Background:: Melanotic schwannoma is a rare tumor with indeterminate biologic behavior and varying treatment recommendations.
Methods:: We report 2 cases of pigmented melanotic schwannoma of the head and neck and perform literature review. The pathologic and immunohistochemical characteristics of melanotic schwannoma are reviewed.
Results:: Two cases of melanotic schwannoma are presented. Both cases underwent surgical resection with one patient receiving adjuvant radiation therapy.
Conclusions:: Melanotic schwannoma is a rare nerve sheath tumor that is frequently mistaken for malignant melanoma. We describe 2 cases of pigmented melanotic schwannoma of the head and neck with different presentations and review the histopathological and immunohistochemical features.
Keywords: Carney complex; neck mass; pigmented melanotic schwannoma; reticulin stain; sympathetic schwannoma.