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We present a case of disseminated aspergillosis treated successfully with high-dose liposomal amphotericin B in a 16-year-old boy with primary immunodeficiency (autoimmune lymphoproliferative syndrome, ALPS). Sites of infection, including the brain, lung and multiple intra-abdominal organs, were studied using 18F-FDG and PET/MRI, which provided important morphological and functional information for monitoring the course of the disease.

Learning Objectives After completing this course, the reader will be able to: Compare the use of i.t. therapy and systemic therapies for patients with neoplastic meningitis.Describe new drugs showing promise for neoplastic meningitis. This article is available for continuing medical education credit at CME.TheOncologist.com Neoplastic meningitis is a result of the spread of malignant cells to the leptomeninges and subarachnoid space and their dissemination within the cerebrospinal fluid. This event occurs in 4%–15% of all patients with solid tumors and represents an important prognostic factor for poor survival. Neoplastic meningitis should be diagnosed in the early stages of disease to prevent important neurological deficits and to provide the most appropriate treatment. Despite new diagnostic approaches developed in recent years, such as positron emission tomography–computed tomography and new biological markers, the combination of magnetic resonance imaging without and with gadolinium enhancement and cytology still has the greatest diagnostic sensitivity. Recently, no new randomized studies comparing intrathecal (i.t.) with systemic treatment have been performed, yet there have been a few small phase II studies and case reports about new molecularly targeted substances whose successful i.t. or systemic application has been reported. Trastuzumab, gefitinib, and sorafenib are examples of possible future treatments for neoplastic meningitis, in order to better individualize therapy thus allowing better outcomes. In this review, we analyze the most recent and interesting developments on diagnostic and therapeutic approaches.

Background: Hemiplegic migraine (HM) is a rare variety of migraine with aura, characterized by motor deficits during the aura, often beginning in childhood. The hemiplegic attacks can be severe and prolonged but the prognosis is usually good. Data on neuroimaging, including diffusion-weighted imaging (DWI) and spectroscopy, during prolonged attacks of HM are quite limited, particularly in children. Case: An eight-year-old female had a prolonged attack of sporadic HM characterized by right-sided hemiplegia, global aphasia, fever and impairment of consciousness. MRI nine hours after hemiplegia onset was negative, while the following MRI scans (days 4 and 11) documented a progressive increase in cortical swelling in the left hemisphere with mild hyperintensity on DWI and mild reduction of apparent diffusion coefficient values. Proton MRI spectroscopy (MRS) (day 15) showed a decrease in the N-acetylaspartate/creatine ratio in the left hemisphere. 99mTc-ECD single-photon emission tomography (SPET) (day 27) showed marked left hemispheric hypoperfusion. The patient recovered completely after 40 days and neuroimaging follow-up (MRI and SPET) after six months was normal. The patient carried a missense mutation of the ATP1A2 gene. Conclusion: Multimodal neuroimaging (MRI, DWI, MRS, SPET) in a prolonged HM attack supports evidence for a primary neuronal dysfunction.

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PURPOSE A morphofunctional approach to the management of brain tumours has been claimed to increase diagnostic accuracy. Among the proposed single-photon emission tomography (SPET) tracers, (99m)Tc-sestamibi is able to distinguish recurrent tumour from radio-necrosis and to identify early response or resistance to chemotherapy. Major drawbacks of sestamibi, that is, poor morphological resolution and the sites of physiological uptake, could be overcome by dual-modality, integrated systems. The purpose of this study was to investigate the real usefulness of (99m)Tc-sestamibi SPET/computed tomography (CT) and to establish a semiquantitative index. METHODS Charts from 33 consecutive patients selected for surgery, who underwent preoperative SPET/CT and magnetic resonance imaging (MRI), were reviewed. Tumours were confirmed histologically after the surgery in all patients and classified according to WHO recommendations. Semiquantitative indexes were obtained on images (maximum likelihood expectation maximization reconstructed) with and without attenuation correction and visual analysis of SPET versus SPET/CT was performed. RESULTS A significant statistical difference was shown between SPET and SPET/CT in terms of the delineation of medial shift, oedema and the ability to distinguish tumour from the skull-meninges complex and plexus. With regard to semiquantitative indexes, a ratio obtained comparing counts/pixel derived from a region of interest in the tumour area with mirrored region of interest in the contralateral site revealed a sensitivity of 90.9% and specificity of 71.45% in discriminating WHO grade 4 gliomas from a lower grade. CONCLUSION SPET/CT can distinguish tumour from the skull and other sites of physiological uptake better than SPET alone (as confirmed by MRI in all cases) and affords a morphological map. The proposed semiquantitative index also seems promising in identifying higher-grade disease. SPET/CT thus seems a useful additional tool in brain tumour management, especially when MRI is not feasible or PET/CT is not available.

