Manolo Piccirilli

Long-term follow-up of intramedullary spinal cord tumors : A series of 202 cases

OBJECTIVE:To review a series of patients who underwent surgical removal of intramedullary spinal cord tumors, focusing on the long-term functional outcome, recurrence rates for the various tumors, and technical problems continually debated in neurosurgical practice. METHODS:From December 1972 to June 2003, 202 patients underwent removal of intramedullary tumors. Lesions were located in the cervical spinal cord in 61 patients (30%), at a dorsal site in 60 (29%), at a cervicodorsal site in 51 (25%), and in the medullary cone in 30 (15%). The most frequent histological tumor types were astrocytomas (86 patients, 42%) and ependymomas (68 patients, 34%). RESULTS:Of the 68 ependymomas, 55 (81%) were completely removed and 13 (19%) incompletely removed. In 66% of the patients (42 patients), the presenting signs and symptoms remained unchanged at long-term follow-up; in 25% (16 patients), they improved; and in 9% (6 patients), the clinical status worsened. Of the 27 Grade I astrocytomas, 22 (81%) were completely removed and 5 (19%) incompletely removed. Functional assessment of the 23 patients available at “late” follow-up showed that 26% (6 of 23 patients) had improved, 9% (2 of 23 patients) had worsened, and 66% (15 of 23 patients) remained unchanged from preoperative status. Conversely, of the 41 Grade II astrocytomas, only 5 (12%) were completely removed, and 10% had improved. None of the 18 Grade III to IV astrocytomas could be completely removed. In 61% (11 of 18 patients), the postoperative functional status worsened. CONCLUSION:Determinant predictors of a good outcome after surgery for intramedullary spinal cord tumors are histological type of lesion, complete removal of the lesion, and a satisfactory neurological status before surgery.

Long-Term Outcome of Esthesioneuroblastoma: Hyams Grade Predicts Patient Survival

Object Esthesioneuroblastoma (ENB) is a rare malignant neuroendocrine tumor originating from the olfactory neuroepithelium in the cribriform plate. Controversy still exists regarding the role of pathologic grading (Hyams grade) in prognostication. This study was undertaken to describe our experience with ENB and assess the role of pathologic grading in patient outcome. Methods This was a retrospective, single-institution experience, including 109 patients with ENB treated at our institution from 1962 to 2009. Multivariate analysis was performed utilizing Cox regression analysis models utilizing age, gender, modified Kadish stage, and Hyams grade. Results Mean age was 49 ± 16 (median 50) years at presentation (range 12 to 90 years). Median follow up was 5.1 years. All-cause mortality was significantly influenced by Hyams grading in univariate (p = 0.04) and multivariate (p = 0.02) analysis, in addition to proven prognostic factors, Kadish staging, lymph node metastasis, and age. Median survival was 9.8 years compared with 6.9 years with low (grade 1 to 2) versus high (grade 3 to 4) Hyams grade. Median overall survival was 7.2 ± 0.7 years. Conclusion ENB has a variable outcome, which is primarily prognosticated by the extent of involvement at presentation (Kadish stage and lymph node metastasis) and higher Hyams pathologic grade.

High-grade intramedullary astrocytomas: 30 years' experience at the Neurosurgery Department of the University of Rome "Sapienza".

OBJECT The goal in this study was to review a series of patients who underwent surgical removal of intramedullary high-grade gliomas, focusing on the functional outcome, recurrence rates, and technical problems continually debated in neurosurgical practice. METHODS Between December 1976 and December 2006, 22 patients underwent removal of intramedullary high-grade gliomas. Lesions were located in the cervical spinal cord in 12 patients, and in the thoracic cord in 10. RESULTS Histological examinations showed 10 Grade III astrocytomas and 12 glioblastomas. Only 2 of the 22 high-grade astrocytomas could be completely removed. The clinical postoperative status worsened in 14 patients (63.6%), was unchanged in 7 patients (31.8%), and there was 1 case of intraoperative death (4.5%). None of the 22 patients showed improvement in their neurological status postoperatively. In this series, excluding the 1 intraoperative death, all patients died of progression of the malignancy. CONCLUSIONS Surgical treatment did not ameliorate the postoperative neurological status; instead, in the majority of cases, it prompted a worsening of the deficit. Radiotherapy and chemotherapy have a little influence on the length of survival. In this series, multimodality treatment of intramedullary high-grade astrocytomas has been shown to increase length of survival without improving the neurological status.

