Langerhans cell histiocytosis in children - a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment.

Langerhans cell histiocytosis is a rare clonal disease characterized by the proliferation of CD1a-positive immature dendritic cells. The purpose of this article was to present an updated review of recent advances in the pathogenesis, clinical features, imaging and treatment of this disease. The discovery of oncogenic BRAF mutations and the presence of proinflammatory cytokines and chemokines confirmed the unusual characteristics of this disease. Currently, children with organ involvement who do not have a good response to chemotherapy and have neurodegeneration or diabetes insipidus are the most problematic patients. Further research is needed to improve the results of treatment.

Sickle cell disease and transcranial Doppler imaging: inter-hemispheric differences in blood flow Doppler parameters.

BACKGROUND AND PURPOSE: to establish reference values of interhemispheric differences and ratios of blood flow Doppler parameters in the terminal internal carotid artery, middle cerebral artery, and anterior cerebral artery in children with sickle cell anemia. METHODS: fifty-seven out of 74 recruited children (mean age, 7.8 ± 3.4 years; range limits, 3-14 years), who were free of neurological deficits and intracranial narrowing detectable by MRA and had flow velocities <170 cm/s by conventional transcranial Doppler ultrasound, underwent transcranial color-coded duplex ultrasonography. Reference limits of flow parameters corrected and uncorrected for the angle of insonation were estimated using tolerance intervals, with P=0.90 for all possible data values from 95% of a population. RESULTS: reference limits for left-to-right differences in cm/s in the mean angle-corrected and uncorrected flow velocities were -56 to 53 and -72 to 75 for middle cerebral artery, -49 to 57 and -81 to 91 for anterior cerebral artery, and -55 to 64 and -73 to 78 for terminal internal carotid artery, respectively. Respective reference limits for left-to-right velocity ratios were 0.31 to 1.84 and 0.38 to 1.75 for middle cerebral artery, 0.48 to 2.99 and 0.46 to 2.89 for anterior cerebral artery, and 0.61 to 2.56 and 0.56 to 2.23 for terminal internal carotid artery. CONCLUSIONS: the study provides reference limits of interhemispheric differences and ratios of blood flow Doppler parameters that may be helpful in identification of intracranial arterial narrowing in children with sickle cell disease undergoing ultrasound screening for stroke prevention.

Glomerular filtration rate and prevalence of chronic kidney disease in Wilms' tumour survivors.

Glomerular filtration rate (GFR) was evaluated in 32 Wilms' tumour survivors (WTs) in a cross-sectional study using 99 Tc-diethylene triamine pentaacetic acid (99 Tc-DTPA) clearance, the Schwartz formula, the new Schwartz equation for chronic kidney disease (CKD), cystatin C serum concentration and the Filler formula. Kidney damage was established by beta-2-microglobulin (B-2-M) and albumin urine excretion, urine sediment and ultrasound examination. Blood pressure was measured. No differences were found between the mean GFR in 99 Tc-DTPA and the new Schwartz equation for CKD (91.8 ± 11.3 vs. 94.3 ± 10.2 ml/min/1.73 m(2) [p = 0.55] respectively). No differences were observed between estimated glomerular filtration rate (eGFR) using the Schwartz formula and the Filler formula either (122.3 ± 19.9 vs. 129.8 ± 23.9 ml/min/1.73 m(2) [p = 0.28] respectively). Increased urine albumin and B-2-M excretion, which are signs of kidney damage, were found in 7 (22%) and 3 (9.4%) WTs respectively. Ultrasound signs of kidney damage were found in 14 patients (43%). Five patients (15.6%) had more than one sign of kidney damage. Eighteen individuals (56.25%) had CKD stage I (10 with signs of kidney damage; 8 without). Fourteen individuals (43.75%) had CKD stage II (6 with signs of kidney damage; 8 without). The new Schwartz equation for CKD better estimated GFR in comparison to the Schwartz formula and the Filler formula. Furthermore, the WT survivors had signs of kidney damage despite the fact that GFR was not decreased below 90 ml/min/1.73 m(2) with 99 Tc- DTPA.

Cerebral blood flow changes in patients with dementia with Lewy bodies (DLB). A study of 6 cases.