Aims and background The incidental finding of nonfunctioning adrenal masses (incidentalomas) is common, but no reliable criteria in differentiating between benign and malignant adrenal masses have been defined. The aim of this preliminary study was to assess the usefulness of adrenal imaging and image-guided fine-needle aspiration cytology in patients with nonfunctioning adrenal incidentalomas with the aim of excluding or confirming malignancy before surgery. Methods Forty-two consecutive patients (18 men and 24 women; median age, 54 years; range, 25-75 years) with incidentally discovered adrenal masses of 3 cm or more in the greatest diameter were prospectively enrolled in the study. All patients underwent helical computerized tomography scan and image-guided fine-needle aspiration cytology, 33 (78.6%) underwent magnetic resonance imaging, and 26 (61.9%) underwent norcholesterol scintigraphy before adrenalectomy. Results The revised final pathology showed 30 (71.4%) benign (26 adrenocortical adenomas, of which 3 were atypical, 2 ganglioneuromas, and 2 nonfunctioning benign pheochromocytomas) and 12 (28.6%, 95% CI = 15-42) adrenal malignancies (8 adrenocortical carcinomas and 4 unsuspected adrenal metastases). The definitive diagnosis of adrenocortical carcinoma was made according to Weiss criteria and confirmed on the basis of local invasion at surgery or metastases. The sensitivity, specificity and accuracy were 75%, 67% and 83% for computerized tomography scan, 92%, 95% and 94% for magnetic resonance imaging, 89%, 94% and 92% for norcholesterol scintigraphy, and 92%, 100% and 98% for fine-needle aspiration cytology. The sensitivity and accuracy of image-guided fine-needle aspiration cytology and magnetic resonance imaging together reached 100%. Immediate periprocedural complications of fine-needle aspiration cytology occurred in 2 (4.7%) patients: self-limited pneumothorax (n = 1), and severe pain (n = 1) requiring analgesic therapy. No postprocedural or late complications were observed. Conclusions With the aim of selecting for surgery patients with a non-functioning adrenal incidentaloma of 3 cm or more in diameter, the combination of magnetic resonance imaging and fine-needle aspiration cytology should be considered the strategy of choice.

Introduction. It is well known that resolution on a gamma camera varies as a function of distance, scatter and the camera’s characteristics (collimator type, crystal thickness, intrinsic resolution etc). Manufacturers frequently provide only a few pre-calculated resolution values (using a line source in air, 10–15 cm from the collimator surface and without scattering). However, these are typically not obtained in situations resembling a clinical setting. From a diagnostic point of view, it is useful to know the expected resolution of a gamma camera at a given distance from the collimator surface for a particular setting in order to decide whether it is worth scanning patients with “small lesion” or not. When dealing with absolute quantification it is also mandatory to know precisely the expected resolution and its uncertainty in order to make appropriate corrections. Aim. Our aims are: to test a novel mathematical approach, the cubic spline interpolation, for the extraction of the full width at half maximum (FWHM) from the acquisition of a line source (experimental resolution) also considering measurement uncertainty; to compare it with the usually adopted methods such as the gaussian approach; to compare it with the theoretical resolution (analytical resolution) of a gamma camera at different distances; to create a web-based educational program with which to test these theories. Methods. Three mathematical methods (direct calculation, global interpolation using gaussian and local interpolation using splines) for calculating FWHM from a line source (planar scintigraphy) were tested and compared. A NEMA Triple Line Source Phantom was used to obtain static images both in air and with different scattering levels. An advanced, open-source software (MATLAB/Octave and PHP based) was created “ad hoc” to obtain and compare FWHM values and relative uncertainty. Results and Conclusion. Local interpolation using splines proved faster and more reliable than the usually-adopted Gaussian interpolation. The proposed freely available software proved effective in assessing both FWHM and its uncertainty.

Fotemustine is a third-generation nitrosourea showing efficacy in various types of tumors such as melanoma and glioma. We reviewed the most important studies on fotemustine treatment in glioma patients analyzing its pharmacological profile and its activity and safety. Fotemustine was used as single agent or in association with new targeted drugs such as bevacizumab; fotemustine was used both as first-line chemotherapy before temozolomide era and in refractory-temozolomide patients during temozolomide era. Finally, analyzing and comparing the activity and safety of fotemustine alone or in combination with bevacizumab versus other nitrosoureas such as lomustine, we may suggest that the combination treatment with bevacizumab and fotemustine may be active and tolerable in patients with high grade gliomas.

