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B Niederle

Publications and source records attributed to B Niederle.

At least 55 records · Page 3Linked to original sources
PubMed2000

New radiopharmaceuticals for receptor scintigraphy and radionuclide therapy.

In vitro data have demonstrated a high amount of receptors for various hormones and peptides on malignant cells of neuroendocrine origin. Among these, binding sites for members of the SST-family (hSSTR1-5) are frequently found, and their expression has led to therapeutic and diagnostic attempts to specifically target these receptors. Receptor scintigraphy using radiolabeled peptide ligands has proven its effectiveness in clinical practice. In addition, initial results have indicated a clinical potential for receptor-targeted radiotherapy. Based on somatostatin (SST) receptor (R) recognition, the novel radiopharmaceuticals 111In/90Y-DOTA-lanreotide developed at the University of Vienna as well as 111In/90Y-DOTA-DPhe1-Tyr3-octreotide (NOVARTIS) both have provided promising data for diagnosis and treatment of hSSTR-positive tumors. SSTR scintigraphy using 111In-DTPA-DPhe1-octreotide has a high positive predictive value for the vast majority of neuroendocrine tumors and has gained its place in the diagnostic work-up as well as follow-up of patients. We have used 111In-DOTA-lanreotide scintigraphy in 166 patients since 1997 and have seen positive results in 93% of patients. In 42 patients with neuroendocrine tumors comparative data were obtained. As opposed to 111In-DTPA-DPhe1-octreotide and 111In-DOTA-DPhe1-Tyr3-octreotide, discrepancies in the scintigraphic results were seen in about one third of patients concerning both the tumor uptake as well as tumor lesion detection. Initial results both with 90Y-DOTA-lanreotide as well as 90Y-DOTA-DPhe1-Tyr3-octreotide has pointed out the clinical potential of radionuclide receptor-targeted radiotherapy. This new therapy could offer palliation and disease control at a reduced cost. The final peptide therapy strategy is most probably cheaper than conventional radiotherapies or prolonged chemotherapies. Overall, receptor-mediated radiotherapy with 90Y-DOTA-lanreotide/90Y-DOTA-DPhe1-Tyr3-octre otide might also be effective in patients refractory to conventional strategies.

Humans↗
PubMed1999

[The value of fine-needle aspiration biopsy (FNAB) in the differential diagnosis of the "cold" thyroid nodule].

INTRODUCTION: According to the literature, fine needle aspiration biopsy (FNAB) should be the first line test to assess thyroid nodules. The value of fine needle aspiration biopsy in the differential diagnosis of "cold" thyroid nodules is discussed. METHODS: 105 consecutive patients undergoing surgery for solitary cold nodule or a dominant cold nodule in multinodular goiter and having a preoperative fine needle aspiration biopsy, are analysed. RESULTS: The cytologic results were benign in 48 cases, malignant in 8 cases and indeterminate (follicular neoplasia) in 29. Twenty biopsies were inadequate. Excluding inadequate and indeterminate biopsy results and calculating microcarcinomas (as incidental findings) as "true negative", sensitivity was 56%, specify 94% and accuracy 88%. There was a negative predictive value of 92% and a positive predictive value of 63%. CONCLUSIONS: Fine needle aspiration biopsy is helpful as an additional test. The indication for surgery for a "cold" thyroid nodule must be established in conjunction with clinical findings and other investigation procedures.

Adolescent↗
PubMed1999

[The value of preoperative localization studies in primary hyperparathyroidism].

Bilateral neck exploration is the standard procedure in primary hyperparathyroidism. Using a bilateral approach, preoperative localization studies are not mandatory. A localized single gland disease is the basis for a minimally invasive procedure. Therefore preoperative localization techniques play an important role in planning (minimally invasive) first time exploration for primary hyperparathyroidism. The combination of (99 m)Tc-SestaMIBI scintigraphy with ultrasound allows correct localization of the parathyroid adenoma in 86 % of all single gland diseases. Multiple gland disease could be predicted in isolated cases only. Computerized tomography and magnetic resonance imaging could not improve the results. Surgery for persistent or recurrent hyperparathyroidism should only be performed after positive localization studies.

Diagnostic Imaging↗
PubMed1999

[Characterization of adrenal gland space-occupying lesions. Can diagnostic imaging replace biopsy?].

