Reconstruction with Mucosal Graft Reduces Recurrence After Endoscopic Surgery of Rathke Cleft Cyst.

OBJECTIVES: Rathke cleft cysts (RCCs) arise from the development of the Rathke pouch. Recurrence is common after either drainage or cyst removal. The endoscopic endonasal approach (EEA) is increasingly utilized for the management of RCC. Various techniques have been described to try to reduce the rates of recurrence. We studied the effect of fenestration with a nasoseptal flap (NSF) on recurrence rates by comparing a cohort of patients undergoing this technique to a cohort of patients undergoing conventional drainage. METHODS: Patients who underwent EEA for RCC between 2011 and 2020 were identified and divided into 2 cohorts: conventional fenestration versus fenestration with NSF. Surgical approach, reconstructive method, and recurrences were recorded. Primary end point was symptomatic or radiographic recurrence. RESULTS: 21 patients were identified undergoing EEA. An NSF was used to line the cyst cavity in 11 cases. Conventional fenestration without mucosal reconstruction was performed in the remaining 10 cases. In the cases without NSF, 5 (50%) developed recurrence requiring revision surgery, while there was only one recurrence in the NSF group (P < 0.05). In patients requiring revision, all had an NSF placed and none had a second recurrence of their RCC. CONCLUSIONS: NSF placement into a fenestrated RCC is useful to prevent cyst reaccumulation and reoperation. Typical fenestration carries an unacceptably high rate of recurrence.

Outcomes of endoscopic endonasal resection of pediatric craniopharyngiomas.

BACKGROUND: Craniopharyngiomas have traditionally been treated via open transcranial approaches. More recent, endoscopic endonasal approaches have been increasingly used; however, there has been limited evaluation of long-term outcomes for this approach. METHODS: A retrospective review was performed to analyze patients with pediatric craniopharyngioma undergoing endonasal endoscopic resection from 2012 to 2020. Demographic information, clinicopathologic factors, and outcomes including follow-up and recurrences were analyzed. RESULTS: All 42 patients, with a mean age of 8.0 years, were included. The median follow-up time was 49 months. Mean tumor diameter was 3.6 cm. All of the tumors had sellar and suprasellar components. The most common presenting symptoms were headaches (64.3%), visual changes (59.5%), and nausea/vomiting (38.1%). Most patients (73.3%) had resolution of their presenting symptoms by their first postoperative visit. Vision improved or remained normal in 69.0% of patients. Postoperatively, incidence of panhypopituitarism or diabetes insipidus developed in 89.7% and 77.8% of patients, respectively. The postoperative cerebrospinal fluid leak rate was 7.1%. The recurrence rate was 9.5%. CONCLUSION: Endoscopic endonasal resection for pediatric craniopharyngiomas can achieve high rates of resection with low rates of disease recurrence when compared with the outcomes of open transcranial resection reported in the literature. However, hypothalamic-pituitary dysfunction remains a significant postoperative morbidity in both approaches.

Supraglottic Squamous Cell Carcinoma: A Population-Based Study of 22,675 Cases.

OBJECTIVES/HYPOTHESIS: The authors used the Surveillance, Epidemiology, and End Results (SEER) database to analyze epidemiological features of patients presenting with supraglottic squamous cell carcinoma (SCCa) and to evaluate treatment trends and outcomes. METHODS: The SEER database was queried for patients with supraglottic SCCa from 1973 to 2013. Information on demographics; tumor size; histologic grade; American Joint Committee on Cancer (AJCC) stage; SEER local, regional, distant stage; and treatment modality were analyzed. RESULTS: There were 22,675 cases of primary supraglottic SCCa identified. The mean age at diagnosis was 62.3 years, with males accounting for 70.3% of all cases. A high percentage of patients presented with stage IV disease (44.9%). The most common treatment modality was radiotherapy (46.6%), followed by combination of surgery and radiotherapy (29.2%) and surgery alone (15.0%). Overall 5-year disease-specific survival (DSS) for all cases was 54.0%. When stratified by treatment modality, 5-year DSS was best for patients receiving surgery alone (64.2%). However, for patients with AJCC stage IV disease, survival was significantly better with combined surgery and radiotherapy (52.5%). CONCLUSION: In general, supraglottic SCCa is treated most commonly with radiotherapy, followed by surgery and radiotherapy. Patients managed surgically had better 5-year DSS when compared to patients treated by other modalities. However, when stratified by stage, patients with AJCC stage IV disease had significantly better survival with combined surgery and radiotherapy. Of patients receiving surgery, supraglottic laryngectomy was found to have a significantly better 5-year DSS when compared to both total laryngectomy and laryngectomy, not otherwise specified. LEVEL OF EVIDENCE: NA Laryngoscope, 129:1822-1827, 2019.

