Suprasellar Melanocytoma with Leptomeningeal Seeding: An Aggressive Clinical Course for a Histologically Benign Tumor

Introduction. Meningeal melanocytoma (MM) is a very rare neuroectodermal neoplasm arising from the leptomeninges. Primary suprasellar melanocytomas are exceedingly rare, with only a handful of cases reported. The systemic spread of a nontransformed meningeal melanocytoma is an unusual occurrence. Herein, we report the first case of a primary sellar melanocytoma with cerebral and spinal meningeal seeding. Case Report. A 30-year-old male with no previous medical history presented to the endocrinology department with a loss of body hair. The endocrine workup concluded with isolated hypogonadotropic hypogonadism. The Magnetic Resonance Imaging (MRI) of the brain and sella revealed a large suprasellar mass continuous with the infundibulum of the pituitary gland. It was heterogeneously hyperintense on T1-, T2-, and FLAIR-weighted images and was enhanced with contrast, along with cerebral and spinal leptomeningeal spread. The patient was referred to the neurosurgery department, and a lumbar spine biopsy was indicated. The histopathological examination was suggestive of a grade I meningeal pigmented melanocytoma. Conclusion. Thus, primary sellar melanocytomas with leptomeningeal spread are an extremely rare phenomenon. Metastatic malignant melanoma should be ruled out. Being aware of differential diagnosis and the unusual behavior of meningeal melanocytoma will be necessary to manage the patient appropriately. Complete tumor resection is the best treatment whenever possible, and radiotherapy should be considered in case of unresectability or partial resection.

S-100-negative, GNA11 mutation-positive intramedullary meningeal melanocytoma of the thoracic spine: A radiographic challenge and histologic anomaly

Background: Intramedullary melanocytomas are exceedingly rare and their management is largely based on case reports and small clinical series. They have characteristic imaging and histologic findings that can aid in their diagnosis. Genetic testing may be required for definitive diagnosis and management guidance in ambiguous cases. Case Description: We present the case of a thoracic intramedullary meningeal melanocytoma in a patient unable to undergo an MRI. Conclusion: This is the first reported S-100-negative case with genetic testing to support the diagnosis of a rare intramedullary melanocytoma.

A National Cancer Database analysis of the patterns of care for meningeal melanocytoma

Aim: To evaluate demographics, treatment patterns, radiotherapy utilization and patient outcomes in meningeal melanocytomas. Materials & methods: The National Cancer Database was queried for meningeal melanocytomas diagnosed in 2002–2016. The effects of demographic, clinical and treatment variables were determined via Kaplan–Meier log-rank and Cox regression analyses. Results: The median and 5-year overall survival were 57.46 months and 48%, respectively. Patients earning ≥ $48K showed improved survival (p = 0.0319). Radiotherapy and chemotherapy were utilized in 37.7 and 9% of patients, respectively. Conclusion: Income significantly affected survival. Surgery remains the mainstay approach. Radiotherapy was delivered in more than one-third of patients but did not impact survival. However, further analyses were limited by poor treatment modality information in the database.

NCOG-57. NEUROPSYCHOLOGICAL FUNCTION IN MENINGEAL MELANOCYTOMA: A CASE REPORT

Meningeal melanocytomas are rare tumors derived from leptomeningeal melanocytes. The aggressive subtype is considered a rare variant of malignant melanoma, carrying a poor prognosis. To date, the extant literature is void of any studies that have systematically assessed cognition or neuropsychological function in these patients. We present a case report of a 61-year-old male, right-hand dominant, Caucasian male with 16 years of formal education. He has an unremarkable medical history, and his presenting symptom were syncopal episodes. An extensive work-up revealed a complex pineal region mass. Serum markers (CEA, AFP, and beta HcG) were negative. The patient continued to deteriorate neurologically and underwent a gross total resection of the mass. At the time of resection, there was evidence of definitive leptomeningeal dissemination. Because of the leptomeningeal spread, he underwent whole brain radiation therapy with a boost to the cerebellum and surgical site. He subsequently required implantation of a ventriculoperitoneal shunt for treatment of communicating hydrocephalus. Prior to WBRT, the patient underwent comprehensive neuropsychological testing. According to the patient and his spouse, they estimated functioning at approximately 50% of normal cognition, with precipitous decline noted prior to resection of the lesion. At the time of the exam, he was dependent in essentially all instrumental activities of daily living. The examination identified a pattern consistent with diffuse subcortical involvement, characterized by impairments in cognitive processing speed, attention, working memory and executive function. Conversely, the patient demonstrated no amnesia or higher cortical dysfunction. Moreover, mood, anxiety, or other psychopathology failed to account for his cognitive impairment. In our review, this is the first case in the literature of a patient with meningeal melanocytoma (with leptomeningeal spread) who has been comprehensively assessed neuropsychologically. Conceptualizations, treatment, and prognosis will be discussed.