Volume 5
Archive

1: Cancer. 2006 Aug 15;107(4):696-704.

 
Primary breast cancer phenotypes associated with propensity for central nervous system metastases.

Tham YL, Sexton K, Kramer R, Hilsenbeck S, Elledge R.

Breast Care Center, Baylor College of Medicine and Methodist Hospital, Houston, Texas 77030, USA.

BACKGROUND: There is anecdotal evidence that the incidence of central nervous system (CNS) metastases in breast cancer patients is increasing. It is unclear whether specific tumor biological properties or the use of systemic therapies influence this risk. METHODS: Using a database of 10,782 patients, 2685 patients were identified who experienced recurrence distantly. Clinical and biological features were analyzed in 2 ways: (1) patients who ever had versus those who never had CNS metastases, and (2) CNS metastases as the first site of recurrence versus those who had other sites. Correlations of survival after CNS metastasis with clinical and biologic features were also analyzed. RESULTS: In the ever versus never analysis, CNS metastases were significantly associated with younger age, premenopausal status, infiltrating ductal carcinoma histology (IDC), estrogen receptor (ER) and progesterone receptor (PR) negativity, low Bcl-2, high S-phase, aneuploidy, and altered p53. Tumor size, lymph node status, and use of adjuvant systemic therapy played little role. HER-2 overexpression was not associated with an increased risk in these patients (none of whom were treated with trastuzumab) (P = .91). However, epidermal growth factor receptor (EGFR) overexpression was associated with increased risk (P = .02). A multivariate analysis revealed ER negativity (odds ratio [OR] 2.8, P < .001), IDC histology (OR 2.5, P = .02), and young age (P < .001) as independent factors for CNS metastases. The clinical and biologic profiles of primary tumors with CNS metastases at first recurrence did not differ from those with CNS metastases after recurrence to other sites, except for HER-2 status. HER-2-positive tumors were not more likely to undergo recurrence initially in the CNS (P =.04). The median survival after CNS metastases was 5.5 months and HER-2-positive patients had a shorter survival. CONCLUSIONS: Younger patients with hormone receptor-negative, highly proliferative, genomically unstable, and p53-altered tumors were at increased relative risk for CNS metastases. HER-2 expression and adjuvant systemic therapies did not increase this risk.

PMID: 16826579 [PubMed - indexed for MEDLINE]

 

2: Childs Nerv Syst. 2006 Oct 13; [Epub ahead of print]

 
Bone mineral density in survivors of childhood brain tumours.

Petraroli M, D'Alessio E, Ausili E, Barini A, Caradonna P, Riccardi R, Caldarelli M, Rossodivita A.

Department of Paediatrics, Universita Cattolica del Sacro Cuore (UCSC), Rome, Italy.

BACKGROUND: Osteopenia has been reported in children surviving acute lymphoblastic leukaemia, apparently as consequence of therapy. Few studies have been published on bone mineral density (BMD) evaluation in children surviving from brain tumours. The endocrine system in these patients is frequently affected as consequence of therapeutic interventions such as cranial irradiation and anti-neoplastic agents: growth hormone deficiency is the most common adverse sequel. The pathogenesis of osteopenia in brain cancer survivors is multi-factorial but still uncertain. OBJECTIVE: The aim of this study is to examine bone mass in 12 brain cancer survivors and its relationship with their hormonal status. RESULTS AND DISCUSSION: We observed that most of the patients had a BMD that was lower than normal in both the lumbar column and in the femoral neck. Bone mass loss was higher in the lumbar region rather than in the femoral neck, due to spinal radiation therapy and to the effect of hormonal deficiencies. Particularly hypogonadism, but also multiple hormonal deficiencies, are associated with lower BMD values. Experience in clinical care of these patients suggests the importance of periodic evaluations of BMD, especially in those with secondary hormone deficiencies. Moreover, the periodic assessment of the hypothalamus-pituitary function is essential for an early diagnosis of hormonal insufficiency, primarily hypogonadism, to precociously detect bone mineral loss and to prevent pathological fractures, thus improving the quality of life.

PMID: 17058089 [PubMed - as supplied by publisher]

 

3: Clin Cancer Res. 2006 Oct 15;12(20 Pt 1):6144-52.

 
A novel one-armed anti-c-Met antibody inhibits glioblastoma growth in vivo.

