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Abstract：
Pathological diagnosis of dermal melanocytic tumors is often problematic owing to histological resemblance. Recently, cutaneous melanocytoma with CRTC1-TRIM11 (CMCT) was added to this category. However, only six cases have been reported so far. We herein present a case of a 77-year-old Japanese man with CMCT. The patient presented a nodule in the right thigh and underwent surgical resection. Histological examination indicated a well-demarcated 6 × 5 mm-sized tumor nodule in the dermis and subcutis. The tumor was amelanotic, consisting of uniform nests and fascicles of spindled, or epithelioid cells. The melanocytic nature was evident by immunohistochemistry. The CRTC1-TRIM11 fusion was detected by TRIM11 immunostaining, chromogenic in situ hybridization, and RT-PCR/direct sequencing. He has been free from the tumor for 1 year after additional resection. The main differential diagnosis of CMCT includes primary and metastatic dermal malignant melanomas (MM) and dermal/subcutaneous clear cell sarcoma (CCS). Additionally, histological overlap with paraganglioma-like dermal melanocytic tumor was considered. Although some investigators argue that CMCT is a variant of CCS, we think it should be separated from CCS, and subcutaneous/dermal CCS should be confined to tumors with EWSR1-ATF1/ CREB1 fusion. However, longer follow-up and more case studies are needed for revealing the true prognosis of CMCT.
摘要：
由于组织学相似性，真皮黑素细胞肿瘤的病理诊断通常是有问题的。最近,皮肤黑素细胞瘤加上CRTC1-TRIM11(CMCT)。然而,到目前为止，只有6例报告。我们在此介绍一名77岁的日本男子患有CMCT的病例。患者出现右大腿结节，并接受了手术切除。组织学检查显示真皮和皮下组织有一个6×5毫米大小的肿瘤结节。肿瘤是无色素性的，由均匀的巢和纺锤状的束组成，或上皮样细胞。黑素细胞性质通过免疫组织化学得到证实。通过TRIM11免疫染色检测CRTC1-TRIM11融合,显色原位杂交，和RT-PCR/直接测序。他在额外切除后1年没有肿瘤。CMCT的主要鉴别诊断包括原发性和转移性真皮恶性黑色素瘤（MM）和真皮/皮下透明细胞肉瘤（CCS）。此外,考虑与副神经节瘤样真皮黑素细胞肿瘤的组织学重叠。尽管一些研究人员认为CMCT是CCS的一种变体，我们认为它应该与CCS分开，皮下/真皮CCS应局限于EWSR1-ATF1/CREB1融合的肿瘤。然而,需要更长时间的随访和更多的病例研究来揭示CMCT的真实预后.