5-Aminolevulinic acid (5-ALA) fluorescence has been proved advantageous in glioma surgery. Conflicting results have been reported by few studies published in literature about intra-operative 5-ALA-induced fluorescence of medulloblastoma (MDB). The aim of this study is to verify if these conflicting results could be explained by intra-tumoral histological and phenotypic differences. In the present case of a 45-year-old patient affected by a cerebellar MDB, histological analysis of cell phenotype and 5-ALA and CD133 correlation were performed in multiple samples according to different fluorescence patterns. Intra-operatively, the tumor appeared unevenly fluorescent under blue-violet light. Histologically, 5-ALA-intense biopsies from inner areas were characterized by a significant amount of cancer cells, whereas 5-ALA faint regions from peripheral areas displayed normal cerebellar features, with MDB cells infiltrating healthy tissues. Presenting our findings, we show the correlation between different 5-ALA fluorescence patterns of medulloblastoma with specific histological and phenotypical features. Thus, we hypothesize that a distinct relationship between CD133 expression and fluorescence accumulation presented in our study could partially explain the divergent results published in literature.

We report on the case of a 61-year-old man with a soft tissue malignant myoepithelioma of the second toe of the right foot. After removal of the primary tumor, the patient developed in-transit metastases of the limb that we later treated with limb perfusion, using extracorporeal circulation with complete response. Following the appearance of lymph node metastases, the patient underwent inguinal, iliac and obturator lymphadenectomy. Subsequent pelvis metastases were treated with chemotherapy and radiotherapy, with complete response. Currently, after 3 years, the patient is alive and no evidence of any residual disease is apparent.

Address for correspondence: Dr Marco Rossato, MD, PhD, University of Padova, Department of Medicine, DIMED, Clinica Medica 3, 35128 Padova, Italy Clinical Pra ● Paraneoplastic syndromes refer to cancer-associated signs and symptoms arising in organs that are remote from the cancer and unrelated to metastases. ● Paraneoplastic neurological syndromes are rare, affecting only 0.01% of patients with cancer. About 3-5% of patients with small-cell lung cancer present paraneoplastic neurological syndromes. ● Generally paraneoplastic neurologic syndromes are due to the production of antibodies against neural antigens expressed by the tumor and that are present in normal neuronal tissues. ● Here we describe the case of a male patient with distal polyneuropathy due to production of auto-antibodies versus myelin associated glycoprotein (MAG) and nonsmall-cell lung cancer expressing MAG on cancer cells. ● The tumor and the immune system are both implicated in the development of paraneoplastic neurological syndromes that are generally thought to

Craniectomy, used to relieve refractory intracranial pressure in traumatic brain injury (TBI), may cause cognitive deficits which could be improved by skull breach repair. This paper studied whether late cranioplasty improves a specific pattern of cognitive functions.A case series of five TBI patients with craniectomy undergoing late cranioplasty (median interval time: 14 months, range: 12-36).Longitudinal neuropsychological and brain MRI assessments 1 week before cranioplasty and 3 months later.After cranioplasty, mean score of the verbal fluency test improved compared to pre-cranioplasty (p = 0.02). Similarly, significant improvements after cranioplasty were observed in other tests scores exploring executive functions, such as working memory (p = 0.03) and speed of attention (p = 0.04), independently from the size and site of cranioplasty.The cognitive improvement induced by cranioplasty, even when performed after a long interval from craniectomy, may be due to the restoration of physiological cerebrospinal fluid circulation which, in turn, allows an efficient brain volume transmission signal circulation. The restoration of this essential way of signal communication seems to affect large-scale neuronal networks responsible for the executive functions.

Objective We describe a patient with prodromal dementia with Lewy bodies (DLB) presenting with drug-induced visual hallucinations (VHs). Case report A 78-year-old woman complained of daytime recurrent VHs characterized by seeing her face and arms covered in fur and viewing moustaches on her daughter's face. VHs started a few days after the beginning of a combination therapy with duloxetine and lorazepam and ceased within 24 h after their discontinuation. Nonamnestic mild cognitive impairment with profound visual perception deficits and very mild extrapyramidal signs, with abnormal brain DaTscan single photon emission tomography, were present. Three years later, cognitive and neurological follow-up assessments supported the diagnosis of DLB. Conclusion Perturbation of cerebral serotonergic tone induced by duloxetine, associated with reduced attentional control due to benzodiazepine use, may be the physiopathological substrate of transient VHs in prodromal DLB.