A Population Based Analysis of Melanoma of the External Ear.

OBJECTIVE: Malignant melanoma accounts for nearly 75% of all skin cancer deaths, and the incidence is on the rise in the United States. External ear melanoma (EEM) is rare, and there is little long-term data regarding the clinical behavior of this melanoma site. This study analyzes the demographic, clinicopathologic, and survival characteristics of EEM. METHODS: The SEER database was queried for EEM cases from 1973 to 2012 (8,982 cases). Data analyzed included patient demographics, incidence trends, and survival outcomes. RESULTS: External ear melanoma occurred most frequently in the sixth and seventh decades of life. Mean age at diagnosis was 65.5 (±16.8) years. However, the incidence of EEM in adolescents and young adults (ages 15-39 yr) has increased by 111.9% from 1973 to 2012. There was a strong male predilection with a male-to-female ratio of 6.40:1. The most common histologic subtype was malignant melanoma, NOS (46.8%), followed by superficial spreading melanoma (21.4%), and lentigo maligna melanoma (17.9%). The majority of cases were localized at the time of presentation (88.0%), with rare distant metastasis (1.9%). The most common treatment modality was surgery alone (97.6%), followed by surgery with radiotherapy (2.3%). Ten-year disease-specific survival was better among those treated with surgery alone (90.7%), than those treated with surgery with radiotherapy (37.1%) (p < 0.0001). Increasing Breslow's thickness and presence of an ulcerating lesion were both associated with poorer survival (p < 0.0001). CONCLUSION: This study represents the largest cohort of EEM. It has an excellent survival outcome with surgery being the treatment of choice.

The role of elective neck dissection in the treatment of maxillary sinus squamous cell carcinoma.

OBJECTIVE: This study is designed to analyze the survival benefits of elective neck dissection (END) in the treatment of squamous cell carcinoma of the maxillary sinus (MS-SCC) with clinically negative neck lymph nodes (N0) and no metastasis (M0). STUDY DESIGN: The aim of this study was to evaluate whether END improves survival in patients with MS-SCC. METHODS: This study is a population-based, concurrent retrospective database analysis of patients diagnosed with N0M0 MS-SCC from 2004 to 2013. Data were acquired from the Surveillance, Epidemiology, and End Results database. Frequency functions, Kaplan-Meier and Cox regression models were queried to analyze demographics, treatment status, and survival outcomes. RESULTS: There were a total of 927 MS-SCC cases in the database between 2004 and 2013. This analysis revealed that for the overall cohort, END significantly and independently reduces the 5-year hazard of death in MS-SCC (hazard ratio [HR] = 0.646, 95% confidence interval [CI] = 0.419-0.873, P = 0.047). For early tumor (T)1/T2 tumors and T4 tumors, END did not independently improve 5-year survival. However, for T3 disease, END significantly reduced the 5-year hazard of death in MS-SCC (HR = 0.471, 95% CI = 0.261-0.680, P = 0.001), regardless of other covariates, including adjuvant radiation. There has been an increase in the percentage of MS-SCC surgeries that have been accompanied by END since SEER started collecting this data, although this did not demonstrate significance (R2 = 0.622). CONCLUSION: END improves disease-specific survival in patients with MS-SCC size > 4 cm and advanced T-stage (American Joint Committee on Cancer AJCC TIII). Therefore, surgeons performing maxillectomies should consider conducting an END concurrent with maxillectomy for those with size > 4 cm advanced stage cancer. LEVEL OF EVIDENCE: 4. Laryngoscope, 1835-1841, 2018.

