Zollinger-Ellison syndrome: Revelation of the gastrinoma triangle.

Zollinger-Ellison syndrome is a complex condition in which one or more tumors form in the patient's pancreas or upper duodenum. These tumors, called gastrinomas, secrete excessive amounts of gastrin, and almost all develop ulcers. The vast majority of gastrinomas are present within the "gastrinoma triangle," which is composed of the porta hepatis, duodenal sweep, and pancreatic head. As surgery remains the treatment of choice, localization of the primary lesion is often challenging but essential. We present a 50-year-old man with a tentative diagnosis of Zollinger-Ellison syndrome. His In-111 pentetreotide scan, fused onto a Tc-99m abdomen image, revealed an avid lesion adjacent to the duodenal loop. Operative resection was performed, and a primary pancreatic gastrinoma was diagnosed by immunohistochemical staining. The neuroendocrine tumors have somatostatin receptors upon them. Therefore, a penteteotide scan, using In-111 radiolabelled somatostatin analogues, is the current technique of choice. This dual-isotope display permits a visual perception of anatomic landmarks around the lesion.

Bisphosphonate-related osteonecrosis of the jaw complicated by Ludwig's angina.

Ludwig's angina is a life-threatening cellulitis that involves the submandibular and sublingual spaces. It often occurs after an infection of the roots of the teeth. However, modern dental care and use of antibiotics for oral infections have made Ludwig's angina rare. We present here a cancer patient exhibiting the sequential features of bisphosphonate related osteonecrosis of the jaw on bone scan complicating with Ludwig's angina. This report highlights the need for medical practitioners to be alert to these rare combinations in the compromised patient after bisphosphonate therapy. To the best of our knowledge, no case of Ludwig's angina secondary to osteonecrosis of the jaw has been reported.

RBC volume deficiency in patients with excessive orthostatic decrease in cerebral blood flow velocity.

BACKGROUND: Orthostatic intolerance (OI) is common but heterogeneous. There is a subgroup of OI patients who have excessive decrease in cerebral blood flow velocity (CBFV) of bilateral middle cerebral arteries (MCAs) during head-up tilt without systemic blood pressure change. This study evaluated the role of blood volume reduction in such patients. METHODS: Patients with idiopathic OI who had excessive orthostatic decrease (>20% of the supine level) in mean CBFV of bilateral MCAs and who also received blood volume determination were collected. The chromium (5¹Cr) dilution method was used for red blood cell (RBC) volume determination in these patients. The blood volume was expressed as a percentage of the expected volume. These patients were further divided into two groups, those with postural tachycardia syndrome (POTS group) and those without (non-POTS group). The data of RBC volume were compared between the two groups. Besides, we used multivariate linear regression to evaluate the factors that predict RBC volume. RESULTS: Twenty-five patients (13 females, median age = 28 years) were enrolled in this study. Nine of these patients had POTS (5 females, median age = 26 years) and 16 did not (8 females, median age = 29.5 years). Compared with the expected volume, the RBC volume was significantly reduced in all patients (median = 82% of the expected volume). Moreover, the RBC volume was significantly lower in the POTS group than that in the non-POTS group (78% vs. 85% of the expected volume, p = 0.013). The orthostatic decrease of MCA flow velocity was 28.3% in the POTS group and 32.5% in the non-POTS group (p = 0.140). The orthostatic pulsatility index increment was 15.4% in the POTS group and 20.5% in the non-POTS group (p = 0.438). Moreover, basic demography and hemoglobin levels were not different between the two groups. After multivariate linear regression (dependent variables including age, sex, body surface, and groups), only the presence of POTS significantly predicted the RBC volume (p = 0.006). CONCLUSION: The results of our study indicated that low RBC volume may play an important role in the pathophysiology of OI in this group of patients. Moreover, its role seems even more relevant in patients with POTS than in those without. Further studies for mechanistic evaluation are needed in the future.

Unusual site of metastasis detected with FDG PET/CT in a case of recurrent rectosigmoid cancer.

