Gonadoblastoma

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Classification according to ICD-10
D39.9 New formation of unsafe or unknown behavior of the female genital organs
D40.9 New formation of unsafe or unknown behavior in the male genital organs
ICD-10 online (WHO version 2019)

The gonadoblastoma (from ancient Greek γόνος, γονάδο gonos, gonado ' gonad ' and βλάστημα blástema ' blastoma ') is a rare, mostly hormone- producing tumor that originates in the germ cells and cells derived from the germinal ridge.

In contrast to germ cell tumors, the original tissues are almost exclusively malformed gonads .

It is a benign tumor consisting of a mixture of tissue from the genital ridge and primordial germ cells with a risk of degeneration.

The first description comes from 1970 by the American pathologist Robert E. Scully.

distribution

The frequency is not known, the tumors are usually discovered before age 30, in 95% a male genotype at but female appearance before, in 80% there is a gonadal dysgenesis

The gonadoblastoma accounts for 0.5% of all testicular tumors .

There is often an association with a dysgerminoma .

Clinical manifestations

Clinical criteria are:

  • Karyotype mostly 46, XY; 45, X / 46, XY; or 45, XO
  • Appearance 80% female

therapy

Although there is hardly any data on the actual degeneration, the preventive removal of incorrectly developed gonads is usually recommended.

literature

  • LM Roth, L. Cheng: Classical gonadoblastoma: its relationship to the 'dissecting' variant and undifferentiated gonadal tissue. In: Histopathology . Vol. 72, No. 4, March 2018, pp. 545-555, doi: 10.1111 / his.13387 , PMID 28881049 (review).
  • TM Ulbright, RH Young: Gonadoblastoma and selected other aspects of gonadal pathology in young patients with disorders of sex development. In: Seminars in diagnostic pathology. Vol. 31, No. 5, September 2014, pp. 427-440, doi: 10.1053 / j.semdp.2014.07.001 , PMID 25129544 .
  • MS Verp, JL Simpson: Abnormal sexual differentiation and neoplasia. In: Cancer Genetics and Cytogenetics. Vol. 25, No. 2, April 1987, pp. 191-218, PMID 3548944 (Review).

Individual evidence

  1. ↑ W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. Springer 1996, ISBN 3-540-60224-0 .
  2. ↑ a b Willibald Pschyrembel : Clinical Dictionary , 266th, updated edition, de Gruyter, Berlin 2014, ISBN 978-3-11-033997-0 , keyword gonadoblastoma
  3. ↑ a b gonadoblastoma. In: Orphanet (Rare Disease Database).
  4. ↑ a b emedicine
  5. ^ RE Scully: Gonadoblastoma. A review of 74 cases. In: Cancer . Vol. 25, No. 6, June 1970, pp. 1340-1356, PMID 4193741 (review).
  6. ↑ a b Urology textbook
  7. ^ YF Lau, Y. Li, T. Kido: Gonadoblastoma locus and the TSPY gene on the human Y chromosome. In: Birth defects research. Part C, Embryo today: reviews. Vol. 87, No. 1, March 2009, pp. 114-122, doi: 10.1002 / bdrc.20144 , PMID 19306348 (Review).

Web links

Commons : Gonadoblastoma  - Collection of images, videos and audio files