Testis and epididymis
Sex cord stromal tumors
General

Author: Swapnil U. Rane, M.D. (see Authors page)

Revised: 14 July 2017, last major update July 2013

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

PubMed Search: Testis sex cord stromal tumors [title]

Cite this page: Sex cord stromal tumors - general. PathologyOutlines.com website. http://pathologyoutlines.com/topic/testissexcordgeneral.html. Accessed September 25th, 2017.
Definition / general
  • Tumors arising from nongerminative stromal cells of testis
  • Consists of multiple tumor types, each strikingly different in clinical features and biology
  • Leydig cell tumor is most common
  • Some arise in setting of androgen insensitivity syndrome (AIS) or adrenogenital syndrome (AGS); designate as tumor-like lesion occurring in specific syndrome
Terminology
  • Terminology analogous to ovarian sex cord stromal tumors but some tumors occur almost exclusively in testis (large cell calcifying Sertoli cell tumor and sclerosing Sertoli cell tumor) while others are extremely uncommon in testis (lipid rich Sertoli cell tumor) (Mod Pathol 2005;18:S81)
  • Broadly divided as pure (only one type) or mixed (two + types)
WHO classification
  • Leydig cell tumors
  • Malignant Leydig cell tumor
  • Sertoli cell tumor
    • Variants
      • Sertoli cell tumor, lipid rich variant
      • Sclerosing Sertoli cell tumor
      • Large cell calcifying Sertoli cell tumor
  • Malignant Sertoli cell tumor
  • Granulosa cell tumor
    • Adult granulosa cell tumor
    • Juvenile granulosa cell tumor
  • Tumors of thecoma / fibroma group
    • Thecoma
    • Fibroma
  • Sex cord / gonadal stromal tumor, incompletely differentiated
  • Sex cord / gonadal stromal tumor, mixed form
  • Malignant sex cord / gonadal stromal tumor
  • Tumors containing both germ cell and sex cord / gonadal stromal tumor
    • Gonadoblastoma
    • Germ cell - sex cord / gonadal stromal tumor, unclassified
Epidemiology
  • Uncommon; 3 - 4% of adult testicular tumors but ~30% of testicular tumors in infants and children
  • Wide age range in general but:
    • Juvenile granulosa cell tumor is most common congenital testicular tumor; more common in neonates and infants, associated with ambiguous genitalia or abnormalities of sex chromosomes
    • Sertoli cell tumors are common before age 1 year; associated with Peutz-Jeghers syndrome and Carney complex
Clinical features
  • Usually presents as testicular mass
  • Unlike ovarian sex cord stromal tumors, functional tumors are generally uncommon except for Leydig cell tumors
Prognostic factors
  • Indolent behavior (unlike germ cell tumors)
  • Stage is most important prognostic factor
  • Stage with TNM or Children's Oncology Group (COG) staging system for germ cell tumors
Treatment
  • High inguinal orchidectomy is treatment of choice
  • Testicular sparing surgery may be attempted in small tumors
  • Retroperitoneal lymph node dissection but only if clinical - radiological evidence of metastatic disease
Positive stains