Importancia del diagnóstico histopatológico en el tratamiento de las lesiones del tronco cerebral en adultos / Relevance of histopathological diagnosis in the treatment of brainstem lesions in adults

Los tumores del tronco cerebral son infrecuentes en la población adulta. Las controversias surgen cuando se considera la necesidad de confirmar el diagnóstico histopatológico en esta área elocuente del cerebro, balanceando los beneficios de obtener un diagnóstico certero y las desventajas de los procedimientos invasivos. Existen escasas publicaciones acerca de su tratamiento quirúrgico en adultos, todas series pequeñas analizadas retrospectivamente. Presentamos nuestra experiencia con el propósito de contribuir al proceso de toma de decisiones. Diez de 13 pacientes fueron intervenidos. Las lesiones se clasificaron en focales (n:7), infiltrativa difusa (n:1), tectal (1) y exofítica (1). El estado neurológico según la escala Karnofsky Performance Status fue ≥ 70 en 6 casos y < 70 en 7. Las muestras fueron obtenidas mediante abordaje microquirúrgico directo o por biopsia estereotáctica. Los hallazgos histopatológicos fueron confirmados en todos los casos: astrocitoma pilocítico (n:1), glioma de bajo grado (n:1), glioblastoma (n:1), hemangioblastoma celular (n:1), subependimoma (n:1), disgerminoma (n:1), y lesiones pseudotumorales (n:4, 3 cavernomas, 1 pseudotumor inflamatorio). La amplia variedad de hallazgos patológicos en esta localización en adultos exige una precisa definición histopatológica, que no solo determina la terapéutica adecuada sino que también previene las consecuencias potencialmente catastróficoas de los tratamientos empíricos.

Brainstem tumors are uncommon beyond childhood. Controversies arise regarding the need of histological diagnosis in this eloquent area of the brain, weighting the benefits of a reliable diagnosis against the disadvantages of invasive procedures. There are scant publications about the surgical management of brainstem tumors in adults, all of them involving small retrospective cohorts. We are reporting our experience with the aim of contributing to the decision making process. Out of a series of 13 patients, 10 were approached surgically. According to Guillamo´s classification the lesions were: focal (n:7), diffuse infiltrative (n:1), tectal (n:1), and exophytic (n:1). According to the Karnofsky Performance Status scale, the neurological status was ≥ 70 in 6 cases and < 70 in 7. Histopathology was confirmed in all 10 treated cases and the samples were obtained by a direct microsurgical approach or by stereotactic biopsy. Histopathological findings were: pilocytic astrocytoma (n:1), low grade glioma (n:1), glioblastoma (n:1), cellular haemangioblastoma (n:1), subependimoma (n:1), pseudotumoral lesions (n:4; 3 cavernomas, 1 inflammatory pseudotumor), and disgerminoma (n:1). As a broad variety of pathologies could be found in this brain localization, an accurate histopathological definition can not only determine the adequate therapy, but also avoid the disastrous consequences of empiric treatments.

Tumefactive demyelinating lesions: A Clinicopathological correlative study.

Tumefactive demyelinating (TDL) lesions are focal zones of demyelination in the central nervous system and they often mimic the neuroimaging features of an intraxial neoplasm. In this report we describe the clinical, neuroimaging and neuropathological features of six cases of TDL. Only in two patients the neuroimaging features in MRI (magnetic resonance imaging) scans were suggestive of TDL while in the other four cases a diagnosis of glioma was suggested. In order to establish a confirmatory diagnosis neuronavigation/stereotactic biopsy was undertaken and the diagnosis of TDL was established in all six cases at histopathology. Two out of six patients did not respond to the conventional corticosteroid therapy and they were treated with plasma exchange. It is being concluded that neuronavigation biopsy, though provide only a small amount of tissue, and is extremely useful in making the diagnosis of TDL.

Brainstem cavernomas: a surgical challenge / Cavernomas de tronco: desafio cirúrgico

Objective: The authors show their experience with brainstem cavernomas, comparing their data with the ones of a literature review. Methods: From 1998 to 2009, 13 patients harboring brainstem cavernomas underwent surgical resection. All plain films, medical records and images were reviewed in order to sample the most important data regarding epidemiology, clinical picture, radiological findings and surgical outcomes, as well as main complications. Results: The mean age was 42.4 years (ranging from 19 to 70). No predominant gender: male-to-female ratio, 6:7. Pontine cases were more frequent. Magnetic resonance imaging was used as the imaging method to diagnose cavernomas in all cases. The mean follow-up was 71.3 months (range of 1 to 138 months). Clinical presentation was a single cranial nerve deficit, VIII paresis, tinnitus and hearing loss (69.2%). All 13 patients underwent resection of the symptomatic brainstem cavernoma. Complete removal was accomplished in 11 patients. Morbidity and mortality were 15.3 and 7.6%, respectively. Conclusions: Cavernomas can be resected safely with optimal surgical approach (feasible entry zone) and microsurgical techniques, and the goal is to remove all lesions with no cranial nerves impairment.

