Brain and spine melanotic schwannoma: A rare occurrence and diagnostic dilemma

Abstract

Purpose Melanotic schwannoma (MS) was formerly known as a rare variant of schwannoma. The behavior of MS is unpredictable, with a tendency for recurrence and metastasis. The purpose of this study was to illustrate the imaging characteristics of these rare and misdiagnosed tumors. The prognosis of MS is discussed, along with the importance of follow-up exams to assess for recurrence and metastasis. Furthermore, we compare our results with those previously published on MS in order to have a better understanding of this rare entity. Methods Three MS cases were encountered between 2008 and 2015 at our institute. All available data were reviewed, including the clinical history, imaging findings, operative notes, and the histopathology results. A follow-up magnetic resonance imaging (MRI) scan was also reviewed up to 23 months post surgery to assess for recurrence. Results Three cases of MS are included: one in the brain and two in the spine. The brain lesion was in the occipital region. The spine lesions were thoracic intramedullary and sacral intradural. All cases were hypointense on T2-weighted imaging. Gross total resection was achieved for all lesions without adjuvant therapy. To date, the brain lesion recurred 15 months after surgery. Conclusions MS is a rare and distinct entity rather than a variant of schwannoma, and it poses both diagnostic and management dilemmas. Although MS has characteristic MRI features, including T1 and T2 shortening, the preoperative diagnosis is always challenging. Accurate diagnosis is crucial for management planning, including long-term follow-up exams to assess for recurrence and metastasis.