Dumbbell-shaped C-2 psammomatous melanotic malignant schwannoma. Case report and review of the literature.

The authors present the case of a dumbbell-shaped malignant psammomatous melanotic schwannoma of the upper cervical spine involving the C-2 sensory root. The family of the patient had a history of other malignant stromal tumors, without the Carney complex genetic pattern. The 30-year-old female patient complained of experiencing cervical pain and cervical muscle contractions for 6 months, and was admitted to the hospital. The cervical T1-weighted magnetic resonance (MR) images revealed the presence of a slightly hyperintense C2-3 intra-extradural lesion, moderately enhancing, which had eroded and enlarged the intervertebral foramen. The patient workup also included computed tomography scans and angiography. A posterior approach was used to perform a C2-3 hemilaminectomy, including opening of the dura mater and gross-total removal of the lesion. Histopathological examination of the lesion revealed it to be a malignant psammomatous melanotic schwannoma. The cerebrospinal MR image of the patient obtained at the 12-month follow-up examination demonstrated the presence of tumor progression into the subarachnoid space at the C-3 level. The strong malignancy potential of the lesion must be considered in the future management of the patient, especially due to the presence in the family of other stromal tumors such as gastrointestinal-stromal tumors and malignant melanomas. The authors review all the literature concerning melanotic schwannomas and report 105 cases of melanotic schwannoma that were not related to the Carney complex. The particular focus of their review is on the characteristics of the malignant progression of melanotic schwannoma, such as local recurrences, metastasis, and survival rate.