To Be or Not to Be a Germ Cell: The Extragonadal Germ Cell Tumor Paradigm

Felici, Massimo De; Klinger, Francesca Gioia; Campolo, Federica; Balistreri, Carmela Rita; Barchi, Marco; Dolci, Susanna

doi:10.3390/ijms22115982

Cited by 29 publications

(29 citation statements)

References 88 publications

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“…The included 32 studies comprised a total of 5095 patients (2639 male, 2027 female, 429 unknown) [ 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 ]. There were 22 prospective [ 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 20 , 21 , 26 , 27 , 31 , 32 , 33 ] and 10 retrospective studies change [ <...>…”

Section: Resultsmentioning

confidence: 99%

“…The included 32 studies comprised a total of 5095 patients (2639 male, 2027 female, 429 unknown) [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][23][24][25][26][27][28][29][30][31][32][33][34][35][36]. There were 22 prospective [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18]20,21,26,27,[31]…”

Section: Overview Of Studiesmentioning

confidence: 99%

“…All studies describe thorough exploration and multiple biopsies of peritoneal surfaces in case of suspicious findings, as well as sending ascites or peritoneal washings for cytological examination in order to stage and treat appropriately. Second look surgery is described in 15 studies for different reasons, ranging from removing residual tumour after initial biopsy to surgery upon deterioration of tumour markers during follow-up [ 4 , 5 , 6 , 7 , 10 , 11 , 13 , 14 , 15 , 16 , 18 , 23 , 25 , 32 , 33 ].…”

Section: Individual Anatomical Localisationsmentioning

confidence: 99%

“…They are predominantly midline tumours occurring from birth to late adulthood [ 3 ]. All are believed to arise from totipotent primordial germ cells and derivatives thereof [ 4 , 5 ]. In childhood, ~50% are gonadal and ~50% extragonadal (~20% intracranial and ~30% extracranial), and clinical presentation depends on tumour site [ 1 , 6 ].…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Treatment and Survival of Malignant Extracranial Germ Cell Tumours in the Paediatric Population: A Systematic Review and Meta-Analysis

Hulsker

Mansori

Fiocco

et al. 2021

Cancers

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Objective: This systematic review and meta-analysis was performed to explore overall survival (OS) and event free survival (EFS) rates internationally over the past two decades and to define specific subgroups with inferior outcomes which may demand different treatment strategies. Methods: The search focused on malignant extracranial germ cell tumours (GCTs) in the paediatric population. The initial database search identified 12,556 articles; 32 articles were finally included in this review, comprising a total of 5095 patients. Results: The studies were heterogeneous, varying from single institution reports to large prospective trials. Older studies, describing eras where non-platinum-based chemotherapy regimens were used, showed clearly worse outcomes. Survival for stage I–II gonadal disease is excellent. On the other hand, patients with an initial alpha-fetoprotein (AFP) > 10,000 ng/mL or kU/L, age > 11 years and stage IV disease confer a survival disadvantage. For testicular disease in particular, lymphovascular invasion and certain histopathological subtypes, such as embryonal carcinoma (EC) and mixed malignant GCTs, survival is poorer. Survival data for sacrococcygeal and mediastinal GCTs show a heterogeneous distribution across studies in this review, independent of year of publication. Patients > 12 years presenting with a mediastinal GCT pose a subpopulation which fares worse than GCTs in other locations or age groups. This is independent of AFP levels, stage of disease or treatment protocol, and these patients may demand a different treatment strategy. Conclusions: This review describes the heterogeneous nature of GCTs in different anatomical locations, impacting on stage at presentation, treatment modalities used and survival data. Despite this heterogeneity, in line with the current developmental biology-based classification system, subpopulations can be defined which have an inferior EFS and OS and where future research and more individualised treatment would help to improve survival.

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Section: Resultsmentioning

confidence: 99%

Section: Overview Of Studiesmentioning

confidence: 99%

Section: Individual Anatomical Localisationsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Treatment and Survival of Malignant Extracranial Germ Cell Tumours in the Paediatric Population: A Systematic Review and Meta-Analysis

Hulsker

Mansori

Fiocco

et al. 2021

Cancers

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“…Further information and clinical outcomes of these tumors were not subject of the present review. We refer the interested readers to our recent reviews [28,29]. Here, we focus on their possible common origin from PGCs.…”

Section: The Pi3k/pten/akt Signaling Pathways In the Generation Of Germ Cell Tumors From Pgcsmentioning

confidence: 99%

PI3K/PTEN/AKT Signaling Pathways in Germ Cell Development and Their Involvement in Germ Cell Tumors and Ovarian Dysfunctions

Felici

Klinger

2021

IJMS

Self Cite

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Several studies indicate that the PI3K/PTEN/AKT signaling pathways are critical regulators of ovarian function including the formation of the germ cell precursors, termed primordial germ cells, and the follicular pool maintenance. This article reviews the current state of knowledge of the functional role of the PI3K/PTEN/AKT pathways during primordial germ cell development and the dynamics of the ovarian primordial follicle reserve and how dysregulation of these signaling pathways may contribute to the development of some types of germ cell tumors and ovarian dysfunctions.

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Retroperitoneal extragonadal germ cell tumor with duodenal infiltration: A challenging endo‐luminal tissue diagnosis

et al. 2022

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A 32‐year‐old Malay male was referred to our hospital for a second opinion. An abdominal and pelvic CT scan at the previous medical facility showed a large retroperitoneal tumor, which was subjected to ultrasound‐guided fine‐needle aspiration cytology (FNAC) with a provisional diagnosis of malignant lymphoma. However, after reviewing the existing results, a repeat biopsy was deemed necessary and this was performed endoluminally via gastroduodenoscopy in view of the close proximity of the tumor and the third part of the duodenum. The first biopsy failed to detect any abnormal cells, but a repeat biopsy with supporting evidence from other laboratory results led to a final diagnosis of extragonadal germ cell tumor (GCT) with duodenal infiltration.

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To Be or Not to Be a Germ Cell: The Extragonadal Germ Cell Tumor Paradigm

Cited by 29 publications

References 88 publications

Treatment and Survival of Malignant Extracranial Germ Cell Tumours in the Paediatric Population: A Systematic Review and Meta-Analysis

Treatment and Survival of Malignant Extracranial Germ Cell Tumours in the Paediatric Population: A Systematic Review and Meta-Analysis

PI3K/PTEN/AKT Signaling Pathways in Germ Cell Development and Their Involvement in Germ Cell Tumors and Ovarian Dysfunctions

Retroperitoneal extragonadal germ cell tumor with duodenal infiltration: A challenging endo‐luminal tissue diagnosis

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