Recurring mixed-type neurothekeoma of the face

Neurothekeomas are rare, benign neoplasms, typically occurring in younger patients with a remarkable predilection for the female population. The nomenclature and derivation of these tumors is controversial. The rarity of this unusual skin tumor in daily routine histopathologic findings prompted the following report.

We report a 17-year-old girl with an asymptomatic nodule at the inner angle of the left eye with slow progression in size within 12 months to 1 cm in diameter. Multiple treatments with laser surgery were performed and yielded no persistent remission of the tumor. Histopathologic examination revealed a non-encapsulated dermal tumor, composed of multiple, closely situated medium-sized nodules, separated by a myxoid collagenrich stroma, without epidermal alteration. In summary, due to the histoarchitecture and immunoprofile of the tumor, a mixed-type cellular neurothekeoma was diagnosed.

We think that it is important to be aware of these uncommon soft tissue lesions and the pitfalls of mixed-type neurothekeomas that often cause diagnostic problems. The aim of this report is to help to avoid misdiagnoses of malignant mesenchymal tumors with serious consequences, including extensive surgical therapy or radiation.