Martens T, Schmidt NO, Eckerich C, Fillbrandt R, Merchant M, Schwall R, Westphal M, Lamszus K.

Department of Neurosurgery, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

PURPOSE: Expression of the receptor tyrosine kinase c-Met and its ligand scatter factor/hepatocyte growth factor (SF/HGF) are strongly increased in glioblastomas, where they promote tumor proliferation, migration, invasion, and angiogenesis. We used a novel one-armed anti-c-Met antibody to inhibit glioblastoma growth in vivo. EXPERIMENTAL DESIGN: U87 glioblastoma cells (c-Met and SF/HGF positive) or G55 glioblastoma cells (c-Met positive and SF/HGF negative) were used to generate intracranial orthotopic xenografts in nude mice. The one-armed 5D5 (OA-5D5) anti-c-Met antibody was infused intratumorally using osmotic minipumps. Following treatment, tumor volumes were measured and tumors were analyzed histologically for extracellular matrix (ECM) components and proteases relevant to tumor invasion. Microarray analyses were done to determine the effect of the antibody on invasion-related genes. RESULTS: U87 tumor growth, strongly driven by SF/HGF, was inhibited > 95% with OA-5D5 treatment. In contrast, G55 tumors, which are not SF/HGF driven, did not respond to OA-5D5, suggesting that the antibody can have efficacy in SF/HGF-activated tumors. In OA-5D5-treated U87 tumors, cell proliferation was reduced > 75%, microvessel density was reduced > 90%, and apoptosis was increased > 60%. Furthermore, OA-5D5 treatment decreased tumor cell density > 2-fold, with a consequent increase in ECM deposition and increased immunoreactivity for laminin, fibronectin, and tenascin. Microarray studies showed no increase in these ECM factors, rather down-regulation of urokinase-type plasminogen activator and matrix metalloproteinase 16 in glioblastoma cells treated with OA-5D5. CONCLUSIONS: Local treatment with OA-5D5 can almost completely inhibit intracerebral glioblastoma growth when SF/HGF is driving tumor growth. The mechanisms of tumor inhibition include antiproliferative, antiangiogenic, and proapoptotic effects.

PMID: 17062691 [PubMed - in process]

 

4: Clin Neuropathol. 2006 Sep-Oct;25(5):232-6.

Rare primary CNS anaplastic large cell lymphoma in an immunocompetent adult: a clinical-pathologic case report and review case of the literature.

Cooper PB, Auerbach A, Aguilera NS, Adair C, Moores L, Geyer D, Rushing EJ.

Department of Neurosurgery, National Capitol Consortium, Walter Reed Army Medical Center, Washington DC, USA.

OBJECTIVE AND IMPORTANCE: Isolated anaplastic large cell lymphoma (ALCL) presenting in the primary central nervous system is distinctly uncommon. The authors describe a case that clinically and radiographically simulated a primary glial neoplasm. CLINICAL PRESENTATION: A 39-year-old immunocompetent male presented with seizures and a rapidly enlarging right occipital/parietal lesion. Magnetic resonance images demonstrated a right occipitoparietal lesion, hypodense on T1WI, with patchy contrast enhancement with gadolinium and significant white matter edema pattern on T2WI along with mass effect and midline shift. INTERVENTION: The patient underwent a frameless stereotactic assisted needle biopsy. There appeared to be a clear demarcation between white matter and tumor with no obvious necrosis. Biopsy showed a proliferation of single cells and poorly cohesive groups of cells with large, pleomorphic nuclei, many containing prominent nucleoli, and a moderate amount of cytoplasm. Immunohistochemical staining revealed CD-30 and ALK-positivity typical of ALCL, a rare form of T-cell lymphoma. An extensive workup revealed neither systemic disease nor evidence of immunocompromise. CONCLUSION: Reported in less than 20 patients, primary ALCL in an immunocompetent patient is rarely found intracranially; however, its ability to mimic glial neoplasms as well as other pathologies underlines its importance.

Publication Types:

• Case Reports

PMID: 17007446 [PubMed - indexed for MEDLINE]

 

5: Clin Neuropathol. 2006 Sep-Oct;25(5):221-6.

Suprasellar and intrasellar paragangliomas.

Voulgaris SG, Partheni M, Tzortzidis F, Ravazoula P, Pessach IS, Papadakis N, Polyzoidis KS.

