Indolent B-cell non-Hodgkin lymphomas in children: high association with inborn errors of immunity.

Indolent lymphomas are rare in children and mostly consist of pediatric type follicular (PTFL) and pediatric marginal zone lymphomas (PMZL) and extranodal marginal zone lymphoma (ENMZL). Twenty children with indolent lymphoma (10 PTFL, 6 PMZL, 3 ENMZL, 1 mixed type) among 307 Non-Hodgkin Lymphoma (NHL) were retrospectively evaluated. The mean age of the entire group was 10.4 ± 4.4 and was significantly lower in PTFL than in PMZL. Seven patients (35%) had an associated inborn error of immunity (IEI) which was higher than that seen in aggressive lymphomas (5.9%) (p < 0.0001). Seventeen patients (85%) had stage I/II disease. Two patients received no treatment after surgery. Eleven patients were treated only with 3-6 courses of rituximab. Four patients received 3-6 courses of R-CHOP protocol. The prognosis was excellent Five years overall and event-free survivals were 100% and 85%, respectively.

Albuminuria is associated with 24-hour and night-time diastolic blood pressure in urinary tract infection with renal scarring.

BACKGROUND: We aimed to detect complications and associated risk factors in patients with renal scarring (RS) secondary to recurrent urinary tract infections (UTI). METHODS: Fifty patients with RS were compared with 25 patients without RS by means of, serum creatinine, 24- hour urinary creatinine clearance, and 24-hour urinary albumin levels. Office blood pressure (BP) examination and ambulatory BP monitoring (ABPM) were also performed. RESULTS: Vesicoureteral reflux was detected in 50 patients. Glomerular filtration rate (GFR) < 90 ml/min/1.73 m2 was observed in 5 patients with RS but in no patient without RS. Albuminuria was significantly higher in patients with bilateral RS and severe RS. Patients with albuminuria had a significantly lower GFR than those without. All patients with ambulatory hypertension (HT) were in the RS group, and 60% of those had isolated nocturnal HT. Compared to those without RS, patients with RS had significantly higher SDS values for all BP readings, 24-hour and nighttime systolic and diastolic BP loads with significantly lower systolic dipping. GFR was negatively correlated with diastolic BP SDS and diastolic BP load in patients with RS. Daytime diastolic BP load was significantly higher in those with severe RS than in those with mild RS. CONCLUSIONS: Isolated nocturnal HT could be an early sign of complications in RS of UTI. Albuminuria is related to increased BP and impaired renal function. Therefore, ABPM and assessing albuminuria should be a routine part of the follow-up. Diastolic BP elevations could be associated with worse outcomes in these patients.

Role of FDG PET/CT in the Evaluation of Therapy Response in Systemic Juvenile Xanthogranuloma.

ABSTRACT: Juvenile xanthogranuloma, a rare type of non-Langerhans cell histiocytosis, is mostly seen in childhood and adolescence and generally manifests as widespread skin lesions. It rarely shows systemic involvement. Although the cutaneous form is often self-limited, systemic form is quite aggressive. Here we report the findings of FDG PET/CT scans during the course of cladribine therapy in a 6-year-old girl with systemic juvenile xanthogranuloma.

Utility of 'dual phase' cone beam computed tomography during radioembolisation in patients with hepatocellular carcinoma: what is really changing in flow dynamics before and after 90Y delivery?

PURPOSE: The aims of the study were: 1) to compare two phases of dual-phase cone beam computed tomography (DP-CBCT) achieved before and after Yttrium-90 (90Y) administration and to evaluate additional benefits during radioembolisation (RE) procedures; and 2) to compare DP-CBCT with pre-procedure contrast enhanced cross-sectional images in terms of tumour detection. MATERIAL AND METHODS: Twenty-three hepatocellular carcinoma patients undergoing RE treatment were scanned with DP-CBCT consisting of early arterial (EA) and late arterial (LA) phases before and after 90Y administration. The CT-like datasets were compared according to embolisation effect, enhancement patterns, lesion detectability, image quality, and artifacts by two interventional radiologists blinded to each other. The compatibility of the two radiologists was evaluated with kappa statistical analysis, and the difference between EA and LA phases was evaluated with marginal homogeneity test. Also, DP-CBCT images were compared with preprocedural cross-sectional images (CT/MRI). RESULTS: For 23 patients 92 data were acquired. Thirteen patients showed a decrease on post-embolisation images both visually and on Hounsfield unit (HU) measurements. No statistical difference was found for tumour detection between EA and LA phases (p = 1.0). Tumour enhancement was visually superior at LA phases whereas EA phases were better for arterial mapping for selective catheterisation. DP-CBCT images were not inferior to preprocedural cross-sectional imaging findings. CONCLUSIONS: DP-CBCT is a promising tool for predicting tumour response to therapy and is not inferior to preprocedural cross-sectional imaging in terms of tumour detection. It allows better assessment during RE procedures because early phases provide good mapping for superselective catheterisation whereas late phases are better for visualisation of tumour enhancement.

