Phosphaturic Mesenchymal Tumors of the Sinonasal Area and Skull Base: Experience at a Single Institution

Objectives: Phosphaturic mesenchymal tumor (PMT) is a rare, polymorphous neoplasm with a highly variable presentation and natural history and unpredictable clinical course. The primary objective was to describe our clinical experience with and management of 4 markedly different cases of sinonasal and skull base PMT. Methods: A retrospective case series with chart review, and relevant literature review, was performed at a tertiary academic medical center between 1998 and 2020. Adult patients treated for PMTs of the sinonasal area and skull base were included. Our main outcome measures included postoperative laboratory findings and radiological evidence of disease remission. Results: Four patients (2 Males, 2 Females; Mean Age: 63.5 years) with PMTs of the skull base have been managed at our institution since 1998. Patient presentations varied, ranging from severe phosphorus wasting and osteoporosis to symptoms secondary to mass effect, including nasal obstruction and rhinorrhea. All 4 patients were eventually found to have elevated levels of fibroblast growth factor 23. Tumors were located in the sinonasal area (right frontal sinus, right ethmoid sinus, and right nasal cavity, respectively) in 3 patients and in the lateral skull base (right jugular foramen) in 1 patient. All 4 patients underwent complete surgical resection of their tumors. PMT tissue pathology was confirmed in all cases. Gross total resection was achieved in all patients. There was no chemical or radiological evidence of disease recurrence in any patients at follow-up. Conclusions: The presentation of skull base PMT is variable, and it may mimic other mass pathologies of the head and neck. Complete surgical resection with negative margins is potentially curative.

Download Full-text

Multi-Disciplinary Skull Base Conference and its Effects on Patient Management

Annals of Otology Rhinology & Laryngology ◽

10.1177/00034894211021251 ◽

2021 ◽

pp. 000348942110212

Author(s):

Nathan Kemper ◽

Scott B. Shapiro ◽

Allie Mains ◽

Noga Lipschitz ◽

Joseph Breen ◽

...

Keyword(s):

Skull Base ◽

Medical Center ◽

Academic Medical Center ◽

Significant Proportion ◽

Internal Auditory Canal ◽

Patient Characteristics ◽

Management Plan ◽

Case Review ◽

Retrospective Case ◽

Lateral Skull Base

Objective: Examine the effects of a multi-disciplinary skull base conference (MDSBC) on the management of patients seen for skull base pathology in a neurotology clinic. Methods: Retrospective case review of patients who were seen in a neurotology clinic at a tertiary academic medical center for pathology of the lateral skull base and were discussed at an MDSBC between July 2019 and February 2020. Patient characteristics, nature of the skull base pathology, and pre- and post-MDSBC plan of care was categorized. Results: A total of 82 patients with pathology of the lateral skull base were discussed at a MDSBC during an 8-month study period. About 54 (65.9%) had a mass in the internal auditory canal and/or cerebellopontine angle while 28 (34.1%) had other pathology of the lateral skull base. Forty-nine (59.8%) were new patients and 33 (40.2%) were established. The management plan changed in 11 (13.4%, 7.4-22.6 95% CI) patients as a result of the skull base conference discussion. The planned management changed from some form of treatment to observation in 4 patients, and changed from observation to some form of treatment in 4 patients. For 3 patients who underwent surgery, the planned approach was altered. Conclusions: For a significant proportion of patients with pathology of the lateral skull base, the management plan changed as a result of discussion at an MDSBC. Although participants of a MDSBC would agree of its importance, it is unclear how an MDSBC affects patient outcomes.

Download Full-text

Imaging and Outcomes for a New Entity: Low-Grade Sinonasal Sarcoma with Neural and Myogenic Features

Journal of Neurological Surgery Reports ◽

10.1055/s-0037-1598198 ◽

2017 ◽

Vol 78 (01) ◽

pp. e15-e19 ◽

Cited By ~ 5

Author(s):

Richard Cannon ◽

Richard Wiggins ◽

Benjamin Witt ◽

Yusuf Dundar ◽

Tawni Johnston ◽

...

