Faculty Bibliography

SMARCA4-deficient thoracic sarcoma (SMARCA4-DTS) is a recently described entity with a poor prognosis that is defined by certain genetic alterations in the BAF chromatin remodeling complex, specifically SMARCA4 and SMARCA2. We present a case of a SMARCA4-DTS in a 59 year-old male with a heavy smoking history who was found to have an unexpected right upper lobe lung mass on routine chest radiograph after a visit to his primary care physician. This led to a biopsy with a diagnosis of poorly differentiated carcinoma at an outside institution. The patient was subsequently seen at our facility for surgical intervention. The right upper lobectomy contained a 7.2-cm poorly differentiated malignancy with slightly discohesive cells arranged in sheets and nests, abundant geographic necrosis, and with many areas showing rhabdoid morphology. The tumor was focally reactive for CK7, AE1/3, Cam5.2, and SALL4 and showed scattered reactivity for CD34 and SOX2. There was complete loss of reactivity for both SMARCA4 and SMARCA2. The histology and immunophenotype were all consistent with the diagnosis of a SMARCA4-DTS. Next-generation sequencing showed a frameshift mutation in the SMARCA4 gene and no abnormality with the SMARCA2 gene. Interestingly, this tumor was confined to the pulmonary parenchyma with no invasion of the visceral pleura nor the mediastinum and with no clinically apparent metastases at the time of presentation. This case is presented to add to the cohort of cases described to date and to discuss the immunohistochemical and molecular findings with regard to SMARCA2.

Patients with acute nonspecific chest pain and low probability for coronary disease remain an important clinical management dilemma. We focus on evidence for imaging, in an integrated decision-making setting. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Pulmonary artery sarcoma is a rare malignancy with poor prognosis which can be misdiagnosed as pulmonary thromboembolism. We present a case of a middle age woman who initially diagnosed with presumptive pulmonary embolism that was later found to have pulmonary artery sarcoma. Symptoms, pathology, imaging characteristics and available treatments are discussed.

Purpose To perform a meta-analysis of the literature to compare the diagnostic performance of fluorine 18 fluorodeoxyglucose PET/CT and diffusion-weighted (DW) MRI in the differentiation of malignant and benign pulmonary nodules and masses. Materials and Methods Published English-language studies on the diagnostic accuracy of PET/CT and/or DW MRI in the characterization of pulmonary lesions were searched in relevant databases through December 2017. The primary focus was on studies in which joint DW MRI and PET/CT were performed in the entire study population, to reduce interstudy heterogeneity. For DW MRI, lesion-to-spinal cord signal intensity ratio and apparent diffusion coefficient were evaluated; for PET/CT, maximum standard uptake value was evaluated. The pooled sensitivities, specificities, diagnostic odds ratios, and areas under the receiver operating characteristic curve (AUCs) for PET/CT and DW MRI were determined along with 95% confidence intervals (CIs). Results Thirty-seven studies met the inclusion criteria, with a total of 4224 participants and 4463 lesions (3090 malignant lesions [69.2%]). In the primary analysis of joint DW MRI and PET/CT studies (n = 6), DW MRI had a pooled sensitivity and specificity of 83% (95% CI: 75%, 89%) and 91% (95% CI: 80%, 96%), respectively, compared with 78% (95% CI: 70%, 84%) (P = .01 vs DW MRI) and 81% (95% CI: 72%, 88%) (P = .056 vs DW MRI) for PET/CT. DW MRI yielded an AUC of 0.93 (95% CI: 0.90, 0.95), versus 0.86 (95% CI: 0.83, 0.89) for PET/CT (P = .001). The diagnostic odds ratio of DW MRI (50 [95% CI: 19, 132]) was superior to that of PET/CT (15 [95% CI: 7, 32]) (P = .006). Conclusion The diagnostic performance of diffusion-weighted MRI is comparable or superior to that of fluorine 18 fluorodeoxyglucose PET/CT in the differentiation of malignant and benign pulmonary lesions. © RSNA, 2018 Online supplemental material is available for this article. See also the editorial by Schiebler in this issue.