C1-esterase inhibitor deficiency in pediatric heart transplant recipients: incidence and findings on ultrasound.

BACKGROUND: Acquired angioedema of the bowel caused by a deficiency of C1-esterase inhibitor can lead to severe abdominal pain with sudden onset, mimicking an acute surgical abdomen. In contrast to hereditary angioedema, which usually manifests in childhood, acquired angioedema is broadly recognized to affect people older than 40 years. OBJECTIVE: To determine the incidence of acquired angioedema in a cohort of pediatric heart transplant recipients and assess imaging findings on ultrasonography. MATERIALS AND METHODS: A cohort of 207 children and adolescents who had undergone heart transplantation were assessed at regular follow-up examinations for incidence of acquired angioedema. All patients received ACE inhibitors and immune inhibitors. Control examinations carried out in 3-month intervals included history, assessment of clinical symptoms, physical examination, US of the abdomen and laboratory blood analysis. In addition, if clinical symptoms were newly encountered, children were admitted between regularly scheduled intervals. We analyzed results of abdominal US for pathological findings of the bowel, and we assessed imaging findings in children diagnosed with acquired angioedema. RESULTS: Acquired angioedema was diagnosed in 3/207 patients (2 girls ages 16 and 17 years and 1 boy age 9 months). These children presented with sudden onset of severe abdominal pain. The bowel wall was thickened in the presence of ascites. After a mean of 72 h, abdominal pains resolved. Thickening of bowel loops dissolved and ascites disappeared. CONCLUSION: Single episodes of acquired angioedema were encountered in 1.4% of our series of pediatric heart transplant recipients. Radiologists should be familiar with this disease so they can diagnose it on US imaging.

Diagnostic value of FDG-PET and HMPAO-SPET in patients with mild dementia and mild cognitive impairment: metabolic index and perfusion index.

OBJECTIVE: The diagnostic potential of F-18-2-fluoro-2-deoxy-D-glucose positron emission tomography (PET) and technetium-99m hexamethylpropylene amine oxime single-photon emission tomography (SPET) in early detection and differential diagnosis of early dementia was evaluated including a comparison of metabolic and perfusion indices (PI). METHODS: Twenty-four patients with initial clinical suspicion of beginning dementia were examined, 12 of them with mild cognitive impairment. All patients underwent SPET and PET within 2 weeks. Data were compared with the final clinical diagnosis at follow-up - 9 with Alzheimer's disease (AD), 1 with frontotemporal dementia, 1 with vascular dementia (VD), 7 with mixed type of dementia (MIX) and 6 without any type of dementia. Metabolic indices (MI) and PI were compared with each other. The regional cerebral blood flow difference (rCBFdiff) calculated as local uptake difference between the right and left hemisphere was measured for patients with VD and MIX. RESULTS: PET showed higher sensitivity and specificity in identifying the different types of early dementia (44--91 and 78--89%, respectively) than SPET (11--64 and 79--89%, respectively), especially in detecting AD (sensitivity 44%, specificity 83%) and MIX (sensitivity 71%, specificity 78%). Especially in patients with mild cognitive impairment, PET was the superior imaging modality for predicting dementia. Using PET, dementia could be excluded in all patients who did not develop dementia during the follow-up. In all patients, a weak correlation between PI and MI was observed (rho=0.64, p<0.002). The rCBFdiff in patients with VD and MIX ranged from 7 to 37%. CONCLUSION: In this study on patients with initial suspicion of beginning dementia who underwent both imaging modalities, PET and SPET, PET was the superior imaging method, especially in the detection of early AD or MIX.

Sentinel node biopsy as staging tool in a multimodality treatment approach to cancer of the oral cavity and the oropharynx.

OBJECTIVES: Feasibility of sentinel lymph node (SLN) biopsy in head and neck cancer as a staging tool embedded in a multimodality regimen including neoadjuvant intraarterial chemotherapy. STUDY DESIGN AND SETTING: 39 patients with oral and anterior oropharyngeal cancer classified N0 by [18 F]FDG-PET underwent SLN scintigraphy. Selective SLN biopsy without elective neck dissection (ND) was performed, immediately followed by radical resection of the primary tumor. Histopathology included step-serial sections and immunocytochemistry. RESULTS: Lymphoscintigraphy detected 104 spots. In 15 patients there was bilateral drainage. 114 SLN were excised due to additional intraoperative discrimination. 95% of visualised SLN could be removed. Histology was positive in 3 patients (8%), all underwent ND which yielded another positive node in 2 cases. Median observation time was 30 months. Two patients (5%) had a neck relapse in combination with a second primary. CONCLUSIONS: SLN biopsy as only surgical staging tool seems to be feasible. SIGNIFICANCE: Method promises reduction of elective ND and morbidity in N0 patients.