Outcomes of Radioembolization in Patients with Chemorefractory Colorectal Cancer Liver Metastasis: a Single-Center Experience.

PURPOSE: We aimed to evaluate the efficacy and outcomes of radioembolization with Yttrium-90 (Y-90) microspheres in patients with unresectable and chemorefractory colorectal cancer liver metastasis (CRCLM). METHODS: This single-center study included 43 patients (34 male, 9 female) who underwent radioembolization with Y-90 for unresectable, chemorefractory CRCLM between September 2008 and July 2014. Overall survival (OS), liver progression-free survival (LPFS), overall response rate (ORR), local disease control rate (LDCR), and relations of these parameters with patient disease characteristics were evaluated. OS and LPFS rates were compared according to microspheres. Survival rates were calculated with Kaplan-Meier method, and potential prognostic variables were evaluated on univariate analyses. RESULTS: Post-procedural median OS was 12.8 months. LPFS was 5.6 months. ORR was 33%, LDCR was 67% on 3rd month follow-up. Low tumor burden (< 25%) was associated with higher median OS after radioembolization (< 25 vs > 25-50% p < 0.0001 and < 25 vs > 50% p = 0.005). Patients with left colon tumors exhibited significantly longer median OS after metastasis than right colon tumors (p = 0.046). Extrahepatic disease and synchronicity showed poorer survival parameters; however, the difference was not significant (p = 0.1 and p = 0.3, respectively). In subgroup analyses, the distribution of patient number and characteristics showed heterogeneity as number of patients with low tumor burden was higher in resin Y-90 group. Resin Y-90 group exhibited significantly higher median OS and LPFS compared to glass Y-90 group (16.5 vs. 7 months, p = 0.001; 6.73 vs. 3.38 months, p = 0.023, respectively). CONCLUSION: Radioembolization is a safe local-regional treatment option in chemorefractory, inoperable CRCLM. Radioembolization at earlier stages may lead to more favorable results especially with lower tumor burden patients.

Intra-Arterial Radionuclide Therapies for Liver Tumors.

Intra-arterial radionuclide therapies serve essentially as internal radiation treatment options for both primary and metastatic liver tumors, which imply delivering implantable radioactive microspheres into branches of hepatic arteries that feed liver tumors to provide a high dose of targeted radiation to tumor tissue, while sparing the healthy liver tissue from hazardous effects of radiation. The principle of this therapeutic option depends on the unique preferential arterial supply of malignant liver tumors in contrast with mostly portal venous supply of normal hepatocytes as well as excess amount of arterial neovascularization in the tumor bed. Therefore, intra-arterial radionuclide therapy can provide very high radiation exposure to tumor tissue, which is impossible to reach with external radiation therapy due to serious side effects and moreover, radiation can be targeted to tumor tissue selectively with less side effects. Yttrium-90 (Y-90), a high-energetic beta emitter is the most preferred radionuclide, which is used to label microspheres. Two types of Y-90 microspheres are commercially available that are made of resin and glass. Many studies in the literature have demonstrated that Y-90 microsphere therapy is an efficient and safe locoregional therapeutic option for unresectable primary and metastatic liver tumors such as hepatocellular carcinoma and liver metastases from colorectal cancer and breast cancer as well as neuroendocrine tumors. Furthermore, limited number of studies has reported its use in some relatively uncommon metastatic liver tumors from melanoma, pancreatic, renal, and lung cancer. Besides Y-90 microspheres, Iodine-131 lipiodol, Rhenium-188 lipiodol, Rhenium-188 microspheres, Holmium-166 chitosan, and Holmium-166 microspheres have been introduced as alternative radiopharmaceuticals for intra-arterial therapy for liver tumors.

SPECT in periodic lateralized epileptiform discharges (PLEDs): a case report on PLEDs.