Semantic dementia (SD) is a neurodegenerative disease belonging to the spectrum of frontotemporal dementia that presents with loss of memory for words and prevalent left temporal pole atrophy. Behavioral disorders, particularly obsessive-compulsive symptoms, are frequent during the course of the disease. We describe a patient presenting with late-onset typical obsessive–compulsive disorder (OCD) that lasted for 10 years as an isolated condition before developing clinical and neuroimaging features of SD. This case alerts clinicians that late-onset OCD may be a psychiatric presentation of a neurodegenerative disorder such as frontotemporal dementia and requires an accurate diagnostic work-up.

This study aimed to evaluate the efficiency of 18F-FDG PET/CT in suspected recurrence of epithelial ovarian cancer, after treatment, comparing outcomes of PET/CT with histological tumor subtype, CA-125 serum levels, and findings of conventional diagnostic imaging modalities (CI).Data from 121 women who underwent FDG PET/CT for suspected recurrence of epithelial ovarian cancer after treatment were reviewed retrospectively.Of all patients, 80% had recurrent disease and 20% were disease-free on the final clinical diagnosis. PET/CT showed true-positive findings in 82% of patients, whereas CI demonstrated true-positives in 70% of cases. At the time of PET/CT scanning, only 55 patients had serum CA-125 level greater than 35 U/mL, whereas 52 patients presented with CA-125 levels in a reference range. PET/CT sensitivity (82%) was significantly higher than that of CA-125 (59%), whereas difference in sensitivity between PET/CT and CI (69%) was limited. PET/CT specificity (87%) was significantly better than that of CI (47%), although no difference in specificity between PET/CT and CA-125 (80%) was found. However, no difference in CA-125 serum levels between patients with local tumor relapse and those with distant metastases was found. PET/CT showed the highest positive predictive value (96%) and negative predictive value (55%) when compared with other modalities. In high-grade tumors (n = 66), PET/CT accuracy was 80%, better than that of serum CA-125 (64%) and that of CI (62%). Equally in low-grade ovarian carcinomas (n = 55), PET/CT accuracy (87%) was significantly higher than that of the tumor marker (60%) and also higher than that of CI (70%).FDG PET/CT was proven to be more efficient than serum CA-125 assay and CI in detecting recurrences of ovarian cancer after treatment. The sensitivity of FDG PET/CT is not influenced by tumor histology. FDG PET/CT should be considered a useful diagnostic tool in the surveillance of patients that received treatment for epithelial ovarian carcinoma.

Owing to the frequent observation of poverty of movements, facial hypomimia and balance impairment, amyotrophic lateral sclerosis (ALS) variant with predominance of upper motor neuron involvement (UMN-ALS) is prone to be diagnosed with Parkinsonism. A clinical assessment, including the velocity-dependent stretch response test to differentiate between pyramidal and extrapyramidal stiffness; the Unified Parkinson's Disease Rating Scale and the Berg Balance Scale to assess degree of bradykinesia and postural instability; and (123)I-FP-CIT scintigraphy evaluation to investigate the nigrostriatal circuit involvement, were carried out to characterize Parkinson-like features in UMN-ALS patients. Sixteen UMN-ALS patients were included in the study. The velocity-dependent stretch response indicated spasticity in all the muscles tested. The degree of stiffness was found to be related to bradykinesia and postural instability. Eleven patients (70%) showed a reduction in striatal (123)I-FP-CIT uptake found to be related to disease duration and patients' ages but not to scores of the functional scales. Slowness of movements and postural instability noted in our patients could be mostly attributed to spasticity. The lack of any correlation between UPDRS or BBS scores and the degree of nigrostriatal impairment on DaTSCAN seems to disprove nigrostriatal circuit involvement in these extrapyramidal-like features.