This paper describes the diagnostic value of new imaging techniques in characterization of adrenal masses and evaluates the role of adrenal biopsy. For differentiation of benign and malignant adrenal lesions, sensitivities and specificities of 85-100% can be reached by unenhanced computed tomography (CT) methods, chemical shift techniques in magnetic resonance imaging (MRI), delayed enhanced CT techniques, and by evaluation of wash-out curves in contrast-enhanced CT. The diagnostic value of all CT techniques depends on threshold values. The value of dynamic contrast-enhanced MRI is under discussion and should be reevaluated by using delayed enhanced series. Biochemical and scintigraphic methods (NP 59 iodine iodomethyl-norcholesterol and MIBG meta-iodobenzylguanidine scintigrams) are valuable for the diagnosis of functional adrenal masses; however, they do not allow differentiation of benign and malignant tumors. According to excellent results of new imaging techniques in characterization of adrenal masses, the indications for fine-needle aspiration biopsy have already regressed, as have complications associated with this invasive technique.

Adrenal Gland Diseases↗
PubMed1999

Anaplastic (undifferentiated) thyroid carcinoma (ATC). A retrospective analysis.

BACKGROUND: Old age, reduced general condition and far advanced tumor stage associated with poor prognosis induced the belief that, apart from verifying the diagnosis of anaplastic thyroid carcinoma (ATC) by biopsy, no additional surgery would be justified. However, in some cases, an ultraradical approach was recommended in order to improve the quality of life and survival. METHODS: These are the results of a retrospective analysis involving 120 patients subjected to restricted radical surgery (excising as much as possible of the tumor and local metastases, foregoing ultraradical removal of vital organs such as esophagus, larynx and trachea). RESULTS: Irrespective of the surgical approach used, 6+/-2% of the patients were alive after 5 years (median survival time: 3.1 months). Patients without tumor residues (R0-resections; extending to soft tissue only; Kaplan-Meier estimate - cumulative survival 15+/-5%) had a significantly better prognosis than patients with tumor residues (R1/R2-resections; no patient survived 5 years; P<0.001). Tumor morphology (spindle cells, giant cells, mixed cells) or differentiated parts of the tumor as well as lymph-node involvement had no statistically significant impact on the prognosis. CONCLUSIONS: In ATC, the objective should be to remove as much of the carcinoma as possible (in the ideal case, a thyroidectomy); if lymph nodes are affected, neck dissection should be the goal, if possible (restricted radical approach, improving quality of life). Ultraradical surgery to include segmental resection of larynx, trachea or esophagus do not seem to be indicated, as prolonged survival is questionable and quality of life is certainly diminished.

Adult↗
PubMed1999

Differentiated thyroid cancer in children and adolescents: the importance of adequate surgery and review of literature.

Within 25 years, 13 girls and 5 boys between the ages of 9 to 20 years were treated for differentiated thyroid cancer (DTC) at our institution. In 67% of the cases the diagnosis was made at an advanced stage indicated by cervical lymphnode metastases, two children showed metastases of the lung. Except for one girl, all patients underwent a total thyroidectomy with various forms of lymph node dissections. All were treated post-surgically with radio-iodine (131I) and all patients received levothyroxine in suppressive doses. The complication rate was low (laryngeal nerve injury in only 3 cases and no permanent hypoparathyroidism) and after a follow-up period of 26 years all patients are alive and without disease. In the case of DTC in children and adolescents, a total thyroidectomy with an appropriate lymph node dissection is the best surgical management. Besides the rather high incidence of local and distant recurrence, particularly in this age group, the detection and treatment of the metastases with radio-iodine are significantly facilitated by this surgical procedure. Additionally it allows the use of serum thyroglobulin as a reliable indicator of recurrence. To prevent high complication rates, surgical treatment should be performed in centers with skilled and experienced endocrine surgeons.

Adolescent↗
PubMed1999

Is medullary thyroid cancer predictable? A prospective study of 86 patients with abnormal pentagastrin tests.