Periodic lateralized epileptiform discharges (PLEDs), which are known as unusual electroencephalogram (EEG) patterns, are described in a patient who had stroke and seizures. This patient underwent Tc-99m HMPAO (hexamethyl propylene amine oxime) brain single photon emission computed tomography (SPECT) imaging both during PLEDs on EEG and after the cessation of PLEDs. The initial SPECT study revealed increased CBF in the left frontal and parietal cortex extending through the left temporal region and in the left basal ganglium. After the PLEDs disappeared, the second SPECT study showed decreased perfusion on the left frontal and parietal region in the brain. Brain SPECT findings supported the contention that PLEDs may be an ictal phenomenon. Here we also present a review on PLEDs and contributions of brain SPECT as a functional imaging modality to investigate the underlying mechanism of this interesting EEG pattern.

In vivo characterisation of parathyroid lesions by use of gamma probe: comparison with ex vivo count method and frozen section results.

OBJECTIVE: In this study we hypothesized that if timing of gamma probe-guided parathyroidectomy were individualized according to an optimal-time-to-surgery technique, in vivo characterization of parathyroid lesions would be possible. We compared our findings with an ex vivo counting method ("20% rule") and frozen section results. STUDY DESIGN AND SETTINGS: Thirty-five patients who were referred for surgical treatment of hyperparathyroidism were studied. Maximum parathyroid to thyroid sestamibi uptake ratio (UR(max)) was measured by use of preoperative dynamic scintigraphy. The interval between sestamibi injection and UR(max) was defined as the optimal time to surgery. On the day of surgery, the patients received the same dose of sestamibi and were taken to the operating room at UR(max) as determined by preoperative scintigraphy. Intraoperative in vivo gamma probe counts from parathyroid lesions were compared with in vivo contralateral background thyroid counts (in vivo/Bkg) and to ex vivo parathyroid counts relative to postexcision background of the adjacent normal tissue (ex vivo/Bkg). RESULTS: A total of 70 excised lesions were evaluated. In vivo/Bkg counts obtained from parathyroid adenoma were significantly different from parathyroid hyperplasia (z = -3.093, P = 0.002) and other lesions (z = -3.958, P = 0.0001). By receiver operating characteristic curve (ROC) analysis, we found the cutoff value for the in vivo/Bkg counts ratio to be 103% to differentiate parathyroid adenoma from hyperplasia with a sensitivity, specificity, and accuracy of 82.5, 65, and 74.4%, respectively. On the other hand, sensitivity, specificity, and accuracy of the ex vivo/Bkg method to differentiate parathyroid adenoma from hyperplasia with a cutoff value of 34.7 was found to be 70.8%, 60%, and 65.9%, respectively. The difference between the accuracy of these 2 tests was not significant statistically (P = 0.137). Sensitivity of frozen section to differentiate parathyroid adenoma and hyperplasia was 76.2% and 33.3%, respectively. CONCLUSIONS: Patient-specific optimal protocol for timing of sestamibi injection together with in vivo/Bkg method is a useful alternative method in guiding the surgeon to differentiate parathyroid adenoma from parathyroid hyperplasia and other tissues and may help surgeons' decisions during the operation. Combined use of in vivo/Bkg and ex vivo/Bkg methods may give more accurate results than frozen section.

Simultaneous surface registration of ictal and interictal SPECT and magnetic resonance images for epilepsy studies.

BACKGROUND: Subtraction of ictal and interictal single photon emission computed tomography (SPECT) images is known to be successful in localizing the seizure focus in the pre-surgical evaluation of patients with partial epilepsy. A computer-aided methods for producing subtraction ictal SPECT co-registered to the magnetic resonance image (MRI) (the SISCOM method) is commonly used. The two registrations involved in SISCOM are (1) between the ictal-interictal SPECT images, which was shown to be the more critical, and (2) between the ictal image and MRI. OBJECTIVE: To improve the accuracy of ictal-interictal registration in SISCOM by registering all three images (ictal, interictal SPECT, MRI) simultaneously. METHODS: The registration problem is formulated as the minimization of a cost function between three surfaces. Then, to achieve a global minimum of this cost function, the Powell algorithm with randomly distributed initial configurations is used. This technique is tested by a realistic simulation study, a phantom study and a patient study. RESULTS: The results of the simulation study demonstrate that, in surface-based registration, the triple-registration method results in a smaller ictal-interictal SPECT registration error than the pair-wise registration method (P<0.05) for a range of values of the cost-function parameter. However, the improved registration error is still larger than that obtained by the normalized mutual information method (P<0.001), which is a voxel-based registration algorithm. The phantom and patient studies reveal no observable difference between registration results. CONCLUSIONS: Although the improved accuracy of triple registration is slightly worse than voxel-based registration, it will soon be possible to apply the results of this study in research utilizing the triple-registration principle to improving voxel-based results of ictal-interictal registration.

