Lal, Devyani

We report a case of sinonasal phosphaturic mesenchymal tumor (PMT) and conduct a systematic review of the literature to highlight a unique paraneoplastic syndrome associated with PMT. We used English language publications from Medline and Cochrane databases (1970–2013) as data sources. A systematic review of the literature was conducted. All reported cases of head and neck PMTs were included. The presence or absence of the associated paraneoplastic syndrome was noted. We found 33 cases of PMT in the head and neck reported in the literature, 17 of which occurred in the sinonasal area. Approximately 5% of all PMTs are located in the head and neck. Just greater than half are concentrated in the sinonasal area, and the remaining involve various bony and soft tissue structures of the head and neck. PMT is sometimes associated with a paraneoplastic syndrome of tumor-induced (oncogenic) osteomalacia (TIO) causing bone pain, muscle weakness, and pathologic fractures. We present the 18th reported case of sinonasal PMT. A smooth mucosa-covered midline intraseptal mass filling the posterior nasal cavity with destruction and erosion of the skull base was found in an adult male. The patient underwent successful endoscopic resection with wide negative margins and is without recurrence at 24-month follow-up. PMT is a benign, locally aggressive tumor with rare malignant transformation. Knowledge of the bony invasion and destruction caused by this tumor is essential in planning surgical resection with wide negative margins. Familiarity with the associated TIO is essential to investigate for and manage any associated bony morbidity.

SummaryBackgroundThe mechanisms and immune pathways associated with chronic rhinosinusitis (CRS) are not fully understood. Immunological changes during acute exacerbation of CRS may provide valuable clues to the pathogenesis and perpetuation of the disease.ObjectiveTo characterize local and systemic immune responses associated with acute worsening of sinonasal symptoms during exacerbation in CRS with nasal polyps (CRSwNP) compared to controls.MethodsThis was a non‐interventional prospective study of individuals with CRSwNP and normal controls. Subjects underwent a baseline visit with collection of nasal secretions, nasal washes, and serum specimens. Within 3 days of acute worsening of sinonasal symptoms, subjects underwent a study visit, followed by a post‐visit 2 weeks later. The sinonasal outcome test‐22 (SNOT‐22) scores and immunological parameters in the specimens were analysed using a novel, unsupervised learning method and by conventional univariate analysis.ResultsBoth CRSwNP patients and control subjects showed a significant increase in SNOT‐22 scores during acute exacerbation. Increased nasal levels of IL‐6, IL‐5, and eosinophil major basic protein were observed in CRSwNP patients. A network analysis of serum specimens revealed changes in a set of immunological parameters, which are distinctly associated with CRSwNP but not with controls. In particular, systemic increases in VEGF and GM‐CSF levels were notable and were validated by a conventional analysis.ConclusionsCRSwNP patients demonstrate distinct immunological changes locally and systemically during acute exacerbation. Growth factors VEGF and GM‐CSF may be involved in the immunopathogenesis of subjects with CRS and nasal polyps experiencing exacerbation.

Objectives/HypothesisTo study differential diagnosis and efficacy of management strategies in patients presenting to an otolaryngologist for sinus pressure, pain, or headache.Study DesignRetrospective analysis at an academic medical center.MethodsPatients were seen in the clinic (2010–2012) for sinus‐related headache, pressure, pain or fullness (study symptoms) by a rhinologist. A retrospective chart review of patients with study symptoms was conducted.ResultsOf 211 patients with study symptoms, 70.62% met American Academy of Otolaryngology–Head and Neck Surgery criteria for sinusitis or had rhinologic disease. Otolaryngic therapy alone (medical or surgical) relieved study symptoms in 51.66%; combined neurology intervention helped another 15.17%. Nearly half of the patients (48.82%) were diagnosed with primary headache disorders. Comorbid rhinologic‐neurologic disease was present in 27.96% and odontogenic disease in 7%. Initial otolaryngology referral was likely unnecessary for 36.49% of the study patients. Sinus computed tomography (CT) was available for 91% of 211 patients, and 80% of scans were positive. Endoscopic sinus surgery (ESS) was used in only 80/211 patients (37.69%) and was effective in 66/211 (31.28%). ESS was most successful in patients receiving concurrent neurological intervention. The Lund‐Mackay CT score did not predict outcomes from ESS. Interdisciplinary otolaryngology‐neurology efforts resulted in a positive outcome for 92.4% of patients.ConclusionsWe present the first series detailing management of patients with sinus‐headache pain in an otolaryngology practice. Such symptoms have multifactorial etiologies. Positive sinus CT results require cautious interpretation. ESS should be judiciously used. Interdisciplinary care is critical for success: approximately 50% of patients benefited from otolaryngic management, 50% needed neurological treatment, and 7% required dental disease management.Level of Evidence4 Laryngoscope, 125:303–310, 2015

ObjectiveIgG4‐related disease (IgG4RD) causing sinonasal and skull base pathology is uncommonly described. We present a series of suspected IgG4RD patients, with a pertinent review of the literature to highlight diagnostic challenges.Study DesignCase series.SettingAcademic tertiary care center.Subjects and MethodsCase series of patients with IgG4RD or suspected IgG4RD involving the sinonasal cavity and skull base.ResultsWe present 4 patients with atypical sinonasal and/or skull base disease who were noted to have IgG4‐positive plasma cell infiltration on immunohistochemistry of biopsy specimens. IgG4RD, a recently described entity affecting multiple organs, is characterized by lymphoplasmacytic infiltration and often elevated serum IgG4. IgG4RD can masquerade as malignancy or infection but responds to glucocorticosteroid and immunosuppressant therapy. IgG4RD has been infrequently reported presenting as sinonasal or skull base lesions, and definitive diagnostic criteria for these regions are not established. In our series, IgG4RD was suspected in all 4 patients, but only 1 met all current criteria for definitive diagnosis. All 4 patients, however, responded to corticosteroid therapy, and 1 was placed on long‐term azathioprine.ConclusionIgG4RD is rarely described in the sinonasal cavity and skull base, and specific diagnostic criteria for such disease have not been defined. We present a series of patients with IgG4‐positive plasma cell inflammatory pathology who were suspected to have IgG4RD. Our series highlights diagnostic challenges associated with these patients. Tumefactive and destructive sinonasal‐skull base lesions with a plasma cell‐rich infiltrate should incite suspicion of IgG4RD, and immunohistochemistry for IgG4‐positive plasma cells should be performed.

BackgroundWe report the first report of an expansile crista galli (CG) mucocele treated surgically by an endoscopic endonasal approach. Only 1 other case of a CG mucocele has been previously reported in the literature. This was treated by a craniotomy approach.Case ReportWe describe the technique employed in endoscopic marsupialization. We also discuss relevant CG anatomy, pneumatization patterns, surgical approaches, and its potential to cause disease, including CG sinusitis and mucocele formation.ConclusionWe present this case to highlight that the growing experience with endoscopic techniques offer us less morbid and more functional alternatives to a variety of lesions that were once tackled by neurosurgical or external approaches. In the contemporary era, the indications for open approaches and craniotomy for frontal sinus and CG lesions is likely limited. We recommend these patients undergo careful evaluation by a surgeon experienced in advanced endoscopic techniques before being advised to undergo open or craniotomy techniques.