Hemangiopericitoma intracraneano: Reporte de un caso y revisión del tema / Intracranial Haemangiopericytoma: Case report and literature review

INTRODUCCIÓN: el Hemangiopericitoma (HPC) es un tumor hipervascular infrecuente que constituye menos del 1% de todos los tumores del Sistema Nervioso Central (SNC) y aproximadamente el 3% de los tumores vinculados a las meninges. Son tumores agresivos que requieren un tratamiento enérgico mediante cirugía y radioterapia, y a pesar del mismo, generalmente son tumores recidivantes, pudiendo generar metástasis a distancia. OBJETIVOS: comunicar el primer caso en que se hace diagnóstico con un panel inmunohistoquímico completo de HPC en Uruguay, y a partir del mismo, se realiza una revisión bibliográfica. MATERIALES Y MÉTODO: en primer lugar se presenta un caso clínico de un paciente que fue asistido en el Hospital de Clínicas. Mediante estudios de imagen se sospecha probable HPC, es intervenido quirúrgicamente de coordinación y el diagnóstico definitivo se realiza mediante estudio de anatomía patológica que incluye técnica inmunohistoquímica. A partir de este caso clínico ilustrativo se realizó una puesta al día sobre esta patología. DISCUSIÓN: a nivel intracraneano, los HPC tienen una distribución similar a los meningiomas, con una base de implantación dural. Tal es su similitud con los meningiomas, que fueron considerados como una variante meningoblástica o angiomatosas de los mismos. Desde 1993 se los clasifica como un grupo aparte, presentando 2 variantes: clásica, de bajo grado (grado II) y anaplásico (grado III). El estudio histopatológico (morfológico e inmunohistoquimico) es fundamental ya que los diferencia definitivamente a los meningiomas de los HCP. En cuanto a los marcadores, y siguiendo la bibliografía referente, nuestro caso reportado fue negativo para CD 34 pero positivo para factor VIIIa. CONCLUSIONES: los HPC del SNC son tumores poco frecuentes, con un comportamiento agresivo, tendencia a recurrir y dar metástasis a distancia. Por lo que es fundamental tener siempre presente este tipo de lesiones en patología tumoral de meninges. Un diagnóstico rápido con estudio histológico y confirmación immunohistoquímica permite llegar al diagnostico y a un tratamiento oportuno agresivo, ya que el mismo es lo único que está demostrado que mejora el pronóstico.

INTRODUCTION: the haemangio-pericytoma (HPC) is a rare hypervascular tumor that accounts for less than 1% of all the Central Nervous System (CNS) tumor and approximately 3% of the tumors involving the meninges. They are aggressive tumors that require an energetic therapy including surgery and radiotherapy, despite which the tumors tend to relapse, potentially giving rise to distant metastases. OBJECTIVES: to report the first case where the diagnosis is reached with the comprehensive immuno-histochemistry panel for HPC in Uruguay, and literature review based on that case. MATERIALS AND METHOD: we describe the case of a patient treated at the Hospital de Clínicas. Imaging studies suggested a potential HPC; the patient underwent scheduled surgery and the final diagnosis was reached through the pathology assessment, using immunohistochemistry. A literature review of the condition is presented on the basis of the clinical report. DISCUSSION: at an intracranial level, HPCs have a distribution similar to that of meningiomas, with a dural implantation. They are so much alike meningiomas that they were considered to be a meningoblastic or angiomatous variant of those tumors. Since 1993 they are classified as a separate group with 2 variations: classical, low-grade (grade II) and anaplastic (grade III). The histology test (morphological and immunohistochemistry) is essential, since it reveals the differences between HCPs and meningiomas. As to markers, in accordance with the bibliography, our case was reported as negative for CD 34 but was positive for factor VIIIa. CONCLUSIONS: SNC HPCs are uncommon tumors that behave aggressively; they tend to relapse and cause distant metastases. Hence, it is always crucial to bear this diagnosis in mind when facing a meningeal tumor. A quick diagnosis including histology and immuno-histochemical confirmation allows for a timely aggressive therapy- the only thing that improves prognosis.