Utility of intraoperative flexible endoscopy in frontal sinus surgery.

BACKGROUND: Surgical management of the frontal sinus can be challenging. Extensive frontal sinus pneumatization may form a far lateral or supraorbital recess that can be difficult to reach by conventional endoscopic surgical techniques, requiring extended approaches such as the Draf III (or endoscopic modified Lothrop) procedure. Rigid endoscopes may not allow visualization of these lateral limits to ensure full evacuation of the disease process. METHODS: Here we describe the utility of intraoperative flexible endoscopy in two patients with far lateral frontal sinus disease. RESULTS: In both cases, flexible endoscopy allowed confirmation of complete evacuation of pathologic material, thereby obviating more extensive surgical dissection. CONCLUSION: In cases where visualization of the far lateral frontal sinus is inadequate with rigid endoscopes, flexible endoscopy can be used to determine the need for more extensive dissection.

Efficacy of Elective Neck Dissection in T1/T2N0M0 Oral Tongue Squamous Cell Carcinoma: A Population-Based Analysis.

OBJECTIVE/HYPOTHESIS: The aim of this population-based study is to analyze the survival benefits of elective neck dissection (END) over neck observation in T1/T2N0M0 oral tongue squamous cell carcinoma (OT-SCC) cases. STUDY DESIGN: Retrospective administrative database analysis. SUBJECTS AND METHODS: The SEER database (Surveillance, Epidemiology, and End Results) was queried for patients diagnosed with T1/T2N0M0 OT-SCC from 1998 to 2011. Data included patient demographics, initial treatment, and survival outcomes. The Kaplan-Meier model and the Cox proportional hazards model were utilized for survival analysis. RESULTS: Out of 7010 T1/T2N0M0 cases, END was performed in 1770 T1 and 950 T2 cases, and the neck was observed in 3278 T1 and 1001 T2 cases. Significantly poorer 5-year disease-specific survival (DSS) rates were noted for the neck observation group when compared with the END group for tumors with moderately differentiated (72.1% vs 86%, P < .0001) and poorly differentiated or undifferentiated (55.6% vs 71.5%, P = .0001) histologic grades. No significant survival benefit was seen between the END group and the neck observation group when tumor size was <1 cm, regardless of histology grade. However, those with tumors >1 cm had a significantly better 5-year DSS with END, except for the well-differentiated tumor cohort, which showed improved survival only when the tumors were >2 cm (5-year DSS: END vs neck observation, 83.5% vs 65.7%, P = .0002). CONCLUSION: END improves DSS versus neck observation in T1/T2N0M0 OT-SCC patients with moderately differentiated, poorly differentiated, or undifferentiated histologic grade tumors >1 cm. Those with well-differentiated tumors benefited from END only when tumor size was >2 cm.

Impact of Nodal Level Distribution on Survival in Oral Cavity Squamous Cell Carcinoma: A Population-Based Study.

OBJECTIVE: Regional lymph node metastasis is an important prognostic factor in squamous cell carcinoma of the head and neck, decreasing survival by up to 50%. Oral cavity squamous cell carcinoma (OC-SCCa) most commonly spreads to levels I, II, and III. STUDY DESIGN: Retrospective analysis of a population-based tumor registry. SETTING: Academic medical center. SUBJECTS AND METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was queried for cases of OC-SCCa from 2004 to 2011 (22,973 cases). Resulting data including patient demographics, clinicopathological features, topographical distribution of nodal metastasis, and survival based on lymph node level involvement were analyzed. RESULTS: In total, 8281 patients were identified with OC-SCCa who underwent neck dissection. Level I, closely followed by levels II and III, represented the most commonly involved nodal basins. The 5-year disease-specific survival (DSS) for patients with only level I, II, or III was 42.0% compared with 30.6% for the level IV group (P < .0001) and 26.4% for the level V group (P < .0001). Surgery with adjuvant radiotherapy improved 5-year DSS for patients with level I to III, level IV, and level V neck disease compared with surgery alone (50.7% vs 48.6%, P = .0109; 39.9% vs 23.2%, P < .0001; and 33.3% vs 9.1%, P = .0005, for levels I-III, IV, and V, respectively). CONCLUSION: Oral cavity squamous cell carcinoma most commonly involves nodal levels I, II, and III. Involvement of nodal level IV or V portends a worse prognosis than patients with only level I to III disease, and multimodality therapy should be considered for these patients.

