Programmed Death-Ligand 1 Expression and Tumor-Infiltrating Lymphocytes in Temporal Bone Squamous Cell Carcinoma.

OBJECTIVES/HYPOTHESIS: The tumor immune microenvironment in temporal bone squamous cell carcinoma (TBSCC), including the programmed death-ligand 1 (PD-L1) expression and tumor-infiltrating lymphocytes (TILs), has not been established. STUDY DESIGN: Retrospective cohort study. METHODS: We performed immunohistochemistry analyses to retrospectively analyze 123 TBSCC cases for PD-L1 expression and TILs and their prognostic significance. We also evaluated the prognostic correlations between these immunomarkers and the therapeutic responses to chemoradiotherapy (CRT). RESULTS: PD-L1 expression (≥1%) was detected in 62 (50.4%) TBSCC cases and was significantly associated with worse prognosis: progression-free survival (PFS), P < .0001; overall survival (OS), P = .0009. A high density of CD8+ TILs was significantly associated with better prognosis (PFS, P = .0012; OS, P = .0120). In contrast, a high density of Foxp3+ TILs tended to be associated with an unfavorable prognosis (PFS, P = .0148; OS, P = .0850). With regard to the tumor microenvironment subtypes defined by CD8+ TILs and PD-L1 expression, the CD8low /PD-L1+ group showed significantly worse prognosis. Among the 36 neoadjuvant CRT-treated cases, PD-L1 expression was significantly associated with worse OS (P = .0132). Among the 32 CRT-treated cases without surgery, a high density of CD8+ TILs tended to be more highly associated with complete response to CRT compared to a low density of CD8+ TILs (P = .0702). CONCLUSIONS: These results indicate that the evaluation of the tumor immune microenvironment may contribute to the prediction of prognoses and the selection of an individualized therapeutic strategy for patients with TBSCC. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:2674-2683, 2021.

Primary Advanced Squamous Cell Carcinoma of the Temporal Bone: A Single-Center Clinical Study.

OBJECTIVES/HYPOTHESIS: The extreme rarity of temporal bone squamous cell carcinoma (TB-SCC) has delayed the accumulation of high-quality clinical evidence. For the purposes of retrospective meta-analysis in the future, a large dataset with information from various institutions would be ideal. Our objective here was to retrospectively review cases of TB-SCC encountered at a single tertiary referral center and explore survival outcomes and prognostic factors. STUDY DESIGN: Retrospective chart review. METHODS: The medical records of all TB-SCC cases were retrospectively reviewed. The resulting dataset contained 71 cases of primary cancer eligible for initial definitive (curative) treatment. RESULTS: T4 status was associated with lower disease-specific 5-year survival than T1 to T3 staging (T1: 100%, T2: 92%, T3: 86%, T4: 51%). Survival was significantly higher in operable than in inoperable cases, even when restricted to advanced (T3/T4) cancers. The tumor extension to the middle ear cavity was observed in 13/17 of T3 cases, but it was not associated with poor survival. In addition, among operable cases, negative surgical margins were associated with significantly higher survival than positive margins. CONCLUSIONS: Definitive treatments can offer disease-specific 5-year survival of over 85% in T1 to T3 cases of TB-SCC. The tumor extension to the middle ear cavity is not associated with poor survival. T4 status, inoperability, nodal invasion, and positive surgical margin are identified as a predictor of poor prognosis. Still, the matter of how to deal with unresectable tumors remains an outstanding issue in the treatment of TB-SCC. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E583-E589, 2021.

Therapeutic effect of Nivolumab for advanced / recurrent temporal bone squamous cell carcinoma.

