Testis & epididymis
Sex cord-stromal tumors
Mixed / unclassified sex cord-stromal


Topic Completed: 1 October 2013

Minor changes: 10 September 2020

Copyright: 2003-2020, PathologyOutlines.com, Inc.

PubMed Search: Sex cord stromal tumors [title]

Swapnil U. Rane, M.D.
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Cite this page: Rane S. Mixed / unclassified sex cord-stromal. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/testismixedscst.html. Accessed September 19th, 2020.
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
Terminology
  • Also includes undifferentiated stromal tumors with no clearly demonstrable differentiation
Epidemiology
  • 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
Etiology
  • 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
Laboratory
  • 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
Treatment
  • 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

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Well circumscribed lower pole testicular tumor

Microscopic (histologic) description
Microscopic (histologic) images

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Tumor is compressing adjacent tunica

Fascicular and storiform spindle cell arrangement

Insular arrangement of cells in hyaline stroma

Irregular ovoid nuclei with longitudinal grooves

Immunohistochemistry
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
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