John P. Marinelli

Quantitative assessment of lung stiffness in patients with interstitial lung disease using MR elastography

To investigate the use of magnetic resonance elastography (MRE) in the quantitative assessment of pulmonary fibrosis by comparing quantitative shear stiffness measurements of lung parenchyma in patients diagnosed with fibrotic interstitial lung disease (ILD) and healthy controls.

Volumetric analysis of olfactory neuroblastoma skull base laterality and implications on neck disease

To determine if the laterality of primary tumors in patients with olfactory neuroblastoma (ONB) influenced the pattern and development of neck disease.

Dural Invasion Predicts the Laterality and Development of Neck Metastases in Esthesioneuroblastoma

Objectives Neck metastases in patients with esthesioneuroblastoma (ENB) constitute the most significant predictor of poor long‐term survival. Recently, researchers discovered the existence of dural lymphatic channels that drain to the cervical lymph nodes. From this physiologic basis, we hypothesized that patients with ENB who develop dural invasion (DI) would exhibit a proclivity for neck metastases. Design Retrospective review. Setting Tertiary referral center. Participants All patients treated for ENB from January 1, 1994 to December 31, 2015. Main Outcome Measures Incidence, laterality, and recurrence rate of neck metastases by DI status. Results Sixty‐one patients were identified (38% female; median age 49, range, 10‐80), 34 (56%) of whom had DI and 27 (44%) did not. Of patients with DI, 50% presented with or developed neck disease following treatment compared with just 22% of those without DI (p = 0.026). Bilateral neck disease was more common in patients with DI (11/34, 32%) compared with those without (2/27, 7%) (p = 0.018). Five‐year regional recurrence‐free survival rates were 88% for those without and 64% for those with DI (p = 0.022). Kadish C patients with DI were more likely to develop regional recurrence when compared with Kadish C without DI and Kadish A/B (p = 0.083). Further, Kadish C patients with DI displayed worse overall survival than Kadish C without DI and Kadish A/B. Kadish D patients displayed the worst overall survival. The difference in overall survival among these four groups was significant (p < 0.001). Conclusion DI by ENB is associated with increased incidence of cervical nodal metastases, bilateral neck disease, worse regional recurrence‐free survival, and poorer overall survival. These data support the division of Kadish C by DI status.

Primary Skull Base Lymphoma: Manifestations and Clinical Outcomes of a Great Imitator

Objectives Primary skull base lymphoma (PSBL) represents a rare manifestation of extranodal lymphoma. Presenting with nonspecific symptomatology and imaging findings, PSBL often masquerades as more common cranial base pathology and thus can present a diagnostic challenge. The objectives of this study were to characterize the manifestations and clinical outcomes of PSBL. Study Design Case series and chart review. Setting Tertiary referral center. Subjects and Methods Review of 48 patients with PSBL treated between 1994 and 2016. Results The median age at diagnosis was 60 years. Diffuse large B-cell lymphoma comprised the most common subtype (56%). Presenting symptoms included diplopia (52%), trigeminal hypesthesia (38%), headache (29%), facial nerve weakness (25%), B-symptoms (fevers, night sweats, and/or weight loss) (25%), and hearing loss (21%). Discrete lesions commonly mimicked meningioma, schwannoma, and nasopharyngeal carcinoma. Diffuse lesions imitated neurosarcoidosis, granulomatosis with polyangiitis, and carcinomatosis meningitis. Only 26% of all initial clinical evaluations suspected lymphoma. The combination of restricted diffusion on diffusion-weighted imaging, an absence of flow voids or surrounding hyperostosis, bony erosion and/or marrow signal replacement, and heterogeneous contrast enhancement facilitated delineation between PSBL and common skull base pathology. Cerebrospinal fluid (CSF) analysis was diagnostic of lymphoma in 24% of cases. Lymphoma within CSF portended significantly worse overall survival (85% vs 18% at 3-years; P < .001). Conclusion A history significant for multiple cranial nerve palsies, B-symptoms, and imaging findings inconsistent with common skull base pathology should raise suspicion for PSBL. CSF analysis in the setting of PSBL can provide diagnostic and prognostic value for patients.

Incidence of Vestibular Schwannoma over the Past Half-Century: A Population-Based Study of Olmsted County, Minnesota

Objective The absence of a centralized health system has limited epidemiologic research surrounding vestibular schwannoma (VS) in the United States. The Rochester Epidemiology Project (REP) comprises a unique medical consortium that covers a complete population of all ages in a well-defined geographic region over the past half-century. The objective of this study was to characterize the incidence of sporadic VS over this extended period. Study Design Population-based study. Setting Olmsted County, Minnesota. Subjects and Methods Review of all VSs diagnosed between January 1, 1966, and December 31, 2016, was conducted with the REP medical records linkage system. Results A total of 153 incident cases of VS were identified. The incidence of VS significantly increased over the past half-century from 1.5 per 100,000 person-years during the first decade to 4.2 in the last decade (P < .001). Incidence increased with age (P < .001): those aged ≥70 years exhibited the highest incidence rate at 18.3 per 100,000 person-years in the last decade. Age at diagnosis significantly increased from a median of 52 years in the first decade to 62 years in the last (P < .001). Despite presenting with fewer symptoms and smaller tumors, the time delay between symptom onset and diagnosis significantly decreased over the past 5 decades (all P < .05). Almost 1 in 4 patients was diagnosed incidentally in the last decade. Conclusion The incidence of VS increased significantly over the past half-century to a rate greater than previously reported. Patients’ ease of access to medical care in Olmsted County and the comprehensive REP system likely contributed to this elevated detection rate of VS.

Esthesioneuroblastoma with distant metastases: Systematic review & meta-analysis

The purpose of this study was to determine the clinical outcomes and review the management strategies for metastatic esthesioneuroblastoma.