OBJECTIVE: The immune checkpoint inhibitor Nivolumab was approved for the treatment of platinum-refractory head and neck squamous cell carcinoma (SCC), expanding the treatment options for recurrent or advanced head and neck SCC. However, since temporal bone squamous cell carcinoma (TB-SCC) is very rare cancer, the effectiveness of Nivolumab remains unclear. We investigated the effects of Nivolumab for TB-SCC. METHOD: Chart information was collected for all patients who underwent the first administration of Nivolumab for recurrent or residual TB-SCC in our hospital between September 2017 and December 2019. Tumor staging followed the modified Pittsburgh classification. Changes in the tumor burden and survival outcome were examined. RESULTS: We examined 9 patients with recurrent or residual TB-SCC who started administration of Nivolumab. In these cases, recurrent or residual SCC was observed after chemotherapy and/or chemoradiotherapy including platinum. The duration of Nivolumab was 2-54 weeks (median 20.0 weeks). The evaluation of the therapeutic effect according to the RECIST method showed partial response in 1 case, stable disease in 2 cases, progressive disease in 4 cases, and size unevaluated in 2 case. Although the number of cases was small, comparing with 5 cases without Nivolumab, these cases showed longer overall survival (1-year OS 33.3% vs 20.0%). CONCLUSION: We used Nivolumab as palliative chemotherapy in 9 patients with recurrent/residual TB-SCC, and we were able to obtain a certain therapeutic effect on TB-SCC as well as other head and neck SCC.

Management of cranial chondroblastoma in adults; a pooled analysis.

OBJECTIVES: Chondroblastoma is a rare bone tumour that often affects children more than adults. The adult cranial affection is a unique presentation of the disease (less than 2%) that mandates specific management. Through this pooled-analysis, we aimed to examine this disease group and obtain reliable scientific conclusions. METHODS: The literature was searched for case-reports of cranial chondroblastoma. Afterwards, a pooled analysis was done for the selected articles. The data in question were the patient's age, gender, disease site, modalities of treatment, disease control, salvage treatment, and disease-specific survival. RESULTS: 122 case reports were retrieved from the literature. The median age at diagnosis was 38.5 years. Up to the moment, the standard of care remains complete resection. The relapse-free survival was 29.5 months, with a three-year relapse rate of 9% and disease-specific mortality of 0%. Most of the relapses were salvaged excellently with excision. The strongest predictor for relapse was the post-resection residual disease, with three-years relapse rates of 23.8% and 2.4% for positive and negative residuals, respectively. Adjuvant Radiotherapy failed to improve the relapse rates. CONCLUSION: Complete surgical excision is the standard of care. Post-surgical residual is the only adverse prognostic factor that predicts poor disease control.

An individual patient data meta-analysis on the effect of chemotherapy on survival in patients with craniofacial osteosarcoma.

Chemotherapy improves the survival of patients with long bone osteosarcomas. However, the benefits of chemotherapy in the treatment of craniofacial osteosarcoma (CFOS) are still controversial. We searched PubMed and EMBASE from February 1997 to December 2016 to identify studies on CFOS. The individual patient data of these studies were pooled into a meta-analysis. Univariate and multivariate survival analyses were performed. Thirteen studies with a total of 184 patients met our inclusion criteria. Positive resection margin was a poor prognostic factor for CFOS in the univariate and multivariate survival analyses. Chemotherapy improved overall survival (OS) and disease-specific survival (DSS) in patients with CFOS who had tumors in the maxilla, positive resection margins, or high-grade tumors. Patients with local tumor recurrence had better OS and DSS when treated with chemotherapy. Chemotherapy improves survival in patients with CFOS with adverse factors, such as tumors with positive margins, high-grade tumors, and recurrent tumors.

Outcomes and prognostic factors in thyroid cancer patients with cranial metastases: A retrospective cohort study of 4,683 patients.

