ACR Appropriateness Criteria® Imaging of Facial Trauma Following Primary Survey.

Maxillofacial trauma patients comprise a significant subset of patients presenting to emergency departments. Before evaluating for facial trauma, an emergency or trauma physician must perform a primary survey to ensure patient stabilization. Following this primary survey, this document discusses the following clinical scenarios for facial trauma: tenderness to palpation or contusion or edema over frontal bone (suspected frontal bone injury); pain with upper jaw manipulation or pain overlying zygoma or zygomatic deformity or facial elongation or malocclusion or infraorbital nerve paresthesia (suspected midface injury); visible nasal deformity or palpable nasal deformity or tenderness to palpation of the nose or epistaxis (suspected nasal bone injury); and trismus or malocclusion or gingival hemorrhage or mucosal hemorrhage or loose teeth or fractured teeth or displaced teeth (suspected mandibular injury). 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.

A Rare Case of Porocarcinoma and Trichoblastoma Arising in a Nevus Sebaceus of Jadassohn.

Nevus sebaceus of Jadassohn, or "organoid nevus," is a common, benign hamartoma of the skin consisting of epithelial and adnexal components. Its natural history and association with neoplastic growths is well documented. The majority of concomitant neoplasms are benign-trichoblastoma and syringocystadenoma papilliferum are most frequently discovered-but malignant tumors have been described. We present the case of a 58-year-old male with a congenital nevus sebaceus of Jadassohn on his left parietal scalp that had been enlarging, changing color, and bleeding over the prior year. Clinical exam and histology disclosed the presence of a trichoblastoma and porocarcinoma arising within the nevus sebaceus. Porocarcinoma is a rare, intermediately aggressive, malignant eccrine gland tumor that is frequently metastasized at presentation. Otolaryngology performed wide local resection with sentinel lymph node biopsy. This case highlights the diversity of tumors associated with nevus sebaceus of Jadassohn, potential for malignant expansion, and necessity for close monitoring and maintaining a low threshold for biopsy in evolving lesions.

Indications and Outcomes of Mohs Micrographic Surgery Using a Multidisciplinary Approach: A Decade of Experience.

BACKGROUND: Traditional approaches of staged outpatient Mohs Micrographic Surgery (MMS) in nonmelanoma skin cancer (NMSC) followed by reconstruction is not possible in a subset of patients. OBJECTIVE: Assess the indications and outcomes of a multidisciplinary approach MMS. METHODS AND MATERIALS: Retrospective, single-surgeon, single Mohs specialist, university-based tertiary care referral practice, including all MMS performed in the operating room setting with concurrent reconstruction in patients from 2008 to 2018 with minimum follow-up of 6 months. Patients with NMSCs who completed multidisciplinary MMS approach were included. Number of Mohs stages, duration of procedure, reconstruction techniques, and complications including flap loss, bleeding, hematoma, wound infections, dehiscence, and local recurrence rates were reviewed. RESULTS: Three hundred twenty patients were included, 160 male and 160 female with mean ages of 71.6 and 72.1 years, respectively. Indications for a multidisciplinary approach MMS were as follows: neuro/psych 22.5%, extensive anticipated defect size 55%, patient request/convenience 4.4%, medical intolerance 5%, multiple reasons 8.1%, and unknown in 5%. Average stage required to clear margins was 1.57 ± 0.64. Mean operative times by increasing Mohs stages up to 3 including reconstruction were 125.1, 159.3, and 195.5 minutes, respectively (p < .00001). CONCLUSION: Indications for a multidisciplinary approach MMS were extensive defects and neuro/psych issues. Advantages include patient tolerance and single-stage procedure.

Oral Hedgehog Pathway Inhibition as a Means for Ocular Salvage in Locally Advanced Intraorbital Basal Cell Carcinoma.

