Testis & paratestis

Sex cord stromal tumors

Mixed and other sex cord stromal tumors

Last author update: 1 October 2013
Last staff update: 28 March 2022

Copyright: 2003-2023, PathologyOutlines.com, Inc.

PubMed Search: Sex cord stromal tumors [title]

Swapnil U. Rane, M.D.
Page views in 2022: 1,969
Page views in 2023 to date: 1,611
Cite this page: Rane S. Mixed and other sex cord stromal tumors. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/testismixedscst.html. Accessed September 27th, 2023.
Definition / general
  • A sex cord stromal tumor of the testis with mixed sex cord stromal elements or undifferentiated features
  • May contain any combination of cell types, including Sertoli cells, Leydig cells, granulosa cells
  • Also includes undifferentiated stromal tumors with no clearly demonstrable differentiation
  • Rare tumor of descended testis; < 1% of testicular tumors (J Urol 2004;172:2370)
  • Can occur in any age group; 1/3 of cases occur in children
  • Account for 2/3 of sex cord tumors occurring in children
  • No specific etiology has been reported for the mixed / undifferentiated sex cord stromal tumors
Clinical features
  • Usually present with a painless testicular swelling
  • May be painful (Ulster Med J 1997;66:54)
  • 15% present with gynecomastia
Comparison of mixed sex cord stromal tumors with and without gonadoblastoma
  • Mixed sex cord stromal tumor with gonadoblastoma:
    • Associated with disorders of sexual development (gonadal dysgenesis [pure or mixed], altered karyotype, pseudohermaphrodites with streak gonads)
    • Leydig-like cells and lutein cells
    • Virilization
    • Proliferative activity only in germ cells
  • Mixed sex cord stromal tumor without gonadoblastoma:
    • Genetically and phenotypically normal individuals
    • No Leydig-like cells and lutein cells
    • No endocrine effects usually; if present, usually feminization
    • Proliferative activity in sex cord stromal and germ cell components
  • Germ cell markers beta-hCG, LDH, alpha fetoprotein are within normal limits
Radiology description
  • Solid tumors but may have cystic areas
Prognostic factors
  • Similar to other sex cord stromal tumors: poor factors are large size, invasive growth pattern, angiolymphatic invasion, nuclear atypia, mitotic activity, necrosis
  • These tumors are usually benign in children < age 10 years
  • ~20% in older patients are malignant
Case reports
  • Similar to other sex cord stromal tumors
    • High inguinal orchidectomy routinely done
    • Testicular sparing surgery may be attempted if tumor is small, well circumscribed without any invasive features, normal tumor markers and frozen section facilities are available for intraoperative diagnosis
    • No proven indication for lymph node dissection if nodes are not enlarged
Gross description
  • Gray, tan or yellow solid nodules
  • Presence of invasion into tunica, rete testis or adjacent structures predicts malignant / aggressive behavior
Gross images

Images hosted on other servers:

Well circumscribed lower pole testicular tumor

Microscopic (histologic) description
Microscopic (histologic) images

Images hosted on other servers:

Tumor is compressing adjacent tunica

Fascicular and storiform spindle cell arrangement

Insular arrangement of cells in hyaline stroma

Irregular ovoid nuclei with longitudinal grooves

Positive stains
Negative stains
Molecular / cytogenetics description
  • Ovarian tumors have c-kit mutations (seen in seminomas) and excess genetic material on #12p (seen in all germ cell tumors) but no genetic aberrations identified in testicular tumors (Virchows Arch 2006;448:612)
Differential diagnosis
  • Pure sex cord stromal tumors: show only one cell type and not more than an occasional microscopic focus of other elements; presence of more than occasional occurrence of two or more sex cord stromal cell types warrants a diagnosis of mixed sex cord stromal tumor
  • Sex cord stromal tumor with entrapped germ cells (Am J Surg Pathol 2000;24:535) and mixed germ cell sex cord stromal tumors: show clusters of germ cells in the center of the tumor; only one sex cord stromal cell type present
Back to top
Image 01 Image 02