PubMed Health⌕ Search

Biomedical subjects

P Ruggieri

Publications and source records attributed to P Ruggieri.

At least 19 recordsLinked to original sources
PubMed2007

The value of interictal diffusion-weighted imaging in lateralizing temporal lobe epilepsy.

BACKGROUND: A subgroup of patients with nonlesional temporal lobe epilepsy (TLE) has no evidence of hippocampal sclerosis on standard temporal lobe protocol MRI. OBJECTIVE: To investigate whether interictal diffusion-weighted imaging adds lateralizing information in patients with TLE with and without lateralizing conventional MRI. METHODS: We studied 22 patients (9 right, 13 left TLE) who had undergone temporal lobectomy and 18 control subjects. We measured hippocampal volumes on high- resolution coronal magnetization-prepared rapid gradient echo scans. Apparent diffusion coefficients (ADCs) for the entire hippocampus and three arbitrarily defined areas of interest within the hippocampal head, body, and tail were measured from the coregistered ADC map. Pathology was reviewed and correlated with imaging findings. RESULTS: Fourteen of 22 patients had hippocampal atrophy on MRI (defined as volume asymmetry greater than 2 SDs compared with asymmetry in the control group). Overall, resected hippocampi (n = 22) were significantly smaller than contralateral hippocampi as well as ipsilateral hippocampi in controls. ADCs were significantly higher in resected hippocampi than contralateral hippocampi as well as ipsilateral hippocampi in controls. These differences were also observed within the three areas of interest. ADCs in the hippocampi contralateral to the epileptogenic zone (n = 22) were also higher than in ipsilateral hippocampi in controls. In the subgroup of eight patients with nonlateralizing conventional MRIs, ADCs of resected hippocampi were not significantly different compared with the contralateral side. Pathology in these patients revealed gliosis only without apparent neuron loss. CONCLUSION: Interictal apparent diffusion coefficients confirm lateralization in patients with hippocampal atrophy on standard temporal lobe protocol MRI. However, they do not provide lateralizing information in patients with nonlateralizing conventional MRI.

Adolescent↗
PubMed2006

Histologically verified lung metastases in benign giant cell tumours--14 cases from a single institution.

From 1975 to 1997, 649 cases of benign giant cell tumours of the bone were treated at the Istituto Rizzoli. Fourteen patients (2.1%) experienced lung metastases after a mean of 35.2 months. The time interval between the diagnosis and the appearance of the lung metastases ranged from 3 months to 11.9 years. Metastasectomy was performed in all patients. Histologically, the metastases were identical to the primary bone lesions. Two patients with unresectable multiple metastases received additional chemotherapy. After a follow-up of 70 months (range: 8.2 to 185 months), all patients are alive. Ten patients showed no evidence of disease, one of these after a second resection of metastases, and four patients presented stable disease with multiple lung metastases. Local recurrence of the bone lesion occurred in seven patients before or simultaneously to the metastases. In contrast to previous reports, we could not detect a predominance of the distal radius, but all of the patients had a stage III tumour according to the Enneking criteria of benign lesions. We conclude that even metastatic benign giant cell tumours have an excellent prognosis after adequate resection. No prognostic factors despite high-grade lesions were detectable.

Adolescent↗
PubMed2005

Seizure outcome after epilepsy surgery in patients with normal preoperative MRI.

OBJECTIVE: To determine outcome after epilepsy surgery in patients with normal preoperative magnetic resonance imaging (MRI). METHODS: 24 adult and paediatric patients with normal preoperative MRIs were studied. They underwent epilepsy surgery between 1994 and 2001 and had at least one year of follow up. RESULTS: At the most recent follow up, nine patients (37%) were seizure-free and 18 (75%) had at least a 90% reduction in seizure frequency with weekly or monthly seizures. Seizure freedom was not significantly different after resections in frontal (5/9) or temporal regions (4/13) (p = 0.24, Fisher's exact test), or among patients with or without localising features on EEG, PET, or ictal SPECT. Subdural grids, used in 15 of 24 patients, helped tailor resections but were not associated with differences in outcome. Histopathology showed cortical dysplasia in 10 patients (42%), non-specific findings in 13 (54%), and hippocampal sclerosis in one (4%). Cortical dysplasia was seen in seven patients with frontal resection (78%) and non-specific findings in nine (69%) with temporal resection. Seizure outcome did not differ on the basis of location of resection or histopathology. CONCLUSIONS: While these results were less favourable than expected for patients with focal epileptogenic lesions seen on MRI, they represented worthwhile improvement for this patient population with high preoperative seizure burden. In this highly selected group, no single test or combination of tests further predicted postoperative seizure outcome.