Calcium is essential for many metabolic process, including nerve function, muscle contraction, and blood clotting. The metabolic pathways that contribute to maintain serum calcium levels are bone remodeling processes, intestinal absorption and secretion, and renal handling, but hypercalcemia occurs when at least 2 of these 3 metabolic pathways are altered. Calcium metabolism mainly depends on the activity of parathyroid hormone (PTH). Its secretion is strictly controlled by the ionized serum calcium levels through a negative feed-back, which is achieved by the activation of calcium-sensing receptors (CaSRs) mainly expressed on the surface of the parathyroid cells. The PTH receptor in bone and kidney is now referred as PTHR1. The balance of PTH, calcitonin, and vitamin D has long been considered the main regulator of calcium metabolism, but the function of other actors, such as fibroblast growth factor-23 (FGF-23), Klotho, and TPRV5 should be considered. Primary hyperparathyroidism and malignancy are the most common causes of hypercalcemia, accounting for more than 90% of cases. Uncontrolled hypercalcemia may cause renal impairment, both temporary (alteration of renal tubular function) and progressive (relapsing nephrolithiasis), leading to a progressive loss of renal function, as well as severe bone diseases, and heart damages. Advances in the understanding of all actors of calcium homeostasis will be crucial, having several practical consequences in the treatment and prevention of hypercalcemia. This would allow to move from a support therapy, sometimes ineffective, to a specific and addressed therapy, especially in patients with chronic hypercalcemic conditions unsuitable for surgery.

Hypercalcemia is a relatively common clinical problem, mainly (>90%) related to primary hyperparathyroidism (HPT) and malignancies. The anatomical and functional imaging techniques available for locating enlarged parathyroid glands include ultrasound (US), computed tomography (CT), magnetic resonance imaging (MRI), and nuclear medicine imaging techniques. The most commonly employed are US and parathyroid scintigraphy, while CT, MRI, positron emission tomography (PET)/CT, and selective venous sampling are generally used in patients with persistent or recurrent HPT, or when findings of non-invasive studies are negative or conflicting. The reported accuracy is 57-93%, 54-93%, and up to 95% for US, (99m)Tc-sestamibi scintigraphy, and the two modalities combined, respectively. A multimodality approach (x-ray, whole-body scintigraphy, CT, MRI, and PET) is usually recommended for whole body assessment in cases of cancer-induced hypercalcemia (CIH). Imaging studies should evaluate each organ (i.e. breast, kidney, prostate, parathyroid) potentially involved in the pathogenesis of hypercalcemia in patients with CIH. In cases of skeletal metastases, when findings on plain x-ray or bone scans are uncertain, any unexplained region of abnormal uptake should be examined by MRI and/or ¹⁸F-fluoro-2- deoxyglucose (FDG)-PET/CT, which has proved more accurate than classical bone scintigraphy, especially for dealing with hematologic malignancies. A number of radionuclide tracers, other than ¹⁸F-FDG, are available for use in selected cases to locate specific tumors (i.e. ⁶⁸Ga for neuroendocrine tumors). This is a review of recently published information on the imaging techniques currently available for patients with hypercalcemia.

Intestinal-type adenocarcinoma of the nasal cavities and paranasal sinuses is a relatively rare tumor. Standard therapeutic modalities include surgery followed by radiotherapy, sometimes with chemotherapy treatment. Despite these treatments, the outcome is poor due to frequent local recurrences constituting the main cause of death among patients; leptomeningeal carcinomatosis is not a frequent event, and its presence indicates short expected survival. The therapy of neoplastic meningitis includes cranial irradiation, intrathecal chemotherapy and high-dose systemic chemotherapy. However, these approaches report important side effects with only modest efficacy. Thus, it is important to discover better treatment for this cancer complication. We present, for the first time, a case of leptomeningeal carcinomatosis from invasive intestinal-type adenocarcinoma treated with temozolomide and cisplatin chemotherapy obtaining a prolonged reduction and stabilization of the lesion improving the clinical condition of the patient.

Intracranial bleeding is rare in patients with low-grade gliomas, above all in adult population. We reviewed the literature of such cases and reported another case of a haemorrhagic low-grade glioma in a 54-year-old woman presenting with a left hemiparesis. Computer tomography (CT) images showed a right basal ganglia haemorrhage with no mass effect. Vascular malformations were ruled out by angiography. Eighteen fluoro-fluoro deossiglucosio (18F-FDG) positron emission tomography (PET/CT) showed a large hypometabolic area corresponding to the lesion. We waited for patient's improvement. Late magnetic resonance images revealed a low-grade glioma at the bleeding site. Tumour was removed and histopathologic examination revealed a WHO grade II mixed glioma. The authors emphasize that this evidence has to be kept in mind since it has important therapeutic implications.

