RESUMO
BACKGROUND: Recent data has raised skepticism regarding the long-term effectiveness of radiotherapy (RxT) in acromegaly and its role as an ancillary tool to neurosurgery (Tx). PATIENTS: We evaluated 72 acromegalic patients previously submitted to RxT. Data were discarded in 23 patients, who were lost to follow-up, operated on after RxT or irradiated with techniques different from external conventional fractionated RxT. Among the remaining 49 (five with mixed GH-prolactin adenoma), 34 were irradiated after surgical failure and 15 as primary treatment. A second cycle of RxT was administered in two. RESULTS: (i) GH/IGF-I. After a median follow-up of 14 years (range 3-41), normal age-matched IGF-I levels were reached in eight patients (16%) after 10 years, and GH levels <2.5 microg/l in six (12%) after 9 years. The rate of persistently pathological hormonal levels was still 90% at 25 years. All patients with GH/IGF-I normalization had undergone irradiation without any antisecretory drug. Neither basal GH nor tumor size affected the outcome of RxT. In three patients (6%) a relapse/worsening occurred. (ii) Tumor size. Tumor shrank after 8.5 years in 24 patients (49%), in nine of whom during GH-suppressive treatment. Tumor shrinkage was not predictive of hormonal normalization. (iii) Side-effects. Hypopituitarism was diagnosed in four patients (selective in three and global in one) and GH deficiency in one. Three patients had neurological side-effects and meningioma was shown in two patients. CONCLUSION: RxT is unable to cure acromegaly, because it seldom achieves hormonal normalization even after a very prolonged follow-up. Concomitant antisecretory treatment seems to counteract its effects. RxT can still play a role in those patients with large tumor remnants, because of its capacity to shrink tumor size.
Assuntos
Acromegalia/radioterapia , Falha de Tratamento , Adenoma/patologia , Adenoma/radioterapia , Adolescente , Adulto , Idoso , Fracionamento da Dose de Radiação , Feminino , Hormônio do Crescimento Humano/análise , Hormônio do Crescimento Humano/sangue , Hormônio do Crescimento Humano/metabolismo , Humanos , Fator de Crescimento Insulin-Like I/análise , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Neoplasias Hipofisárias/metabolismo , Neoplasias Hipofisárias/patologia , Neoplasias Hipofisárias/radioterapia , Prolactinoma/patologia , Prolactinoma/radioterapia , Radioterapia/efeitos adversos , Estudos RetrospectivosRESUMO
OBJECTIVE AND DESIGN: A decrease of GH levels below 2 microg/l after an oral glucose tolerance test (OGTT) is still currently accepted as the gold standard for assessing cure in surgically treated acromegaly. Whether glucose-induced suppression of GH is accompanied by a restoration of normal GH late rebound has not yet been evaluated in this disease. In order to assess the restoration of normal GH regulation after removal of a pituitary adenoma, we have evaluated GH changes after an OGTT in a series of selected acromegalic patients (transsphenoidal surgery and lack of pituitary failure). METHODS: Twenty-nine patients (13 male, 16 female, age range 27-70 years) entered the study. Their neuroradiological imaging before neurosurgery showed microadenoma in 7, intrasellar macroadenoma in 8 and macroadenoma with extrasellar extension in 14. Plasma GH levels were assayed up to 300 min after glucose administration (75 g p.o.) and IGF-I on basal samples. RESULTS: Basal GH levels were below 5 microg/l in 20 patients and below 2 microg/l in 5 of these. Normal age-adjusted IGF-I levels were observed in 12 patients. GH values were suppressed below 2 microg/l during an OGTT in 13 patients, and below 1 microg/l in 7 of these. In 9 patients out of these 13, a marked rise in GH levels occurred after nadir. Baseline and nadir GH values of these 9 patients were not different from the corresponding values of the other 4 patients without OGTT-induced late GH peaks. CONCLUSIONS: GH rebound after GH nadir occurs in acromegalic patients considered as cured on the basis of OGTT-induced GH suppression and/or IGF-I normalization. The restoration of this physiological response could be regarded as a marker of recovered/preserved integrity of the hypothalamic-pituitary axis. Even though the reason for this GH rebound has not yet been elucidated (GHRH discharge?/end of somatostatin inhibition?), the lack of late GH peak in the patients regarded as cured by the usual criteria could be due to injury to the pituitary stalk caused by the adenoma or by surgical manipulation.
