Exoftalmos y dolor ocular como presentación clínica de linfoma de Hodgkin con afectación intracraneal al diagnóstico / Exophthalmos and ocular pain as clinical debut of intracranial Hodgkin's lymphoma at diagnosis

La afectación intracraneal del linfoma de Hodgkin (LH) es extremadamente rara, especialmente como forma de presentación de la enfermedad. Muestra un patrón radiológico inespecífico, pudiendo ser confundido con otras entidades de mayor frecuencia y pronóstico radicalmente distinto. Anatomopatológicamente se caracteriza por la presencia de células grandes binucleadas (células de Reed-Sternberg) eIntracranial involvement in Hodgkin's lymphoma (HL) is extremely unusual, especially at the time of diagnosis. Because of its non-specific radiological behaviour, it can be confused with more common entities with a radically different prognosis. Pathologically, large and bi-nucleated cells, called Reed-Sternberg cells, embedded in an inflammatory network.

In this report we describe the clinical case of a patient, with no medical history, with left ocular pain and exophthalmos as presentation of intracranial HL at diagnosis and review the most current literature. Intracranial involvement is often associated with extracranial disease. Therefore, a systemic study including body computed tomography, bone marrow biopsy and ophthalmological evaluation is necessary. Intracranial lesions respond favourably to treatment and the prognosis depends on the extracranial involvement. To date, there is no standardised management scheme for these patients. For us, the primary role of surgery in this context is to perform a biopsy to confirm the histological diagnosis (AU)mbebidas en un entramado inflamatorio. Presentamos el caso de una paciente con dolor ocular y exoftalmos izquierdo como presentación clínica de afectación intracraneal por LH al diagnóstico de su enfermedad y revisamos la literatura más reciente al respecto. En pacientes con LH intracraneal es necesario realizar un estudio de extensión con tomografía computarizada corporal, biopsia de médula ósea y examen oftalmológico. Se asocia con gran frecuencia a enfermedad extracraneal, que marca el pronóstico. La lesión intracraneal presenta buena respuesta al tratamiento, que no sigue un esquema estandarizado. El papel de la cirugía es la realización de una biopsia para confirmar el diagnóstico (AU)

Exophthalmos and ocular pain as clinical debut of intracranial Hodgkin's lymphoma at diagnosis.

Intracranial involvement in Hodgkin's Lymphoma (HL) is extremely unusual, especially at the time of diagnosis. Because of its non-specific radiological behaviour, it can be confused with more common entities with a radically different prognosis. Pathologically, large and bi-nucleated cells, called Reed-Sternberg cells, embedded in an inflammatory network. In this report we describe the clinical case of a patient, with no medical history, with left ocular pain and exophthalmos as presetation of intracranial HL at diagnosis and review the most current literature. Intracranial involvement is often associated with extracranial disease. Therefore, a systemic study including body computed tomography, bone marrow biopsy and ophthalmological evaluation is necessary. Intracranial lesions respond favourably to treatment and the prognosis depends on the extracranial involvement. To date, there is no standardised management scheme for these patients. For us, the primary role of surgery in this context is to perform a biopsy to confirm the histological diagnosis.

Meningitis neoplásica en tumores sólidos: revisión actualizada de diagnóstico, pronóstico, manejo terapéutico y direcciones futuras / Neoplastic meningitis in solid tumours: Updated review of diagnosis, prognosis, therapeutic management, and future directions