The aim of this study was to evaluate the sensitivity, specificity and accuracy of axillary ultrasonography (US) and (99m)Tc-sestamibi scintimammography (SSM) in patients with breast cancer (BC) undergoing curative surgery.A series of 77 consecutive women (median age 54 years, range 36-70) with primary BC underwent both US and SSM from 2 to 15 (median 4) days prior to curative surgery. The results of imaging studies were compared against the final pathology. Breast-conserving surgery with axillary node (AN) dissection was performed in 49 (63.6%) patients, and modified radical mastectomy in 28 (36.4%) patients, according to the tumour staging.Final pathology showed 5 pT1bN0, 1 pT1bN1, 28 pT1cN0, 19 pT1cN1, 7 pT2N0, and 17 pT2N1 BC. Overall, 719 AN were removed of which 106 (14.7%) were metastatized nodes (median 3, range 1-5 per patient). The sensitivity, specificity and accuracy were 67.6%, 80.0%, and 74.0% for US, 78.4%, 85.0%, and 81.8% for SSM, and 91.9%, 92.5%, and 92.2% for US and SSM together, respectively. There was a significant difference (p0.05) in the number of metastatized AN between patients with metastases correctly detected and undetected by both US (3.1+/-1.3 vs. 2.0+/-0.7) and SSM (3.2+/-1.3 vs. 1.7+/-0.7).Although the results of each diagnostic tests are strictly dependent on the number of the metastatized AN, the combination of axillary US and SSM is a sensitive low-cost procedure that should be suggested in all patients with BC, when a preoperative evaluation of the AN status is required.

Summary The complementary role of sestamibi scintimammography (SSM) in patients with breast cancer (BC) is well established. The aim of this study was to establish whether a relationship exists between sestamibi uptake, evaluated as a tumour-to-background ratio (TBR), and the main prognostic factors of BC. SSM with the measurement of TBR was performed preoperatively in 102 women (median age 57 years, range 32–81 years) who underwent curative surgery for primary BC. Final pathology showed 4 (3.9%) with pT1a, 17 (16.7%) with pT1b, 44 (43.1%) with pT1c and 37 (36.3%) with pT2 breast carcinomas. The overall sensitivity of SSM was 80.4%. An ANOVA showed significant ( P 0.01 ) differences between the TBR of patients with G1 vs. G3 tumours, and between the TBR of those with G2 vs. G3 breast carcinomas. Moreover, there was a difference ( P = 0.021 ) between the TBR of patients ( n = 12 , 11.8%) with CEA serum levels >10 ng/ml (2.031±0.420), and those with normal ( n = 90 , 88.2%) CEA values (1.713±0.446), whilst no difference ( P =NS) was found between patients ( n = 27 , 26.5%) with CA 15-3 >30 U/ml (1.893±0.401) and those with normal ( n = 75 , 73.5%) CA 15-3 values (1.699±0.462). There was a mild inverse correlation between TBR and both the oestrogen ( R = 0.25 , P = 0.011 ) and the progesterone receptor ( R = 0.23 , P = 0.02 ) rate. The logistic regression analysis showed that only size and CA 15-3 serum levels represent true independent parameters, but the function was able to predict only 11 out of 21 (52.4%) patients with false-negative SSM. TBR is independent of age and mainly correlates with the size of the tumour. There are no reliable preoperative prognostic factors that are really useful for improving SSM sensitivity in patients with small breast carcinomas.

As observed by other authors, normal adrenal medullary tissue frequently gives an apparently positive meta-iodobenzylguanidine (MIBG) scan in cases studied using 123I-MIBG and less frequently 131I-MIBG. The aim of this study was to assess the usefulness of a scoring system, based on different uptakes of the radiopharmaceutical, to improve the accuracy of 123I-MIBG scintigraphy in patients with either adrenal or extra-adrenal pheochromocytomas. Charts from 67 consecutive patients (29 males and 38 females, median age 48 years, range 14–80 years) with suspected pheochromocytoma (either sporadic or familial: multiple endocrine neoplasia (MEN) 2a, MEN2b, Von Hippel–Lindau, neurofibromatosis type 1) who underwent 123I-MIBG scintigraphy (scans acquired 4–24 h after injection) from 1991 to 2004, were independently reviewed by two experienced nuclear medicine physicians using liver uptake as a reference (scores: 1, uptake absent or less than the liver; 2, equal to the liver; 3, moderately more intense than the liver; 4, markedly more intense than the liver). Interfering medications were discontinued for the appropriate time before MIBG injection. Histological data were obtained for all patients who underwent adrenalectomy. Scintigraphies were classified as positive using the following criteria: extra-adrenal focal uptake, adrenal enlargement together with non-homogeneous uptake and adrenal uptake more intense than the liver (score 3–4). After surgical resection, as confirmed by histological findings and long-term follow-up (range 1–14 years, average 9.25 years), 43 patients were considered true positives using the proposed scoring system, 20 were true negatives, four were false negatives and none was false positive. In conclusion, the proposed scoring system demonstrated high specificity (100%), sensitivity (91.5%) and accuracy (94%) in the management of pheochromocytoma. Positive predictive value and negative predictive value were 100% and 83.3% respectively. Normal adrenal tissue uptake was correctly discriminated from pheochromocytomas in 18 out of 20 patients, with adrenal uptake equal to the liver (grade 2), using the proposed cut-off level.