Assuntos
Acromegalia/cirurgia , Hormônio do Crescimento/sangue , Acromegalia/sangue , Acromegalia/fisiopatologia , Adulto , Idoso , Glicemia/análise , Feminino , Teste de Tolerância a Glucose , Humanos , Fator de Crescimento Insulin-Like I/análise , Masculino , Pessoa de Meia-Idade , Fatores de Tempo , Resultado do TratamentoRESUMO
The effects of positive end-expiratory pressure (PEEP) on central venous and intracranial (ICP) pressures were evaluated in 10 patients with posterior fossa tumors, in both supine and sitting positions. With patients in the supine position, intrathoracic PEEP-dependent venous hypertension was clearly transmitted to the intracranial compartment but without intracranial hypertension. On the contrary, with patients in the sitting position PEEP had no influence in almost half of our patients. In patients with radiological or clinical signs of increased ICP, however, the combination of head flexion and rotation with institution of PEEP caused a dangerous increase in ICP, even when the patients were in the sitting position. The need for early withdrawal of cerebrospinal fluid in these patients is stressed.
Assuntos
Neoplasias Encefálicas/cirurgia , Pressão Intracraniana , Complicações Intraoperatórias/etiologia , Respiração com Pressão Positiva , Adolescente , Adulto , Criança , Fossa Craniana Posterior/cirurgia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Papiledema/etiologia , PosturaRESUMO
The clinical correlation between the degree of HCV variability and the response to anti-HCV treatment in HIV positive patients infected with HCV genotype 3a is unknown. In this study, 27 HIV positive and 5 HIV negative patients with HCV genotype 3a infection were treated with interferon-alpha-2b with or without ribavirin. Nine patients (5 HIV positive) achieved a sustained virological response (SR) and 23 (only one HIV negative) were non-responders (NR). Sequence analyses of the partial E2 domain and the non-structural 5A protein were performed at baseline in all patients, and before and during treatment in the HIV positive NRs. There was no difference in the mean number of amino acid mutations from HCV 3a prototype, within E2 region, between the HIV positive and HIV negative patients: 17 (range 11-25) vs 16 (range 14-17). The mean baseline number of mutations in E2 region, was similar in HIV positive SRs and NRs: 18 (range 14-25) vs 16 (range 11-19). Phylogenetic analysis of HCV paired serum samples at baseline and during treatment revealed identical E2 sequence in 5/21 HIV positive NR patients, whereas 6 other sequences were strictly related to baseline E2 domain and the remaining 10 were divergent. The mean number of amino acid mutations in the NS5A protein at baseline, was 1 (range 0-3) in HIV negative patients and 2 (range 0-4) in HIV positive ones. This region was highly conserved in all isolates of HIV positive NRs analysed during treatment. These results suggest that genetic variability at baseline within the E2 region and NS5A protein of HCV 3a strain obtained from HIV positive and HIV negative patients is not associated with treatment response. Furthermore, the anti-HCV treatment did not influence HCV heterogeneity within the E2 and NS5A domains in HIV positive patients infected with HCV genotype 3a.