BACKGROUND: The aim of this study was to show the application of cerebral blood flow SPECT (rCBF SPECT) study in dementia with Lewy bodies (DLB). MATERIAL AND METHODS: (99m)Tc-ECD regional cerebral blood flow SPECT scanning was performed using a triple head, high resolution gamma camera on a group of six patients who ful- -filled criteria for clinical diagnosis of DLB. All patients were examined neurologically by a neurologist specialized in movement disorders. Detailed neuropsychological examination was performed on each patient with a psychological tests battery by an experienced neuropsychologist. Qualitative and quantitative analysis was performed utilizing an asymmetry index for unilateral perfusion deficits and a comparison to cerebellar perfusion to assess regional cerebral perfusion. A control group of 20 patients was studied to assess normal values, utilizing an asymmetry index for unilateral perfusion deficits, and a comparison to cerebellar perfusion was performed to assess regional cerebral perfusion. RESULTS: In four cases rCBF SPECT images showed patterns of bilateral hypoperfusion of the temporal, parietal and occipital lobes. In two other cases parietal deficits were observed. CONCLUSIONS: Functional neuroimaging with the use of CBF SPECT may contribute to clinical diagnosis of DLB.

Evaluation of surgical gamma probes for sentinel node localization in cervical and vulvar cancer.

BACKGROUND: Sentinel node (SN) scintigraphy for cervical and vulvar cancer guides the gynaecological oncologist in finding the metastatic lymph nodes during lymphadenectomy. The role of the surgical gamma probe in the sentinel node concept in gynaecological oncology is to localise (SN) both intra-operatively and transcutaneously. Intra-operative hand-held collimated gamma probes are increasingly used for detection of the sentinel lymph node. MATERIAL AND METHODS: A comparative evaluation of hand held gamma probes: Neoprobe 1,500, Europrobe, Gamma Finder, Gamma Ray Prospector GRP1 and GPR2 was performed using different detection methods. Laboratory tests were performed in which sensitivity, spatial resolution and angular sensitivity were evaluated. RESULTS: The results for each gamma probe were summarised and discussed. CONCLUSION: Awareness of a gamma probeís capabilities and limitations should be considered in the appropriate selection of a device.

Evaluation of sentinel node detection in vulvar cancer.

BACKGROUND: In vulvar cancer, in a large portion of patients with early stages of the disease, the inguinal lymphadenectomy not only does not influence the overall survival and recurrence rate but may increase the incidence of complications. Sentinel lymph node (SN) detection is a promising technique for detecting groin lymph nodes, which may in future lead to less extensive use of surgical treatment. The aim of the study was to evaluate the feasibility of the sentinel node detection technique in patients with vulvar cancer. MATERIAL AND METHODS: Between the years 2003 and 2005, we performed intraoperative lymphatic mapping on 10 patients with planoepithelial vulvar cancer. In eight cases, vulvar lesion was localized centrally, around the clitoris. The extent of the surgery included radical vulvectomy with bilateral inguinal lymphadenectomy in nine cases and unilateral inguinal lymphadenectomy in one case. For the lymphatic mapping, we employed two detection methods: (99m)Tc-labelled radiocolloid (activity 35-70 MBq) and blue dye (3-5 ml). Both techniques were used in six cases (60%), blue dye only in three cases and radiocolloid only in one case. RESULTS: In each patient, we detected at least one sentinel lymph node. Sentinel nodes were localized in 14 of 19 operated groins (73.7%); a total of 25 SNs in all. The mean number of SNs for one groin was 1.78. Nodal metastases were found in four cases. In three cases, metastases were detected only in the SN. In one patient, two SNs with metastases were found in one groin and in the contralateral groin (without any SN) there was one unchanged node, which transpired to be metastatic. This can be explained by a complete overgrowth of neoplasm in the lymph node resulting in lymph flow stasis and disabling tracer uptake. In five cases, an SN was found only in one groin ó the first case is described above, in the second case the vulvar tumor was localized laterally, opposite to the groin without any SN. In the remaining three cases, we have used only one method of SN detection. CONCLUSIONS: Lymphatic mapping in vulvar cancer based on the combined detection technique is a highly accurate method after adequate training of the surgeons.

[Usefulness of FDG-PET for the diagnosis of brain tumors]. / Przydatnosc FDG-PET w diagnostyce guzów mózgu.