Adolescent↗
PubMed2004

Temporal lobe volumes in patients with hippocampal sclerosis with or without cortical dysplasia.

BACKGROUND: Recent MRI-based volume reconstruction studies in intractable temporal lobe epilepsy (TLE) due to hippocampal sclerosis (HS) suggested atrophy that extends to the adjacent neocortical areas. OBJECTIVE: To study the extent of temporal lobe volume (TLV) abnormalities in patients with pathologically confirmed HS (with or without cortical dysplasia [CD]) who underwent anterior temporal lobectomy for the treatment of drug-resistant TLE. METHODS: Fifty patients (right TLE: n = 24; left TLE: n = 26) were found to have HS (hippocampal cell loss of >30%). Associated neocortical CD was seen in 20 patients (43%). MRI-based TLVs and hippocampal and hemispheric volume reconstructions in all patients were compared between pathologic groups and with volumes acquired from 10 age-matched control subjects. RESULTS: TLVs ipsilateral to the epileptogenic zone in patients with TLE were smaller than TLVs in control subjects (p < 0.01). In patients with left TLE, TLVs ipsilateral to the epileptogenic zone were smaller than contralateral TLVs (left: 66.6 +/- 8.3 cm3, right: 74.9 +/- 10.0 cm3; p < 0.001). In patients with right TLE, there were no significant asymmetries. The contralateral TLVs (regardless of the side of surgery) were smaller in the HS + CD group than the HS group (HS + CD group: 74.9 +/- 8.6 cm3, HS group: 79.7 +/- 6.6 cm3; p < 0.05). Patients with HS + CD had a tendency to have less hippocampal atrophy and slightly smaller TLVs ipsilateral to the epileptogenic zone, accounting for significantly smaller TLV/hippocampal volume ratios compared with patients with HS alone. CONCLUSIONS: Drug-resistant TLE due to HS is associated with extrahippocampal temporal lobe atrophy. The presence of bilateral temporal lobe atrophy is suggestive of a more widespread (bilateral) temporal lobe involvement in patients with HS and CD.

Adolescent↗
PubMed2003

Unilateral absence of the basal ganglia plus epilepsy without motor symptoms.

A patient with absence of the basal ganglia and refractory epilepsy without impairment of pyramidal or extrapyramidal motor function is reported. Imaging findings suggest a vascular insult as etiology. Preserved motor function could be explained by neuronal plasticity involving contralateral corticostriatal and pallidothalamic connections and points to a lesion received in early pregnancy.

Adolescent↗
PubMed2003

Phase I study of thalidomide for the treatment of plexiform neurofibroma in neurofibromatosis 1.

An open-label phase I trial of thalidomide (TL) in 20 patients with neurofibromatosis 1 (NF1) treated symptomatic plexiform neurofibroma (PNF). TL was well tolerated in doses up to 200 mg/d. Adverse reactions included transient somnolence in four, evanescent rash in two, and reversible mild peripheral neuropathy in two patients. Four patients showed less than 25% reduction in the tumor size. TL may have a role in the treatment of PNF and should be explored in a larger controlled study, possibly using higher doses of TL.

Adolescent↗
PubMed2003

Hamartomas and epilepsy: clinical and imaging characteristics.

PURPOSE: Cerebral hamartomas are lesions marked by a disorganized arrangement of mature neural elements and represent a rare cause of medically intractable focal epilepsy. We present the clinical presentation and imaging findings of this rare entity. METHODS: History and neurophysiological studies of 14 patients with pathologically confirmed hamartomas who had surgery for intractable focal epilepsy were reviewed. MRIs were available for review in 10 patients. RESULTS: The lesions were most commonly located in the temporal and frontal lobes. Seizure semiology was concordant with the anatomic location of the hamartoma in all patients. Nine of the thirteen patients (69%) with the hamartoma confined to one lobe had interictal spikes and sharp waves at the corresponding electrodes. The ictal pattern was confined to the same lobe of the hamartoma in five of nine patients with ictal recordings. Although imaging characteristics were variable, all patients had signal increase on T2-weighted images and 50% of them had mild mass effect. Neocortical involvement was present in the majority of patients (7/10), blurring of the gray/white matter interface was seen in seven patients. Five of those seven patients were found to have associated cortical dysplasia by pathology. CONCLUSION: Hamartomas represent a rare entity and may cause devastating epilepsy. Imaging characteristics are difficult to distinguish from those of some other developmental tumors. Hamartomas are frequently associated with microscopic cortical dysplasia (CD), thus underlining their malformative etiology.