Assuntos
Heterogeneidade Genética , Infecções por HIV/complicações , Hepacivirus/genética , Hepatite C/tratamento farmacológico , Adulto , Alanina Transaminase/sangue , Sequência de Aminoácidos , Contagem de Linfócito CD4 , Análise Mutacional de DNA/métodos , Feminino , Genótipo , Hepacivirus/classificação , Hepacivirus/efeitos dos fármacos , Hepatite C/complicações , Hepatite C/virologia , Humanos , Interferon alfa-2 , Interferon-alfa/uso terapêutico , Masculino , Pessoa de Meia-Idade , Dados de Sequência Molecular , Mutação/genética , Filogenia , RNA Viral/sangue , RNA Viral/efeitos dos fármacos , Proteínas Recombinantes , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Ribavirina/uso terapêutico , Alinhamento de Sequência , Homologia de Sequência de Aminoácidos , Resultado do Tratamento , Proteínas do Envelope Viral/genética , Carga Viral/métodos , Proteínas não Estruturais Virais/genética , Proteínas Virais/efeitos dos fármacos , Proteínas Virais/genéticaRESUMO
The amino terminal region of the non structural gene 3 (NS3) of hepatitis C virus (HCV) is a chymotripsinlike serine-protease responsible for cleavage of the non structural proteins of Hepatitis C virus (HCV). In order to investigate the genetic variation of this region, we developed a nested PCR to obtain NS3 protease sequences from 54 patients chronically infected with HCV genotypes 1a, 1b and 3, respectively. Comparison of nucleotide and amino acids sequences of NS3 protease domain with consensus sequence obtained within the same genotype, showed 3.73% nucleotide divergence and 1.64% amino acid divergence in isolates of genotype 3a, whereas isolates 1a exhibited 4.45% nucleotide and 4% amino acid change, respectively. Finally, NS3 sequence from 1b isolates revealed 6.47% nucleotide and 3.5 % aa changes. Comparison of consensus amino acid sequences derived from isolates 1a, 1b and 3, with the HCV prototypes showed a low amino acid sequence diversity. However, the consensus sequence of HCV genotype 3 isolates showed an amino acid changed from the prototype, that was located within a region important for enzyme structure and activity. These results indicated that the NS3 protease gene is highly conserved within the same HCV genotype. The domains involved in enzyme function were highly conserved in 1a and 1b strains, whereas consensus sequence of isolates 3a showed that the majority of these strains were not perfectly conserved in one of such regions. These findings altogether suggested that the NS3 protease enzyme of HCV may constitute an important target for antiviral therapy, but the NS3 protease variability of isolates 3 within a region that is a potential target for antiviral therapy could pose a problem for structure based drug development.
Assuntos
Hepacivirus/genética , Hepatite C/virologia , Proteínas não Estruturais Virais/genética , Adulto , Sequência de Aminoácidos , Substituição de Aminoácidos/genética , Substituição de Aminoácidos/fisiologia , Domínio Catalítico/genética , Sequência Consenso/genética , Sequência Conservada/genética , Sequência Conservada/fisiologia , DNA Complementar/química , DNA Complementar/genética , Feminino , Genótipo , Hepacivirus/classificação , Humanos , Masculino , Pessoa de Meia-Idade , Dados de Sequência Molecular , Filogenia , RNA Viral/sangue , RNA Viral/isolamento & purificação , Reação em Cadeia da Polimerase Via Transcriptase Reversa/métodos , Alinhamento de Sequência , Análise de Sequência de DNA , Homologia de Sequência de Aminoácidos , Serina Endopeptidases/genéticaRESUMO
A surgical series of 59 patients with cerebellar or spinal cord hemangioblastomas or von Hippel-Lindau's (VHL) syndrome is analyzed. The presence of the tumor is easily detected by Computerized Tomography (CT) and Nuclear Magnetic Resonance (NMR), but angiography is still necessary for a correct surgical planning. The value of a sharp distinction among patients with single hemangioblastomas and the ones with Lindau's disease and VHL syndrome is stressed. In fact patients with single cerebellar or spinal hemangioblastomas have a good prognosis, while patients with disseminated hemangioblastomas have a rather poor outlook. Neuropathological studies with immunohistochemical techniques have been performed to identify the nature of the stromal cells of the hemangioblastomas: their origin from glial, endothelial and monociticphagocitic elements seems excluded.
Assuntos
Neoplasias Cerebelares/fisiopatologia , Neoplasias Cerebelares/cirurgia , Hemangiossarcoma/fisiopatologia , Hemangiossarcoma/cirurgia , Neoplasias da Medula Espinal/fisiopatologia , Neoplasias da Medula Espinal/cirurgia , Doença de von Hippel-Lindau/cirurgia , Adolescente , Adulto , Idoso , Neoplasias Cerebelares/patologia , Criança , Diagnóstico Diferencial , Feminino , Seguimentos , Proteína Glial Fibrilar Ácida/análise , Hemangiossarcoma/patologia , Humanos , Imuno-Histoquímica , Espectroscopia de Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Muramidase/análise , Estudos Retrospectivos , Neoplasias da Medula Espinal/patologia , Vimentina/análise , alfa 1-Antiquimotripsina/análise , alfa 1-Antitripsina/análise , Doença de von Hippel-Lindau/patologia , Doença de von Hippel-Lindau/fisiopatologia , Fator de von Willebrand/análiseRESUMO
The clinical, radiological and histological findings of a huge olfactory grooves meningioma in a 14 years-old girl are described. Good result after complete removal is stressed and concerning literature is reviewed.