Sphenoid sinus malignancies: a population-based comprehensive analysis.

BACKGROUND: Sinonasal malignancies are rare, representing less than 1% of all cancers, with the sphenoid sinus accounting for 1% to 2% of these cases. Sphenoid sinus malignancies exhibit very poor outcomes. There is a paucity of literature describing their histopathological features, incidence trends, treatment, and survival. We seek to elucidate these factors using a national population-based resource. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was used to identify malignant sphenoid sinus tumors. The results were analyzed for demographics, incidence, and clinicopathologic trends. Survival was calculated using Kaplan-Meier analysis. RESULTS: The search identified 472 cases. The mean and median age at diagnosis was 60.0 years. Males represented 54.9% of cases. By race/ethnicity, 82.4% were white and 8.5% were black. The four most common histopathologies were squamous cell neoplasms (29.4%), adenocarcinomas (14.4%), non-Hodgkin's mature B-cell lymphomas (13.1%), and unspecified epithelial neoplasms (11.0%). The overall incidence from 2000 to 2012 was 0.030 per 100,000. Kaplan-Meier analysis demonstrated an overall 5-year disease-specific survival (DSS) of 48.1%. Of the most common histopathological subtypes, 5-year DSS was best for mature B-cell NHL (64.0%) and worst for unspecified epithelial neoplasms (25.6%). CONCLUSION: Sphenoid sinus malignancies are rare, with high prevalence in white males. The most common histopathology is squamous cell neoplasms. They exhibit significant locoregional extension. Of the common sphenoid sinus malignant subtypes, 5-year DSS is best for mature B-cell NHL and worst for unspecified epithelial neoplasms.

Utility of Surgery/Radiotherapy in Distant Metastatic Head and Neck Squamous Cell Carcinoma: A Population-Based Approach.

OBJECTIVES: The aim of this study is to analyze the survival benefits of surgery and/or radiation therapy over no therapy in patients with metastatic (M1) squamous cell carcinoma of the head and neck region (HN-SCC). STUDY DESIGN: Retrospective administrative database analysis. SUBJECTS AND METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was queried for M1 HN-SCC cases from 1988 to 2012 (6663 patients). Patient demographics, initial treatment, and survival outcomes were analyzed. Survival was analyzed with the Kaplan-Meier model. RESULTS: Of the 6663 patients identified with M1 HN-SCC in the SEER database, 1669 patients received no therapy; 2459 patients, radiotherapy; 570 patients, surgery; and 1100 patients, surgery with adjuvant radiotherapy. The mean survival was 8.44 months for patients who did not undergo any therapy. In comparison, patients who underwent radiotherapy alone, surgery alone, or surgery with radiotherapy had mean survivals of 18.03 (P < .0001), 31.07 (P < .0001), and 39.93 (P < .0001) months, respectively. The 5-year disease-specific survival rates were 6.35% for no therapy, 17.51% for radiotherapy alone, 30.50% for surgery alone, and 33.75% for surgery with radiotherapy (P < .0001). Site-specific analysis revealed that surgery and/or radiation provides disease-specific survival benefit as compared with no therapy at all subsites within the head and neck region. CONCLUSIONS: Surgery and/or radiation-which has been shown to improve quality of life in patients with advanced cancer-is associated with an increased survival when utilized in patients with distant metastatic disease.

Sinonasal fibrosarcoma: analysis of the Surveillance, Epidemiology, and End Results database.