Keyword(s):

Skull Base ◽

Surgical Outcomes ◽

Medical Center ◽

Academic Medical Center ◽

Case Series ◽

Intracranial Extension ◽

Low Grade ◽

Skull Base Tumor ◽

Imaging Characteristics ◽

Sinonasal Mass

Objectives Low-grade sinonasal sarcoma with neural and myogenic features (LGSSNMF) is a new, rare tumor. Our goal is to describe the imaging characteristics and surgical outcomes of this unique skull base malignancy. Design Retrospective case series. Setting Academic medical center. Participants There were three patients who met inclusion criteria with a confirmed LGSSNMF. Main Outcome Measures Imaging and histopathological characteristics, treatments, survival and recurrence outcomes, complications, morbidity, and mortality. Results Patients presented with diplopia, facial discomfort, a supraorbital mass, and nasal obstruction. Magnetic resonance imaging and computed tomography imaging in all cases showed an enhancing sinonasal mass with associated hyperostotic bone formation that involved the frontal sinus, invaded the lamina papyracea and anterior skull base, and had intracranial extension. One patient underwent a purely endoscopic surgical resection and the second underwent a craniofacial resection, while the last is pending treatment. All patients recovered well, without morbidity or long-term complications, and are currently without evidence of disease (mean follow-up of 2.1 years). One patient recurred after 17 months and underwent a repeat endoscopic skull base and dural resection. Conclusions The surgical outcomes and imaging of this unique, locally aggressive skull base tumor are characterized.

Download Full-text

Surgical Treatment of Sinonasal Mucosal Melanoma in Patients Treated with Systemic Immunotherapy

Journal of Neurological Surgery Part B Skull Base ◽

10.1055/s-0040-1701219 ◽

2020 ◽

Author(s):

Tiffany N. Chao ◽

Edward C. Kuan ◽

Charles C. L. Tong ◽

Michael A. Kohanski ◽

M. Sean Grady ◽

...

Keyword(s):

Surgical Resection ◽

Metastatic Disease ◽

Initial Treatment ◽

Medical Center ◽

Academic Medical Center ◽

Case Series ◽

Mucosal Melanoma ◽

Primary Site ◽

Definitive Treatment ◽

Systemic Immunotherapy

Abstract Objectives Surgical resection is widely accepted as a critical component for definitive treatment of sinonasal mucosal melanoma. Systemic immunotherapy, including multiple newer agents, has been used to treat metastatic or unresectable disease. In this study, we examine its efficacy in locoregional control when used in conjunction with surgical resection for primary mucosal lesions. Design Present study is a retrospective review of all patients at a tertiary academic medical center with primary sinonasal mucosal melanoma and distant metastatic disease. Results A total of four patients were identified. In all cases, patients were treated with a combination of surgical resection of the primary tumor and systemic immunotherapy. Three patients were initially treated with surgery at the primary site followed by immunotherapy for distant metastases. Response to immunotherapy at the sites of primary and metastatic disease was seen in two patients. All four patients developed progression or recurrence at the primary site following initiation of immunotherapy for which they underwent surgical resection. One patient remains in follow-up without evidence of disease 20 months after initial treatment; three succumbed to the disease at 135, 37, and 16 months after initial treatment. Conclusion Surgical resection for local control plays a critically important role in the treatment of sinonasal mucosal melanoma regardless of the presence of metastases and whether immunotherapy will be given. This case series suggests that, though immunotherapy may demonstrate efficacy in managing distant disease, surgery should remain the first-line treatment for the primary site.

Download Full-text

Multicranial neuropathy secondary to endolymphatic sac tumor

International Journal of Otorhinolaryngology and Head and Neck Surgery ◽

10.18203/issn.2454-5929.ijohns20213657 ◽

2021 ◽

Author(s):

Tanvir Hussain ◽

Thomas J. Crotty ◽

Eoin F. Cleere ◽

Mel Corbett ◽

Aishan Patil ◽

...

Keyword(s):

Skull Base ◽

Surgical Resection ◽

Cranial Nerves ◽

Endolymphatic Sac ◽

Endolymphatic Sac Tumor ◽

Von Hippel Lindau ◽

Lateral Skull Base ◽

Complete Surgical Resection ◽

Close Proximity ◽

History Of

<p class="abstract">Endolymphatic sac tumors (ELST) are rare neuroectodermal neoplasms arising from the epithelium of the endolymphatic sac or duct. Despite their benign histopathological features, ELSTs frequently demonstrate a locally destructive growth pattern with involvement of the skull base and cranial nerves. While ELSTs may arise sporadically, the majority of cases occur in association with Von Hippel-Lindau (VHL) syndrome. ELSTs are commonly diagnosed late due to their slow growing nature and non-specific symptomatology. Surgical resection is the treatment of choice. However, due to the location of these tumors in the lateral skull base surgical intervention carries a high risk of injury to critical neurovascular structures in close proximity. We presented the case of a 51 years old gentleman with a three-months history of hearing loss and otalgia. He subsequently developed multiple cranial neuropathies and was diagnosed with a sporadic ELST. He underwent a complete surgical resection and received adjuvant radiotherapy. </p>

Download Full-text

Characteristics and Outcomes Among Hospitalized COVID-19-Positive Patients in a Nonurban Environment

Military Medicine ◽

10.1093/milmed/usab044 ◽

2021 ◽

Author(s):

Robert P Lennon ◽

Theodore J Demetriou ◽

M Fahad Khalid ◽

Lauren Jodi Van Scoy ◽

Erin L Miller ◽

...