BACKGROUND: Cranial metastasis of thyroid cancer is rare. The aim of this study was to analyse the clinical characteristics, treatments and outcomes of thyroid cancer patients with cranial metastasis and to identify the associated prognostic factors. MATERIALS AND METHODS: Between January 1977 and August 2017, a total of 4683 patients were histologically confirmed to have thyroid cancer. Among them, 25 patients (0.53%) were identified as having cranial metastases, and their medical records were reviewed. The Kaplan-Meier method with a log-rank test was performed with cancer-specific survival as the main outcome. Cox regression analysis was used to examine the potential prognostic factors influencing patient survival. RESULTS: Of the 25 patients, 21 were female, and 4 were male. The median age at the time of diagnosis of cranial metastasis was 63 years. Sixteen patients had metastases to the brain, and nine patients had metastases involving the skull only. Papillary carcinoma and follicular carcinoma accounted for 84.0% of cases. Twenty-four cases (96.0%) had extracranial metastases at the time of diagnosis of cranial metastases. Twenty patients received surgery, and 4 patients received palliative radiotherapy. One patient received supportive care only. The median cancer-specific survival after the diagnosis of cranial metastases was 27 months. According to the Kaplan-Meier test, 3 factors had a significant impact on survival, the metastatic site, histological types and surgical resection. According to the Cox regression analysis, skull metastases (HR: 0.274, 95% CI: 0.083-0.904, p = 0.033) and surgical resection (HR: 0.134, 95% CI: 0.019-0.929, p = 0.042) were identified as independent prognostic factors for a better outcome. CONCLUSIONS: Surgical resection is the mainstay therapy for thyroid cancer patients with cranial metastasis. Cranial metastases involving the skull only are associated with a better outcome.

Ewing sarcoma of the head and neck: The Mayo Clinic experience.

BACKGROUND: Treatment options of Ewing sarcoma of the head and neck include surgery, radiotherapy (RT), and chemoradiotherapy. However, local control can be challenging. METHODS: We conducted a retrospective review of all patients with head and neck Ewing sarcoma treated from 1972 to 2015 at a single tertiary care hospital. RESULTS: Seventeen patients met criteria (median 21 years, range 5-58 years; 5 women). Mean follow-up was 10.4 years (range 2.2-39 years). Tumors occurred commonly in the cervical spine (5/17), the skull (3/17), and the paranasal sinuses (3/17). A total of 14 of 17 patients underwent surgical resection, 9 with gross total resection. After multimodality therapy, the 5-year overall survival (OS) and recurrence-free survival (RFS) was 87% and 75%, respectively. CONCLUSION: Combined multimodal treatment resulted in a 5-year OS and RFS of 87% and 75%, respectively. Aggressive surgical resection with adjuvant chemoradiotherapy should be considered. Although negative margin surgery is the goal, subtotal resection may be acceptable in the setting of adjuvant treatment.

Long-term Outcomes After Radiosurgery for Temporal Bone Paragangliomas.

OBJECTIVES: To determine the long-term outcome after stereotactic radiosurgery (SRS) for temporal bone paragangliomas. MATERIALS AND METHODS: We retrospectively reviewed the medical records of 11 patients with temporal bone paragangliomas (10 patients with a glomus jugulare tumor and 1 patient with a glomus tympanicum tumor) treated between January 1997 and July 2012 at the University of Florida with SRS to a median dose of 15 Gy in 1 fraction. Ten previously unirradiated patients received SRS as did 1 patient who received prior fractionated radiotherapy (FRT) and then received salvage SRS for a local recurrence. The major outcome endpoint was local control, meaning no further growth or shrinkage on follow-up computed tomography or magnetic resonance imaging scans. RESULTS: The median follow-up time was 5.3 years. Two patients developed a local recurrence after SRS, including the patient who received salvage SRS after prior FRT. The overall local control rates at 5 and 10 years were both 81%. The cause-specific survival rates at 5 and 10 years were both 88%. The distant metastasis-free survival rates at 5 and 10 years were both 100%. The overall survival rates at 5 and 10 years were both 78%. There were no severe complications. CONCLUSIONS: SRS for benign head and neck paragangliomas is a safe and efficacious treatment associated with minimal morbidity. SRS is suitable for patients with skull base tumors <3 cm when FRT is logistically unsuitable. Surgery is reserved for patients in good health whose risk of associated morbidity is low. Observation is a reasonable option for asymptomatic patients with a limited life expectancy.

Prognostic Factors and Survival Outcome in Patients with Chordoma in the United States: A Population-Based Analysis.