La meningitis neoplásica (MN) es una complicación metastásica relativamente frecuente en pacientes con cáncer, con alta morbilidad neurológica y en general pobre pronóstico. La prevalencia está en torno al 5-15% de los tumores sólidos, y los más frecuentes son el cáncer de mama, el de pulmón y el melanoma. La clínica se produce por afectación de hemisferios cerebrales, nervios craneales, médula y raíces nerviosas, siendo en muchos casos multifocal, y presenta a menudo síntomas y signos de hipertensión intracraneal. Las principales herramientas diagnósticas son la exploración neurológica, la resonancia magnética cerebral y medular con contraste, y el análisis y la citología del líquido cefalorraquídeo (LCR), aunque recientemente se están investigando técnicas como la detección de células tumorales y ADN circulante en el LCR, que aumentan la sensibilidad diagnóstica. Con las terapias disponibles en la actualidad el objetivo del tratamiento no es curativo, sino retrasar y disminuir los síntomas y preservar la calidad de vida de los pacientes, e implica un enfoque multimodal que puede incluir radioterapia, quimioterapia intratecal y/o sistémica y cirugía. El tratamiento debe ser individualizado y se basa principalmente en guías y opiniones de expertos. Actualmente se están llevando a cabo ensayos clínicos prometedores de fármacos contra dianas moleculares e inmunoterápicos. Este artículo es una revisión actualizada de la MN, e incluye epidemiologia, presentación clínica, diagnóstico, pronóstico, manejo y opciones terapéuticas; se dirige al neurólogo general, y en particular al neurólogo que ejerce su práctica en centros con pacientes oncológicos. (AU)

Neoplastic meningitis (NM) is a relatively frequent metastatic complication of cancer associated with high levels of neurological morbidity and generally poor prognosis. It appears in 5%-15% of patients with solid tumours, the most frequent being breast and lung cancer and melanoma. Symptoms are caused by involvement of the cerebral hemispheres, cranial nerves, spinal cord, and nerve roots, and are often multifocal or present with signs and symptoms of intracranial hypertension. The main diagnostic tools are the neurological examination, brain and spinal cord contrast-enhanced magnetic resonance imaging, and cerebrospinal fluid analysis including cytology, although studies have recently been conducted into the detection of tumour cells and DNA in the cerebrospinal fluid, which increases diagnostic sensitivity. With the currently available therapies, treatment aims not to cure the disease, but to delay and ameliorate the symptoms and to preserve quality of life. Treatment of NM involves a multimodal approach that may include radiotherapy, intrathecal and/or systemic chemotherapy, and surgery. Treatment should be individualised, and is based mainly on clinical practice guidelines and expert opinion. Promising clinical trials are currently being conducted to evaluate drugs with molecular and immunotherapeutic targets. This article is an updated review of NM epidemiology, clinical presentation, diagnosis, prognosis, management, and treatment; it is aimed at general neurologists and particularly at neurologists practicing in hospital settings with oncological patients. (AU)

Neoplastic meningitis in solid tumours: updated review of diagnosis, prognosis, therapeutic management, and future directions.

Neoplastic meningitis (NM) is a relatively frequent metastatic complication of cancer associated with high levels of neurological morbidity and generally poor prognosis. It appears in 5%-15% of patients with solid tumours, the most frequent being breast and lung cancer and melanoma. Symptoms are caused by involvement of the cerebral hemispheres, cranial nerves, spinal cord, and nerve roots, and are often multifocal or present with signs and symptoms of intracranial hypertension. The main diagnostic tools are the neurological examination, brain and spinal cord contrast-enhanced magnetic resonance imaging, and cerebrospinal fluid analysis including cytology, although studies have recently been conducted into the detection of tumour cells and DNA in the cerebrospinal fluid, which increases diagnostic sensitivity. With the currently available therapies, treatment aims not to cure the disease, but to delay and ameliorate the symptoms and to preserve quality of life. Treatment of NM involves a multimodal approach that may include radiotherapy, intrathecal and/or systemic chemotherapy, and surgery. Treatment should be individualised, and is based mainly on clinical practice guidelines and expert opinion. Promising clinical trials are currently being conducted to evaluate drugs with molecular and immunotherapeutic targets. This article is an updated review of NM epidemiology, clinical presentation, diagnosis, prognosis, management, and treatment; it is aimed at general neurologists and particularly at neurologists practicing in hospital settings with oncological patients.