BACKGROUND: Basal cell cancer is the most common cutaneous malignancy. It rarely presents with locally advanced or metastatic disease. Rare presentations such as intraorbital invasion remain a difficult clinical problem with significant potential morbidity. There is no review of sonic hedgehog pathway inhibitors (HPIs) for intraorbital basal cell cancer, and evidence regarding optimal management is limited. OBJECTIVE: To evaluate the evidence for the management of intraorbital basal cell cancer with HPIs. METHODS: A search to identify evidence for treatment intraorbital basal cell cancers with HPIs to date was performed in PubMed database and OVID using the phrases "basal cell cancer/carcinoma/BCC," "intraorbital," "orbital," "ocular," "periocular," "vismodegib," "GDC-0449," "sonidegib," and "LDE224," in various combinations with Boolean operators "AND" and "OR." RESULTS: Rigorous clinical trials have previously reported the use of vismodegib and sonidegib in locally advanced and metastatic basal cell carcinoma (BCC). However, specific descriptions of treatment of intraorbital tumors are rarely presented in detail adequate for analysis. Twenty-two cases of intraorbital BCC treated with vismodegib have been described in the literature, and no cases using sonidegib were identified. These vary in quality, but highlight important questions regarding optimal treatment duration, follow-up, and adjunctive therapies. Reports describing locally advanced BCC in various facial and periocular locations, but without specific mention of intraorbital invasion, were excluded. CONCLUSION: Vismodegib is an attractive eye and vision-sparing option in patients with locally advanced intraorbital basal cell cancer whose other options often include exenteration, radiation, or other radical surgery.

Desmoplastic Melanoma of the Head and Neck: Incidence and Survival, 1992-2013.

Objective To describe the epidemiological characteristics and survival of desmoplastic melanoma of the head and neck (DMHN) and discuss the factors influencing survival variation among DMHN, DM of other sites (DMnHN), and conventional melanoma of the head and neck (CMHN). Study Design Retrospective cohort study. Setting Surveillance, Epidemiology, and End Results (SEER) database (years 1992-2013). Subjects and Methods Incidence and survival data for 1095 patients with DMHN, 1139 patients with DMnHN, and 40,257 patients with CMHN were obtained. Kaplan-Meier and Cox proportional hazards regression models were used to calculate survival outcomes. Results Patients with DMHN were diagnosed at greater Breslow thickness ( P < .001), stage ( P < .001), and Clark's level ( P < .001) compared to DMnHN and CMHN. Kaplan-Meier survival analysis demonstrated disease-specific survival (DSS) at 5 and 10 years for DMHN to be 80.5% and 74.7%, respectively, compared with 89.1% and 86%, respectively, for DMnHN and 88.1% and 83%, respectively, for CMHN (log-rank test; P < .001). On multivariate Cox regression analysis, age at diagnosis ( P < .001), Breslow depth >4.00 mm ( P = .006), lymph node status ( P < .001), and presence of ulceration ( P < .001) were found to be independent predictors of DSS for DMHN. Conclusion The increasing incidence and poor survivability of DMHN compared to DMnHN and CMHN are parsimoniously explained by the later stage of disease and depth of invasion at diagnosis, highlighting the importance of improved diagnosis and awareness of DMHN.

Reporting of Postoperative Pain Management Protocols in Randomized Clinical Trials of Mandibular Fracture Repair: A Systematic Review.

IMPORTANCE: The control of pain associated with mandibular fractures is an important treatment outcome that affects function, adherence to treatment regimens, and patient comfort and satisfaction. OBJECTIVE: To explore the pain management protocols reported in studies of mandibular fractures, including the reporting of quality-of-life measures. EVIDENCE REVIEW: PubMed/MEDLINE, EMBASE, Cochrane CENTRAL, and clinicaltrials.gov were searched for randomized clinical trials published from 1970 to July 2014. We followed PRISMA reporting standards to assess study eligibility and extract data. Studies of patients older than 16 years who underwent operative mandibular fracture management were included. The primary data collected included the type of analgesic prescribed, associated adverse effects of the analgesic, method of pain assessment, and use of quality-of-life measures. A pain attentiveness score was assigned to studies based on the comprehensiveness of the information reported. Several variables were reviewed to determine the factors that predict reporting of pain-related data. Assessments of risk for bias were performed using the Cochrane Collaboration's domain-based evaluation method. FINDINGS: The initial search identified 111 articles, of which 38 met inclusion criteria. Among the 38 reviewed articles, there were 38 trials and 1808 unique patients represented. Among the 38 articles, the procedures reported included maxillomandibular fixation only in 6 (16%), open reduction with internal fixation only in 20 (53%), and both in 12 (32%). Specific analgesics prescribed were reported in only 5 of the 38 studies (13%), and 3 of these used a combination of nonsteroidal anti-inflammatory drugs and acetaminophen (paracetamol). Thirteen studies (34%) reported pain assessments and 5 (13%) included quality-of-life measures. Geographic region was the only variable that predicted pain attentiveness, with studies from Europe (3 of 11 studies [27%]) and Asia (6 of 16 studies [38%]) most likely to have a high pain attentiveness score. A low rating was least common in the United States (2 of 5 studies [40%]) (P = .047, Fisher exact test). Most of the studies had unclear (n = 27) or high (n = 6) risks for bias in the key domains assessed. CONCLUSIONS AND RELEVANCE: Pain management is a neglected outcome in randomized clinical trials of mandibular trauma; most studies did not describe the specific analgesics used. Many randomized clinical trials (13 [34%]) assessed pain levels among patients without providing information about the agents prescribed. The incorporation of validated pain measures and quality-of-life scores in future studies of mandibular trauma would focus attention on this key outcome measure.