BACKGROUND: Primary fibrosarcoma of the sinonasal region is an infrequently occurring malignant neoplasm. Fibrosarcomas are most commonly found in the extremities, with only 1% of fibrosarcomas reported in the head and neck region. This study analyzes the demographic, clinicopathologic, and survival characteristics of sinonasal fibrosarcoma (SNFS). METHODS: The Surveillance, Epidemiology, and End Results (SEER) database (1973 to 2012) was queried for SNFS cases. Data were analyzed with respect to various demographic and clinicopathologic factors. Survival was analyzed using the Kaplan-Meier model. RESULTS: Fifty-one cases of fibrosarcoma were identified in the sinonasal region. The mean age at diagnosis was 54.5 years and the mean survival was 119.7 months. There was no gender predilection with a male-to-female ratio of 1.04:1. The maxillary sinus was the most common site of involvement (54.9%), followed by the nasal cavity (23.5%). Five-year survival analysis revealed an overall survival rate of 71.7%, disease-specific survival rate of 77.8%, and relative survival (RS) rate of 78.8%. Disease-specific survival was better among those treated with surgery (with [76.2%] or without [87.5%] adjuvant radiotherapy) than those treated with primary radiotherapy alone (33.3%) (p = 0.0069). CONCLUSION: SNFS is a rare entity. This study represents the largest series of SNFS to date. The mainstay of treatment for this tumor is surgical resection with or without radiotherapy.

Squamous cell carcinoma of the maxillary sinus: A population-based analysis.

OBJECTIVES/HYPOTHESIS: Squamous cell carcinoma (SCC) accounts for > 90% of head and neck cancers and 60% to 75% of malignancies of the paranasal sinuses. The most commonly affected paranasal sinus is the maxillary. Epidemiologic, incidence, and survival trends have been studied for maxillary sinus SCC (MSSCC), but far less is known about its metastatic potential. STUDY DESIGN: Retrospective database analysis. METHODS: The Surveillance, Epidemiology, and End Results database was used to extract frequency, incidence, and survival data for MSSCC between 2004 and 2012. The resultant cases were stratified according to patient demographics and collaborative stage information, including extent of disease, lymph node involvement, TNM staging, and regional and distant metastasis. RESULTS: A total of 854 cases of MSSCC were identified. The mean age at diagnosis was 66.6 years, with 87.4% presenting at > 50 years. Most patients (65.1%) were male. Whites accounted for 74.6% of cases. A majority (64.3%) of cases presented with stage IV disease. Overall 5-year disease-specific survival was 23.4%. Neck involvement was seen in 7.6% of T1 tumors, 22.2% of T2 tumors, 18.5% of T3 tumors, and 12.2% of T4 tumors. Distant metastasis was not seen in T1-T3 tumors, but was present in 6.8% of T4 tumors. CONCLUSIONS: MSSCC is a rare entity with poor overall prognosis. The majority of patients included in this study were white males aged ≥50 years, with most tumors presenting at advanced stages. Nodal involvement and distant metastasis are poor prognostic indicators. T1-T3 tumors did not metastasize to distant sites. LEVEL OF EVIDENCE: 4.

A population-based analysis of Head and Neck hemangiopericytoma.

OBJECTIVES/HYPOTHESIS: Hemangiopericytomas (HPC) are tumors that arise from pericytes. Hemangiopericytomas of the head and neck are rare and occur both extracranially and intracranially. This study analyzes the demographic, clinicopathologic, treatment modalities, and survival characteristics of extracranial head and neck hemangiopericytomas (HN-HPC) and compares them to HPCs at other body sites (Other-HPC). METHODS: The Surveillance, Epidemiology, and End Results (SEER) database (1973-2012) was queried for HN-HPC (121 cases) and Other-HPC (510 cases). Data were analyzed comparatively with respect to various demographic and clinicopathologic factors. Disease-specific survival (DSS) was analyzed using the Kaplan-Meier model. RESULTS: There was no significant difference in age at time of diagnosis between HN-HPC and Other-HPC. Head and neck HPC was most commonly located in the connective and soft tissue (18.4%), followed by the nasal cavity and paranasal sinuses (8.5%). Head and neck HPCs were smaller than Other-HPC (P < 0.0001) and more likely to be a lower histologic grade (P < 0.0097). The primary treatment modality for HN-HPC was surgery alone, used in 55.8% of cases. The 5-, 10-, and 20-year DSS for HN-HPC were 84.0%, 79.4%, and 69.4%, respectfully. Higher histologic grade and the presence of distant metastases were poor prognostic factors for HN-HPC. CONCLUSION: Head and neck HPCs are rare tumors. This study represents the largest series of HN-HPCs to date. Surgery alone is the primary treatment modality for HN-HPC, with a favorable prognosis. Adjuvant radiotherapy does not appear to confer a survival benefit for any body site. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:643-650, 2016.

