A long-standing controversy exists regarding the concept of testicular mixed germ cell-sex cord stromal tumor (MGC-SCST) as a distinct entity, or whether instead it simply denotes a sex cord-stromal tumor with entrapped germ cells. Recently, Michalova et al. detected chromosomal aneuploidy as well as additional morphological evidence including abnormal mitoses and extratesticular invasion in some of their cases, thus strongly supporting the neoplastic nature of the germ cell component . Both genetic and epigenetic alterations likely account for the differences in histological appearance and clinical behavior depending on the sex of the individual. Moreover, testicular MGC-SCST is even less common than the corresponding ovarian neoplasm.
In 1981, Bolen reported the first case of testicular MGC-SCST . Matoska and Talerman  reported the second case of testicular MGC-SCST, 9 years after Talerman described the first two cases of ovarian MGC-SCST [4, 5]. In a recent commentary published in Virchows Archiv, Oosterhuis envisioned that in some cases, the development of testicular MGC-SCST could begin with entrapment of tubules within a gonadal sex cord-stromal tumor followed by selection of spermatogonial cells that have acquired chromosomal aberrations that confer to them both survival and growth advantage . The author, however, believes that the majority of cases of testicular MGC-SCST arise through simultaneous transformation of germ cells and sex cord elements .
It is well established that testicular MGC-SCST is clinically non-aggressive, whereas ovarian MGC-SCST often shows aggressive behavior. The most likely explanation for this observation is that ovarian MGC-SCST is a premalignant neoplasm often associated with malignant germ cell tumors, whereas the corresponding testicular tumors are benign. The association of ovarian MGC-SCST with malignant germ cell tumors likely accounts for its earlier clinical detection.
In summation, we strongly support the viewpoint that testicular MGC-SCST is a distinct neoplasm rather than being simply a sex cord-stromal tumor with entrapped germ cells. Both genetic and epigenetic variations likely account for the differences between testicular and ovarian MGC-SCST.
Michalova K, McKenney JK, Kristiansen G et al (2020) Novel insights into the mixed germ cell-sex cord stromal tumor of the testis: detection of chromosomal aneuploidy and further morphological evidence supporting the neoplastic nature of the germ cell component. Virchows Arch 477(5):615–623
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Roth, L.M. On the existence of testicular mixed germ cell-germ cell sex cord-stromal tumor as a distinct entity. Virchows Arch (2021). https://doi.org/10.1007/s00428-021-03024-6