University of Ioannina Medical School, Regional University Hospital of Ioannina, Department of Neurosurgery, Ioannina 45110, Greece. iliaspessach1980@yahoo.gr

Neoplasms of the sellar region are entities with a large differential diagnosis. Although paraganglionic cells have not been demonstrated in the pituitary or adjacent structures, the existence of sellar region paragangliomas is well-documented. To elucidate, in this area the nature of these unusual tumors is relatively difficult. Clinical history, physical examination, radiographic investigation as well as intraoperative gross observation are the same as those of sellar meningioma or pituitary adenoma. Immunohistochemistry, using neuroendocrine markers and electron microscopy are the two definitive diagnostic methods to differentiate among these entities. The clinical management, the possible pathogenesis of the tumor, the importance of immunohistochemistry in making the diagnosis and the clinical outcome of these patients are discussed.

Publication Types:

• Case Reports

PMID: 17007444 [PubMed - indexed for MEDLINE]

 

6: Clin Neuropathol. 2006 Sep-Oct;25(5):216-20.

Cyclooxygenase-2 (cox-2) expression and angiogenesis in intracranial ependymomas.

Onguru O, Kurt B, Gunhan O, Soylemezoglu F.

Department of Pathology, Gulhane Military Medical Academy, Hacettepe University Faculty of Medicine, Ankara, Turkey. onguruo@yahoo.com

AIM: Cyclooxygenase-2 (Cox-2), the inducible key enzyme in the biosynthesis of prostaglandins, appears to play a role in the regulation of progression, invasiveness and angiogenesis of various neoplasms including some glial tumors. Little is known about the role of Cox-2 in angiogenesis and proliferation of ependymomas. We studied Cox-2 expression, Ki-67 labeling index (Ki-67 LI) and microvessel density (MVD) in 30 intracranial ependymomas and analyzed the relationship among these parameters to evaluate their importance in the tumor biology of ependymomas. RESULTS: The mean Ki-67 LI for all tumors ranged from 1 - 50% (mean 9%). Statistically significant difference was present for Ki-67 LI between ependymomas (grade II, WHO) and anaplastic ependymomas (grade III, WHO) (p < 0.001) (mean Ki-67 LI for ependymoma, 2.8%, for anaplastic ependymomas, 15.6%). Anaplastic ependymomas did not demonstrate a greater vascularization than ependymomas, and the MVD values were 84.5 +/- 39.7 for ependymomas, and 90.6 +/- 61.4 for anaplastic ependymomas. Cox-2 immunohistochemical expression was observed in 19 tumors (63%). Although Cox-2 expression was slightly higher in anaplastic ependymomas, it was not statistically significant. No correlation was found between Cox-2 expression and MVD and Ki-67 LI. CONCLUSION: Similar to morphologic and prognostic heterogeneity in ependymomas, Cox-2 expression, MVD and Ki-67 LI also show a great variability. Other factors may be more important for the proliferation and angiogenesis of ependymomas.

PMID: 17007443 [PubMed - indexed for MEDLINE]

 

7: Eur J Cancer. 2006 Sep;42(13):2064-80.

 
Childhood central nervous system tumours--incidence and survival in Europe (1978-1997): report from Automated Childhood Cancer Information System project.

Peris-Bonet R, Martinez-Garcia C, Lacour B, Petrovich S, Giner-Ripoll B, Navajas A, Steliarova-Foucher E.

National Childhood Cancer Registry, Spain (RNTI-SEOP) and Instituto Lopez Pinero (CSIC-Universitat de Valencia), Faculty of Medicine, Avd. Blasco Ibanez, 15, 46010-Valencia, Spain. rafael.peris@uv.es

This paper describes the incidence and survival of childhood central nervous system (CNS) tumours in Europe for the period 1978-1997. A total of 19,531 cases, aged 0-14 years, from the ACCIS database were analysed by five regions: the British Isles, East, North, South, and West. Overall age-standardised incidence rate (ASR) of CNS tumours in Europe (1988-1997) was 29.9 per million, with the highest rates in the North. Astrocytoma (ASR=11.8), primitive neuroectodermal tumours (PNET) (ASR=6.5) and ependymoma (ASR=3.4) were the most frequent types. Incidence increased significantly during 1978-1997, on average by 1.7% per year. Diagnostic methods may partially explain incidence rates and trends, although a role of variations in risk factors cannot be excluded. Overall 5-year survival was 64% and varied between 72% in the North and 53% in the East. PNET had the poorest prognosis (49%) and astrocytoma the best (75%). Survival has improved by 29% since late 1970s. The positive trends were seen in all regions, although the interregional differences persisted, as a reflection of the different healthcare systems.