Assuntos
Neoplasias Meníngeas/cirurgia , Meningioma/cirurgia , Adolescente , Feminino , Humanos , Neoplasias Meníngeas/diagnóstico por imagem , Meningioma/diagnóstico por imagem , Bulbo Olfatório , RadiografiaRESUMO
An interaction between the protein kinase (PKR)-eIF2-alpha phosphorylation homology domain (PePHD) within the E2 protein of hepatitis C virus (HCV) and cell protein kinase (PKR) may affect the control of protein synthesis and cell growth. In an attempt to investigate the genetic variability of the E2-PePHD domain in hepatocellular carcinoma (HCC), we studied sera and liver tissues from HCC patients. The partial E2-PePHD region was analysed by direct sequencing of the sera of 47 HCCs in cirrhotic livers and 31 cases of chronic active hepatitis (CAH), and tumoral and non-tumoral liver tissues from 13 HCC patients. A similar number of mutations was detected within the E2 domain in the HCC and CAH cases, but nine of the 47 HCCs (19%) showed an amino acid (aa) mutation at position 660, eight of which involved a change in the same aa (alanine instead of serine; A/S). No such mutation was detected in any of the PePHD sequences from the CAH patients: this difference was statistically significant (P = 0.008). The aa change at position 660 was also found in two sequences from tumoral but not non-tumoral tissue from the same liver. The analysis of 461 sequences obtained from GenBank supports the conclusion that the observed aa change is an infrequent event in HCV-infected patients, thus suggesting that it could be associated with HCC.
Assuntos
Carcinoma Hepatocelular/genética , Efrina-B2/genética , Hepacivirus/genética , Neoplasias Hepáticas/genética , Mutação , Idoso , Idoso de 80 Anos ou mais , Carcinoma Hepatocelular/virologia , Feminino , Regulação Neoplásica da Expressão Gênica , Marcadores Genéticos , Hepatite C Crônica/diagnóstico , Hepatite C Crônica/genética , Humanos , Neoplasias Hepáticas/virologia , Masculino , Pessoa de Meia-Idade , Probabilidade , RNA Viral/análise , Sensibilidade e Especificidade , Técnicas de Cultura de Tecidos , Proteínas não Estruturais Virais/genéticaRESUMO
Two cases of metastases from carcinoma to intracranial meningioma are presented. In the first case the presence of the malignant tumor was unsuspected at the time of intracranial operation. In the second case, a breast cancer had been previously removed, and unusual cranial computed tomographic findings were noted. The literature on metastases to intracranial tumors is reviewed, and the association of meningioma and carcinoma of the breast is emphasized.
Assuntos
Neoplasias Encefálicas/secundário , Carcinoma/patologia , Meningioma/secundário , Adenocarcinoma/patologia , Idoso , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/cirurgia , Neoplasias da Mama/patologia , Feminino , Humanos , Neoplasias Renais/patologia , Neoplasias Pulmonares/patologia , Masculino , Meningioma/diagnóstico por imagem , Meningioma/cirurgia , Pessoa de Meia-Idade , CintilografiaRESUMO
A case of an intracranial meningioma containing a metastasis from breast cancer is reported. CT demostrated a hypodense area within the meningioma and angiography showed a relatively less evident blush in the same area. The correlation of the neuroradiological with the pathological findings is discussed.
Assuntos
Adenocarcinoma/secundário , Neoplasias Encefálicas/secundário , Neoplasias da Mama/patologia , Neoplasias Meníngeas/diagnóstico por imagem , Meningioma/diagnóstico por imagem , Adenocarcinoma/patologia , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/patologia , Angiografia Cerebral , Feminino , Humanos , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios XRESUMO
We describe an exceptional case of an intrinsic, spontaneous haematoma of the oculomotor nerve and review the literature. We conclude that, even in an elderly patient, an isolated cranial nerve palsy should be extensively investigated and, when necessary, surgically treated, since a good functional recovery is possible.