Objetivo: Os autores mostram sua experiência com cavernomas de tronco cerebral, comparando seus dados com os de uma revisão da literatura. Métodos: De 1998 a 2009, 13 pacientes com cavernoma de tronco cerebral foram submetidos a ressecção cirúrgica. Todos os filmes, prontuários e imagens foram revisados para exposição dos dados mais importantes, como epidemiologia, detalhes clínicos, achados radiológicos e resultados cirúrgicos, bem como as principais complicações. Resultados: A média de idade foi de 42,4 anos (variação de 19 a 70). Não houve predominância de gênero na taxa masculino versus feminino, 6:7. Os casos pontinos foram os mais frequentes. Ressonância nuclear magnética foi o método de imagem para o diagnóstico de cavernomas em todos os casos. A média do acompanhamento foi de 71,3 meses (variação de 1 a 138 meses). A apresentação clínica mais frequente foi a paresia do VIII nervo craniano, tinitus e perda auditiva (69,2%). Todos os 13 pacientes com cavernomas de tronco sintomáticos foram submetidos à ressecção cirúrgica. A remoção total foi realizada em 11 pacientes. A morbidade e a mortalidade foram de 15,3 e 7,6%, respectivamente. Conclusão: Os cavernomas podem ser seguramente ressecados por meio de acessos cirúrgicos ideais (zonas de entrada seguras) e técnicas de microcirurgia, sendo que o objetivo é remover toda a lesão sem o comprometimento dos nervos cranianos.

Tectal plate tumors

Tumores na região do teto mesencefálico são raros. Vários tipos de lesões como tumores, lesões vasculares, inflamatórias e infecciosas localizam-se nesta região. Nós revimos o tratamento adotado em sete pacientes com diferentes tipos de lesões tectais: cinco pacientes apresentando gliomas de baixo grau, um paciente com lesão metastática proveniente de câncer de pulmão e um com cavernoma. O tratamento cirúrgico com abordagem direta da lesão foi realizado em três casos (devido ao aumento do volume tumoral ou quando houve necessidade da confirmação diagnóstica). Nos demais casos o tratamento para a hidrocefalia não-comunicante foi o método empregado. O prognóstico dessas lesões é baseado no tipo de patologia em questão. Em nossa série, com exceção do caso de metástase e do paciente com cavernoma, as demais lesões foram gliomas de baixo grau. Estas lesões representam um subgrupo diferenciado de tumores de tronco encefálico, apresentando bom prognóstico e tendo comportamento benigno com sobrevida elevada. Acreditamos que tumores da região tectal devam ser avaliados caso a caso. Na hipótese diagnóstica de uma lesão benigna, o tratamento do principal complexo sindrômico – hidrocefalia não-comunicante – é provavelmente a melhor conduta a ser empregada.

Non-Hodgkin's lymphoma of the brainstem with atrial septal mass.

Simultaneous occurrence of cardiac and central nervous system tumors have been rarely reported. A 23 years male presented to us with right cerebello-pontine (CP) angle symptoms and signs. Cranial imaging showed a mass lesion in the right pons infiltrating into the right and middle cerebellar peduncles. There was also a cardiac-atrial septal mass. The brainstem lesion was found to be a non-Hodgkin's lymphoma where as the cardiac lesion was not accessible. Central nervous system lymphomas are reported rarely and the prognosis is poor. The chances of the cardiac lesion in this patient also being a lymphoma are high, as it was a infiltrative cardiac mass, infiltrating the atrial septum.

Multiple cranial neuropathy: a common diagnostic problem.

Syndrome of multiple cranial palsies is a common clinical problem routinely encountered in neurological practice. Anatomical patterns of cranial nerves involvement help in localizing the lesion. Various infections, malignant neoplasms and autoimmune vasculitis are common disorders leading to various syndromes of multiple cranial nerve palsies. A large number of diffuse neurological disorders (e.g. Gullian-Barre syndrome, myopathies) may also present with syndrome of multiple cranial nerve palsies. Despite extensive biochemical and radiological work-up the accurate diagnosis may not be established. Few such patients represent "idiopathic" variety of multiple cranial nerve involvement and show good response to corticosteroids. Widespread and sequential involvements of cranial nerves frequently suggest possibility of malignant infiltration of meninges, however, confirmation of diagnosis may not be possible before autopsy.