Evidence-Based Facial Fracture Management.

With demands for an evidence-based approach to patient care, the management of facial fractures will come under increasing scrutiny because there is an overall deficiency in higher level clinical evidence. This article reviews the management of facial fractures, focusing on an evidence-based approach. It focuses on select areas of facial trauma in which there is controversy and presents randomized studies and meta-analysis to help define best practice. The article notes the many areas where the evidenced-based literature is weak and looks at the future of evidence-based facial trauma care.

Utility of computed tomography scans in predicting need for surgery in nasal injuries.

In many centers, computed tomography (CT) scan is preferred over plain film radiographs in the setting of acute nasal injury because CT scan is thought to be more sensitive in predicting nasal bone fracture. However, the usefulness of CT scans in predicting the need for surgery in acute nasal injury has not been well-studied. We conducted a retrospective review of 232 patients with known nasal bone fracture and found very similar rates of surgery in patients with a diagnosis of nasal fracture by CT scan as by nasal radiographs (41 and 37%, respectively). This suggests that experienced clinical examination remains the gold standard for determining the need for surgery in isolated nasal trauma, regardless of CT findings.

Early tracheotomy in elderly patients results in less ventilator-associated pneumonia.

OBJECTIVES: To determine if the timing of tracheotomy in elderly patients results in less ventilator associated-pneumonia, mortality, and morbidity. STUDY DESIGN: Historical cohort study. SUBJECTS AND METHODS: This study included 158 ICU patients aged >65 who underwent tracheotomy from March 2003 to June 2007. Patient demographics, outcomes, and ventilation data were collected and analyzed. RESULTS: The early tracheotomy group (continuous intubation time <7 days) included 43 patients, and 115 patients were included in the late group. There were no statistically significant differences in the demographics of the two groups. A statistically significant difference in the rate of ventilator-associated pneumonia was noted in the early versus late tracheotomy group (-0.29% VAP, 95% CI: -0.46, -0.12). There were more intubations per patient noted in the early tracheotomy group versus the late tracheotomy group (0.70 intubations, 95% CI: 0.41, 0.99). The early tracheotomy group has a lower total ICU admission time (-9.5 days, 95% CI: -21.81, -2.25) and total hospital admission time (-10 days, 95% CI: -33.69, -2.249). There was no difference in mortality, although there was a trend of lower mortality in the early tracheotomy group (-11.3% mortality, 95% CI: -0.27, -0.05). CONCLUSION: Early tracheotomy in elderly patients is associated with less ventilator-associated pneumonia, more frequent intubations, less total admission time, and a trend toward lower mortality.

Soft tissue sarcoma in the setting of chronic cutaneous graft versus host disease after allogenic bone marrow transplantation.

Second primary cancers are approximately 2.1-2.8 times more common in survivors of bone marrow transplant than in the age-matched general population. We describe a patient who developed high-grade sarcoma in two disparate sites that were clinically involved by chronic cutaneous graft versus host disease (GVHD). This occurred 3.5 years after bone marrow transplant for acute myelogenous leukemia (AML). This suggests that malignant sarcomas may develop in the setting of chronic GVHD, and close surveillance of GVHD-related nodules is warranted.

Facial fracture repair in the traumatic brain injury patient.