Adolescent↗
PubMed2001

Fluid-attenuated inversion recovery: correlations of hippocampal cell densities with signal abnormalities.

BACKGROUND: Hippocampal sclerosis (HS) is characterized by hippocampal atrophy and increased signal on T2-weighted images and on fluid-attenuated inversion recovery (FLAIR) images. OBJECTIVE: To quantitate cell loss and compare it with signal abnormalities on FLAIR images. METHODS: Thirty-one patients with temporal lobe resection, pathologically proven HS, and Engel class I and II outcome were included: 20 with HS only and 11 with HS associated with pathologically proven cortical dysplasia (dual pathology). The signal intensity on FLAIR was rated as present or absent in the hippocampus and correlated with the neuronal losses in the hippocampus. RESULTS: FLAIR signal increases were present in 77% (24/31) of all patients studied. In patients with isolated HS, 90% (18/20) had ipsilateral signal increases, but in patients with dual pathology, only 55% (6/11; p < 0.02) showed FLAIR signal increase. Hippocampal cell losses were significantly higher in the isolated HS group. The average cell loss in patients with FLAIR signal abnormalities was 64.8 +/- 8.0% as compared with only 32.7 +/- 5.1% in patients with no FLAIR signal abnormalities. There was a significant positive correlation between the presence of signal abnormality and average hippocampal cell loss in both pathologic groups. CONCLUSIONS: Ipsilateral FLAIR signal abnormalities occur in the majority of patients with isolated HS but are less frequent in those with dual pathology. The presence of increased FLAIR signal is correlated with higher hippocampal cell loss.

Adult↗
PubMed2001

Seizure outcome after functional hemispherectomy for malformations of cortical development.

MRI features were correlated with postsurgical seizure outcome in patients with hemispheric malformations of cortical development (MCD). After functional hemispherectomy, 5 of 6 patients (83%) with hemimegalencephaly had persistent, although markedly improved, seizures; 5 of 6 patients (83%) with relative preservation of part of one lobe or atrophy were seizure free. Hemimegalencephaly and other types of hemispheric MCD appear to differ in prognosis for freedom from seizures after functional hemispherectomy.

Brain↗
PubMed2001

Temporal lobe epilepsy due to hippocampal sclerosis in pediatric candidates for epilepsy surgery.

OBJECTIVE: To characterize the clinical, EEG, MRI, and histopathologic features and explore seizure outcome in pediatric candidates for epilepsy surgery who have temporal lobe epilepsy (TLE) caused by hippocampal sclerosis (HS). METHODS: The authors studied 17 children (4 to 12 years of age) and 17 adolescents (13 to 20 years of age) who had anteromesial temporal resection between 1990 and 1998. RESULTS: All patients had seizures characterized by decreased awareness and responsiveness. Automatisms were typically mild to moderate in children and moderate to marked in adolescents. Among adolescents, interictal spikes were almost exclusively unilateral anterior temporal, as opposed to children in whom anterior temporal spikes were associated with mid/posterior temporal, bilateral temporal, extratemporal, or generalized spikes in 60% of cases. MRI showed hippocampal sclerosis on the side of EEG seizure onset in all patients. Fifty-four percent of children and 56% of adolescents had significant asymmetry of total hippocampal volumes, whereas the remaining patients had only focal atrophy of the hippocampal head or body. Subtle MRI abnormalities of ipsilateral temporal neocortex were seen in all children and 60% of adolescents studied with FLAIR images. On histopathology, there was an unexpectedly high frequency of dual pathology with mild to moderate cortical dysplasia as well as HS, seen in 79% of children and adolescents. Seventy-eight percent of patients were free of seizures at follow-up (mean, 2.6 years). A tendency for lower seizure-free outcome was observed in patients with bilateral temporal interictal sharp waves or bilateral HS on MRI. The presence of dual pathology did not portend poor postsurgical outcome. CONCLUSIONS: TLE caused by HS similar to those in adults were seen in children as young as 4 years of age. Focal hippocampal atrophy seen on MRI often was not reflected in total hippocampal volumetry. Children may have an especially high frequency of dual pathology, with mild to moderate cortical dysplasia as well as HS, and MRI usually, but not always, predicts this finding. Postsurgical seizure outcome is similar to that in adult series.

Adolescent↗
PubMed2001

Postictal diffusion-weighted imaging for the localization of focal epileptic areas in temporal lobe epilepsy.

