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Gonadoblastoma

  • Living reference work entry
  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 56 Accesses

Synonyms

Gonadoblastoma; Mixed germ cell; Sex cord; Stromal tumor

Definition

Neoplasm resembling normal developing gonad (hence the name) composed of germ cell type and sex cord cells similar to immature granulosa cells.

Clinical Features

  • Incidence

    Very rare neoplasia.

  • Age

    1st–2nd decade of life.

  • Sex

    Male individuals.

  • Site

    Descended or undescended testis.

  • Etiology

    It frequently occurs in patients with DSD (Disorders of Sex Development) such as Turner Syndrome, Swyer Syndrome, and Complete Androgen Insensitivity Syndrome. All these disorders have in common a mutation in one or multiple genes implicated in normal development of gonads, such as WT1, SOX9, SF1, DHH, ARX, and TSPY. These mutations lead to altered maturation of both sex cord cells component and germ cells component: the former resemble granulosa cells instead of normal Sertoli cells while the latter are prone to neoplastic transformation toward GCNIS-like cells (Ulbright and Young 2014).

  • Treatment

    Surgical excision of the...

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References and Further Reading

  • Kao, C.-S., Ulbright, T. M., & Idrees, M. T. (2014). Gonadoblastoma: An immunohistochemical study and comparison to Sertoli cell nodule with intratubular germ cell neoplasia, with pathogenetic implications. Histopathology, 65, 861–867.

    Article  Google Scholar 

  • Kersemaekers, A.-M. F., Stoop, H., Oosterhuis, J. W., et al. (2006). Gonadoblastoma arising in undifferentiated gonadal tissue within dysgenetic gonads. The Journal of Clinical Endocrinology & Metabolism., 91, 2404–2413.

    Article  Google Scholar 

  • Scully, R. E. (1970). Gonadoblastoma. A review of 74 cases. Cancer, 25, 1340–1356.

    Article  CAS  Google Scholar 

  • Ulbright, T. M., & Young, R. H. (2014). Gonadoblastoma and selected other aspects of gonadal pathology in young patients with disorders of sex development. Seminars in Diagnostic Pathology, 31, 427–440.

    Article  Google Scholar 

  • Ye, H., & Ulbright, T. M. (2012). Difficult differential diagnoses in testicular pathology. Archives of Pathology & Laboratory Medicine, 136, 435–446.

    Article  Google Scholar 

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Author information

Authors and Affiliations

  1. Histopathology and Molecular Diagnostics, University Hospital Careggi, Florence, Italy

    Caterina Fattorini & Maria Rosaria Raspollini

  2. Pathology Service, Champalimaud Clinical Center, Lisbon, Portugal

    Antonio Lopez-Beltran

  3. Unit of Anatomic Pathology, Department of Surgery, Cordoba University Medical School, Cordoba, Spain

    Antonio Lopez-Beltran

Authors
  1. Caterina Fattorini
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  2. Antonio Lopez-Beltran
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  3. Maria Rosaria Raspollini
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Corresponding author

Correspondence to Maria Rosaria Raspollini .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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Cite this entry

Fattorini, C., Lopez-Beltran, A., Raspollini, M.R. (2019). Gonadoblastoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4819-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_4819-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

  • eBook Packages: Springer Reference MedicineReference Module Medicine

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