PURPOSE: To review the surgical complications of patients who had facial fractures repairs in the setting of a traumatic brain injury (TBI). PATIENTS AND METHODS: A review of all individuals admitted with the diagnosis of TBI based on an evaluation by the neurotrauma service who also underwent facial fracture repair was performed. More than 600 charts were reviewed and 99 patients met study criteria. Univariate and mulitvariate logistic regression model analysis were performed comparing the complication rate in the immediate postoperative period to the patients' age, gender, mechanism of injury, zone of facial injury, preoperative Glasgow Coma score, presence of multisystem injury, mechanism of TBI and treatment, length of time from injury to surgical repair and length of surgical procedure. RESULTS: Of the 99 individuals studied, there was an 11% complication rate (8 minor, 3 major) in the immediate postoperative period. After univariate analysis, the length of time from injury to surgical repair, zone 1 facial injury and low Glasgow Coma score were all factors associated with increased complications. Multivariate logistic regression model analysis revealed that the odds of a patient sustaining a postoperative complication was 1.298 as the hour of procedure increased by 1 hour (95% CI, 1.065-1.582) and was 1.152 as the days of repair increased by 1 day (95% CI, 1.030-1.288). CONCLUSIONS: The overall complication rate of facial fracture repair in the TBI patient was 11%. A prolonged surgical procedure and delay in surgical repair were associated with higher complication rates as identified by multivariate logistical regression analysis.

Lipoplasty of the face and neck.

PURPOSE OF REVIEW: Facial aesthetic surgery has gained wider acceptance and demand for it is increasing. Patients seeking a more youthful facial look often request lipoplasty. This article reviews the recent advances in lipoplasty and related fat contouring for the face and neck. RECENT FINDINGS: Lipoplasty of the face and neck continues to be popular. There have been improvements in instrumentation, with laser and powered lipoplasty improving the efficiency of fat removal. Lipoplasty indications for neck lipodystrophy have been extended to patients previously only offered neck lifting. Additionally, limited procedures for patients with isolated anterior neck deformities, including direct lipectomy and skin excision, are gaining in popularity. Considerable attention in the lay and professional literature has been paid to mesotherapy for dissolving unwanted fat. Evidence supporting its efficacy is elusive. Finally, there remains enthusiasm for injection fat transfer for facial volume restoration as a component of rejuvenation. SUMMARY: The treatment of lipodystrophy of the face and neck involves the removal of undesirable fat and the transfer of fat to other areas to produce improved aesthetic results. With the current emphasis on restoring volume, lipoplasty and fat transfer will continue to be important in facial plastic surgery.

Treatment of idiopathic sudden sensorineural hearing loss.

CONCLUSION: The therapeutic role of corticosteroids and/or corticosteroids with antiviral agents for sudden sensorineural hearing loss (SSNHL) has yet to be fully elucidated; however, in cases where deafness is profound and of recent onset, a therapeutic trial is indicated. OBJECTIVES: To investigate treatment regimens and their efficacies, as well as evaluating the potential prognostic correlates and allowing comparison between local and national standards of care for SSNHL. PATIENTS AND METHODS: A retrospective evidence-based case series of 143 patients seen at the University of Rochester, Department of Otolaryngology between 1999 and 2002 was investigated. Treatment modalities included (1) observation, (2) steroids, and (3) steroids with antivirals. RESULTS: The study demonstrates that steroid treatment, alone or in combination with antivirals, results in a significant improvement rate compared with observation. Results indicate that the more expediently a patient with SSNHL is seen by an otolaryngologist, the better their prognosis.

Review of nasopharyngeal carcinoma.

We review the literature on nasopharyngeal carcinoma that has been published within the past 5 years. Nasopharyngeal carcinoma is a highly morbid disease, and survival is poor. Its management remains extremely difficult, not just for otolaryngologists but for radiation oncologists and medical oncologists, as well. A clear understanding of its etiology is still lacking, but nasopharyngeal carcinoma is widely suspected to be the result of both a genetic susceptibility and exposure to environmental factors or Epstein-Barr virus infection. With no clear cause, treatment is controversial. For example, an optimal radiation regimen has not been determined, reports in the literature regarding the role of chemotherapy for advanced disease are conflicting, and treatment of local recurrences is unsettled. Still, advances in immunologic research and chemotherapy offer hope for better control of the disease. We hope that our assessment of the recent literature will provide otolaryngologists with a more clear understanding of the etiology and management of nasopharyngeal carcinoma.

Hyaluronan and its receptors in mucoepidermoid carcinoma.