Pattern of drainage in sentinel lymph nodes after intra-arterial chemotherapy for oral and oropharyngeal cancer.

PURPOSE: Prerequisite for lymphoscintigraphy-guided sentinel lymph node (SLN) biopsy in head and neck cancer patients has been a previously untreated primary tumor to avoid disturbance of the lymphatic flow. We evaluated the feasibility in a multimodality treatment approach to oral and oropharyngeal cancer comprising presurgical intra-arterial chemotherapy of the primary tumor. PATIENTS AND METHODS: In a prospective study of 35 consecutive patients with primary oral and anterior oropharyngeal cancer T1-4N0 treated with superselective intra-arterial chemotherapy with high-dose cisplatin, SLN scintigraphy was performed on the day of surgery, including gamma camera imaging before and hand-held gamma probe identification of the SLN during surgery. SLN biopsy was performed in 50 neck sides, followed by radical resection of the primary in the same session. Elective neck dissection was not carried out. The pattern of lymphatic drainage was recorded and compared with the literature on well-known patterns found in lymphographic studies and metastatic disease in neck dissection specimens of patients without presurgical treatment. RESULTS: Drainage patterns of SLN related to different primary locations mapped the mentioned comparison patterns. SLN drainage patterns gave strong evidence that there are SLN in levels 3 and 4 with direct drainage from the primary. After a median observation time of 24 months, no patients had a neck relapse. CONCLUSIONS: The results suggest that intra-arterial chemotherapy of the primary tumor does not significantly alter lymphatic drainage and that SLN biopsy following intra-arterial chemotherapy is feasible and offers valid results comparable to those obtained from patients without presurgical treatment.

The influence of CA 125 and CEA levels on the results of (18)F-deoxyglucose positron emission tomography in suspected recurrence of epithelial ovarian cancer.

BACKGROUND AND PURPOSE: The follow-up of epithelial ovarian cancer (OCA) consists of clinical investigation, sonography, and tumor markers (TMs), especially CA 125. If tumor recurrence is suspected, other imaging modalities including positron emission tomography (PET) with (18)F-deoxyglucose (FDG) are often used. While there is still no consensus about the method of choice and the timing of its application, this study aims to find a TM threshold at which a PET would be appropriate. MATERIAL AND METHODS: A total of 90 PET studies and the associated CA 125 values (normal value < 35 U/ml) were available in 71 patients during the follow-up after primary therapy for OCA. In 48 studies a CEA value (normal value < 3 ng/ml) was also available. The results of PET imaging were related to the level of TM increase. RESULTS: In 23/90 studies the PET scan was normal. These patients had a median CA 125 of 13.3 U/ml (range 4.2-168 U/ml). In 67/90 studies the PET indicated a potential recurrence of OCA and the median CA 125 was 166.7 U/ml (range 13.3-4,060 U/ml). The TM levels were significantly different (p < 0.001, U-test). With one exception, there were no normal PET scans above CA 125 levels of 30 U/ml; between 20 and 30 U/ml PET was positive in 4/7 studies. CONCLUSION: In suspected recurrence of OCA, if imaging modalities are to be used, an FDG PET may be considered. Since the costs of this investigation are high, it should be restricted to clinical situations where it is likely to be most effective. In this study a PET indication is worthwhile at CA 125 levels of approximately 30 U/ml.

Transplantation of progenitor cells after reperfused acute myocardial infarction: evaluation of perfusion and myocardial viability with FDG-PET and thallium SPECT.

