Managing stage 4 ameloblastoma with dual BRAF/MEK inhibition: A case report with 8-year clinical follow-up.

We present 8-year follow-up on the first patient with stage 4 ameloblastoma carrying a BRAF V600E mutation treated with dual BRAF/MEK inhibition (BRAF/MEKi). He experienced a durable clinical response while on dabrafenib (BRAFi) and trametinib (MEKi) without toxicity nor evidence for drug-resistant tumor progression. He was asymptomatic when he self-discontinued therapy after 4 years of sustained clinical response. He did not return for follow-up until 2.5 years later with onset of painful mandibular tumor recurrence associated with recurrent bilateral lung metastases. He was rechallenged with dabrafenib/trametinib and experienced another prompt tumor response and remains in a second durable clinical remission (currently > 16 months) on continuous dual targeted therapy. We discuss the implications of this case study for future treatment strategies.

Percutaneous radionuclide ablation of axial aneurysmal bone cysts.

OBJECTIVE: The purpose of our study was to retrospectively examine the efficacy of intralesional injection of 32P chromic phosphate, a beta-emitting colloidal radiopharmaceutical, in the treatment of aneurysmal bone cysts of the axial skeleton. Five patients with large aneurysmal bone cysts were managed with injection of 32P chromic phosphate into their tumors under CT guidance. With only a single minor complication, all lesions were observed to ossify on follow-up CT, with an average follow up of 2 years. CONCLUSION: CT-guided injection of axial aneurysmal bone cysts with 32P chromic phosphate leads to excellent local lesion control. In addition, the morbidity associated with this procedure is lower than that associated with surgical or other nonsurgical treatments.

Lessons from a standardized program using PET-CT to avoid neck dissection after primary radiotherapy for N2 squamous cell carcinoma of the oropharynx.

OBJECTIVES: To report the results of a standardized program using positron emission tomography (PET)-computed tomography (CT) approximately 12 weeks after primary radiotherapy to determine the need for a planned neck dissection in patients with radiographic N2 squamous cell carcinoma (SCC) of the oropharynx. METHODS: Fifty consecutive patients with T1-4 and hemineck radiographic stage N2A-B SCC of the oropharynx for whom the only indication for planned neck dissection was a positive PET-CT performed ∼12 weeks after completing primary treatment with radiotherapy. RESULTS: Results of PET-CT to identify residual neck disease were as follows: sensitivity and positive predictive value, 0%; specificity, 89%; negative predictive value, 91%; potential neck recurrence from using this 12-week PET-CT program, 2%. The time between negative PET-CT and detection of neck recurrence was 0.5, 0.6, 1.2, and 2.0 years. The rate of successful (>1 year) salvage of neck recurrence was 25% (1/4). CONCLUSIONS: PET-CT approximately 12 weeks after radiotherapy for oropharyngeal cancer is an excellent way to identify patients who do not need neck dissection. Approximately half of neck recurrences present over 1 year after negative PET-CT and the chance of successful salvage is low.

Displacement of the Spleen Mimicking Renal Cell Cancer Recurrence Post-Nephrectomy: A Case Report.

Local regional recurrence of renal cell cancer post-nephrectomy most often occurs within three years after surgery. Post-nephrectomy, many processes may mimic RCC recurrence. We present the case of a 75 year-old Caucasian male patient with a mass in his renal fossa post-nephrectomy for renal cell cancer, suggesting local recurrence. Use of the technetium-99m sulfur colloid scan showed that the mass was his spleen which had been displaced into the renal fossa. With high index of suspicion, characterization of these processes as splenic in origin would prevent subjecting patients to risks of biopsy or even surgery.

Advantages of azithromycin over erythromycin in improving the gastric emptying half-time in adult patients with gastroparesis.

BACKGROUND/AIMS: Current therapy for gastroparesis with prokinetic agents is limited by options and side effects. One macrolide, erythromycin (ERY), is associated with possible sudden cardiac death from QT prolongation due to P450 iso-enzyme inhibition. An alternative, azithromycin (AZI), lacks P450 inhibition. We compared the effect on gastric emptying half-times (t½) between AZI and ERY in patients diagnosed with gastroparesis by gastric emptying scintigraphy. METHODS: Patients stopped medications known to affect gastric emptying prior to the study, and then ingested 1 scrambled egg meal labeled with 18.5-37 MBq of technetium-99m sulfur colloid followed by continuous imaging for 120 minutes, at 1 minute per frame. A simple linear fit was applied to the rate of gastric emptying, and gastric emptying t½ was calculated (normal = 45-90 minutes). At 75-80 minutes, if the stomach had clearly not emptied, patients were given either ERY (n = 60) or AZI (n = 60) 250 mg IV and a new post-treatment gastric emptying t½ was calculated. RESULTS: Comparison of gastric emptying t½ showed a similar positive effect (mean gastric emptying t½ for AZI = 10.4 ± 7.2 minutes; mean gastric emptying t½ for ERY = 11.9 ± 8.4 minutes; p = 0.30). CONCLUSIONS: AZI is equivalent to ERY in accelerating the gastric emptying of adult patients with gastroparesis. Given the longer duration of action, better side effect profile and lack of P450 interaction for AZI as compared with ERY, further research should evaluate the long term effectiveness and safety of AZI as a gastroparesis treatment.

Erythromycin improves gastric emptying half-time in adult cystic fibrosis patients with gastroparesis.

