Abstract：
CRTC1::TRIM11 cutaneous tumor (CTCT) is a rare skin tumor of uncertain differentiation. In the 49 reported cases, only four cases showed regional or distant metastasis, but follow-up remains limited. Herein, we present a case of metastatic CTCT with ulceration, a histological feature that has not been previously described. A 75-year-old male with a 2-month history of toe ulceration underwent a shave biopsy, which showed a dermal nodular neoplasm that was immunoreactive for SOX10 and S100, negative for Melan-A, and was initially diagnosed as melanoma. Upon pathology review at our institution, the tumor was composed of intersecting fascicles and nests of epithelioid and spindle cells. Additional immunohistochemistry revealed immunoreactivity of the tumor for MiTF and NTRK and negativity for HMB-45 and PRAME. Next-generation sequencing identified CRTC1::TRIM11 fusion, leading to a revised diagnosis of CTCT. The patient proceeded to a toe amputation and sentinel lymph node (SLN) biopsy 5 months after the shave biopsy. The amputation showed residual CTCT and a focus on lymphovascular invasion. The SLN revealed multifocal subcapsular metastases. The patient was started on adjuvant nivolumab and showed biopsy-proven recurrence in the right inguinal lymph nodes and imaging findings suspicious for pulmonary metastases 8 months after the excision. In summary, we present a case of CTCT with ulceration and lymphovascular invasion. We also provide additional evidence that a subset of CTCT behaves aggressively. The optimal surgical and medical treatments are unknown.
摘要：
CRTC1::TRIM11皮肤肿瘤(CTCT)是一种罕见的分化不确定的皮肤肿瘤。在报告的49起案件中，只有4例出现局部或远处转移，但后续行动仍然有限。在这里，我们介绍了一例伴有溃疡的转移性CTCT,以前没有描述过的组织学特征。一名有2个月脚趾溃疡病史的75岁男性接受了剃须活检，表现为对SOX10和S100免疫反应的真皮结节性肿瘤，对Melan-A阴性，最初被诊断为黑色素瘤。根据我们机构的病理学检查，肿瘤由交叉的神经束和上皮样细胞和梭形细胞的巢组成。其他免疫组织化学显示肿瘤对MiTF和NTRK的免疫反应性以及对HMB-45和PRAME的阴性。下一代测序鉴定出CRTC1::TRIM11融合，导致CTCT的修订诊断。患者在剃刮活检后5个月进行脚趾截肢和前哨淋巴结（SLN）活检。截肢显示CTCT残留，重点是淋巴血管浸润。SLN显示多灶性包膜下转移。患者开始接受nivolumab辅助治疗，术后8个月，右侧腹股沟淋巴结活检证实复发，影像学发现可疑肺转移。总之,我们介绍了一例伴有溃疡和淋巴管浸润的CTCT病例。我们还提供了额外的证据，表明CTCT的一个子集具有攻击性。最佳的手术和药物治疗是未知的。