Although magnetic resonance imaging (MRI) and X-ray computed tomography (CT) are the two imaging modalities used conventionally for the morphological OUN imaging, there are many cases for positron emission tomography (PET) diagnostic. Basal radiopharmaceutic used in PET is a 18F-2-Fluoro-2-deoxy-D-glucose (18FDG). Use of PET imaging provides in vivo biochemical and physiological data and therefore can provide unique functional information about tumors compared to histological or anatomic neuroradiological methods. PET can provide information regarding tumor grading, delineation of tumors margins, differentiation of recurrent brain tumors from necrosis (golden standard), guidance for stereotactic biopsy, response to therapy and prognosis. New value in brain tumor imaging is combining two essential and complementary imaging modalities PET and multisection CT to create one integrated unit that allows both functional and anatomic imaging in a single study. This allows PET-CT for more accurate tumor localization and variability assessment changes impressions and increases diagnostic confidence. The first PET-CT centre in Poland is localised in Cancer Centre in Bydgoszcz. Scanning is performed on a dedicated Biograph (PET-CT) LSO scanner (SIMENS, Germany), and cyclotron RDS-111 (CTI, USA), FDG production laboratory (NUCLEAR INTERFACE, Germany, COMECER, Italy) and FDG quality control (RAYTES, Germany) are radiopharmaceutical production unit.

Decortication in chronic pleural empyema - effect on lung function.

OBJECTIVE: The aim of the present study is to evaluate the lung function before and after the lung decortication in patients with chronic pleural empyema (CPE). METHODS: Twenty-six patients with diagnosis of CPE were evaluated in a prospective manner by lung perfusion scintigraphy, blood gas analysis and spirometry before and 35 weeks (+/-17) after the lung decortication. RESULTS: Preoperative scintigraphy showed reduction of lung perfusion on the affected side to 24.5% (+/-12.6%) in 11 right side empyemas (predicted value 55%) and to 18% (+/-8%) in 15 left side empyemas (predicted value 45%). The postoperative measurements showed improvement in perfusion to 45.2% (+/-7.7%) in patients with right side empyema and 34.1% (+/-8.5%) with the left side affection. The preoperative vital capacity (VC) was reduced to 62.3% (+/-13.8%) of the predicted value and forced expiratory volume in 1s (FEV1) to 50% (+/-15.5%) of the predicted value. Postoperatively, slight improvement was achieved to 79.8 % (+/-12.9%) for VC and 69.2% (+/-12.7%) for FEV1. Blood gas analysis showed decreased values in majority of the patients before operation and significant improvement in postoperative evaluation. CONCLUSIONS: Perfusion and spirometry improves significantly in patients with CPE after the lung decortication but function of the affected lung remains impaired. There was no influence of the age, gender, side of the disease, bacteriology or duration of the empyema before operation on lung function.

Myocardial perfusion in women with systemic lupus erythomatosus and no symptoms of coronary artery disease.

BACKGROUND: The aim of the study was to assess myocardial perfusion in women with systemic lupus erythomatosus (SLE) and no symptoms of coronary artery disease (CAD). MATERIAL AND METHODS: Twenty two women with SLE of mean age 40.5 +/- 7.2 were enrolled in the study. The average duration time of the disease was from 2 to 19 years, mean 8 +/- 4.6 years. The inclusion criterion was the absence of stenocardial symptoms. The myocardial perfusion was studied by using Single Photon Emission Computerized Tomography (SPECT) utilising (99m)Tc-MIBI and a triple-head gamma-camera. We also analyzed risk factors of heart ischemic disease in our group. RESULTS: Myocardial perfusion stress scanning showed abnormal perfusion in 12 patients, 54.5% of the whole group, mostly in the anterior wall. At rest hypoperfusion abnormalities were found in 7 individuals. In patients with positive myocardial perfusion, out scanning, risk factors of CAD were more pronounced than in a sub-group with a negative result of myocardial perfusion scanning. CONCLUSIONS: In young women with SLE and no symptoms of coronary artery disease, myocardial perfusion defects may be detected by means of myocardial perfusion scintigraphy. Exercise and resting electrocardiography tests could be not sufficient for CAD diagnosis in women with SLE. The presence of coronary artery disease risk factors in women with SLE could be an indication to perform myocardial perfusion SPECT scanning.

Application of rhenium-188 HEDP in bone metastases therapy.

Radionuclide bone metastases therapy is a major achievement of nuclear medicine. Development of less radiotoxic and more effective radiopharmaceuticals is therefore a challenge for radiopharmacists and industry. This paper reviews the application of rhenium-188 HEDP as a reactor- or generator-produced nuclide for bone metastases therapy.