Clinical outcome after myocardial infarction depends on the extent of irreversibly damaged myocardium. Implantation of bone marrow-/circulating blood-derived progenitor cells has been shown to improve contractile cardiac function after myocardial infarction in both experimental and initial clinical studies. In the present study, first observations of the effect of local intracoronary progenitor cell infusion on the regeneration of infarcted cardiac tissue after acute myocardial infarction was evaluated by means of 18F-fluorodeoxyglucose positron emission tomography (PET) and 201Tl single-photon emission computed tomography (SPECT). Twenty-six patients underwent intracoronary infusion of bone marrow-derived (BMCs) (15 patients) or circulating blood-derived endothelial progenitor cells (EPCs) (11 patients) 4+/-2 days after acute myocardial infarction. Based on a left ventricular segmentation model (17 segments), mean signal intensities as a parameter of viability and perfusion in the infarct zone and non-infarct areas were calculated quantitatively by PET and SPECT at baseline and at 4 months of follow-up. Transplantation of progenitor cells was associated with a significant increase in the mean signal intensity (MSI) in the infarct zone from 54.5% (25th and 75th percentiles: 47.7%, 60.0%) to 58.0% (52.7%, 66.7%) on PET (P=0.013) and from 58.0% (49.5%, 63.0%) to 61.5% (52.5%, 70.2%) on SPECT (P=0.005). Global left ventricular ejection fraction (LVEF) increased from 53.5% (42.6%, 60.0%) to 58.0% (53.0%, 65.8%) (P<0.001). In the five patients without an increase in MSI on PET, LVEF changed from 60.0% (50.0%, 64.0%) to 72.0% (64.0%, 75.5%) at follow-up. PET and SPECT did not show any significant changes in MSI in the non-infarct areas [from 73% (68.5%, 76.2%) to 73% (69.7%, 78.0%) for PET and from 72.0% (66.5%, 77.6%) to 73.0% (67.5%, 78.2%) for SPECT]. There were no significant differences in myocardial viability and perfusion between BMC and EPC infusion. These preliminary results show that coronary stenting and transplantation of progenitor cells result in a significant increase in myocardial viability and perfusion. Therapeutic effects can be reliably measured by PET and SPECT.

Uterine contractility and directed sperm transport assessed by hysterosalpingoscintigraphy (HSSG) and intrauterine pressure (IUP) measurement.

BACKGROUND: Uterine peristalsis sustains sperm transport and can be detected by hysterosalpingoscintigraphy (HSSG). This study is the first to be designed to investigate utero-tubal transport function by HSSG and uterine contractility by intrauterine pressure measurement (IUP) consecutively on the same day in the periovulatory phase. METHODS: Twenty-one female subjects (mean age 28.4 years) without a gynecologic history were examined sequentially by HSSG and IUP on the same day to evaluate uterine contractility in relation to the utero-tubal transport function. In HSSG, intact transport function was visualized by the rapid uptake of 99m-technetium-marked albumin aggregates through the female genital tract. In IUP, the frequency of uterine contractions (UC/min), amplitude of uterine contractions and basal pressure tone were detected via a intrauterine catheter. HSSG and IUP were embedded in cycle monitoring with measurement of LH and estradiol. RESULTS: In HSSG, a positive transport of inert particles was assessed in 20 of 21 subjects, in 76% to the side of the dominant follicle or on both sides of the oviduct, and in 19% a strict contralateral transport could be observed. In only one subject (5%), no transport was assessed. The mean value of uterine contractions was 3.4 UC/min (SD +/- 0.7), the mean amplitude was 12.0 mmHg (SD +/- 4.25 mmHg). Basal pressure tone was 70.7 mmHg. There was a statistically significant correlation with estradiol levels: none of the subjects with less than 3 UC/min showed an estradiol level higher than 100 pg/mL; nearly every patient (one exception) with more than 3 UC/min had an estradiol level higher than 100 pg/mL (p < 0.0001, Fisher's exact test). CONCLUSIONS: Intact periovulatory utero-tubal transport function can be documented by HSSG and is caused by directed uterine contractility, measured consecutively by IUP. Uterine contractility is influenced by rising estradiol levels. Directed uterine contractility and intact utero-tubal transport function are considered necessary for intact sperm transport, mainly to the side bearing the dominant follicle to maximize fertility.

PET imaging of Rosai-Dorfman disease: correlation with histopathology and ex-vivo beta-imaging.

We report the case of a woman who developed an early relapse of a squamous cell carcinoma (SCC) and was thus restaged twice within a year using [(18)F]fluorodeoxyglucose (FDG) positron emission tomography (PET). While there was no evidence of metastatic tumor outspread, focally increased FDG uptake was visible in numerous nodes but showed no change during the period between the two PET scans. These nodes, predominantly located at the proximal extremities, ranged in size from about 1 cm to over 6 cm. They were located subcutaneously, showed a red/bluish livid color and were of stout consistency. These nodes occurred first after radiochemotherapy for a non-Hodgkin's lymphoma (NHL) about 6 years earlier and slowly increased in size and number. One node of the right forearm was resected and ex-vivo beta-imaging, directly measuring the positron emission of the intranodal FDG distribution, was done and showed an overall increased glucose utilization with distinct spots of high metabolism. Histopathological work-up of the tumor showed widespread granulomatous tissue with lymphocyte follicles. Immunostaining showed the tumor to be positive for S100, CD68 and vimentin. Rosai-Dorfman disease (RDD) was diagnosed and no evidence of a potential relapse of the previous NHL was detected. RDD is a rare disease that is associated with the multifocal growth of benign tumors. The lesions are metabolically highly active. The correlation of the beta-imaging and histopathological results showed a high metabolism within granulomatous tissue with more intense metabolism within lymphocyte follicles.