Objective To describe a patient with behavioral variant frontotemporal dementia (bvFTD) presenting with impulse control disorders (ICDs) which responded to fluvoxamine and topiramate. Case report A 64-year-old woman was affected by several ICDs. At disease onset, she suffered from impulsive smoking and overeating which caused a body weight increase of 20 kg in 6 months. Later on she manifested binge-eating behavior and skin-picking compulsion. Presence of progressive frontal cognitive impairment (Mini Mental State Examination 24/30) and evidence of hypoperfusion of the anterior cingulate and dorsolateral frontal cortex with brain single-photon emission computed tomography scan contributed to the diagnosis of bvFTD. Use of combination treatment with selective serotonin reuptake inhibitor drugs and topiramate improved all these symptoms. Conclusion This case extends the clinical phenotype of repetitive and compulsive habits in bvFTD to encompass symptoms suggestive of ICDs. It is proposed that fluvoxamine and topiramate may be considered as treatment options in these conditions.

44 consecutive patients with confirmed primary hyperparathyroidism (HPT) undergoing surgery were prospectively enrolled in the study. There were 13 (29.5%) men and 31 (70.5%) women with an overall median age of 59 years (range 23-78 years). Prior to successful parathyroidectomy both (99)Tc(m)-sestamibi/(99)Tc(m)-pertechnetate subtraction scintigraphy (SS) and helical CT were performed, and the results of imaging studies were compared against intraoperative findings. Final histopathology showed 40 (90.9%) solitary parathyroid (PT) adenomata (median size 18 mm, range 8-40 mm), that were in an ectopic location in 13 (32.5%) patients. Moreover, 3 (6.8%) patients had multiglandular disease (one patient with two PT adenomata, two patients with PT hyperplasia), while one (2.3%) patient had a PT carcinoma. The sensitivity and positive predictive value were 86.0% and 97.4% for SS, 88.1% and 94.9% for CT, and 100% and 97.4% for the combination of SS and CT, respectively. Calcium and parathyroid hormone (PTH) serum levels, and the mean size of the removed PT glands of patients with false negative results were lower than that of those with true positive results, but the difference was not significant. Among patients with solitary PT tumours (N=41) the sensitivity was 88.1% and 90.3% for SS and CT-scan, respectively. In conclusion, our study confirms the usefulness of SS, which should be the initial test for patients undergoing parathyroidectomy. However, the strategy of performing two tests in each patient with primary HPT could be of limited utility when the initial SS is positive.

The aim of this study was to evaluate the accuracy of fine-needle aspiration (FNA) cytology and thyroid scintigraphy (TS) in patients with solitary thyroid nodules. We retrospectively reviewed a series of 657 consecutive patients (531 (80.8%) women and 126 (19.2%) men, median age 45 years, range 16-81 years) with solitary thyroid nodules. Prior to surgery, all patients underwent FNA cytology whilst 99mTc-pertechnetate TS was performed in 496 (75.5%) patients. Final histopathology showed 533 (81.1%) benign nodules, including 251 (38.2%) follicular adenomas and 124 (18.9%) thyroid carcinomas. The sensitivity, specificity, positive predictive value, negative predictive value and accuracy in the detection of thyroid cancer were 95.8%, 21.1%, 22.6%, 95.5% and 35.7% for TS, and 81.4%, 99.4%, 97.1%, 95.8% and 96.0% for FNA cytology. The presence of thyroid cancer was confirmed in 92 out of 407 (22.6%) patients with a "cold" nodule, in 4 (4.5%) patients who had normal or increased uptake on TS, in 101 out of 104 (97.1%) patients with smears suggesting malignancy, and in 3 out of 533 (0.6%) smears suggesting benign lesions. In conclusion, the specificity of TS is low and this technique should be abandoned as a routine test in patients with nontoxic thyroid nodules. However, a more careful evaluation should be suggested for patients with cold thyroid nodules and a FNA cytology that reads follicular neoplasm.