OBJECTIVE: To evaluate prognostic factors of patients with chordoma through a population-based analysis. METHODS: Surveillance, Epidemiology, and End Results (SEER) database was queried for patients with chordoma from 1973 to 2013. Kaplan-Meier univariate analysis and Cox regression multivariate analysis were performed to examine prognostic factors in overall survival (OS) and disease-specific survival (DSS). RESULTS: One thousand five hundred ninety-eight patients with chordoma are identified. Kaplan-Meier analysis showed that OS and DSS were 61% and 71% at 5 years and 41% and 57% at 10 years. Multivariate Cox regression analysis demonstrated that independent predictors of OS and DSS are age at diagnosis (hazard ratio [HR]= 2.80 [95% confidence interval {CI}, 2.12-3.70], P < 0.001; HR = 1.60 [95% CI, 1.18-2.16], P = 0.002), surgical treatment (HR = 0.62 [95% CI, 0.52-0.73], P < 0.001; HR = 0.64 [95% CI, 0.52-0.79], P < 0.001), radiation therapy (HR = 1.23 [95% CI, 1.07-1.42], P = 0.004; HR = 1.29 [95% CI, 1.09-1.54], P = 0.004), tumor size (HR = 1.53 [95% CI, 1.32-1.78], P < 0.001; HR = 1.62 [95% CI, 1.35-1.94], P < 0.001) and distant metastasis (HR = 3.40 [95% CI, 2.45-4.71], P < 0.001; HR = 3.77 [95% CI, 2.61-5.45], P < 0.001). CONCLUSION: We report the largest study to date to evaluate prognostic factors of patients with chordoma. Multivariate analysis demonstrated that older age, greater tumor size, and distant metastasis were correlated with decreased survival, whereas surgical resection was correlated with increased survival. Patients receiving radiation therapy also showed decreased survival, likely an indication of the patients' advanced stage of disease, making them poor surgical candidates.

Secondary Craniofacial Sarcomas Following Retinoblastoma: A Systematic Review.

OBJECTIVE: We conducted the largest systematic review of individual patient data to characterize secondary craniofacial sarcomas following retinoblastoma. METHODS: We conducted a systemic search of the PubMed databases and compiled a comprehensive literature review. Student t tests were used to evaluate differences between variables. Kaplan-Meier analysis was used to estimate survival. Statistical significance was assessed using a log-rank test. RESULTS: We analyzed 220 cases of secondary craniofacial sarcomas, including 112 osteosarcomas. The average age (±SD) of onset for retinoblastoma was 1.20 ± 2.77 years. External-beam radiotherapy was delivered in 207 patients (94.0%) and chemotherapy was delivered in 53 patients (24.0%) patients. The latency period between retinoblastoma diagnosis and the onset of secondary sarcomas was 12 years. Cranial extension was found in 66 patients (30.0%). The median overall survival was worse with cranial extension (P = 0.0073). In cranial extended patients, the median survival in patients who received chemotherapy was 41 months, whereas patients who did not receive chemotherapy had a median survival of 12 months (P = 0.0020). CONCLUSIONS: The risk of incidence of secondary sarcomas in retinoblastoma patients warrants longer follow-up periods. Moreover, chemotherapy should be considered as a potential treatment option for secondary cranial sarcomas following retinoblastoma.

Multicenter experiences in temporal bone cancer surgery based on 89 cases.

OBJECTIVE: To present outcomes of extensive surgery: lateral, subtotal, total petrosectomies in patients with temporal bone invasion resulting from specific primary cancers. STUDY DESIGN: Retrospective case review. SETTING: Four tertiary referral centers. MATERIAL: 89 patients with cancer of the temporal bone treated between January 2006 and December 2010. INTERVENTION: Multidisciplinary team approach including surgical resection, reconstruction, and postoperative radiotherapy. MAIN OUTCOME MEASURE: Disease-specific survival, overall survival. RESULTS: In 27.0% of the patients, relapse was reported, with an average of 6.3 months after surgery; 31 patients (34.8%) died during the follow-up. The average mortality was 22.1 months. Fifty-four patients (58.7%) stayed alive during the time of observation. The average survival time was 42.0 months. The median time of survival with relapse was 12 months (range: 1-51 months). The three-year disease-free rate was 38.0% and the overall survival rate was 58.7%. CONCLUSIONS: Petrosectomy is an effective treatment for malignant temporal bone invasion. The probability of a good outcome was statistically decreased with a high T grade, positive margins, and salvage surgery. Younger age is connected with better prognosis. One of the major tasks remains to improve detection and to shorten the time to diagnosis, keeping in mind that symptoms are insidious and in younger people, the time before diagnosis was longer.

