Germ Cell Tumor and Distribution of Demographic Features in a Main Tertiary Hospital in Iran A R T I C L E I N F O A B S T R A C T Aims Ovarian cancer is the 4th cause of women’s mortality occurring due to cancer. Malignant Article Type germ cell tumors (GCTs) account for 5% of malignant ovarian tumors and 70% of ovarian Original Research tumors in women between the ages of 10-30 years old. The aim of the present study was to detect the frequency of malignant ovarian germ cell tumor and distribution of demographic Authors features in the most crowded gynecology oncology clinic. 1 Setareh Akhavan MD, Materials and Methods This cohort descriptive-analytical study was conducted on cases 2 Jila Agah* MD, with malignant ovarian tumor managed in Vali-Asr hospital, Tehran, Iran, from 2001 to 2018 Abbas Alipour3 PhD (n=1540). The malignant germ cell tumors cases were extracted (n=128) and evaluated in point of epidemiologic and demographic data via the software SPSS 24. Findings 128 patients (8.3%) had GCTs. The average age was 23.88±7.85 years. 79.7% lived in the city, 76.6% had medium economic status and 53.6% had normal body mass index. Premature puberty was revealed in one person. Karyotype XY was detected in 5 persons. How to cite this article About 70.3% of the patients had no parity.
    [Show full text]
  • Dysgerminoma: Diagnostic Impasse
    International Surgery Journal Anandakannan I et al. Int Surg J. 2020 Nov;7(11):3811-3814 http://www.ijsurgery.com pISSN 2349-3305 | eISSN 2349-2902 DOI: http://dx.doi.org/10.18203/2349-2902.isj20204700 Case Report Dysgerminoma: diagnostic impasse Indrajit Anandakannan*, Shanthi Ponnandai Swaminathan, Vikas Kawarat, Rajeshwari Mani, Kannan R. Institute of General Surgery, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India Received: 13 August 2020 Revised: 07 October 2020 Accepted: 12 October 2020 *Correspondence: Dr. Indrajit Anandakannan, E-mail: [email protected] Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. ABSTRACT Dysgerminoma is a rare malignant ovarian tumour in women of reproductive age group, featuring lower abdominal pain and abdominal mass with elevated tumour markers. The tumour grows rapidly and diagnosed at an earlier stage. We present a short-statured 20 years girl with intermittent abdominal pain and distention for 4 months, on examination huge abdominal mass of size 20x15 cm, firm in consistency was palpable, lower limit not ascertained. Routine blood investigations normal, thyroid function test showed hypothyroidism. A plain abdominal radiograph shows a large homogenous mass fitting mid part of abdomen with displaced bowel loop. Contrast-enhanced computed tomography abdomen and pelvis show up 20x18.5x9.5 cm well defined heterogeneous mass, with areas of necrosis suggesting mesenchymal tumour or germ cell tumour.
    [Show full text]
  • Handbook for Children with Germ Cell Tumors
    Handbook for Children with TM Germ Cell Tumors Winter 2018 from the Cancer Committee of the American Pediatric Surgical Association ©2018, American Pediatric Surgical Association I CONTRIBUTORS Brent R. Weil, MD Boston Children’s Hospital Boston, MA Rebecca A. Stark, MD UC Davis Children’s Hospital Sacramento, CA Deborah F. Billmire, MD Riley Children’s Hospital Indianapolis, IN Frederick J. Rescorla, MD Riley Children’s Hospital Indianapolis, IN John Doski MD San Antonio, TX NOTICE The authors, editors, and APSA disclaim any liability, loss, injury or damage incurred as a consequence, directly or indirectly, of the use or application of any of the contents of this volume. While authors and editors have made every effort to create guidelines that should be helpful, it is impossible to create a text that covers every clinical situation that may arise in regards to either diagnosis and/or treatment. Authors and editors cannot be held responsible for any typographic or other errors in the printing of this text. Any dosages or instructions in this text that are questioned should be cross referenced with other sources. Attending physicians, residents, fellows, students and providers using this handbook in the treatment of pediatric patients should recognize that this text is not meant to be a replacement for discourse or consultations with the attending and consulting staff. Management strategies and styles discussed within this text are neither binding nor definitive and should not be treated as a collection of protocols. TABLE OF CONTENTS 1. Introduction 2. One-Minute Reviews 3. Classification and Differential Diagnosis 4. Staging 5. Risk Groups 6.
