Trichoblastic carcinoma is a rare neoplasm thought to arise from malignant degeneration of benign follicular tumors. Its clinical features, optimal treatment, and outcomes remain largely unknown. We performed a comprehensive review of the existing trichoblastic carcinoma literature. PubMed and Google Scholar were searched for keywords related to trichoblastic carcinoma, and additional articles were found in reference lists. Cases with a histopathologic diagnosis of trichoblastic carcinoma were summarized. A total of 93 cases have been described, all in case reports or case series. The average age was 65, with 66.7% of cases in males. A minority of patients had familial tumor syndromes or a history of radiation at the site, and only one was reported to be immunosuppressed. The most common site was the face (48.4%), and the majority developed de novo (87.1%). The clinical presentation was highly variable. Although most cases (82.8%) were successfully treated with surgery alone, a subset of patients had more aggressive disease including local progression or recurrence in five cases, nodal metastases in five cases, and distant metastases in three cases. Trichoblastic carcinoma is a rare malignancy with the potential for aggressive behavior. Further research is needed to better characterize this neoplasm.
Keywords: Adnexal neoplasms; Follicular neoplasms; Malignant trichoblastoma; Trichoblastic carcinoma.
© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.