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      Is Open Access

      Gonadal germ cell tumors in children : A retrospective review of a 10-year single-center experience

      review-article
      , MD a , , , MD b , , MD b , , MD a , , MD a
      Medicine
      Wolters Kluwer Health
      children, germ cell tumor, gonadal, yolk sac tumor

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          Abstract

          Background:

          The true incidence of gonadal germ cell tumors (GCTs) in children is unknown. Few studies have been published concerning about pediatric gonadal GCTs. The aim of this study is to review and analyze clinical data on the diagnosis and management of gonadal GCTs in children.

          Methods:

          Between 2005 and 2015, 127 pediatric patients (<14 years old) with gonadal GCTs admitted to our institute were reviewed. Clinical features, imaging and laboratory studies, surgical approaches, as well as pathological diagnoses were recorded.

          Results:

          The series comprised 53 males with testicular GCTs and 74 females with ovarian GCTs. Their median age was 5.8 years old. Palpable mass was the main clinical manifestation of testicular GCTs, while abdominal pain and abdominal distention were the most frequent presenting symptoms of ovarian GCTs. Both computed tomography and magnetic resonance imaging showed a high diagnostic yield. AFP levels were elevated in most malignant GCTs, markedly elevated in yolk sac tumors. All patients were treated surgically. Mature teratoma was the most common type of benign GCTs, while yolk sac tumor was the most common type of malignant GCTs.

          Conclusion:

          Gonadal GCTs in children have various of pathological types, as well as clinical manifestations. Imaging and laboratory data could be useful for differentiation of malignant from benign tumors. Final diagnosis depends on pathology. Surgical excision of the gonadal GCTs is the prior option.

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          Most cited references27

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          Surgery for ovarian masses in infants, children, and adolescents: 102 consecutive patients treated in a 15-year period.

          Ovarian pathology, although rare in children, must be included in the differential diagnosis of all girls who present with abdominal pain, an abdominal mass, or precocious puberty.
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            Germ-cell tumors in childhood and adolescence. GPOH MAKEI and the MAHO study groups.

            In mature and immature teratoma the treatment is surgical. The risk of recurrence can be estimated from the parameters primary site (with the coccygeal tumors being most at risk), histological grade of immaturity and completeness of the primary resection including the adjacent organ of origin (coccyx, ovary, testis etc.). In case of a microscopically complete tumor resection there is no role for adjuvant chemo- or radiotherapy irrespective of the histological grade of immaturity. Malignant germ-cell tumors (GCT) account for 2.9% of all malignant tumors of children younger than 15 years of age. More than half of the tumors occur at extragonadal sites such as the ovaries (26%), the coccygeal region (24%), the testes (18%) and the brain (18%) represent then primary sites. In patients with extensive tumor growth, metastatic disease or secreting intracranial tumors a delayed tumor resection after preoperative chemotherapy is preferable. In these patients malignant non-seminomatous GCT may be diagnosed clinically due to the increased serum or cerebrospinal fluid levels of the tumor markers AFP and/or beta-HCG. Current risk adapted treatment protocols containing cisplatinum allow long-term remissions in about 80% including patients with bulky or metastatic tumors. In the cisplatinum era the prognostic factors like histology, primary site of the tumor and initial tumor stage have partly lost their former impressive significance in infants and children. On the other hand the completeness of the primary tumor resection according to oncological standards has been established as the most powerful prognostic parameter superior to tumor marker levels or primary site of the tumor.
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              Management of germ cell tumors with somatic type malignancy: pathological features, prognostic factors and survival outcomes.

              Germ cell tumors with somatic type malignancy are rare, occurring in approximately 2.7% to 8.6% of germ cell tumor cases. Prognostic factors and optimal management remain poorly defined. The Indiana University testis cancer database was queried from 1979 to 2011 for patients demonstrating germ cell tumor with somatic type malignancy at orchiectomy or subsequent resection. Patients with transformation to primitive neuroectodermal tumor only were excluded from study due to distinct management. Chart review, pathological review and survival analysis were performed. A total of 121 patients met the study inclusion criteria. The most common somatic type malignancy histologies were sarcoma (59), carcinoma (31) and sarcomatoid yolk sac tumor (17). Of these patients 32 demonstrated somatic type malignancy at germ cell tumor diagnosis. For those with delayed identification, median time from germ cell tumor to somatic type malignancy diagnosis was 33 months. This interval was longest for carcinomas (108 months). At a median followup of 71 months, 5-year cancer specific survival was 64%. Predictors of poorer cancer specific survival included somatic type malignancy diagnosed at late relapse (p = 0.017), referral to Indiana University for reoperative retroperitoneal lymph node dissection (p = 0.026) and grade (p = 0.026). None of these factors maintained prognostic significance on multivariate analysis. Somatic type malignancy histology subtype, stage, risk category and number of resections were not predictive of cancer specific survival. Germ cell tumor with somatic type malignancy is associated with poorer cancer specific survival than traditional germ cell tumor. Established prognostic factors for germ cell tumor lose predictive value in the setting of somatic type malignancy. Aggressive and serial resections are often necessary to optimize cancer specific survival. Tumor grade is an important prognostic factor in sarcomas and sarcomatoid yolk sac tumors. Copyright © 2014 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.
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                Author and article information

                Journal
                Medicine (Baltimore)
                Medicine (Baltimore)
                MEDI
                Medicine
                Wolters Kluwer Health
                0025-7974
                1536-5964
                June 2017
                30 June 2017
                : 96
                : 26
                : e7386
                Affiliations
                [a ]Department of Pediatric Surgery, The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University
                [b ]Department of Pediatric Surgery, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, China.
                Author notes
                []Correspondence: Xiaokun Lin, Department of Pediatric Surgery, The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325027, China (e-mail: linxk2000@ 123456163.com ).
                Article
                MD-D-17-01780 07386
                10.1097/MD.0000000000007386
                5500093
                28658171
                494c76b9-d79f-474b-915f-605043db8ce3
                Copyright © 2017 the Author(s). Published by Wolters Kluwer Health, Inc.

                This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial License 4.0 (CCBY-NC), where it is permissible to download, share, remix, transform, and buildup the work provided it is properly cited. The work cannot be used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc/4.0

                History
                : 24 March 2017
                : 18 May 2017
                : 4 June 2017
                Categories
                6200
                Research Article
                Systematic Review and Meta-Analysis
                Custom metadata
                TRUE

                children,germ cell tumor,gonadal,yolk sac tumor
                children, germ cell tumor, gonadal, yolk sac tumor

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