Literature DB >> 35554637

How to classify, diagnose, treat and follow-up extragonadal germ cell tumors? A systematic review of available evidence.

Stefanie Schmidt1, Carsten Bokemeyer2, Christian Winter3,4, Friedemann Zengerling1,5, Jonas Busch6, Julia Heinzelbecker7, David Pfister8, Christian Ruf9, Julia Lackner5, Peter Albers10, Sabine Kliesch11.   

Abstract

PURPOSE: To present the current evidence and the development of studies in recent years on the management of extragonadal germ cell tumors (EGCT).
METHODS: A systematic literature search was conducted in Medline and the Cochrane Library. Studies within the search period (January 2010 to February 2021) that addressed the classification, diagnosis, prognosis, treatment, and follow-up of extragonadal tumors were included. Risk of bias was assessed and relevant data were extracted in evidence tables.
RESULTS: The systematic search identified nine studies. Germ cell tumors (GCT) arise predominantly from within the testis, but about 5% of the tumors are primarily located extragonadal. EGCT are localized primarily mediastinal or retroperitoneal in the midline of the body. EGCT patients are classified according to the IGCCCG classification. Consecutively, all mediastinal non-seminomatous EGCT patients belong to the "poor prognosis" group. In contrast mediastinal seminoma and both retroperitoneal seminoma and non-seminoma patients seem to have a similar prognosis as patients with gonadal GCTs and metastasis at theses respective sites. The standard chemotherapy regimen for patients with a EGCT consists of 3-4 cycles (good vs intermediate prognosis) of bleomycin, etoposid, cisplatin (BEP); however, due to their very poor prognosis patients with non-seminomatous mediastinal GCT should receive a dose-intensified or high-dose chemotherapy approach upfront on an individual basis and should thus be referred to expert centers Ifosfamide may be exchanged for bleomycin in cases of additional pulmonary metastasis due to subsequently planned resections. In general patients with non-seminomatous EGCT, residual tumor resection (RTR) should be performed after chemotherapy.
CONCLUSION: In general, non-seminomatous EGCT have a poorer prognosis compared to testicular GCT, while seminomatous EGGCT seem to have a similar prognosis to patients with metastatic testicular seminoma. The current insights on EGCT are limited, since all data are mainly based on case series and studies with small patient numbers and non-comparative studies. In general, systemic treatment should be performed like in testicular metastatic GCTs but upfront dose intensification of chemotherapy should be considered for mediastinal non-seminoma patients. Thus, EGCT should be referred to interdisciplinary centers with utmost experience in the treatment of germ cell tumors.
© 2022. The Author(s).

Entities:  

Keywords:  Chemotherapy; Extragonadal germ cell tumors (EGCTs); Non-seminoma; Primary mediastinal germ cell tumors; Primary retroperitoneal germ cell tumors; Seminoma

Year:  2022        PMID: 35554637     DOI: 10.1007/s00345-022-04009-z

Source DB:  PubMed          Journal:  World J Urol        ISSN: 0724-4983            Impact factor:   4.226


  42 in total

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Authors:  R Harbour; J Miller
Journal:  BMJ       Date:  2001-08-11

Review 2.  Extragonadal germ cell tumors.

Authors:  H-J Schmoll
Journal:  Ann Oncol       Date:  2002       Impact factor: 32.976

3.  Positron emission tomography and clinical predictors of survival in primary extragonadal germ cell tumors.

Authors:  T Buchler; P Dusek; A Brisuda; K Simonova; P Fencl; J Jarkovsky; M Babjuk; J Abrahamova
Journal:  Klin Onkol       Date:  2012

4.  A principal component analysis is conducted for a case series quality appraisal checklist.

Authors:  Bing Guo; Carmen Moga; Christa Harstall; Don Schopflocher
Journal:  J Clin Epidemiol       Date:  2015-08-22       Impact factor: 6.437

Review 5.  Management of Germ Cell Tumours of the Testes in Adult Patients: German Clinical Practice Guideline, PART II - Recommendations for the Treatment of Advanced, Recurrent, and Refractory Disease and Extragonadal and Sex Cord/Stromal Tumours and for the Management of Follow-Up, Toxicity, Quality of Life, Palliative Care, and Supportive Therapy.

Authors:  Sabine Kliesch; Stefanie Schmidt; Doris Wilborn; Clemens Aigner; Walter Albrecht; Jens Bedke; Matthias Beintker; Dirk Beyersdorff; Carsten Bokemeyer; Jonas Busch; Johannes Classen; Maike de Wit; Klaus-Peter Dieckmann; Thorsten Diemer; Anette Dieing; Matthias Gockel; Bernt Göckel-Beining; Oliver W Hakenberg; Axel Heidenreich; Julia Heinzelbecker; Kathleen Herkommer; Thomas Hermanns; Sascha Kaufmann; Marko Kornmann; Jörg Kotzerke; Susanne Krege; Glen Kristiansen; Anja Lorch; Arndt-Christian Müller; Karin Oechsle; Timur Ohloff; Christoph Oing; Ulrich Otto; David Pfister; Renate Pichler; Heinrich Recken; Oliver Rick; Yvonne Rudolph; Christian Ruf; Joachim Schirren; Hans Schmelz; Heinz Schmidberger; Mark Schrader; Stefan Schweyer; Stefanie Seeling; Rainer Souchon; Christian Winter; Christian Wittekind; Friedemann Zengerling; D H Zermann; Roger Zillmann; Peter Albers
Journal:  Urol Int       Date:  2021-01-22       Impact factor: 2.089

6.  Klinefelter's syndrome associated with mediastinal germ cell neoplasms.

Authors:  C R Nichols; N A Heerema; C Palmer; P J Loehrer; S D Williams; L H Einhorn
Journal:  J Clin Oncol       Date:  1987-08       Impact factor: 44.544

7.  [OCEBM levels of evidence system].

Authors:  N Durieux; S Vandenput; F Pasleau
Journal:  Rev Med Liege       Date:  2013-12

8.  Hematologic malignancies associated with primary mediastinal germ-cell tumors.

Authors:  C R Nichols; R Hoffman; L H Einhorn; S D Williams; L A Wheeler; M B Garnick
Journal:  Ann Intern Med       Date:  1985-05       Impact factor: 25.391

9.  Assessing bias in studies of prognostic factors.

Authors:  Jill A Hayden; Danielle A van der Windt; Jennifer L Cartwright; Pierre Côté; Claire Bombardier
Journal:  Ann Intern Med       Date:  2013-02-19       Impact factor: 25.391

10.  Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement.

Authors:  David Moher; Alessandro Liberati; Jennifer Tetzlaff; Douglas G Altman
Journal:  PLoS Med       Date:  2009-07-21       Impact factor: 11.069

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