PMID: 16919771 [PubMed - indexed for MEDLINE]

 

8: Int J Cancer. 2006 Oct 25; [Epub ahead of print]

 
Aclarubicin-loaded cationic albumin-conjugated pegylated nanoparticle for glioma chemotherapy in rats.

Lu W, Wan J, Zhang Q, She Z, Jiang X.

Department of Pharmaceutics, School of Pharmacy, Fudan University, Shanghai, People's Republic of China.

Traditional glioma chemotherapy with those second-line drugs such as anthracyclines usually failed because they are inaccessible to blood-brain barrier (BBB) in tumor. In our study, we incorporated aclarubicin (ACL) into cationic albumin-conjugated pegylated nanoparticle (CBSA-NP-ACL) to determine its therapeutic potential of rats with intracranially implanted C6 glioma cells. When labeled with fluorescent probe, 6-coumarin, CBSA-NP was shown to accumulate much more in tumor mass than nanoparticle without conjugated CBSA (NP) 1 hr post intravenous injection, as well as better retention after 24 hr. Tumor drug concentration of CBSA-NP-ACL displayed 2.6- and 3.3-fold higher than that of NP-ACL and ACL solution 1 hr post injection, while 2.7 and 6.6-fold higher after 24 hr, respectively. Moreover, using tumor microdialysis sampling, AUC(0-24 hr) of free drug amount in tumor interstitium delivered by CBSA-NP-ACL was about 2.0- and 2.7-fold higher than that of NP-ACL and ACL solutions, respectively. When the tumor rat model was subjected to 4 cycles of 2 mg/kg of ACL in different formulations, a significant increase of median survival time was found in the group of CBSA-NP-ACL compared with that of saline control animals, animals treated with NP-ACL and ACL solution. By terminal deoxynucleotidyl transferase-mediated dUTP nick-end-labeling, CBSA-NP-ACL can extensively make the tumor cell apoptosis. Histochemical evaluation by periodic acid Shiff staining and biochemical analysis depicted that the incorporation of ACL into CBSA-NP reduced its toxicity to liver, kidney and heart. Besides, CBSA-NP-ACL was not shown to open tight junction evaluated by BBB coculture. It was concluded that CBSA-NP-ACL could have a therapeutic potential for treatment of glioma. (c) 2006 Wiley-Liss, Inc.

PMID: 17066446 [PubMed - as supplied by publisher]

 

9: Int J Radiat Oncol Biol Phys. 2006 Oct 20; [Epub ahead of print]

 
Validation of the RTOG recursive partitioning analysis (RPA) classification for small-cell lung cancer-only brain metastases.

Videtic GM, Adelstein DJ, Mekhail TM, Rice TW, Stevens GH, Lee SY, Suh JH.

Department of Radiation Oncology, Cleveland Clinic Foundation, Cleveland, OH.

PURPOSE: The Radiation Therapy Oncology Group (RTOG) developed a prognostic classification based on a recursive partitioning analysis (RPA) of patient pretreatment characteristics from three completed brain metastases randomized trials. Clinical trials for patients with brain metastases generally exclude small-cell lung cancer (SCLC) cases. We hypothesize that the RPA classes are valid in the setting of SCLC brain metastases. METHODS AND MATERIALS: A retrospective review of 154 SCLC patients with brain metastases treated between April 1983 and May 2005 was performed. RPA criteria used for class assignment were Karnofsky performance status (KPS), primary tumor status (PT), presence of extracranial metastases (ED), and age. RESULTS: Median survival was 4.9 months, with 4 patients (2.6%) alive at analysis. Median follow-up was 4.7 months (range, 0.3-40.3). Median age was 65 (range, 42-85). Median KPS was 70 (range, 40-100). Number of patients with controlled PT and no ED was 20 (13%) and with ED, 27 (18%); without controlled PT and ED, 34 (22%) and with ED, 73 (47%). RPA class distribution was: Class I: 8 (5%); Class II: 96 (62%); Class III: 51 (33%). Median survivals (in months) by RPA class were: Class I: 8.6; Class II: 4.2; Class III: 2.3 (p = 0.0023). CONCLUSIONS: Survivals for SCLC-only brain metastases replicate the results from the RTOG RPA classification. These classes are therefore valid for brain metastases from SCLC, support the inclusion of SCLC patients in future brain metastases trials, and may also serve as a basis for historical comparisons.