Keyword(s):

New York ◽

Medical Center ◽

Academic Medical Center ◽

Military Medicine ◽

Case Series ◽

Outcome Data ◽

Urban Environments ◽

Urban Setting ◽

Published Data ◽

Retrospective Case

ABSTRACT Introduction Virtually all hospitalized coronavirus disease-2019 (COVID-19) outcome data come from urban environments. The extent to which these findings are generalizable to other settings is unknown. Coronavirus disease-2019 data from large, urban settings may be particularly difficult to apply in military medicine, where practice environments are often semi-urban, rural, or austere. The purpose of this study is compare presenting characteristics and outcomes of U.S. patients with COVID-19 in a nonurban setting to similar patients in an urban setting. Materials and Methods This is a retrospective case series of adults with laboratory-confirmed COVID-19 infection who were admitted to Hershey Medical Center (HMC), a 548-bed tertiary academic medical center in central Pennsylvania serving semi-urban and rural populations, from March 23, 2020, to April 20, 2020 (the first month of COVID-19 admissions at HMC). Patients and outcomes of this cohort were compared to published data on a cohort of similar patients from the New York City (NYC) area. Results The cohorts had similar age, gender, comorbidities, need for intensive care or mechanical ventilation, and most vital sign and laboratory studies. The NYC’s cohort had shorter hospital stays (4.1 versus 7.2 days, P < .001) but more African American patients (23% versus 12%, P = .02) and higher prevalence of abnormal alanine (>60U/L; 39.0% versus 5.9%, P < .001) and aspartate (>40U/L; 58.4% versus 42.4%, P = .012) aminotransferase, oxygen saturation <90% (20.4% versus 7.2%, P = .004), and mortality (21% versus 1.4%, P < .001). Conclusions Hospitalists in nonurban environments would be prudent to use caution when considering the generalizability of results from dissimilar regions. Further investigation is needed to explore the possibility of reproducible causative systemic elements that may help improve COVID-19-related outcomes. Broader reports of these relationships across many settings will offer military medical planners greater ability to consider outcomes most relevant to their unique settings when considering COVID-19 planning.

Download Full-text

Perioperative outcomes for patients on chronic buprenorphine undergoing spine surgery: A case series

Journal of Opioid Management ◽

10.5055/jom.2021.0655 ◽

2021 ◽

Vol 17 (7) ◽

pp. 171-177

Author(s):

Ashley L. Sharp, MD ◽

Stephanie Gilbert, MD ◽

Danielle N. Perez, MD ◽

Kerstin Kolodzie, MD, PhD, MAS ◽

Matthias Behrends, MD

Keyword(s):

Pain Management ◽

Outcome Measures ◽

Spine Surgery ◽

Elective Surgery ◽

Medical Center ◽

Academic Medical Center ◽

Case Series ◽

Perioperative Outcomes ◽

Perioperative Pain ◽

Perioperative Pain Management

Objective: Pain management following spine surgery can be challenging as patients routinely suffer from chronic pain and opioid tolerance. The increasing popularity of buprenorphine use for pain management in this population may further complicate perioperative pain management due to the limited efficacy of other opioids in the presence of buprenorphine. This study describes perioperative management and outcomes in patients on chronic buprenorphine who underwent elective inpatient spine surgery.Design: The authors performed a retrospective chart review of all patients 18 years of age taking chronic buprenorphine for any indication who had elective inpatient spine surgery at a single institution. Perioperative pain management data were analyzed for all patients who underwent spine surgery and were maintained on buprenorphine during their hospital stay.Setting: The study was performed at a single tertiary academic medical center. Main outcome measures: The primary outcome measures were post-operative pain scores and analgesic medication requirements.Results: Twelve patients on buprenorphine underwent inpatient spine surgery. Acceptable pain control was achieved in all cases. Management included preoperative dose limitation of buprenorphine when indicated and the extensive use of multimodal analgesia.Conclusion: The question whether patients presenting for painful, elective surgery should continue using buprenorphine perioperatively is an area of controversy, and the present manuscript provides more evidence for the concept of therapy continuation with buprenorphine.

