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Expression pattern of clinically relevant markers in paediatric germ cell- and sex-cord stromal tumours is similar to adult testicular tumours

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  3. Autopsy after transcatheter aortic valve implantation

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  2. Neuronal mechanisms of mutations in SCN8A causing epilepsy or intellectual disability

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  3. Decline in severe spastic cerebral palsy at term in Denmark 1999-2007

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  4. Characterization of Human Adrenal Steroidogenesis during Fetal Development

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Paediatric germ cell tumours (GCTs) are rare and account for less than 3 % of childhood cancers. Like adult GCTs, they probably originate from primordial germ cells, but the pattern of histopathological types is different, and they occur predominantly in extragonadal sites along the body midline. Because they are rare, histology of paediatric GCTs is poorly documented, and it remains unclear to what extent they differ from adult GCTs. We have analysed 35 paediatric germ cell tumours and 5 gonadal sex-cord stromal tumours from prepubertal patients aged 0-15 years, to gain further knowledge, elaborate on clinical-pathological associations and better understand their developmental divergence. The tumours were screened for expression of stemness-related factors (OCT4, AP-2γ, SOX2), classical yolk sac tumours (YSTs; AFP, SALL4), GCTs (HCG, PLAP, PDPN/D2-40), as well as markers for sex-cord stromal tumour (PDPN, GATA4). All YSTs expressed AFP and SALL4, with GATA4 present in 13/14. The majority of teratomas expressed SOX2 and PDPN, whereas SALL4 was found in 8/13 immature teratomas. Adult seminoma markers AP-2γ, OCT4, SALL4 and PDPN were all expressed in dysgerminoma. We further report a previously unrecognised pathogenetic relationship between AFP and SALL4 in YST in that different populations of YST cells express either SALL4 or AFP, which suggests variable differentiation status. We also show that AP-2γ is expressed in the granulosa layer of ovarian follicles and weakly expressed in immature but not in mature granulosa cell tumours. Our findings indicate that the expression pattern of these antigens is similar between paediatric and adult GCTs, even though they develop along different developmental trajectories.

Original languageEnglish
JournalVirchows Archiv : an international journal of pathology
Volume465
Issue number5
Pages (from-to)567-77
Number of pages11
ISSN0945-6317
DOIs
Publication statusPublished - Nov 2014

    Research areas

  • Adolescent, Adult, Child, Child, Preschool, Endodermal Sinus Tumor, Female, Humans, Immunohistochemistry, Infant, Infant, Newborn, Male, Neoplasms, Germ Cell and Embryonal, Ovarian Neoplasms, Seminoma, Teratoma, Testicular Neoplasms, Transcription Factors, Tumor Markers, Biological, alpha-Fetoproteins

ID: 44856987