Assuntos
Hematoma/diagnóstico , Doenças do Nervo Oculomotor/diagnóstico , Idoso , Doenças dos Nervos Cranianos/etiologia , Feminino , HumanosRESUMO
Twenty-four cases of supratentorial primary arachnoid cysts operated with different techniques are reviewed: both cyst shunting or extirpation may lead to a good early clinical result, but the former is less dangerous and more indicated in a particular group of patients; late results are better in the shunted patients. CT scan is almost always diagnostic and is mandatory postoperatively to assess the late results.
Assuntos
Aracnoide-Máter , Cistos/cirurgia , Adolescente , Adulto , Idoso , Doenças do Sistema Nervoso Central/diagnóstico , Doenças do Sistema Nervoso Central/diagnóstico por imagem , Doenças do Sistema Nervoso Central/cirurgia , Derivações do Líquido Cefalorraquidiano , Criança , Pré-Escolar , Cistos/diagnóstico , Cistos/diagnóstico por imagem , Eletroencefalografia , Estudos de Avaliação como Assunto , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios XRESUMO
Microsurgery for supratentorial aneurysm was performed in 19 patients over the age of 65 with good results in 74%. Mortality and complications are discussed in the light of the preoperative neurological status and general condition. The indications for surgery are outlined.
Assuntos
Aneurisma Intracraniano/cirurgia , Idoso , Feminino , Seguimentos , Halotano , Humanos , Hipertensão/complicações , Hipestesia/complicações , Hipotensão Controlada , Aneurisma Intracraniano/complicações , Masculino , Microcirurgia , Paralisia/complicações , Hemorragia Subaracnóidea/complicações , Inconsciência/complicaçõesRESUMO
The first known case of association of giant cell arteritis and normal pressure hydrocephalus is described. The arteritis was cured with corticosteroid therapy while hydrocephalus required ventriculo-peritoneal shunt. The high protein content of cerebrospinal fluid of this patient is probably the pathogenetic mechanism of cerebrospinal fluid block, leading to occult obstructive hydrocephalus.
Assuntos
Proteínas do Líquido Cefalorraquidiano/análise , Arterite de Células Gigantes/complicações , Hidrocefalia de Pressão Normal/complicações , Hidrocefalia/complicações , Idoso , Derivações do Líquido Cefalorraquidiano , Feminino , Arterite de Células Gigantes/líquido cefalorraquidiano , Humanos , Hidrocefalia de Pressão Normal/líquido cefalorraquidiano , Hidrocefalia de Pressão Normal/etiologia , Cavidade Peritoneal , Prednisona/uso terapêuticoRESUMO
We describe here a case of holocord subependymoma in an adolescent patient, completely removed with two surgical procedures. We discuss the histological features, the diagnostic difficulties and the surgical techniques used in this case.
Assuntos
Glioma/cirurgia , Neoplasias da Medula Espinal/cirurgia , Adolescente , Glioma/diagnóstico , Glioma/patologia , Humanos , Masculino , Metrizamida , Mielografia , Neoplasias da Medula Espinal/diagnóstico , Neoplasias da Medula Espinal/patologia , Tomografia Computadorizada por Raios XRESUMO
The surgical treatment of syringomyelia is still debatable and the result are often poor. Several surgical procedures, based on various proposed etiopathologies, have been developed but in many cases have proved completely ineffective. We have evaluated the follow-up of 69 syringomyelic patients, some operated on, some not, in the search for clues to the management of the disease. For this purpose we devised a rating system, which we describe. 31 patients underwent surgery while 38 received no treatment. We found that half of the patients deteriorated, whether they were operated on or not; only 1 in 5 improved and the rest remained stable. For surgical treatment to be successful, the disease must be in rapid evolution but without definite paraparesis.