    [Show full text]
  • Ovary (Germ Cell Tumour) Fact Sheet
    Cancer Association of South Africa (CANSA) Fact Sheet on Germ Cell Tumour of the Ovary Introduction Germ cell tumours begin in the reproductive cells (egg or sperm) of the body. Ovarian germ cell tumours usually occur in teenage girls or young women and most often affect just one ovary. [Picture Credit: Germ Cell Ovarian Tumours] The ovaries are a pair of organs in the female reproductive system. They are situated in the pelvis, one on each side of the uterus. Each ovary is about the size and shape of an almond. The ovaries make ova (eggs) and female hormones. Ovarian germ cell tumour is a general name that is used to describe several different types of cancer. The most common ovarian germ cell tumour is called dysgerminoma. Acosta, A.M., Sholl, L.M., Cin, P.D., Howitt, B.E., Otis, C.N. & Nucci, M.R. 2020. Aims: Tumours of the female genital tract with a combination of malignant Mullerian and germ cell or trophoblastic tumour (MMGC/T) components are usually diagnosed in postmenopausal women, and pursue an aggressive clinical course characterised by poor response to therapy and early relapses. These clinical features suggest that MMGC/T are somatic in origin, but objective molecular data to support this interpretation are lacking. This study evaluates the molecular features of nine MMGC/T, including seven tumours containing yolk sac tumour (YST), one tumour containing choriocarcinoma and one tumour containing epithelioid trophoblastic tumour. The objectives were to: (i) investigate whether MMGC/T show a distinct genetic profile and (ii) explore the relationship between the different histological components.
    [Show full text]
  • CREOG Review: Oncology
    CREOG Review: Oncology I. Basic Science/Mechanisms of Disease A. Genetics Chromosomal Abnormalities: 1. Abnormalities of nuclear content: anueploidy 2. Deletions 3. Duplications 4. Rearrangements 5. Mutations of genes = proto-oncogenes, tumor suppressor genes, DNA repair genes - Proto-oncogenes: • Encode growth factors, membrane/cytoplasmic receptors and other proteins • Mutations behave in dominant fashion Æ alteration of one allele is enough to promote cancer progression • c-erb B and HER-2/neu Æ endometrial cancer • c-myc Æ cervical cancer - Tumor suppressor genes: • Prevent cell division or trigger cell death • Typically autosomal recessive • Include: p53, Rb, p16, NF1, WT1, BRCA1, BRCA2 • Mutations are “loss-of-function” mutations such as frameshifts, nonsense, deletions • p53 mutation Æ chemoresistance to platinum-based therapy - DNA repair genes: • Encode protein products that repair or remove damaged DNA • Example: DNA mismatch repair errors cause 25% of endometrial cancers; deficient DNA repair predisposes to HNPCC ** Normal tissue demonstrates a balance between cell proliferation and cell death When proliferation exceeds cell death Æ hyperplasia When death exceeds cell proliferation Æ atrophy Cell cycle G1 (Gap 1) Æ Synthesis (S) Æ G2 (Gap 2) Æ Division G1: preparation for DNA synthesis Synthesis: DNA synthesis, DNA content doubles G2: Resting period Division: Mitosis Phases of cell cycle most sensitive to chemotherapy - Alkylating agents: - attack the negatively charged sites on the DNA (oxygen, nitrogen, phosphorous and sulfur atoms), bind to DNA, leads to DNA strand breaks and DNA strand cross-linking causing cell death - active in every phase of the cell cycle - Examples: Cyclophosphamide, Ifosfamide, Melphalan, Chlorambucil, Dacarbazine, Procarbazine, Busulfan, and Thiotepa.
    [Show full text]
  • Overview of Gynecologic Oncology
    Overview of Gynecologic Oncology “The Blue Book” R. Kevin Reynolds, MD 11th Edition, Revised February 2010 www.med.umich.edu/obgyn/gynonc Gyn Oncology.......................................................................734-764-9106 Cancer Center Answer Line ...............................................800-865-1125 Contents Gyn Tumors Page Breast Cancer ......................................................................... 1 Cervical Cancer 6 Endometrial Cancer................................................. 17 Gestational Trophoblastic Neoplasia 24 Ovarian Cancer ....................................................... 29 Sarcomas 44 Vaginal Cancer........................................................ 51 Vulvar Cancer 53 Associated Treatment Modalities Nutrition, Fluid and Electrolytes 66 Radiation Therapy ................................................... 71 Chemotherapy 75 Perioperative Management ..................................... 94 Tools and Equipment for the Art of Surgery 108 Appendix Out-of-Date Staging Rules .................................... 123 GOG Toxicity Criteria 125 Performance Status............................................... 129 Web Resources 130 Special thanks to William Burke, MD, and to Catherine Christen, PharmD Favorite Quotes "Statistics are no substitute for judgment." Henry Clay "A leading authority is anyone who has guessed right more than once." Frank A. Clark "Well done is better than well said." Ben Franklin "Trust me. I'm a doctor" Donald H. Chamberlain, MD "To err is human; to repeat the
    [Show full text]
  • Immature Ovarian Teratoma in a 21 Year-Old Woman: a Case Report
