Objective: Meningeal melanocytomas of the central nervous system are extremely rare, with an incidence of 1 per 10 million individuals. Cases of primary cerebellopontine angle melanocytoma (PCPAM) have only been described in single case reports. The goal of the present study was to analyze the surgical management of PCPAM, with a particular focus on early and late treatment outcomes and recurrence rates.
Methods: The patients who had undergone surgery for PCPAM from January 2004 to May 2018 were identified by a local database query. The patients were evaluated for initial symptoms, pre- and postoperative facial and cochlear nerve function, complications, and recurrence rate by reviewing the patients' medical records.
Results: We identified 4 patients with PCPAM of >1500 cerebellopontine angle lesions (∼0.2%) that had been surgically treated at our department in the past 14 years. Of the 4 patients, 2 were men and 2 were women, with a mean age of 47 years. Anatomical facial and cochlear nerve preservation was achieved in all 4 patients. One patient experienced a new moderate facial palsy immediately after surgery (House-Brackmann grade III). Of the 4 patients, 3 had undergone radiotherapy and 1 had undergone ion beam therapy for tumor recurrence (6 years after surgery). Of the 4 patients, 3 had presented with tumor recurrence at 2, 3, and 6 years of follow-up respectively. The long-term follow-up examination had not yet been conducted for 1 patient.
Conclusions: At long-term follow-up, 3 patients had developed recurrence. Because of the high recurrence rate of PCPAM, we believe that radiotherapy in addition to surgery should be considered in the future to avoid early recurrence.
Keywords: BRAF; Black tumor; Cerebellopontine angle; Melanocytoma of intermediate grade; Meningeal melanocytoma; Pigmented tumor; V600E mutation status.
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