Peng et al. (2019) held the opinion that the criterion should be relaxed up to 4 cm. PNETs of VHL patients were considered not at risk of metastasis with a diameter below 1.2 cm and high risks of malignancy and metastasis with a diameter greater than 3 cm. For patients with a PNET diameter between 1.2 and 3 cm, only those with VHL missense mutations were observed to develop metastasis (Tirosh et al., 2018). Here, VHL is linked to primitive neuroectodermal tumor.