Ganglioglioma anaplásico: diseminación leptomeníngea cervico-dorso-lumbar. A propósito de un caso / Anaplasic ganglioglioma: Cervical-dorso-lumbar leptomeningeal dissemination. About a case

Los ganglioglioma son tumores bien diferenciados, de crecimiento lento, compuestos por una mezcla de células ganglionares maduras y gliales. La mayoría son de grado I de la OMS. Aparecen predominantemente en niños y adultos jóvenes. La mayoría se localizan a nivel del lóbulo temporal, y la sintomatología más frecuente son las crisis epilépticas de difícil control farmacológico. En general tienen buen pronóstico tras la resección quirúrgica. La variante anaplásica, considerada grado III de la OMS, presenta mayor agresividad clínica y radiológica. La diseminación leptomeníngea es excepcional en estos tipos de tumores, pero cuando es diagnosticada presenta un curso rápidamente progresivo y fatal para el paciente (AU)

Gangliogliomas are well-differentiated, slow-growing tumors. The majority are grade I of WHO. It appears predominantly in children and young adults. Most are located at the temporal lobe, and as symptomatology more frequent epileptic seizures of difficult pharmacological control. In general, they have a good prognosis after surgical resection. The anaplasic variant, considered to be grade III of the WHO, presents greater clinical and radiological aggressiveness. Leptomeningeal dissemination is exceptional in these types of tumors, but when diagnosed it presents a rapidly progressive and fatal course for the patient (AU)

Anaplasic ganglioglioma: Cervical-dorso-lumbar leptomeningeal dissemination. About a case. / Ganglioglioma anaplásico: diseminación leptomeníngea cervico-dorso-lumbar. A propósito de un caso.

Gangliogliomas are well-differentiated, slow-growing tumors. The majority are gradeI of WHO. It appears predominantly in children and young adults. Most are located at the temporal lobe, and as symptomatology more frequent epileptic seizures of difficult pharmacological control. In general, they have a good prognosis after surgical resection. The anaplasic variant, considered to be gradeIII of the WHO, presents greater clinical and radiological aggressiveness. Leptomeningeal dissemination is exceptional in these types of tumors, but when diagnosed it presents a rapidly progressive and fatal course for the patient.

Neoplastic meningitis in solid tumours: Updated review of diagnosis, prognosis, therapeutic management, and future directions. / Meningitis neoplásica en tumores sólidos: revisión actualizada de diagnóstico, pronóstico, manejo terapéutico y direcciones futuras.

Neoplastic meningitis (NM) is a relatively frequent metastatic complication of cancer associated with high levels of neurological morbidity and generally poor prognosis. It appears in 5%-15% of patients with solid tumours, the most frequent being breast and lung cancer and melanoma. Symptoms are caused by involvement of the cerebral hemispheres, cranial nerves, spinal cord, and nerve roots, and are often multifocal or present with signs and symptoms of intracranial hypertension. The main diagnostic tools are the neurological examination, brain and spinal cord contrast-enhanced magnetic resonance imaging, and cerebrospinal fluid analysis including cytology, although studies have recently been conducted into the detection of tumour cells and DNA in the cerebrospinal fluid, which increases diagnostic sensitivity. With the currently available therapies, treatment aims not to cure the disease, but to delay and ameliorate the symptoms and to preserve quality of life. Treatment of NM involves a multimodal approach that may include radiotherapy, intrathecal and/or systemic chemotherapy, and surgery. Treatment should be individualised, and is based mainly on clinical practice guidelines and expert opinion. Promising clinical trials are currently being conducted to evaluate drugs with molecular and immunotherapeutic targets. This article is an updated review of NM epidemiology, clinical presentation, diagnosis, prognosis, management, and treatment; it is aimed at general neurologists and particularly at neurologists practicing in hospital settings with oncological patients.

[Liposomal cytarabine for the treatment of leptomeningeal dissemination of central nervous system tumours in children and adolescents]. / Citarabina liposomal para el tratamiento de la diseminación leptomeníngea en tumores del sistema nervioso central en niños y adolescentes.

Leptomeningeal dissemination in paediatric central nervous system (CNS) tumours is associated with a poor outcome, and new therapeutic strategies are desperately needed. One of the main difficulties in the treatment of CNS tumours is blood brain barrier penetration. Intrathecal therapy has shown to be effective in several paediatric tumours. The aim of this article is to review the data available on the use of liposomal cytarabine for paediatric patients with leptomeningeal dissemination of CNS tumours, including the pharmacology, administration route, safety and efficacy data.