Atypical cellular neurothekeoma of the oral mucosa: A rare case report and literature review

• Published in: Journal of cutaneous pathology 2024-12, Vol.51 (12), p.930-937
• Main Authors: Bezerra, Hélen Kaline Farias, Nakamura, Gabriele Prospero, Mariz, Bruno Augusto Linhares Almeida, Lopes, Rodrigo Nascimento, Prado, José Divaldo, Roza, Ana Luiza Oliveira Corrêa, Vargas, Pablo Agustin
• Format: Article
• Language: English
• Subjects: CD34 antigen; CD63 antigen; Cell proliferation; cellular neurothekeoma; Female; floor of the mouth; Humans; Literature reviews; Middle Aged; Mouth; Mouth Mucosa - metabolism; Mouth Mucosa - pathology; Mouth Neoplasms - diagnosis; Mouth Neoplasms - metabolism; Mouth Neoplasms - pathology; Mucosa; nerve sheath myxoma; Neurothekeoma - diagnosis; Neurothekeoma - metabolism; Neurothekeoma - pathology; oral mucosa; S100 protein; Stroma; Tumor cells
• ISSN: 0303-6987; 1600-0560; 1600-0560
• DOI: 10.1111/cup.14693

Cellular neurothekeoma (CN) is a benign dermal neoplasm that most often affects the head and neck region and rarely occurs in the oral mucosa. We report a rare case of CN with atypical features on the floor of the mouth and summarize the reported cases of oral CN in English‐language literature. A 62‐year‐old woman presented with a 6‐month history of a painless mass on the floor of the mouth. Histopathological analysis of the excised specimen revealed a proliferation of neoplastic cells with oval to spindle morphology arranged in a vaguely nested and multinodular architecture separated by scarce hyaline collagen within a predominantly myxoid‐rich stroma. The tumor cells were positive for NSE, and CD63 (NKI/C3), and negative for S100 protein, CD34, and SMA. Thus, the final diagnosis was CN. In addition, we summarized all clinicopathological data on oral CNs reported in the English‐language literature. Nineteen cases were reviewed. Among them, only one case affected the floor of the mouth of a young girl, in contrast to the present case that occurred in an elderly woman. CN is particularly rare in this location and may be a diagnostic challenge for oral pathologists due to its rarity and morphological similarity with other lesions.