Primary malignant schwannoma of the cervical plexus with melanocytic differentiation

Orbital and myocardial metastases of a primary pulmonary melanotic schwannoma

“…Although these lesions are of neural origin, a number of cases have been reported in the stomach, bone and soft tissues. Other unusual sites include the heart, liver and skin [3]. Primary pulmonary involvement, as in our case, is extremely rare.…”

“…About 20% of patients have multiple tumours, and in such patients, there is an ever higher probability that other manifestations of the complex will be present [3]. However, our patient had no evidence of the complex.…”

“…The tem melanocytic schwannoma was coined by Fu et al in 1975 [2]. Despite the similarity in their names, the lesion is a distinctive neoplasm of adult life that differs signifi cantly from classic schwannoma in age of presentation and clinical behaviour [1][2][3][4]. Although these lesions are of neural origin, a number of cases have been reported in the stomach, bone and soft tissues.…”

“…Due to its neural origin, pain and neurologic defi cits in the affected part are the most common presentations [2,3]. The presence of melanin gives the A thorough pathological study is pivotal for a correct diagnosis [1][2][3][7][8][9]. The neoplastic cells are predominantly spindled, with ill-defi ned cytoplasms, although there are usually also epithelioid cells that blend seamlessly with the fusiform ones.…”

“…The tumour cells strongly express the S-100 protein and HMB-45, both present in melanoma and in other tumour types. The S-100 protein is composed of two subunits, alpha and beta, which are present in melanomas; however, in melanotic schwannomas, only the beta subunit is expressed [3]. Ultrastructurally, the cells resemble Schwann cells, with cytoplasmic processes in the form of lateral interdigitations and intercellular unions.…”