Download Full-text

Growth and Management of Repaired Complete Tracheal Rings after Slide Tracheoplasty

Otolaryngology ◽

10.1177/0194599819841893 ◽

2019 ◽

Vol 161 (1) ◽

pp. 164-170 ◽

Cited By ~ 5

Author(s):

Lyndy J. Wilcox ◽

Claudia Schweiger ◽

Catherine K. Hart ◽

Alessandro de Alarcon ◽

Nithin S. Peddireddy ◽

...

Keyword(s):

Medical Center ◽

Academic Medical Center ◽

Balloon Dilation ◽

Case Series ◽

Slide Tracheoplasty ◽

Time Period ◽

Academic Medical ◽

Operative Notes ◽

Over Time

ObjectiveThis study documents the growth and course of repaired complete tracheal rings over time after slide tracheoplasty.Study DesignCase series with review.SettingTertiary pediatric academic medical center.Subjects/MethodsMedical records of pediatric patients with confirmed tracheal rings on bronchoscopy who underwent slide tracheoplasty between January 2001 and December 2015 were reviewed. Patients who had operative notes documenting tracheal sizing over time were included. Exclusion criteria included tracheal stenosis not caused by complete tracheal rings, surgical repair prior to presentation at our institution, or lack of adequate sizing information. The postoperative follow-up was examined and airway growth over time documented.ResultsOf 197 slide tracheoplasties performed during the study time period, 139 were for complete tracheal rings, and 40 of those children met inclusion criteria. The median age at time of surgery was 7 months, and the median initial airway size was 3.9 mm (n = 34). The median growth postoperatively was 1.9 mm over a median follow-up period of 57 months (0.42 mm/year), which is similar to growth rates of unrepaired complete tracheal rings ( P = .53). Children underwent a median of 10 postoperative endoscopies, with time between endoscopies increasing further out from surgery. The most commonly performed adjunctive procedure was balloon dilation.ConclusionsThis is the first study documenting continued growth of repaired complete tracheal rings after slide tracheoplasty. Postoperative endoscopic surveillance ensures adequate growth. Intervals between airway endoscopies can be increased as the child gets older, as the airway increases in size, and as long as symptoms are minimal.

Download Full-text

Management and Surveillance of Frontal Sinus Violation following Craniotomy

Journal of Neurological Surgery Part B Skull Base ◽

10.1055/s-0038-1676826 ◽

2019 ◽

Vol 81 (01) ◽

pp. 001-007 ◽

Cited By ~ 1

Author(s):

Alexander Farag ◽

Marc R. Rosen ◽

Natalie Ziegler ◽

Ryan A. Rimmer ◽

James J. Evans ◽

...

Keyword(s):

Literature Review ◽

Frontal Sinus ◽

Medical Center ◽

Academic Medical Center ◽

Case Series ◽

Endoscopic Endonasal Approach ◽

Endonasal Approach ◽

Endoscopic Endonasal ◽

Incomplete Removal ◽

Intraoperative Management

Objectives In the setting of craniotomy, complications after traversing the frontal sinus can lead to mucocele formation and frontal sinusitis. We review the etiology of frontal sinus violation, timeline to mucocele development, intraoperative management of the violated sinus, and treatment of frontal mucoceles. Design Case series in conjunction with a literature review. Participants A total of 35 patients were included in this meta-analysis. Nine of these patients were treated at a tertiary academic medical center between 2005 and 2014. The remaining patients were identified through a literature review for which 2,763 articles were identified, of which 4 articles met inclusion criteria. Main Outcomes Measures Etiology of frontal violation, timeline to mucocele development, and method of management. Results The overall interval from initial frontal sinus violation until mucocele identification was 14.5 years, with a range of 3 months to 36 years. The most common cause of mucocele formation was obstruction of the frontal recess with incomplete removal of the frontal sinus mucosa. The majority of patients were successfully managed with an endoscopic endonasal approach. Conclusions Violation of the frontal sinus during craniotomy can result in mucocele formation as an early or late sequela. Image guidance may help avoid unnecessary frontal sinus violation. Mucoceles may develop decades after the initial frontal sinus violation, and long-term follow-up with imaging is recommended. While the endoscopic endonasal approach is usually the preferred method to treat these lesions, it may be necessary to perform obliteration or cranialization in unique situations.

Download Full-text