BACKGROUND: The aim of this prospective study was to distinguish biochemically between C-cell hyperplasia (CCH) and medullary thyroid cancer (MTC) before surgery. METHODS: Eighty-six consecutive patients with an abnormal stimulated calcitonin level (> 100 pg/mL) underwent thyroidectomy and lymph node dissection. In sporadic MTC, histopathologic findings and postoperative biochemical outcomes were documented prospectively and correlated with preoperative basal and stimulated calcitonin levels. RESULTS: Analysis of variance revealed a highly significant difference in basal/stimulated calcitonin levels (P < .0001), with a comparison of CCH (n = 39 patients) and sporadic MTC (n = 38 patients). With a comparison of sporadic MTC N0 M0 (n = 25 patients) and N1 M0/1 (n = 12 patients), the basal calcitonin level was significantly different (P < .05). There was a close correlation between the n-log of basal/stimulated calcitonin level and the n-log of the tumor volume; there were also different distributions of the n-log of basal/stimulated calcitonin level among CCH, MTC N0, and MTC N1. Assuming that a basal calcitonin level of more than 64 pg/mL and/or a stimulated calcitonin level of more than 560 pg/mL implies MTC, 31 of 38 patients with sporadic MTC were detected before surgery. Three patients were predicted false positive (neoplastic CCH). Patients with stimulated calcitonin levels of less than 129 pg/mL had CCH only. Patients with basal calcitonin levels of less than 22 pg/mL and sporadic MTC (7/38 patients) were node negative. CONCLUSIONS: All patients with abnormal pentagastrin tests showed C-cell pathologic evidence. Sporadic MTC was predicted in 81% of the patients; CCH or N0 was predicted in 36% of the patients. Central neck dissection is recommended to avoid difficult reoperations. Lateral neck dissection is possible "on demand."

Adult↗
PubMed1999

Basal calcitonin levels and the response to pentagastrin stimulation in patients after kidney transplantation or on chronic hemodialysis as indicators of medullary carcinoma.

Plasma concentrations of calcitonin (hCT) were determined in 150 patients with chronic renal failure on chronic hemodialysis therapy (CHD) and in 800 patients after successful kidney transplantation (KT). Basal hCT concentrations exceeded 10 pg/mL in 44 of 150 patients (29%) with CHD and in 48 of 800 (6%) in patients with KT. Among these patients with elevated basal hCT, pentagastrin-stimulated concentrations of hCT exceeded 100 pg/mL in 4 patients with CHD and in 7 with KT. Thyroidectomy was performed in 8 patients (5 with KT, 3 with CHD) revealing the presence of medullary thyroid carcinoma (MTC) (n = 2) or of C-cell hyperplasia (n = 6). Two patients with C-cell hyperplasia had the neoplastic form of this disorder. One patient with MTC and 1 with C-cell hyperplasia also presented a papillary microcarcinoma. Stimulated concentrations of hCT were only moderately elevated in the remaining 3 patients and follow-up rather than surgery was deemed appropriate due to their concomitant severe medical problems. In conclusion, basal concentrations of hCT higher than 10 pg/mL are more common in patients with CHD (29%) and after successful KT (6%) than previously described in patients with thyroid nodular disease (3%). In spite of various additional factors complicating the interpretation of elevated hCT in CHD, pentagastrin-stimulated values above 100 pg/mL must be considered to indicate the presence of C-cell hyperplasia and/or of medullary thyroid carcinoma. Although thyroidectomy would theoretically be the therapy of choice, the potential benefit of the operation must be seen in the context of the patient's general condition.

Adult↗
PubMed1999

Characterization of adrenal masses using MR imaging with histopathologic correlation.

OBJECTIVE: The purpose of this study was to evaluate the sensitivity, specificity, and accuracy of MR imaging in the characterization of adrenal masses by correlating imaging findings with histopathologic results. In addition, adrenal tumors that were of an indeterminate nature on MR imaging were analyzed. SUBJECTS AND METHODS: For 114 patients with 134 adrenal masses, MR findings were compared with histologic results. In all patients, MR imaging was performed using T2-weighted fast spin-echo imaging and unenhanced and gadolinium-enhanced T1-weighted spin-echo imaging. Chemical-shift imaging was performed in 92 patients and dynamic gadolinium-enhanced studies in 108 patients. Chemical-shift images were analyzed quantitatively and qualitatively, and dynamic gadolinium-enhanced studies were qualitatively assessed. RESULTS: The sensitivity of MR imaging in differentiating between benign and malignant adrenal masses was 91%, the specificity was 94%, and the accuracy was 93%. The diagnosis at MR imaging differed from that at histology in 12 (9%) of 134 patients. Results of quantitative analyses of chemical-shift imaging techniques showed significant differences between adenomas and nonadenomas (-36.0% versus -3.7%; p < .001). Qualitative analysis provided a similar diagnostic confidence compared with quantitative analysis. Both chemical-shift and dynamic gadolinium-enhanced studies proved to be unreliable in characterizing borderline tumors (epithelial tumors with high malignant potential). Moreover, such imaging failed to allow correct diagnosis of adenomas in two patients. CONCLUSION: The characterization of an adrenal mass can be made with high sensitivity and specificity using MR imaging. The increased reliance on MR imaging seems to be based mainly on findings from chemical-shift and dynamic gadolinium-enhanced studies. The need to perform histologic sampling of incidentally discovered adrenal masses may be reduced to some problematic lesions, which will remain during the era of MR imaging.