    Genera of l P l r a a n c r t u i c o e J Stavrou, J Gen Pract 2016, 4:2 Journal of General Practice DOI: 10.4172/2329-9126.1000232 ISSN: 2329-9126 CaseResearch Report Article OpenOpen Access Access Immature Ovarian Teratoma in a 21 Year-Old Woman: A Case Report and Review of the Literature Sofoklis Stavrou*, Ekaterini Domali , Ioannis Paraoulakis , Dimitrios Haidopoulos ,Nikolaos Thomakos , Dimitrios Loutradis ,Peter Drakakis Department of Obstetrics and Gynecology,University of Athens, Alexandra Hospital, 11528, Athens, Greece Abbreviations: IOTA: International Ovarian Tumor Analysis; MRI: Magnetic Resonance Imaging; CT: Computed Tomography; 3D: 3 Dimensional; NCCN: National Comprehensive Cancer Network; ECOG: Eastern Cooperative Oncology Group. Introduction Figure 1: 3D images of ovarian lesion; A: color score IV with centrally Ovarian tumors can be categorized into epithelial, germ cell, sex distributed vessels; B: uterus surrounded by ascites; C: multilocular-solid cord stromal or metastatic ones [1]. The ovarian teratomas are divided mass, containing>5loci and irregular solid part while external wall was into mature teratomas, immature teratomas and monodermal teratomas appeared interrupted. (like struma ovarii, carcinoid tumors, and neural tumors). They represent the largest part of germ cells neoplasms [1,2]. Mature cystic teratomas (dermoid cysts) constitute the majority of ovarian germ cell with specific tumor markers .So, tumor markers Ca 15-3, Ca19-9, Ca tumors accounting for 20% of all ovarian neoplasms [3]. Struma ovarii 125, and CEA were fluctuated in normal ranges. Serum levels of alpha- tumors represent uncommon ovarian tumors containing completely or fetoprotein (a-FP) were significantly elevated reaching the plateau of mainly thyroid tissue [3].
    [Show full text]
  • Mature Teratomas
    MATURE TERATOMAS 30% Ovarian tumors Mostly dermoid cysts May be seen at any age Complications: infection, torsion, rupture with pseudomalignant change outside ovary, melanosis peritonei, hemolytic anemia, limbic encephalitis Dermoid Cyst Dermoid Cyst Dermoid Cyst with Abundant Brain Dermoid Cyst Sieve –Like Pattern Cerebellum Fetal Cartilage (not immature!) Selected Home Truths 1. If it looks like a fibroid grossly do not put through any sections. You will just end up confused! (AT Hertig, M.D.) 2. Nothingggg looks more malignant on high power than normal proliferative endometrium (AT Hertig, M.D.) 3. If it grossly looks like a dermoid cyst, it is a dermoid cyst! UNCOMMON TISSUES IN DERMOID Salivary gland 16% Thyroid 13% GI epithelium 12% Lung 3% Retina 2% PttProstate 1% Pituitary 1% Pancreas 1% From Blackwell et al. Am J Obset Gynecol 51:151, 1946 MALIGNANT CHANGE IN DERMOID CYST • About 1% of cases, disproportionately in postmenopausal years • Great majjyority are sq uamous cell carcinoma • Others are mostly melanoma and rare sarcomas • Thorough sampling to show association with dermoid can be crucial • Theoretically includes mucinous tumors but by convention they are considered separately Squamous Cell Ca in Dermoid Squamous Cell Ca in Dermoid Struma with Dermoid Cystic Struma Cystic Struma Cystic Struma Solid Struma Struma Carcinoid Insular Carcinoid PRIMITIVE GERM CELL TUMORS Dysgerminoma Yolk sac tumor Embryonal carcinoma Choriocarcinoma Immature Teratoma Polyembryoma Mixed forms (mostly 1 and 2) Remember some, mostly 1, arise out
    [Show full text]
  • Klinefelter Syndrome and Germ Cell Tumors: Review of the Literature Kimberley Bonouvrie1,2 , Jutte Van Der Werff Ten Bosch2,3 and Machiel Van Den Akker2,3*
    Bonouvrie et al. International Journal of Pediatric Endocrinology (2020) 2020:18 https://doi.org/10.1186/s13633-020-00088-0 REVIEW Open Access Klinefelter syndrome and germ cell tumors: review of the literature Kimberley Bonouvrie1,2 , Jutte van der Werff ten Bosch2,3 and Machiel van den Akker2,3* Abstract Objective: The most common presentation of Klinefelter syndrome (KS) is infertility and features of hypogonadism. Currently no consensus exists on the risk of malignancy in this syndrome. Several case reports show an incidence of extragonadal germ cells tumors (eGCT) of 1.5 per 1000 KS patients (OR 50 against healthy population). Malignant germ cell tumors are rare in children. They account for 3% of all children cancers. Young patients with a germ cell tumor are not routinely tested for Klinefelter syndrome. This can therefore result in underdiagnosing. Literature data suggest a correlation between eGCT and KS. To the best of our knowledge there is no precise description of the primary locations of germ cell tumors in KS patients. The purpose of this study is to evaluate age groups and primary locations of extragonadal germ cell tumors in Klinefelter patients. With this data we investigate whether it is necessary to perform a cytogenetic analysis for KS in every eGCT patient. Study design: This study is based on case report publications in PubMed/Medline published until march 2020 that described “Klinefelter Syndrome (MeSH) AND/OR extragonadal germ cell tumors”. Publications were included when patients age, location and histology of the germ cell tumor was known. Two double blinded reviewers selected the studies.Results: 141 KS patients with eGCTs were identified.