Assuntos
Siringomielia/fisiopatologia , Adulto , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Musculares/etiologia , Dor/etiologia , Prognóstico , Siringomielia/complicações , Siringomielia/cirurgiaRESUMO
In some acromegalic patients medical treatment does not succeed in normalizing GH/IGF-I values. Data showing IGF-I suppression in acromegaly by estrogen and by tamoxifen use prompted us to reevaluate the effects of estroprogestins (EP) supplementation on GH/IGF-I levels in acromegalic women resistant or only partially sensitive to medical treatment. Eight active acromegalic women (30-52 yr, 4 with regular menses) entered a prospective open pilot study. Three of them, resistant to medical treatment, were off therapy; the remaining five, partially sensitive, maintained it at the maximally effective dosages throughout the study. Patients were treated with a triphasic pill (ethynil-estradiol 30-40-30 microg/day and desogestrel 50-70-100 mg/day) for 13 +/- 7 months. IGF-I levels fell from 512 (median, interquartile 436-657) microg/l to 282 (244-526) microg/l (p=0.0414); the decrease was observed in 6 patients (75%), and normal values were reached in 4 (50%). GH levels did not change [basal 7.6 (6.2-8.6) microg/l, final 7.6 (6.5-8.3) microg/l]. Effectiveness of treatment was not dependent on concomitant anti-GH treatment or gonadal status. In all patients IGF-I levels re-increased after EP withdrawal. This pilot study shows a marked IGF-I lowering effect of pill in acromegalic women, and warrants a prospective randomized study in patients resistant or partially sensitive to other medical treatments.
Assuntos
Acromegalia/sangue , Acromegalia/tratamento farmacológico , Anticoncepcionais Orais Sintéticos/farmacologia , Desogestrel/farmacologia , Etinilestradiol/farmacologia , Fator de Crescimento Insulin-Like I/efeitos dos fármacos , Adulto , Anticoncepcionais Orais Sintéticos/uso terapêutico , Desogestrel/uso terapêutico , Estrogênios/farmacologia , Estrogênios/uso terapêutico , Etinilestradiol/uso terapêutico , Feminino , Humanos , Fator de Crescimento Insulin-Like I/metabolismo , Pessoa de Meia-Idade , Projetos Piloto , Progestinas/farmacologia , Progestinas/uso terapêutico , Estudos ProspectivosRESUMO
The case of a patient who had a relapse of cerebral Whipple's disease (WD) one year after discontinuation of a two-years' antibiotic treatment is reported. Neither the clinical course nor the results of magnetic resonance imaging (MRI) and routine examination of the cerebrospinal fluid (CSF) allowed the caring physician to predict the relapse. Retrospective analysis of serial specimens of CSF showed that slight CSF leucocytosis and intrathecal synthesis of IgA might have suggested persistence of infection. The decision to stop antibiotic therapy in cerebral WD is difficult, but evaluation of cell counts and of intrathecal synthesis of IgA may help in the decision. Some patients may need to take treatment indefinitely.
Assuntos
Encefalite/líquido cefalorraquidiano , Encefalite/etiologia , Doença de Whipple/líquido cefalorraquidiano , Doença de Whipple/complicações , Encefalite/imunologia , Humanos , Imunoglobulina A/sangue , Imunoglobulina A/líquido cefalorraquidiano , Imunoglobulina G/sangue , Imunoglobulina G/líquido cefalorraquidiano , Imunoglobulina M/sangue , Imunoglobulina M/líquido cefalorraquidiano , Leucocitose , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Recidiva , Doença de Whipple/imunologiaRESUMO
Between 1956 and 1987 operations were performed on 36 patients below the age of 20 years for epidermoid and dermoid cysts of the central nervous system. Seventeen tumors were intracranial intradural lesions (47%): 12 were located in the supratentorial region (71%) and 5 were located in the infratentorial region (29%). Ten of these tumors (59%) were seated along the midline structures. The clinical presentation was consistent with the location of the tumors. The neuroradiological diagnosis was mostly made with the aid of pneumoencephalography, computed tomography (CT), nonionic contrast medium CT cisternography, and magnetic resonance imaging. Complete removal of the tumor contents was performed in all cases but one, although the completeness of removal of the tumor capsule could not be exactly estimated in some patients. At late follow-up only two tumor recurrences were observed. Radical removal of the tumor capsule of these congenital tumors, even when it is connected to vital neurovascular structures, seems advisable in patients who become symptomatic within the first two decades of life.