Subglottic Squamous Cell Carcinoma: A Population-Based Study of 889 Cases.

OBJECTIVE: Subglottic squamous cell carcinoma (SCCa) is a rare malignancy representing <5% of all laryngeal cancers. Patients often present with late-stage disease, and survival outcomes are reportedly worse than those for SCCa in other regions of the larynx. STUDY DESIGN: Analysis of a population-based tumor registry. SETTING: Academic medical center. SUBJECTS AND METHODS: The US National Cancer Institute's Surveillance, Epidemiology, and End Results database was queried for cases of subglottic SCCa from 1973 to 2011 (889 cases). Resulting data were analyzed, including patient demographics, therapeutic measures, and survival outcomes. RESULTS: Subglottic SCCa most frequently occurred in the fifth to seventh decade of life, with a mean age at diagnosis of 65.7 ± 11.3 years. There was a strong male predilection, with a male:female ratio of 3.83:1. Most patients were stage III and IV (64.4%) per the American Joint Committee on Cancer. The most common treatment modality was a combination of radiotherapy and surgery (38.8%), followed by radiotherapy alone (33.9%), and surgery alone (17.0%). Overall 5-year disease-specific survival rate was 53.7%. When stratified by treatment modality, 5-year disease-specific survival was 62.4% for surgery alone, 56.7% for radiotherapy alone, and 55.1% for surgery with adjuvant radiotherapy (P = .3892). CONCLUSION: This study represents the largest cohort of subglottic SCCa. It shows a strong predilection for men in the US population. Surgery with adjuvant radiotherapy was the most commonly employed treatment modality. No statistically significant differences were observed in 5-year DSS by treatment modality.

Squamous cell carcinoma of the nasal cavity: A population-based analysis.

OBJECTIVES/HYPOTHESIS: Squamous cell carcinoma of the nasal cavity (NCSCC) is an infrequent malignancy that has been historically difficult to characterize. This study provides new insight into NCSCC utilizing a population-based database. We analyze the propensity for cervical and distant metastasis from NCSCC, as well as survival outcomes. STUDY DESIGN: Retrospective database analysis. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database (2004-2012) was queried for NCSCC cases. Data were analyzed with respect to various demographic and clinicopathologic factors. The results were further examined for regional and distant metastasis. Survival was analyzed using the Kaplan-Meier model. RESULTS: A total of 1,180 cases of NCSCC were identified in the SEER database between 2004 and 2012. The mean age at diagnosis was 65.8 years. American Joint Committee on Cancer stage was known in 1,050 cases, of which 53.4% were stage I, 13.3% were stage II, 10.2% were stage III, and 23.0% were stage IV. By tumor (T) stage classification, T1 was the most common (56.6%), followed by T4 (19.3%). Most cases had no nodal (N) involvement at diagnosis (90.8%). Cervical nodal involvement was present in 9.1% of cases, while distant metastasis was seen in 1.9%. Five-year disease-specific survival was 69.5% overall, 39.6% in cases with neck involvement and 0.0% for metastatic cases. CONCLUSIONS: This study represents the only known population-based investigation of NCSCC. Metastasis to cervical nodes or distant sites, especially with T1 tumors, is rare. However, any cervical involvement or distant metastasis discovered on presentation is a poor prognostic indicator. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:560-565, 2016.

Comparative Analysis of Head and Neck and Non-Head and Neck Malignant Peripheral Nerve Sheath Tumors.