Lymphoscintigraphy in radical vaginal trachelectomy and pelvic lymphadenectomy.

BACKGROUND: Radical hysterectomy has traditionally been the treatment of choice for early invasive cervical carcinoma. Validation of the sentinel lymph node concept in gynaecology has led to less radical surgery. If preservation of fertility is an issue, then radical trachelectomy is an accepted approach. CASE REPORT: A 29-year-old female presented with stage IB1 cervical cancer was treated by radical vaginal trachelectomy and pelvic lymphadenectomy as definitive treatment. Two obturator nodes visualised on lymphoscintigraphy and one parametrial not visualized on lymphoscintigraphy were "hot" and blue during surgery, and identified as sentinel nodes. All 18 nodes were non-metastatic. CONCLUSION: Preoperative lymphoscintigraphy is a useful tool in the preoperative localisation of sentinel lymph nodes, and should be undertaken in cases of early invasive cervical carcinoma where a radical trachelectomy is performed to preserve fertility.

The role of sentinel node detection techniques in vulvar and cervical cancer.

The sentinel node is the first lymph node that receives the lymph drainage from the primary tumour. The pathological status of the sentinel node should reflect the histopathology of the entire regional lymph drainage area--both vulvar and cervical cancer spread through the lymphatic system. In gynaecological oncology recent studies have confirmed the utility of the sentinel node concept in vulvar and cervical cancer. Three techniques for sentinel node localisation are available. The preoperative lymphoscintigraphy and intraoperative hand-held gamma probe detection require the administration of the technetium-99m-labelled colloid around the tumour. The other method is based on the injection of the patent blue dye--during the surgery of the sentinel node because of the dye uptake becomes visible. Following detection, the sentinel lymph node can be removed separately and assessed with ultrastaging and immunohistochemical staining. In the early stages of vulvar and cervical cancer the lymph nodes metastases rate is relatively low--in most cases lymphadenectomy is not necessary. The determination of the regional lymph nodes' pathological status may limit the extent of the surgical treatment. The sentinel node detection rate is relatively high and depends on the applied technique. This technique may play an important role in the treatment of vulvar and cervical cancer. This paper describes the details of sentinel node identification and reviews the literature.

The influence of depth of marker administration on sentinel node detection in cervical cancer.

BACKGROUND: Regional lymph node surgical management is an integral part of cervical cancer therapy. In gynaecological oncology, recent studies have confirmed the utility of the sentinel node concept in vulvar and cervical cancer. The method of the marker's administration is considered to play an important role in sentinel node detection. MATERIAL AND METHODS: 60 patients with cervical cancer (stage IB-IIA) underwent SLN detection during radical abdominal hysterectomy. The patients were randomly divided into two groups: the first group of 30 patients with 0.5-1cm deep marker injection, the second with sub-epithelial marker injection. Gamma-camera scanning, as well as hand-held probe detection was applied. RESULTS: All hot nodes visualised on lymphoscintigraphy were "hot" when using the hand-held gamma probe. Deep marker injection revealed a sentinel node in 27 patients (90%) on both sides, in 3 patients (10%) only on one side. Only 40 (67%) sentinel nodes were blue-stained. Sub-epithelial marker administration revealed a sentinel node on both sides in all 30 patients (100%). In 28 patients (93.3%) the sentinel nodes were radioactive and blue-stained, in one case not-blue stained on either side, in one case blue stained only on one side. CONCLUSIONS: The sentinel node detection rate in cervical cancer is relatively high and depends on the applied technique. The superficial administration of radiocolloid and the blue dye into the cervix provides a higher sentinel node detection rate than deep administration in cervical cancer patients.

Lung perfusion scintigraphy in the diagnosis of peripheral pulmonary stenosis in patients after repair of Fallot tetralogy.