Role of scintigraphy in congenital thyroid anomalies.

Thyroid scintigraphy using Tc-99m pertechnetate is a frequently performed procedure in routine nuclear medicine practice. The indications for thyroid scintigraphy are investigation of hyperthyroidism, nodularity of the gland, cause of thyroid stimulating hormone elevation and localization of an ectopic thyroid gland. In the pediatric population, the most common request is for the evaluation of neonatal hypothyroidism. This imaging procedure is helpful in the identification of the underlying cause as well as in making a differential diagnosis. Early diagnosis is essential for appropriate therapy planning in this age group, and thyroid scintigraphy provides important diagnostic data. This article is written to review the scintigraphic findings in various congenital thyroid anomalies and to underline its use in the differential diagnosis.

Losartan renography for the detection of renal artery stenosis: comparison with captopril renography and evaluation of dose and timing.

PURPOSE: Radionuclide renography with angiotensin converting enzyme (ACE) inhibition plays an important role in the diagnosis of haemodynamically significant renal artery stenosis. Angiotensin receptor antagonists inhibit the renin angiotensin system at different levels from ACE inhibitors by selectively blocking the binding of angiotensin II to AT1 receptors. The AT1 angiotensin receptor antagonist losartan has recently been used clinically in the treatment of hypertension. However, the available data on the use of losartan with renography for the detection of renovascular hypertension are limited and contradictory. The purpose of this prospective study was to compare the effectiveness of losartan renography and captopril scintigraphy in revealing renal artery stenosis. METHODS: A total of 61 renal units in 32 patients with hypertension were studied in two groups based on the losartan dosage (50 mg in group A and 100 mg in group B). Group A consisted of 17 patients, in whom 19 renal units had angiographically proven renal artery stenosis (>or=50%). In group B, there were 15 patients, in whom 20 renal arteries were stenotic. All of the patients underwent three renographies (baseline, captopril renography and early losartan renography). Early losartan renography was performed at 1 h after oral losartan administration in both groups. In group B, seven patients underwent additional losartan renography (late losartan) performed 3 h after oral losartan administration; these patients composed group B1. RESULTS: The sensitivities of captopril and losartan studies were 63.2% and 42% in group A, 65% and 65% in group B and 55.6% and 66.6% in group B1, respectively. CONCLUSION: From our preliminary results, we conclude that losartan is not superior to captopril renography for the detection of haemodynamically significant renal artery stenosis. However, a high dose (100 mg) of losartan provided higher sensitivity than the lower dose (50 mg). Late losartan scintigraphy provided similar diagnostic efficacy to early losartan renography.

Lung hilar Ga-67 uptake in patients with lymphoma following chemotherapy.

Scintigraphic characteristics of lung hilar Ga-67 uptake (HU) and their relationship with the etiology (benign vs. malignant) of the hilar lesions in lymphoma patients following chemotherapy were retrospectively investigated. A total of 161 lymphoma patients were included in the study. The presence/absence of HU and if present, symmetry/asymmetry and intensity of HU (on the basis of a 3 scale grading system) were visually and semiquantitatively assessed on transaxial sections of thorax Ga-67 SPECT. By drawing ROIs over right and left hilum, asymmetry index (AI%) was also calculated. HU was categorized as benign or malignant depending on the radiological correlation and clinical follow-up. In the malignant group, the majority of patients (85.7%) had grade 2 or grade 3 uptake and all had asymmetric pattern. However, in the benign group, grade 1 uptake was more common (66%) and was mainly symmetric (94.6%) in appearance. AI% in the malignant group (73.7 +/- 36.6) was significantly higher than in the benign group (5.7 +/- 4.9) confirming the marked asymmetry in malignant patients.

Testing different storage conditions for (99m)Tc-MAG3 kit: can hot fractioning reduce the cost per unit dose?

Since its release for routine clinical use, (99m)Tc-mercaptoacetyltriglycine (MAG3) has become an important alternative to (131)I-labeled orthoiodohippuran. The cold kit for MAG3 is expensive, especially in developing countries. Therefore, unique storage conditions should be provided for cost reduction. Cold fractioning is a well-known procedure but has special requirements, such as a nitrogen tank and a laminar flow hood. The aim of this study was to prolong the shelf life of (99m)Tc-labeled MAG3 by a hot fractioning method, which separates the patient doses after (99m)Tc labeling. The radiochemical purity of the (99m)Tc-labeled MAG3 kit was tested under different storage conditions. Hot fractioning of the (99m)Tc-labeled MAG3 kit was found to be a possible alternative to cold fractioning for routine clinical studies.