PURPOSE: Diffusion-weighted MR imaging (DWI) is a novel technique to delineate focal areas of cytotoxic edema of various etiologies. We hypothesized that DWI may also detect the epileptogenic region and adjacent areas during the ictal and early postictal periods in patients with temporal lobe epilepsy (TLE). METHODS: We studied patients with intractable TLE (n = 9), due to hippocampal sclerosis (HS, n = 7), left mesial temporal lobe tumor (n = 1), and of unknown etiology (n = 1). Informed consent was obtained before inclusion in the study. All patients with single short seizures were scanned immediately after EEG-documented seizures (between 45 and 150 min); one of two patients in status was scanned 14 h after cessation of seizures. DWI results were analyzed visually and by calculating apparent diffusion coefficient (ADC) maps. RESULTS: We found significant decreases in ADC postictally in one of six patients with TLE due to HS and single short seizures. One patient with an incompletely resected temporal lobe tumor also exhibited ADC abnormalities. One patient in focal status epilepticus revealed a decrease in ADC, and one patient with a continuous aura had no DWI abnormality. CONCLUSIONS: Postictal DWI technique may occasionally help delineate epileptic areas in some patients with TLE. Yield is low in patients with HS and single short seizures: it may be higher in patients with tumor or status epilepticus.

Adolescent↗
PubMed2001

Telangiectatic osteosarcoma of the extremity: neoadjuvant chemotherapy in 24 cases.

Between April 1990 and December 1994, we treated 24 patients with telangiectatic osteosarcoma (TO) of the extremities with neoadjuvant chemotherapy using 2 protocols. Surgery consisted of limb salvage in 21 patients and amputation or rotation plasty in 3. The histologic response to chemotherapy was good (90% or more tumor necrosis) in 23 patients, of whom 12 had total necrosis. With a mean follow-up of 74 (60-96) months, 20 patients remained continuously free of disease and 4 relapsed with lung metastases. There were no local recurrences. Comparing these results to the ones achieved in 269 contemporary patients with conventional osteosarcoma of the extremities using the same protocols for chemotherapy, we found a significantly better histologic response to chemotherapy (96% vs 68% of good histologic response; p = 0.004) and disease-free survival (83% vs 55%; p = 0.01) in the TO group. We conclude that TO, once considered a lethal tumor, seems to be even more sensitive to chemotherapy than conventional osteosarcoma, and that most of these patients may be cured without amputation.

Adolescent↗
PubMed2001

Osteoid osteoma and osteoblastoma of the sacrum.

This retrospective study examined 10 patients with osteoid osteoma and 11 patients with osteoblastoma localized in the sacrococcyx. In the sacrum, the diagnosis was delayed compared to other sites. Curettage through a posterior approach is the treatment of choice. Radiotherapy as well as embolization of feeding arteries may be used for the most aggressive lesions. Prognosis is generally good with a low incidence of local recurrence (<10%).

Adolescent↗
PubMed2000

Long-term outcome for patients with nonmetastatic osteosarcoma of the extremity treated at the istituto ortopedico rizzoli according to the istituto ortopedico rizzoli/osteosarcoma-2 protocol: an updated report.

PURPOSE: To provide an estimate of long-term prognosis for patients with osteosarcoma of the extremity treated in a single institution with neoadjuvant chemotherapy and observed for at least 10 years. PATIENTS AND METHODS: Patients with nonmetastatic osteosarcoma of the extremity were preoperatively treated with high-dose methotrexate, cisplatin, and doxorubicin (ADM). Postoperatively, good responders (90% or more tumor necrosis) received the same three drugs used before surgery, whereas poor responders (less than 90% tumor necrosis) received ifosfamide and etoposide in addition to those three drugs. RESULTS: For the 164 patients who entered the study between September 1986 and December 1989, surgery was a limb salvage in 136 cases (82%) and a good histologic response was observed in 117 patients (71%). At a follow-up ranging from 10 to 13 years (median, 11.5 years), 101 patients (61%) remained continuously free of disease, 61 relapsed, and two died of ADM-induced cardiotoxicity. There were no differences in prognosis between good and poor responding patients. ADM-induced cardiotoxicity (six patients), male infertility (10 of the 12 assessable patients), and second malignancies (seven patients) were the major complications of chemotherapy. Despite the large number of limb salvages performed, only four local recurrences (2.4%) were registered. CONCLUSION: With an aggressive neoadjuvant chemotherapy, it is possible to cure more than 60% of patients with nonmetastatic osteosarcoma of the extremity and amputation may be avoided in more than 80% of them. Because local or systemic relapses, myocardiopathies, and second malignancies are possible even 5 years or more after the beginning of treatment, a long-term follow-up is recommended for these patients.

