Faculty Bibliography

PURPOSE/OBJECTIVE:The purpose of this study is to measure the performance of restricted diffusion of the pericholecystic hepatic parenchyma for distinguishing between acute and chronic cholecystitis. METHODS:, apparent diffusion coefficient (ADC) maps) were reviewed and graded in a blinded fashion by two abdominal fellowship-trained radiologists for the presence of restricted diffusion in the pericholecystic hepatic parenchyma and the gallbladder wall. Sensitivity, specificity, and inter-observer agreement for individual imaging signs were calculated using surgical pathology as the reference standard for acute cholecystitis. RESULTS:Of the 266 patients, 113 were treated conservatively and 153 underwent cholecystectomy. Restricted diffusion of the pericholecystic hepatic parenchyma showed low sensitivity (reviewer 1, 40%; reviewer 2, 30%) and moderately high specificity (reviewer 1, 84%; reviewer 2, 75%) for distinguishing acute and chronic cholecystitis. Restricted diffusion in the gallbladder wall showed low sensitivity (reviewer 1, 30%; reviewer 2, 7%) and high specificity (reviewer 1, 93%; reviewer 2, 97%) for distinguishing acute and chronic cholecystitis. CONCLUSION/CONCLUSIONS:Diffusion-weighted imaging of the pericholecystic hepatic parenchyma shows moderately high specificity for distinguishing between acute and chronic cholecystitis.

Atypical teratoid/rhabdoid tumor (AT/RT) is a malignant tumor that is commonly associated with biallelic alterations of SMARCB1. Recurrent or refractory AT/RT has not been molecularly characterized as well. We present the case of a child with recurrent AT/RT who underwent clinically integrated molecular profiling (germline DNA and tumor DNA/RNA sequencing). This demonstrated a somatic lesion in CDKN1C alongside hallmark loss of SMARCB1. This data allowed us to explore potential personalized therapies for this patient and expose a molecular driver that may be involved in similar cases.

Background/UNASSIGNED:Surgical resection is the most effective curative therapy for non-small cell lung cancer (NSCLC). However, many patients are unable to tolerate resection secondary to poor reserve or comorbid disease. Radiofrequency ablation (RFA) and microwave ablation (MWA) are methods of percutaneous thermal ablation that can be used to treat medically inoperable patients with NSCLC. We present long-term outcomes following thermal ablation of stage IA NSCLC from a single center. Methods/UNASSIGNED:Patients with stage IA NSCLC and factors precluding resection who underwent RFA or MWA from July 2005 to September 2009 were studied. CT and PET-CT scans were performed at 3 and 6 month intervals, respectively, for first 24 months of follow-up. Factors associated with local progression (LP) and overall survival (OS) were analyzed. Results/UNASSIGNED:. 17 months, P=0.18). Median OS with LP was 32 months compared to 39 months without LP (P=0.68). Three patients underwent repeat ablations. Mean time to LP following repeat ablation was 14.75 months. One patient had two repeat ablations and was disease free at 40-month follow-up. Conclusions/UNASSIGNED:Thermal ablation effectively treated or controlled stage IA NSCLC in medically inoperable patients. Three-year OS exceeded 50%, and LP did not affect OS. Therefore, thermal ablation is a viable option for medically inoperable patients with early stage NSCLC.

CONTEXT/BACKGROUND:- Neoplasms originating in the thalamus are rare overall (1% of all brain tumors); however, they comprise approximately 5% of pediatric intracranial tumors and approach 15% of all malignant pediatric intracranial tumors in some series. OBJECTIVE:- To update readers about the current understanding of the diverse histology, biology, and behavior of pediatric thalamic tumors. Histologic verification is now thought to be critical for planning treatment, and, as a result, biopsy and total/subtotal resections are much more common today than in the past. DATA SOURCES/METHODS:- A PubMed search using the keywords "pediatric + thalamic + glioma" yielded 45 publications with a total of 445 cases of thalamic gliomas in patients less than 18 years of age. We found only 9 substantial institutional series tabulating all encountered thalamic histologic types in children. This survey confirmed a high proportion of astrocytomas, 81% (214 of 265), of which approximately two-thirds were diffuse astrocytomas (146 of 214) and one-third were pilocytic astrocytomas (68 of 214). Of the diffuse astrocytomas, 34% (49 of 146) were low grade (World Health Organization grade II) and 55% (81 of 146) were high grade (World Health Organization grade III or IV), making the latter subgroup the largest single category of all pediatric thalamic tumors. Oligodendrogliomas and ependymomas (mostly anaplastic in both cases) comprised 10% and 3% of all pediatric thalamic tumors, respectively. CONCLUSIONS:- Tissue diagnosis is now thought crucial for prognostication and treatment, particularly as more potentially therapeutic molecular targets are discovered. Secure diagnosis allows identification of tumors for which resection is more feasible and beneficial.

Immune reconstitution inflammatory syndrome (IRIS) is a common complication during treatment for natalizumab-associated progressive multifocal leukoencephalopathy (PML). Although severe IRIS can result in acute worsening of disability and is associated with poor prognosis, effective immune reconstitution may account for the high survival rate of this cohort of PML patients. We present pathological evidence of chronic IRIS 3.5 years after diagnosis with natalizumab-associated PML. Our case showed that the IRIS initially developed after plasma exchange therapy and resolved clinically and radiologically following a combination treatment with corticosteroids, maraviroc, and cidofovir. Autopsy 3.5 years later revealed evidence of grey-white matter junction demyelinating lesions characteristic of PML and perivascular leukocyte infiltrates predominated by CD8⁺ T-lymphocytes, and polymerase chain reaction analysis demonstrated the presence of JC viral DNA in this tissue, indicative of persistent PML-IRIS. While clinical symptoms of PML-IRIS typically stabilize within 6 months, our case report suggests that prolonged low-grade inflammation may persist in some patients. Better assays are needed to determine the prevalence of prolonged low-grade IRIS among PML survivors.

Amyloid mesenteric angiopathy is difficult to diagnose as gastrointestinal (GI) signs are nonspecific and radiological studies rarely identify amyloid deposits with certainty. The objective of this study was to highlight the pathological findings of amyloid mesenteric angiopathy in cases of senile systemic amyloidosis. Literature review, author's research data, and practice experience were used. Systemic amyloidosis should be considered in the differential diagnoses when evaluating nonspecific and unexplained GI in patient population aged> 55 years to decrease the morbidity and fatal outcomes.

The elongation or calcification of the stylohyoid ligament that leads to pressure symptoms, or entrapment of nearby glossopharyngeal nerve or carotid artery, is known as Eagle syndrome. A PubMed search leads to finding of rare fatality among the 49 reported cases. In the present case, the deceased was a 40-year-old male who choked on his food. We hypothesise that the impaction of food in the upper respiratory tract, as well as the inability to intubate the person, were both the result of the calcified stylohyoid ligament.