PMTNS are thought to be an exceedingly rare group of tumors not captured by tumor registries. The study was done to determine the relative incidence, clinical presentation, diagnostic findings, patient management, and outcome.
The researchers retrospectively searched the Section of Neuro‐Oncology database at the Yale Cancer Center. It particularly for patients with primary or metastatic melanotic lesions of the nervous system. The researchers recorded demographic data, clinical presentation, histopathological and imaging findings, therapy, and outcome for patients with PMTNS.
One hundred sixteen patients in total with melanocytic lesions were identified, including four patients with PMTNS. The incidence of PMTNS was therefore calculated as 3.4%. Histology of PMTNS patients revealed melanocytoma in 3 patients and psammomatous melanotic schwannoma in one patient. Symptoms attributed to the mass tumor effect. MRI showed hyperintensity on pre‐contrast T1‐weighted imaging, hypointensity on T2‐weighted imaging, and homogeneous contrast enhancement in all PMTNS patients.
The study concluded that the nervous system’s primary melanotic tumors are rare jumpy system tumors. The outcome appears excellent, and complete surgical resection may form the basis for a favorable outcome. Radiation therapy may represent a therapeutic approach for residual or relapsed disease.
Reference: https://onlinelibrary.wiley.com/doi/10.1111/ene.14437