Adolescent↗
PubMed1999

Cytogenetic and CGH studies of four neuroendocrine tumors and tumor-derived cell lines of a patient with multiple endocrine neoplasia type 1.

A malignant insulinoma (LOHG-I), a carcinoid of the lung (LOHG-L), a parathyroid adenoma (LOHG-NSA), and a fibroma (LOHG-F) were obtained from a patient with multiple endocrine neoplasia type 1 (MEN1). Long-term cultures were established. Essential neurobiological properties of the cell lines were proven immunocytochemically and by electron microscopy. Molecular analysis of the germline DNA showed a 4 bp deletion in exon 3 of the MEN1 gene. Cytogenetic and CGH analyses of the tumors/tumor cell lines revealed diploidy and balanced and unbalanced structural aberrations different for each tumor. Chromosomes 6q21, 11q and 17q were most frequently involved in clonal structural aberrations.

Adenoma↗
PubMed1998

Stage-dependent therapy of rectal carcinoid tumors.

Although malignant behavior of rectal carcinoid tumors is rare, the risk of metastases and death does exist. Adaptation of therapy according to the estimated malignancy seems necessary. To develop a stage-dependent therapy, 31 patients with rectal carcinoid tumors measuring 5 to 50 mm in diameter were analyzed retrospectively. Malignancy was estimated according to tumor size, infiltration depth, and histopathology. There were 18 tumors within the mucosa and submucosa (T1), 7 tumors with muscularis propria invasion (T2), and carcinoid tumor penetrating the full rectal wall (T3) or spreading to surrounding tissue (T4) in 6 patients. Altogether 20 patients (65%) were treated with a minimally invasive intervention: endoscopic polypectomy (EP) in 12 and transanal excision (TE) in 8 patients. In 11 patients (35%) aggressive surgical procedures--anterior resection (AR) in 4 and abdominoperineal resection (APR) in 7--were performed. After a mean +/- SD follow-up of 86.0 +/- 61.3 months, tumor recurrence was not seen in any of the 20 patients with minimally invasive treatment, and all were still alive. No severe complications associated with surgical procedures were detected. In contrast, 5 of the 10 patients with advanced tumor stage died from their disease despite aggressive surgery (AR, APR). In conclusion, depending on tumor stage, treatment of rectal carcinoids includes EP, TE, or extended resection. Minimally invasive techniques are safe treatments for small to medium-size T1/T2 rectal carcinoids. Extended surgery cannot improve the overall survival of those with advanced tumors (T3/T4, N1, M1) but can be beneficial for preventing local complications.

Adult↗
PubMed1998

Prophylactic thyroidectomy in 75 children and adolescents with hereditary medullary thyroid carcinoma: German and Austrian experience.

When mutations of the RETproto-oncogene were found in 1993 to account for hereditary medullary thyroid carcinoma (MTC), surgeons obtained the opportunity to operate on patients prophylactically (i. e., at a clinically asymptomatic stage). Whether this approach is justified, and, if so, when and to which extent surgery should be performed remained to be clarified. A questionnaire was sent to all surgical departments in Germany and Austria. All of the patients who fulfilled the following criteria were enrolled: (1) preoperatively proved RET mutation; (2) age </= 20 years, (3) clinically asymptomatic thyroid C cell disease; and (4) TNM classification pT0-1/pNX/pN0-1/M0. Seventy-five patients were identified, and fifteen mutations were detected in six codons. Two adolescents had unilateral pheochromocytomas as part of the multiple endocrine neoplasia II (MEN-II) syndrome. No hyperparathyroidism was noted. All patients underwent total thyroidectomy, and 57 patients went on to have lymph node dissection. Parathyroid glands were removed in 34 patients and autografted in 11. Histopathology revealed MTC in 46 patients (61%, youngest 4 years); C cell hyperplasia (CCH) only was detected in the other 29 patients. Three patients had lymph node metastases (LNMs) the youngest being age 14 years. Calcitonin levels were not useful for differentiating between CCH and MTC, but in all patients with LNMs at least the stimulated calcitonin levels were assayed. After surgery, five patients (6.7%) sustained permanent hypoparathyroidism, and one patient (1.3%) had a permanent unilateral recurrent nerve palsy. All but three patients (96%) were biochemically cured. In conclusion, prophylactic total thyroidectomy can be performed safely in experienced centers. We recommend prophylactic total thyroidectomy at age 6. Cervicocentral lymph node dissection should be included when calcitonin levels are elevated or if patients are older than 10 years. Bilateral lymph node dissection should be performed if LNMs are suspected or when patients with elevated calcitonin are older than 15 years.