BACKGROUND: The frequency of peripheral pulmonary artery stenosis in patients after surgical repair of tetralogy of Fallot (TOF) ranges from 20 to 40%. This can be either primary or secondary to the surgical intervention. The influence of resulting lung perfusion alterations on the life quality of patients is difficult to predict. The aim of this study was to compare the utility of the diagnostic procedures in this group of patients, with particular focus on lung perfusion scintigraphy. MATERIAL AND METHODS: This study comprised 104 patients who underwent repair of TOF at ages from 5 months to 25 years. The patients have been followed up for from 4.2 to 25 years. On the basis of chest x-ray peripheral pulmonary artery stenosis was suspected in 11 patients, in 12 on the basis of echocardiography examination. RESULTS: Lung perfusion scintigraphy has been performed on 87 patients. The disturbances in lung perfusion (mostly in the left lung) were show by means of lung perfusion scintigraphy in 43 (49%) of patients. In 27 of them heart catheterisation has been performed. Angiography revealed stenosis of the lung artery branch in 15/43 (34.9%) patients with abnormal perfusion lung scan and in 4/44 (9%) in patients with normal perfusion lung scan. Intervention procedures were carried out on 10 patients. CONCLUSIONS: Lung perfusion scintigraphy may prove a valuable, non-invasive screening tool in the assessment of patients after TOF repair, although both false-negative and false-positive results may happen. Therefore, it should play an auxiliary role together with other diagnostic modalities.

[Evaluation of positron emission tomography by using F-18-fluorodeoxyglucose in diagnosis of recurrent colorectal cancer]. / Ocena pozytonowej tomografii emisyjnej z uzyciem F-18-fluorodeoksyglukozy w rozpoznawaniu wznowy raka jelita grubego.

UNLABELLED: Accurate detection of recurrent colorectal carcinoma remains a clinical challenge. 18-F-fluorodeoxyglucose-positron emission tomography is new imaging technique that allows direct evaluation of cellular metabolism. This method is mainly used in oncology, especially in fusion with computer tomography (FDG-PET/CT, PET/CT Fusion). The aim of the study was to evaluate the usefulness of FDG-PET/CT in diagnosis of recurrent colorectal cancer. MATERIAL AND METHODS: The results of FDG-PET/CT scan performed in 120 patients who had suspected recurrence disease were analyzed retrospectively. The definite diagnosis was established on the basis of histopathological examination or clinical follow-up. FDG-PET/CT results were compared with 76 computer tomography (CT), 30 magnetic resonance imaging (MRI), 81 ultrasonography (US), 75 chest roentgenogram (X-ray) and 91 carcinoembryonic antigen (CEA) test. RESULTS: Recurrence colorectal cancer was demonstrated on FDG-PET-CT in 69 patients (57.5%). In 24 patients local recurrence, 33 liver metastases, 23 pulmonary metastases and in 36 other metastases were found. A total of 116 suspicious lesions were identified. The final diagnosis of recurrence was obtained in 56 patients (46.6%). Sensitivity, specificity, and accuracy for malignant findings were: for FDG-PET/CT 98, 94 and 97%; for CEA test 68, 82 and 72%; for CT scan 85, 91 and 89%; for RMI scan 96, 86 and 93%; for ultrasound test 71, 96 and 82%; for X-ray 87, 96 and 92%, respectively. FDG-PET-CT influenced surgical decisions in 23.6% of cases. CONCLUSIONS: FDG-PET/CT is a very useful diagnostic method in case of patients with suspected recurrence of colorectal carcinoma. Its usefulness is higher than the serum level of CEA and other conventional imaging modalities (CT MRI, US, X-ray).

The usefulness of sentinel lymph node detection in vulvar cancer - a short communication.

BACKGROUND: The sentinel lymph node (SLN) biopsy is a solution for decreasing the extent of surgery with a significant reduction of the incidence of complications without influencing treatment results. MATERIAL AND METHODS: We performed the sentinel lymph node procedure in 24 women with vulvar cancer. In 14 cases, only the blue dye technique was applied, and in 10 cases (99m)Tc-labelled nanocolloid with blue dye was administered simultaneously. The extent of the surgery included radical vulvectomy in 23 patients and a wide local excision in 1 patient. In 15 patients unilateral inguinofemoral lymphadenectomy was performed and in 9 cases bilateral lymphadenectomy. The total number of operated groins was 39. RESULTS: SLNs were detected in 34/39 of operated groins (87.2%). In 4 cases (16.6%) tumour metastases to the lymph nodes were found. In total, 10 metastatic lymph nodes were detected in 9 sentinel-nodes and in 1 non-sentinel node. In three patients the nodal metastases were found only in the sentinel nodes. In one patient the metastases were found in the contralateral groin in two SLNs. There were no false negative sentinel lymph nodes. With the sole use of blue dye, SLNs were found in 79.5% of groins. The additional administration of the radiocolloid improved SLN detection to 88.9% of groins. CONCLUSIONS: The parallel use of the (99m)Tc labelled radiocolloid and blue dye enables high sentinel node detection rates by adequately trained surgeons.