The World Health Organization in its 2019 Classification of Breast Tumors termed breast sebaceous carcinoma as invasive breast carcinoma of no special type (IBC-NST), with a sebaceous pattern. Approximately 30 cases of IBC-NST with a sebaceous pattern have been reported in the literature, and in all cases the expression of mismatch repair proteins in tumors was normal. Here, we report a case of IBC-NST with a sebaceous pattern and high-frequency microsatellite instability (MSI-H). This case was a sporadic sebaceous pattern of IBC-NST with MSI-H and was unrelated to Muir-Torre syndrome. Its histopathological characteristics were similar to those of MSI-H-associated triple-negative breast carcinoma (TNBC) with a high histological grade but were without tumor-infiltrating lymphocytes (TILs). The tumor did not recur after 20 months of follow-up.

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