Adolescent↗
PubMed1998

Impairment of endothelium-independent vasodilation in patients with hypercalcemia.

OBJECTIVE: Patients with primary hyperparathyroidism (PHPT) and/or hypercalcemia are at increased risk for myocardial ischemia. Whether PHPT is associated with altered endothelium-dependent dilation, vascular smooth muscle cell function, or both is unknown. This study was performed to test the hypothesis that endothelium-dependent, flow-mediated dilation (FMD) and/or endothelium-independent, nitroglycerin-induced dilation (NMD) is impaired in the preclinical phase of vascular disease in patients with PHPT. METHODS: Twenty-six PHPT patients (mean +/- SD; age 55 +/- 15 y, serum calcium 3.00 +/- 0.37 mmol/l, serum phosphate 0.79 +/- 0.21 mmol/l, iPTH 249 +/- 262 pg/ml) with no evidence of coronary artery disease (CAD) as well as 26 normocalcemic control subjects (CTL; age 51 +/- 12 y) were studied. FMD following reactive hyperemia and NMD after 0.8 mg nitroglycerin (NTG) were assessed in the brachial artery by using high resolution ultrasound (7 MHz). RESULTS: NMD was impaired in PHPT patients compared to CTL (11.9 +/- 3.9% vs. 15.6 +/- 5.7%; p = 0.012). FMD was similar in both study groups (11.6 +/- 4.6% vs. 12.6 +/- 4.9; NS). The ratio of FMD to NMD was significantly different between PHPT patients and CTL (0.98 +/- 0.19 vs 0.81 +/- 0.25, p = 0.0009). On multiple stepwise regression analysis serum calcium was independently associated with the FMD/NMD ratio (r = 0.34, p = 0.017). CONCLUSIONS: Endothelium-independent vasodilation is impaired in PHPT patients without clinical evidence of coronary artery disease compared to normocalcemic CTL, while endothelium-dependent dilation was similar in both study groups. Thus, altered arterial reactivity in the course of PHPT may predominantly involve the arterial media and not the endothelium as observed previously in patients with various stages of atherosclerosis.

Adult↗
PubMed1998

First continuous human pheochromocytoma cell line: KNA. Biological, cytogenetic and molecular characterization of KNA cells.

Pheochromocytomas are rare tumours, with an incidence of 1-2 per million which arise from chromaffin cells of the adrenal medulla. They occur sporadically or as part of dominantly inherited cancer syndromes like multiple endocrine neoplasia 2 (MEN2A and 2B) and others. Continuous cell lines, not available so far, are essential tools for studies in these tumours. A continuous cell line (KNA) was established from a sporadic pheochromocytoma of the right adrenal gland of a 73-year-old woman. The KNA cells grow as suspensions of spheroids and show the morphological and immunocytochemical characteristics of neuronal chromaffin cells, such as neuroendocrine granules, and positive reactions to chromogranin- and related peptide-, neuron specific enolase and vasoactive intestinal peptide antibodies. Neurite-like processes are formed after addition of nerve growth factor. Chromosomal analyses revealed a diploid (46,XX,n = 50) to hypodiploid (43-45,XX,n = 15) karyotype. In hypodiploid metaphases most frequently #19, #17, #21 and #22 were missing. Chromosome arms 1p and 4q showed apparently consistent interstitial deletions: 6q, 8q, 13q and 22q showed clonal interstitial deletions. The cell line shows a heterozygous sequence variant TGC (cysteine) to TGG (tryptophan) in codon 611 in exon 10 of the RET proto-oncogene. So far, PC-12, a rat adrenal pheochromocytoma, has been the only continuous pheochromocytoma cell line available. KNA represents the first report on a human continuous pheochromocytoma cell line, the first report of structural chromosome aberrations in pheochromocytomas and the first report of a RET mutation TGC to TGG in exon 10 of the RET proto-oncogene in a sporadic pheochromocytoma.

Adrenal Gland Neoplasms↗
PubMed1998

Effects of parathyroid hormone and serum calcium on the phenotype and function of mononuclear cells in patients with primary hyperparathyroidism.