Extra central nervous system metastases from cerebral glioblastoma multiforme in elderly patients. Clinico-pathological remarks on our series of seven cases and critical review of the literature

Aims and background The aim of the study was to evaluate the treatment of the extracranial metastases from glioblastoma multiforme in the elderly, discussing their uncommon occurrence and their pathogenesis. Methods The authors report seven cases of elderly patients (mean age, 69 years), with an initial diagnosis of cerebral glioblastoma multiforme, treated by a grossly total surgical removal and followed by adjuvant radiotherapy (64 Gy in 6 weeks, using Linac) and adjuvant chemotherapy (temozolomide both concomitant and sequential to radiotherapy). Results All patients presented a postoperative course characterized by good functional and clinical conditions (Karnofsky performance scale ≥70), which remained unchanged for a mean period of about 21 months (range, 16–23), with no neuroradiological signs of lesion regrowth. After this interval, new clinical signs occurred, and their clinical and radiological investigation showed metastatic repetitions in different sites: lung, liver, humerus and lymph nodes. All the metastases were surgically treated, but regrowth of the brain tumor and progression to deep important neural structures caused the patients’ exitus after a mean interval of about 10 months (range, 8–12) from the diagnosis of metastasis. Conclusions We found 128 cases of extra CNS metastases in the English literature. The main features of the patients of the previous reports and of those of the present series were analyzed. The main modalities of glioblastoma multiforme spread, the few theories about the rarity of metastasis, and the probable biological, histological and immunogenetic mechanisms involved in the pathogenesis are described. Although several studies have reported a poor outcome in elderly patients, they affirm that the treatment of those with a Karnofsky performance status >60 should be just as aggressive as in younger patients. This allows them to obtain a longer survival time and to also treat metastases, which are uncommon particularly in the elderly.

Extradural spinal cavernous angiomas: report of seven cases

The authors describe seven cases of extradural spinal cavernous angioma. Although cavernoma itself is not rare, the extradural spinal localization is uncommon and makes preoperative differential diagnosis difficult. Routine MRI investigation has aided neurosurgeons in evaluating the true incidence of these vascular malformations, which was understimated in the past. The data published so far have not entirely clarified the treatment of choice for these lesions. Considering their rarity in this site, their presenting symptoms and the difficulties involved in neuroradiological diagnosis, the authors discuss the role of surgery as the principal form of treatment and review the relevant literature. Seven patients (4 male, 3 female) were admitted to our Institute of Neurosurgery between 1992 and 2004, with a 5–6 month history (range=2–365 days) of low back pain or radicular pain, sometimes associated with paresthesia. All patients had a CT scan, as well as MRI with gadolinium when possible, which detected an extradural roundish lesion: differential diagnosis was very difficult, especially between neurinoma and cavernoma. Treatment was always surgical and resection of the lesion radical. Postoperatively, all patients presented complete regression of clinical symptoms. In all cases histological diagnosis was cavernous angioma. Postoperative MRI with gadolinium or CT scan with IV contrast, performed before discharge, confirmed radical removal of the vascular malformation in all cases. Our experience confirms that surgery should be the treatment of choice for these lesions, in view of both their tendency to bleed and their straightforward surgical removal.