BACKGROUND: Hyaluronan (HA) is a prominent extracellular matrix component undergoing continuous production and degradation. Increased HA levels have been described in a variety of tumors. The objective of this study was to examine the staining patterns of HA and two of its associated receptors (CD44 and HARE) in relation to the metastatic potential of mucoepidermoid carcinoma (MC). Immunohistochemical staining of preserved surgical specimens was used. METHODS: Tissues from 12 patients with a histologic diagnosis of salivary MC (10 parotid, one submandibular gland, one minor salivary gland) were studied. Half (six of 12) of the patients had regional metastases. Tumor, normal salivary tissue, and regional lymph nodes were stained for HA, CD44, and HARE expression. Specimens were graded for staining intensity and a percent of the specimen stained. RESULTS: Normal salivary tissue did not demonstrate epithelial cell surface HA expression, whereas HA was expressed on tumor cells and in regional lymph nodes containing metastases. These differences were both significant using Student's t test (p < .00002, and p < .0022, respectively). Tumors with positive nodes tended to have greater cell surface HA. Decreased expression or downregulation of HARE was also noted in involved lymph nodes. No differences in CD44 expression were seen between primary specimens and lymph nodes. The observed staining patterns for CD44 and HARE were not reflective of the metastatic potential of the primary MC. CONCLUSIONS: Increased HA expression was seen on mucoepidermoid carcinoma cells compared with adjacent normal salivary gland epithelium. This observation may assist in explaining the development of regional metastasis in these tumors. We did not identify specific HA, CD44, or HARE staining patterns in primary lesions that were predictive of regional metastases.

Infectious mononucleosis and corticosteroids: management practices and outcomes.

OBJECTIVE: Although many studies have examined the effects of systemic corticosteroid therapy (SCT) on the clinical course of infectious mononucleosis (IM), few have evaluated the influence of these studies on treatment patterns and outcomes. The purpose of this study was to review current therapeutic strategies and outcomes in uncomplicated and complicated IM. DESIGN: Retrospective case series. SETTING: Tertiary care center. PATIENTS: We identified 206 immunocompetent patients with IM diagnosed during the previous 5 years. Patient information, including age, sex, history and physical findings at presentation, pertinent laboratory data, management practices, and treatment outcomes, were analyzed. INTERVENTIONS: Systemic corticosteroid therapy was used in 44.7% of patients. Evaluation of treatment indications for SCT revealed that 8.0% of the study population qualified by traditional criteria for the use of corticosteroids; 92.0% of patients received SCT for other indications. Factors associated with the observed increase in corticosteroid use included a history of repeat visits, inpatient admission, and otolaryngology consultation. MAIN OUTCOME MEASURES: Diagnosis was made on the basis of a positive heterophil antibody test (monospot test) with appropriate clinical findings (97.5% of patients) or by the presence of lymphocytosis with appropriate clinical findings (2.4% of patients). RESULTS: Systemic corticosteroid therapy was not positively associated with fever, decreased oral intake, tonsillar hypertrophy, or duration of symptoms. No significant differences in incidence of disease complications, rates of hospital admission, or length of hospital stay were noted between the steroid and nonsteroid treatment groups. CONCLUSIONS: Despite consistent and uniform acceptance in the medical literature that SCT in the setting of IM should be reserved for patients with impending airway obstruction, corticosteroids continue to be used on a much broader scale at this tertiary care institution. This observation suggests that clinicians see value in SCT for treatment of IM beyond the classically accepted reasons. Moreover, despite previous reports of possible adverse consequences of SCT in IM, our review failed to demonstrate any such trend.

Craniomaxillofacial fractures during recreational baseball and softball.

PURPOSE: Baseball and softball are leading causes of sports-related facial trauma in the United States. We review our institutional experience (Strong Memorial Hospital, Rochester, NY) with these injuries and discuss measures to reduce their incidence. PATIENTS AND METHODS: We review our institutions experience with facial fractures sustained during the course of a softball or baseball game over a 12-year period. A total of 38 patients were identified and medical records analyzed for patient demographics, type of impact, and fracture location. RESULTS: The male-to-female ratio was 3.2:1; mean age was 24.2 years, with 17 (45%) of the injuries occurring in the pediatric population. The majority of the injuries were caused by direct impact with the ball (68%), while player-player collisions (18%) and impact from a swung bat (13%) were responsible for the remaining injuries. There were a total of 39 fractures; 18 fractures (46%) involved the midface (level 2), skull (level 1) fractures accounted for 12 (31%), while 9 (23%) were mandibular (level 3) fractures. CONCLUSION: With 68% of the injuries resulting from a ball impact, we endorse the recommendations of the Consumer Product Safety Commission for the use of low-impact National Operating Committee on Standards for Athletic Equipment-approved baseballs and softballs for youth and recreational leagues.