BACKGROUND: Several studies have demonstrated specific influence of parathyroid hormone (PTH) on immune parameters, especially on T- and B-cell function, migration of polymorphonuclear leucocytes (PMNLs) and antibody synthesis, in patients with secondary hyperparathyroidism and chronic renal failure and recently also in patients with primary hyperparathyroidism (pHPT). METHODS: We therefore examined 12 patients with pHPT before and 6 months after parathyroidectomy (PTX) and nine sex- and age-matched control subjects to determine the impact of PTH and serum calcium concentrations on several immune parameters, including (a) serum concentrations of immunoglobulins, (b) immunophenotype of peripheral blood lymphocytes, (c) phytohaemagglutinin (PHA)-induced lymphocyte proliferation and (d) monocytic surface marker expression. RESULTS: Serum concentrations of immunoglobulins (IgG, IgA, IgM) were unaffected by elevated serum PTH and calcium levels. T lymphocytes (CD3), B lymphocytes (CD19), NK cells (CD16/56) and monocytes (CD16) revealed a normal distribution and were not different before and after PTX in patients with pHPT when compared with the control group. CD4+ T-helper lymphocytes were significantly elevated pre- and post-operatively in patients with pHPT. The lymphocyte proliferation response to PHA in the highest concentration (12.5 micrograms L-1) tested was significantly suppressed in patients with pHPT preoperatively when compared with the patients post-operatively and the control group. In addition, both CD4+ and CD8+ lymphocytes showed a lower expression of activation markers, interleukin 2 (IL-2) receptor (CD25) and transferrin receptor (CD71), which could be partially restored 6 months after PTX, but did not reach normal values. CONCLUSION: In summary, in contrast to the findings in patients with secondary HPT, pHPT appears to be associated with less alterations of immune functions. Chronically elevated serum PTH and calcium concentrations in patients with pHPT induce a higher percentage of CD4+ helper T lymphocytes and a suppressed lymphocyte response to PHA as well as a reduced expression of activation markers on peripheral blood lymphocytes.

Adult↗
PubMed1998

C-cell hyperplasia and medullary thyroid carcinoma in patients routinely screened for serum calcitonin.

Routine screening of calcitonin serum levels in patients with nodular thyroid disorders has led to an increased rate of total thyroidectomies. We investigated prevalence and interrelationship of C-cell hyperplasia (CCH) and medullary thyroid carcinoma (MTC) in patients with thyroid and parathyroid disorders that showed increased calcitonin serum levels detected by routine screening. Within two years, 30 (mean age, 60 +/- 14 years) of 667 patients had a pentagastrin-stimulated calcitonin level of more than 100 pg/mL. All 30 underwent total thyroidectomy and were tested for germ-line mutations of the ret protooncogene. Entire surgical specimens were blocked, and C-cell disorders were assessed using conventional histology and immunohistochemistry. C-cell hyperplasia was defined by the presence of more than 50 C cells/l low-power field in both lobes and was classified as focal, diffuse, nodular, or neoplastic. Nineteen patients (female/male = 14/5) had MTC, and 11 males but no females had CCH only. Six of 16 patients with sporadic MTC had concomitant CCH. Three patients were index cases of new MTC families. We conclude that MTC with concomitant CCH is an unreliable marker for hereditary MTC risk and that CCH has a preneoplastic potential in the absence of germ-line mutations. In this series, CCH alone was not found in females.

Adult↗
PubMed1998

Location of a VIPoma by iodine-123-vasoactive intestinal peptide scintigraphy.

A major problem in patients with small endocrine tumors is the difficulty in localizing the primary tumor site. Many endocrine tumors possess larger amounts of high affinity vasoactive intestinal peptide (VIP) binding sites compared with normal tissue or blood cells. We used radiolabeled VIP to localize the tumor site in a patient with Verner-Morrison syndrome (VMS). Under octreotide therapy, the VIP levels had declined in this patient, but a tumor site could not be detected by conventional techniques or by radiolabeled octreotide. However, using 123I-VIP, the tumor was detectable in the pancreatic tail. Surgical resection of the tumor was followed by complete remission of the VMS. Expression of VIP binding sites in the tumor was confirmed by a radioreceptor assay and showed cross-competition between VIP and octreotide. The identity of the VIP binding site in the tumor was analyzed by Northern blotting and revealed the expression of somatostatin receptor subtype 3, which binds both somatostatin-14 and VIP with higher affinity than octreotide. Iodine-123-VIP scintigraphy would be an effective tracer to identity the tumor site in VMS patients.

Adult↗