Meningeal Solitary Fibrous Tumor

We report a rare case of a patient with meningeal solitary fibrous tumor. A 60-year-old woman presented with right leg monoparesis. Brain magnetic resonance imaging demonstrates a well enhancing huge mass, located in left parietal lobe. Cerebral angiography demonstrating increased vascularity in area of the tumor, which had feeder vessels extending from the internal carotid artery and external carotid artery. A presumptive diagnosis of meningioma or hemangiopericytoma was considered. At surgery, the consistency was firm and had destroyed the dura and skull. A gross total resection was performed. Immunohistochemically, tumor was strongly, and widely, positive for CD34 and vimentin. There was no staining for epithelial membrane antigen(EMA), S-100 protein, cytokeratin, and glial fibrillary acidic protein (GFAP). Differential diagnosis of intracranial solitary fibrous tumor includes fibroblastic meningioma, meningeal hemangiopericytoma, neurofibroma, and schwannoma.

Spinal Cord Tumors: An Analysis of 654 Cases(1973-1999)

OBJECTIVE: The spinal cord tumors(including vertebral tumors) are increasingly diagnosed and operated due to development of refined diagnostic and therapeutic tools. It is necessary to re-evaluate clinical features and surgical results of spinal cord tumors with increasing cases and developing treatment modalities. The authors reviewed the spinal cord tumor cases to evaluate their clinical characteristics. MATERIALS AND METHODS: The retrospective review of 654 cases of spinal cord tumors between 1973 and 1999 was done. The clinical features, pathological analysis and surgical results were analyzed and compared to the literature. The results of the study are analyzed with a more detailed consideration of each of major pathologies: neurogenic tumors, meningeal tumors, neuroepithelial tumors, and metastatic tumors. RESULTS AND CONCLUSION: The spinal cord tumor was most common in the 5th decade of age(145 cases, 22.1%) and 78 cases(11.9%) were found in children under 15 years of age. The ratio of male to female was 1.2:1. The pathologic diagnosis was neurogenic tumor in 266 cases(40.7%), neuroepithelial tumor in 131(20.0%), metastatic tumor in 118(18.0%), and meningeal tumor in 94(14.4%) in the order of frequency. The tumor was located most frequently in the thoracic area(36.5%) and in the intradural extramedullary space(38.1%). The most common initial presentation was pain(40.1%) and the mean duration for presentation to operation was 14.8 months. The total or gross total removal was possible in 404 cases(61.7%) and the surgical result on the postoperative one month was recovery or improvement in 424 cases(64.8%), stationary in 188(28.7%), progression in 42(6.4%). As a surgical complication, there was a spinal deformity(12 cases), wound infection(5 cases), aspiration pneumonia(5 cases) etc. Neurogenic tumors and menigiomas showed good surgical results, whereas neuroepithelial tumors(except ependymoma) and metastatic tumors showed relatively poor prognosis.

Spinal Cord Tumors : An Analysis of 175 Cases(1973-1986)

The authors analyzed 175 cases of spinal cord tumor who had been operated upon at the Department of Neurosurgery of the Seoul National University Hospital from 1973 to 1986. The tumor was most common in the 5th decade of age(47 cases, 26.9%) and 24 cases(13.7%) were children. And the ratio of male to female was 1.2:1. The pathologic diagnosis was neurogenic tumor in 73 cases(41.7%), metastatic tumor in 29 cases(16.6%), glial tumor in 24 cases(13.7%), and meningeal tumor in 23 cases(13.1%) in the order of frequency, and the tumors of dysembryogenesis were only 2 cases(1.1%). The tumors were located most frequently in the thoracic area(79 cases, 45.1%) and I the intradural extramedullary space(79 cases, 45.1%). The most common initial presentation was pain(86 cases, 49.1%) and the mean duration of presentation was 20.6 months. On admission 70 cases(40.0%) were in the 'paraparesis stage'. The mean value of the CSF protein was 741mg%. The rate of positivity of the plain spinal radiologic study was 55.7% and the most common positive findings was the increased interpedicular distance(46 cases, 29.1%). the total or gross total removal was possible in 105 cases (60.0%). The surgical result within the postoperative one month was recovery in 52 cases(29.7%), improvement in 76 cases(43.4%), stationary state in 36 cases (20.6%), progression in 11 cases(6.3%). After 20.1 months of the mean follow-up duration in 109 cases(62.3%), the result was recovery in 69 cases(63.3%), improvement in 16 cases(14.7%), stationary state in 8 cases(7.3%), progression in 16 cases(14.7%). As a major complication, there was a spinal deformity in 6 cases(3.4%), pulmonary embolism in 2 cases(1.1%), postoperative hematoma and meningitis in 1 case(0.6%) each. Ten cases(5.7%) were followed up to their deaths. And the authors discussed the clinical features of the spinal cord tumors reported in the literatures and the results of the present study with a more detailed consideration on each of major pathologies, neurogenic tumors, meningeal tumors, glial tumors, and metastatic tumors.