Metastatic colon cancers to the oral cavity are unusual, involving a majority of the mandible rather than the oral soft tissues. We describe a rare site of gingival metastasis in a case of rectosigmoid cancer. The patient was a 74-year-old woman who developed swelling and persistent bleeding of the right mandibular gingiva after tooth extraction. A PET/CT using 18F-FDG depicted multiple metastatic lesions in the neck, lung, abdominal wall, and mandibular gingiva. Histologic study of the gingival lesion confirmed the diagnosis of gingival metastasis from colon adenocarcinoma.

Hallucal sesamoiditis manifested on bone scan.

The first metatarsal articulation bears one third of the weight of the forefoot. The hallucal sesamoids are embedded in the flexor hallucis brevis and connected by the intersesamoid ligament and plantar plate. The sesamoid apparatus acts as a pulley to help pull the big toe down against the ground during walking. Repetitive pressure, force, or tension can cause sesamoiditis. If the impact is great enough, the bones can break. Here we present a woman with big toe pain during walking. Our case highlights the role of 99mTc-MDP bone scan in reaching the diagnosis of hallucal sesamoiditis.

Compartment syndrome following robotic-assisted prostatectomy: rhabdomyolysis in bone scintigraphy.

Major urologic surgery performed in the lithotomy position sometimes results in the serious complication of rhabdomyolysis. A 56-year-old man was admitted to the hospital for prostate adenocarcinoma. A whole-body bone scan was performed to exclude bony metastases, which demonstrated no bone lesions but showed intense soft-tissue activity in gluteus maximus muscles, findings suggestive of a myopathy. He had just undergone right nerve-sparing radical prostatectomy in the lithotomy position for 6 hours and presented with swollen bilateral thighs. Elevation of creatine kinase level confirmed muscle injury.

Supernumerary breast masquerading as metastasis on serial 67Ga scan.

We report the incidental discovery of supernumerary breast changes on sequential 67Ga scans in a woman with dermatofibrosarcoma protuberans excised 5 years before. The appearance of the accessory breast fluctuated with physiological 67Ga uptake of the breast tissue was evident. Awareness of the concomitant changes in accessory breast with breast tissue could help narrow the differential diagnosis.

Incidental finding of autosomal dominant polycystic kidney disease with liver involvement on Tc-99m sestamibi scintigraphy.

Autosomal dominant polycystic kidney disease is a systemic hereditary disease characterized by renal cysts and sometimes involvement of the liver. We present a 65-year-old woman with autosomal dominant polycystic kidney disease on regularly hemodialysis who recently experienced intermittent right upper quadrant abdominal pain and elevated intact parathyroid hormone for more than a year. She was referred for double-phase Tc-99m sestamibi scintigraphy, under the impression of hyperparathyroidism. Apart form increased uptake in the right thyroid bed, the images showed a large photon-deficient area in the upper portion of the abdomen corresponding to the liver.

Epileptic negative myoclonus: SPECT, PET, and video/EEG studies and the dramatic effects of levetiracetam.

An 18-year-old woman presented with epileptic negative myoclonus (ENM) as her major seizure pattern for 4years. Her seizures were characterized by intermittent postural lapse of the right limbs for a period of hours to 2 days. Ictal electroencephalography (EEG)-electromyography showed a silent period that was time-locked to generalized spike-wave discharges. Video/EEG monitoring demonstrated marked improvement of ENM after oral administration of levetiracetam. Cranial magnetic resonance imaging was normal, but 2-deoxy-2-[(18)F]fluoro-d-glucose positron emission tomography during frequent ENM showed hypometabolism in the left frontoparietal cortex. Technetium-99m-ethyl cysteinate dimer single-photon emission computed tomography revealed hyperperfusion over the left parietal cortex and putamen. Here, we document the short-term effects of levetiracetam in this subject with nearly isolated ENM and the neuroimaging results during ENM. Long-term follow-up is in progress to evaluate the clinical evolution and long-term effects of levetiracetam on ENM.

PET imaging of brain astrocytoma with 1-11C-acetate.

