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Sex cord stromal tumors
Mixed / unclassified sex cord - stromal


Swapnil U. Rane, M.D.

Topic Completed: 1 October 2013

Revised: 28 March 2019

Copyright: 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Sex cord stromal tumors [title]

Swapnil U. Rane, M.D.
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Table of Contents
Definition / general | Terminology | Epidemiology | Etiology | Clinical features | Laboratory | Radiology description | Prognostic factors | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Immunohistochemistry | Differential diagnosis
Cite this page: Rane S. Mixed / unclassified sex cord - stromal. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/testismixedscst.html. Accessed February 21st, 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

Images hosted on other servers:

Well circumscribed lower pole testicular tumor

Microscopic (histologic) description
Microscopic (histologic) images

Images hosted on other servers:

Cords separated by hyalinized stroma

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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