Adolescent↗
PubMed2000

Neoadjuvant chemotherapy for Ewing's sarcoma of bone in patients older than thirty-nine years.

The aim of this study was to determine whether the behaviour of Ewing's sarcoma of bone in adult patients is the same as that observed in children and adolescents. We reviewed the clinical features and outcomes of 23 patients over the age of 39 (17 males, 6 females) who had been treated with neoadjuvant chemotherapy between 1983 and 1995 at our institution. The most common primary sites of tumor were the extremities (16 cases); tumor volume was more than 100 ml in 17 patients, and elevated serum LDH levels were found in 6 cases. The local treatment was surgery in 8 cases, surgery plus radiotherapy in 8, and radiotherapy alone in 7 cases. Chemotherapy comprised a 4-drug regimen in 10 patients, while the other 13 patients received 6 drugs. At a follow-up of 8.8 years (3.5-15) 13 patients remained continuously free of disease and 10 relapsed. The 5-year disease-free survival and overall survival rates were 53% and 59%, respectively. Clinical features, dose intensity, and toxicity of chemotherapy, as well as the outcome of these 23 patients were found to be exactly comparable to the findings observed in 327 patients younger than 40 years treated at our institution in the same period, with the same therapy. We conclude that Ewing's sarcoma of bone in adults is no different from that occurring in children, and we therefore recommend the inclusion of all adult patients in multidisciplinary treatment trials of this tumor.

Adult↗
PubMed2000

Analysis of 12q13-15 genes in parosteal osteosarcoma.

The region q13-15 of chromosome 12 frequently is altered in human sarcomas, and several genes, such as SAS, CDK4, and MDM2, have been found to be amplified in bone and soft tissue sarcomas. These genes and their products were studied by quantitative polymerase chain reaction and immunohistochemical analysis in 25 parosteal osteosarcoma samples (22 Grades I or II, three dedifferentiated) to evaluate if the possible alterations detected of the genes on chromosome 12 could have a role in the development of this rare bone tumor. Immunohistochemical analysis was performed on formalin fixed, paraffin embedded tumor sections to evaluate CDK4 and MDM2 protein expression. To measure the degree of SAS and CDK4 gene amplification, quantitative polymerase chain reaction was done on deoxyribonucleic acid derived from the same samples. The results showed that CDK4 protein was expressed in 92% of the cases. Strong and uniform CDK4 and MDM2 immunoreactivity was found respectively in three of three and two of three dedifferentiated parosteal osteosarcomas. SAS and CDK4 genes were found to be amplified fourfold in two Grade II tumors and in one dedifferentiated tumor. These findings, which should be investigated further, might suggest a possible role of the chromosome 12 genes in the pathogenesis of parosteal osteosarcoma.

Bone Neoplasms↗
PubMed1999

Italian Cooperative Study for the treatment of children and young adults with localized Ewing sarcoma of bone: a preliminary report of 6 years of experience.

BACKGROUND: In 1991, the Italian Association for Pediatric Hematology-Oncology and the National Council of Research (CNR) initiated an Italian Cooperative Study (SE 91-CNR Protocol) with the main objective of improving the overall survival (SUR) and the event free survival (EFS) of children and young adults with localized Ewing sarcoma and primitive neuroectodermal tumors of bone compared with a previous study (IOR/Ew2 Protocol). METHODS: Between November 1991 and November 1997, 165 patients were enrolled in this study, 160 of whom were evaluable. The patients were treated with a multimodal approach characterized by intensified chemotherapy, hyperfractionated and accelerated radiation therapy, and the addition of ifosfamide and etoposide to standard chemotherapy with vincristine, actinomycin-D, doxorubicin, and cyclophosphamide. RESULTS: After a median follow-up of 37 months, 126 of the 160 evaluable patients remained free of disease recurrence. Thirty-one patients developed a disease recurrence (20 with disseminated disease). CONCLUSIONS: The 3-year SUR and EFS rates found in the current study (83.6% and 77.8%, respectively) may be considered satisfactory. Only age at diagnosis < or =14 years and a good histologic response appeared to affect the outcome of patients with localized Ewing sarcoma positively. These results appear to demonstrate the efficacy of the addition of ifosfamide in induction chemotherapy to four-drug standard combination chemotherapy, as confirmed by the improved outcome in terms of 3-year EFS reported in the SE 91-CNR Protocol compared with the IOR/Ew2 Protocol (77.8% vs. 60.7%). In addition, the better outcome also could be explained by the change in treatment strategy with a trend toward the use of more surgery than radiation therapy compared with the authors' previous protocol.

Adolescent↗