Prognostic value of the seventh AJCC/UICC TNM classification of intestinal-type ethmoid adenocarcinoma: Systematic review and risk prediction model.

BACKGROUND: The purpose of this study was to propose a prognostic classification of intestinal-type adenocarcinoma (ITAC) based on literature search and prognostic modeling of cohort data. METHODS: We first conducted a literature search to assess the homogeneity of the reported estimates of 5-year survival and to identify the influence of T classification. We then pooled prospective data from 3 large French and Italian series to predict time to all-cause mortality. The sample was randomly split to derive and then to validate the proposed prognostic model. RESULTS: Literature analysis confirmed the heterogeneity in 5-year survival rates, partly explained in subsets of homogeneous T-values. The sample included 223 patients, randomly separated into a derivation (n = 141) and a validation set (n = 82). Invasion of the sphenoid lateral and/or posterior walls and dura/cerebral invasion were systematically associated with a poor survival. CONCLUSION: The incorporation of the invasion of the sphenoid lateral or posterior walls should be considered for ITAC management and prognostication. © 2017 Wiley Periodicals, Inc. Head Neck 39: 668-678, 2017.

Survival impact of local extension sites in surgically treated patients with temporal bone squamous cell carcinoma.

OBJECTIVES: Temporal bone squamous cell carcinoma (TSCC) is a rare malignancy. Due to its low incidence rate, studies involving TSCC treatment are limited. The aim of this study is to define the prognostic factors of surgery for TSCC by evaluating our clinical experience. METHODS: We reviewed the clinical charts of patients presenting at the University of Tokyo Hospital between 2001 and 2014 and identified 33 patients with TSCC who had been treated with surgery as initial curative treatment. RESULTS: Lateral and subtotal temporal bone resections were performed in 17 and 16 patients, respectively. The 5-year disease-specific and overall survival rate were 71 and 62%, respectively. The significant poor prognostic factors were pathological T4 (P = 0.03), dural invasion (P = 0.008), temporomandibular joint invasion (P = 0.04), and a positive surgical margin (P = 0.009). CONCLUSION: We demonstrated that the outcome of curative surgery for TSCC as initial treatment was favorable. However, because of the difficulty to ensure an adequate or clear surgical margin due to anatomical complexity, the surgical indication for T4 TSCC with temporomandibular joint invasion should be reconsidered.

Combined approaches to the skull base for intracranial extension of tumors via perineural spread can improve patient outcomes.

Many neoplasms of the head and neck extend centripetally, gaining access to the central nervous system via nerves through the skull base foramina. Often patients with perineural spread have been excluded from aggressive interventions given the overall poor prognosis and technical difficulty when addressing the perineural components. However, in carefully selected patients combined surgical approaches can provide the greatest potential for disease control as well as neural decompression for symptom relief. We performed a retrospective chart review of 20 consecutive patients who underwent skull base approaches for resection of tumors with intracranial extension via perineural spread from 2011 to 2014. Patients were evaluated for symptom change, surgical approaches, histopathology, adjuvant therapy, outcome, and prognosis. The most common presenting symptoms were pain or cranial nerve palsies. 55% of patients underwent endoscopic endonasal approaches, 50% transcranial approaches, and 15% underwent transfacial approaches. Overall 85% of patients reported symptom improvement in the post-operative period while 40% were completely asymptomatic following surgical resection. Ultimately, we observed a 45% mortality rate with an average survival of 8 months after diagnosis. In carefully selected patients, an aggressive multidisciplinary approach using a combination of surgical avenues to the skull base for the treatment of intracranial tumor via perineural extension can improve patient quality of life.

Cutaneous squamous cell carcinoma of the scalp in the immunocompromised patient: review of 53 cases.