OBJECTIVES: Malignant peripheral nerve sheath tumors (MPNSTs) are a group of tumors that arise from peripheral nerves or from the various elements of the nerve sheath, including Schwann cells and perineural fibroblasts. Head and neck MPNSTs (HN-MPNSTs) are rare, accounting for 8% to 16% of all soft tissue sarcomas. This study analyzes the demographic, clinicopathologic, and survival characteristics of HN-MPNSTs and establishes comparisons with MPNSTs at other body sites (other-MPNSTs). STUDY DESIGN: Analysis of population-based tumor registry. SETTING: Academic medical center. SUBJECTS AND METHODS: The SEER database (Surveillance, Epidemiology, and End Results; 1973-2012) was queried for HN-MPNSTs (324 cases) and other-MPNSTs (1680 cases). Data were analyzed comparatively with respect to various demographic and clinicopathologic factors. Disease-specific survival was analyzed with the Kaplan-Meier model. RESULTS: Mean age at diagnosis for HN-MPNST was 49.1 years, compared with 46.1 years for other-MPNSTs (P = .0169). There was a sex predilection for males in HN-MPNSTs (60.2%) versus a female predilection for other-MPNSTs (54.2%; P < .0001). Average tumor size for HN-MPNSTs was 4.9 cm, compared with 8.7 cm for other-MPNSTs (P < .0001). HN-MPNSTs were more commonly of low histologic grade types, whereas other-MPNSTs were mostly of high histologic grade (P = .0073). HN-MPNSTs had a higher 5-year disease-specific survival than other-MPNSTs (65.1% vs 57.4%; P = .0209). CONCLUSIONS: HN-MPNSTs are rare entities. This study represents the largest series of HN-MPNSTs to date. Although HN-MPNSTs and other-MPNSTs share a common histology, there are important clinical differences between the 2 groups.

Cost-Effectiveness Analysis of Microscopic and Endoscopic Transsphenoidal Surgery Versus Medical Therapy in the Management of Microprolactinoma in the United States.

BACKGROUND: Although prolactinomas are treated effectively with dopamine agonists, some have proposed curative surgical resection for select cases of microprolactinomas to avoid life-long medical therapy. We performed a cost-effectiveness analysis comparing transsphenoidal surgery (either microsurgical or endoscopic) and medical therapy (either bromocriptine or cabergoline) with decision analysis modeling. METHODS: A 2-armed decision tree was created with TreeAge Pro Suite 2012 to compare upfront transsphenoidal surgery versus medical therapy. The economic perspective was that of the health care third-party payer. On the basis of a literature review, we assigned plausible distributions for costs and utilities to each potential outcome, taking into account medical and surgical costs and complications. Base-case analysis, sensitivity analysis, and Monte Carlo simulations were performed to determine the cost-effectiveness of each strategy at 5-year and 10-year time horizons. RESULTS: In the base-case scenario, microscopic transsphenoidal surgery was the most cost-effective option at 5 years from the time of diagnosis; however, by the 10-year time horizon, endoscopic transsphenoidal surgery became the most cost-effective option. At both time horizons, medical therapy (both bromocriptine and cabergoline) were found to be more costly and less effective than transsphenoidal surgery (i.e., the medical arm was dominated by the surgical arm in this model). Two-way sensitivity analysis demonstrated that endoscopic resection would be the most cost-effective strategy if the cure rate from endoscopic surgery was greater than 90% and the complication rate was less than 1%. Monte Carlo simulation was performed for endoscopic surgery versus microscopic surgery at both time horizons. This analysis produced an incremental cost-effectiveness ratio of $80,235 per quality-adjusted life years at 5 years and $40,737 per quality-adjusted life years at 10 years, implying that with increasing time intervals, endoscopic transsphenoidal surgery is the more cost-effective treatment strategy. CONCLUSIONS: On the basis of the results of our model, transsphenoidal surgical resection of microprolactinomas, either microsurgical or endoscopic, appears to be more cost-effective than life-long medical therapy in young patients with life expectancy greater than 10 years. We caution that surgical resection for microprolactinomas be performed only in select cases by experienced pituitary surgeons at high-volume centers with high biochemical cure rates and low complication rates.