Expression of urokinase-type plasminogen activator and its receptor in keloids.

BACKGROUND: The urokinase-mediated plasminogen activation (uPA) system plays a central role in a number of cellular processes including tissue remodeling, cell migration, and angiogenesis. Elevated uPA activity has also been seen with tumor invasion and metastasis in a variety of malignancies. Keloids represent an aberrant form of wound healing characterized by uncontrolled growth with invasion beyond the margins of the original wound. The regulation of this cellular process remains poorly understood. We hypothesize that keloids will have increased staining percentage for uPA and its receptor (uPAR) compared with normal scars. To our knowledge, no previous studies have examined the relationship of uPAR in keloid formation. DESIGN: Analysis of uPAR expression by immunohistochemistry in paraffin sections from 20 keloids and 18 normal scars. Expression was graded by a dermatopathologist according to percentage of cells staining for uPAR. SETTING: University Medical Center (Division of Otolaryngology-Head and Neck Surgery) and the Department of Dermatology at the University of Rochester Medical and Dental School, Rochester, NY. RESULTS: Of the 20 keloids, 8 (40%) strongly expressed uPAR (>50% of cells), while only 4 (22%) of 18 normal scars had similar staining. More than half of the normal scars stained minimally for uPAR (<5% staining). There was a strong expression of uPAR in the extracellular matrix in 14 (70%) of the 20 keloids, while no scar showed uPAR in the extracellular matrix. CONCLUSION: Our observation suggests that the uPA system is involved in the expansion of keloids beyond the wound margins in part through the degradation of the extracellular matrix, a finding that is supported by the strong expression of uPAR in the extracellular matrix and collagenous cords in most keloids studied.

Urokinase-type plasminogen activator receptor expression in adenoid cystic carcinoma of the skull base.

BACKGROUND: Head and neck adenoid cystic carcinoma (ACC) is a malignancy of the salivary and lacrimal glands with a variable growth pattern and propensity for perineural spread. Involvement of the skull base indicates a poor prognosis. Despite surgical resection and adjuvant radiotherapy, tumor recurrence and metastases are common. The urokinase-type plasminogen activator and its receptor (uPAR) have an important role in tumor invasion and metastasis. The expression of uPAR is predictive of poor outcomes in many tumors. This study examines the expression of human uPAR in ACCs involving the skull base. OBJECTIVES: To determine uPAR expression in ACCs of the skull base by immunohistochemical analysis and compare expression with tumor histologic findings and clinical outcomes. STUDY DESIGN: Analysis of uPAR in archival ACC specimens and a retrospective medical chart review. SETTING: Multidisciplinary cranial base program at a university medical center with tertiary referral pattern. RESULTS: Ten (83%) of 12 tumors stained positive for uPAR. Three of 3 patients who died of ACC and 6 of 6 patients alive with disease expressed uPAR. Only 1 of 3 patients free of disease was uPAR positive. CONCLUSIONS: In most patients with ACC of the skull base, uPAR was expressed. Its expression seems to be a negative prognostic factor. However, the small study sample limits our observations. Additional study of uPAR expression in ACC at other anatomic sites is required.

Plasma cell tumors of the skull base: four case reports and literature review.

Four patients (mean age, 46 years; range, 28 to 60) with skull base plasmacytomas are presented along with a review of the literature examining the progression of anterior and central skull base plasma cell tumors to multiple myeloma. The primary sites were the nasopharynx, orbital roof, sphenoid, and clivus. Three patients presented with extensive local spread of tumor. Two patients were diagnosed with multiple myeloma and treated with chemotherapy. Patients with an isolated plasmacytoma were treated with external beam radiation. A partial or complete response to therapy was seen in all four cases. Skull base plasma cell tumors are uncommon with only a limited number of published reports. Previous literature demonstrates that nasopharyngeal extramedullary plasmacytomas have a lower rate of progression to multiple myeloma than other sites. Patients with clivus, sphenoid, and petrous apex-based plasma cell tumors appear to have a higher risk of developing multiple myeloma than patients with plasma cell tumors involving the nasopharynx.