Abstract
Simple SummaryMolecular profiling of unclassified neoplasms has been pivotal in the characterization of new entities. Cutaneous melanocytic tumor with CRTC1::TRIM11 fusion (CMTCT) is a newly described neoplasm that falls into the broad category of diagnostically challenging dermal proliferations with melanocytic differentiation. The aim of this review is to educate colleagues about the clinical, histopathologic, and molecular features of CMTCT, and provide important information on the differential diagnosis.“Cutaneous melanocytic tumor with CRTC1::TRIM11 fusion” (CMTCT) is a recently described entity belonging to the family of superficial tumors displaying melanocytic differentiation. Thirteen cases have been reported so far, on the head and neck, extremities, and trunk of adults of all ages (12 cases) and one in an 11-year-old child. Histopathologically, it is a nodular or multilobulated tumor composed of spindle and epithelioid cells arranged in nests, fascicles, or bundles that are surrounded by thin collagenous septa. By immunohistochemistry, the tumor shows variable immunoreactivity for S100-protein, SOX10, and MITF, as well as specific melanocytic markers such as MelanA and HMB-45. The neoplasm’s biologic behavior remains uncertain since the reported cases are limited and the follow-up is short (median 12 months). However, local recurrence and synchronous distant metastasis after 13 years of initial resection has been described in one case. Herein, we present a comprehensive literature review of CMTCT hoping to raise awareness among the dermatopathologists of this potentially novel entity.
Highlights
The histopathologic diagnosis of dermal/subcutaneous proliferations with melanocytic differentiation can be challenging due to existing similarities among different entities and their broad differential diagnosis
Cellier et al [1] first reported five cases in 2018, and described it as a relatively well-circumscribed neoplasm composed of atypical spindle and epithelioid cells immunoreactive for melanocytic markers, such as SOX10, MITF, and
[1] Primary dermal melanoma (PDM) presents as a single nodule or multilobulated tumor composed of spindle, or epithelioid cells arranged in sheets or nests [29,30]
Summary
The histopathologic diagnosis of dermal/subcutaneous proliferations with melanocytic differentiation can be challenging due to existing similarities among different entities and their broad differential diagnosis. This includes the newly described superficial melanocytic tumor with CRTC1::TRIM11 fusion. Cellier et al [1] first reported five cases in 2018, and described it as a relatively well-circumscribed neoplasm composed of atypical spindle and epithelioid cells immunoreactive for melanocytic markers, such as SOX10, MITF, and. The histopathologic differential diagnosis includes cutaneous clear cell sarcoma, primary dermal and metastatic melanoma, atypical blue nevus, Spitz tumor, myoepithelial tumor, epithelioid schwannoma, malignant peripheral nerve sheath tumor (MPNST), and paraganglioma-like dermal melanocytic tumor. CMTCT’s biologic behavior has not yet been determined, as the reported median follow-up is relatively short (12 months); one case did recur and metastasized to the regional lymph nodes and lung after 13 years, which prompted the use of the
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