OBJECTIVE: We analyzed outcomes from immunocompromised patients with scalp squamous cell carcinoma (SCC) treated with surgical excision with and without radiation in order to explore 3-year disease-free survival and overall survival. STUDY DESIGN: The study design was a retrospective chart review. SETTING: This study was conducted in a private practice setting. SUBJECTS AND METHODS: The study included 53 immunocompromised patients with an average age of 63.2 years, with scalp squamous cell carcinoma. Pre-operative imaging dictated the extent of resection. Patients with bony involvement received wide local excision including full-thickness craniectomy and cranioplasty. Patients without bony involvement underwent wide local excision and outer-table calvarial resection. All patients were recommended to have post-operative radiation. Patients were followed for a minimum of 3 years. RESULTS: A total of 53 patients were included in the study. Six patients had pre-operative CT showing bone involvement and were treated with full-thickness craniectomy along with post-operative radiation. Fourteen patients without bone involvement on pre-operative CT were found to have positive bone involvement on final pathology. Forty-five patients underwent post-operative radiation. Patients treated with adjuvant radiation demonstrated a 3-year survival of 80 % and the overall survival was 62 %. In the surgery-only group, the 3-year survival was 62.5 % and the overall survival was 32.5 %. CONCLUSIONS: Immunocompromised patients with scalp SCC have a poor prognosis. Early detection and treatment are crucial. Based on our results, we recommend wide local excision with at least outer-table calvarial resection, and post-operative radiation. Despite aggressive therapy, patients may still have distant, local, or regional recurrence. LEVEL OF EVIDENCE: level 2b (retrospective cohort).

Primary extradural meningiomas in head: a report of 19 cases and review of literature.

Primary extradural meningiomas (PEMs) in head were rare tumors. Here we analyzed 231 cases of PEMs in head (including our 19 cases) reported in the literature during the CT era. We found that PEMs in head accounted for 0.8%-1.8% of all meningiomas. The constituent ratio of male PEMs in head increases markedly. PEMs in head have bimodal distribution of ages. The most common presenting symptom was a mass in the region of the lesion. The average duration of symptom was 2.38 years. The skull convexities, paranasal sinuse and nasal cavity, and middle ear ranked as the top three of all sites of tumors. The most common type was Type II (calvarial or diploic). Among 231 cases, total, subtotal and partial removals of tumors were achieved in 89%, 5.5% and 3.1% respectively, and no death occurred perioperatively in all patients. 90% were benign, 5.6% atypical and 3.9% malignant in the 231 cases. The most common histopathological subtype was meningothelial meningioma. The recurrence and tumor-related death rates were 22.4% and 8.2% respectively during a mean 3.03-year follow-up. Our results demonstrate that PEMs in head have some marked clinical characteristics compared with primary intradural meningiomas. Total tumor removal together with a wide excision of all involved tissues followed by the reconstruction of tissue defects is the best surgical project. The prognoses are good in the benign cases after complete surgical resections.

Recurrent squamous cell carcinoma of the temporal bone: critical analysis of cases with a poor prognosis.

PURPOSE: Temporal bone squamous cell carcinoma (TBSCC) is an uncommon, aggressive malignancy with a significant recurrence rate. We reviewed our experience with recurrent TBSCCs. MATERIALS AND METHODS: Clinicopathological and therapeutic variables potentially associated with disease-free survival (DFS) and disease-specific survival (DSS) were assessed in 17 TBSCC patients who died of their disease after treatment. RESULTS: TBSCC recurrences were treated with surgery in 12 cases (palliative in 11, with curative intent in 1) and palliative chemotherapy in 5; the median DFS and DSS were 6 and 16 months, respectively. The mean DFS and DSS were longer in patients who had primary lateral temporal bone resection (LTBR) rather than subtotal temporal bone resection (STBR) (p=0.0173 and p=0.03, respectively). Patients given non-surgical palliative treatment for recurrences had a longer mean DSS than those who underwent surgery (trend toward significance, p=0.09). CONCLUSIONS: Our results reflect the aggressive nature of TBSCC recurrences. Our findings seem to support the use of non-surgical treatments (chemotherapy, radiotherapy, or specialist palliative care) in patients with loco-regionally advanced recurrent TBSCC. Salvage surgery might be considered for early recurrences when radicality is still achievable. Precise guidelines for the rational follow-up of surgically-treated TBSCCs need to be shared between tertiary centers.