Several studies suggested that preoperative localization of abnormal parathyroid (PT) glands may be useful in reducing operative time facilitating parathyroidectomy, especially in patients with ectopic PT glands. At present, noninvasive techniques used to evaluate patients with primary HPT include (1) 99mTc-sestamibi scintigraphy, (2) high-resolution neck ultrasonography, (3) CT scanning, and (4) magnetic resonance imaging (MRI). The sensitivity and positive predictive value of each technique range from 70% to 90%, and a combination of two of more tests may significantly improve the results. In the minimally-invasive era both radioguided and video-assisted parathyroidectomy require an accurate preoperative localization of the abnormal PT glands, and PT imaging should be obtained before surgery in all patients with primary hyperparathyroidism, with the aim of reducing operative time and hospital stay.

A series of 112 consecutive patients with primary hyperparathyroidism who underwent both high-resolution neck ultrasonography (US) and 99mTc-sestamibi/99mTc-pertechnetate subtraction scintigraphy (SS) prior to successful parathyroidectomy was reviewed. There were 29 (25.9%) men and 83 (74.1%) women, with a median age of 58 years (range 13-78 years). Patients were divided into two groups, according to the preoperative US findings: group A (87 patients, 77.7%) without thyroid diseases, and group B (25 patients, 22.3%) with either multinodular goitre or a solitary nontoxic thyroid nodule. In group B patients partial or total thyroidectomy was also performed, according to the intraoperative findings and frozen-section examination results. Final histopathology showed 99 (88.4%) solitary parathyroid (PT) adenomas and 3 (2.7%) PT carcinomas, while 10 (8.9%) patients had a multiglandular disease. The sensitivity and positive predictive value (PPV) were (group A vs group B) 79.8% vs 70.8% (P=0.25) and 95.7% vs 94.4% (P=0.58) for US, and 83.3% vs 87.0% (P=0.47) and 95.9% vs 90.9% (P=0.32) for SS respectively. Better but similar (P=not significant) results were obtained in patients with solitary PT tumours: 81.5% vs 77.8% (US) and 85.0 vs 94.1% (SS) sensitivity; 97.1% vs 93.3% (US) and 95.8% vs 88.9% (SS) PPV. Overall, the combination of US and SS was 92.9% sensitive (group A=93.1%, group B=92.0%; P=0.55), and the PPV reached 100% in each group. In conclusion, in patients with primary hyperparathyroidism the results of both US and SS are independent of coexistent thyroid disease, especially in patients with solitary PT tumours.

The aim of this study was to evaluate the sensitivity and positive predictive value (PPV) of dexamethasone-suppression norcholesterol scintigraphy (NCS), computed tomography (CT) scanning and magnetic resonance imaging (MRI) in patients with primary aldosteronism (PA) who had undergone unilateral adrenalectomy. A series of 49 patients with confirmed PA was reviewed. There were 18 (36.7%) men and 31 (63.3%) women, with a median age of 47 years (range, 23-66 years). NCS was performed in all patients, and 46 (93.9%) and 31 (63.2%) underwent CT scan and MRI, respectively. Final pathology showed an aldosterone-producing adenoma in 45 (91.8%) patients, unilateral nodular cortical hyperplasia (NCH) in two (4.1%) and unilateral microscopic cortical hyperplasia (MCH) in two (4.1%). No aldosterone-producing carcinoma or bilateral adenomas were found. The greatest diameter of the removed adrenal tumour was in the range 8-40 mm (median, 14 mm). The PPV of adrenal imaging was 97.6% for NCS, 85.0% for CT scan (P=0.04) and 83.3% for MRI (P=0.03), and the sensitivity was 85.4%, 85.0% and 74.1%, respectively (P=NS). The age and the main biochemical parameters did not differ significantly (P=NS) between patients with true positive and false negative results of the imaging procedures. NCS accurately depicted all patients with NCH and MCH, whilst CT scan and MRI failed to diagnose such unilateral adrenal gland hyperfunction in two and three patients, respectively. The overall sensitivity of combined NCS and CT scan was 100%. In conclusion, in patients with PA, both NCS and CT scan are necessary to confirm the exclusive unilateral adrenal hyperfunction and, subsequently, to establish the appropriate treatment.

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