PURPOSE: The purpose of this study was to assess the use of 1-(11)C-acetate (ACE) as a metabolic tracer for the detection and characterisation of astrocytomas. METHODS: Positron emission tomography (PET) studies with ACE and 2-(18)F-fluoro-2-deoxy-D-glucose (FDG) were performed sequentially in 26 patients with primary astrocytomas. Images were analysed by visual interpretation and determination of the tumour to cortex ratio (T/C ratio) and standardised uptake value (SUV). The tumour uptake was visually scored into three grades as compared with the contralateral cortex: clearly lower (-), almost equal (+) and clearly higher (++). RESULTS: There were 85% of astrocytomas with ++ ACE uptake, 15% with + ACE uptake and none with - ACE uptake. Only 19% of astrocytomas had ++ FDG uptake. Thirty-seven percent of high-grade astrocytomas had + FDG uptake and 37% had - FDG uptake. The sensitivity and specificity of the FDG T/C ratio in discriminating high-grade from low-grade astrocytomas were 79% and 100%, respectively, at the cutoff value of 0.75. Using 2.33 as the cutoff value of the ACE T/C ratio, the sensitivity and specificity were 42% and 86%, respectively. FDG was better than ACE in discriminating high-grade from low-grade astrocytomas. T/C ratios and SUVs of FDG uptake of tumours correlated with the histological grades, but those of ACE uptake did not. CONCLUSION: ACE appears to be a promising tracer for use in the detection of primary astrocytomas, but is of limited value in the differentiation of high- and low-grade astrocytomas. ACE is complementary to FDG for the diagnosis and characterisation of astrocytoma.

Xanthogranulomatous pyelonephritis treated by partial nephrectomy.

Xanthogranulomatous pyelonephritis is an uncommon disease in children. We report of a 2-month-old girl with urinary tract infection and with a renal mass detected by ultrasound scan. The preoperative differential diagnoses were Wilms tumor, multicystic dysplastic kidney, renal abscess, and mesoplastic nephroma. The subsequent histopathological findings allowed the diagnosis of xanthogranulomatous pyelonephritis (XGP). Although XGP is rare at this age, it must be considered in the differential diagnosis of a child presenting with renal mass, either with or without associated urolithiasis, anemia, and elevated inflammatory markers. The long-standing mainstay of therapy for diffuse XGP has been nephrectomy. However, we report the successful preservation of renal mass with partial nephrectomy for diffuse XGP. The affected kidney grew normally and preserved residual function after the operation. Hence we strongly suggest parenchymal saving in pediatric cases of XGP to preserve renal function.

High prevalence of a novel mutation (2268 insT) of the thyroid peroxidase gene in Taiwanese patients with total iodide organification defect, and evidence for a founder effect.

The mutation of the thyroid peroxidase (TPO) gene that causes the total iodide organification defect (TIOD) is a common and severe condition leading to dyshormonogenesis of the thyroid gland in Caucasians. However, the role of TIOD in Chinese patients with thyroid dyshormonogenesis is unknown. In this study we followed 16 patients from 16 unrelated families in Taiwan and performed perchlorate discharge examination. Seven patients had TIOD and 2 had the partial iodine organification defect (PIOD) among the 16 families. These 9 patients underwent screening in search of TPO gene mutations. Three new mutations (2268 insT, 2243 delT, and G157C) were detected in the 7 patients with TIOD, whereas no mutation in the TPO gene was found in the 2 patients with PIOD. The 2268 insT mutation was noted to be the most common among these TIOD patients (12 of 14 studied alleles, 86%). With 3 intragenic polymorphic markers, we found that the alleles carrying the 2268 insT mutation in Taiwan Chinese TIOD patients were tightly linked to a specific haplotype. The allele frequencies of this haplotype in the 8 patients with homozygous 2268 insT (5 unrelated families, 10 studied alleles) and in 49 normal individuals (98 studied alleles) were 1.00 and 0.02, respectively (P < 0.0001). This indicates that this common novel mutation among Taiwanese patients with TIOD is due to a founder effect.