BACKGROUND: Gastrointestinal manifestations are frequently encountered in cystic fibrosis patients. Gastroparesis evidenced by a variety of diagnostic methods has been described in patients with cystic fibrosis, predominantly in children and in individuals with advanced lung disease. The presence of gastroparesis in adult patients with different degrees of lung involvement and its response to the acute and chronic administration of macrolides have not been reported. METHODS: Using the University of Florida Cystic Fibrosis database we identified symptomatic patients who had gastroparesis confirmed by a prolonged half-time during gastric emptying scintigraphy. RESULTS: Of 86 cystic fibrosis patients, periodically followed in our institution, we found five who had classical symptoms and prolonged gastric emptying half-time. Age 25.2+/-8 years, 80% females, BMI 22+/-9 kg/m(2), HbA1c 5.8+/-0.6 g/dl, FEV1 53.2+/-15% of predicted. Gastric emptying half-time was 191.4+/-91.4 min (range 100-300 min) and decreased to 12.2+/-6 min (range 5-20 min) after IV administration of erythromycin (p=0.043). Patients were followed up for 3+/-2.1 years. All patients but one, who was taking opiods, had good clinical response to PO macrolides. CONCLUSIONS: Gastroparesis occurs in patients with cystic fibrosis, even in patients with relatively preserved lung function and in those without cystic-fibrosis related diabetes. Macrolides may be an effective therapy in cystic fibrosis patients with gastroparesis when administered acutely or chronically.

Measurement of cerebrovascular flow reserve in pediatric patients with sickle cell disease.

PURPOSE: To report a method for determining absolute percentage change in cerebral blood flow (measurement of cerebrovascular reserve) before and after acetylazolamide (Diamox) administration in children with sickle cell anemia. MATERIALS AND METHODS: Thirty-six symptomatic sickle cell disease patients (48 studies) were evaluated. After the injection of either Tc-99m bicisate ethyl cysteinate dimer (ECD) or hexamethyl propylene amine oxime (HMPAO), both whole body scans (with geometric mean correction) and single photon emission computed tomography (CT) were performed pre- and post-Diamox administration with calculation of percentage brain uptake on the whole body images for both examinations and determination of cerebrovascular reserve (percentage change in brain uptake post-Diamox). Evaluation for regional cerebral perfusion change was also performed. RESULTS: The cerebrovascular reserve measurement was 17.6% +/- 43.5% (mean +/- 1 SD). Thirty-three of 48 studies (69%) showed an abnormal cerebrovascular reserve, while only 6 of 48 studies (12.5%) showed Diamox-induced regional perfusion changes in the brain. No statistically significant relationship was found between the occurrence of a regional perfusion abnormality versus loss of cerebrovascular reserve (P = 0.75, Fisher exact test), suggesting that these are independent variables. The cerebrovascular reserve was reproducible, with an average standard deviation of +/-0.54%. CONCLUSION: A new, simple method for calculation of cerebrovascular reserve is presented; this method is reproducible and appears to be an independent variable in the evaluation of cerebrovascular status in sickle cell anemia patients. It should allow further characterization of this complex patient population, and possibly assist in detection of patients at risk for developing "silent" or overt stroke.

Gallium uptake in complicated pancreatitis: a predictor of infection.

OBJECTIVE: A retrospective evaluation was performed of the use of gallium imaging in patients with known severe pancreatitis to detect infection in pancreatic and peripancreatic fluid collections. MATERIALS AND METHODS: Gallium-67 single-photon emission computed tomography (SPECT) studies were retrospectively reviewed in patients with complicated pancreatitis. Only patients who had undergone interventional procedures within 10 days of the scanning were included in our analysis. A total of 23 scans from 20 patients were reviewed. SPECT imaging was typically performed 48-72 hr after injection of the gallium. All studies were correlated with conventional CT findings. Findings from subsequent interventions (results of aspiration, Gram stains, or cultures) were used as evidence of infection. RESULTS: Twenty patients underwent either percutaneous or surgical drainage within 10 days of their gallium scanning. One patient underwent gallium scanning on three different occasions and underwent three different interventional procedures after each of the gallium scans, bringing the total number of cases in our study to 23. Of these 23 cases, 18 patients (78%) with gallium scans showing positive findings for infection had infected fluid; five patients (22%) with negative findings for infection on gallium scans had sterile fluid (p < 0.00001). No false-positive scans were found among our study cases, and we found no correlation between the uptake of gallium and the presence or absence of pancreatic necrosis. CONCLUSION: Gallium does not actively accumulate in all patients with severe pancreatitis, and gallium uptake does not correlate with the presence or absence of necrosis. In patients with severe pancreatitis complicated by fluid collections or inflammatory masses, gallium SPECT is a useful predictor of infection and can be used to help guide subsequent intervention. Gallium SPECT allows targeting sites of infected fluid in patients with multiple fluid collections and potentially obviates intervention in patients with sterile fluid collections.

Dual-radionuclide simultaneous biliary and gastric scintigraphy to depict surgical treatment of bile reflux.

Biliary diversion procedures are performed during gastric surgery to decrease bile reflux. A 1-day dual-radionuclide examination was studied to determine its potential in the evaluation of the effectiveness of the Braun enteroenterostomy in reducing bile reflux and its effects on gastric emptying. Orally ingested gallium 67-labeled egg and intravenously administered technetium 99m diisopropyl-imino-diacetic acid were imaged simultaneously. This provided a way to depict both bile reflux and gastric emptying on the same day in patients who underwent gastric surgery. Overall, the Braun enteroenterostomy trades bile reflux, a symptomatic and premalignant disease, for gastroparesis, a less severe and often treatable disease.