Background: Melanotic schwannoma is a pigmented nerve tumor that may be located in the skin and express local aggressivity. This tumor may occur singly. It may also be part of the Carney complex which consists of various, but specific, tumors.
Objective: We report two cases of subcutaneous melanotic schwannoma localized on the trunk in two men aged 37 and 45 years.
Methods: Conventional histology and immunohistochemistry were performed.
Results: One melanotic schwannoma was associated with a cutaneous atypical myxoma and multiple melanocytic lesions, all being part of the Carney complex. The other case had no associated signs. In both cases, the melanotic schwannoma was completely excised and did not recur.
Conclusion: Melanotic schwannoma is rare and curable by surgery. It must not be confused with malignant melanoma and other pigmented neoplasms. The Carney complex should be carefully ruled out.
Schwann Cell S100 Protein Neurofibroma Factor XIIIa Cardiac Myxoma
This is a preview of subscription content, log in to check access
No sources of funding were used to assist in the preparation of this manuscript. The authors have no conflicts of interest that are directly relevant to the content of this manuscript.
Bouziani A, Kammoun N, Zidi B, et al. Le schwannome mélanotique: une observation avec revue de la littérature. Arch Anat Cytol Pathol 1994; 2: 46–53Google Scholar
Burns K, Silva FG, Forde KA, et al. Primary melanotic schwannoma of the stomach: evidence of dual melanocytic and schwannoma differentiation in extra-axial site in a patient without neurofibromatosis. Cancer 1983; 52: 1432–41PubMedCrossRefGoogle Scholar
Noubari BA, Chiaramonte I, Magro G, et al. Spinal malignant melanotic schwannoma. J Neurosurg Sci 1998; 42: 245–9PubMedGoogle Scholar
Carney JA, Gordon H, Carpenter PC, et al. The complex of myxomas, spotty pigmentation and endocrine over-activity. Medicine 1985; 64: 270–82PubMedCrossRefGoogle Scholar
Carney JA, Hruska LS, Beauchamps GD, et al. Dominant inheritance of the complex of myxomas, spotty pigmentation, and endocrine overactivity. Mayo Clin Proc 1986; 61: 165–72PubMedGoogle Scholar
Danoff A, Jormark S, Lorber D, et al. Adrenocortical micronodular dysplasia, cardiac myxomas, lentigines, and spindle cell tumors. Arch Intern Med 1987; 147: 443–8PubMedCrossRefGoogle Scholar
Thornton CM, Handley J, Bingham EA, et al. Psammomatous melanotic schwannoma arising in the dermis in a patient with Carney’s complex. Histopathology 1992; 20: 71–3PubMedCrossRefGoogle Scholar
Utiger CA, Headington JT. Psammomatous melanotic schwannoma: a new cutaneous marker for Carney’s complex. Arch Dermatol 1993; 129: 202–4PubMedCrossRefGoogle Scholar
Carney JA, Ferreiro JA. The epithelioid blue nevus: a multicentric familial tumor with important associations, including cardiac myxoma and psammomatous melanotic schwannoma. Am J Surg Pathol 1996; 20: 259–72PubMedCrossRefGoogle Scholar
Monteagudo C, Ferrandez A, Gonzalez-Devesa M, et al. Psammomatous malignant melanoma arising in an intradermal naevus. Histopathology 2001; 39: 493–7PubMedCrossRefGoogle Scholar
Aso M, Hashimoto K, Eto H, et al. Expression of Schwann cell characteristics in pigmented nevus. Immunohistochemical study using monoclonal antibody to Schwann cell associated antigen. Cancer 1988; 62: 938–43PubMedCrossRefGoogle Scholar
Gray MH, Smoller BR, McNutt NS, et al. Immunohistochemical demonstration of factor XIIIa expression in neurofibromas. A practical means of differentiating these tumors from neurotized melanocytic nevi and schwannomas. Arch Dermatol 1990; 126: 472–6PubMedCrossRefGoogle Scholar