PMID: 17056192 [PubMed - as supplied by publisher]

 

10: J Clin Oncol. 2006 Oct 1;24(28):4570-4.

 
Long-term follow-up of high-dose methotrexate-based therapy with and without whole brain irradiation for newly diagnosed primary CNS lymphoma.

Gavrilovic IT, Hormigo A, Yahalom J, DeAngelis LM, Abrey LE.

Department of Neurology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.

PURPOSE: We previously reported a series of patients treated with high-dose methotrexate (MTX) -based chemotherapy, with or without whole brain radiotherapy. The purpose of this report is to update the initial results and provide long-term data regarding overall survival, patterns of relapse, and the risk of treatment-related neurotoxicity. PATIENTS AND METHODS: Fifty-seven patients with an average age of 65 and median Karnofsky performance score of 70 were treated; all patients have been observed longitudinally with serial magnetic resonance imaging scans and neurologic examinations. RESULTS: The overall median survival was 51 months with a median follow-up of 115 months for surviving patients. Twenty-five patients relapsed or developed progressive disease; median progression-free survival was 129 months. Seventeen patients developed treatment-related neurotoxicity; all but one had received whole brain radiotherapy as a component of treatment. Seventy-four percent of patients younger than 60 years who received both MTX-based chemotherapy and whole brain radiotherapy were alive at last follow-up. Median survival for patients older than 60 years was 29 months regardless of whether or not they received whole brain radiotherapy. CONCLUSION: Long-term follow-up of our initial cohort confirms the observation of excellent overall survival, particularly for those patients younger than age 60 at diagnosis. For older patients, it appears to be reasonable to defer whole brain radiotherapy in an effort to minimize treatment-related neurotoxicity.

PMID: 17008697 [PubMed - indexed for MEDLINE]

 

11: J Neurol. 2006 Aug;253(8):1092-3. Epub 2006 Apr 28.

 
Multicystic tumor in the fourth ventricle: consider neurocysticercosis.

Costa CU, von Einsiedel HG, Disko R, Berthele A.

Publication Types:

• Case Reports
• Letter

PMID: 16649100 [PubMed - indexed for MEDLINE]

 

12: J Neurol. 2006 Aug;253(8):1094-6. Epub 2006 Apr 11.

 
Unusual MRI findings in primary central nervous system lymphoma presenting diffuse linear enhancements located in the perivascular space.

Ichikawa Y, Maeda M, Ishida M, Takeda K, Hirano T.

Publication Types:

• Case Reports
• Letter

PMID: 16609808 [PubMed - indexed for MEDLINE]

 

13: Neurology. 2006 Oct 24;67(8):1509-12.

 
The role of surgical biopsy in the diagnosis of glioma in individuals with neurofibromatosis-1.

Leonard JR, Perry A, Rubin JB, King AA, Chicoine MR, Gutmann DH.

Department of Neurology, St. Louis Children's Hospital and Washington University School of Medicine, 660 S. Euclid Avenue, St. Louis, MO 63110, USA.

Most gliomas in neurofibromatosis type 1 (NF1) are pilocytic astrocytomas (PAs) of the optic pathway occurring in young children. However, some individuals develop gliomas that lack the typical NF1-associated clinical features or radiographic appearance. We identified 17 atypical presentations from a review of 100 patients with NF1-associated gliomas. Biopsy showed that 9 were not classic PAs. These data highlight the value of biopsy in NF1-associated gliomas with unusual clinical or radiographic presentations.

PMID: 17060590 [PubMed - in process]

 

14: Pediatr Hematol Oncol. 2006 Dec;23(8):631-7.

 
Late response to radiochemotherapy in pediatric glioblastoma: report on two patients treated according to HIT-GBM protocols.

Classen CF, Warmuth-Metz M, Papke K, Trotter A, Wolff JE, Wagner S.

Children's Hospital, Wedau Kliniken, Klinikum Duisburg, Duisburg, Germany. cfclassen@gmx.de

High-grade gliomas in children are rare and the best treatment is undetermined. The German language group study HIT-GBM compares various induction protocols for subsequent patient cohorts. Currently, cisplatinum, etoposide, ifosfamide, and vincristine are given simultaneously with extended-field radiotherapy. Imaging is done 3 weeks after to define treatment response, followed by 6-weekly controls during consolidation with lomustine, vincristine, and prednisone. The authors report on 2 patients with incompletely resected glioblastoma multiforme in which response was lacking 3 weeks after radiochemotherapy but became evident 12 weeks later. This suggests that later time points are required to assess induction protocol response.