Image-guided, intensity-modulated radiation therapy (IG-IMRT) for skull base chordoma and chondrosarcoma: preliminary outcomes.

BACKGROUND: We report our preliminary outcomes following high-dose image-guided intensity modulated radiotherapy (IG-IMRT) for skull base chordoma and chondrosarcoma. METHODS: Forty-two consecutive IG-IMRT patients, with either skull base chordoma (n = 24) or chondrosarcoma (n = 18) treated between August 2001 and December 2012 were reviewed. The median follow-up was 36 months (range, 3-90 mo) in the chordoma cohort, and 67 months (range, 15-125) in the chondrosarcoma cohort. Initial surgery included biopsy (7% of patients), subtotal resection (57% of patients), and gross total resection (36% of patients). The median IG-IMRT total doses in the chondrosarcoma and chordoma cohorts were 70 Gy and 76 Gy, respectively, delivered with 2 Gy/fraction. RESULTS: For the chordoma and chondrosarcoma cohorts, the 5-year overall survival and local control rates were 85.6% and 65.3%, and 87.8% and 88.1%, respectively. In total, 10 patients progressed locally: 8 were chordoma patients and 2 chondrosarcoma patients. Both chondrosarcoma failures were in higher-grade tumors (grades 2 and 3). None of the 8 patients with grade 1 chondrosarcoma failed, with a median follow-up of 77 months (range, 34-125). There were 8 radiation-induced late effects-the most significant was a radiation-induced secondary malignancy occurring 6.7 years following IG-IMRT. Gross total resection and age were predictors of local control in the chordoma and chondrosarcoma patients, respectively. CONCLUSIONS: We report favorable survival, local control and adverse event rates following high dose IG-IMRT. Further follow-up is needed to confirm long-term efficacy.

The role of radiotherapy and intrathecal CNS prophylaxis in extralymphatic craniofacial aggressive B-cell lymphomas.

To define the role of radiotherapy and intrathecal prophylaxis in extralymphatic craniofacial involvement (ECFI) of aggressive B-cell lymphoma, we analyzed 11 consecutive German High-Grade Non-Hodgkin Lymphoma Study Group trials. ECFI occurred in 290/4155 (7.0%) patients (orbita, 31; paranasal sinuses, 93; main nasal cavity, 38; tongue, 27; remaining oral cavity, 99; salivary glands, 54). In a multivariable analysis adjusted for International Prognostic Index rituximab improved event-free and overall survival both in patients with and without ECFI. Three-year event-free (79% vs 79%; P = .842) and overall survival (86% vs 88%; P = .351) rates were similar in 145 patients receiving and 57 not receiving radiotherapy. Without rituximab, the 2-year cumulative rate of central nervous system (CNS) disease was increased in 205 ECFI patients compared with 2586 non-ECFI patients (4.2% vs 2.8%; P = .038), whereas this was not observed with rituximab (1.6% in 83 ECFI vs 3.4% in 1252 non-ECFI patients; P = .682). In 88 ECFI patients who received intrathecal prophylaxis with methotrexate, the 2-year rate of CNS disease was 4.2% compared with 2.3% in 191 patients who did not (P = .981). In conclusion, rituximab eliminates the increased risk for CNS disease in patients with ECFI. This retrospective analysis does not support intrathecal prophylaxis or radiotherapy to ECFI patients in complete remission/unconfirmed complete remission. These findings should be confirmed in a prospective study.

Temporal bone carcinoma. Current diagnostic, therapeutic, and prognostic concepts.

Temporal bone carcinoma is an uncommon aggressive malignancy. Its low incidence and the absence of a globally accepted staging system still make it difficult to compare different centers' approaches and results. In this review of the main available studies dealing with temporal bone carcinoma since 1995, we consider its rational preoperative staging and assessment, compare the effectiveness of different treatments by tumor stage, and outline the main actuarial prognostic factors.