Testis and epididymis
Nonneoplastic testicular lesions
Splenogonadal fusion syndrome

Author: Sean Williamson, M.D. (see Authors page)

Revised: 11 April 2017, last major update December 2012

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Splenogonadal fusion syndrome

Cite this page: Splenogonadal fusion syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/testissplenogonadal.html. Accessed September 25th, 2018.
Definition / general
  • Also called ectopic scrotal spleen, testicular splenic fusion
  • Rare congenital condition of gonad fusing with ectopic splenic tissue; patients usually present before age 20, 50%+ are less than age 10
  • In males, involves left testis only; occurs in females also
  • Fusion may be continuous (attaching to spleen) or discontinuous (intrascrotal splenic nodules attached to testis, spermatic cord, epididymis, appendix of testis or appendix of epididymis)
    • Continuous form is associated with limb bud anomalies such as peromelia (severe congenital anomalies of extremities identical to thalidomide embryopathy) and micrognatia (small jaw)
    • Discontinuous type is rarely associated with cardiac defects
Case reports
Gross description
  • Splenic tissue well demarcated from gonad
Microscopic (histologic) description
  • Normal splenic parenchyma with variable fibrosis, thrombi, calcification, fatty degeneration, hemosiderin
Microscopic (histologic) images

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Case of the Week #309

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Various images

Various Images

Differential diagnosis
  • Lymphoproliferative tumor
  • Sarcoma
  • Teratoma