Familial Testicular Tumor



Familial Testicular Tumor


Gladell P. Paner, MD








Image shows mixed TGCT that consists of seminoma image, embryonal carcinoma image, and mature teratoma image. In young adults, mixed TGCT is the 2nd most common testicular tumor after pure seminoma.






Large-cell calcifying Sertoli cell tumor shows distinctive large cells with abundant eosinophilic cytoplasm & calcifications; it is associated with Carney complex & Peutz-Jeghers syndrome. (Courtesy S. Shen, MD.)


FAMILIAL TESTICULAR GERM CELL TUMORS


Terminology



  • Abbreviations



    • Testicular germ cell tumor (TGCT)


    • Familial testicular germ cell tumor (FTGCT)


    • Hereditary testicular germ cell tumor (HTGCT)


  • Definitions



    • FTGCT



      • Affected individuals from families with ≥ 2 cases of TGCT


    • HTGCT



      • FTGCT with consistent passage of susceptibility gene via Mendelian inheritance


      • No definitive human susceptibility gene identified so far


      • Existence not yet firmly established


Epidemiology



  • Incidence



    • In USA, there will be 7,920 cases of testicular cancers estimated in 2013


    • Incidence increased 3-6% annually since the 1970s


    • 95% of testicular tumors are TGCT


    • ˜ 1.5% of patients with TGCT reported positive family history of TGCT



      • ˜ 120 FTGCT cases per year


  • Age range



    • 3 distinct age groups of TGCT



      • Mostly young adults between 20 and 35 years (pure and mixed germ-cell tumor [GCT])


      • Neonates and infants (mostly pure teratoma and yolk sac tumor)


      • Older men (spermatocytic seminoma)


    • Most reported FTGCT cases under 1st group


    • Diagnosis of FTGCT is 2-3 years younger than in usual TGCT

Tags:
Jul 6, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Familial Testicular Tumor

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