PMID: 17065139 [PubMed - in process]

 

15: Radiology. 2006 Nov;241(2):614-7.

 
Case 100: spinal epidural meningioma.

El Khamary SM, Alorainy IA.

College of Medicine and King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia. selkhamary@hotmail.com

PMID: 17057078 [PubMed - in process]

 

16: Rev Neurol. 2006 Aug 16-31;43(4):207-12.

 
[Tumour of the corpus callosum: the association between interhemispheric disconnection and an anterograde amnesia syndrome]

[Article in Spanish]

Bustamante J, Lopera F.

Universidad de Antioquia, Facultad de Medicina, Grupo de Neurociencias, Medellin, Colombia.

INTRODUCTION: Sperry, or interhemispheric disconnection, syndrome was reported in patients who had undergone surgical section of the corpus callosum carried out in an attempt to control medication-resistant epilepsy. It has occasionally been linked to tumours of the corpus callosum and, although even more rarely, it has also been associated to an amnesic syndrome. In this paper we report the anatomical and neuropsychological findings in a patient with interhemispheric disconnection syndrome associated to a hippocampal-type amnesic syndrome, caused by a tumour in the splenius of the corpus callosum that extended into the fornix. CASE REPORT: A 52-year-old white male who visited because of loss of memory; on admission to hospital the physical examination revealed a certain degree of asomatognosia with regard to the left-hand side of the body. An axial tomography brain scan showed a dense central lesion in the brain that extended laterally and occluded the body of both lateral ventricles. A biopsy study revealed an undifferentiated astrocytoma that affected the corpus callosum and the fornix. CONCLUSIONS: Sperry, or interhemispheric disconnection, syndrome produced by a tumour in the splenius of the corpus callosum is very likely to course with an amnesic syndrome due to disconnection caused by destruction of the fornix. This association, which characterised our patient's clinical picture, has only previously been described in three cases.

Publication Types:

• Case Reports

PMID: 16883509 [PubMed - indexed for MEDLINE]

 

17: Rev Neurol. 2006 Aug 16-31;43(4):197-200.

 
[Intracranial and intramedullary peripheral nerve sheath tumours. Case reports from 20 autopsies]

[Article in Spanish]

Aguirre-Quezada DE, Martinez-Anda JJ, Aguilar-Ayala EL, Chavez-Macias L, Olvera-Rabiela JE.

Unidad de Patologia, Hospital General de Mexico, Facultad de Medicina, Universidad Autonoma de Mexico. Mexico DF, Mexico.

INTRODUCTION: Tumors arising from the sheath of peripheral nerves, both intracranial and intraspinal, are uncommon and are sometimes of difficult clinical diagnosis, especially when they occur in unusual sites. Schwannomas, neurofibromas and perineuromas are depicted in this descending order of frequency. Most are sporadic and some can be part of hereditary syndromes. Histological malignancy of this neoplasm is rare. MATERIALS AND METHODS: The clinical and pathological findings of 20 autopsy cases of intracranial and intraspinal peripheral nerve tumors are analyzed. The average age at presentation was 35 years and the male/female ratio was 1:1. RESULTS: 19 were schwannomas, 13 of the 8th cranial nerve (two associated with neurofibromatosis type 2), two originated in the trigeminal and one in the 12th nerves. Three were intraspinal, one of this underwent malignant changes and was part of neurofibromatosis type 1 (NF-1), another was an intraspinal lumbar mass with schwannomatosis and the third was a case of multiple intraspinal neurofibromas as a part of NF-1. 14 cases were surgically treated and the causes of death were ischemic lesions due to the large size of the tumors. The correct clinical diagnosis was made in 14 patients. In 11 instances there was corroboration by biopsy. Three were misdiagnosed and three were autopsy findings. CONCLUSIONS: In this series more cases were sporadic. No sex predominance was encountered. The importance of early detection on intracranial and intraspinal peripheral tumors is paramount, since the large size of these histologically benign neoplasms makes them biologically malignant.

PMID: 16883507 [PubMed - indexed for MEDLINE]