    [Show full text]
  • OVARIAN GERM CELL TUMORS Union for International Cancer Control 2014 Review of Cancer Medicines on the WHO List of Essential Medicines
    OVARIAN GERM CELL TUMORS Union for International Cancer Control 2014 Review of Cancer Medicines on the WHO List of Essential Medicines OVARIAN GERM CELL TUMORS Executive Summary Ovarian germ cell tumors (OGCTs) are derived from primordial germ cells of the ovary. These tumors affect both adults and children and therefore the recommendations apply to the EML lists for both adults and children (regimen distinctions are included in the present briefing). OGCTs include dysgerminomas and nondysgerminomas, which are subdivided to immature teratoma, embryonal cell carcinoma, yolk sac tumors, primary ovarian (nongestational) choriocarcinomas, polyembryoma, and mixed germ cell tumors. OGCTs comprise of 2-3% of all malignant ovarian tumors registered globally, with highest rates in young women of 15 to 30 years old [1]. Since 1990, the standard regimen for OGCT treatment has been a combination of bleomycin, etoposide and cisplatin, referred to as “BEP” regimen. The standard of care in the United States, Europe, and other high-income settings is a combination of the three chemotherapy agents plus surgery. This allows to achieve a survival rate of 95-100% at 5 years among patients receiving adjuvant BEP regimen after surgery for stage I disease, and 70-85% among patients with metastatic disease (II-IV stages) on BEP regimen [2,3]. The chance of survival without chemotherapy is 0%. Addition of filgrastim to chemotherapy when a patients’ absolute neutrophil count decreases to less than 500 prevents the occurrence of severe neutropenia and infection. Using filgrastim helps to maintain appropriate dose intensity, which is vital in the treatment of germ cell tumors, as well as other curable tumors.
    [Show full text]
  • Therapeutic Challenges for Cisplatin-Resistant Ovarian Germ Cell Tumors
    cancers Review Therapeutic Challenges for Cisplatin-Resistant Ovarian Germ Cell Tumors Ugo De Giorgi 1,*, Chiara Casadei 1, Alice Bergamini 2, Laura Attademo 3, Gennaro Cormio 4, Domenica Lorusso 5, Sandro Pignata 3 and Giorgia Mangili 2 1 Department of Medical Oncology and Hematology, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, 47014 Meldola, Italy; [email protected] 2 Department of Obstetrics and Gynaecology, San Raffaele Scientific Institute, 20132 Milan, Italy; [email protected] (A.B.); [email protected] (G.M.) 3 Department of Urology and Gynecology, Istituto Nazionale Tumori IRCCS Fondazione G. Pascale, 80138 Naples, Italy; [email protected] (L.A.); [email protected] (S.P.) 4 Gynecologic Oncology Unit, IRCCS Istituto Oncologico Giovanni Paolo II, 70124 Bari, Italy; [email protected] 5 Gynecologic Oncology Unit, Department of Woman, Child Health and Public Health, Fondazione Policlinico Universitario Agostino Gemelli, IRCCS, 00168 Rome, Italy; [email protected] * Correspondence: [email protected] Received: 28 August 2019; Accepted: 15 October 2019; Published: 17 October 2019 Abstract: The majority of patients with advanced ovarian germ cell cancer are treated by cisplatin-based chemotherapy. Despite adequate first-line treatment, nearly one third of patients relapse and almost half develop cisplatin-resistant disease, which is often fatal. The treatment of cisplatin-resistant disease is challenging and prognosis remains poor. There are limited data on the efficacy of specific chemotherapeutic regimens, high-dose chemotherapy with autologous progenitor cell support and targeted therapies. The inclusion of patients in clinical trials is strongly recommended, especially in clinical trials on the most frequent male germ cell tumors, to offer wider therapeutic opportunities.
    [Show full text]