Positron emission tomography in patients with Hodgkin's disease: correlation to histopathologic subtypes.

The aim of this study was to evaluate the initial staging and restaging of Hodgkin's disease (HD) according to histopathologic subtype (HST) using fluorine-18-deoxyglucose-positron emission tomography (PET). Special attention was paid to the accuracy of PET for detection of bone marrow infiltration (BMI). 44 patients with HD (m:f = 28:16, mean age 36 +/- 15 years) underwent PET; 16 were primary stagings and 28 restaging examinations. PET results were compared with methods of conventional staging including computed tomography (CT) and bone marrow biopsy. Viable tumor tissue was detected by PET in 25/44 cases, 16 nodular sclerosis (NS), 4 mixed cellularity (MC), 3 lymphocyte predominance (LP) and 2 cases with a nonclassified subtype (NC). FDG tumor uptake, measured as standard uptake value (SUV), ranged from 1.7 to 13. Maximum SUV in NS was 5.2 +/- 1.5 (2.5-7.3), 3.2 +/- 2.4 for MC, 2.6 +/- 0.7 for LP, and 9.1 +/- 3.8 for NC, respectively. In 7% of all patients (3/44) bone marrow infiltrations were detected by PET. PET is known for its superior detection of viable tissue in HD. In this study it was shown that HST does not influence the intensity of glucose metabolism, although 2 patients with NC showed the highest SUVs. In addition PET accurately detected focal BMI and may thus be applied before BMB to guide its optimal use.

rhTSH stimulation before radioiodine therapy in thyroid cancer reduces the effective half-life of (131)I.

UNLABELLED: Recombinant human thyroid-stimulating hormone (rhTSH) is effectively used for exogenous thyroid-stimulating hormone (TSH) stimulation before diagnostic (131)I scintigraphy. It is not yet widely used for preparation of patients receiving a therapeutic amount of radioiodine. METHODS: The results of 64 consecutive therapeutic applications of rhTSH with regard to clinical tolerance and side effects were evaluated in comparison with 163 radioiodine therapies (RITs) done on patients with hypothyroidism after thyroxine withdrawal during the same period. All therapies-applying 1.1-10 GBq of (131)I-used a standardized protocol of patient preparation and activity application. RITs were followed by daily whole-body uptake measurements for 2-6 d, and a biexponential curve fit was used to obtain a short initial and afterward a long effective half-life of (131)I. Patients after rhTSH were evaluated as a whole group (group A, n = 64) and as a subset of that group with normal thyroglobulin (hTG) levels (group D, n = 18). Patients after endogenous TSH stimulation were evaluated as a whole group (group B, n = 163), as a subset of that group excluding all ablative RITs (group C, n = 113), and as a subset of that subset with normal hTG levels (group E, n = 87). RESULTS: rhTSH-stimulated patients showed significantly higher TSH values than did endogenously stimulated patients (P < 0.001). Furthermore, the effective half-life of (131)I was significantly prolonged after endogenous stimulation (e.g., 0.43 d for group A vs. 0. 54 d for group B, P < 0.001). All rhTSH applications were tolerated well and without serious side effects. The only side effects were 2 cases of nausea and headache. CONCLUSION: The use of rhTSH for stimulation of TSH before RIT is safe but also significantly reduces the effective half-life of (131)I. This is mainly due to a reduced renal iodine clearance in the hypothyroid state, but the bioavailability of radioiodine may be slightly overestimated because of larger amounts of intestinal (131)I after endogenous TSH stimulation.

Enchondroma: a benign osseous lesion with high F-18 FDG uptake.

A woman was referred for fluorodeoxyglucose positron emission tomography for the staging of a malignant melanoma. Although no signs of metastatic melanoma were evident on the whole-body scan, focally increased uptake within the femoral metaphysis was noted. Radiographic and magnetic resonance examinations revealed an enchondroma as the cause of the increased uptake. Histopathologic verification was obtained. The final diagnosis was actively proliferating enchondroma. A grade I chondrosarcoma could be ruled out. Enchondromas may be responsible for focally increased FDG uptake in bone lesions and must be considered when positron emission tomographic scans obtained with FDG are evaluated in cancer staging.