Variante amelanótica en melanoma cerebral primario: a propósito de un caso / Primary intracranial melanoma, amelanotic variant: Case report

El melanoma cerebral primario es un tumor muy infrecuente (0,07% de las neoplasias primarias del SNC). Generalmente muestra un abundante contenido en melanina, y solo en contadas ocasiones se han descrito variantes hipoamelanóticas. Presentamos el caso de una paciente con clínica de cefalea, paresia braquial izquierda y síndrome lobar frontal. La RM mostró una masa frontal derecha con captación homogénea de contraste. Como tratamiento, se realizó una resección quirúrgica completa. El estudio anatomopatológico fue diagnóstico para melanoma, con muy escaso contenido en melanina y alto índice proliferativo. Se realizó un estudio de extensión exhaustivo para descartar otra localización primaria. Debido a varias complicaciones intercurrentes, la paciente evolucionó desfavorablemente, sin llegar a recibir otros tratamientos. La variante amelanótica de los melanomas cerebrales primarios no ha sido descrita con detalle previamente. Repasamos la literatura al respecto y discutimos los detalles de manejo y diagnóstico de esta entidad clínica (AU)

Primary brain melanoma is a very rare tumour (only 0.07% of primary CNS neoplasms) which usually shows with abundant melanin content; whereas hypo/melanotic variants have been scarcely described. We introduce the case of a female patient with headache, left brachial paresis and frontal lobar syndrome. The MRI image showed a right frontal mass with homogeneous contrast uptake. As treatment, a complete surgical resection was performed. Pathology was diagnostic for melanoma, with very low melanin content and a high proliferative index. A thorough extension study was performed to rule out an extracranial primary origin. Due to several intercurrent complications, the patient evolved unfavorably, not being able to receive further treatment. The amelanotic variant of primary intracranial malignant melanomas has not been described in detail previously. We will review the literature, focusing on the particularities of management and diagnosis of this clinical entity (AU)

Primary intracranial melanoma, amelanotic variant: Case report.

Primary brain melanoma is a very rare tumour (only 0.07% of primary CNS neoplasms) which usually shows with abundant melanin content; whereas hypo/melanotic variants have been scarcely described. We introduce the case of a female patient with headache, left brachial paresis and frontallobar syndrome. The MRI image showed a right frontal mass with homogeneous contrast uptake. As treatment, a complete surgical resection was performed. Pathology was diagnostic for melanoma, with very low melanin content and a high proliferative index. A thorough extension study was performed to rule out an extracranial primary origin. Due to several intercurrent complications, the patient evolved unfavorably, not being able to receive further treatment. The amelanotic variant of primary intracranial malignant melanomas has not been described in detail previously. We will review the literature, focusing on the particularities of management and diagnosis of this clinical entity.

Glioblastoma with primitive neuronal component: A case report and considerations of fluorescence-guided surgery.

BACKGROUND: Glioblastoma with primitive neuronal components (GB/PNC) is an extremely rare type of glioblastoma characterized by presenting histological and cytogenetic features of both entities. The mixed nature of these tumors limits the imaging diagnosis and supposes a therapeutic dilemma. CASE DESCRIPTION: We present the case of a 77-year-old female with a GB/PNC who is treated with surgery and adjuvant radiochemotherapy according to the STUPP protocol, where an abnormal uptake of 5-aminolevulinic acid (5-ALA) is evident during surgery in probable relation to the mixed nature of GB/PNC. CONCLUSION: GB/PNC is extremely rare tumors. Given its low prevalence, there are no studies that refer to the macroscopic characteristics of the tumor as well as evidence of the effectiveness of adjuvant treatment. Fluorescence-guided resection with 5-ALA is the surgical treatment of choice in surgery for high-grade gliomas; however, in GB/PNC, it may not be as useful since PNC may have less fluorescent marker uptake and be more dimly visualized when excited by light using the surgical microscope.