Differential expression of neurogenins and NeuroD1 in human pituitary tumours

Basic helix-loop-helix (bHLH) transcription factors are involved in neuroendocrine cell growth and differentiation. Though NeuroD1 is viewed as corticotroph specific, its overexpression in non-corticotroph pituitary adenomas (PAs) may reflect the activation of molecular pathways involving other bHLH factors, like neurogenins. To search for neurogenin-NeuroD1 molecular pathways in the human normal and tumoural pituitary. Fifty-one PAs--22 clinically non-secreting (CNS) and 29 secreting respectively--and normal human pituitaries (NP) were studied for NeuroD1 and neurogenins (Ngn1, Ngn2 and Ngn3) gene expression by RT-PCR and quantitative real-time RT-PCR (qRT-PCR). Immunohistochemistry for Ngn2/3 was performed in some cases. NeuroD1, Ngn2, Ngn3 and Ngn1 were observed in up to 84.3, 76.5, 30.4 and 9.1% of PA respectively, only NeuroD1 and Ngn2 being frequently overexpressed when compared with NP. Whereas NeuroD1 expression was higher in corticotroph and CNS adenomas (P=0.0001 versus Pit-1-dependent PA), Ngn2 expression was higher in secreting PA, especially in Pit-1-dependent PA (P=0.007 and P=0.0006 versus CNS respectively). Pit-1-dependent PA which received pre-operative pharmacological treatment expressed higher Ngn2 levels than untreated cases (P=0.025). Nuclear Ngn2 was observed in NP and in most PA, especially ACTH- and GH-secreting adenomas. Nuclear Ngn3 was observed in a minority of secreting PA. Ngn2 is normally expressed in the anterior pituitary and frequently expressed in PA, but does not account for NeuroD1 overexpression where present. Owing to their low and inconstant expression, the biological significance of Ngn1/3 in the adult pituitary is uncertain.

Neurinoma of the hypoglossal nerve in the submandibular space: case report and review of the literature.

SummaryNeurinomas of the hypoglossal nerve extending extra-cranially are rare; a schwannoma in a 63-year-old woman causing nerve palsy is reported. The tumour presented as a slow-growing mass in the right submandibular space; a surgical approach with transcervical exploration was performed. The post-operative course was uneventful.

Glioblastoma multiforme and breast cancer: report on 11 cases and clinico-pathological remarks.

The association between breast cancer and glioblastoma multiforme has not been amply analyzed in the literature. We describe 11 female patients with a diagnosis of glioblastoma multiforme who were treated when younger for breast cancer. We believe that this association is not due to chance but rather to genetic changes in hormone status and in particular to sex hormones. Another important point of view is represented by the chemotherapy treatment of breast cancer, which could have a carcinogenic effect and explain the growth of glioblastoma. This consideration, in our opinion, is important, because more effort should be made to understand the pathogenesis of glioblastoma multiforme and to improve the therapeutic approaches.

Spontaneous regression of a thoracic calcified disc herniation in a young female: A case report and literature review

Thoracic intersomatic disc herniation (TDH) is a rare entity, hose incidence is about one case over one million of habitants er year [1]; incidence of asymptomatic one is approximately 37% 2]. The majority of dorsal disc herniations goes through a calcifiation process, first described by Von Luschka, and may penetrate he dura mater [3]. Intervertebral disc calcification (IDC) is common n adults and it is often observed in degenerating discs. While in hildren treatment should be conservative, given its benignity and ikelihood to spontaneously regress, in adults it is considered to be n expression of disc degeneration and, despite its rate of regression s unknown, it is likely to be rare. For patients showing an active ymptomatology, treatment should be surgical. While few years go surgery was followed by important and often life-threatening omplications, nowadays new surgical techniques, above all mininvasive, come in handy [3]. We describe a case of a spontaneous egression of a dorsal calcified extruded disc herniation with an ssociated calcification of the intersomatic disc, which, till date, it s the only case reported in literature, CT and MRI documented.

Benign osteoblastoma of the odontoid process of the axis with secondary aneurysmal bone cyst component: a case report

The authors report their experience on one patient with osteoblatoma of the odontoid process of the axis with secondary aneurysmal bone cyst. According to their knowledge, this is the first case, reported in the literature, of this kind of lesion in that particular anatomical region. Because of the rarity of this lesion, it was difficult to have a certain preoperative diagnosis. Therefore, the patient underwent a biopsy via a transoral route. The biopsy was performed over the noncalcified component of the lesion. The intraoperative histological examination showed the benign nature of the lesion. Thereafter, the lesion was totally removed, succeeding in preserving the remaining part of the odontoid process and the anterior arch of C1. In the follow-up, there was no evidence of cranio-vertebral instability. The histological examination revealed an osteoblastoma of the odontoid process of the axis with a secondary aneurysmal bone cyst. To the best of our knowledge, this is the first case reported in the literature.