HMB-45 could turn out positive in PEComa and related tumors, melanotic schwannoma, clear cell sarcoma, sex cord-stromal tumors, MiT family translocation renal cell carcinomas, pheochromocytoma, and rare cases of salivary gland tumors (as previously specified, it reacts with the fibrillar matrix in stage II melanocytes and should be more appropriately considered an organelle-specific marker, rather than a lineage-specific marker) [44,45,46,47]. The gene discussed is PMEL; the disease is hereditary clear cell renal cell carcinoma.