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Paget's disease of bone is a localized disorder of bony resorption. The mechanism underlying the development of the disease remains controversial. There is substantial evidence suggesting a genetic basis for Paget's disease in some patients. A viral etiology of Paget's disease has been advocated. A further hypothesis implicating an immunological mechanism for this disease is based on growing evidence reviewed in the text. The presented case showed clinical and X-ray features typical of a very aggressive form of Paget's disease. We hypothesize that the extreme local aggressiveness of this case was secondary to the patient's concomitant immunosuppression due to an extended therapy following renal transplant.

The filum terminale is an exceptional location for isolated hemangioblastoma. Only five cases are reported in literature, prior to the magnetic resonance imaging era. A 57-year-old man was referred to our Department with a prolonged history of progressive back pain, particularly severe when recumbent, and recurrent unilateral sciatalgia. Computed tomography demonstrated a non-homogeneous mass at L4 level. Magnetic resonance imaging displayed earliness and homogeneous enhancement of the mass with tortuous vessels above the rostral pole of the tumour, suggesting the diagnosis of a vascular tumour. The tumour was totally removed. Histological examination confirmed the hemangioblastoma diagnosis. The case indicates that hemangioblastoma, although uncommon, must be taken into consideration in cauda equina tumour diagnosis, and also emphasises the specificity of magnetic resonance features.

We report a patient in whom the distal radius was resected for a giant cell tumour and the bone defect was replaced using a vascularized proximal fibular graft. The graft was viable and hypertrophied and normal callus formed on the distal radius. Due to chronic instability of the wrist the patient underwent revision arthrodesis 1 year after resection. Microscopic studies of the epishyseal region of the fibula showed wide necrosis of the graft with active creeping substitution. Despite the good technical result of the vascularized fibular graft, the vascularization was incomplete in the proximal epiphysis. We discuss possible reasons for this.

We describe a surgical technique for replacing the hip joint capsule using synthetic mesh after oncological resections of the proximal femur that resulted in gross intraoperative instability of the prosthetic reconstruction. The results of its use in 13 patients, 6 of whom also had pelvic resections, are described. These patients were selected from a total group of 88 patients undergoing proximal femoral replacement, 75 of whom did not require capsular replacement (none of these 75 patients have experienced dislocation). In the group requiring capsular reconstruction, 1 of 4 patients with bipolar hemiarthroplasty and 4 of 9 patients with total hip replacements experienced dislocation after operation. Of the dislocated total hip replacements, 1 remains chronically dislocated, and 3 were successfully stabilized by open reduction with further capsular augmentation. Given that the resections involved removal of most of the soft tissues stabilizing the hip joint, we believe that the technique of capsular reconstruction is useful in this difficult group of patients.

A variety of reconstructive procedures have been suggested for stabilizing the ulnar shaft following resection of the distal ulna for tumour. We present the results of a series of nine distal ulnar tumour resections in which four different stabilization techniques were employed. We based our results on an evaluation of function, pain, motion, strength and instability. We obtained good or excellent results in seven patients treated with a soft tissue stabilization of the ulnar stump. One patient did not undergo any stabilization procedure and scored fair in our system. A further patient who required a radiocarpal arthrodesis also had an inferior result. These results suggest that soft tissue stabilization of the ulnar stump should be performed whenever possible.

Primary central nervous system non-Hodgkin's malignant lymphomas (PCNSNHML) have undergone a remarkable increase in incidence in the last years. Clinically PCNSNHML may present with the symptoms that mimic many others neurological conditions. Radiologically they appear as iso or hyperdense deposits, often multiple, usually supratentorial in periventricular region. Histologically although most lesions are high grade B cell lymphomas, T cell lymphomas are being recognised with increasing frequency. Actually a substantial improvement in survival has been obtained using chemotherapy as sole treatment, eventually followed by radiotherapy for recurrences. The prognosis, however, remains poor in comparison with nodal lymphomas. It is improved by T-cell phenotype, a Karnofsky performance status more than 50%, age at onset less than 50 years and negativity for bcl-2 oncogene of neoplastic cells.

A series of 6 patients with primary T-cell lymphomas of the central nervous system (PTCLCNS) is presented. The clinical, histological and immunohistological findings are shown. The lesions were infratentorial in the 34% and sopratentorial in the 66% of the cases. Of the 5 cases with an available follow-up only one died in the first year after diagnosis and one is alive and well after 42 months. These data agree with those of others series of PTCLCNS in the literature. In conclusion our data and those of the literature suggest that immunophenotyping should routinely be carried in primary non-Hodgkin's lymphomas of the CNS (PNHLCNS), because the T-cell forms run a more favourable course than the B-cell varieties.

We report on data gathered from five European centres regarding 147 primary non-Hodgkin Lymphomas (NHLs) of the Central Nervous System (CNS) in HIV-negative patients. The results lead us to make the following considerations: i) there has been a significant and progressive increase in the frequency of observation of this pathology during the course of the last two decades; ii) the pathology lacks specific characteristic symptoms; iii) the radiological profile, as observed by CAT and/or MNR, most frequently corresponds to an isodense or slightly hyperdense lesion which has clear margins and is capable of assuming the contrast medium homogeneously; iv) the tumour most often has a single supratentorial localisation; v) high grade B-cell lymphomas account for 66% of the observations, low grade B-cell varieties being relatively rare and cases of T-cell derivation exceptional (6/147); vi) immunohistochemistry allows the differential diagnoses with respect to primitive or secondary non-lymphoid tumours, and provides confirmation of the histogenetic assessment made on morphological grounds; vii) the course of the disease is not significantly influenced by the histotype, the phenotype, the number of lesions present or the chemotherapy regimen, but rather by the employment of combined surgery and radio- or radiochemotherapy. This study represents the largest series of CNS NHLs so far reported, and as such, provides precise clinico-pathological indications which were only partially obtainable from the relatively small previously published series. Some concluding remarks are made as to the genesis of CNS NHLs, along with some practical suggestions for reaching a better understanding of their complex biology.

We report an unusual and possibly unique example of cerebral ganglioglioma with an anaplastic oligodendroglial component. The latter was documented on both morphological and immunohistochemical grounds. Immunohistochemically, the anaplastic cells were strongly positive with the monoclonal antibody anti-Leu-7, while they lacked glial fibrillary acid protein, vimentin and neurofilaments.

We reviewed the cases of 16 patients with primary intra-cranial lymphomas. The clinical and radiological data proved to be helpful in suggesting the diagnosis, but only tumoral biopsy provided unequivocal diagnosis. In our cases radiotherapy proved to be the most effective treatment

To assess the value of transbronchial lung biopsy in the evaluation of disease activity in pulmonary sarcoidosis, lung biopsy specimens obtained from 37 patients with this disease and their cellular patterns of bronchoalveolar lavage were studied. Morphologic analysis has showed peculiar lesions: predominant interstitial alveolitis consisting of mononuclear cells and scattered neutrophils, and eosinophils, diffuse in 13 cases and focal in 11 cases, interstitial nodular clusterings of mononuclear cells (five cases), diffuse intraalveolar infiltration of macrophages (one case), granulomas (27 cases), hyaline membranes (one case), intraalveolar (two cases) and interstitial (six cases) fibrosis, and finally cuboidal metaplasia of alveolar lining cells (eight cases). Hyaline membranes were always combined to a diffuse alveolitis consisting of some neutrophils. Only diffuse alveolitis was significantly associated with a high lymphocytosis in BAL (p less than 0.05).